Peds- acute infectious emergencies; hepatology; congenital; diarrhea; GI; abuse

Down syndrome

-trisomy 21 -certain features= syndrome Dx/ syx in older (4 or 5 yrs): -hypotonia, tendency to keep mouth open, mental retardation, bachycephaly (cranial looks flat), microencephaolapthy (below the 3rd percentile on chart (note that 95th percentile at like 2 or 4 months can be bc tumor or hydrocephalus); head circumference is smaller), late fontanelle closure so can be 2 years and still open.; ** In eyes you get speckling of iris (Brushfield spots), fine lens opacities. Kids with Down syndrome have glasses.-hearing problems -hypoplastic of teeth -short fingers -single crease or simian crease -40% will have endocardia cushion defects. Easily operatable but have to dx early!! -males: smaller junk & females infertile Dx/ syx in neonates: -hypotonia -poor Moro reflex (when they come out and back in a ball) -hyperflexibility of joints -simian crease- crease that goes all the way around the palm of hand-40% of time -excess neck skin -flat facial profile -low set ears -clenodactyly- pinky finger is curved Progression: -muscle tone gets better with age. Developmental exam: gross motor exam. Fine motor, speech, personal & social. -motor delay & global delay -Snore a lot so airway obstruction -slow, friendly, lie music, mimicry ***Major cause for early mortality is congenital heart defects (endocardial cushion defects) -survival after 1st year is like 60% So it's pretty high -Many have atlantoaxial dislocation & with neck injury they'd be paralysis. Scenario) Down syndrome kid wants sports clearance. Get a x-ray Types: -Full trisomy (they have all the features) -Mosaicism (2.4%): Some cells (20% of cells) have trisomy but 80% cells are normal -translocation (D to G or G to G)- paternally translated, so not all of this is bc mom -faulty chromosomal distribution in Down syndrome more likely to occur in older age -Down syndrome rates with maternal age: 15 - 29 yrs. / 1 in 1,500 30 - 34 yrs. / 1 in 800 35 - 39 yrs. / 1 in 270 40 - 44 yrs. / 1 in 100 over 45 yrs. / 1 in 50 -reoccurrence of Down syndrome is 1% Dx- carrier type -charts can help dx disorders. Can dx turner's if person is super short

What syndrome will you see butterflies on an x ray?

Alagille disease

Answers to neonatal (1, 2, 3)

Answers and Explanations 1. The answer is C [XII.A.3]. Esophageal atresia in a newborn is characterized by increased oral secretions as a result of the accumulation of saliva in the proximal esophageal pouch. Respiratory distress may occur if the infant aspirates this saliva. The presence of a distal tracheoesophageal fistula may also result in the passage of gastric contents to the trachea and lung, exacerbating the respiratory problem. Half of children with esophageal atresia have other congenital malformations, such as congenital heart disease. Both pneumonia and persistent pulmonary hypertension of the newborn also present with respiratory distress, but without increased oral secretions. Respiratory distress syndrome occurs less commonly in term infants, and increased oral secretions are not expected. Congenital diaphragmatic hernia usually presents with acute respiratory distress soon after birth in a newborn with a scaphoid abdomen. Bowel sounds can be heard on auscultation of the chest. 2. The answer is B [I.G.6 and I.H.1.b]. The most likely cause of an abdominal mass detected during the newborn period is of renal origin, with hydronephrosis being the most common cause. In female infants, an ovarian cyst, which is usually a benign tumor, is not as common as hydronephrosis. Wilms tumor and multicystic kidneys may present as abdominal masses but are also less common causes. Hydrometrocolpos, a retention of vaginal secretions, most commonly presents just after birth as a small cyst located between the labia, although during childhood, it may present as a lower midline abdominal mass. 3. The answer is A [X.C.1]. Breastfeeding jaundice is typically associated with indirect, or 162 unconjugated, hyperbilirubinemia and is caused by suboptimal milk intake during the first week of life, which causes weight loss, poor hydration, and decreased stool output. The treatment of breastfeeding jaundice is hydration, which typically includes increasing the frequency of breastfeeding, along with observation and serial bilirubin assessments. Breast milk jaundice, which occurs later, after the first week of life, is thought to be associated with high levels of lipase and β-glucuronidase within breast milk. Choledochal cysts, biliary atresia, and neonatal hepatitis are more typical causes of direct, or conjugated, hyperbilirubinemia. 4. The answer is B [XII.C]. Omphalocele is more frequently associated with congenital malformations, such as congenital heart defects, and with genetic conditions, such as trisomy 13, and less commonly with trisomy 18, but not with trisomy 21. Omphalocele and gastroschisis are easily distinguished and diagnosed by inspection. An omphalocele occurs centrally through the umbilical ring, whereas gastroschisis is a lateral abdominal wall defect in which the abdominal contents herniate into the amniotic cavity and are directly exposed to amniotic fluid. Because of this difference in clinical presentation, both omphalocele and gastroschisis are diagnosed clinically without the need for radiographic confirmation. In gastroschisis, exposure to the amniotic fluid may cause inflammation of the bowel with subsequent bowel damage and risk of bowel obstruction. 5. The answer is E [VIII.A, VIII.C, and VIII.E]. One of the most common causes of persistent pulmonary hypertension of the newborn (PPHN) is perinatal asphyxia, resulting in increased pulmonary vascular resistance and significant right-to-left shunting through the foramen ovale or the ductus arteriosus. Oxygen is the most potent vasodilator of pulmonary vessels, and in most cases, increases of both alveolar and arterial partial pressures of O2 produce a decrease in pulmonary vascular resistance and reversal of low blood flow to the lungs. By definition, PPHN excludes the presence of congenital heart disease. If left untreated, the hypoxemia caused by PPHN worsens the increased pulmonary vascular resistance, resulting in many cases in irreversible disease and death. In addition, PPHN occurs most commonly in near-term and full-term infants, as well as in postterm infants. 6. The answer is B [VI.A-H]. Respiratory distress syndrome (RDS), which is most common in premature male infants, is caused by a lack or deficiency of surfactant, with alveolar atelectasis and hypoventilation. Chest radiographic findings usually include a diffuse ground glass pattern with air bronchograms. Pneumonia and sepsis should always be included in the differential diagnosis of RDS because their clinical presentations may be quite similar. Persistent pulmonary hypertension of the newborn (PPHN) is more common in term infants than in premature infants and results most frequently from perinatal asphyxia and meconium aspiration syndrome (MAS). Fluid retention in the lungs may cause respiratory distress, but it is usually mild. The chest radiograph usually shows normal or increased lung volume with increased vascular markings. Bronchopulmonary dysplasia (BPD) is a chronic complication of RDS. Some causes of cyanotic congenital heart disease may cause hypoxemia and respiratory distress after birth; however, the chest radiograph does not show a ground glass appearance, nor air bronchograms. 7. The answer is A [X.A-G]. Newborn infants who breastfeed have higher peak serum bilirubin a markedly elevated direct bilirubin level is consistent with a choledochal cyst, a disorder that causes obstruction of the biliary tree. Both breastfeeding and breast milk jaundice are characterized by indirect, not direct, hyperbilirubinemia. Crigler-Najjar syndrome, or hereditary deficiency of glucuronyl transferase, would also be expected to result in an indirect, or unconjugated, hyperbilirubinemia. ABO incompatibility would lead to hemolysis, leading to an elevation of indirect bilirubin. 10. The answer is B [XII.E]. Necrotizing enterocolitis (NEC) is one of the most common surgical 163 values. However, hyperbilirubinemia alone is not a reason to discontinue breastfeeding. Bilirubin encephalopathy is caused only by unconjugated (indirect) bilirubin because of the ability of unconjugated bilirubin to cross the blood-brain barrier. Encephalopathy caused by indirect hyperbilirubinemia does occur in healthy term newborns, and for this reason, high bilirubin levels in this group of infants should not be ignored. Benign physiologic indirect hyperbilirubinemia is expected to peak in term infants at 3-4 days of life and in preterm infants at 5-7 days of life. 8. The answer is D [I.K.11]. Nevus flammeus or "port wine stain" located over the V-1 branch of the trigeminal nerve may herald Sturge-Weber syndrome, with its associated, and potentially very significant, underlying intracranial vascular malformations and calcifications. Pustular melanosis is a benign rash, characterized by small, dry vesicles over a dark macular base, more frequently seen in African American infants. Nevus simplex is the most common vascular lesion of infancy and is also completely benign and often transient, appearing as a "salmon patch" or "stork bite" on the nape of the neck. Milia are benign very small cysts formed around the pilosebaceous follicles that appear as tiny whitish papules over the nose, cheeks, forehead, and chin. Erythema toxicum neonatorum (ETN) is a benign rash usually present in the first 72 hours of life and seen in approximately 50% of all infants. ETN is characterized by erythematous macules, papules, or pustules on the trunk and extremities. 9. The answer is E [Figures 4-3 and 4-4]. This infant's presentation with hyperbilirubinemia and conditions in neonates, occurring most commonly in premature infants. Clinical features include abdominal distension, abdominal tenderness, residual gastric contents, bilious vomiting or bilious nasogastric aspirate, bloody stools, and, at times, abdominal wall erythema. Classic radiographic findings include abdominal distension, air-fluid levels, thickened bowel walls, and pneumatosis intestinalis (air within the bowel wall). In contrast, the double-bubble sign on abdominal radiographs is pathognomonic of duodenal atresia, which classically presents with nonbilious emesis and abdominal distension, but not with bloody stools. The presence of a soap-bubble appearance on abdominal radiographs is characteristic of meconium ileus, a presentation of cystic fibrosis during the neonatal period. Infants with meconium ileus would not be expected to pass bloody stools on the third day of life, but may require pancreatic enzyme supplementation if they are ultimately diagnosed with fat malabsorption and cystic fibrosis. There is no known association between Down syndrome and NEC, although there is an association between Down syndrome and duodenal atresia. 11. The answers are E and B, respectively [Table 4.1]. The Apgar scoring system provides a simple, systematic, and objective assessment of intrapartum stress and neurologic depression. The female newborn earns 2 points for a heart rate >100 beats/minute, 1 point for slow and irregular respirations, 1 point for having some flexion of the extremities, 1 point for reflex irritability or grimace when a catheter is placed in her nose, and 1 point for her peripheral cyanosis or acrocyanosis, for a total Apgar score of 6 points. The male newborn earns 1 point for a heart rate <100 beats/minute, 1 point for slow and irregular respirations, 1 point for having some flexion of the extremities, 0 points for the absence of reflex irritability when a catheter is placed into his nose, and 0 points for his cyanosis, for a total Apgar score of 3 points. 12. The answer is A [IV.D.2 and V.A] The 100% oxygen test helps distinguish whether cyanosis is caused by cardiac or respiratory disease. When administered 100% oxygen, infants with primary lung pathology, such as neonatal pneumonia, meconium aspiration syndrome, transient tachypnea of the newborn or respiratory distress syndrome, have a very significant increase in PaO2 levels. In contrast, patients with cyanotic congenital heart disease would not be expected to have such a significant rise in their PaO2 level. For example, patients with Tetralogy of Fallot, a cyanotic congenital heart disease associated with reduced pulmonary blood flow, would not be expected to respond with any increase of significance in the PaO2 level when given 100% oxygen. Note that other cyanotic congenital heart diseases with normal or increased pulmonary blood flow, like truncus arteriosus, may have some increase in the PaO2 level, but nowhere near as much of an increase as that seen in infants with primary pulmonary disease.

Symptoms of Hepatitis/ importance of liver

-Hypoglycemia bc normally it helps with glucose metabolism -helps break down toxins -if liver didn't break stuff down you could get build up of toxic ammonia bc you get ammonia production from breaking down proteins -hyperammonia in blood can go to the brain and can cause coma -circulation in liver (hepatic) vs circulation in kidney (renal): portal circulation!! Blood from GI tract comes into liver via the portal vein. The portal vein brings in metabolites, toxins —-> if the pressure goes up here, you can get portal hypertension

Antibiotic associated diarrhea differential dx

-be V careful!! -could be clostridium difficule colitis -could be pseudomembrane colitis -if you don't stop antibiotics, & keep giving them when they have C dif...... It'll cause perforation. C dif toxin test comes back in 2 hrs. if it's pos, stop antibiotics. Treat with flagel or metronidazole. STOP ANTIBIOTIC.

Rocky Mountain spotted fever

-differential dx for meningococcal bc both have petechial rash and both are toxic. And Abs are V different so have to know difference -other dx: other ticke borne diseases like Lyme -went camping or hiking -fever, myalgia, body ache, sore throat, fatigue, abdominal pain, arthralgia, myalgia, rash (petechial), fever -pulmonary edema or periorbital edema not coming from the heart!! -photophobia, seizures, irritability, petechial rash -***KEY in lab: electrolytes: Hyponeutremia Tx: doxycycline even if it's a young child.......

Fragile X Syndrome (Martin-Bell Syndrome)

-on X you have trinucelotide repeats (50 of them) -syx: autism, crooked jaw, orchidism, pragmatism (jaw protruding), bigger testicles, conduct disorder -normally, you should ahve less than 50 CG repeats, but here you can have more than 200 CG repeats -bc of conduct disorder, 5% of incracinated ppl have fragile X syndrome

Glucose-galactose malabsorption

V rare -malabsorption of glucose and galactose -transporter deficiency

Look at a child and be able to judge gestational age of child based on jubelle??

-1 (sticky, friable skin)= pre-term to 5 (leathery wrinkled skin)= post-term -normal baby=1 which has skin that's smooth and pink -lanugo (pre-term and post-term are both bald). But normal baby has abundant lanugo -plantar surface- look at creases. The more pre-term, the less creases there are -Breast- normal = no bud -eye- normal- little recoil & curved= 1 -testes- in pre mature baby might not have descended yet. In post-term, the testes and rugae are a lot -pre-term baby you'll just see the clit & vaginal opening, but normal and more post-term baby you'll see more fat around labia majora

Hep B

-1.25 million people in US are carriers. If woo ppl screened in NY 2/100 would be carrier for hepatitis B. -biggest problem in other countries can be perinatal and childhood— infant transmission, But in US and Canada it's sexual and needles. US biggest problem is needles!! Dx: -Dx: hepatitis B viral DNA to test for chronic infection or not. If hep B viral DNA is persistent, it's chronic -Dx: have Hb surface antigen for usually 6 months. More than 10^5 DNA. Persistent or intermittent elevated AST & ALT. Liver biopsy: if necroinflammatory > 4, then it's chronic hepatitis. -Dx: inactive carrier state: people who are positive for Hb surface S antigen for greater than 6 months BUT everything else is neg. So not a lot of HBV DNA and normal AST/ALT levels. Necroinflamm is normal. -Dx: resolved hep B: Hb surface antigen is negative. No HBV DNA. Normal ALT Other manifestations: -serum sickness -polyaterteris nodosa -glomemlonephritis in adults!! Defs consider hep B or C -popular acrodermatitis -aplastic anemia (always think underlying hep b) Sequels of hep B: -chronic active —> cirrhosis —> portal hypertension -confection with Hep B & C -hepatocellular carcinoma (7th most common cancer worldwide) Vaccine: -hep B vaccine prevents hepatocellular carcinoma Hep B vaccine- mandated!! Every child has to get 1st dose of Hep B within 48 hrs. 2nd dose b/w 1-2 months. Third dose b/w 6 and 18 months of age. -If a mother has hep B OR if we don't know mom's hep B status, we give baby Hep B immunoglobulin in 24 hrs and the hep B vaccine. -new recommendation: if baby born with hep B mom, do everything the same but recheck tigers at 9 months and revaccinate if needed -Tx: no tx really. Can suppress replication. Antiviral: Lamivudine. IFN-alpha. Nothing great tho. Very expensive

Celiac disease— incidence, what specific marker can you look for?? This is the most sensitive marker for making dx of celiac disease

-AKA gluten sensitivity enteropathy -used to be Scandinavian but everywhere now -autoimmune -2 presentations: in kids as chronic diarrhea or older teens/ adults -syx) recurrent abdominal pain, diarrhea, autoimmune condition so may see it with other autoimmune - -incidence: 1/133 -to dx used to do biopsy but now can use marker called anti tissue transglutaminase antibody IgA. If this marker is high, you have celiac disease ***always on boards -Scalaping on biopsy -slide: villous blunting= celiac disease. Villous not finger like bc of all the inflammation.

Jaundice- pg 150 in BRS -breast milk jaundice

-ANy jaundice in the first 24 hrs of life is never normal (it's NOT physiologic jaundice) -physiological jaundice is always indirect/ unconjugated -visible jaundice in neonate is when serum bilirubin is above 5 -it'll peak on 3rd day of life. Peaks a lil slower in pre-term -breast milk jaundice- increased fat and you're reabsorbing more bilirubin. Get higher levels of indirect bilirubin. Rarely > 20%. At 20 and over, you get irreversible damage in the brain because that much of unconjugated bilirubin can pass through BBB and kills neural cells. This affects basal ganglia and BRAINSTEM nuclei. -TX: discontinue breast feeding and eventually you can resume after 24 hrs and it's fine! -Rapid early tx of that high of bilirubin levels can prevent kernicterus!! It starts with hypotonia and ends with eye muscle paralysis and hearing paralysis. Increased arched back. Permanent neurological damage. Yellow staining of brain by unconjugated.

Meningitis

-Bacterial meningitis- not as much with vaccines. If you do tap and see pus, you know it's bacterial. When pus heals, there's gonna be some obstruction. Can have hydrocephalus and deafness after so make sure you give steroids here!! If baby who doesn't have closed sutures, check head circumference -viral meningitis- enteroviral meningitis seen every year. -Tx: ampicillin bc need to cover for listeria, group B strep, & E. coli, steph, strap. -older kids: H flu, Neisseria meningitidis, Strep pneumonia -Younger kids: Group B strep, E. coli, staph aureus -warning: when you have increased ICP, and do a spinal tap, what can happen? Draining fluid with ICP —-> herniation and die... some kids you need to CT scan and make sure there's not a shift of ventricles. -in meningitis: use 3/4 of starting fluid. Make sure they aren't hypoglycemia or acidosis. Make sure no DIC TX: support all systems. Don't fluid overload. If we don't know viral or bacterial start both: Acyclovir & Vancomyson F/U: hearing F/U!! Bc of high deaf incidence Biggest late complication: hydrocephalus, CN, mental retardation -specific meningitis: meningococcal meningitis: routinely vaccinate: MenVeo: age 11 & booster at age 16. Men B: age 16 & booster 4 weeks later—- Men B is not mandatory bc not V common; more prevalent in oceans. Risk: tight places like college. If you're traveling to endemic country can do earlier -prophylaxis: Rifampin, ceftriaxone, si[profloxacin if >18 y/o. Azithromycin in resistant organisms. -meningococcemia- very high mortality. Gangrene and can have above knee amputation. thrombocytopenia. Purpura rash Lekocytosis in white count. CSF: high white count, cell count, polynuclear count BUT glucose is low bc organisms are eating the glucose! Shock is a big thing. Course: very fast and mortality is very high

Diarrhea, why's this more common in younger kids? What's chronic diarrhea and what are the causes? -what's excessive fermentation mean?

-Bc infant has so much liquid!! So even just a little GI motility will cause diarrhea -Chronic diarrhea- more than 2 weeks Causes: 1) digestion impaired in lumen: A) cystic fibrosis- not making enough enzymes that come into duodenum so you have lack of digestion B) bile interruption like in biliary atresia. Not enough bile coming in to GI tract; you need bile to absorb fats. Lack of bile, will cause malabsorption of fats 2)Malabsorption- probs with villi -celiac disease; chrohns, UC are V important -post enteritis syndrome- after baby has rotavirus, microvilli have been destroyed and need time to recover. When you give baby milk, it doesn't have microvilli to process milk. lactose intolerance. Gradually reintroduce cheese after GI infection -giardiasis- Protozoa that causes malabsorption of fats -immunodeficiency- more prone to infections -a beta lipoproteinemia- malabsorption of fat -lymphangiectasia- malabsorption of fat -excessive fermentation is lactase deficiency— can be congenital or late-onset

Botulism

-Bioterrorism or farming -inhalation or iatrogenic - acute, Flaccid, descending, symmetric paralysis, but HERE you have cranial nerve involvement & no senssory involvement -scenario: baby: floppy baby. They don't feed well. Cry & voice is weak. Ptosis or eye dropping. Maybe no gag reflex. May have respiratory probs. -Think differential dx: spinal muscular atrophy, myasthenia Gravis, duscenne or UMN -Dx: clostridium found in stool -Tx: AVOID AMINOGLYCOSIDES bc it enhances the effects -Gram + bacteria can survive boiling temps (heat labile & sturdy toxins) -2-4 mo of age= peak -we're type B and mississippi is type A-can come from -sauteed onions, potatoses, canned foods canm get it from there -Avoid honey, wash everything well. Wash objects well. -Irreversible toxin. Blocks Ach release. You can't reverse binding!! You have to wait it out

Role of intestinal biopsy: what would you see for celiac, a beta lipoproteinemia, chrohns, & lactase deficiency

-Celiac disease- you would see villous/ partial villous atrophy -A beta lipoproteinemia- you'd see fat filled enterocytes -crohn's- intense inflammation Lactase deficiency- normal biopsy! Microvilli you'd have to see under electron microscopy

Rashes: diaper dermatitis vs. candida diper dermatitis vs staph diaper dermatitis

-Diaper dermatitis- **spares the intertriginous areas (the folding areas) -involves convex surfaces -goes into lower abdomen tooo -Tx: local hygiene, topical steroids, aquafor!! -candida diaper dermatitis -rash is worse in the creases bc -maculopapular rashes even in places far away from the actual rash! Might be one on the leg or arm -don't scape these lesions!! It won't have the actual fungi in it! -Tx: anti-fungal creams. Steroids can make this worse!!! If it's super inflamed can use some steroids -staphylococcal diaper dermatitis -may have small or large lesions. Color of scales around it like impetigo -can get combo! Fungi and bacterial

Encephalitis vs meningitis vs meningoencephalitis

-Encephalitis- inflammation of brain -meningitis- inflammation of meninges -meningoencephalitis- inflammation of brain and meninges

Deletions of short arm (4p, 5p, 6p -)

-Hypertelerism- if you can place another eye between the eyes, they have this -microcephalic -low ear set -very typical cry like a cat! Goes away after 6 mo

Face & body rash: milia

-Milia- obstruction of sweat glands retain vesicles. They can look like white papules but Do NOT mistake for pustules!! Do NOT give antibiotics. resolves on its own. -can have miliaria rubra (red) or miliaria crystallina (white crystal like) -Miliaria: white yellow papules seen mostly on face in newborn. On central mid face of baby. DOn't do anything; it's normal! Mongolian spots- normal! Esp in African Americans. Slate-grey lesion over the lumbosacral area of black infant. It should go away in a year (2 or 3) -Erythema Toxicum Neonatorum- it appears very early. Central rash. Primarily on the TRUNK/ some extremities but Not rlly on palms and soles. It occupies large areas. It has raised and red papules. There might be a lot of postules looking things in it. **If you do a culture (Gram or wreight stain), you won't find any bacteria! You'll find only eosinophils in lesions— giveaway that it's erythema toxicum neonatorum. No tx needed -Transient Neonatal Pustular Melanosis (TNPM)- Transient! Pustular phase initially but in a couple of hrs, the pustules are gone! The top flap is gone and you're left with a pigmented lesion. If you culture, you get lots of PMNs and no bacteria -sebaceous gland hyperplasia & neonatal acne- newborn can have neonatal acne and even bleeding and stuff bc of mom's hormones. Do NOT use acne treatment. No intervention needed. -Cutis Marmorata- problem if baby is withdrawing from drugs. Neonatal abstinence syndrome. In the beginning, it's not a problem. Down syndromes have this and it'll persist -hemangiomas- 10-40% of babies have it. Strawberry hemangiomas. Cavernous hemangioma. Port wine stain- big patch covering skin. -some hemangiomas can eat platelets and you can get thrombocytopenia -Nevis simplex (pr "salmon patch") in newborns, you can get a specific spot on forehead/ upper eye lid/ nape of neck ("stork bite") -Nevis flammeus or "port wine stain" can become darker with age. If these are located in trigeminal V1 branch area tho... think sturge Weber -sturge Webber- if you have hemangiomas midline, close to the face, can be assoc with seizures -Nevi- can have predisposition to cancer. Dermatologists track this throughout their life.

Scenario) unusual small bruises over bony prominences in babies— should you suspect abuse? When might you suspect abuse? Are bite marks sus? -what should you make sure you rule out? -what types of burns are sus? -what do you see in oral injuries?

-No, it's normal to have bruises over knees & shins -It's NOT normal to have bruises under the scapula on the back -Bite marks are always SUS -make sure you rule out platelet disorders! Why you need to order labs -sus burns- if they say it's from splashing oil but the distribution is more stocking glove distribution with sharp demarcated lines -branding injury (small circle can indicate cigarette) -immersion scalds most common -oral injuries: if you're trying to force bottle in mouth while the baby is really crying you can tear the frenulum

Complications in premature babies

-PDA- in normal weight babies they close by themselves but in pre-me baby who is already struggling with breathing, they do not tolerate PDA well. We close it. -Pulmonary interstitial emphysema (PIE)- if there's fluid or air b/w, and you're forcing air in there, now you have oxygen rich air in alveoli. Blood flowing through alveoli but oxygen can't get back in blood and CO2 can't get back in alveoli. You need to increase pressure to force oxygen out into endothelial lining. But increased pressure worsens PIE. -pneumothorax- small can resolve but large an collapse lung. Sometimes it can go to heart and mediastinum= pneumopericardium & pneumoperiosteum -Bronchopulmonary Dysplasia (BPD)- they've been on oxygen and pressure for awhile and get scarring in bronchi -retinopathy of prematurity- damage of retina from too much oxygen!! FiO2 kept to keep stats b/w 95-99%. Not 100!!

Giardia -if you have a kid with recurrent Giardia infections, consider they might have.....

-Protozoa infection -scenario) abdominal pain. -pos stool o & p -if you have a kid with recurrent Giardia infections, consider they might have .... IgA defiency!!

Bone- osteomyelitis & septic arthritis

-Syx: V nonspecific in newborn. That's why you squeeze every limb and if baby starts crying- you know there's pain in the bone there. -Syx: Older child . Initially it's in cortex, break the periosteum and then spread everywhere. -the starting point- nidus- progresses rapidly. If close to joint, it can go in joint and cause septic arthritis. Of middle in shin, it can rupture and affect nearby muscles. -x ray: may initially be normal but eventually periosteal lifting & inflammation, Early on, need bone scan **Most common bone involved- femur, then tibia, then humerus. "keep the FAITH"- Femur, Tibia, Humerus Most common bug in neonates & older children- staph aureus (used to be H flu but not anymore) -Under 3- kingella is actually the most common bug that causes this, but any older than 3, it's staph aureus -specific scenarios: -teen hitting someone in face and breaking your skin with their teeth- most common infection: anaerobes— very high chance to get osteomyelitis with anaerobes- make sure antibiotic covers anaerobes!! -with cat and dog bite: antibiotic should cover for pasturella -when you step on a nail & get osteomyelitis/ septic arthritis- have to cover for pseudomonas -sickle cell- cover for salmonella -diabetic- cover for fungal -think fungal infections if talking about vertebral body, skull, jaw Types of arthritis dx by joint fluid analysis: 1) septic arthritis- cartilage. it forms clots. Very high white counts, leukocytosis. Polymorphonuclear cells. Kosher criteria for septic arthritis (4 questions): 1a) is child weight bearing 1b) does child have high acute phase reactance like is ESR high (more than 40)? 1c) Is white count more than 12,000? 1d) Is there fever more than 38.5 C or 101.3 F? grade: yes (1) or no (0). prob 4/4= 99%. 3/4= 93%. 2/4= 40%. 1/4= 0% VS 2) Reactive arthritis- ropy, stringy, mostly lymphocytic. Not that much cell count VS 3) Autoimmune arthritis like idiopathic- white count is high but not that high. Glucose is fine. Not so much cell count. Most common stuff in different ages: -older ages: broadly asked most common bacteria & bone: staph & femur. Remember STDs like gonococcal arthritis -younger ages: toddler group specifically: kingella kingae. Not just staph but CA-MRSA (community associated methicillin resistant staph aureus). Septic arthritis associated with long-time catheterizxation like IV lines Things associated with reactive arthritis: -STDs in older kids like gonococcal arthritis ***Reiter's syndrome from chlamydia infection- triad syndrome: "can't see, can't pee, can't climb a tree"= conjunctivitis, urethritis, arthritis -younger kids: catheters or IV -Lyme disease- assoc with septic arthritis -Kawasaki disease- assoc with septic arthritis

Edwards syndrome

-Trisomy 18 -very feeble cry so usually have to be resuscitated -clenched hands -prominent occiput -50% die within one week -low dermal ridges -most die within 1 year and really don't live past 10 yrs

IgA defiency

-V common -secretory IgA 1/300 -most common primary immunodeficiency -more prone to get giardia -high incidence of chronic diarrhea -can ahve severe anaphylactic reactions to blood products! -GI malignancy

Turner syndrome

-XO female -if female pt is below the 3rd percentile in height, think turners! -broad chest, widely spaced nipples -renal: horseshoe kidney -heart: Bicuspid aortic valve OR coarctition of aorta **** COMPARE to Down syndrome where you have endocardial cushion defects -hearing impairment -web neck -IQ: about 90 -if they're mosaic for turners, 80% cells are normal & 20% are turner's -scenario- pt comes bc fertility issues or can't get her period— could be turner's mosaics! Infertility just depends on if the fertility stuff affected in the 20%

Infant of substance abusing mother (ISAM)

-all babies will have some probs but some babies don't go into full withdrawal. MRIs and CTs are normal so can't see damage but functional damage is there. These kids may not have seizures when born but may have intellectual disabilities like ADHD later on. -syx in kids: spitting up, inconsolable. -monitoring: finnegan scoring system mainly for opioids so it has limitations but still used.

Eosinophilic gastroenteropathy... he skipped it...

-allergy to cow milk -Increased IgE levels -eosinophilia -reversible after elimination

NAFLD (non-alcoholic fatty liver disease)

-asymptomatic but elevated AST & ALT -symmtptomatic: fatigue, Right upper quadrant pain, metabolic syndrome or Type II diabetes

How to test for gastroespageal reflux:

-barium swallow. Give baby barium to swallow and look to see if barium comes up at all on scan. You get false negatives 20% of time bc you might not have reflux at 9 am but you do have at later on at night. NOT gold standard test. This helps you figure out if there is gastric outlet obstruction (problem in pyloris or duodenum) -esophageal 24 pH study- at top of feeding tube it measures pH. Put it in esophagus and it'll show you how much acid is there. Helps you quantify how long these episodes are and when reflux is getting worse and with event correlation. Event correlation= when trying to correlate turning blue with the reflux. Mom hits the event marker button when baby turns blue. If you have event correlation, you can say that the reflux is what caused the episode of turning blue -endoscopy- GOLD STANDARD. Lower esophageal biopsy -milk scintiscan- use is child has recurrent pneumonia. If you want to figure out if aspiration is causing the pneumonia. feed baby milk, use gamma ray scan., and there should be no lung activity. 4 hrs later, if you find the tracer in the lungs, that means they have aspirated the milk

GERD gastroesophageal reflux

-doesn't have to be a disease -baby spitting up can just be reflux which is normal. If they're going weight it's okay -if baby is failing to thrive (not gaining weight), that's a problem! -make sure they aren't turning blue! If they are, it's an ALTERNATIVE (acute life threatening episode) -need to intervene if they aren't gaining weight (failure to thrive) or turning blue -teens might have persistent vomiting and hematasis (puking out blood) -sandifer syndrome- bc of altered tone in lower esophageal sphincter -asthma- make sure they're using it right. If they don't have good control of asthma you always ask if they have associated gastroesophageal reflux! Recurrent wheezing, rule out gastroesphageal reflux -rule out recurrent pneumonia (the aspirations may look like reflux) -Don't know how many SIDS cases are bc of reflux (back to back!!) -in older kids, reflux can present as heart burn. Worse at night. You're laying down so stomach acids come up (why baby also gets reflux bc they're always laying down) -saliva is basic. Acts like a buffer. We make less saliva at night!! -sponataneous swallowing in daytime- don't have that at night

APGAR score

-for respiration, it's too hard to look at chest going up and down so for respiration you measure it based on crying! -color: if baby has pink body and blue extremities this is a 1!! But this is NORMAL -if baby is pink ALL over then give it a 2. -most babies will have some degree of cyanosis and that's normal **Know how to calcualate APGAR score -we do at it birth, and then 5 minutes, and then 10 minutes -APGAR scores can guide your resuscitation effort methodology -we used to think low APGAR scores were indicative of future successfulness, but not true. V resilient

Constipation

-functional constipation- most common cause of constipation in kids. Mom brings kids in and it really hurts. They want to hold the poop. Change the diet and make sure there's more fiber. Can use gentle laxative like oil or Mirilax. -anatomical constipation causes- V rare. Hirschprungs disease- the last 3 cm of rectum hav no ganglion cells, it can't relax. It will stay spammed or closed. To dx this you can do barium and you'll see a "rat tail" and have to do biopsy. Tx: always surgery. If you put bulb in his Hep rungs, the pressure won't fall, it might stay the same! = lack of receptive relaxation. If biopsy has no ganglion cells, you can confirm hirschprung's disease. -Tx: always surgical!

causes of hepatitis

-in teenagers it's infectious mononucleosis caused by EBV, hep A, B, and C= most common reason!! -can be caused by metabolic disorders like Williams disease, hemochromatosis, alpha one antitrypsin deficiency, or NAFLD -neonatal hepatitis & obstructive cholangiopathy

Things you ask yourself when baby comes out: -what's the indicating for respiratory support and what do you do? -What's indication for chest compressions?

-is the baby to term? -what is the tone? -Is the baby crying/ breathing? -if baby isn't crying, rub their back and suction the secretion (with a bulb syringe) -DO we suction the mouth or nose first?? -answer: the mouth just bc there is more fluid there -check breathing & HR -breathing can check by feeling pulsatations in the cord or auscultation -syx of respiratory distress: blue, grunting, retracting, flaring, gasping -we don't start with 100% oxygen!! Don't push over 95%. We start them based on their stats. Takes 10 minutes to come to 90%. Bc too much oxygen can cause oxidative damage -if you're heart rate is less than 100, start with respiratory support, which is positive pressure ventilation and monitor their sats. IF you start doing ventilation and HR is still less than 100, make sure they're EFFECTIVE ventilations, make sure airway is clear, put towels under the baby's shoulder blades to extend baby's head a little back. Make sure their airway is not kinked airway/ no obstruction -if HR goes below 60, start chest compressions (compress with 2 fingers, NOT on diploid process, depth: 1.5 in or 4 cm OR u can put both hands on ack and thumbs touching lower part of sternum= better way) -if consistently below 60, can give them epi

Life threatening congenital anomalies you should check for within 1 hour after birth: -choanal atresia -Pierre robin syndrome -diaphragmatic hernia -tracheoesophageal fistula

-look at pic! 1) Choanal atresia- the nasal airways aren't completely patent. Membrane is blocking it so to check, you just run a tube up there! -example scenario where this wasn't performed and baby comes in to see doc bc baby has compensated by the nose blockage by breathing through their mouth BUT when you feed them, they can no longer breathe through their mouth! 2) Pierre robin syndrome- V small jaw. Hardly have a chin and chin is set backwards. Tongue is normal size and it obstructs airway. Micrognathia, airway obstruction. Need to lift jaw up to provide patent airway 3) diaphragmatic hernia- part of your stomach is in your chest. The more positive ventilation you give, the worse the respiratory distress gets 4) tracheoesophageal fistula- usually won't catch this at first bc they present with recurrent aspiration pneumonia -Gi issue -neuro issue -cardio issue

Myocarditis

-more viral!! -cocksachie, echo, polio -morbidity from influenza comes bc of myocarditis -Rocky Mountain spotted fever -Syx: really SOB. Mother says baby gets tired just sucking the bottle (30 min to drink). And need to rest in b/w feeding. Can be sudden -bacterial etiology: -syx: septic shock, cardiogenic shock, CHF -Kawasaki can cause myocarditis after 1st week -Rickettsia can cause too -DX: chest X ray -syx: lower height of waves!!! Tiny waves— 1st thing you should do— check machine's setting!! Think myocarditis.

Drug induced hepatitis

-most common is dose dependent- Acetaminophen (Tylenol)- taken more than 140 mg of Tylenol. Can cause hepatic failure -metabolic toxicity with statins -dx: extra eosinophils

Birth injuries -Most common thing you see in vaginal deliveries but not in c-sections -caput succadaneum -cephal hematoma & how to tell the difference b/w the two

-most common vaginal delivery injury: -sutures may override on the head and can be weirdly shaped- called molding. Normal. Won't see this in c-section! The worse the molding, usually the quicker it reverts (gets better). Face & eyes may seem squished at first, but gets better after a day -caput succadaneum- pelvic brim is like a rubber band so below the brim, you have swollen area but above that, it's fine!Circular swelling on scalp, feel;s boggy to touch, no pain to touch, but swelling is crossing suture line. Whatever the shape of the band constricting, is the shape of the swelling -cephal hematoma- traumatic delivery or we used forceps to get baby out. Might fracture the skull & cause bleeding under the periosteum. SUB PERISOTEUM and are limited by the sutures!! **If you have a left cephal hematoma on your left parietal bone, it won't cross sagittal or coronal suture but in caput saccadaneum it WILL cross the suture lines

Heart- endocarditis

-pancarditis- everything is inflamed. Endocarditis, myocarditis, pericarditis inflam -endocarditis- bacterial etiology. Hardware in heart with port. Staph aureus & epidermidis. Strep pnemonia & viridae -scanerio: fever: unknown origin. Has had some kind of heart repair. A fever with underlying cardiac history- Think this!! Most babies will already have some kind of murmur and now you have a CHANGE in the murmur- think endocarditis. -can get petechiae or Roth's spots- little hemorrhages. Emboli phenomenon and spleen will try to clean it and can get spleenomegaly Dx: echo and blood culture (x2 or x3) with fever spike. Take blood during fever!! Elevated see rate. Hematuria. Tx: broad spectrum Abx at first— then narrow after lab results for sensitivity come back -if its acute rheumatic fever- sterptosin test- positive

Definitions- perinatal, neonatal, preterm vs post term, SGA & AGA & LGA; LBW & VLBW; IUGR

-perinatal- gestation 20th week to 28th DOL after birth -neonatal- birth to 28 DOL (days of life) -preterm- less than 37 weeks (66 weeks and 6 days is pre term). Term (37-42 weeks). Post term- anything after 42 weeks -birth WEIGHT tells you gestational age. SGA (10th percentile), AGA, LGA (>90th percentile) -low birth weight (LBW) = < 2500 g & vs VLBW (<1500 g) -Intrauterine growth retardation (IUGR)- fetal growth is in 5th percentile for gestational age]. Head, length, then weight. SO in pre term baby they might be fine height and length but will be low weight! -FGR (fetal growth retardation)- fetal weight is below the 10th percentile for gestational age & aren't growing as much. Much better to be asymmetric (bc this affects weight but preserves length & head circumference). -symmetric FGR affects all parameters

neonatology- factors affecting neonatal outcome: prenatal

-poor nutritional status is biggest factor- earlier the nutrition is affected, the more symmetric the retardation -exposure to drugs & probs hep C positive Major causes of mortality fetus: -placental insufficiency Congenital malformation Intrauterine infection (CMV etc) -hydrosphere fetails (Rh group) Mortality in preterm infant: -respiratory distress- especially premature babies -intraventricular hemorrhages- graded 1-4. Grade 1 will heal spontaneously -necrotizing enterocolitis- why we feed later on, not at first! Gut perforation is BAD (Think head, lungs, gut) Mortality in full term babies: -more related to congenital anomalies -cord wrapped around neck- birth asphyxia -meconium aspiration pneumonia -ex) baby gets stuck in vaginal deliver. Head comes out but can't get rest out. In distress, the baby's anal tone will relax (sticker, gooey substance- like chewing gum mass; is the baby's head is still in—- the meconium will go into the lungs and it's V hard to get it out) -baby is covered in werneckes caseosa (very cheesy)

Tx of gastric reflux

-postural! Raise foot end of crib -thickening feeds- take one spoon of cereal/ enfameal formula and put it with milk and by thickening it it won't come back up in the esophagus -to neutralize acid- can use famotidine. If you want more acid suppression, you can use PPI like omeprazole or Prilosec -surgical treatment id nothing else works: fundoplication

Pre-term vs term vs post-term baby posture

-pre-term baby doesn't have a lot of posture. Stays in what position you put it in -pre-mature babies let you do whatever -in mature babies and post-term, they can't resist a little! -popliteal angle- pre-term baby it's V easy to bring foot to ear but the more term you get, the harder it is to do this! -scarf sign- allow across the chest- easy with pre-me baby. Harder with term baby and even harder with post-term baby

Epiglottis is

-problem more in adults now that're not vaccinated -not as common in kids bc we're vaccinating -immigrants coming in- don't have to be vaccinated coming in -Syx: you don't have much time. Acute onset of fever, stridor, and respiratory distress. -what prevents you from breathing like when you swallow? Epiglottitis!! -So if u have a problem with epiglottitis on top of trachea, you literally can't breathe. Airway is sealed -Tx: airway is sealed. Only way to open is tracheostomy. When you put a pen in pt's trachea if nothing else Tx: most important thing to do. KEEP KID CALM. Before a kid cries, they will take a deep breathe in which creates more neg pressure & the epiglottitis will drop! Give tubing to mom and have her give it to kid. Don't examine child without a surgeon by your side!! Do this all in operating room! Surgeon on standby. Swab and intubate. And they'll be better -Syx: dysphagia, coughing -Look at the posterior pharyngeal space -can also happen in abcess!! If it blocks ur throat. ALl conditions mimic this. Anaphylaxis— anything with throat closing up. All these are your differential dx -Difference: steroptococcyl pharyngitis or mono- progressive! Fever & worse over days -Foreign body obstruction, can be all of a sudden like epiglottitis tho -Croup- slower onset than epiglottitis. Starts to cough, low grade fever, -strider (upper airway & inspiratory) -wheezing (lower airway and expiratory)

Fractures indicating abuse -which 2 fractures are pathognomonic??

-spiral humeral fracture: arm has been twisted -spiral fracture of the femur -spiral is sus but not pathognomic -corner fractures AKA bucket-handle AKA metaphysical fracture-elbow fractures, if you're shaking the child & violently yanking them -oblique fractures of the humerus & femur are also sus -bone scans can reveal occult fractures (healed fractures) -CT scan or fundoscopy (retinal hemorrhage) for hemorrhaging The 3 S: SusPeCT- spinous process, scapula, sternum PMS- Posterior rib fractures, Metaphyseal long bones, spiral fractures -pathognomic fractures: 1) multiple, unexplained long bone and rib fractures 2) corner fracture

Pericardium-pericarditis

-the outermost layer of the heart -2 layers with fluid in b/w -outer layer-pain sensitive -inner layer- pain insensitive -usually viral etiology. Used to be bacterial (H flu) but not anymore with vaccinations -scenario: early on chest discomfort. Eventually, once the fluid is a lot, you've lost the pain!! -mild pericardial effusion: asymptomatic -Lots of pericardial effusion: prevents heart pumping. Low cardiac output.

-Shaken baby syndrome or abusive head trauma now- high yield -differential dx for shaken baby syndrome

-unresponsive: meningitis, sepsis, metabolic, no broken bones on x-ray, or say that kids have already died (SIDS) -CT scans showed some of these kids had intracerebral hemorrhages (not picked up by x-rays). Pattern in bleeds= shaken baby syndrome -Occurs when child is held by forearm and jerked violently back and forth. This is what causes the bleeds. Head is large and elastic and underdeveloped cervical ligaments -soft calvarium; large subarachnoid space; bridging veins b/w dura & cerebral cortex can tear -increased plasticity and axons of unmyelinated brain make it more vulnerable to shearing injuries -includes: TRIAD: subdural hematoma, retinal hemorrhage, & metaphyseal (long bone) chip fractures all bc of shaking so in the brain, eye, and other long bones= "BEB" -no external signs if abuse!! Just internal bleeding -to dx: funduscopy: can also look in yees. Dilate eye, look in fundus of eye and you can see the retinal hemorrhages Differential dx: -purpura due to bleeding disorders like vasculitis (Hendrickson Purpura is Vasculitis)= bruises & hematomas on butt, legs & upper extremities). If you see "bruises" EVERYWHERE think VASCULITIS -thrombocytopenia or clotting factor deficiencies -Mongolian spots- don't mistake these for bruises -coin rubbing- immigrant parents do this to help bring fever down. Not really abuse -cupping- bigger circular bruises with central sequalae

Guillain-Barré syndrome

-usually occurs after some virus infection (like respiratory virus or some GI like campylobacter!!) -sometimes can occur after vaccination. -Pregnancy, immune can also cause GBS -Inflammation of the myelin sheath of peripheral nerves, characterized by rapidly worsening muscle weakness that may lead to temporary paralysis; also known as infectious polyneuritis. -aryflexia -no sensory involvement -mainly symmetric -CN can be involved -DX: spinal tap: protein will be much higher than your cell count- called cytoalbuminogenic disassociation -In contrast to CSF, your cell count will be V high. Not in GBS -If ASYMMETRIC or sharp sensory level or only in one limb- think os something else -Most recover. If phrenic involved, may have Resp probs -Tx: immunoglobulin. prognosis is good. Most pt don't need immunoglobulin IV! -Just do supportive care :) most pt recover. -steroids don't help

1) Child risk factors for child abuse? 2) what's the consensus on spanking? 3) most common triggering events 4) some tests to run if you suspect abuse

1) less than 3 yrs -infants separated from moms at birth bc premature Ex) baby born early. Born and then put in ICU. It's going to have a lot of needs. At high risk!! Separated from mom, increased needs, & more difficult -All NICU babies that're discharged, have a home evaluation. Called HANS in KY -congenital anomalies & chronic illness -difficult children -foster children 2) consensus on spanking is that American academy of pediatrics say no to it but as long as it doesn't leave any marks.... It's not considered child abuse 3) crapping & crying (toilet training & crying) 4) CBC, hemoglobin, hematocrit, prothrombin time (PT), PTT -SCAN (suspected child abuse & neglect) series

Fontanelle; eyes; ears; nose; lip/ throat/ genitalia/ hip

Anterior fontanelle should always be open. Posterior fontanelle may or may not be open -most congenital probs start with eyes (well-spaced, slant like in down syndrome's, location, iris-colobomas, red reflex; do NOT check for alignment or strabismus bc ocular muscles aren't strong in new born -ears: most important- shape and location -low set ears in Down syndrome -cauliflower ears -what other organ is forming at the same time as ear? Kidney!! If you have cauliflower ears, always check for renal problem! -before discharge, every baby has a hearing test done.It's a stimulation test and just tells you if you have the pathway. You find out later on if something is wrong with hearing. -nose- bridge of nose isn't developed and nose is saddle like. Sometimes it makes eyes look weird!= pseudostrabismus. Make sure the posterior nares are open. As molding goes down, nose will look better -lip/ throat- absence of angular muscle is most common cause of a weird lip. Look at tongue ties & look to see if face is asymmetrical when they cry. Roof of mouth: rule out cleft palate!! Can still have cleft palate even if they don;t have cleft lip. It's inherited! Bifold uvula or cleft can come all the way out so different stages of cleft. Can even feel for soft mucosa gums bc clefting can be under there -two white looking pearl lesions= epstein pearls on the middle of palate- normal! Nothing to do with it -For neck.... You can have lost of ****ed up stuff. When child sticks tongue out or swallows, the thyroglossal cysts move with the tongue -chest- pectus like in Marfan's. Don't forget the distal pulses bc you don't wanna miss coarction of the aorta!! -penile exams in male- have to clear them for circumcision. Make sure there is no hooking or curving. WHat condition do you not wanna circumcise male?? Hypospadius. Wanna use that skin later on for reconstruction! -if you can't tell gender, wait for chromosomal gender (you give the chromosomal analysis) -omphjoloclele or meningocele -Hip exams!! CHD (congenital hip dysplasia). Femoral head needs to stay in acetabulum and this is ongoing process. When the head is in and out, the cup doesn't grow around it. DO the Barlow & ortalani tests (do this every well check until child walks).

Congenital abnormalities definitions

Cause: 1) malformation- intrinsically abnormal developmental process like gene mutation 2) deformation- extrinsic disturbance like complete compression of fetus bc lack of amniotic fluid. -Ex) clubbed feet bc baby doesn't have enough room. Normal tissue but something happens to tissue 3) disruption- extrinsic disturbance to one region. Like a finger cut off bc amniotic band. Normal tissue but something happens. -Ex) Pia Robin syndrome- cleft lip/ palate, micrognathia (small tongue) but tongue looks big bc jaw is so small 4) dysplasia- abnormal organization of tissue Ex) cyst in kidney. Renal cystic disease (Formation= whole scale. D= extrinsic cause). -Vater associations: -V for vertebral association -A for anal fistula —T-E for T-E fistula, esophageal fistula -Radial dysplasia or Renal anomaly

Chrohns vs ulcerative cholitis

Chrohns: -can affect any part of GI tract -transmural inflammation- inflamm everywhere -syx are very variable) ex: duodenal ulcers, chronic diarrhea (happens usually bc villous damage), fistula formation, -Malabsorption- villous damage & inflammation -Terminal ileum- Less bile salt absorption & fat malabsorption -Malabsorbed carbs - fermentation & osmotic diarrhea -Colonic involvement - absorption of water -Endoscopic biopsies diagnostic -slide: blue= marked inflammation. V characteristic of chrohns. Non-caseating Granulomas- most common: chrohns -biopsy: nodules, yellow parts UC: -only affect colonic mucosa -starts at anal and goes upwards (only get inflam in rectal mucosa, sigmoid, left, transverse & right colon) -no inflammation in duodenum -only mucosal. Doesn't affect all the layers. So you won't see fistulas in UC -scenario) bloody diarrhea, frank rectal blooding, abdominal pain -biopsy: diagnostic. Inflammation, big pink. Can have exudate. Loss of landmarks -slide: crypts and in lumen of crypts you have crypt abscesses- always indicative of UC Comparing the 2: -In UC, ALWAYS rectal bleeding. Chrohns can have rectal bleeding if out affected anus part. -if you palpate belly, chrohns is transmural inflamm- gut bowel loops stick together & you'd feel that bulge/mass in Chrohns but NOT UC -rectal disease always there in UC. If rectum is spared, it canNOT be UC -if rectum is spared, think chrohns -scenario) rectum involvement, no sigmoid, no left colon, transverse colon patches, PATCHY inflammation - SKIP areas= always Chrohns!! UC never has skip lesions -UC never has skip lesions bc it's continuous disease. When one thing is affected, it'll affect the next thing next to it. No skipping!! -strictures & fistula formation common in chrohns bc transmural inflammation -Risk for colon cancer is V high in UC. If things aren't going well with UC pt, take out colon bc it'll also prevent cancer -fistula & skin tags- has to be chrohns

Different phases of lactose intolerance

Congenital lactose def- V rare Post enteritis lactase def- much commoner Adult onset -lactose breathe test -greater than 25 of hydrogen in breathe -cure/ tx: eliminate lactose

Autoimmune hepatitis

Dx: -Type 1: classic: ANA pos, SMA positive antinuclear or anticytoplasmic Ab are pos, -Type 2: anti-liver kidney microsomal (anti LKM) Ab -Dx: might have other autoimmune disorders like Diabetes, Graves, or Lupus so they would have autoimmune haptitis. If their AST/ ALT is elevated, they have autoimmune hep C -Dx: liver biopsy. Huge expansion of portal triad. -Tx: easily treatable! Long-term immunosuppressive or steroids

Ques for neonatal (1, 2, & 3):

Review Test 1. Soon after birth, a term newborn infant presents with increased oral secretions and mild respiratory distress. Which of the following is the most likely diagnosis? A. Persistent pulmonary hypertension of the newborn B. Pneumonia C. Esophageal atresia D. Respiratory distress syndrome (surfactant deficiency syndrome) E. Diaphragmatic hernia 2. An abdominal mass is detected on examination of a 2-day-old infant in the newborn nursery. Which of the following is the most likely cause of this abdominal mass? A. Ovarian cyst B. Hydronephrosis C. Wilms tumor D. Multicystic kidney E. Hydrometrocolpos 3. The parents of a 5-day-old term infant notice that he is jaundiced. Your physical examination is remarkable only for scleral icterus and jaundice. The infant's total bilirubin level is 15 mg/dL, with a direct component of 0.4 mg/dL. Which of the following is the most likely diagnosis? A. Breastfeeding jaundice B. Choledochal cyst C. Biliary atresia D. Neonatal hepatitis E. Breast milk jaundice 4. You are called to the delivery room to evaluate a newborn infant born at 37 weeks' gestation who has an abdominal wall defect noted on delivery. Based on your initial physical examination, you diagnose an omphalocele. Which of the following statements is consistent with this clinical diagnosis? A. To rule out gastroschisis definitively, an abdominal computed tomographic scan is necessary. B. Compared with gastroschisis, omphalocele is more frequently associated with other congenital malformations. C. This abdominal wall defect is just lateral to the umbilicus. D. The incidence of bowel obstruction is higher in this infant than in one with gastroschisis. E. Omphaloceles may be associated with trisomy 21. 5. You are evaluating a 3-day-old infant with significant respiratory distress. He was delivered by emergency cesarean section at 42 weeks' gestation because of fetal distress. You note that he has an oxygen saturation of 76% in room air that increases to 95% with administration of 100% oxygen. Which of the following statements most accurately supports your suspected diagnosis of persistent pulmonary hypertension of the newborn (PPHN)? A. This patient is likely to have an associated cyanotic congenital cardiac defect. B. PPHN occurs most frequently in premature infants but may occur in postterm infants. C. PPHN usually resolves spontaneously. D. This infant is likely to have significant left-to-right shunting. E. Adequate oxygenation is the best preventive measurement and treatment. 6. A male infant was born at 32 weeks' gestation via cesarean section because of bleeding from placenta previa. Soon after birth, he developed respiratory distress requiring supplemental oxygen and mechanical ventilation. Chest radiograph shows decreased lung volumes and a diffuse ground glass pattern with air bronchograms. Which of the following is the most likely cause of this condition? A. Persistent pulmonary hypertension of the newborn (PPHN) B. Deficient surfactant C. Fluid retention in the lungs D. Bronchopulmonary dysplasia E. Congenital heart disease 7. The parents of a term infant diagnosed with physiologic jaundice are very concerned that their child is at risk for brain damage. Which of the following statements regarding the infant's hyperbilirubinemia is most accurate? A. Breastfeeding, compared with formula feeding, is associated with higher peak serum bilirubin levels. B. Serum conjugated bilirubin concentration is the best predictor of bilirubin encephalopathy. C. Bilirubin encephalopathy does not occur in healthy term infants. D. Increased conjugated (direct) bilirubin levels cause neuronal damage, including choreoathetoid cerebral palsy, hearing loss, and opisthotonus. E. This infant's jaundice is expected to peak at 10-14 days of life. 8. A 2-day-old term male infant is being evaluated before discharge from the nursery. The parents are concerned about a skin rash on his face. As you perform the physical examination, you contemplate skin disorders that are benign compared with those that may indicate underlying pathology. Which of the following skin findings is most likely to be associated with underlying pathology? A. Pustular melanosis B. Nevus simplex C. Milia D. Nevus flammeus E. Erythema toxicum neonatorum (ETN) 9. At a routine health maintenance visit, a 2-week-old infant appears jaundiced. Laboratory evaluation reveals a total bilirubin level of 12.6 mg/dL with a direct bilirubin level of 6.9 mg/dL. Which of the following is the most likely diagnosis? A. Breastfeeding jaundice B. Breast milk jaundice C. Crigler-Najjar syndrome D. ABO incompatibility E. Choledochal cyst 10. A female infant born at 30 weeks' gestation develops abdominal distension, abdominal tenderness, and bloody stools on the third day of life. Which of the following statements regarding the most likely diagnosis is correct? A. The diagnosis is supported by a double-bubble sign on abdominal radiographs. B. The diagnosis is supported by pneumatosis intestinalis on abdominal radiographs. C. The diagnosis is supported by a soap-bubble appearance on abdominal radiographs. D. The infant will ultimately require pancreatic enzyme supplementation. E. The diagnosis has an increased association with Down syndrome. Questions 11 and 12: The response options for statements 11 and 12 are the same. You will be required to select one answer for each statement in the set. A. 2 B. 3 C. 4 D. 5 E. 6 F. 7 G. 8 In each case, select the infant's 1-minute Apgar score. 1. At 1 minute of life, a newborn's respiratory rate is slow and irregular with a heart rate of 120 beats/minute. There is some flexion of her upper and lower extremities; she grimaces when a catheter is placed into her nose; and she appears to be pink and well perfused, except for some cyanosis of the distal extremities. 2. At 1 minute of life, a newborn's respiratory rate is slow and irregular with a heart rate of 80 beats/minute. There is some flexion of his upper and lower extremities; he does not respond when a catheter is placed into his nose; and he is blue and pale. You're called to the Nursery to evaluate a cyanotic infant with significant respiratory distress. After 100% oxygen is administered to the infant, there is almost no improvement in the PaO2 (only 10 mm Hg). Which of the following is the patient's most likely diagnosis? A. Tetralogy of Fallot B. Pneumonia C. Respiratory Distress Syndrome (RDS) D. Meconium aspiration syndrome E. Transient tachypnea of the newborn

Infectious Encephalitis vs encephalopathy vs herpes simplex encephalitis

INFECTIOUS ENCEPHALITIS: -severe headache, nausea, vomiting -memningeal irritation signs: can't flex their chin to their neck. When you try to flex knee and hip and extend their leg will give them back pain= kernicks and bedinskys signs are positive -if you have encephalitis with virus- can have those syx too -focal neurological deficits- seizures. Can lose bladder control ENCEPHOLOPATHY: -mass effects: hemorrage or abcess or rupture in brain or cyst. Can have mass effects -remote controlled effects: ingestion of toxin Or -metabolic disorders (hyponutremia) can lead to encephalopathy -scenario: pt isn't really conscious. In stupor or coma. This is encephalopathy -HX for encephalitis: Important for tick bites, environment, travel DX: CSF! -EEG for seizures -Cat scan for hemorrhage MRI: tumors or rabies TX: respiration, cardiac support, antivirals, antibiotics, fix metabolic stuff. - With intracranial problems, Put them on antacids bc high chance for stress ulcers & gastritis. H2 agonist -In herpes simplex encephalitis- CSF has higher red blood count but not as much WBC

How do you evaluate protein malabsorption? What specific factors can you look at?

IS it being caused by: 1a) protein malabsorption- serum albumin (albumin has a V long half life of 24 days so it's gonna take time for albumin to go down); prealbumin (half life 9 days; can show earlier) -why would someone have low albumin levels but no diarrhea?? Kidney problem! Just bc there's low albumin, it's not automatically GI prob. It can be that ur intake and absorption is good but prob is when it gets to kidney! -scenario) all history is good. No diarrhea. Albumin came back as 2 instead of 4. No albumin is urine. What's the cause of this? Cirrhosis of liver! You can also have fluid overload so albumin is normal- just diluted. -summary here on low albumin levels: suspect GI issue (if diarrhea present); suspect kidney issue (if albumin in urine); suspect liver if none in urine 1b) protein malabsorption- fecal alpha 1-antitrypsin- losing protein in stool 1c) pancreatic enzyme analysis- not V common 2a) carbohydrate malabsorption- D xylose tolerance- For every 1 kg, give child 1 gram of D Xylose. Ex) 35 kg child so give him 35 grams of D xylose in mouth. Then check D xylose level after 1 hr. Normally, it goes up 25 mg per deciliter. If child has a prob with carbohydrates, it won't go up that much! It might go up 10 mg per deciliter. -helps determine the integrity of billows surface area 2b) carbohydrate malabsorption- breathe H2 analysis- If 35 kg child, give 35 grams of lactose, after an hour, look to see how much hydrogen they're exhaling in their breath. Normally, lactose will be digested and absorbed. You shouldn't have any hydrogen bc lactose should be broken down into glucose & galactose. -in lactose intolerance, Lactose stays in gut, lactose is fermented, and it's going to make hydrogen. Hydrogen is exhaled. -Proxy for malabsorption 2c) carbohydrate malabsorption- stool pH &b reducing substances -if stool pH is acidic, there's a ma;absorption of carbohydrates 3a) -fat malabsorption- fecal fat analysis. Take drop of stool and under microscope If you see fat globules, you aren't supposed to see them. Not easiest 3b) fat malabsorption- fecal fat 72 hr. Know how much intake and measure how much is excreted. You should only be excreting 4% so out of 100g if you' true excreting 10 g, that's not normal. Not easiest 3c) serum retinol & Vit E levels- V helpful! Vitamin A & E are fat soluble vitamins so if you're absorbing these two vitamins, you probs don';t have fat malabsorption

Hypoglycemia

Less than 40 is a problem. -you want it to be over 45 -if symptomatic (irritated, floppy) -do not let baby get hypoglycemia!!

Rare causes:

Munchasuen syndrome by proxy: Laxative abuse -mom was putting laxatives in baby's milk to get secondary gain -carcinoid tumors -endocrinopathies- hyperthyroidism can cause diarrhea

Hep C

Properties: -lacks proofreading so it has a lot of antigenic diversity -No vaccine bc it keeps replicating and making different subtypes -causes innate immune response & T cells which causes inflammation -2-3% in the world. The burden of healthcare on system bc so many people have hep C -40-60% people get chronic disease -biggest cause: drug use with needles Dx: -ELISA- Ab test. (Can still be false positive) so do the next 2 tests: -more specific test: RIBA-Radio Immune Blot Assay or PCR-HCV RNA test (V sensitive test) -children born to mom's with Hep C: 5% get infection. Early on, Ab test will be positive but should screen around 18 months & it can be neg! -snowflake inclusions on slide Tx: -Direct Acting Antivirals (DAA's)- V effective. Can cure pt in 12 weeks!! But V V expensive

Most common problem in newborn

Respiratory distress- breathing too fast or too slow or using accessory muscles, grunting, flaring, retraction -depends on age of child -pre-term babies- most common reason is lack of surfactant that causes respiratory distress syndrome -full term babies-most common reason is meconium aspiration or polycythemia (can aspirate anything) -common to both pre and full term infant: sepsis!! -Group B sepsis -transient tachypnea- this transitioning of replacing fluid with air takes some time -spontaneous pneumothorax- bleb of lung -diaphragmatic hernia- stomach is sitting in your left thoracic cavity and the stomach descends and it presses on lung -metabolic errors- eventually will get acidosis and respiratory distress TESTS: -chest x ray: to see if there's fluid or air. Is heart normal? ** V typical pattern of respiratory distress in premature babies: each alveoli is collapsed and opaque so now they look like white tiny dots! Snowstorm appearance -arterial blood gas- oxygenation based on PO2, ventilation based on PCO2, and acidosis based on pH -white count- infections! -babies get hypoglycemia V early so check blood glucose!! -Ex) you're increasing your FiO2, but your PO2 isn't improving so you're thinking cardiac problem

Pyloric stenosis

Scenario) 4 week/ 6 week old baby, baby has been VOMITING (not spitting up). Projectile vomiting in baby less than 6 weeks be very careful of pyloric stenosis!! You get Projectile vomiting. Giant peristalsis waves. If you palpate, you should be able to palpate a pyloric mass (hypertrophy) -ultrasound: dx: gold standard: will see thickening of pyloris. Upper GI series isn't preferred -Tx: always surgery. Pyloromyotomy; make sure you treat the electrolyte (hypochloremic (bc stomach has lot of chloride), hypokalemia & metabolic alkalosis (bc you're throwing up all the acid) ****electrolyte imbalance for pyloric stenosis: hypochloremic, hypokalemia, & metabolic alkalosis -seen more in males. Seen in 2nd/ 3rd week of life

Hep A

Scenario: -Hep A- Picorna virus. fecal oral. See it a lot in day care settings. Changes diaper and get it. Contaminated water/ food -Syx: most of the time it's asymptomatic!! But it's a problem bc it can still be shed. Most common syx is jaundice. Which is how it's brought under attention -case scenario: 6 yr old comes in with low grade fever, maybe vomited 2 days ago, sclera is yellow!! ALT & AST elevation, elevation of bilirubin, IgM antibody for 6 mo, IgG persists for the rest of your life Tx: symptomatic! If their glucose is low, you can give that Vaccine --HepA vaccine went down from vaccine BUT it started to go up with people who had addiction and homelessness -Hep A vaccine at 1 year and 2nd dose at 18 months. Vaccine became available at 2003. Vaccine recommended for everyone! But kids are required to get it for school

Different stages of reflux

Stage 1 Stage 2 Stage 3 Stage 4a

Peritonsilar abcess vs retropharyngeal abcess— how does the uvula look in each?

What would you seee? Uvula is sideways displaced to the other side, touching the other tonsil!! Retropharyngeal abcess- uvula is gonna be pushed Forward bc abcess is coming in from the back!! "Hot potatoe voice"- it sounds muffled Tx: drain & antibiotic

Bardet-Biedl Syndrome

_obesity!!!

sandifer syndrome (GI)

cerebral palsy kids might have altered tone of esophageal sphincter, and they're trying to get food down so they arch their back. Mistaken as a seizure but they're just trying to get food down. -think about putting your head back in the "sand"- baby does same motion