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What is the associated disorder with Hirschsprung disease? Where is the obsturction usually located? How is the consistency of teh meconium? What is teh "squirt sign"?

Down syndrome. Rectosigmoid. Normal meconium consistency. Positive squirt sign


What is the associated disorder with Meconium ileus? How is the consistency of the meconium? What is the "squirt sign"?

CF. Inspissated. Negative squirt sign.


What is the treatment for sickle cell patients presenting with stroke symptoms?

Exchange transfusion


What is the management of duodenal obstruction 2/2 duodenal hematoma?

NG suction with parenteral nutrition


What is the presentation of Zenker's diverticulum? What is the test of choice?

Dysphagia, regurgitation, foul smelling breath, aspiration, palpable mass. Contrast esophagram is the test of choice.


What is ppx treatmetn for renal transplant patients?

Oral TMP-SMX for preventing PCP, flu vaccine, pneumoccocus vaccine and Hep B vaccine


What is the renin and aldosterone levels for patinets with hypertension and hypokalemia in secondary hyperaldosteronism? What are some causes?

Both are elevated. Renovascular HTN, Malignant HTN, Renin secreting tumor, diuretic use


What is the renin and aldosterone levels for patinets with hypertension and hypokalemia in PRIMARY hyperaldosteronism? What causes it? Why is there not significant hypernatremia?

Renin is low, aldosterone is high. Aldosterone-producing tumor, bilateral adrenal hyperplasia. Aldosterone escape


What is the most common complication of nephrotic syndrome? What can it lead to?

Hypercoagulability 2/2 urinary loss of antithrombin3, altered protein C adn S levels, increased platelet aggregation. Renal vein thrombosis.


What are other complications of nephrotic syndrome?

Protein malnutrition, iron-resistant anemia, Vit D deficiency 2/2 cholecalciferol binding protein deficiency. Decreased thyroxine level due to loss of TBG.


What is a feared complication of antithyroid drugs? What are presenting signs? What is teh next step?

Agranulocytosis (WBC


What is the workup for abnormal uterine bleeding?

Endometrial biopsy. IF no atypia or kids desired, progestin therapy. IF no kids desired or complicated, hysterectomy


What is the most common congenital cyanotic heart disease in neonates? What are findings? What is treatment?

Transposition of great vessels. Single S2 +/- VSD. Egg on a string (narrow mediastinum). Prostaglandinss


What are the findings for TOF?

Harsh pumonic stenosis murmur, VSD, boot shaped heart


What are the findings for Tricuspid atresia?

Single S2, VSD murmmur, minimal pulmonary flow


What are the findings for Truncus arteriosus?

Single S2 ,systolic ejection murmmur, increased pulmonary blood flow, edema


What are the findings for Total anomalous pulmonary venous return with obstruction?

severe cyanosis and respiratory distress. Pulmonary edema ( snowman sign) and enlarged supracardiac veins and SVC


What are the symptoms of borderline personality disorder? Treatment?

unstable relationships. mood lability, anger, impulsivity, recurrent suicidal behavior. Tx is psycotherapy with dilaectical behavior therapy. Other tx are 2nd gen antipsychotics, mood stabilizers, antidepressants.


What is the presentation of diamond black fan? What is seen on peripheral smear? Treatment?

Macrocyutic pure red aplasia associated with short stature, webbed neck, cleft lip, shielded chest, triphalangeal thumbs. No hypersegmaentaiton of neutrophils. Corticosteroids


What is the presentation of Fanconi anemia?

AR disorder with pancytopenia, macrocytosis, cafe au laits spots, microcephaly, absent thumbs, horsehsoe kidneys, microphthalmia


What causes Whopping cough? What are the three phases?

Bordetella pertussis. Catarrhal, paroxysmal, Convalescent.


What are teh features of paroxysmal phase of whooping cough? Labs? Treatment? Prevention?

Coughing paroxysms, posttussive emesis, (apnea, cyanosis in infants less than 6 months). Lymphocytosis. Macrolides are first line treatment.. Acellular pertussis vaccine.


What are the presenting symptoms of brain abscess? What re predisposing factors?

Fever, headache, focal neurological deficits, seizures. Congenital heart disease and recurrent sinusitis.


What are teh PFT findings in ankylosing spondylitithis?

Restricitve lung disease 2/2 limited chest and spinal mobility. Reduced VC adn reduced TLC with normal FE1/FVC


What blood abnormality is found in SLE?

Pancytopenia 2/2 immune mediated destruction of all 3 cell lines. Anemia can be due to ACD, Renal insufficiency, Iron defieicny anemia and autoimmune hemolutic anema.


What causes elevated A-a gradient? How can it be corrected?

Intersitial lung disease, V/Q mismatch 2/2 pulmonary edema from fluid collection in lungs, shunts. Supplemental oxygen can correct hypoxemia, V/Q mismatch, A-a gradient


How is ehrlichiosis transmitted? Where is it located? Symotoms? Labs? Dx? Tx?

Lone star tick. South and south central central US. FLu-like illness, neurologic symotoms, rash is uncommon. Leukopenia and thrombocytopenia. Elevated LFTs and LDH. Intracytoplasmic morulae in monocytes. Empiric doxycycline


What is the most common site of colon cancer metastasis? How does it manifest and how is it diagnosed?

Liver, RUQ pain, elevated LFTs, firm hepatomegaly. CT abdomen


What are the majority of renal stones composed of? What is the shape? Causes?

Calcium oxalate crystals which are envelope-shaped. Fat malabsorption leading up to freeing oxalate from caclium and causing it to (oxalic acid) free for absorption


What is the presentation of TOF? What kind of pulmonary markings?

PROVE - pulmonic stenosis right ventricular hypertrophy, overriding aorta, VSD. Decreased pulmonary markings.


What is the presentation of Hypoplastic left heart syndrome?

Absent plse with single S2. Incresed Right ventricular impulse. GRAY rather than bluish cyanosis.


What is the presentation of Truncus arteriosus? What kind of pulmonary markings?

Severe dyspnea, early and frequent resiratory infections. Pulmonary HTN after 4 months is feared complciations. VSD. Single S2. Increased pulmonary markings with cardiomegaly.


Which congenital heart defect shows a snowman/figure of 8 on echo? How does it present?

TAPVR without obstruction. Right heart failure and tachypnea.


What heart condition does a child have who has failre to thrive after several months and a holocystolic mrmmur?

VSD leading to RVH from Eisenmenger syndrome.


What is the presentation of PDA?

Machinery like murmur, Wide pulse pressure, boudnig pulses


What is the presentation of long QT syndrome?

Hearing loss, syncope, family Hx of sudden cardiac death


What is the treatment for VSD?

Diuretics and digoxin


What is the most serious complication of hyperbilirubinemia in the new born?

Hyptonia, seizures, choreoathetosis, hearing loss


When should you consider exchange transfusion for hyperbilirubinemia?

If bilirubin rises to 20-25 mg/dL


What is the most common complications of TF fistula? Which one is the most common type?

Recurrent Aspiration PNA. EA with distal TEF.


What causes the hypokalemia in pyloric stenosis? How is it treated?

Aldosterone release 2/2 increased volume loss. Replace fluids and electroytes. NGT decompresion


What are the components of CHARGE syndrome?

C -coloboma of the eye, CNS abnormalities, H - heart defects; A - Atresia of the choanae; R - rtrdation of growth; G - GU defects; E - Ear anomalies


What is the presentation of duodenal atresia? What conditions is it associated with?

Bilious vomiting; double bubble, associated with Trisomy 21 and Annular pancreas, No respiratory distress


What is the pathophysiology of Hirschprung disease? Symptoms?

Congenital lack of innervation in Auerbach plexus; no meconiium pasage for 1st 48 hours, large bowel obstruction 2/2 obstruction; inability to pass flatus. Distended bowel loops with lack of air in rectum. Associate with Down syndrome.


What are the components of VACTREL

Vertebral defects, Anal atresia, Cardiac defects, Tracheo-esophageal fistula, Renal anomalies, and Limb abnormalities.


Do biliary atresia and pyloric stenosis have Bilious vomiting?



What are the 5 causes of bilious vomiting? What test do you use for each?

Meconium Ilieus and HiRSchesprung (contrast enema showing mcirocoon for meconium ileus and RectoSigmoid transition zone for Hirschprung), Duodenal malrotation (double bubble), volvulus (upper Gi series showign ligament of Treitz on R side), intusscucseption


What is the presentation of Intussussecption

Currant jelly stool, bilious vomiting, colicky abdominal pain


What is the presentation of angiofibroma?

Epistaxis, bone erosion, localized mass


What is the presentation of necrotizing enterpcolitis? Immediate treatment?

Prematurity, vomiting, abdominal distend ion, fever. CT shows air in portal vein and air within bowel wall is seen on AXR. immediate treatment with Vancomycin, gentamicin, metronidazole. NPO, fluids, NGT, surgery if abx don't help