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STEP2
Pediatrics UWORLD
| Question | Answer |
|---|---|
| Age at which kids develop B-cell deficiency problems? | 4-6 months |
| Which type of cell is defective in children with recurrent opportunistic/fungal infections? | T-cells |
| What are the 4 B-and T-cell immunodeficiencies? | SCID Ataxia-Telangiectasia Hyper IgM-syndrome Wiskott-Aldrich syndrome |
| What are classic (triad) findings of Ataxia telangiectasia? | ↓ IgA, cerebellar Ataxia, and Spider Angiomas |
| What are signs that indicate suspicion for CGD? | Inflammatory bowel disease, granulomas on intestinal bx, and person/family member with HX of recurrent infections |
| What test confirms CGD? | Oxidative burst test |
| What are the 3 Phagocytic immunodeficiencies? | 1. Leukocyte Adhesion defect 2. Chediak-Higashi syndrome 3. CGD |
| What is the worst arrhythmia which kids with long QT interval are at risk for? | Torsade de pointes |
| What conditions ↑ risk for polymorphic ventricular tachycardia in Long QT syndrome pts? | ↑↑ HR and ↑↑ sympathetic activity |
| What medications are used to prevent PVT in long QT interval patients? | ß-blockers |
| How does the "knee-chest" position in ToF help to relieve tet spells? | ↑↑ SVR, ↑↑ pulmonary blood flow, and improve hypoxemia |
| Common manifestations of possible hearing loss | Inattention, social disinterest, speech delay, and HX of recurrent ear infections |
| What is the 1st step in management is suspected hearing loss? | Audiometry |
| What types of HPV are seen in warts seen in the true vocal cords? | HPV 6 and 11 |
| How is respiratory papillomatosis presented in a child vertically transmitted? | Hoarseness of voice due to "finger-shaped" growths on true vocal cords |
| Common name for Otitis Externa | Swimmer's ear |
| Which otitis type is associated with pain with ear manipulation (pulling), externa or media? | Otitis externa |
| What is the most common source of infection leading to Otitis Externa? | Outdoor water sources |
| Is cerumen acidic or basic? | Acidic |
| Kid pulling on ear lobe constantly. MC DX? | Otitis media |
| MC pathogen causative of Otitis Externa? | Pseudomonas aeruginosa |
| Antibiotic TX for Otitis externa? | Amoxicillin ± clavulanate |
| What are the 3 criteria to diagnose OE? | 1. S/S for 10 days or more w/out improvement 2. S/S with severe fever (≥39C (102.2F) + drainage ≥ 3days 3. S/S biphasic illness ± Fever |
| What is the defect associated with fetal hyperglycemia during 1st trimester? | Malformation |
| What are the defect associated with fetal hyperglycemia after 1st trimester? | Macrosomia, birth injury, and hypoglycemia. |
| What is the MC complication in infants of DM mothers? | Hypoglycemia |
| What enzyme is missing in von Gierke disease? | Glucoese-6-phosphatase |
| What are the main S/S of von Gierke disease? | Hypoglycemia (±seizures), lactic acidosis, hyper-uricemia, and hyper-lipidemia |
| Doll-like face | von Gierke disease |
| What physical features of von Gierke disease? | "Doll-like face", thin extremities, short-stature, and protuberant abdomen |
| Is von Gierke disease seen with or without hepatomegaly? | Hepatomegaly that causes the protuberant abdomen |
| What is the MC lead point resulting in recurrent intussusception in kids? | Meckel diverticulum |
| What is an atypical location for intussusception? | Small bowel |
| What test is used to diagnose Meckel diverticulum? | Nuclear scintigraphy |
| What is intraosseous access? | Technique in which bone marrow cavity is used as a non-collapsible vascular entry point. |
| When do you use intraosseous access? | Emergency situations in which a peripheral access cannot be obtained |
| Which requires higher skill level: Intraosseous access or Central Venous catheter placement? | Central Venous catheter |
| How is the Red Cell Distribution (RDW) in thalassemia minor? | Normal |
| What does an elevated reticulocyte count mean? | Compensation by the bone marrow in response to anemia |
| What does Splenic sequestration mean? | Fatal complication of SCD in which blood cells and platelets become entrapped within the spleen. |
| What are the S/S of Splenic sequestration? | Anemia, splenomegaly, and hypovolemic shock |
| What is the initial treatment protocol for SCA complicated by splenic sequestration? | IV fluids and Packed RBC transufion |
| What causes Urticarial transfusion reatcion? | Preformed recipient IgE antibodies reacting against a solutble allergen in the donated plasma |
| What is the treatment for Urticaral transfusion recation? | Stop transfusion and diphendydramine |
| What is the PPX for better survival in SCA patients? | Pneumococcal vaccination + Penicillin prophylaxis |
| What are sutble clues for SCA? | - ↑↑ Reticulocyte count - Previous hospital admission due to pain - Hematuria |
| What are physical features of Peutz- |
Created by:
rakomi
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