Pediatrics Elite High Yield Notes PDF

Pediatrics Elite High Yield Notes Www.Medicalstudyzone.com 1 Notes by MedSN httpst.meUSMLEWorldStep2CK  PEDIATRICS GENERAL PRINCIPLES............

Pediatrics Elite High Yield Notes Www.Medicalstudyzone.com 1 Notes by MedSN httpst.meUSMLEWorldStep2CK  PEDIATRICS GENERAL PRINCIPLES.........................................................................................................................1 Autism : 18m and 2yr , depression screen at 12yrs and yearly , visual at 4yr Lipid once between 7-12yr [MS3D - Mentalhealth, Sexualhealth, Substance use,Safety, Dyslipidemia] ROUTINE SCREENINGS.............................................................................................................................2 >15:3 doses 97% micro 3.5CM IN NEONATES = HEPATOMEGALY Www.Medicalstudyzone.com 1 httpst.meUSMLEWorldStep2CK  2 PEDIATRICS ROUTINE SCREENINGS Uric Acid Crystals in Diapers: 1. Autism screening is performed at ages 18 months and 2 years 1. “Pink stains” or “brick 2. Depression screening starts at age 12 years (repeated annually) dust” in neonatal 3. Visual acuity screening is routinely done at age 4 years diapers represent uric acid crystals Routine adolescent screening 2. Uric acid excretion is MS3D Category Screening method* high at birth and decreases until Mental health Validated depression questionnaire adolescence Confidentia l discuss ion about sexual act ivity Sexual health Gono rrhea & chlamyd ia test ing if sexually active HIV testing (once at age 21 days) 5. MARKED peripheral leukocytosis with neutrophilia 6. Biopsy of infected tissue shows lack of neutrophils 7. Culture shows mostly staph aureus and gram – bacilli NOT SEEN IN MILD INTEGRIN DEFECTS - SO DONT RELY ON THIS ! HYPER-IGE SYNDROME 1. Also called Job syndrome 2. Inheritance pattern: Autosomal Dominant 3. Presentation: a. Recurrent skin abscesses b. Recurrent pulmonary infections c. Recurrent S aureus infections d. Characteristic facial features (eg, broad nose) e. Bony abnormalities (eg, scoliosis) ANAPHYLACTIC VS URTICARIAL TRANSFUSION REACTIONS Transfus ion reactions Anaphy lactic Urticaria ! Anti -lgA ant ibodies (lgG or lg E) in Preformed rec ipient lgE antibodies Mechan ism lgA-defic ient patient against donor aga inst soluble alle rgen in donated blood lgA plasma Onset Seco nds to minutes Hours Respi ratory distress /wheeze Angioedema Hives Find ings Hypote nsion Itching Hives Immed iate cessat ion of transfus ion Immed iate cessation of transf usion Epinep hrine Antih istam ines Treatment Antih istam ines , oxyge n, fluids & Resume transfus ion if patie nt is vasopressors otherwise asymp tomat ic Www.Medicalstudyzone.com 21 httpst.meUSMLEWorldStep2CK  23 PEDIATRICS STANDARD IMMUNIZATIONS Standard pediatric immun izations in the United States. Polio Inactivated (killed). Hepatitis A. Influenza (injection) Toxoid (inactivated tox in). Diphtheria, tetanus. Rotavirus Measles Live attenuated. Mumps Rubella Varicella. Hepatitis B. Pertussis Haem ophi/us influenzae type B Subunit/conjugate. Pneumococcal. Meningococcal. Human papillomavirus Www.Medicalstudyzone.com 22 httpst.meUSMLEWorldStep2CK  24 PEDIATRICS DTAP VACCINATION 1. Schedule a. 5 doses given at ages: i. 2, 4 & 6 months ii. 15-18 months iii. 4-6 years 2. Complications a. Seizures i. Not a contraindication if they are uncomplicated i.e, short ( -0 0 8 -a.~ Cl> 6 0) C :;::; ·e 4 - 0 > 0 :it 2 0 0 5 10 15 20 25 30 35 40 45 50 55 60 65 70 Www.Medicalstudyzone.com 171 httpst.meUSMLEWorldStep2CK  175 PEDIATRICS MICROCOLON 1. Pathognomonic for Cystic fibrosis 2. Viscous meconium obstruction of terminal ileum underused and contracted colon 3. Administration of hyperosmolar enema (eg, Gastrografin) can break up inspissated meconium and dissolve the obstruction 4. Surgery is required if therapeutic enema fails Differentiating features of Hirschsp rung disease and meconium ileus Hirschsprung disease Mecon ium ileus Assoc iated Down syndrome Cystic fibrosis disorder Typica l level of Rectosigmoid Ileum obstruct ion Meconium Normal lnspissated cons istency "Squirt sign " Positive Negative Hirschsprung Disease Www.Medicalstudyzone.com 172 httpst.meUSMLEWorldStep2CK  176 PEDIATRICS PEDIATRIC CONSTIPATION 1. Treatment a. Behavioral modification i. water intake ii. dietary fiber intake iii. Limiting cow’s milk to oz per day 3oz = concerning iv. Sitting on toilet after each meal b. Oral Laxative (if behavioral modification fails) i. Polyethylene glycol ii. Mineral oil c. Suppositories or enema (if oral laxatives fail) Pediatric const ipation Initiation of solid food & cow's milk Risk factors Toilet train ing School entry. Painful/ hard bowel movements Clinical features Stool withho lding. Encopresis Ana l fissures Complications. Hemorrhoids Enuresis /urinary tract infectio ns j Dietary fiber & water intake. Limit cow's milk intake to 3 = concerning Www.Medicalstudyzone.com 174 httpst.meUSMLEWorldStep2CK  178 PEDIATRICS PRUNE BELLY SYNDROME 1. Defect in abdominal musculature 2. Intestinal loops seen through thin abdominal wall 3. Intestines covered by skin GASTROSCHISIS 1. Isolated defect 2. Herniation of uncovered bowel lateral to umbilicus 3. Features a. Free-floating intestines in amniotic sac seen in prenatal ultrasound b. Oligohydramnios c. Continued exposure of intestines to amniotic fluid chronic inflammation intestinal thickening i. This leads to risk of necrotizing enterocolitis and bowel obstruction short bowel syndrome 4. Treatment a. Cover with sterile saline dressings b. Plastic wrap them to minimize heat and fluid losses c. Decompress stomach by placing NG tube d. Administer antibiotics e. Surgical repair Www.Medicalstudyzone.com 175 httpst.meUSMLEWorldStep2CK  179 PEDIATRICS REYE SYNDROME 1. Due to aspirin ingestion in children 2. Features a. Encephalopathy b. Hepatic dysfunction c. Cerebral edema d. ICP Latest Reye syndrome Etiology Pediatric aspi rin use during influenza or va ricella infectio n Ac ute liver failure Clinical features Encep halopathy AST, A LT Laboratory findings. PT, I R, PTT NH3 Treatment Suppo rtive ALT = alanine aminotransferase; AST = aspartate aminotransferase; NH3 = ammonia. latest Www.Medicalstudyzone.com 176 httpst.meUSMLEWorldStep2CK  180 PEDIATRICS EVALUATION OF BILIARY EMESIS Evaluation of bilious emesis in the neonate Bilious emesis Stop feeds NG tube decompression IV fluids I Abdominal x-ray I I l l l Free air, Dilated loops NG tube in Double hematemesis , of bowel misplaced bubble sign unstable vital signs duodenum I Surgery I Contrast enema Upper GI series Rectosigmoid Ligament of Treitz transition zone on the right side of abdomen 1 1 Meconium ileus Hirschsprung IMalrotation I Duodenal disease atresia (c)IJWnrlci Www.Medicalstudyzone.com 177 httpst.meUSMLEWorldStep2CK  181 EVALUATION OF BILIOUS EMESIS IN NEONATE Www.Medicalstudyzone.com httpst.meUSMLEWorldStep2CK  182 PEDIATRICS MIDGUT VOLVULUS 1. Primary predisposing factor is malrotation of midgut in early fetal development 2. Presentation a. Age 3.5CM IN NEONATES = HEPATOMEGALY 2. Labs: a. direct bilirubin b. Normal or mildly elevated AST and ALT 3. Diagnosis a. First step abdominal ultrasound i. Findings: abnormal/absent gallbladder; absent bile duct; triangular cord sign (fibrous remnants seen above the porta hepatis) b. Liver biopsy plugging and proliferation of bile ducts as well as portal tract edema and hepatic fibrosis Www.Medicalstudyzone.com 183 httpst.meUSMLEWorldStep2CK  188 PEDIATRICS c. Gold Standard cholangiogram 4. Treatment a. Hepatoportoenterostomy (Kasai procedure) i. Creation of path for bile to flow from liver to small bowel b. Liver transplant Biliary atres ia Pathogenesis Extrahepatic bile duct fibros is Asymptomat ic at birth Clinic al Infants age 2-8 weeks : findi ngs o Jaundice, acholic stools , dark urine o Hepatomega ly Direct hyperb ilirub inemia Ultrasound: o Absent/abnormal gallb ladder &/or CBD Liver biopsy : Diagnostic o lntrahepat ic bile duct proliferat ion evaluation o Porta l tract edema o Fibrosis lntraoperative cholang iography (gold standard) : o Biliary obstruction Surg ical hepatoportoenterostomy (Kasai procedure) Treatme nt Liver transplant CBD = common bile duct. LIVER EDGE IN NEONATES 1. Liver edge >3.5 cm is abnormal in neonates. 2. Liver edge. cm is normal Www.Medicalstudyzone.com 184 httpst.meUSMLEWorldStep2CK  189 PEDIATRICS NECROTIZING ENTEROCOLITIS: 1. Can be caused by: a. Prematurity b. When there is reduced mesenteric perfusion, such as: i. Congenital heart disease ii. Hypotension LBW30k platelets tin transfus ion. Genetidmetabo lic disease : hypot hyro idism or hyperthy roidism ; genetic trisomy (13, 18, 21) Asymp tomatic (most com mon) Ruddy skin Clin ical Hypog lyce mia, hyperbilirubinemia presentat ion. Resp iratory distress , cyanosis, apnea. Irritability, j itteriness Abdom inal distension. Intraveno us fluids Treatment Glucose Partial exc hange transfusion 1. Increased RBC mass leads to increased uptake of glucose and calcium, hence causing: a. Hypoglycemia b. Hypocalcemia 2. Treatment a. Hydration by oral feeds b. Glucose-containing parenteral fluids Www.Medicalstudyzone.com 212 httpst.meUSMLEWorldStep2CK  217 PEDIATRICS DIAMOND-BLACKFAN ANEMIA 1. Clinical features a. Macrocytic anemia i. Reticulocytopenia b. Craniofacial abnormalities i. Mild hypertelorism ii. Flat nasal bridge c. Triphalangeal thumbs 2. Other features include: a. Short stature b. Webbed neck c. Cleft palate 3. Normal platelets and WBC 4. Treatment a. Mainly corticosteroids b. RBC transfusion in refractory cases Diamond-B lackfa n anem ia Pathogenesis Conge nital erythroid aplasia Craniofacial abno rmalities Clinical findings Triphalangeal thu mbs Increased risk of maligna ncy Macrocy tic anemia Labo ratory fi ndings Reticulocytope nia Norma l platelets, white blood cells Corticostero ids Treatme nt Red blood cell transfusions Www.Medicalstudyzone.com 213 httpst.meUSMLEWorldStep2CK  218 PEDIATRICS INHIBITOR DEVELOPMENT IN FACTOR VIII DEFICIENCY: 1. Approximately 25% of patients with severe hemophilia undergo inhibitor development a. Suspect inhibitor development in case of: i. Increased bleeding frequency ii. Hemorrhage refractory to treatment 2. Regular screening for inhibitor development is performed when: a. Patients with hemophilia receive factor infusions b. When inhibitor is clinically suspected 3. Treatment a. Bypassing products i. In case of factor VIII inhibitor, give recombinant activated factor VII, activated prothrombin complex concentrates ii. These agents work downstream of coagulation cascade TRANSIENT ERYTHROBLASTOPENIA OF CHILDHOOD 1. Presentation: 6 months to 5 years 2. Features a. Normocytic normochromic anemia i. Hb level 3-8 g/dl b. Extremely low reticulocyte count c. Normal WBC and platelets SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS 1. Presents in early childhood 2. Look for: a. Large joint pain with rash and high fever 3. Treatment a. Aspirin *Note: aspirin in children can only be given in Kawasaki Disease and Juvenile Idiopathic Arthritis* Www.Medicalstudyzone.com 214 httpst.meUSMLEWorldStep2CK  219 PEDIATRICS ANEMIA OF PREMATURITY Anemia of prematurity Impaired erythropoietin production Etiology Short red blood cell life span Iatrogenic blood sampling Clinical Usually asymptomat ic manifestations Tachycardia , apnea , poor weight gain Low hemoglobin & hematocrit Laboratory Low reticulocyte count findings Normocytic , normochromic red blood cells Minimize blood draws Treatment Iron supplementation Transfusions 1. Normally when a baby is born, EPO concentration decreases in the blood because of high oxygen supply to tissues a. Hence, RBC count decrease occurs at age 2-3 months (according to RBC age) 2. But if a baby is born preterm, it gets anemia of prematurity because: a. Short RBC life span (40-50 days) b. Frequent phlebotomy in NICU 3. Most cases are asymptomatic 4. Anemia of prematurity is a diagnosis of exclusion 5. Normocytic normochromic anemia 6. Treatment a. Minimize blood draws b. Iron supplementation c. RBC transfusion i. In symptomatic babies ii. It will further suppress EPO levels and delay recovery iii. Supplemental EPO is not effective Www.Medicalstudyzone.com 215 httpst.meUSMLEWorldStep2CK  220 PEDIATRICS VITAMIN K DEFICIENCY Vitam in K deficiency Inadequate dietary intake (eg, malnutrition) Disorders of fat malabsorption o Cystic fibrosis o Biliary atresia Risk factors Disorders of intestinal inflammation o Celiac disease o Inflammatory bowel disease Decreased production by bacterial flora (eg, frequent antibiotic use) Easy bruising Clinica l Mucosa! bleeding featu res Gastrointestinal bleeding Laboratory t PT &INR findings Normal aPTT (unless severe deficiency) HEMOGLOBIN ELECTROPHORESIS FINDINGS IN SICKLE CELL AND THALASSEMIA: Hemog lobin electrophores is patterns in sickle cell & beta-thalassem ia Cond ition Hemoglob in A Hemoglob in A2 Hemoglobin F Hemoglob in S Normal 95%-98% ~2.5% ,. fl) C :0 30 0 0) ~ 2 20 0 0 10 6 12 18 24 30 36 6 12 18 24 30 36 42 Prenatal age (weeks) BIRTH Postnatal (weeks) Www.Medicalstudyzone.com 217 httpst.meUSMLEWorldStep2CK  222 PEDIATRICS FOLATE DEFICIENCY ANEMIA Folate defic iency anemia Chronic hemo lysis (eg , sickle ce ll disease ) Poo r dietary intake Etiology Malabsorp tion (eg , gas tric bypass ) Med ica ·ons (eg , me hotrexa e) Clinica l features Dysp nea , fatigue, pa llor, weakness NON MEGALOBLASTIC MACROCYTIC Macrocy tic anemia DIAMOND BLACKFAN Laboratory Poo r re iculocyte respo nse (low to norma l) ALCOHLICS AND LIVER find ings Hype rsegme nted neutrop hils Low serum folate Treatment Folic acid suppleme nta ion HEMOLYTIC UREMIC SYNDROME Hemolytic uremic syndrome Initial insult from Shiga toxin (Escherichia coli serotype 0 157:H7) Pathogenesis Vascular damage & microthrombi formation Preceding bloody diarrhea Fatigue, pallor Clinical features Bruising, petechiae Oliguria, edema Hemolytic anemia (schistocytes, bilirubin) Laboratory findings Thrombocytopenia Acute kidney inj ury ( BU I creatinine) Fluid & electrolyte management Treatment Blood transfusions Dialysis DO NO T GIVE ANTIBIOTICS IN A BLOODY DIARRHEA HUS PT AS IT INCREASES THE RICK FOR HUS Www.Medicalstudyzone.com 218 httpst.meUSMLEWorldStep2CK  223 PEDIATRICS APLASTIC ANEMIA Aplastic anemia PANCYTOPENIA Pathogenes is Bone marrow failure due to hematopoietic stem cell deficiency (CD34 +) Auto immune Infections (eg, parvov irus B 19, Epstein-Barr virus) Causes. Drugs (eg, carbamazepine, chloramphenicol, sulfon amides). Exposure to rad iation or toxins (eg , benzene, solvents) Laboratory studies : Pancytope nia Anem ia (fatigue, weak ness, pallor) Clinical & Thrombocy topen ia (mucosa l bleeding, easy bruising, petechiae) laboratory find ings. Leukopenia (recurrent infections) Biopsy : Hypoce llular bone marrow with fat and stromal cells Www.Medicalstudyzone.com 219 httpst.meUSMLEWorldStep2CK  224 PEDIATRICS TRANSFUSION REACTIONS Immu nolog ic blood transfusion react ions Transfusion Onse e Cause Key features reaction Ang ioedema , Recipient anti- hypotension , Within lgA antibodies resp iratory Anaph y lactic seconds to directed aga inst d istress /wh eezi ng, minutes dono r blood lgA shock. lgA deficient recipient. Fever, flank pain, ABO hemoglob inuria Ac ute hemo lytic Within incompatibility. Dissem inated 1 hr (often clerical intravascular error) coagulation. Positive Coombs test Feb rile Cytokine non hemolytic (most common Within 1-6 hr accumu lation during blood. Fever & chills HAVE TO DO LEUKOREDUCTION reaction) storage Recipient lgE Urticar ia! Within 2-3 hr against blood produ ct. Urticaria component. Respiratory distress Transfus ion- Within Donor anti-. Noncardiogen ic related acute leukocy te pulmonary edema with 6 hr lung inju ry antibodies bilateral pulmonary infiltrates Often asymptomatic Anamnestic. Laboratory evidence of Delayed With in days hemoly tic anemia hemolytic to weeks antibody response. Positive Coombs test , positive new antibody screen. Rash , fever , Graft versus DonorT- gastrointestinal Within weeks host lymphocytes symptoms , pancytopenia "Time after transfusion initiation. Www.Medicalstudyzone.com 220 httpst.meUSMLEWorldStep2CK  225 PEDIATRICS TRANSFUSION-ASSOCIATED CIRCULATORY OVERLOAD Transfusion-associated circulatory overload TACO TRALI Age 60 Risk factors Unde rlying card iac or renal cond ition Large transfusio n volume or fast infus ion rate Resp iratory distress Clinical features j Heart rate (