Primary 2 Final exam CONDENSED

Key findings of ITP

bruising, petechiae, mucosal bleeding, enlarged liver, spleen, lymph nodes, fever, weight loss

First line treatment in ITP

corticosteroids

clinical findings of Von Willebrand Disease

mucous membrane bleeding, easy bruising, decreased vWF, normal platelet count

Management of Von Willebrand Disease

desmopressin and Factor VII-vWF

clinical findings of pancytopenia

infection, bleeding

Treatment for pancytopenia

hematopoietic stem cell transplantation, splenectomy

What most commonly causes Lymphocytosis

viral illnesses: MMR, Varicella, Mono, Hepatitis, EBV

Clinical features of Fanconi Anemia

hematologic manifestations, SOB, chest pain, dizziness, fatigue, prolonged bleeding, petechiae

Clinical findings of TEC

previous infection, breath holding spells

What triggers TEC

viral trigger (paravirus B18, herpes, echovirus)

Beta Thalassemia labs

Elevated Hgb A2

Hgb 4-10

Labs for Vitamin B12/Folate deficiency

CBC, reduced reticulocytes, macro ovalocytes, hyper segmented neutrophils, high methylmalonic acid

Lab findings for Hemolytic Anemia

elevated reticulocyte count

Sickle Cell Management

NSAIDs, opioids, pneumo/meningococcal vaccines, prophy penicillin

What medication reduces pain and crisis in sickle cell

hydroxyurea

Labs for central hypothyroidism

Low free T4, Normal or low TSH

Lab values for subclinical hypothyroidism

TSH mildly elevated, free T4 normal

Hashimoto’s history and exam

growth delay, fatigue, weight gain, family hx, goiter, midline facial abnormalities

what is sick euthyroid syndrome

alteration in thyroid levels in systemic illness

Common lab finding in sick euthyroid syndrome

decreased total and free T3

What is MODY

Monogenic, inherited nonautoimmune form of diabetes

characteristics of MODY

impaired insulin secretion, no insulin resistance, absence of pancreatic autoimmunity

Prediabetes lab results

Fasting glucose: 100-125

A1c: 5.7-6.4

OGTT 2 hour glucose 140-199

Lab findings for Hypoparathyroidism

Low calcium and PTH, high phosphorus

Delayed puberty onset

5 years of thelarche - menarche for girls

4.5 years - full portal maturity in boys

Adrenal crisis symptoms

lethargy, vomiting, abdominal pain, hypoglycemia, hypotension, AMS

History and exam of constitutional growth delay

bone age delayed, growth rate aligns with bone age, family history, short stature

What is a basal/bolus approach in T1DM

Basal - between meals and overnight

Bolus - during meals/snacks

Management of SCID

pneumocystis and fungal prophy, isolation, IVIG, hematopoietic stem cell transient

What is Ataxia Telangiectasia

genetic defect that prevents ability to repair damaged DNA

Management of ataxia telangiectasia

IVIG, betamethasone

Diagnostic findings of Wiskott Aldrich Syndrome

Low IgM, High IgA and IgE

What can hypoparathyroidism cause in neonates

tetany from hypocalcemia

What does IgM do

first to respond, activates complement system

What does IgG do

passes placenta to baby, immune memory

What does IgA do

mucosal protection, across membranes

What does IgE do

Allergic reactions and parasite defense

Manifestations of CVID

sinopulmonary infections, diarrhea, hepatitis

Management for CVID

IVIG

Manifestations of Hyper IgE syndrome

recurrent infections, eczema history

Clinical findings in SLE

Butterfly/malar rash, fever, fatigue, joint pain

Pharmacological management for SLE

NAIDs, oral steroids, immunosuppressants

Clinical findings of fibromyalgia

widespread pain for longer than 3 months, interferes with ADLs, insomnia

Clinical findings of femoral anteversion

family history, W sitting, kissing patella, stumbles over feet

Blount disease

affects the proximal tibial epiphysis, brace if under 3, surgery if over 4

Physiologic genu varum

angle less than 15 degrees up to age 3

When does genu valgum resolve

by 4-6 years of age

Internal tibial torsion

most common cause of in toeing in children age 2, abnormal rotation of the tibia

External tibial torsion

TFA indicates >30 degrees, r/t IT band contracture

Clinical findings of Muscular Dystrophies

symptom onset 2-6 years, toe walking, defined calves, gluteal atrophy, gower sign

Scoliosis management

observation is less than 20 degrees

brace if 25-45 degrees OR 5+ degree progression

surgery if 45 degrees or more

Leukemia symptoms

fever, repeated infections, weight loss, pallor, hepatosplenomegaly, petechiae, lymphadenopathy

Labs for leukemia

CBC, peripheral smear, bone marrow aspiration, CSF

Clinical findings of Hodgkin Lymphoma

painless enlargement of lymph nodes, chronic cough, fever, drenching night sweats

Pattern of B’s in Hodgkin Lymphoma

B cell malignancy, Past EBV infection, Binucleated cells (reed sternberg), Bi modal

Clinical features of Retinoblastoma

Leukocoria, strabismus, orbital inflammation

pertinent history of Wilms Tumor

abdominal mass that does not cross midline, HTN, fever, weight loss

Neuroblastoma

abdominal mass/distention that crosses midline

Pathophysiology of Tumor Lysis Syndrome

Hyperuricemia from purine metabolism, hyperphosphatemia, low calcium, high phos

Which late effect requires the MOST frequent monitoring:

secondary malignancy in the radiation field

Mentzer index (MCV/RBC)

<13 = thalassemia trait

>13 = iron deficiency

G6PD common triggers

aspirin, sulfonamides, antimalarials, anthelmintics, quinolones, fava beans

First line therapy for Diamond Blackfan Anemia

corticosteroids (prednisone)

Common complication of sickle cell anemia

splenic sequestration

G6PD symptoms

dark urine, jaundice, recent viral illness

How does beta thalassemia typically present

asymptomatic, mild hypochromic anemia, iron does not help

Vegan child with pallor, weakness, beefy red tongue is deficient in what vitamin

B12

What is used to diagnose Scoliosis

Cobb Method, greater than 10 is scoliosis

Blount disease affects what bone

tibia

Gower sign is associated with what

Duchenne muscular dystrophy

Tibial torsion typically resolves when

age 8