Pediatrics Flashcards
(165 cards)
What three qualities characterize constitutional bone delay?
Delayed growth spurt
Delayed puberty
Delayed bone age
MCC of short stature and pubertal delay in adolescents?
Constitutional growth delay.
When does constitutional growth delay occur?
Children born with normal weight and height at birth experience a slowed height velocity between 6 months to 3 years.
In constitutional growth delay, when should normal growth resolve?
At 3 years of age the child regains normal growth velocity and follows the growth curve at the 5th to 10th percentile. However, puberty and adolescent growth spurt are delayed, though they will occur. They will reach a normal adult height.
3yo boy presents with fever, cough, SOB. He recently recovered from Giardia and has an Hx of lobar pneumonia and ear infxns that were recurrent since age 6months. He has a mild fever. PE reveals small tonsils and crackles in the right lower lobe. He has normal growth. Cause of reccurrent infxns?
X-linked agammaglobinemia (Bruton agammaglobinemia). This results from failure of B cell development. Small or absent lymph tissue with recurrent sinopulmonary and GI infxns occur after maternal antibody protection wanes. T cells are normal. Rx: Ig replacement and antibiotics.
X-linked agammaglobinemia (Bruton agammaglobinemia) presents as?
B cell failure causing small/absent lymphatics with recurrent sinopulmonary infxns and GI infxns after maternal antibodies wear off (after 6 months).
Pts. with recurrent severe viral, fungal, and bacterial infxns who are also failure to thrive. Dx?
Adenosine deaminase deficiency. This causes SCID (severe combined immunodeficiency).
Patients with complement deficiencies are at increased risk of what diseases?
Disseminated bacterial infxns, especially those caused by encapsulate species (e.g. S. pneumoniae, N. meningitidis). Giardia is not problematic in complement deficiency.
Chronic granulomatous disease is due to?
Impaired oxidative burst that leads to recurrent skin/pulmonary infxns with catalase+ organisms (S. aureus, Serratia marcescens)
DiGeorge syndrome (thymic hypoplasia) results in?
Hypocalcemia
Cardiac defects
Failure to thrive
Recurrent infxns (loss of T cell maturation)
Common variable imunodeficiency presents with?
Normal B cells, but reduced IgG, IgM, and IgA.
Selective IgA deficiency results in what Sx?
Usually asymptomatic, but some have recurrent respiratory, GI, urogenital infxns due to low serum IgA. IgM is normal.
Patients with selective IgG subclass deficiencies have?
Recurrent infxns with low or normal total IgG and normal IgM.
Young child presents with severe and recurrent sinopulmonary infxns as well as tympanostomy tube Hx and poor growth. His IgG and IgA are markedly decreased, but his elevated IgM is normal. Dx?
Hyper IgM syndrome. Presents with recurrent sinopulmonary infxns and poor growth. The lack of normal CD40 ligands prevents B cells switching to produce IgG and IgA, thus, encapsulated bacteria cause infxns often Opportunistic infxns (PCP) also can occur. Rx: IV Ig and ABx.
Does a child with a grade III murmur that increases with standing, but decreases with laying supine warrant an echo?
Yes. The decreased preload when standing results in low venous return and preload leading to worsened obstrxn due to lower LV size. Harsh, holosystolic, or diastolic murmurs are concerning.
Does a child with a grade II murmur that decreases with standing, but increases with laying supine warrant an echo?
No. This is more likely to be an innocent murmur. Grade 1 or 2 murmurs which decrease with standing and valsalva and are louder when supine are typically benign.
Newborn presents with a 5-cm skin patch of dark skin with overlying hair on the upper back. Likely Dx?
Congenital melanocytic nevus. Solitary, hyperpigmented lesions with increased density of overlying dark, coarse hair. 5% increase in melanoma risk.
Asian newborn presents with dark, blue-gray patches on the skin, especially the lower back. Dx?
Mongolian patch aka congenital dermal melanocytosis. These are poorly circumscribed and often fade with time.
Blanchable red patch in newborn on one side of the face that overlies the eye. Dx?
Nevus flammeus aka port-wine stain. These tend not to regress.
Blanching pink patches that fade with time. Tend to be located on the eyelids, glabella, and nape of neck in kids. Dx?
Nevus simplex.
1 month old presents with frequent regurgitation of mild and painless bloody stools. He appears to have eczema on physical exam. Dx?
Milk-protein induced enterocolitis. A non-IgE mediated immunologic response to proteins in formula or milk causes inflammation in the colon leading to the Sx. Prognosis is excellent and most kids tolerate milk/soy products by 1 year old.
Children with sickle cell trait are at increased risk for?
Renal issues, especially painless hematuria resulting from sickling in the renal medulla. Isosthenuria (trouble concentrating urine) is also common and can present as nocturia and polyuria.
Sickle cell trait Hgb electrophoresis pattern?
50-60% Hgb A and 35-45% Hgb S.
Sickle cell disease Hgb electrophoresis pattern?
85-95% Hgb S and 5-15% Hgb F.