UW 2 Flashcards
Causes of precocious puberty
-central ==> high FSH & LH -peripheral ==> low FSH & LH (e.g. excess peripheral conversion, estrogen-producing ovarian cyst, CAH
NF1 features (7)
Two or more of the following
6 Cafe au lait spots
2 Neurofibromas
Freckling of the axillary or inguinal area
Optic glioma
2 Lisch nodules (Iris hamartomas)
Bone abnormality (eg. scoliosis)
First degree relative with NF1
Evaluation of precocious puberty
bone age normal ==> premature thelarche (breast development) vs. premature adrenarche (pubic hair development)
advanced ==> basal LH basal
LH high ==> central
low ==> GnRH stimulation test
low LH ==> peripheral
high LH ==> central
Neurologic deficits in B12 deficiency
loss of distal vibration sense & proprioceptive sense memory loss, irritability, dementia
Tx of megaloblastic anemia w/folic acid alone
if 2/2 B12 deficiency, folic acid will correct anemia but accelerate neurologic sx of B12 deficiency
Most common cause of constrictive pericarditis in Africa, India, China = ?
TB
Pagets Disease presentation
osteoclast dysfxn ==> increased bone turnover may be asx can lead to fx, bone pain, skeletal deformities can lead to H/As and hearing loss if bones of head/ear involved
Dxx for nephritic/nephrotic sd with low C3
- Postinfectious
- Membranoproliferative (including mixcrioglobilinemia)
- Lupus nephritis
Mixed cryoglubulinemia presentation
Palpable purpura
Nephritic/Nephrotic Sd
Low C3
Hep C (90%)
Nephritic Sd findings (6)
Proteinuria
Hematuria
Azotemia
RBC casts
Oliguria
Hypertension
Types of Nephritic Sd
Inmune Complex (postinfectious, igA, henoch-sholein)
Pauci-inmune (No IG deposits on inmunofluorescence)
Granulomatosis with polyangitis, microscopic polyangitis, eosinophilic granulomatosis
Anti-GMB disease Goodpasture, Alport
Renal disease with pulmonary symptoms (hemoptisis) dxx
- Goodpasture (no ulcers) 2. Granulomatosis with polyagitis
a. Kidney + lungs + sinus
b. kidney + lungs
c. kidney + asthma
a. granulomatosis with polyangitis
b. microscopic polyangitis / goodpasture
c. Chrug-Strauss (eosinophilic granulomatosis with polyangitis)
Pauci-inmune glomerulonephritis
a. Granulomatosis with polyangitis URT involvement (may see ulcers and polyps). Cavitary lesions (hemoptysis) PR3-ANCA/C-ANCA (antiproteinase 3)
b. Microscopic polyangitis No URT involvement. MPO-ANCA/p-ANCA (antimyeloperoxydase)
c. Eosinophilic granulomatosis with polyangitis (churg- strauss) Asthma, sinusitis, skin nodules, peripheral neuropathy MPO-ANCA/p-ANCA (antimyeloperoxydase) Eosinphilia, Elevated IgE
ALL treated with corticosteroids, cyclophosphamide or rituximab
Immune complex glomerulonephritis
a. Postinfectious: Common in kids. 2-4 weeks after infection. Low C3
b. Ig A (berger): Common in adults. During infection, Normal C3. Most common with GI infection (IgA produccing musocsa
c. Henoch-Shonlein purpura Like Berger + purpura (arthralgias, abdominal pain, IgA+)
Anti GMB glomerulonephritis
a. Goodpasture Hemoptisis, dyspnea, respiratory failure
b. Alport Fxx of renal failure. Serineural deafness
Treatment to consider in nephritic sd
- IECA (for all types of hypertension and/or proteinuria)
- Glucocorticoids
- Steroids, Cyclophosphamide, and rituximab
- Plasma exchange
Nephrotic sd causes
M and M FAN
Minimal change (most common in kids)
Membranous (highest rate of thrombosis), most common in white adults, 30% overall)
Focal segmental (most common in adults, espacially african american)
Amyloidosis
Diabetic
Lupus
Important complication in mebranous neprhopathy
Highest rate of thrombosis Renal vein trhombosis (hematuria, flank pain and scrotal edema)
Histology finding in MPGN
Tram track double layered basement mambrane/ Intermembranous dense deposits Low C3