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
Action of cyclosporin and SE
Inhibits interleukin 2 transcription
nephrototoxycity (most common)
tremor
hta
gum hypertrophy
hyperkaliemia
Mycophenolate side effects
Marrow supresion
Azathioprine
diarhee leukopenia hepatotoxicity
Calcium renal stone
Radioopaque. Most common Square
Calcium phosphate: high pH
Calcium oxalate: low pH
Struvite stone
Mg amonium phosphate “Paralelepipedo” Radioopauqe. Urease producing organism: Proteus high pH
Uric acid stone
Radiolucid
diamond shaped
Low pH
Hydrate and alkalinize urine
Cystine stones
Hexagonal. radiolucent
Defect aminoacid transport: COLA Cystine, ornithine, lysine and arginine
Family hx
Hydration, Na restriction, urine alkalinization
Kidney stone management according to size
<5mm: may pass spontaneously
<10mm: may pass with CCBs or Tamsulosin tx
5-20mm: shock wave lithotripsy
>20mm: percutaneous neprholithotomy
Young patient from developing country with progressive dyspnea, nocturnal cough and hemoptysis
Rheumatic heart disease
Mitral valve stenosis causes
Rheumatic fever (developing countries) Uncommon in the US
Presentation of Mitral stenosis
Progressive symptoms Dyspnea Orthopnea Hemoptysis =======> Eventualy Right heart failure
Left atria enlargment: Afib (thrombus formation), Stroke dysphagia and hoarness, elevation of main bronchus
Mitral stenosis auscultation
Opening snap with mid diastolic rumble at the apex
Rocky Mountain spotted fever
Riketsia Fever, malaise Maculopapular rash that starts in wrist and ankles… becomes petechial/purpuric… spreads centrally (involve hands and soles)
Altered mental status and DIC may be seen in severe cases
Treat with doxycycline
Ixodes tick is the vector of?
Babesia
Lyme (Borelia)
Anaplasma
Anemias algorithm pg 188 first aid
See algorithm Mycrocitic (5) Normocytic (14) Macrocytic (7)
Hereditary Spherocytosis
Autosomal Dominant
Fx of splenectomy/hemolytic anemia
Clasic triad: splenomegaly, hemolytic anemia, jaundice
Labs in Hereditary Spherocytosis
Normocytic anemia. Reticulocytosis
normal MCV, elevated MCHC and RDV, Coombs -
Indirect hyperbilirrubinemia
May present with cholecystitis due to pigmented gall stones
Diagnosis: Eosin-5 maleimide flow cytometry, acidified glycerol lysis test, osmotic fragility test
Autoinmune hemolytic anemia vs. Hereditary spherocytosis
Both are hemolitic anemia with spherocytes
Only AIHA is direct Coombs +
Types of Autoinmune hemolytic anemia
Warm: IgG (SLE, CLL, lymphoma, penicillin, rifampin, phenytoin and a-methyldopa)
Cold: IgM (Mycoplasma, EBV, Waldesntrom macroglobulinemia)
POSITIVE: direct coombs
Complications of Mumps
Orchitis
Can cuase aseptic meningitis and trasient sesoryneural hearing loss
Pancreatitis has been described
Preterm premature rupture of membranes vs. premature rupture of membranes
PRM: 1hr before onset of labor (precipitated by urogenital infections, cervival incompetence,)
PPRM: <37weeks
Prolonged: >18hr (need ATB prohylaxis)
Agammaglobulinemia
boy (x linked recesive), starting at 6motnhs
Infections with H influenza and S. neumonia and Pseudomona
B cell deficiency
Low Ig level
Confirm with B and T-cell subsets (B absent, T high)
Absent lymphoid tissue (eg. no tonsils)
B cell Disorders (4)
Agammaglobulinemia: H. influenzae, Pseudomona and pneumococcal infections
Common variable inmunodeficiency: (both B and T defect). All Ig levels low
IgA deficiency: anaphylactic reaction to blood transfusion. Recurrent GI and Resp infections
Hyper IgM sd: severe recurrent sinopulmonary infection
Multiple Myeloma
CRAB
Calcium (elevated), renal involvement, anemia/amyloidosis, Bone (lytic lesions) / back pain
Multiple Myeloma: Monoclonal M protein
Increased risk for infections (respiratory and UTI most common) due to impaired antibody production
Diagnosis of Chronic granulomatous disease
dihydrodamine 123 test
nitroblue tetrazolium test
Chronic granulomatous disease
Increased risk of catalase + organisms (Candida, S. aureus, Serratia, Aspergillus, E. coli, Klebsiella)
Severe pyogenic infections
X linked or autosomal resecive
Treatment of Chronic granulomatous disease
Prophylaxis with TMP-SMX
Interferon
Bone marrow transplant
Obstructive pattern on spirometry
FEV1/FVC: <70%
FEV1: decreased
FVC: normal/decreased
FRC: increased
TLC: increased
Managment of Neonatal respiratory distress sd
- CPAP or intubation
- Surfactant administration
Pretreat with corticosteroids in patients with risk of preterm delivery in the next 7 days (24 to 37 weeks)
Xray findings in:
a. NRDS
b. Transient tachypnea od the new born
c. Meconium aspiration
a. Ground-glass apearance, lack of focal opacities
b. Due to amniotic liquid retantion. Perihiliar streaking
c. Coarse, irregular infiltrates, lung expansion, pneumothoroax
Secondary syphilis
Fever, malaise, generalized lyphadenopaties
Symetric, difuse maculo papular rash in palms and soles
Condyloma lata (highly contagious)
Treat with Penicillin G
Treatment of Multiple Sclerosis
Acute: High dose corticosteroids, plasma exchange
Disease modifiying medications:
- Interferon (ABC) (decrease number of relapses)
- Ocrelizumab (progressive MS)