3/23 GI and Renal Flashcards
Hemophagocytic lymphohistocytosis (HLH)
AR defect in perforin-related genes or linked to EBV infection.
Over active immune system attacks bone marrow, liver and brain in little kids
see macrophages engulfing RBCs
Signs and symp of Diffuse liver injury with no signs of cirrhosis
JAUNDICE
Steatosis, acute inflammatory infiltrate
accumulation of eosinophilic material in hepatocytes
During thoracentesis why insert needle on the lower margin of the intercostal space
To avoid stabbing the intercostal a, n, v
Mutation in Duchenne muscular dystrophy
X linked FRAMESHIFT
(out of frame)
dystrophin gene (usually anchors muscle fibers to extra cellular matrix
Mutation in Becker muscular dystrophy
x-linked NON frameshift (in-frame deletions)
Myotonia, muscle wasting, cataracts, testicular atrophy, frontal balding
Myotonic type 1
AD
CTG repeat
MY Tonia My testicles, My toupee, My ticker (arrhythmias)
Drugs that cause malignant hyperthermia
Sevoflurane, Desflurane, isoflurane, succinylcholine
tx: dantrolene
IV drug user with leukopenia, diffuse powdery interstitial infiltrates and + Methenamine silver stain
Pneumocystis jirovecii
Tx for pneumocystis jirovecii
Trimethoprim-sulfamethoxazole
MOA: Block folate synthesis at pteridine and PABA nucleotide formation
Forehead lesion with palisading nuclei
Basal cell carcinoma
What does basal cell carcinoma look like
Pink, pearly nodules with telangiectasis, rolled borders and central crusting
SE of haloperidol
EPS Acute dystonia (first several hrs) Akinesia (first several days) Akathisia (weeks) Tardive dyskinesia (mo)
Acute respiratory acidosis
Low pH
High pCO2
normal or slightly elevated HCO3 (
inheritance of classical galactosemia
AR
inheritance of lesch-Nyhan
x-linked recessive
X linked recessive disorders
Be Wise, Fool’s GOLD Heeds Silly HOpe
Burton agammaglobulinemia Wiskott-Aldrich Fabry G6PD Ocular albinism Lesch-nyhan Duchenne (and Becker) Hunter syndrome Hemophilia A and B Ornithine transcarbamylase def
Where is the majority of water reabsorbed in the kidneys?
PCT (60%)
depending loop-20%; DT and CD 20%
Where is K+ reabsorbed?
66% in the PT
25-30% thick ascending loop
where is K+ levels regulated?
Alpha intercalated cells of the collecting tubules
(H+/K+ ATPase)
Depletion of K+ stimulates alpha intercalated cells to reads K+
Increased K load stimulated principal cells to secrete K+
Who gets IgA nephropathy?
children (hence-schonlein purpura)
Episodic hematuria with RBC casts
Triad and cause of HSU
Triad: anemia, Thrombocytopenia, acute renal failure
Due to micro thrombi forming on damaged endothelium
With GI illness
Thrombocytopenia thrombotic purpura-HUS
A thrombotic microangiopathy
Fever, neuro symptoms, renal failure, anemia, thrombocytopenia w/ GI illness
Normal PT and aPTT
Thrombotic microangiopathy syndromes
TTP-HUS and DIC
Put activation in arterioles and caps
diffuse microvascular thrombosis (brain, kid, heart)
microangiopathic hemolytic anemia with schistocytes
thrombocytopenia
What is the most important poor prognostic factor for PSGN?
Increased age of onset
Also pre-existing kidney disease
Eosinophilic glassy casts
MM
or chronic pyelonephritis but they resemble thyroid tissue
Cause of acute interstitial nephritis
Penicillin, NSAIDs, PPI, sulfonamides, diuretics
See eosinophils in pee, fever, rash, acute renal failure
Gross painless hematuria in an older adult
Urinary tract cancer (urothelial or RCC)
Renal cell carcinoma histology
Clear cells–> filled with glycogen and lipids
Lipid peroxidation associated with…
inflammation, atherosclerosis and tumorigenesis
how is PAH secreted?
Carrier protein mediated process so it can be saturated at high blood concentrations
membranous nephropathy, flank pain, gross hematuria, right-sided varicocele, elevated lactate dehydrogenase
Renal vein thrombosis
What does a isolated right-sided varicocele indicate?
IVC occlusion by a malignant tumor or thrombus
Antithrombin deficiency
Inherited–> only a problem if give heparin (increased PTT)
Acquired–> renal failure/nephrotic syndrome–> antithrombin loss in urine–> decreased inhibition of factors IIa and Xa
Risk factor for calcium kidney stones?
Hypercalciuria Idiopathic but maybe: Increased GI abs increased mobilization from bone Decreased renal tubular Ca reabs
Other risks: hyperoxaluria, hyperuricosuria, low urine volume, hypocitraturia
Why patients with calcium stones remain normocalcemic
intact regulation of serum Ca by Vit D and PTH
Associations with renal papillary necrosis
SAAD Sickle cell dz and trait Acute pyelo Analgesics (NSAIDs) DM
Difficulty starting and maintaining stream of urine + nocturnal enuresis and daytime uncontrolled voiding w/o feeling a full bladder
Overflow incontinence
impaired detrusor contractility or bladder outlet obstruction
can be due to DM neuropathy
Which fluids increase in concentration along the proximal tubule? which decrease?
increase: PAH, creatinine, insulin, urea
Decrease: Bicarb, glucose, aa
primary virulence factor of E. coli causing UTIs
P fimbriae
allows adhesion to uroepithelium
ApoE
Found on chylomicrons and VLDLs–> responsible for up take
NOT on LDL
deficiency–dysbetalipoproteinemia (palmar xanthomas and premature atherosclerosis)
Extrinsic clotting pathway factor
VII