Quick associations :) Flashcards
What is the most common cause of nephrotic syndrome in blacks and hispanics?
FSGS
Which nephrotic syndrome is associated with obesity, heroin use, IF tx, CKD due to congenital absence or surgical removal?
FSGS
Which nephrotic syndrome is associated with Hodgkin lymphoma?
MCD
Which nephrotic syndrome is associated with chronic conditions (multiple myeloma, TB, RA)?
Amyloidosis
Which nephrotic syndrome is associated with an Ab to PLA2-R, drugs, infections, SLE, or solid tumors?
Membranous nephropathy
Which nephrotic syndrome has a “spike and dome” appearance on EM?
Membranous nephropathy
Which nephrotic syndrome is associated with HBV and HCV?
Type I membranoproliferative GN
Which nephrotic syndrome is associated with C3 nephritic factor Ab?
Type II membranoproliferative GN
Which nephrotic syndrome has a “tram track” appearance?
Type I membranoproliferative GN
Which nephrotic syndrome has eosinophilic nodular glomerulosclerosis (Kimmelstiel-Wilson lesions) on LM?
Diabetic glomerulonephropathy
What is the most common cause of nephrotic syndrome in Caucasian adults?
Membranous glomerulonephropathy
Which kidney pathology is associated with “crescents?”
RPGN (nephritic)
What do the crescents of RPGN consist of?
Fibrin and plasma proteins with glomerular parietal cells, monocytes, and macrophages
What disease processes can lead to RPGN?
Goodpasture syndrome, Wegener granulomatosis, Microscopic polyangiitis, Churgg-Strauss
What is the most common cause of death in SLE?
Diffuse proliferative glomerulonephritis
What is the most common cause of nephropathy worldwide?
IgA nephropathy (Berger disease)
What is Alport syndrome?
Mutation in type IV collagen –> thinning and splitting of glomerular BM. X linked. Isolated hematuria. Glomerulonephritis, deafness, and eye problems.
IgA nephropathy is seen with what disease?
Henoch-Schonlein purpura
What kind of kidney stones look like coffin lids?
Struvite
What kind of kidney stones look hexagonal?
Cystine
What kind of kidney stones look rhomboid or rosette-lke?
Uric acid
Ethylene glycol, VitC abuse, or Crohn disease can lead to what kind of kidney stones?
Ca oxalate
What is the tx for recurrent calcium kidney stones?
Thiazides and citrate
What causes struvite kidney stones?
UTI with urease positive bugs
Renal oncocytomas arise from which cells?
Collecting duct cells
What is the WAGR complex?
Wilms tumor, Aniridia, Genitourinary malformation, mental Retardation
Transitional cell carcinoma (kidney) arises from what cells?
Urothelial cells
Drug-induced interstitial nephritis (tubulointerstitial nephritis) can progress to what?
Renal papillary necrosis
How does Drug-induced interstitial nephritis present?
Either asymptomatic or with fever, rash, hematuria, oliguria, CVA tenderness about 2 weeks after starting drug
What parts of the kidney are highly susceptible to ischemic injury?
PCT and thick ascending limb
What part of the kidney is highly susceptible to nephrotoxic injury?
PCT
What is renal papillary necrosis associated with?
DM, acute pyelonephritis, chronic phenacetin use (acetaminophen), HbS/SS
How does renal papillary necrosis present?
Gross hematuria and proteinuria
Hypersegmented PMNs are seen in what?
B12/folate deficiency
What kind of gene malfunctioning leads to alpha-thalassemia?
Alpha globin gene deletion
What kind of gene malfunctioning leads to bet-thalassemia?
Point mutations in splice sites and promoter sequences
What blood disease presents with a crew cut appearance on xray and chipmunk facies?
Beta thalassemia major
Beta thalassemia major predisposes to aplastic crisis by which bug?
Parvovirus B19
What leads to basophilic stippling of RBCs?
Lead poisoning, anemia of chronic disease, EtOH abuse, lead poisoning, and thalassemias
What is first line treatment for lead poisoning?
Dimercaprol and EDTA
Orotic aciduria leads to what kind of anemia?
Megaloblastic macrocytic anemia
What is the pathophysiology of orotic aciduria?
Defect in UMP synthase; cannot convert orotic acid to UMP in de novo pyrimidine synthase pathway. Tx is UMP.
What is Fanconi anemia?
DNA repair defect
What are the lab findings of anemia of chronic disease?
Low Fe, low TIBC, high ferritin
What is the treatment for hereditary spherocytosis?
Splenectomy
What is HbC defect?
Glutamic acid –> lysine mutation at residue 6 in beta globin. Patients with HbSC have milder disease than those with HbSS.
Patients with Paroxysmal nocturnal hemoglobinuria have increased risk for what disease?
AML - 10% develop this
What is the pathophysiology of paroxysmal nocturnal hemoglobinuria?
Impaired GPI anchor for decay-accelerating factor that protects RBC from complement –> more complement destruction.
What are the clinical findings of paroxysmal nocturnal hemoglobinuria?
Coombs negative hemolytic anemia, pancytopenia, and venous thrombosis
What are the lab findings of paroxysmal nocturnal hemoglobinuria?
CD55/59 negative RBCs on flow cytometry
What is the tx of paroxysmal nocturnal hemoglobinuria?
Eculizumab
What is the mutation of HbS?
Substitution of glutamic acid with valine at position 6 of globin gene
What is the treatment of HbSS?
Hydroxyurea (increases fetal Hb)
Is transferrin production high or low in pregnancy and OCP use?
High (primary)
What are the presenting symptoms of acute intermittent porphyria?
5 Ps: Painful abdomen, Port wine-colored urine, Polyneuropathy, Psych disturbances, Precipitated by drugs, alcohol, starvation
What is the treatment of acute intermittent porphyria?
Glucose and heme which inhibit ALA synthase
What is the affected enzyme and the presenting symptom of porphyria cutanea tarda?
Uroporphyrinogen decarboxylase; blistering cutaneous photosensitivity
What is the presentation of Wiskott-Aldrich syndrome?
Thrombocytopenic purpura, eczema, recurrent infections
What is the presentation of Job syndrome?
FATED: course Facies, cold staphylococcal Abscesses, retained primary Teeth, hyper IgE, Derm problems (eczema)
What is the treatment of vWF disease?
DDAVP which releases vWF stored in endothelial W-P bodies
What are the lab findings of Antithrombin III deficiency?
No direct effect on PT, PTT, or thrombin time, but diminishes increase in PTT following heparin administration because heparin works on antithrombin
If a patient has skin and subcutaneous tissue necrosis after warfarin administration, what disease might they have?
Protein C or S deficiency
Packed RBCs are used to treat what?
Acute blood loss, severe anemia
Fresh frozen plasma is used to treat what?
DIC, cirrhosis, warfarin OD, exchange transfusions in TTP/HUS (increases coag factor levels)
What does cryoprecipitate contain? What does it treat?
Fibrinogen, factor VIII, factor XIII, vWF, and fibronectin; Coag factor deficiencies involving fibrinogen and factor VIII
What is a leukemoid reaction?
Acute inflammatory response to infection. Increased WBC count with increased neutrophils and neutrophil precursors such as band cells (left shift); increased leukocyte ALP. Contrast with CML (also increased WBC count with left shift, but less leukocyte ALP).
What type of cancer presents with constitutional “B” signs and contiguous spread?
Hodgkin lymphoma
What are the CD markers of Reed-Sternberg cells (B cell origin)?
CD15 and CD30
Which form of Hodgkin lymphoma has the best prognosis?
Lymphocyte-rich
Which form of Hodgkin lymphoma has the worst prognosis?
Lymphocyte-mixed or depleted forms
Which form of Hodgkin lymphoma is the most common?
Nodular sclerosing
EBV can lead to what kind of lymphomas?
Burkitt lymphoma and Hodgkin lymphoma
Describe the genetics of Burkitt lymphoma
t(8;14) translocation of c-myc (8) and heavy-chain Ig (14)
Describe the genetics of diffuse large B cell lymphoma
t(14;18) or sporadic
Describe the genetics of Mantle cell lymphoma
t(11;14) - translocation of cyclin D1 (11) and heavy chain Ig (14)
Describe the genetics of Follicular lymphoma
t(14;18) - translocation of heavy-chain Ig (14) and bcl-2 (18)
Which lymphoma is CD5+?
Mantle cell
Follicular lymphoma can progress to what?
Diffuse large B cell lymphoma
How does adult T-cell lymphoma present?
Cutaneous lesions, lytic bone lesions, hypercalcemia
What is mycosis fungoides/Sezary syndrome?
Mature T cell neoplasm; Adults present with cutaneous patches/plaques/tumors with potential to spread to lymph nodes and viscera. Circulating malignant cells seen in Sezary syndrome.
Monoclonal gammopathy of undetermined significance (MGUS) is an asymptomatic precursor to what?
Multiple myeloma
What is Pseudo-pelger-huet anomaly?
Neutrophils with bilobed nuclei typically seen after chemotherapy
Which cancer has smudge cells in the blood smear?
Small lymphocytic lymphoma/chronic lymphocytic leukemia
Which leukemia stains TRAP positive?
Hairy cell leukemia
What is the treatment for hairy cell leukemia?
Cladribine (2-CDA)
What is the median age of onset of AML?
65 YO
Auer rods are seen where?
In AML
The philadelphia chromosome (t[9;22] bcr-abl) is associated with what neoplasm?
CML
CML responds to what treatment?
Gleevac (imatinib)
How does Langerhans cell histiocytosis present?
In a child as lytic bone lesions and skin rash or as recurrent otitis media with a mass involving the mastoid bone.
The cells of Langerhans cell histiocytosis express what?
S-100 and CD1a
How does Langerhans cell histiocytosis look on EM?
Birbeck granules (tennis rackets)
Chronic myeloproliferative disorders are associated with what mutation?
JAK2
How does PV present?
Intense itching after a hot shower
Which endocrine hormones use cGMP for signaling?
ANP, NO
Which endocrine hormones use IP3 for signaling?
GnRH, oxytocin, ADH (V1), TRH, histamine (H1), AT-II, gastrin
Which endocrine hormones use a steroid receptor for signaling?
VitD, estrogen, testosterone, progesterone, T3/T4, cortisol, aldosterone
Which endocrine hormones use an intrinsic tyrosine kinase for signaling?
Insulin
Which endocrine hormones use cAMP for signaling?
Glucagon, GHRH, calcitonin, PTH, MSH, ADH (V2), hCG, CRH, TSH, ACTH, FSH/LH
Which endocrine hormones use a receptor-associated tyrosine kinase for signaling?
Prolactin, immunomodulators, GH
What is the enzyme of thyroid peroxidase?
Oxidation and organification of iodide as well as coupling of monoiodotyrosine (MIT) and di-iodotyrosine (DIT)
What is the Wolff-Chaikoff effect?
Excess iodine temporarily inhibits thyroid peroxidase leading to less iodine organification and less T3/T4
What is Cushing disease?
ACTH-secreting pituitary adenoma
How does cortisol suppress the immune system?
Inhibits histamine, IL-2, and PLA2
What is Conn syndrome?
Aldosterone-secreting adrenal adenoma
What is Waterhouse-Friderichsen syndrome?
Acute primary adrenal insufficiency due to adrenal hemorrhage associated with Neisseria meningitidis septicemia, DIC, and endotoxic shock
What is the most common tumor of the adrenal medulla in children?
Neuroblastoma -
What is the most common tumor of the adrenal medulla in adults?
Pheochromocytoma - from chromaffin cells - rule of 10s
Hashimoto thyroiditis has an increased risk of what malignancy?
non-Hodgkin lymphoma
What are the findings of cretinism (congenital hypothyroidism)?
Pot-bellied, pale, puffy face, protruding umbilicus, protuberant tongue, poor brain development
What is subacute thyroiditis (de Quervain)?
Self-limited hypothyroidism often following a flu-like illness. Granulomatous inflammation. High ESR, jaw pain, tender thyroid.
What is Riedel thyroditis?
Thyroid replaced by fibrous tissue (hypothyroid). Fibrosis may extend to local structures, mimicking anaplastic carcinoma. Fixed, hard and painless goiter.
What is the most common thyroid cancer?
Papillary carcinoma. Excellent prognosis. Orphan Annie eyes. Psammoma bodies, nuclear grooves, increased risk with ret and BRAF mutations, childhood irradiation.
Medullary carcinoma of the thyroid arises from what cells?
Parafollicular “C cells” - produces calcitonin. Sheets of cells in an amyloid stroma. Hypocalcemia. Neuroendocrine.
What is osteitis fibrosa cystica?
Cystic bone spaces filled with brown fibrous tissue. A consequence of hyperparathyroidism. Stones, bones, groans, and pyschiatric overtones.
What is renal osteodystrophy?
Renal disease –> less VitD –> hypocalcemia –> secondary hyperparathyroidism –> bone lesions
Describe pseudohypoparathyroidism
Aka Albright hereditary osteodystrophy - aut dom. Unresponsiveness of kidney to PTH. Hypocalcemia, shortened 4th/5th digits, short stature.
What are the findings of SIADH?
Excessive water retention, hyponatremia with continued urinary Na excretion, urine osmolarity > serum osmolarity
What is the pancreatic histology in DM Type 2?
Islet amyloid polypeptide (IAPP) deposits
What is the most common malignancy of the small intestine?
Carcinoid tumor
Where are Zollinger-Ellison tumors usually found?
Pancreas or duodenum
Which MEN is associated with Marfanoid habitus?
MEN2B
Which fungi is acquired through bird or bat droppings?
Histoplasmosis
What is the prophylaxis treatment for PCP?
TMP-SMX
How does Sporothrix present?
Spores traumatically introduced into skin, local pustule or ulcer with nodules along draining lymphatics (ascending lymphangitis). Little systemic illness. Tx: Itraconazole or potassium iodide.
How does congenital toxoplasmosis present?
Chorioretinitis, hydrocephalus, and intracranial calcifications
What disease does Naegleria fowleri cause?
Rapidly fatal meningoencephalitis, primary amebic encephalitis (PAM)
What is the MOA of chloroquine?
Blocks Plasmodium heme polymerase
If resistant to chloroquine, which meds are used for malaria?
Mefloquine or atovaquine/proguanil. If life-threatening, use IV quinidine.
If infected with vivax/ovale, which medication is added on in addition to regular ones?
Primaquine for hypnozoite
How does Babesiosis present?
Fever and hemolytic anemia. NE US. Maltese cross.
How is Babesiosis treated?
Atovaquone and azithromycin.
How does visceral Leishmaniasis present?
Spiking fevers, hepatosplenomegaly, pancytopenia. 100% fatal if untreated. Macs contain amastigotes.
What is the pathophysiology of osteopetrosis?
Failure of normal bone resorption due to defective osteoclasts. Thick dense bones that are prone to fracture.
What is the most common mutation involved with osteopetrosis?
Mutation in carbonic anhydrase II - osteoclast can’t create acidic environment it needs
Which intracranial hemorrhage can cross suture lines and which cannot?
Subdural can, epidural cannot
Which intracranial hemorrhage can cross falx and tentorium and which cannot?
Epidural can, subdural cannot
Intraparenchymal hemorrhages usuall occur where?
Basal ganglia and internal capsule
What is a Charcot-Bouchard microaneurysm?
Associated with chronic hypertension; affects small vessels like in the basal ganglia and thalamus
What event leads you to be “wet wobbly and wacky?”
Normal pressure hydrocephalus
Tabes dorsalis affects what part of the spinal cord?
Demyelination of dorsal columns and roots
MS affects what part of the spinal cord?
Random asymmetric lesions; mostly white matter of cervical region
AML affects what part of the spinal cord?
Both UMN and LMN, ant horns and lateral corticospinal tract
VitB12 or VitE deficiciency affects what part of the spinal cord?
Demyelination of dorsal columns, lateral corticospinal tracts, and spinocerebellar tracts; ataxic gait, paresthesia, impaired position and vibration sense
Which CN passes through the cribriform plate?
CN1
Which CNs pass through the middle cranial fossa and through the sphenoid bone?
CNs II-VI
What passes through the optic canal?
CN II, ophthalmic artery, central retinal vein
Which CNs pass through the superior orbital fissure?
CN III, IV, V1, VI, ophthalmic vein, sympathetic fibers
Which CNs pass through the foramen rotundum?
CN V2
Which CNs pass through the foramen ovale?
CN V3
What passes through the foramen spinosum?
Middle meningeal artery
Which CNs pass through the posterior cranial fossa and through the temporal or occipital bones?
CN VII-XII
Which CNs pass through the internal auditory meatus?
CNs VII and VIII
What passes through the jugular foramen?
CN IX, X, XI jugular vein
Which CNs pass through the hypoglossal canal?
CN XII
Which CNs pass through the foramen magnum?
Spinal roots of VN XI, brain stem, vertebral arteries
What passes through the cavernous sinus?
CN III, IV, V1, V2, VI and postganglionic sympathetic fibers en route to the orbit
Uvula deviation indicates what?
CN X lesion - uvula deviates away from side of lesion
Jaw deviation indicates what?
CN V motor lesion - jaw deviates toward side of lesion
What are the 3 muscles of mastication?
Masseter, temporalis, medial pterygoid
What is a Marcus Gunn pupil?
Afferent pupillary defect. Due to optic nerve damage or severe retinal injury.
What is scotoma?
Loss of central vision