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Tom wangers high yield - path Flashcards

1
Q

Honeycomb lung

A

Ideopathic Pulmonary fibrosis. Fibrosis mediated by TGF-B from injured Pneumocytes

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2
Q

unexplained Pleural Effusion in young woman

A

Lupus (until proven otherwise)

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3
Q

Egg shell Calcification of Hilar Nodes

A

Silicosis. Quartz–>Birefringent

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4
Q

Calcified plaque on Dome of Diaphragm/pleura

A

Asbestosis

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5
Q

Curshman Spirals and Charcot Leiden crystals

A

Asthma

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6
Q

Nasal Polyps in a child

A

Cystic Fibrosis

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7
Q

Nasal Polyps in an adult female

A

long-time aspirin use or allergies

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8
Q

Vinyl Chloride, Arsenic, Thorotrast

A

Liver Angiosarcoma

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9
Q

Myasthenia Gravis

A

Thyoma or Thyroid Follicular B cell hyperplasia

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10
Q

Bilateral Internuclear Opthalmoplegia

A

Multiple Sclerosis

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11
Q

Non-bilious projectile vomiting in a 3-5 week old

A

Congenital Pyloric Stenosis. Can be acquired if infant treated w/ erythromycin when

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12
Q

False diverticulum through Cricopharyngeus m.

A

Zenker Diverticulum

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13
Q

Giant rugal folds w/ mucus cell hyperplasia

A

Menetrier’s disease. Protein Losing Enteropathy, Atrophy of parietal cells, increased risk for adenocarcinoma

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14
Q

Bleeding in Gastric Ulcer

A

Left Gastric Artery

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15
Q

Perforation of Posterior Duodenal Ulcer

A

Pancreatitis or bleeding from Gastroduodenal artery

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16
Q

High Gastrin, low stomach acidity

A

Autoimmune chronic atrophic Gastritis. pernicious anemia

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17
Q

High Gastrin, high stomach acid

A

Zollinger Ellison. Pancreas mass, multiple/distal ulcers, MEN1

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18
Q

Signet Ring cells in Ovaries

A

Krukenberg Tumor. from Diffuse Gastric Adenocarcinoma

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19
Q

Severe pain in LUQ after eating

A

Ischemic colitis. splenic flexure

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20
Q

Pseudopolyps and crypt abscesses

A

Ulcerative colitis. Risk of Toxic Megacolon

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21
Q

Submucosal noncaseating granulomas

A

Crohn’s Disease

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22
Q

Skin flushing, diarrhea, Tricuspid Regurgitation

A

Carcinoid tumor from Ileum metastasized to liver. increased 5-HIAA in urine; must be in liver for Carcinoid Syn.

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23
Q

AST: ALT at least 2:1 and

A

Alcoholic Liver disease. GGT and ALP also elevated, but GGT vastly more elevated

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24
Q

Increased ALP and GGT

A

Cholestasis. Inc ALP w/o inc GGT–> osteoblastic activity in bone

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25
Q

Beading of the Bile ducts on ERCP

A

Primary Sclerosing Cholangitis. Jaundice, Severe Pruritis, and Hepatosplenomegaly

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26
Q

Antimitochondrial Antibodies. mediated by what cell?

A

Primary Biliary Sclerosis. CD8 Tcell mediated granulomatous destruction of intrahepatic bile duct epithelium

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27
Q

Cirrhosis in a child

A

A1AT deficiency

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28
Q

Strictures, cobblestone, creeping fat

A

Crohn’s Disease. String sign

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29
Q

Tumor at Junction of R and L Hepatic Ducts

A

Klatskin Tumor. Type of Cholangiocarcinoma

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30
Q

Superficial Migratory Thrombophelbitis (moving DVTs)

A

Pancreatic Cancer (Trousseau’s Syndrome). CA19-9 monitored for remission; Painless Jaundice

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31
Q

RBC casts and linear immunostaining

A

Goodpastures disease. Anti-basement membrane Ab (collagen type 4)

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32
Q

Deposits IN the Glomerular BM

A

Type 2 Membranoproliferative Glomerulonephritos. C3 Nephritic Factor and very low levels of serum C3–>C3 nephritic factor stabilizes C3 convertase. C3 Nephritic Factor and very low levels of serum C3–>C3 nephritic factor stabilizes C3 convertase

(Tram Tracks and subendothelial deposits in type 1. )

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33
Q

Fever, Flank Pain, WBC casts

A

Acute Pyelonephritis

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34
Q

Thyroidization of the kidney

A

Chronic Pyelonephritis

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35
Q

Bilateral Renal Cell Carcinoma, Hemangioblastoma in retina or cerebellum

A

Von Hippel Lindau. VHL on Chr 3; clear cells contain lipid and glycogen; Cerebellar Hemangiomas

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36
Q

Unilateral palpable flank mass in child w/ HTN

A

Wilm’s Tumor. Contains abortive glomeruli and tubules, primitive blastemal cells,( and rhabdomyoblasts?)

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37
Q

Sudden onset of testicular pain post trauma and absent cremaster reflex

A

Testiticular torsion

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38
Q

Biggest risk factor for testicular cancer

A

Cryptorchid testicle. especially if intra-abdominal

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39
Q

Testicular cancer in a guy >50

A

Malignant Lymphoma

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40
Q

Delayed puberty and anosmia

A

Kallmann’s Syndrome. Failure of migration of GnRH secreting cells and olfactory bulbs

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41
Q

Best Marker for Menopause

A

Increased FSH. LH will also be increased, estradiol will be decreased

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42
Q

Most common cause of infertility & Ectopic Pregnancy

A

PID

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43
Q

Tumor with Reinke Crystals

A

Leydig Cell Tumor. may produce androgens

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44
Q

First test if Pregnant woman w/ vaginal bleeding

A

Pelvic Ultrasound. Do NOT do pelvic exam; Painless (placenta previa); Painful + Contractions (Abruptio Placentae)

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45
Q

Amioncentesis with Inc AFP? Dec AFP?

A

Inc: Neural Tube Defect; Dec: Down Syndrome. Down Syndrome : Dec AFP, Dec urine estriol, and Increased B-hCG and inhibin A

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46
Q

Hypothyroidism w/ germinal centers on biopsy

A

Hashimoto’s Thyroiditis. Lymphocyte infilitrate; if mixed immune infiltrate and painful, think thyroiditis (DeQuervain)

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47
Q

Cancers secreting PTHrP

A

SqCC of Lung and Renal Cell Carcinoma

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48
Q

Syndromes w/ Pheochromocytoma

A

Neurofibromatosis 2, MEN IIa and IIb, Von Hippl Lindau (bilateral)

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49
Q

Chaotic eye movements and myoclonic jerks in a child

A

Neuroblastoma. Usually located in Adrenal gland; N-Myc amplification; Homer Wright Rosettes

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50
Q

Secretory Diarrhea and Achlorhydria

A

VIPoma. can be tx w/ octreotide

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51
Q

Eczema, Thrombocytopenia, and Recurrent infections

A

Wiscott Aldrich. X linked deficiency of B and T lymphocytes with eczema and thrombocytopenia

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52
Q

Port wine stain w/ ipsilateral leptomeningeal angioma, seizures

A

Sturge Weber Syndrome

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53
Q

Ash Leaf spots, CNS hamartomas, subependymal astrocytomas, cardiac rhabdomyoma, renal angiomyolipoma

A

Tuberous Sclerosis

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54
Q

Bitemporal Hemianopia and headaches in a child

A

Craniopharyngioma. Derived from Rathke’s pouch, oral ectoderm, same as Anterior Pituitary

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55
Q

Bitemporal Hemianopia and headaches in an adult

A

Pituitary Adenoma. Most commonly prolactinoma

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56
Q

Solid tumor w/ small blue cells in cerebellum of child

A

Medulloblastoma. Drop Metastasis

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57
Q

Pseudopalasading tumor cells around necrosis

A

Glioblasoma multiforme

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58
Q

New Onset Seizures due to whorled tumor

A

Meningioma. Psammoma bodies

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59
Q

Tinnitis, Vertigo, and Hearing loss

A

Meniere Disease. Increased volume of endolymph in inner ear due to defective reabsorption

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60
Q

Fluctuating Consciousness, hallucinations, and dementia; late Parkinsonian effects

A

Lewy Body Dementia

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61
Q

coarse facial features, clouded corneas, high plasma lysosomal enzymes, restricted joint movement

A

I cell Disease. Failure of Golgi to add Mannose-6-Phosphate signal to proteins destined for Lysosomes–>excreted instead

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62
Q

Bronchiectasis/Recurrent Pulmonary infections, infertility, and Situs Inversus

A

Kartagener’s Disease. dynein arm defect in cilia

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63
Q

Telangiectasia, Epsitaxis, GI bleeding, skin discoloration, AV Malformations

A

Osler-Weber-Rendu Syndrome (Hereditary hemorrhagic telangiectasia)

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64
Q

Duchenne Muscular Dystrophy mutation

A

X-linked Frameshift mutation

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65
Q

Becker Muscular Dystrophy mutation

A

X-linked Point mutation

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66
Q

Difficulty releasing grib/doorknob, muscle wasting, frontal balding, cataracts, testicular atrophy

A

Myotonic Muscular Dystrophy. CTF trinucleotide repeats in DMPK gene; Distal Weakness in hands/feet; Type 1 fibers more affected

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67
Q

Macroorchidism, Large Jaw, large/everted ears, mitral valve prolapse, developmental delay

A

Fragile X Syndrome. X linked trinucleotide repeats affecting the METHYLATION of FMR1 gene (fragility only describes in vitro not in vivo);

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68
Q

Rocker bottom feet, lowset ears, micrognathia

A

Edwards. Trisomy 18

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69
Q

Rocker bottom feet, cleft lip/palate, holoprosencephaly, polydactyly

A

Patau. Trisomy 13

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70
Q

Microcephaly, High pitched crying, epicanthal folds, severe intellectual disbility, VSD

A

Cri du chat. Chr 5 short arm deletion

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71
Q

Asymptomatic accumulation of fructose in blood/urine

A

Essential Fructosuria (Defect in Fructokinase)

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72
Q

Intellectual disability, jaundice, failure to thrive, hepatomegaly, and cataracts in an infant

A

Classic Galactosemia: Defect in Galactose-1-P Uridyltransferase–>accumulation of galactitol and depletion of phosphate

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73
Q

Infantile Cataracts and failure to track objects with galactose in blood/urine

A

Galactokinase Deficiency: accumulation of Galactitol due action of Aldolase reductase on galactose

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74
Q

Hypoglycemia, jaundice, cirrhosis, and vomiting in an infant after consuming juice/fruit

A

Fructose Intolerance: Deficiency of Aldolase B (Auto rec), accumulation of Fructose-1-p causes decreased phosphate, which inhibits glycogenolysis and gluconeogenesis

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75
Q

Hyperammonemia with excess orotic acid

A

Ornithine Transcarbamylase Deficiency: X-linked recessive, often evident in first few days of life: Increased Orotic Acid, Decreased BUN, Hyperamonemia symptoms (Kernicterus, somnolence, vomiting, cerebral edema)

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76
Q

Excess orotic acid with Megaloblastic Anemia

A

Orotic Aciduria: deficiency of UMP Synthase, Megaloblastic anemia NOT treated with folate or vit B12 supplement

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77
Q

Intellectual disability, mousy.musty body odor, seizures, and eczema corrected with Tetrahydrobiopterin supplementation

A

Malignant PKU: Deficiency in Dihydrobiopterin Reductase; May also present with Prolactinemia because Tetrahydrobiopterin is also cofactor for Tyrosine hydroxylase–> decreased activity causes decreased DOPA, which causes decreased Dopamine, which removes inhibiton of prolactin secretion

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78
Q

Dark connective tissue, brown pigmented sclera, urine turns black w/ prolonged air exposure, Debilitating arthralgia

A

Alkaptonuria: Deficiency of Homogenisate oxidase–> accumulation of homogentisic acid and failure to breakdown tyrosine to Fumarate

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79
Q

Osteoporosis, Tall stature, kyphosis, lens subluxation (down and in), thrombosis/atherosclerosis w/ stroke and MI risk

A

Homocystinuria (Elevated Homocystine in urine): Due to Deficiency of either: MethionineCystathionine–>Cysteine

  • Cystathionine Synthase –>supplement with inc cysteine, B6/pyridoxine, and dec methionine
  • Homocysteine Methyltransferase (Methionine Synthase) Deficiency – inc methionine in diet
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80
Q

Renal Cystine stones (hexagonal)

A

Cystinuria. Hereditary defect in Renal Proximal tubule and intestinal reaborption of Cysteine, Ornithine, Lysine, and Arginine

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81
Q

Severe retardation, disability, and sweet smelling urine

A

Maple Syrup Urine Disease. Deficiency in Alpha-Ketoacid Dehydrogenase (uses thiamine)–>blocks degradation of Branched amino acids (Valine, Leucine, Isoleucine), Isoleucine causes burnt sugar urine smell

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82
Q

Glycogen storage disease causing cardiomyopathy

A

Pompe Disease (Type II). Deficiency in Lysosomal a-1,4-glucosidase (acid maltase)

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83
Q

Glycogen storage disease causing painful muscle cramps, myoglobinuria, and arrhthmias

A

McArdle Disease (Type V). Deficiency in Myophosphorylase (skeletal muscle glycogen phosphorylase)

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84
Q

Glycogen storage disease with hypoglycemia and accumulation of short outer Dextrin-like structures

A

Cori Disease (Type III). Deficiency in Debranching enzyme (a-1,6-glucosidase)

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85
Q

Lysosomal Storage disease with Aseptic necrosis of the femur, pancytopenia, hepatosplenomegaly, and bone crisises

A

Gaucher’s Disease. Most common LSD, Gaucher cells (lipid-laden macrophages that look like crumpled paper)
Glucocerbrosidase deficiency–>accumulation of Glucocerebroside

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86
Q

Jaundice (CB) in a less than 2 month old

A

biliary atresia (+acholic stool)

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87
Q

Jaundice (CB) in a 2-10 year old

A

Biliary cyst. (70% have abnormal pancreatobiliary junction). If UCB, think Crigler Najjar

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88
Q

Microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure

A

Hemolytic Uremic Syndrome. Shigella or EHEC

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89
Q

Diarrhea that is initially watery then becomes bloody and rose spots appear on the abdomen

A

Salmonella typhi. Can cause dramatic inflammation in Peyer’s pathes–> intestinal hemorrhage/perforation and death; can colonize the gallbladder

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90
Q

Retroperitoneal Hematoma following trauma (Motor crash)

A

Bleeding from Pancreas. Spleen is intraperitoneal

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91
Q

Pt w/ S3 gallop, orthopnea/dyspnea, and MR is tx w/ diuretic and both heart sounds disappear; What was the pathophys of the MR?

A

Functional Mitral Regurgitation. S3 gallop indicated volume overload, which the diuretic treated; elimination of the holosystolic murmur @ the apex means the valves were normal, just stretched apart

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92
Q

Cerebral tumor: S-100+, elongated cell w/ regular oval nuclei, and alternating areas of high and low cellularity

A

Schwannoma

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93
Q

Well circumscribed tumor at grey/white matter junction

A

Metastatic brain tumor. often multiple, often from lungs

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94
Q

Multiple liver nodules w/o increased AFP

A

Metastatic liver cancer. Usually from colon

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95
Q

Fixed wide splitting of S2

A

ASD

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96
Q

Inspiration decreases the A2/P2 splitting

A

Paradoxical Split S2. Aortic stenosis or Left Bundle Branch block

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97
Q

Both Aortic and Pulmonic regurgitation is heard where?

A

Left Sternal border. Aortic Regurg not heard at the aortic area (stenosis/sclerosis of valves only)

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98
Q

Systolic murmur at the Left Sternal Border

A

Hypertrophic Cardiomyopathy. Murmur involves the hypertrophied interventricular septum and MITRAL VALVE

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99
Q

Valsalva increased the intensity of one murmur only, which?

A

Hypertrophic Cardiomyopathy. Obstruction greater at lower ventricular volume

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100
Q

Sign a person w/ severe volume overloaded or hypertrophied heart failure has Afib

A

Disappearence of S4. S4 is caused by atrial contraction

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101
Q

Murmur that radiates to carotids and causes Pulsus Parvus et tardus

A

Aortic stenosis. intensity increases w/ increased venous return (opposite of hypertrophic cardiomyopathy)

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102
Q

Young pt w/ recurrent episodes of syncope and deafness

A

Jervell Lange Nielsen Syndrome. Autosomal recessive Long QT Syndrome; Romano-Ward is AutoDom (cardiac only)

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103
Q

Progressive lengthening of the PR interval until beat is dropped, followed by normal PR

A

Mobitz Type 1 Second Degree Heart Block

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104
Q

Pacemaker for the ventricles in 3rd degree Heart block

A

AV node (45-55 bpm)

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105
Q

Recominant form of B-type Natuiretic peptide used to treat heart failure

A

Nesiritide. BNP is normally released form the Ventricular Myocytes and acts like ANP–>vasodilation, decrease Na reabsorption in collecting tubule, Increase GFR (constrict efferent and dilate afferent)

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106
Q

Reccurent Epistaxis,spider like skin lesions, and guiaiac positive stool

A

Osler-Weber-Rendu Syndrome (Hereditaty hemorrhagic telangiectasia)

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107
Q

Ischemic Neuron Injury histology

A

“Red Neuron” w/ eosinophilic cytoplasm, Loss of Nissl, Pykinosis of nucleus

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108
Q

Axonal Damage/Axonal Reaction Histology

A

Wallerian degeneration: Swollen neuron body, dispersion of Nissl, eccentric nucleus w/ large nucleolus

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109
Q

Ulcers where in the GI tract are virtually never malignant

A

Duodenal ulcers. Gastric ulcers have a high potential of being malignant and must be biopsied

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110
Q

Fern Leaf marks (Lichtenburg figures), Secondary burns, and death via arrthymia

A

Person died from lightening strike. Lichtenberg figures are characteristic; death via arrhythmia or respiratory depression

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111
Q

Bilateral absence of the Vas Deferens

A

Cystic Fibrosis. Azoospermia

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112
Q

Parotid tumor with germinal centers

A

Warthin Tumor (Most common parotid tumor is pleomorphic adenoma (stroma and epithelial))

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113
Q

2 things suspected if abdominal pain with meals

A

gastric ulcer and ischemic colitis. Ischemic colitis is typically 30-60 min post meal and pt has wt loss

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114
Q

Histology of Reye Syndrome

A

Microvesicular fatty change. NOT necrosis

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115
Q

Air in the gallbladder/biliary tree w/ bowel obstruction

A

Gallstone ileus. Fistula between gallbladder and small intestine; due to longstanding cholelithiasis

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116
Q

Cerebellar defects/atrophy/difficulty walking, spider angiomas, and recurrent respiratory infections

A

Ataxia-Telangectasia. Defect in DNA double strand break repair

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117
Q

Reccurent Hemorrhagic strokes in an elderly person

A

Cerebral Amyloid Angiopathy. Ab amyloid, usually lobar strokes and are less severe

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118
Q

Child presents w/ enlarged testicle w/ fluid in scrotum

A

Hydrocele. Fluid in Tunica Vaginalis, communicating hydrocele if processus vaginalis is patent; Tunica vaginilis derived from the peritoneum; highly associated w/ inguinal hernia

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119
Q

Acute Painless monocular vision loss

A

Central Retinal Artery occlusion. Pale retina w/ Cherry Red macula; commonly associated w/ Afib

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120
Q

Coffee ground emesis

A

Blood exposed to gastric acid

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121
Q

BRAF V600E mutation. Treatment for dz?

A

Malignant Melanoma. Vemurafenib–inhibits B-Raf w/ V600E mutation; Often metastasizes to the brain, causing seizures

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122
Q

Jejunal and distal duodenal ulcers, diarrhea

A

Gastrinoma/Zollinger Ellison

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123
Q

Severe Abdominal Pain, Port-wine colored urine, polyneuropathy, and psychological disturbances

A

Acute Intermittant Porphyria. Defect in Porphrobilinogen Deaminase causes buildup of porphrobilinogen and ALA; TX w/ glucose and Heme to inhibit ALAS. Associated with CYP inducers. Defect in Porphrobilinogen Deaminase causes buildup of porphrobilinogen and ALA; TX w/ glucose and Heme to inhibit ALAS

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124
Q

Neutrophils with bilobed nuclei (no effect on activity)

A

Pelger-Huet Anomaly. seen after chemotherapy–>two nuclear masses connected w/ a thin chromatin filament

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125
Q

Autoimmune hemolytic anemia, smudge cells, CD5/Cd23+

A

CLL

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126
Q

Lytic bone lesions in a child, skin rash, +/- recurrent otitis media w/ a mass involving the mastoid bone

A

Langherhans cell Histiocytosis. Birbeck granules, S-100+ (mesodermal origin)

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127
Q

Scalp rash, Lytic skull defects, Diabetes Insipidus, and exophalthmos is a child

A

Hand-Christian-Schuller Diesease. Malignany proliferation of Langerhans cells

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128
Q

3 conditions associated w/ lytic bone lesions

A

Multiple Myeloma, Langerhans cell Histiocytosis, Adult T-cell Lymphoma

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129
Q

Complications of Paget’s disease of bone

A

High Output Heart Failure (AV shunts in bone)

Osteosarcoma. Also associated w/ paramyxoviruses

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130
Q

Trabecular thinning w/ fewer interconnections

A

Osteoporosis. ALL labs NORMAL

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131
Q

Persistance of primary, unmineralized spongiosa in the medullary canals w/ no mature trabecullae

A

Osteopetrosis. unlike normal bone, where marrow replaces the primary spongiosa–>Pancytopenia. TX w/ BM transplant b/c osteoclasts are from BM

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132
Q

Osteoid matrix accumulation around trabeculae

A

Osteomalacia/rickets. Decreased Ca, PO4 with increased ALP and PTH

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133
Q

Mosiac pattern of woven and lamellar bone

A

Paget Disease of Bone. Isolated increase in ALP

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134
Q

Polyostotic fibrous dysplasia, precocious puberty, Cafe-au-Lait macules

A

McCune-Albricht Syndrome. Multiple unilateral bone lesions

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135
Q

Hyperkeratosis and parakaratosis? which layer?

A

Psoriasis. Increased thickness of stratum corneum (hyper) and retained nuclei in stratum corneum (para)

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136
Q

Epidermal hyperplasia in response to GI malignancy or insulin resistance. which layer?

A

Ancanthosis Nigrecans. Ancanthosis = epidermal hyperplasia (increased spinosum)

137
Q

Hyperorality, hypersexuality, disinhibited behavior

A

Kluver Bucy Syndrome. Associated w/ bilateral Amygdala lesions–>HSV-1

138
Q

Dysphagia, hoarseness, Checkerboard loss of pain and temp (ipsi face, contra body)

A

Wallenberg Sydrome/Lateral Medullary Syndrome. PICA stroke

139
Q

Stroke causing facial N paralysis

A

AICA. facial nucleus effects are specific to AICA strokes–> Lateral Pontine Syndrome

140
Q

Contralateral Paralysis w/ ipsilateral CN3 lesion

A

Weber Syndrome. Midbrain stroke (paramedian branch of PCA)

141
Q

Ipsilateral CN3 lesion and Ipsilateral paralysis

A

Transtentorial/Uncal herniation. b/c contralateral midbrain peduncle compressed (fibers cross over below)

142
Q

Berry Aneursym in posterior communicating hits what CN

A

CN3. Eyes “down and out”

143
Q

Infant w/ hypotonia and tongue fasciculations

A

Spinal Muscular Atrophy/Werndig Hoffman

144
Q

Duchenne Muscular Dystrophy mutation

A

dystrophin. X linked. frameshift mutation.

point mutation: becker

145
Q

Pt unable to look upward

A

Parinaud sydrome. Compression of Superior collilculi, usually by a Pinealoma

146
Q

Hypertension and proteinuria (prego)

A

Hydatiform mole. after 20 weeks is Pre-eclampsia

147
Q

Precocious puberty and testicular mass in young boy

A

Leydig Cell Tumor

148
Q

Granulomatous heart nodules

A

Aschoff Bodies/Rheumatic Fever

149
Q

Most common cause of Increased AFP on amniocentesis

A

Dating Error. If pathology, neural tube defects

150
Q

Lack of Xylose absorption

A

Small Bowel Disease. Xylose does not require pancreatic enzymes. Pancreatic problems will have normal xylose absorption

151
Q

Korotkoff sounds heard only on expiration at pressures >10mmHg they are heard thought respiratory cycle

A

Pulses paradoxus. Tamponade, constrictive pericarditis, severe Obstructive lung disease, restrictive cardiomyopathy

152
Q

Air in biliary tree and bowel obstruction

A

Gallstone Ileus

153
Q

Connective Tissue in muscle converting to bone (metaplasia) post-trauma

A

Myositis ossificans

154
Q

Non-caseating granulomas in the axillary lymph nodes

A

Cat Scratch disease (Bartonella hensae)

155
Q

Factors that increase angiogenesis

A

VEGF, FGF, PDGF. VEGF and FGF are commonly produced by tumors

156
Q

Three deficiencies that cause delayed wound healing

A

Vitamin C, Copper, Zinc: Dehiscence is rupture of a wound

157
Q

Keloid is caused by excess what

A

Type 3 collagen

158
Q

Rebleeding after surgery or wisdom teeth pull, hemarthrosis

A

Hemophilia

159
Q

Embolism w/ cholesterol clefts on histology

A

Atherosclerotic embolus

160
Q

Pt w/ broken long bone has trouble breathing and petechiae on chest

A

Fat embolus

161
Q

Embolism w/ squamous cells and keratin debris in postgravid

A

Amniotic fluid embolism. Squamous cells/keratin from fetal skin, associated w/ DIC

162
Q

Remnants in RBCs

A

Basophilic Strippling: rRNA : Lead, ACD, EToH, Thal
Heinz Bodies: Hemoglobin: G6PD (bite cells)
Howell Jolly bodies: Nuclear remants (asplenic pts: Sicke cell, hereditery sphercytosis post surgery)
Reticulocytes- RNA

163
Q

Extravascular Hemolysis vs Intravascular hemolysis labs

A

Extra: inc unconjugated bilirubin/bilirubin gallstones
Intra: Hemoglobinemia, Hemoglobinurea, Hemosiderinuria, Decreased Haptoglobin (Haptoglobin binds free Hb in blood; Hemopexin binds free heme)

164
Q

Major cause of death->Paroxysmal Nocturnal Hemoglobinuria

A

Thrombosis (hepatic, portal, cerebral). Pancytopenia due to lack of CD55/Decay Accelerating Factor due to loss of GPI anchors

165
Q

> 20% blasts leukemia that infiltrates the gums

A

Acute Monocytic Leukemia

166
Q

blood cancers associated w/ Down syndrome before age 5? After age 5?

A

Acute Megakaryoblastic Leukemia. After age 5 Down is associated w/ ALL

167
Q

Most common cause of death in CLL

A

Infection. Hypogammaglobulinemia due to cancer production of non-produtive Ab; Smudge cells and AIHA

168
Q

Lytic bone lesions w/ a rash in a pt from Japan/Caribbean

A

Adult T cell Leukemia. CD4 T cells and’ HTLV1 virus

169
Q

Pautrier Microabscesses

A

Mycosis Fungoides. Cd4 tcell tumor in skin, cerebriform nuclei when progresses to Sezary Syndrome

170
Q

Differentiates CML from Leukemoid Reactions

A

Leukocyte Alkaline Phosphatase stain Negative

Basophilia and t(9,22) Philly Chr

171
Q

Itching after showering, facial plethora, blurry vision, headache, Peptic ulcers, Gout

A

Polycythemia Vera: Increased risk for venous thrombosis; Symptoms due to increased blood viscosity; Increased Hematocrit w/ low EPO; Itching and peptic ulcers are due to increased histamine release from increased mast cells

172
Q

What Myeloproliferative Disorder is not associated w/ gout

A

Essential Thrombocytosis. excess platelets, which never have a nucleus

173
Q

Most common cause of death in Multiple Myeloma

A

death: Infection. s/s: Bone Lesions/pain, Hypercalcemia, Renal Insufficiency, Anemia, M spike, AL amyloidosis, Rouleaux, Eosinophilic casts of bence jones +Tamm Horsfall proteins

174
Q

B-cell lymphoma w/ IgM spike

A

Waldenstrom Macroglobulinemia. also called Lymphoplasmacytic Lymphoma; Hyperviscosity due to IgM pentamers (visual & neurological defects and bleeding due to platelet aggregation)

175
Q

Aortic Dissection is associated w/

A

Hypertension (old people)- Hyaline arteriolosclerosis of vaso vasorum
Marfan/Ehlers Danlos- Cystic Medial Necrosis. (s/s sharp tearing chest pain that radiates to the back)

176
Q

Hypotension, pulsatile abdominal mass, flank pain

A

ruptured abdominal aortic aneurysm

177
Q

Recurrent apthous ulcers, genital ulcers, and uveitis

A

Behcet Syndrome. Due to immune complex deposition in small vessels

178
Q

Hyperplasia of what cells occurs in Chronic autoimmune gastritis

A

Antral G cells. Increased gastrin release due to achlorhydria

179
Q

Duodenal Atresia vs Jejunal/Ileal atresia

A

Duodenal is due to failure to canalize, Jejunal is due to vascular interruption/ischemia. Duodenal atresia presents as polyhydramnios, bilous vomitting, and double bubble sign on x-ray

180
Q

Unexplained blood in the stool of a young child w/ abd pain

A

Meckel’s Diverticulum w/ gastric tissue

181
Q

Macrophages w/ PAS+ staining lysosomes in small bowel lamina propria, fat malabsorption and steatorrhea

A

Whipple disease (Trophyerma whippelii). Fat malabsortption due to macrophage compression of lacteals

182
Q

Atherosclerosis of SMA

A

Ischemic colitis at the splenic flexure. Postprandial pain and weight loss

183
Q

Thin, older person w/ sudden onset diabetes, suspect:

A

Pancreatic carcinoma. Smoking is the most common cause.

184
Q

Severe pruritis, pale stools, and dark urine

A

Biliary tract obstruction. (conjugated hyperbilirubinemia)

185
Q

Stain used to differentiate Iron vs. Lipofuschin accumulation in the liver

A

Prussian Blue. used in Dx of Hemochromatosis, Lipofuschin is peroxidized lipids in “wear and tear”

186
Q

Causes of Renal Papillary Necrosis

A

SCD/Trait, Chronic analgesic abuse (aspirin), Diabetes, Severe acute pyelonephritis, Phenoacetin (APAP). Presents as gross hematuria and flank pain

187
Q

Differentiation of Post-strep glomerulonephritis vs Membranous Nephropathy on EM

A

Both have subepithelial deposits but Membranous is covered by BM (spike-immune complex and dome-BM)

188
Q

Hematuria, palpable flank mass, and flank pain

A

Renal Cell Carcinoma. May also have paraneoplastic syndrome: EPO, Renin, ACTH, PTHrP

189
Q

Renal Cell Carcinoma is associated w/ what gene loss

A

VHL (chr 3, tumor suppressor). either sporadic (smoking, unilateral) or Von Hippl Lindau Disease (bilateral, +hemangioblastoma and Pheochromocytoma)

190
Q

Painless hematuria in a smoker

A

Urothelial/Transitional cell Carcinoma of bladder. Flat (early p53 mutation) or Papillary

191
Q

Secondary amenorrhea due to overaggressive D&C

A

Asherman Syndrome. will not respond to progesterone challenge due to loss of basalis and scarring

192
Q

Endometrial hyperplasia most likely to progress to carcinoma when what is seem? Exception to this rule?

A

Simple hyperplasia w/ atypia. atypia is most important. Only except to this rule is with prostate cancer in which the artecture of the cell is more importan and graded than atypia

193
Q

Older man w/ low back pain w/ Increased ALP, normal GGT

A

Prostatic adenocarcinoma w/ spread to lumbar spine.Osteoblastic lesion, PSA and Porstatic Acid Phosphatase (PAP) also elevated

194
Q

Apoptotic bodies seen in acute viral hepatitis

A

Councilman bodies

195
Q

Chronic fatigue, mild weight gain, and inc creatinin kinase

A

Hypothyroidism

196
Q

Adult Polycystic Kidney disease is associated w/ what cardiac path and GI path

A

Mitral Valve prolapse; Diverticulosis

197
Q

Anti-phospholipase A2 antibodies

A

Membranous Nephropathy

198
Q

Vasculitis of graft vessels w/ dense interstitial lymphocyte infiltrate

A

Acute graft rejection. Reaction against Donor MHC–>This is different from Chronic rejection, in which recipient cells accept donor MHC but react against the proteins presented by the donor MHC

199
Q

Bone Metastasis from Prostate and Breast

A

Prostate: Blastic; Breast: Blastic and Lytic

200
Q

What must be tested if a pt is being treated for Hyperthyroidism

A

WBC with differential.Methimazole and Propylthiouracil both cause agranulocytosis

201
Q

Ab present in T1DM

A

Anti-glutamic acid Decarboxylase

202
Q

First Cardiac sign of Congestive Heart Failure

A

S3. Best heard at apex in Left lateral decubitus position

203
Q

What changes in the JVP curve in Afib

A

No A wave. A wave corresponds to atrial contraction; also no S4 if one was present

204
Q

Why are bibasilar crackles sometimes not heard in pts w/ chronic Left heart failure

A

Increased lymphatics in the lung drains the transudate

205
Q

What factor from the ventricles can be used in CHF Dx?

A

BNP. Brain Natriuretic Peptide; If normal, LHF can be excluded

206
Q

Person has AMI w/ normal coronary arteries

A

Cocaine use

207
Q

Mitral Stenosis can cause what GI symptom

A

Dysphagia for solids. LA is posteriorly located right over the esophagus–>dilation of LA in MS causes dysphagia

208
Q

Mitral valve prolapse due to myxomatous degeneration is associated w/ excess production of what

A

Dermatan sulfate. Floppy, hooded valves, mid systolic click w/ murmur (click due to restrain by chordae)

209
Q

Decreased preload causes midsystolic click in MVP to:

A

Move closer to S1

210
Q

How do you differentiate Aortic Stenosis and Hypertrophic Cardiomyopathy based on murmur?

A

AS: Intensity increases w/ INCREASED preload
HypCard: Intensity increases w/ DECREASED preload (standing with decrease the preload and will increase the intensity of HCM). Anterior leaflet of MV is drawn against the hypertrophied septum–> outflow obstruction

211
Q

Murmur in AR that signifies need for replacement

A

Austin Flint murmur. Caused by functional mitral stenosis due to AR blood flow hitting the anterior mitral valve leaflet

212
Q

Huge C-V wave on JVP

A

Tricuspid regurgitation

213
Q

Lymphocytic infiltrate in the myocardium

A

Coxsackie virus B. Most common cause of myocarditis and pericarditis

214
Q

Cardiac path associated w/ organic solvents/sniffing glue

A

Dilated cardiomyopathy

215
Q

Restrictive cardiomyopathy in a child

A

Endocardial fibroelastosis. Thick fibroelastic tissue in the endocardium

216
Q

Cardiac defects associated w/ Conotruncal abnormalities

A

Transposition of great vessels, Tetrology of Fallot, Persistant Truncus Arteriosus. Due to failue to migration/rotation of neural crest cells (truncal and bulbar ridges)

217
Q

People w/ Aortic stenosis are @ risk for what electrical abnormality

A

Afib; Also @ risk for syncope, angina, and dyspnea on exertion

218
Q

1 risk factor for native valve bacterial endocarditis in US

A

Mitral valve prolapse

219
Q

Hypotension, tachycardia, hypoglycemia, and hyperpigmentation

A

Adrenal crisis. TX w/ corticosteroids due to low circulating cortisol

220
Q

Polymyositis vs Dermatomyositis HIstology

A

Poly: CD8+ Tcells w/ endomysial inflammatin
Dermato: CD4+ Tcells w/ perimysial inflammation

221
Q

Loss of development, verbal abilities intellect, and ataxia in a 1-4 yo girl with hand-wringing

A

Rett Disorder. X-linked, fatal in utero in males

222
Q

Contralateral hemiparesis w/ ipsilateral tongue deviation

A

Medial Medullary Syndrome (ASA)

223
Q

Checkerboard loss of pain and temp, dysphagia, hoarseness, vertigo, nystagmus

A

Lateral Medullary/Wallenberg syndrome. PICA, dysphagia and hoarseness are the most specific (Nucleus ambiguus)

224
Q

Facial Paralysis, decreased taste from anterior 2/3 of tongue, decreased pain/temp on face,

A

Lateral Pontine Syndrome (AICA)

225
Q

Opthalmoplegia in all directions, loss of sensation on forehead and maxillary region, loss of corneal reflex

A

Cavernous Sinus Syndrome :mass effect, fistula, thrombosis; CN 3, 4, 6, V1, V2; Visual acuity not effected

226
Q

Rapidly progressive dementia w/ myoclonus

A

Creutzfield-Jakob disease

227
Q

Sudden onset of back pain, hematuria, and oliguria in a pt w/ anion gap metabolic acidosis

A

Ethylene glycol intoxication w/ calcium oxylate kidney stone formation

228
Q

Eosinophilic casts

A

Multiple Myeloma or Chronic Pyelonephritis. Eosinophilurea (eosinophil cells) associated w/ drug-induced interstitial nephritis

229
Q

Hypoglycemia with low keytones

A

Acyl-CoA Dehydrogenase deficiency.Defect in beta oxidation or ketone body synthesis

230
Q

Calluses are marked by increase in what cell layer

A

Stratum CORNEUM (Hyperkeritosis)

231
Q

Causes of Macroglossia

A

Myxedema (severe hypothyroidism), Down Syndrome, Acromegaly, Amyloidosis, MEN2B, Plummer Vinson Syndrome, Kawasaki, creatinism, Beckwith-Wiedemann sundrome.

232
Q

Infection associated w/ Glossitis

A

Scarlet Fever (Erythrogenic Toxin) and Hairy Leukoplakia. Also Nutritional Deficiencies: Iron, B12, Folate, Vit C, Pellagra/Niacin

233
Q

Infectious Esophagitis

A

HSV: punch out lesions; CMV- Linear ulcers, Candida thrush

234
Q

Melena (dark, tarry stools) indicates what about bleed

A

Bleed is proximal to duodenojejunal junction. Hb converted to hematin by acid

235
Q

5 things raising suscpicion for Zolliner Ellison Syndrome

A

Multiple ulcers, ulcers distal to 1st part of duodenum, PUD+diarrhea, ulcers resistant to therapy, MEN1. Also if secretin administration increases gastrin

236
Q

Biggest risk factor for gastric adenocarcinoma

A

H. pylori. Nitrosamines/Smoked food (Japan) and pernicious anemia also important

237
Q

Air in urine and recurrent UTI’s in an old person

A

Colovesicular fistula due to Diverticulosis

238
Q

GI tumor that secretes protein and K+ rich mucus

A

Villous adenoma in colon

239
Q

Portal HTN, ascites, and splenomegaly w/o hepatomegaly in a skinny non-drinker and normal transaminases

A

Portal Vein Thrombosis. Causes: Pylephlebitis (inflammation from appendicitis), Polycythema vera, Paroxysmal Nocturnal hematuria

240
Q

What pain drug is contraindicated in acute cholecystitis

A

Morphine. morphine constricts the sphincter of Oddi; use meperidine or fentanyl

241
Q

Painful swelling of the tibial tuberosity at the insertion of the patella in a boy 11-15 years old

A

Osgood-Schlatter disease. permanently “knobby” knees

242
Q

Proximal joint pain associated w/ Temporal Arteritis

A

Polymyalgia Rheumatica

243
Q

Munro Microabscesses

A

Psoriasis (Neutrophils in stratum CORNEUM)

244
Q

Upper 1/3 of the vagina is columnar epithelium

A

Vaginal adenosis. Related to Diethylstilbestrol (DES), failure of squamous replacement of upper vaginal epithelium

245
Q

Common non-neoplastic cause of death w/ cervical cancer

A

Renal failure to due hydronephrosis. Invasion of bladder wall and obstruction of ureters–>post-renal azotemia

246
Q

Transient, peritoneal abdominal pain mid-cycle in female

A

Mittelschmerz. Peritoneal irritation from ruptured follicle

247
Q

Ovarian Teratoma w/ functioning thyroid tissue

A

Struma ovarii

248
Q

Call-Exner Bodies tumor? Reinke crystals tumor?

A

Call-Exner bodies (resemble follices) –Granulosa-Theca cell tumor: may produce E
Reinke crystals– Leydig cell Tumor: may produce T

249
Q

Opening of the prepuce is too small

A

Phimosis. Can result from Balanoposthitis (infection of glans/prepuce)

250
Q

Greatest risk factor for Squamous cell carcinoma of penis

A

Non-circumsized w/ poor hygeine. And smoking.

251
Q

Increased FSH, Dec Inhibin, normal Testosterone and LH

A

Defect in Sertoli cells. Can be caused by Varicocele!

252
Q

What indicates erectile dysfunction is psychogenic

A

Presence of Nocturnal Penile Tumesence

253
Q

Small cystic spaces in the Internal capsule or Thalamus

A

Lacunar infarcts. Due to Lipohyalinosis of Lenticulostriate arteries w/ HTN or diabetes

254
Q

Albinism, increased pyogenic infections, giant granules in leukocytes, peripheral neuropathy

A

Chediak-Higashi Syndrome. Failure of phagosome-lysosome fusion

255
Q

Mutated in Hyper-IgM Syndrome

A

CD40L or CD40. prevents B cells isotype switching

256
Q

Anti-U1 ribonucleoprotein

A

anti Smith Ab. Mostly SLE but also in Mixed Connective Tissue disease, systemic sclerosis, and polymyositis

257
Q

Pentad of findings for HUS and TTP

A

Microangiopathic Hemolytic Anemia
Thrombocytopenia.
Fever, renal insufficiency, CNS Abnormalities. Both have hemolytic anemia and Thrombocytopenia
TTP has CNS abnormalities more

258
Q

Nitrogen gas emboli are associated w/ what other symptom

A

joint and muscle pain. chronic form can cause necrosis of bone= Ciasson’s dz

259
Q

Unlike in Thalassemia, Extramedullary hematopoesis in Sickle Cell Disease does not include what organ

A

Spleen. Due to autosplenectomy; Both have “crew cut” skull, chipmunk faces, and hepatomegaly

260
Q

Aplastic anemia due to defective telomerase

A

Dyskeratosis Congenita

261
Q

Aortic Valve stenosis vs. Chronic Rheumatic fever of aortic valve

A

Rheumatic fever leads to fusion of the commissures. (Commisures are NOT FUSED in “wear and tear” AS) RF usually also w/ mitral stenosis.

262
Q

Lung changes in cigarette smoke

A

Increased mucus production, decreased ciliary movement, decreased alveolar macrophage activity

263
Q

Two characteristic CXR findings of emphysema

A

Flat Diaphragm and vertical heart

264
Q

Calcified “coin lesion” in the lung containing lung tissue and Cartilage

A

Bronchial Hamartoma

265
Q

Lung cancer that only grows along bronchioles and alveoli

A

Broncioalveolar Carcinoma. CLARA cells!

266
Q

Unique metastasis site of lung cancer

A

Adrenal gland

267
Q

Obstructing stone in salivary gland leading to?

A

Sialadenitis. Staph infection

268
Q

Fibrosis in Ideopathic Pulmonary Fibrosis and Cirrhosis is mediated by:

A

TGF-B.Ideopathic pulmonary fibrosis is Honeycomb lung

269
Q

Ovarian tumor associated w/ pleural effusion and ascites

A

Fibroma. Cluster of s/s called Meig’s Syndrome

270
Q

Course facial features, umbilical hernia, macroglossia, mental retardation, short stature, skeletal abnomalitis in young kid

A

Cretinism. Most commonly due to thyroid agenesis. Or lack iodine.

271
Q

Signs of Hypocalcemia

A

Trousseau’s and Chvosek signs

circumoral numbness and tingling

272
Q

Low Ca, high PTH, short height and 4th and 5th metacarpals

A

Albright’s Hereditarry Osteodystrophy or Pseudohypoparathyroidism

273
Q

Death in Uncal herniation

A

Duret Hemorrhage (Rupture of paramedian artery)

274
Q

Three findings of Multiple Sclerosis Spinal Tap

A

Lymphocytes, Oligoclonal IgG band, Myelin Basic Protein

275
Q

Supratentorial cystic tumor in a child w/ HX of seizures

A

Ganglioglioma. Mural nodule.

276
Q

Extreme Friendliness w/ strangers, Elfin face, intellectual disability, hypercalcemia

A

Williams Syndrome. Deletion of long arm of Chr 7

277
Q

Lesioned in Hemiballismus

A

Contralateral subthalamic nucleus (normally it helps inhibit movement in basal ganglia pathway)

278
Q

Hypotonia and tongue fasciculations in a newborn

A

Spinal Muscular Atrophy/Werndig Hoffman

279
Q

Renal tumor w/ highly eosinophilic cells and central scar

A

renal oncocytoma. Benign epithelial tumor. Large number of mitocondria.

280
Q

Fever, rash, hematuria, CVA tenderness, and eosinophilia 1-2 weeks after starting a drug

A

Drug-induced Interstitial Nephritis (Tubulointerstitial nephritis). Eosinophils in urine; NSAIDS, Diuretics, Penecillin, Sulfonamides

281
Q

Granulosa cells in eosinophilic fluid

A

Call-Exner bodies–Granulosa-Theca cell tumor

282
Q

Failure of copper absorption leading to Kinky hair, growth retardation, and arterial fragility/aneurysms/rupture

A

Menkes Disease: Decrease activity of Lysyl Oxidase (collagen synthesis) due to failure of ATP-mediated transport of copper from the intestinal epithelium to the blood–> decreased Ceruloplasmin

283
Q

Rh- mother reaction against Rh+ fetus

A

Erythroblastosis fetalis. if daughter survives, no risk of that happening when they get pregnant b/c daughter is Rh+

284
Q

In pts w/ COPD what controls respirations

A

Peripheral Chemoreceptor response to O2. Chronically elevated CO2 blunts the central response to CO2 and central chemoreceptors do not respond to O2; If pt is given high O2 ventillation, respiratory drive is lost (O2 must be below 70 to stimulate peripheral chemoreceptors

285
Q

Aphasia, olfactory hallucinations, and personality changes

A

HSV-1 Temporal Lobe encephalopathy

286
Q

Pts w/ RCA MI may have Bradycardia due to? Tx?

A

Infarct of SA and AV nodes (RCA supplies). TX w/ atropine to decrease muscarinic input except in glaucoma pts

287
Q

Crohn’s pts are at risk for what two types of stones

A

Calcium Oxylate kidney stones. Increased Oxylate absorption

Cholesterol gallstones. Decreased Bile acid reabsorption/cycling

288
Q

Neurologic Manifestation of Wilson’s Disease

A

Basal Ganglia Degeneration (parkinsonian symptoms). Also dementia, dyskinesia, and dysarthria

289
Q

Two things that cause increased skin pigmentation

A

Addison’s Disease (ACTH) and Hemochromatosis

290
Q

Overproduction of abnormal platelets w/ bleeding and thrombosis

A

Essential Thrombocytosis. Chronic Myeloproliferative Disorder, 50% have JAK2 mutations, Bone marrow contains enlarge megakaryocytes

291
Q

3 key complications of Sjogren’s Syndrome

A

Dental Carries (Xerostomia), Corneal damage (Xeropthalmia), MALT Lymphoma

292
Q

Rosenthal Fibers

A

Pilocystic Astrocytoma

293
Q

Focal Segmental Glomerulosclerosis associations

A

HIV, Sickle Cell Disease, Heroin Abuse, obesity. AA, Hispanics.

294
Q

Membranous Nephropathy associations

A

HepB, Solid Tumors, Anti-Phospholipase A2, SLE. All in a white person

295
Q

If a hyperthyroid pt has fever and sore throat, what test should be done

A

CBC or WBC b/c Methimazole and PTU both cause agranulocytosis

296
Q

Histology of Preeclampsia

A

Fibrinoid Necrosis of the placental vessels

297
Q

Most common manifestations of systemic amyloidosis. Spot for biopsy?

A

Amyloid Nephropathy (Nephrotic), Restricted cardiomyopathy, Tongue enlargement. Abdominal fat pad and rectum are easy spots for biopsy

298
Q

Builds up in urine of SCID pts

A

Deoxyadenosine (if Adenosine Deaminase deficient). Also caused by IL-2 receptor mutations and MHC Class II deficiency

299
Q

Antiphospholipid antibody syndrome presents w/ prolonged PTT, how does it present clinically

A

Arterial and Venous thrombosis (DVT, Hepatic Vein thrombosis/Budd-Chiari, Recurrent Pregnancy loss (placental thrombosis)

300
Q

Good Prognosis ALL

A

t(12,21)

301
Q

Leukemia w/ hypogammaglobulinemia (infection risk), autoimmune hemolytic anemia, Richter Transformation, and smudge cells

A

Chronic Lymphocytic Leukemia. Richter transformation to Diffuse Large B cell lymphoma

302
Q

Intermediate Size B Cell Lymphoma? Large sized?

A

Intermediate- Burkitts; Large: Diffuse Large B cell

303
Q

Adolescent w/ pathological fracture due to mass w/ eosinophils and langerhans cells

A

Eosinophilic Granuloma. Type of Langerhans Cell Histiocytosis–>S-100+ and Birbeck granules

304
Q

Arteriolosclerosis of the renal arterioles due to long standing HTN or diabetes causing glomerular scarring and CRF

A

Arteriolonephrosclerosis

305
Q

Mechanism of Abdominal Aortic Aneurysm

A

Atherosclerosis decreases O2 diffusion to the media (abd aorta has no vasa vasorum)

306
Q

Wilm’s Tumor + progressive glomerular disease and male pseudohermaphroditism

A

Denys-Drash Syndrome

307
Q

Wilm’s Tumor + muscular hemihypertrophy, neonatal hypoglycemia, and organomegaly (tongue)

A

Beckwith–Wiedemann syndrome. WT2 gene imprinting

308
Q

Graves Ab bind TSH receptor on retro-orbital and pre-tibial fibroblasts leading to production of what substance

A

Glycosaminoglycans (chondroitin sulfate and hyaluronic acid). IgG can cross the placenta causing thyroid enlargement in a newborn w/ possibly airway compromise

309
Q

Addison disease is usually autoimmune, if it is caused by metastatic cancer, where is the cancer from?

A

Lungs. Also can be caused by TB

310
Q

Systemic Effects of Cushings (not fat distribution)

A

Muscle weakness, Osteoporosis, Immunesuppression, hypertension

311
Q

What exacerbates the septic hypotension in Waterhouse Friederichsen syndrome

A

Lack of Cortisol. Neisseria meningitidis sepsis w/ DIC and destruction of the adrenal glands

312
Q

Pt presents w/ acute mastitis that did not improve w/ antibiotics (swollen/inflammed breast), Consider what?

A

Inflammatory Carcinoma. Inflamed swollen breast w/ peau de orange; can occur in breastfeeding women; tumor cells in the dermal lymphatics

313
Q

Bile pigment deposited in hepatic parenchyma and green-brown plugs in dilated bile canaliculi

A

Cholestasis

314
Q

Herald patch followed by multiple plaques w/ Collarette scale

A

Pityiasis rosea. Christmas tree distribution on back

315
Q

Cholesterol Gallstones

A

Most common

316
Q

Black Pigment Gallstones

A

Extravascular HA

317
Q

Brown Stones Gallstones

A

Infection of Common Bile Duct

318
Q

Dysphagia of Solids Only

A

Peristaltic stricture (GERD), esophageal ring (intermittant), cancer.

319
Q

Dysphagia of Solids and liquids

A

Neuromuscular causes: achalasia, esphageal spasm, dsymotility due to systemic sclerosis

320
Q

Small Cell Lung Paraneoplastic Syndromes

A

ADH, ACTH, Eaton-Lambert

321
Q

SqCC of Lung Paraneoplastic Syndromes

A

PTHrP

322
Q

Renal Cell Carcinoma Paraneoplastic Syndromes

A

EPO, ACTH, PTHrP, L Varicocele

323
Q

Leber’s Hereditary Optic Neuropathy

A

Mitochondrial Inheritance

324
Q

Myoclonic epilepsy and Ragged Red Fibers

A

Suspect if seizures, myoclonic, lactic acidosis, ragged red fibers/muscle histology change. Mitochondrial Inheritance

325
Q

MELAS

A

Suspect if seizures, myoclonic, lactic acidosis, ragged red fibers/muscle histology change Mitochondrial Inheritance

326
Q

Type 1 Renal Tubular Acidosis

A

Failure of a-intercalated cells to secrete H+

327
Q

Type 2 Renal Tubular Acidosis

A

Defect in PCT HCO3 reabsorption

328
Q

Type 3 Renal Tubular Acidosis

A

Failure to respond to aldosterone/lack of aldosterone

329
Q

Heart Organ Specific Chronic Rejection

A

Atherosclerosis

330
Q

Lungs Organ Specific Chronic Rejection

A

Bronchiolitis obliterans

331
Q

Liver Organ Specific Chronic Rejection

A

Vanishing Bile ducts

332
Q

Kidney Organ Specific Chronic Rejection

A

Vascular fibrosis, glomerulopathy

333
Q

Glomerular EM: Foot Process Effacement

A

FSGS, Minimal Change

334
Q

Glomerular EM: Subepithelial Deposits

A

Membranous Nephropathy

335
Q

Glomerular EM: Tram Track

A

MPGN Type 1

336
Q

Glomerular EM: deposits

A

MPGN Type 1

337
Q

Glomerular EM: Mesangial deposits

A

Diabetic Nephropathy

338
Q

Glomerular EM: Intramembranous Deposits

A

MPGN Type 2 (C3 Nephritic Factor)

339
Q

Friedrich Ataxia. defect? death via?

A

Death from Hypertrophic cardiomyopathy; Impaired mitochondrial iron homeostasis

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