Pathophys flashcards
Honeycomb lung
Ideopathic Pulmonary fibrosis Note: Fibrosis mediated by TGF-B from injured Pneumocytes
unexplained Pleural Effusion in young woman
Lupus (until proven otherwise)
Egg shell Calcification of Hilar Nodes
Silicosis Note: Quartz–>Birefringent
Calcified plaque on Dome of Diaphragm/pleura
Asbestosis
Curshman Spirals and Charcot Leiden crystals
Asthma
Nasal Polyps in a child
Cystic Fibrosis
Nasal Polyps in an adult female
long-time aspirin use or allergies
Vinyl Chloride, Arsenic, Thorotrast
Liver Angiosarcoma
Myasthenia Gravis
Thyoma or Thyroid Follicular B cell hyperplasia
Bilateral Internuclear Opthalmoplegia
Multiple Sclerosis
Non-bilious projectile vomiting in a 3-5 week old
Congenital Pyloric Stenosis Note: Can be acquired if infant treated w/ erythromycin when under 6 weeks old for pertussis
False diverticulum through Cricopharyngeus m.
Zenker Diverticulum
Giant rugal folds w/ mucus cell hyperplasia
Menetrier’s disease Note: Protein Losing Enteropathy, Atrophy of parietal cells, increased risk for adenocarcinoma
Bleeding in Gastric Ulcer
Left Gastric Artery
Perforation of Posterior Duodenal Ulcer
Pancreatitis or bleeding from Gastroduodenal artery
High Gastrin, low stomach acidity
Autoimmune chronic atrophic Gastritis Note: pernicious anemia
High Gastrin, high stomach acid
Zollinger Ellison Note: Pancreas mass, multiple/distal ulcers, MEN1
Signet Ring cells in Ovaries
Krukenberg Tumor Note: from Diffuse Gastric Adenocarcinoma
Severe pain in LUQ after eating
Ischemic colitis Note: splenic flexure
Pseudopolyps and crypt abscesses
Ulcerative colitis Note: Risk of Toxic Megacolon
Submucosal noncaseating granulomas
Crohn’s Disease
Skin flushing, diarrhea, Tricuspid Regurgitation
Carcinoid tumor from Ileum metastasized to liver Note: increased 5-HIAA in urine; must be in liver for Carcinoid Syn.
AST: ALT at least 2:1 and
Alcoholic Liver disease Note: GGT and ALP also elevated, but GGT vastly more elevated
Increased ALP and GGT
Cholestasis Note: Inc ALP w/o inc GGT–> osteoblastic activity in bone
Beading of the Bile ducts on ERCP
Primary Sclerosing Cholangitis Note: Jaundice, Severe Pruritis, and Hepatosplenomegaly
Antimitochondrial Antibodies
Primary Biliary Sclerosis Note: CD8 Tcell mediated granulomatous destruction of intrahepatic bile duct epithelium
Cirrhosis in a child
A1AT deficiency
Strictures, cobblestone, creeping fat
Crohn’s Disease Note: String Sign
Tumor at Junction of R and L Hepatic Ducts
Klatskin Tumor Note: Type of Cholangiocarcinoma
Superficial Migratory Thrombophelbitis (moving DVTs)
Pancreatic Cancer (Trousseau’s Syndrome) Note: CA19-9 monitored for remission; Painless Jaundice
RBC casts and linear immunostaining
Goodpastures disease Note: Anti-basement membrane Ab (collagen type 4)
Deposits in the Glomerular BM
Type 2 Membranoproliferative Glomerulonephritos Note: C3 Nephritic Factor and very low levels of serum C3–>C3 nephritic factor stabilizes C3 convertase
Fever, Flank Pain, WBC casts
Acute Pyelonephritis
Thyroidization of the kidney
Chronic Pyelonephritis
Bilateral Renal Cell Carcinoma, Hemangioblastoma in retina or cerebellum
Von Hippel Lindau Note: VHL on Chr 3; clear cells contain lipid and glycogen; Cerebellar Hemangiomas
Unilateral palpable flank mass in child w/ HTN
Wilm’s Tumor Note: Contains abortive glomeruli and tubules, primitive blastemal cells, and rhabdomyoblasts
Sudden onset of testicular pain post trauma and absent cremaster reflex
Testiticular torsion
Biggest risk factor for testicular cancer
Cryptorchid testicle Note: especially if intra-abdominal
Testicular cancer in a guy >50
Malignant Lymphoma
Delayed puberty and anosmia
Kallmann’s Syndrome Note: Failure of migration of GnRH secreting cells and olfactory bulbs
Best Marker for Menopause
Increased FSH Note: LH will also be increased, estradiol will be decreased
Most common cause of infertility & Ectopic Pregnancy
PID
Tumor with Reinke Crystals
Leydig Cell Tumor Note: may produce androgens
First test if Pregnant woman w/ vaginal bleeding
Pelvic Ultrasound Note: Do NOT do pelvic exam; Painless (placenta previa); Painful + Contractions (Abruptio Placentae)
Amioncentesis with Inc AFP? Dec AFP?
Inc: Neural Tube Defect; Dec: Down Syndrome Note: Down: Dec AFP, Dec urine estriol, and Inc B-hCG and inhibin A
Hypothyroidism w/ germinal centers on biopsy
Hashimoto’s Thyroiditis Note: Lymphocyte infilitrate; if mixed immune infiltrate and painful, think thyroiditis (DeQuervain)
Cancers secreting PTHrP
SqCC of Lung and Renal Cell Carcinoma
Syndromes w/ Pheochromocytoma
Neurofibromatosis 2, MEN IIa and IIb, Von Hippl Lindau (bilateral)
Chaotic eye movements and myoclonic jerks in a child
Neuroblastoma Note: Usually located in Adrenal gland; N-Myc amplification; Homer Wright Rosettes
Secretory Diarrhea and Achlorhydria
VIPoma Note: can be tx w/ octreotide
Eczema, Thrombocytopenia, and Recurrent infections
Wiscott Aldrich Note: X linked deficiency of B and T lymphocytes with eczema and thrombocytopenia
Port wine stain w/ ipsilateral leptomeningeal angioma, seizures
Sturge Weber Syndrome
Ash Leaf spots, CNS hamartomas, subependymal astrocytomas, cardiac rhabdomyoma, renal angiomyolipoma
Tuberous Sclerosis
Bitemporal Hemianopia and headaches in a child
Craniopharyngioma Note: Derived from Rathke’s pouch, oral ectoderm, same as Anterior Pituitary
Bitemporal Hemianopia and headaches in an adult
Pituitary Adenoma Note: Most commonly prolactinoma
Solid tumor w/ small blue cells in cerebellum of child
Medulloblastoma Note: Drop Metastasis
Pseudopalasading tumor cells around necrosis
Glioblasoma multiforme
New Onset Seizures due to whorled tumor
Meningioma Note: Psammoma bodies
Tinnitis, Vertigo, and Hearing loss
Meniere Disease Note: Increased volume of endolymph in inner ear due to defective reabsorption
Fluctuating Consciousness, hallucinations, and dementia; late Parkinsonian effects
Lewy Body Dementia
coarse facial features, clouded corneas, high plasma lysosomal enzymes, restricted joint movement
I cell Disease Note: Failure of Golgi to add Mannose-6-Phosphate signal to proteins destined for Lysosomes–>excreted instead
Bronchiectasis/Recurrent Pulmonary infections, infertility, and Situs Inversus
Kartagener’s Disease Note: dynein arm defect in cilia
Telangiectasia, Epsitaxis, GI bleeding, skin discoloration, AV Malformations
Osler-Weber-Rendu Syndrome (Hereditaty hemorrhagic telangiectasia)
Duchenne Muscular Dystrophy mutation
X-linked Frameshift mutation
Becker Muscular Dystrophy mutation
X-linked Point mutation
Difficulty releasing grib/doorknob, muscle wasting, frontal balding, cataracts, testicular atrophy
Myotonic Muscular Dystrophy Note: CTF trinucleotide repeats in DMPK gene; Distal Weakness in hands/feet; Type 1 fibers more affected
Macroorchidism, Large Jaw, large/everted ears, mitral valve prolapse, developmental delay
Fragile X Syndrome Note: X linked trinucleotide repeats affecting the METHYLATION of FMR1 gene (fragility only describes in vitro not in vivo);
Rocker bottom feet, lowset ears, micrognathia
Edwards Note: Trisomy 18
Rocker bottom feet, cleft lip/palate, holoprosencephaly, polydactyly
Patau Note: Trisomy 13
Microcephaly, High pitched crying, epicanthal folds, severe intellectual disbility, VSD
Cri du chat Note: Chr 5 short arm deletion
Asymptomatic accumulation of fructose in blood/urine
Essential Fructosuria (Defect in Fructokinase)
Intellectual disability, jaundice, failure to thrive, hepatomegaly, and cataracts in an infant
Classic Galactosemia Note: Defect in Galactose-1-P Uridyltransferase–>accumulation of galactitol and depletion of phosphate
Infantiile Cataracts and failure to track objects with galactose in blood/urine
Galactokinase Deficiency Note: accumulation of Galactitol due action of Aldolase reductase on galactose
Hypoglycemia, jaundice, cirrhosis, and vomiting in an infant after consuming juice/fruit
Fructose Intolerance Note: Deficiency of Aldolase B (Auto rec), accumulation of Fructose-1-p causes decreased phosphate, which inhibits glycogenolysis and gluconeogenesis
Hyperammonemia with excess orotic acid
Ornithine Transcarbamylase Deficiency Note: X-linked recessive, often evident in first few days of life: Increased Orotic Acid, Decreased BUN, Hyperamonemia symptoms (Kernicterus, somnolence, vomiting, cerebral edema
Excess orotic acid with Megaloblastic Anemia
Orotic Aciduria Note: deficiency of UMP Synthase, Megaloblastic anemia NOT treated with folate or vit B12 supplement
Intellectual disability, mousy.musty body odor, seizures, and eczema corrected with Tetrahydrobiopterin supplementation
Malignant PKU Note: 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
Dark connective tissue, brown pigmented sclera, urine turns black w/ prolonged air exposure, Debilitating arthralgia
Alkaptonuria Note: Deficiency of Homogenisate oxidase–> accumulation of homogentisic acid and failure to breakdown tyrosine to Fumarate
Osteoporosis, Tall stature, kyphosis, lens subluxation (down and in), thrombosis/atherosclerosis w/ stroke and MI risk
Homocystinuria Note: 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
Renal Cystine stones (hexagonal)
Cystinuria Note: Hereditary defect in Renal Proximal tubule and intestinal reaborption of Cysteine, Ornithine, Lysine, and Arginine
Severe retardation, disability, and sweet smelling urine
Maple Syrup Urine Disease Note: Deficiency in Alpha-Ketoacid Dehydrogenase (uses thiamine)–>blocks degradation of Branched amino acids (Valine, Leucine, Isoleucine), Isoleucine causes burnt sugar urine smell
Glycogen storage disease causing cardiomyopathy
Pompe Disease (Type II) Note: Deficiency in Lysosomal a-1,4-glucosidase (acid maltase)
Glycogen storage disease causing painful muscle cramps, myoglobinuria, and arrhthmias
McArdle Disease (Type V) Note: Deficiency in Myophosphorylase (skeletal muscle glycogen phosphorylase)
Glycogen storage disease with hypoglycemia and accumulation of short outer Dextrin-like structures
Cori Disease (Type III) Note: Deficiency in Debranching enzyme (a-1,6-glucosidase)
Lysosomal Storage disease with Aseptic necrosis of the femur, pancytopenia, hepatosplenomegaly, and bone crisises
Gaucher’s Disease Note: Most common LSD, Gaucher cells (lipid-laden macrophages that look like crumpled paper)
Glucocerbrosidase deficiency–>accumulation of Glucocerebroside
Jaundice (CB) in a less than 2 month old
biliary atresia (+acholic stool) Note: If UCB, think Crigler Najjar
Jaundice in a 2-10 year old
Biliary cyst Note: (70% have abnormal pancreatobiliary junction)
Microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure
Hemolytic Uremic Syndrome Note: Shigella or EHEC
Diarrhea that is initially watery then becomes bloody and rose spots appear on the abdomen
Salmonella typhi Note: Can cause dramatic inflammation in Peyer’s pathes–> intestinal hemorrhage/perforation and death; can colonize the gallbladder
Retroperitoneal Hematoma following trauma (Motor crash)
Bleeding from Pancreas Note: Spleen is intraperitoneal
Pt w/ S3 gallop, orthopnea/dyspnea, and MR is tx w/ diuretic and both heart sounds disappear; What was the pathophys of the MR?
Functional Mitral Regurgitation Note: S3 gallop indicated volume overload, which the diuretic treated; elimination of the holosystolic murmur @ the apex means the valves were normal, just stretched apart
Cerebral tumor: S-100+, elongated cell w/ regular oval nuclei, and alternating areas of high and low cellularity
Schwannoma
Well circumscribed tumor at grey/white matter junction
Metastatic brain tumor Note: often multiple, often from lungs
Multiple liver nodules w/o increased AFP
Metastatic liver cancer Note: Usually from colon
Fixed wide splitting of S2
ASD
Inspiration decreases the A2/P2 splitting
Paradoxical Split S2 Note: Aortic stenosis or Left Bundle Branch block
Both Aortic and Pulmonic regurgitation is heard where?
Left Sternal border Note: Aortic Regurg not heard at the aortic area (stenosis/sclerosis of valves only)
Systolic murmur at the Left Sternal Border
Hypertrophic Cardiomyopathy Note: Murmur involves the hypertrophied interventricular septum and MITRAL VALVE
Valsalva increased the intensity of one murmur only, which?
Hypertrophic Cardiomyopathy Note: Obstruction greater at lower ventricular volume
Sign a person w/ severe volume overloaded or hypertrophied heart failure has Afib
Disappearence of S4 Note: S4 is caused by atrial contraction
Murmur that radiates to carotids and causes Pulsus Parvus et tardus
Aortic stenosis Note: intensity increases w/ increased venous return (opposite of hypertrophic cardiomyopathy)
Young pt w/ recurrent episodes of syncope and deafness
Jervell Lange Nielsen Syndrome Note: Autosomal recessive Long QT Syndrome; Romano-Ward is AutoDom (cardiac only)
Progressive lengthening of the PR interval until beat is dropped, followed by normal PR
Mobitz Type 1 Second Degree Heart Block
Pacemaker for the ventricles in 3rd degree Heart block
AV node (45-55 bpm)
Recominant form of B-type Natuiretic peptide used to treat heart failure
Nesiritide Note: 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)
Reccurent Epistaxis,spider like skin lesions, and guiaiac positive stool
Osler-Weber-Rendu Syndrome (Hereditaty hemorrhagic telangiectasia)
Ischemic Neuron Injury histology
“Red Neuron” w/ eosinophilic cytoplasm, Loss of Nissl, Pykinosis of nucleus
Axonal Damage/Axonal Reaction Histology
Swollen neuron body, dispersion of Nissl, eccentric nucleus w/ large nucleolus Note: Wallerian degeneration
Ulcers where in the GI tract are virtually never malignant
Duodenal ulcers Note: Gastric ulcers have a high potential of being malignant and must be biopsied
Fern Leaf marks (Lichtenburg figures), Secondary burns, and death via arrthymia
Person died from lightening strike Note: Lichtenberg figures are characteristic; death via arrhythmia or respiratory depression
Bilateral absence of the Vas Deferens
Cystic Fibrosis Note: Azoospermia
Parotid tumor with germinal centers
Warthin Tumor Note: Most common parotid tumor is pleomorphic adenoma (stroma and epithelial)
2 things suspected if abdominal pain with meals
gastric ulcer and ischemic colitis Note: Ischemic colitis is typically 30-60 min post meal and pt has wt loss
Histology of Reye Syndrome
Microvesicular fatty change Note: Not necrosis
Air in the gallbladder/biliary tree w/ bowel obstruction
Gallstone ileus Note: Fistula between gallbladder and small intestine; due to longstanding cholelithiasis
Cerebellar defects/atrophy, spider angiomas, and recurrent respiratory infections
Ataxia-Telangectasia Note: Defect in DNA double strand break repair
Reccurent Hemorrhagic strokes in an elderly person
Cerebral Amyloid Angiopathy Note: Ab amyloid, usually lobar strokes and are less severe
Child presents w/ enlarged testicle w/ fluid in scrotum
Hydrocele Note: Fluid in Tunica Vaginalis, communicating hydrocele if processus vaginalis is patent; Tunica vaginilis derived from the peritoneum; highly associated w/ inguinal hernia
Acute Painless monocular vision loss
Central Retinal Artery occlusion Note: Pale retina w/ Cherry Red macula; commonly associated w/ Afib
Coffee ground emesis
Blood exposed to gastric acid
BRAF V600E mutation
Malignant Melanoma Note: Vemurafenib–inhibits B-Raf w/ V600E mutation; Often metastasizes to the brain, causing seizures
Jejunal and distal duodenal ulcers, diarrhea
Gastrinoma/Zollinger Ellison
Severe Abdominal Pain, Port-wine colored urine, polyneuropathy, and psychological disturbances
Acute Intermittant Porphyria Note: Defect in Porphrobilinogen Deaminase causes buildup of porphrobilinogen and ALA; TX w/ glucose and Heme to inhibit ALAS
Neutrophils with bilobed nuclei (no effect on activity)
Pelger-Huet Anomaly Note: seen after chemotherapy–>two nuclear masses connected w/ a thin chromatin filament
Autoimmune hemolytic anemia, smudge cells, CD5/Cd23+
CLL
Lytic bone lesions in a child, skin rash, +/- recurrent otitis media w/ a mass involving the mastoid bone
Langherhans cell Histiocytosis Note: Birbeck granules, S-100+ (mesodermal origin)
Scalp rash, Lytic skull defects, Diabetes Insipidus, and exophalthmos is a child
Hand-Christian-Schuller Diesease Note: Malignany proliferation of Langerhans cells
3 conditions associated w/ lytic bone lesions
Multiple Myeloma, Langerhans cell Histiocytosis, Adult T-cell Lymphoma
Complications of Paget’s disease of bone
High Output Heart Failure (AV shunts in bone)
Osteosarcoma Note: Also associated w/ paramyxoviruses
Trabecular thinning w/ fewer interconnections
Osteoporosis Note: All labs normal
Persistance of primary, unmineralized spongiosa in the medullary canals w/ no mature trabecullae
Osteopetrosis Note: unlike normal bone, where marrow replaces the primary spongiosa–>Pancytopenia
Osteoid matrix accumulation around trabeculae
Osteomalacia/rickets Note: Decreased Ca, PO4 with increased ALP and PTH
Mosiac pattern of woven and lamellar bone
Paget Disease of Bone Note: Isolated increase in ALP
Polyostotic fibrous dysplasia, precocious puberty, Cafe-au-Lait macules
McCune-Albricht Syndrome Note: Multiple unilateral bone lesions
Hyperkeratosis and parakaratosis
Psoriasis Note: Increased thickness of stratum corneum (hyper) and retained nuclei in stratum corneum (para)
Epidermal hyperplasia in response to GI malignancy or insulin resistance
Ancanthosis Nigrecans Note: Ancanthosis = epidermal hyperplasia (increased spinosum)
Hyperorality, hypersexuality, disinhibited behavior
Kluver Bucy Syndrome Note: Associated w/ bilateral Amygdala lesions–>HSV-1
Dysphagia, hoarseness, Checkerboard loss of pain and temp (ipsi face, contra body)
Wallenberg Sydrome/Lateral Medullary Syndrome Note: PICA stroke
Stroke causing facial N paralysis
AICA Note: facial nucleus effects are specific to AICA strokes–> Lateral Pontine Syndrome
Contralateral Paralysis w/ ipsilateral CN3 lesion
Weber Syndrome Note: Midbrain stroke (paramedian branch of PCA)
Ipsilateral CN3 lesion and Ipsilateral paralysis
Transtentorial/Uncal herniation Note: b/c contralateral midbrain peduncle compressed (fibers cross over below)
Berry Aneursym in posterior communicating hits what CN
CN3 Note: Eyes “down and out”
Infant w/ hypotonia and tongue fasciculations
Spinal Muscular Atrophy/Werndig Hoffman
Duchenne Muscular Dystrophy mutation
Friedrich Ataxia Note: Death from Hypertrophic cardiomyopathy; Impaired mitochondrial iron homeostasis
Pt unable to look upward
Parinaud sydrome Note: Compression of Superior collilculi, usually by a Pinealoma
Hypertension and proteinuria
Hydatiform mole Note: after 20 weeks is Pre-eclampsia
Precocious puberty and testicular mass in young boy
Leydig Cell Tumor Note: Reinke Crystals
Granulomatous heart nodules
Aschoff Bodies/Rheumatic Fever
Most common cause of Increased AFP on amniocentesis
Dating Error Note: If pathology, neural tube defects
Lack of Xylose absorption (does not require pancreatic enzymes
Small Bowel Disease Note: Pancreatic problems will have normal xylose absorption
Korotkoff sounds heard only on expiration at pressures >10mmHg they are heard thought respiratory cycle
Pulses paradoxus Note: Tamponade, constrictive pericarditis, severe Obstructive lung disease, restrictive cardiomyopathy
Air in biliary tree and bowel obstruction
Gallstone Ileus
Connective Tissue in muscle converting to bone (metaplasia) post-trauma
Myositis ossificans
Non-caseating granulomas in the axillary lymph nodes
Cat Scratch disease (Bartonella hensae)
Factors that increase angiogenesis
VEGF, FGF, PDGF Note: VEGF and FGF are commonly produced by tumors
Three deficiencies that cause delayed wound healing
Vitamin C, Copper, Zinc Note: Dehiscence is rupture of a wound
Keloid is caused by excess what
Type 3 collagen
Rebleeding after surgery or wisdom teeth pull, hemarthrosis
Hemophilia
Embolism w/ cholesterol clefts on histology
Atherosclerotic embolus
Pt w/ broken long bone has trouble breathing and petechiae on chest
Fat embolus
Embolism w/ squamous cells and keratin debris in postgravid
Amniotic fluid embolism Note: Squamous cells/keratin from fetal skin, associated w/ DIC
Remnants in RBCs
Basophilic Strippling: rRNA
Heinz Bodies: Hemoglobin
Howell Jolly bodies: Nuclear remants
Reticulocytes- RNA Note: Lead poisoning, Anemia of chronic disease, Alcohol abuse, Thalassemia
Glucose-6-P Dehydrogenase deficiency (bite cells)
Asplenic patients (Sickle cell Disease or Hereditary Spherocytosis post surgery)
-
Extravascular Hemolysis vs Intravascular hemolysis labs
Extra: inc unconjugated bilirubin/bilirubin gallstones
Intra: Hemoglobinemia, Hemoglobinurea, Hemosiderinuria, Decr Haptoglobin Note: Haptoglobin binds free Hb in blood; Hemopexin binds free heme
Major cause of death->Paroxysmal Nocturnal Hemoglobinuria
Thrombosis (hepatic, portal, cerebral) Note: Pancytopenia due to lack of CD55/Decay Accelerating Factor due to loss of GPI anchors
> 20% blasts leukemia that infiltrates the gums
Acute Monocytic Leukemia
AML associated w/ Down syndrome before age 5
Acute Megakaryoblastic Leukemia Note: After age 5 Down is associated w/ ALL
Most common cause of death in CLL
Infection Note: Hypogammaglobulinemia due to cancer production of non-produtive Ab; Smudge cells and AIHA
Lytic bone lesions w/ a rash in a pt from Japan/Caribbean
Adult T cell Leukemia Note: CD4 T cells and’ HTLV1 virus
Pautrier Microabscesses
Mycosis Fungoides Note: Cd4 tcell tumor in skin, cerebriform nuclei when progresses to Sezary Syndrome
Differentiates CML from Leukemoid Reactions
Leukocyte Alkaline Phosphatase stain Negative
Basophilia and t(9,22) Philly Chr
Itching after showering, facial plethora, blurry vision, headache, Peptic ulcers, Gout
Polycythemia Vera Note: 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
What Myeloproliferative Disorder is not associated w/ gout
Essential Thrombocytosis Note: excess platelets, which never have a nucleus
Most common cause of death in Multiple Myeloma
Infection Note: Bone Lesions/pain, Hypercalcemia, Renal Insufficiency, Anemia, M spike, AL amyloidosis, Rouleaux, Eosinophilic casts of bence jones +Tamm Horsfall proteins
B-cell lymphoma w/ IgM spike
Waldenstrom Macroglobulinemia Note: also called Lymphoplasmacytic Lymphoma; Hyperviscosity due to IgM pentamers (visual & neurological defects and bleeding due to platelet aggregation)
Aortic Dissection is associated w/
Hypertension (old people)- Hyaline arteriolosclerosis of vaso vasorum
Marfan/Ehlers Danlos- Cystic Medial Necrosis Note: sharp tearing chest pain that radiates to the back
Hypotension, pulsatile abdominal mass, flank pain
ruptured abdominal aortic aneurysm
Recurrent apthous ulcers, genital ulcers, and uveitis
Behcet Syndrome Note: Due to immune complex deposition in small vessels
Hyperplasia of what cells occurs in Chronic autoimmune gastritis
Antral G cells Note: Increased gastrin release due to achlorhydria
Duodenal Atresia vs Jejunal/Ileal atresia
Duodenal is due to failure to canalize, Jejunal is due to vascular interruption/ischemia Note: Duodenal atresia presents as polyhydramnios, bilous vomitting, and double bubble sign on x-ray
Unexplained blood in the stool of a young child w/ abd pain
Meckel’s Diverticulum w/ gastric tissue
Macrophages w/ PAS+ staining lysosomes in small bowel lamina propria, fat malabsorption and steatorrhea
Whipple disease (Trophyerma whippelii) Note: Fat malabsortption due to macrophage compression of lacteals
Atherosclerosis of SMA
Ischemic colitis at the splenic flexure Note: Postprandial pain and weight loss
Thin, older person w/ sudden onset diabetes, suspect:
Pancreatic carcinoma Note: smoking is most common cause
Severe pruritis, pale stools, and dark urine
Biliary tract obstruction Note: (conjugated hyperbilirubinemia)
Stain used to differentiate Iron vs. Lipofuschin accumulation in the liver
Prussian Blue Note: used in Dx of Hemochromatosis, Lipofuschin is peroxidized lipids “wear and tear”
Causes of Renal Papillary Necrosis
SCD/Trait, Chronic analgesic abuse (aspirin), Diabetes, Severe acute pyelonephritis Note: Presents as gross hematuria and flank pain
Differentiation of Post-strep glomerulonephritis vs Membranous Nephropathy on EM
Both have subepithelial deposits but Membranous is covered by BM (spike-immune complex and dome-BM)
Hematuria, palpable flank mass, and flank pain
Renal Cell Carcinoma Note: May also have paraneoplastic syndrome: EPO, Renin, ACTH, PTHrP
Renal Cell Carcinoma is associated w/ what gene loss
VHL (chr 3, tumor suppressor) Note: either sporadic (smoking, unilateral) or Von Hippl Lindau Disease (bilateral, +hemangioblastoma and Pheochromocytoma)
Painless hematuria in a smoker
Urothelial/Transitional cell Carcinoma of bladder Note: Flat (early p53 mutation) or Papillary
Secondary amenorrhea due to overaggressive D&C
Asherman Syndrome Note: will not respond to progesterone challenge due to loss of basalis and scarring
Endometrial hyperplasia most likely to progress to carcinoma
Simple hyperplasia w/ atypia Note: atypia is most important
Older man w/ low back pain w/ Increased ALP, normal GGT
Prostatic adenocarcinoma w/ spread to lumbar spine Note: Osteoblastic lesion, PSA and Porstatic Acid Phosphatase (PAP) also elevated
Apoptotic bodies seen in acute viral hepatitis
Councilman bodies
Chronic fatigue, mild weight gain, and inc creatinin kinase
Hypothyroidism
Adult Polycystic Kidney disease is associated w/ what cardiac path and GI path
Mitral Valve prolapse; Diverticulosis
Anti-phospholipase A2 antibodies
Membranous Nephropathy
Vasculitis of graft vessels w/ dense interstitial lymphocyte infiltrate
Acute graft rejection Note: 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
Bone Metastasis from Prostate and Breast
Prostate: Blastic; Breast: Blastic and Lytic
What must be tested if a pt is being treated for Hyperthyroidism
WBC with differential Note: Methimazole and Propylthiouracil both cause agranulocytosis
Ab present in T1DM
Anti-glutamic acid Decarboxylase
First Cardiac sign of Congestive Heart Failure
S3 Note: Best heard at apex in Left lateral decubitus position
What changes in the JVP curve in Afib
No A wave Note: A wave corresponds to atrial contraction; also no S4 if one was present
Why are bibasilar crackles sometimes not heard in pts w/ chronic Left heart failure
Increased lymphatics in the lung drains the transudate
What factor from the ventricles can be used in CHF Dx?
BNP Note: Brain Natriuretic Peptide; If normal, LHF can be excluded
Person has AMI w/ normal coronary arteries
Cocaine use
Mitral Stenosis can cause what GI symptom
Dysphagia for solids Note: LA is posteriorly located right over the esophagus–>dilation of LA in MS causes dysphagia
Mitral valve prolapse due to myxomatous degeneration is associated w/ excess production of what
Dermatan sulfate Note: Floppy, hooded valves, mid systolic click w/ murmur (click due to restrain by chordae)
Decreased preload causes midsystolic click in MVP to:
Move closer to S1
How do you differentiate Aortic Stenosis and Hypertrophic Cardiomyopathy based on murmur?
AS: Intensity increases w/ INCREASED preload
HypCard: Intensity increases w/ DECREASED preload Note:
Anterior leaflet of MV is drawn against the hypertrophied septum–> outflow obstruction
Murmur in AR that signifies need for replacement
Austin Flint murmur Note: Caused by functional mitral stenosis due to AR blood flow hitting the anterior mitral valve leaflet
Huge C-V wave on JVP
Tricuspid regurgitation
Lymphocytic infiltrate in the myocardium
Coxsackie virus Note: Most common cause of myocarditis and pericarditis
Cardiac path associated w/ organic solvents/sniffing glue
Dilated cardiomyopathy
Restrictive cardiomyopathy in a child
Endocardial fibroelastosis Note: Thick fibroelastic tissue in the endocardium
Cardiac defects associated w/ Conotruncal abnormalities
Transposition of great vessels, Tetrology of Fallot, Persistant Truncus Arteriosus Note: Due to failue to migration/rotation of neural crest cells (truncal and bulbar ridges)
People w/ Aortic stenosis are @ risk for what electrical abnormality
Afib; Also @ risk for syncope, angina, and dyspnea on exertion
1 risk factor for native valve bacterial endocarditis in US
Mitral valve prolapse
Hypotension, tachycardia, hypoglycemia, and hyperpigmentation
Adrenal crisis Note: TX w/ corticosteroids due to low circulating cortisol
Polymyositis vs Dermatomyositis HIstology
Poly: CD8+ Tcells w/ endomysial inflammatin
Dermato: CD4+ Tcells w/ perimysial inflammation
Loss of development, verbal abilities intellect, and ataxia in a 1-4 yo girl with hand-wringing
Rett Disorder Note: X-linked, fatal in utero in males
Contralateral hemiparesis w/ ipsilateral tongue deviation
Medial Medullary Syndrome (ASA)
Checkerboard loss of pain and temp, dysphagia, hoarseness, vertigo, nystagmus
Lateral Medullary/Wallenberg syndrome Note: PICA, dysphagia and hoarseness are the most specific (Nucleus ambiguus)
Facial Paralysis, decreased taste from anterior 2/3 of tongue, decreased pain/temp on face,
Lateral Pontine Syndrome (AICA)
Opthalmoplegia in all directions, loss of sensation on forehead and maxillary region, loss of corneal reflex
Cavernous Sinus Syndrome Note: mass effect, fistula, thrombosis; CN 3, 4, 6, V1, V2; Visual acuity not effected
Rapidly progressive dementia w/ myoclonus
Creutzfield-Jakob disease
Sudden onset of back pain, hematuria, and oliguria in a pt w/ anion gap metabolic acidosis
Ethylene glycol intoxication w/ calcium oxylate kidney stone formation
Eosinophilic casts
Multiple Myeloma or Chronic Pyelonephritis Note: Eosinophilurea (eosinophil cells) associated w/ drug-induced interstitial nephritis
Hypoglycemia with low keytones
Acyl-CoA Dehydrogenase deficiency Note: Defect in beta oxidation or ketone body synthesis
Glomerular capillary wall collapse
Focal Segmental Glomerulonephritis in HIV pt Note: poor prognosis
Calluses are marked by increase in what cell layer
Stratum Corneum Note: Hyperkeratosis
Causes of Macroglossia
Myxedema (severe hypothyroidism), Down Syndrome, Acromegaly, Amyloidosis, MEN2B, Plummer Vinson Syndrome, Kawasaki
Infection associated w/ Glossitis
Scarlet Fever (Erythrogenic Toxin) and Hairy Leukoplakia Note: Also Nutritional Deficiencies: Iron, B12, Folate, Vit C, Pellagra/Niacin
Infectious Esophagitis
HSV: punch out lesions; CMV- Linear ulcers, Candida thrush
Melena (dark, tarry stools) indicates what about bleed
Bleed is proximal to duodenojejunal junction Note: Hb converted to hematin by acid
5 things raising suscpicion for Zolliner Ellison Syndrome
Multiple ulcers, ulcers distal to 1st part of duodenum, PUD+diarrhea, ulcers resistant to therapy, MEN1 Note: Also if secretin administration increases gastrin
Biggest risk factor for gastric adenocarcinoma
H. pylori Note: Nitrosamines/Smoked food (Japan) and pernicious anemia also important
Air in urine and recurrent UTI’s in an old person
Colovesicular fistula due to Diverticulosis
GI tumor that secretes protein and K+ rich mucus
Villous adenoma in colon
Portal HTN, ascites, and splenomegaly w/o hepatomegaly in a skinny non-drinker and normal transaminases
Portal Vein Thrombosis Note: Pylephlebitis (inflammation from appendicitis), Polycythema vera, Paroxysmal Nocturnal hematuria
What pain drug is contraindicated in acute cholecystitis
Morphine Note: morphine constricts the sphincter of Oddi; use meperidine or fentanyl
Painful swelling of the tibial tuberosity at the insertion of the patella in a boy 11-15 years old
Osgood-Schlatter disease Note: permanently “knobby” knees
Proximal joint pain associated w/ Temporal Arteritis
Polymyalgia Rheumatica
Munro Microabscesses
Psoriasis Note: Neutrophils in stratum corneum
Upper 1/3 of the vagina is columnar epithelium
Vaginal adenosis Note: Related to Diethylstilbestrol, failure of squamous replacement of upper vaginal epithelium
Common non-neoplastic cause of death w/ cervical cancer
Renal failure to due hydronephrosis Note: Invasion of bladder wall and obstruction of ureters–>post-renal azotemia
Transient, peritoneal abdominal pain mid-cycle in female
Mittelschmerz Note: Peritoneal irritation from ruptured follicle
Ovarian Teratoma w/ functioning thyroid tissue
Struma ovarii
Call-Exner Bodies tumor? Reinke crystals tumor?
Call-Exner bodies–Granulosa-Theca cell tumor
Reinke crystals– Leydig cell Tumor Note: Resemble follicles, may produce estrogen
May produce androgens
Opening of the prepuce is too small
Phimosis Note: Can result from Balanoposthitis (infection of glans/prepuce)
Greatest risk factor for Squamous cell carcinoma of penis
Non-circumsized w/ poor hygeine
Increased FSH, Dec Inhibin, normal Testosterone and LH
Defect in Sertoli cells Note: Can be caused by Varicocele
What indicates erectile dysfunction is psychogenic
Presence of Nocturnal Penile Tumesence
Small cystic spaces in the Internal capsule or Thalamus
Lacunar infarcts Note: Due to Lipohyalinosis of Lenticulostriate arteries w/ HTN or diabetes
Albinism, increased pyogenic infections, giant granules in leukocytes, peripheral neuropathy
Chediak-Higashi Syndrome Note: Failure of phagosome-lysosome fusion
Mutated in Hyper-IgM Syndrome
CD40L or CD40 Note: prevents B cells isotype switching
Anti-U1 ribonucleoprotein
Mixed Connective Tissue disease Note: elements of SLE, systemic sclerosis, and polymyositis
Pentad of findings for HUS and TTP
Microangiopathic Hemolytic Anemia
Thrombocytopenia
Fever, renal insufficiency, CNS Abnormalities Note: Both have hemolytic anemia and Thrombocytopenia
TTP has CNS abnormalities more
HUS has renal insufficiency more
Nitrogen gas emboli are associated w/ what other symptom
joint and muscle pain Note: chronic form can cause necrosis of bone
Unlike in Thalassemia, Extramedullary hematopoesis in Sickle Cell Disease does not include what organ
Spleen Note: Due to autosplenectomy; Both have “crew cut” skull, chipmunk faces, and hepatomegaly
Aplastic anemia due to defective telomerase
Dyskeratosis Congenita
Aortic Valve stenosis vs. Chronic Rheumatic fever of aortic val
Rheumatic fever leads to fusion of the commissures Note: RF usually also w/ mitral stenosis, which is absent in “wear and tear” AS
Lung changes in cigarette smoke
Increased mucus production, decreased ciliary movement, decreased alveolar macrophage activity
Two characteristic CXR findings of emphysema
Flat Diaphragm and vertical heart
Calcified “coin lesion” in the lung containing lung tissue and Cartilage
Bronchial Hamartoma
Lung cancer that only grows along bronchioles and alveoli
Broncioalveolar Carcinoma Note: Clara Cells
Unique metastasis site of lung cancer
Adrenal gland
Obstructing stone in salivary gland leading to Staph infection
Sialadenitis
Fibrosis in Ideopathic Pulmonary Fibrosis and Cirrhosis is mediated by:
TGF-B Note: Ideopathic pulmonary fibrosis is Honeycomb lung
Ovarian tumor associated w/ pleural effusion and ascites
Fibroma Note: Meig’s Syndrome
Course facial features, umbilical hernia, macroglossia, mental retardation, short stature, skeletal abnomalitis in young kid
Cretinism Note: Most commonly due to thyroid agenesis
Signs of Hypocalcemia
Trousseau’s and Chvosek signs
circumoral numbness and tingling
Low Ca, high PTH, short height and 4th and 5th metacarpals
Albright’s Hereditarry Osteodystrophy or Pseudohypoparathyroidism
Death in Uncal herniation
Duret Hemorrhage Note: Rupture of paramedian artery
Three findings of Multiple Sclerosis Spinal Tap
Lymphocytes, Oligoclonal IgG band, Myelin Basic Protein
Supratentorial cystic tumor in a child w/ HX of seizures
Ganglioglioma
Extreme Friendliness w/ strangers, Elfin face, intellectual disability, hypercalcemia
Williams Syndrome Note: Deletion of long arm of Chr 7
Lesioned in Hemiballismus
Contralateral subthalamic nucleus
Hypotonia and tongue fasciculations in a newborn
Spinal Muscular Atrophy/Werndig Hoffman
Renal tumor w/ highly eosinophilic cells and central scar
renal oncocytoma Note: Benign epithelial tumor
Fever, rash, hematuria, CVA tenderness, and eosinophilia 1-2 weeks after starting a drug
Drug-induced Interstitial Nephritis (Tubulointerstitial nephritis) Note: Eosinophils in urine; NSAIDS, Diuretics, Penecillin, Sulfonamides
Granulosa cells in eosinophilic fluid
Call-Exner bodies–Granulosa-Theca cell tumor
Failure of copper absorption leading to Kinky hair, growth retardation, and arterial fragility/aneurysms/rupture
Menkes Disease Note: 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
Rh- mother reaction against Rh+ fetus
Erythroblastosis fetalis Note: if daughter survives, no risk of that happening when they get pregnant b/c daughter is Rh+
In pts w/ COPD what controls respirations
Peripheral Chemoreceptor response to O2 Note: 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
Aphasia, olfactory hallucinations, and personality changes
HSV-1 Temporal Lobe encephalopathy
Pts w/ RCA MI may have Bradycardia due to:
Infarct of SA and AV nodes (RCA supplies) Note: TX w/ atropine to decrease muscarinic input except in glaucoma pts
Crohn’s pts are at risk for what two types of stones
Calcium Oxylate kidney stones
Cholesterol gallstones Note: Increased Oxylate absorption
Decreased Bile acid reabsorption/cycling
Neurologic Manifestation of Wilson’s Disease
Basal Ganglia Degeneration (parkinsonian symptoms) Note: Also dementia, dyskinesia, and dysarthria
Two things that cause increased skin pigmentation
Addison’s Disease (ACTH) and Hemochromatosis
Overproduction of abnormal platelets w/ bleeding and thrombosis
Essential Thrombocytosis Note: Chronic Myeloproliferative Disorder, 50% have JAK2 mutations, Bone marrow contains enlarge megakaryocytes
3 key complications of Sjogren’s Syndrome
Dental Carries (Xerostomia), Corneal damage (Xeropthalmia), MALT Lymphoma
Rosenthal Fibers
Pilocystic Astrocytoma
Focal Segmental Glomerulosclerosis associations
HIV, Sickle Cell Disease, Heroin Abuse, obesity
Membranous Nephropathy associations
HepB, Solid Tumors, Anti-Phospholipase A2, SLE
If a hyperthyroid pt has fever and sore throat, what test should be done
CBC or WBC b/c Methimazole and PTU both cause agranulocytosis
Histology of Preeclampsia
Fibrinoid Necrosis of the placental vessels
Most common manifestations of systemic amyloidosis
Amyloid Nephropathy (Nephrotic), Restricted cardiomyopathy, Tongue enlargement Note: Abdominal fat pad and rectum are easy spots for biopsy
Builds up in urine of SCID pts
Deoxyadenosine (if Adenosine Deaminase deficient) Note: Also caused by IL-2 receptor mutations and MHC Class II deficiency
Antiphospholipid antibody syndrome presents w/ prolonged PTT, how does it present clinically
Arterial and Venous thrombosis (DVT, Hepatic Vein thrombosis/Budd-Chiari, Recurrent Pregnancy loss (placental thrombosis)
Good Prognosis ALL
t(12,21)
Leukemia w/ hypogammaglobulinemia (infection risk), autoimmune hemolytic anemia, Richter Transformation, and smudge cells
Chronic Lymphocytic Leukemia Note: Richter transformation to Diffuse Large B cell lymphoma
Intermediate Size B Cell Lymphoma? Large sized?
Intermediate- Burkitts; Large: Diffuse Large B cell
Adolescent w/ pathological fracture due to mass w/ eosinophils and langerhans cells
Eosinophilic Granuloma Note: Type of Langerhans Cell Histiocytosis–>S-100+ and Birbeck granules
Arteriolosclerosis of the renal arterioles due to long standing HTN or diabetes causing glomerular scarring and CRF
Arteriolonephrosclerosis
Mechanism of Abdominal Aortic Aneurysm
Atherosclerosis decreases O2 diffusion to the media (abd aorta has no vasa vasorum)
Wilm’s Tumor + progressive glomerular disease and male pseudohermaphroditism
Denys-Drash Syndrome
Wilm’s Tumor + muscular hemihypertrophy, neonatal hypoglycemia, and organomegaly (tongue)
Beckwith–Wiedemann syndrome Note: WT2 gene imprinting
Graves Ab bind TSH receptor on retro-orbital and pre-tibial fibroblasts leading to production of what substance
Glycosaminoglycans (chondroitin sulfate and hyaluronic acid) Note: IgG can cross the placenta causing thyroid enlargement in a newborn w/ possibly airway compromise
Addison disease is usually autoimmune, if it is caused by metastatic cancer, where is the cancer from?
Lungs Note: also caused by TB
Systemic Effects of Cushings (not fat distribution)
Muscle weakness, Osteoporosis, Immunesuppression, hypertension
What exacerbates the septic hypotension in Waterhouse Friederichsen syndrome
Lack of Cortisol (permissive for alpha1) Note: Neisseria meningitidis sepsis w/ DIC and destruction of the adrenal glands
Pt presents w/ acute mastitis that did not improve w/ antibiotics (swollen/inflammed breast), Consider what?
Inflammatory Carcinoma Note: Inflamed swollen breast w/ peau de orange; can occur in breastfeeding women; tumor cells in the dermal lymphatics
Bile pigment deposited in hepatic parenchyma and green-brown plugs in dilated bile canaliculi
Cholestasis
Herald patch followed by multiple plaques w/ Collarette scale
Pityiasis rosea Note: Christmas tree distribution on back
