GI Flashcards
Plummer Vinson Syndrome
Esophageal web and atrophic glossitis. Presents as microcytic iron def anemia, dysphagia, and beefy red tongue
cholangioncarinoma is a complication of
primary sclerosing cholangitis; Chinese liver fluke
Weil dx associated with
lepto
swimming in contaminated water`
necrotizing migrating erythema
glucogonoma`
CA19-19 marker for
pancreatic carcinoma
Mutation in FGF
Achondroplasia - arrests maturation of chondrocytes
Mutation in collage gene
Osteogenesis imperfecta
Mutation in lysyl hydroxylase gene
Ehlers-Danlos syndrome (hyperfelxability of joints and fragility of skin, heart valves, and other organ tissues)
Pott’s disease
Tuberculous spondylitis - vertebral body as almost completely replaced by tuberculous tissue. Intervertebral disk preserved.
Eosinophilic granuloma
Langerhans cell histiocytosis (localized form)
Typically one or two lytic areas in bones of the axial or vertebrae or skull.
70% of LCH
Hand-Schuller-Christian disease
Langerhans cell histiocytosis (disseminated variant)
kids 2-5
Radiolucent bony lesions
Involvement of jaw bone = floating teeth
Infiltration of retro orbital space = exopthalmous
Crusty, red, weepy skin lesion a hair line and extensors surfaces.
Deafness may result if involve mastoid air cells
Diabetes inspidus of involve hypothalmaic stalk
TRIAD: radiolucent lesions of the skull, DI, exopthalmous
Letterer-Siwe dx
Langerhans cell histiocytosis (fulminate and often fatal generalized dx)
Brown tumor associated with
Primary hyperparathryoidism
brown due to himosiderin laden macrophages and areas of hemorrhage from microfractures (with reactive woven bone)
Hydrops fatalis
Severe edema due to congestive heart failure caused by severe anemia
–often due to Erythroblastosis fetalis
Eosinophilic esophagitis
probably related to allergy
esophagus has transverse ridges and appears like a trachea.
mild peripheral eosiniophilia
c-kit associated with
gastrointestinal stromal tumors
spindle shaped cells present (DE-differentiated)
Inherited disease associated with peritonitits, arthritis, and pluritis?
Familial Mediterranean Fever
Gardner syndrome triad:
Familial adenomatous polposis, fibromatosis, and ostemoas (benign surface skull tumors)
Gilbert syndrome
most common inherited disorder of bilirubin conjugation
decreased levels of uridine diphosphate glucuronyl transferase
AR
increased UCB
Crigler-Najjar syndrome
mutation in the gene coding for UDPGT –> enzyme is severely lacking or completely absent
increase UCB
Kernicterus can be fatal
Dubin Johnson Syndrome
deficiency of bilirubin canalicular transport protein
AR
increased CB
Liver is dark (differs from rotor syndrome)
anti-mitochondrial antibodies
primary biliary cirrhosis
anti-smooth muscle antibodies
autoimmune hepatitis
most frequent sarcoma after radiation
malignant fibrous histocytoma - soft tissue tumor with centers of histocytic (macrophage) differentiation
