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What is The test used to diagnose Dubin Johnson syndrome is and explain in detail?
Answer is B (Bromsulphalein / Sulfobromophthalein) The appearance of late rise in plasma level of Bromsulphalein, BSF / Sulfobromophthalein in the clearance of this dye has been considered as a oathormontie sign of Dubin Johnson syndrome - Metabolism of Bromsulphalein in D-J syndrome; Digestive diseases and Sciences Vol/17 numbers 6. Patients with D - J syndrome exhibit a characteristic rise in plasma concentration of Bromsulphalein at 90 minutes after injection due to reflux of conjugated BSP into circulation from Hepatocyte. - Harrison Bromsulphalein test for Dubin Johnson syndrome : Harrison 16th / 1821 Dubin Johnson syndrome is characterized by a Familial / Autosomal Recessive defect in Hepatic excretory function (fom hepatocyte to biliary canaliculi ) Biliary excretion of a number of anionic compounds is compromised including that of sulfohromphthalein (Bromsulphalein, BSP), a synthetic dye (cholecystographic agent). To test for D -- J syndrome, the role of disappearance of BSP from plasma is determined after its bolus intravenous administration. Patients with D - J syndrome exhibit a characteristic rise in its plasma concentration at 90 minutes after injection due to reflux of conjugated BSP into circulation from Hepatocyte. -- Harrison Dubin Johnson Syndrome : Review During Johnson syndrome is an autosomal recessive() hereditary disorder presenting with conjugated hyperbilirubinemiaQ due to defect in hepatic excretory function across the canalicular membrane of hepatocyte.() Clinical presentation * Most patient are asymptomatic (subclinical chemical hyperbilirubinemia) * Unexplained low grade jaundice during teenage years Often exacerbated by : -- Oral contraceptive use -- Pregnancy -- Intercurrent illness * Physical examination is usually normal (occasional patient may have hepatosplenomegaly) Laboratory studies * Coniuifated Ilvperbilirubinemia :Total bilirubin is typically between 2 -- 5 mg/dl (34 -- 85 ymol/L) May occasionally he Normal or as high as 20-25 mg/dI(340-430 ,umol/L) * Bilirubinitria :Typically present as hyperbilirubinemia is conjugated * Other Routine Liver Function Tests including Serum Transaminases and Alkaline Phosphatase are essentially Normal Diagnosis Bromsulphalein test :Elaborated above Histology : Corporphyrin test Deposition of melanin like pigment occurs in the (Preferential urinary excretion of corporphyrin-l) liver causing liver to appear dark or completely black. Corporphrins are byproducts of heme biosnthesis There are two naturally occurring corporphyrins -- I & III Pigment is thought to be derived from epinephrine Normally approx. 75 % of corporpyrin in urine is isomer III metabolites that are not excreted normally In urine of patients with D-J Syndrome total corporphyrin content in urine is norma but > 80 % is Isomer I