Dubin-Sprinz syndrome

デュビン・ジョンソン症候群

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• 1. 高抱合型ビリルビン血症関連の遺伝性疾患inherited disorders associated with conjugated hyperbilirubinemia [show details]
  … Dubin and Johnson and Sprinz and Nelson described patients with predominantly… and MRP2 (causing Dubin-Johnson syndrome). The genetic basis of Rotor syndrome has not been identified . Although Dubin-Johnson syndrome and Rotor syndrome have…
• 2. 肝酵素および肝機能検査値の異常が認められた患者に対するアプローチapproach to the patient with abnormal liver biochemical and function tests [show details]
  …in conjugated bilirubin is found in two rare inherited conditions: Dubin-Johnson syndrome and Rotor syndrome. Dubin-Johnson syndrome and Rotor syndrome should be suspected in patients with mild hyperbilirubinemia …
• 3. 既存の慢性肝疾患を有する女性における妊娠pregnancy in women with pre existing chronic liver disease [show details]
  …syndrome is not exacerbated by pregnancy . By comparison, the conjugated hyperbilirubinemia of Dubin-Johnson syndrome may worsen during gestation but returns to baseline levels after delivery . Examples of…
• 4. 黄疸または無症候性高ビリルビン血症成人患者に対する診断的アプローチdiagnostic approach to the adult with jaundice or asymptomatic hyperbilirubinemia [show details]
  …liver test abnormalities is found in two rare inherited conditions: Dubin-Johnson syndrome and Rotor syndrome. Dubin-Johnson syndrome and Rotor syndrome should be suspected in patients with mild hyperbilirubinemia …
• 5. ビリルビン代謝bilirubin metabolism [show details]
  …(eg, alcoholic or viral hepatitis, cholestasis of pregnancy) and inherited disorders (eg, Dubin-Johnson syndrome, Rotor syndrome, benign recurrent intrahepatic cholestasis). It can also be caused by a variety …

English Journal

• PMID 30020687

• Primary Sclerosing Cholangitis: A Concise Review of Diagnosis and Management.

• Fricker ZP, Lichtenstein DR.
• Digestive diseases and sciences. 2019 Mar;64(3)632-642.
• Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterized by progressive idiopathic stricturing of the biliary system, typically leading to cirrhosis, end-stage liver disease, and colonic or hepatobiliary malignancy. Its presentation is often that of asymptomatic alka
• PMID 30725292
• PMID 30725679

Related Links

• Dubin-Sprinz syndrome - RightDiagnosis.com

Dubin-Sprinz syndrome symptoms, causes, diagnosis, and treatment information for Dubin-Sprinz syndrome (Dubin-Johnson Syndrome) with alternative diagnoses, full-text book chapters, misdiagnosis, research treatments ...

• Dubin-Johnson syndrome - Genetics Home Reference - NIH

Dubin-Johnson syndrome is a condition characterized by jaundice, which is a yellowing of the skin and whites of the eyes. In most affected people jaundice appears during adolescence or early adulthood. Jaundice is typically the only ...

• Sprinz-Dubin syndrome | definition of Sprinz-Dubin syndrome ...

Looking for online definition of Sprinz-Dubin syndrome in the Medical Dictionary? Sprinz-Dubin syndrome explanation free. What is Sprinz-Dubin syndrome? Meaning of Sprinz-Dubin syndrome medical term. What does Sprinz ...

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★リンクテーブル★

リンク元	「デュビン・ジョンソン症候群」
関連記事	「syndrome」

「デュビン・ジョンソン症候群」

　　[★]

英

Dubin-Johnson syndrome, DJS

同

Dubin-Johnson症候群、デュービン・ジョンソン症候群

デュビン・スプリンツ症候群 Dubin-Sprinz syndrome

検査

• (腹腔鏡などによる)肉眼所見：黒色肝
• ブロムスルファレイン試験 BSP試験：45分前まではブロムスルファレインの血中濃度は低下傾向にあるが、45分以降血中濃度が再上昇していく。グルクロン酸抱合を受けたBSPであるとされる。　→　肝細胞にtrapされて抱合を受けるが、排泄できないのでleakしてきたのであろうか？？

体質性黄疸 (IMD.875)

体質性黄疸	遺伝形式*	ビリルビン型	ビリルビン値	予後
Crigler-Najjar症候群I型	常染色体劣性	間接型(非抱合)	21-31mg/dl	致死的
Crigler-Najjar症候群II型	常染色体劣性		8-18mg/dl	良好
Gilbert症候群	常染色体優性		<6mg/dl	きわめて良好
Dubin-Johnson症候群	常染色体劣性	直接型(抱合)	2-5(-25)mg/dl	きわめて良好
Rotor症候群	常染色体劣性		2-5(-20)mg/dl	きわめて良好
*劣性か優性かについては例外あり

参考

• 1. [charged] Inherited disorders associated with conjugated hyperbilirubinemia - uptodate [1]

「syndrome」

　　[★]

• n.

• 症候群