77:
148:
47:
304:
A test of MRP2 activity can also be done to differentiate between Dubin–Johnson syndrome and Rotor syndrome. The clearance of bromsulphthalein is used to determine this, the test for which is called bromsulphthalein clearance test. 100 units of BSP is injected intravenously and then the clearance. In
313:
Dubin–Johnson syndrome is a benign condition and no treatment is required. However, it is important to recognize the condition so as not to confuse it with other hepatobiliary disorders associated with conjugated hyperbilirubinemia that require treatment or have a different prognosis.
130:
Around 80 to 99% of people with Dubin–Johnson syndrome have jaundice, abnormal urinary color, biliary tract abnormality, and conjugated bilirubinemia. Around 30 to 79% of people with the disorder have abnormality of the gastric mucosa. Other rare symptoms include fever and fatigue.
117:
in the serum. Classically, the condition causes a black liver due to the deposition of a pigment similar to melanin. This condition is associated with a defect in the ability of hepatocytes to secrete conjugated bilirubin into the bile, and is similar to
720:
705:
139:
The conjugated hyperbilirubinemia is a result of defective endogenous and exogenous transfer of anionic conjugates from hepatocytes into bile. Impaired biliary excretion of bilirubin glucuronides is due to a mutation in the canalicular
194:
For the first two days of life, healthy neonates have ratios of urinary coproporphyrin similar to those seen in patients with Dubin–Johnson syndrome; by 10 days of life, however, these levels convert to the normal adult ratio.
305:
case of Dubin–Johnson syndrome, clearance of bromsulphthalein will be within 90 minutes, while in case of Rotor syndrome, the clearance is slow, i.e., it takes more than 90 minutes for clearance.
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853:
322:
Prognosis is good, and treatment of this syndrome is usually unnecessary. Most patients are asymptomatic and have normal lifespans. Some neonates present with cholestasis.
1400:
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1186:
1385:
186:
In patients with Dubin–Johnson syndrome, this ratio is inverted, with coproporphyrin I being 3–4 times higher than coproporphyrin III. Analysis of urine
141:
493:
167:. It is an autosomal recessive disease and is likely due to a loss of function mutation, since the mutation affects the cytoplasmic/binding domain.
1078:
846:
1405:
1273:
467:
735:
668:
1410:
1071:
839:
385:
1131:
620:"Chronic idiopathic jaundice with unidentified pigment in liver cells; a new clinicopathologic entity with a report of 12 cases"
420:
Strassburg CP (2010). "Hyperbilirubinemia syndromes (Gilbert-Meulengracht, Crigler-Najjar, Dubin-Johnson, and Rotor syndrome)".
122:. It is usually asymptomatic, but may be diagnosed in early infancy based on laboratory tests. No treatment is usually needed.
939:
343:
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156:
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323:
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1312:
1063:
819:
1013:
360:
175:
A hallmark of Dubin–Johnson syndrome is the unusual ratio between the byproducts of heme biosynthesis:
190:
shows a normal level of coproporphyrin, but the I isomer accounts for 80% of the total (normally 25%).
1308:
1198:
1147:
662:
76:
1390:
1347:
1163:
969:
724:
216:. An isoform of this protein is localized to the apical hepatocyte membrane, allowing transport of
110:
494:"Dubin-Johnson syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program"
459:
386:"Dubin-Johnson syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program"
85:
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61:
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964:
631:
583:
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1257:
1119:
1098:
930:
674:
393:
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normal with >80% being isomer 1 (normal urine contains more of isomer 3 than isomer 1)
213:
144:. A darkly pigmented liver is due to polymerized epinephrine metabolites, not bilirubin.
1351:
1335:
1048:
289:
237:
180:
119:
729:
1379:
808:
636:
619:
578:
561:
164:
682:
212:
Plentiful canalicular multiple drug-resistant protein causes bilirubin transfer to
797:
751:
781:
274:
221:
217:
608:
Up-to-date: "Inherited disorders associated with conjugated hyperbilirubinemia"
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114:
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331:
151:
Dubin–Johnson syndrome has an autosomal recessive pattern of inheritance.
697:
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206:
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46:
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1154:
709:
228:(GGT) help in diagnosing pathologies involving biliary obstruction.
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1330:
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1252:
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1228:
1193:
1181:
1138:
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1114:
346:
and
Isadore Dubin first described Dubin–Johnson syndrome in 1954.
261:
160:
146:
113:, benign disorder that causes an isolated increase of conjugated
862:
740:
1067:
835:
205:, the liver will have a dark pink or black appearance due to
560:
Rocchi E, Balli F, Gibertini P, et al. (June 1984).
458:
Gensler, Ryan; Delp, Dean; Rabinowitz, Simon S. (2020).
422:
Best
Practice & Research: Clinical Gastroenterology
687:
562:"Coproporphyrin excretion in healthy newborn babies"
280:
gallbladder can be visualized by oral cholecystogram
1322:
1295:
1220:
1173:
1106:
1032:
1002:
987:
948:
931:
Gunther disease/congenital erythropoietic porphyria
919:
894:
870:
772:
691:
566:
Journal of
Pediatric Gastroenterology and Nutrition
94:
84:
60:
36:
31:
155:Dubin–Johnson syndrome is due to a defect in the
415:
413:
411:
1211:Progressive familial intrahepatic cholestasis 2
1187:Progressive familial intrahepatic cholestasis 3
464:NORD (National Organization for Rare Disorders)
224:conjugates back into the blood. High levels of
334:and icterus (yellowing of the eyes and skin).
1079:
847:
8:
1396:Syndromes affecting the hepatobiliary system
183:III to coproporphyrin I ratio around 3–4:1.
1086:
1072:
1064:
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891:
854:
840:
832:
688:
75:
45:
28:
635:
577:
142:multiple drug-resistance protein 2 (MRP2)
618:Dubin, IN; Johnson, F (September 1954).
522:
520:
518:
242:
377:
236:Dubin–Johnson syndrome is similar to
7:
1401:Membrane transport protein disorders
488:
486:
484:
453:
451:
1386:Accessory digestive gland disorders
1132:Surfactant metabolism dysfunction 3
232:Differentiation from Rotor syndrome
14:
283:gallbladder cannot be visualized
637:10.1097/00005792-195409000-00001
579:10.1097/00005176-198406000-00017
295:high with <70% being isomer 1
240:, but can be differentiated by:
90:Jaundice, otherwise asymptomatic
470:from the original on 2016-04-25
266:normal histology and appearance
940:Hepatoerythropoietic porphyria
1:
1406:Autosomal recessive disorders
989:Hereditary hyperbilirubinemia
975:Erythropoietic protoporphyria
269:liver has black pigmentation
40:Conjugated Hyperbilirubinemia
911:Acute intermittent porphyria
671:at Medline Plus encyclopedia
179:Unaffected subjects have a
1432:
226:gamma-glutamyl transferase
15:
1411:Heme metabolism disorders
1361:
1286:Dilated cardiomyopathy 1O
1160:Harlequin-type ichthyosis
960:Hereditary coproporphyria
498:rarediseases.info.nih.gov
434:10.1016/j.bpg.2010.07.007
390:rarediseases.info.nih.gov
53:
44:
1246:Pseudoxanthoma elasticum
549:. Elsevier. p. 639.
460:"Dubin Johnson Syndrome"
157:multiple drug resistance
18:Stevens–Johnson syndrome
16:Not to be confused with
1148:Retinitis pigmentosa 19
1024:Lucey–Driscoll syndrome
1019:Crigler–Najjar syndrome
936:Porphyria cutanea tarda
547:Robbins Basic Pathology
366:Crigler–Najjar syndrome
324:Hormonal contraceptives
1234:Dubin–Johnson syndrome
1044:Dubin–Johnson syndrome
809:Dubin–Johnson syndrome
675:Dubin–Johnson syndrome
669:Dubin-Johnson syndrome
663:Dubin-Johnson syndrome
529:Dubin–Johnson Syndrome
254:Dubin–Johnson syndrome
152:
107:Dubin–Johnson syndrome
32:Dubin–Johnson syndrome
1313:Adrenomyeloneuropathy
1164:Lamellar ichthyosis 2
545:Kumar, Vinay (2007).
526:Suzanne M Carter, MS
150:
23:Genetic liver disease
1309:Adrenoleukodystrophy
897:early mitochondrial:
865:metabolism disorders
624:Medicine (Baltimore)
1348:Gallbladder disease
1144:Stargardt disease 1
970:Variegate porphyria
951:late mitochondrial:
111:autosomal recessive
1014:Gilbert's syndrome
773:External resources
665:at MedlinePlus.gov
361:Gilbert's syndrome
330:may lead to overt
153:
126:Signs and symptoms
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26:Medical condition
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1366:ABC transporters
1095:Genetic disorder
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1007:
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965:Harderoporphyria
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500:. Archived from
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396:on 14 April 2021
392:. Archived from
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159:protein 2 gene (
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1099:ABC transporter
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214:bile canaliculi
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135:Pathophysiology
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27:
24:
21:
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11:
5:
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1416:Rare syndromes
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1393:
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1352:Sitosterolemia
1339:
1336:Sitosterolemia
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1049:Rotor syndrome
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692:Classification
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657:External links
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630:(3): 155–198.
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260:Appearance of
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209:accumulation.
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181:coproporphyrin
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683:Rare Diseases
681:'s Office of
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504:on 2021-09-30
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344:Frank Johnson
342:Pathologists
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165:chromosome 10
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922:cytoplasmic:
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588:11380/612304
572:(3): 402–7.
569:
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546:
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527:
506:. Retrieved
502:the original
497:
472:. Retrieved
463:
425:
421:
398:. Retrieved
394:the original
389:
380:
341:
321:
312:
303:
288:Total urine
253:
248:
235:
211:
199:
197:
193:
174:
154:
138:
129:
106:
105:
1035:conjugated:
782:MedlinePlus
275:Gallbladder
222:glutathione
218:glucuronide
200:post mortem
109:is a rare,
37:Other names
1391:Hepatology
1380:Categories
804:Patient UK
758:DiseasesDB
508:2021-09-30
474:2021-09-30
372:References
188:porphyrins
71:hepatology
67:Pediatrics
1364:see also
1101:disorders
994:bilirubin
886:porphyrin
872:Porphyria
793:eMedicine
534:eMedicine
328:pregnancy
318:Prognosis
309:Treatment
171:Diagnosis
115:bilirubin
96:Prognosis
62:Specialty
55:Bilirubin
815:Orphanet
646:13193360
468:Archived
442:20955959
400:11 April
356:Jaundice
350:See also
332:jaundice
86:Symptoms
877:hepatic
798:med/588
752:D007566
596:6737185
338:History
292:content
207:pigment
203:autopsy
1206:ABCB11
1155:ABCA12
787:000242
741:237500
644:
594:
440:
73:
1343:ABCG8
1331:ABCG5
1304:ABCD1
1281:ABCC9
1274:TNDM2
1265:ABCC8
1253:ABCC7
1241:ABCC6
1229:ABCC2
1194:ABCB7
1182:ABCB4
1139:ABCA4
1127:ABCA3
1115:ABCA1
730:277.4
715:E80.6
262:liver
161:ABCC2
1323:ABCG
1296:ABCD
1270:HHF1
1221:ABCC
1199:ASAT
1174:ABCB
1107:ABCA
879:and
863:Heme
763:3982
747:MeSH
736:OMIM
725:9-CM
642:PMID
592:PMID
438:PMID
402:2019
326:and
220:and
100:Good
1350:4,
820:234
721:ICD
706:ICD
679:NIH
677:at
632:doi
584:hdl
574:doi
532:at
430:doi
198:In
1382::
1311:,
1272:,
1162:,
1146:,
818::
807::
796::
785::
761::
750::
739::
728::
713::
710:10
640:.
628:33
626:.
622:.
590:.
582:.
568:.
564:.
517:^
496:.
483:^
466:.
462:.
450:^
436:.
426:24
424:.
410:^
388:.
69:,
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1346:(
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1158:(
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884:(
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648:.
634::
598:.
586::
576::
570:3
511:.
477:.
444:.
432::
404:.
20:.
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