PERSISTENT NONHEMOLYTIC HYPERBILIRUBINEMIA ASSOCIATED WITH LIPOCHROMELIKE PIGMENT IN LIVER CELLS: REPORT OF FOUR CASES
Authors: Helmuth Sprinz, Robert S. Nelson
Journal: Annals of Internal Medicine (1954)

Article1 November 1954PERSISTENT NONHEMOLYTIC HYPERBILIRUBINEMIA ASSOCIATED WITH LIPOCHROMELIKE PIGMENT IN LIVER CELLS: REPORT OF FOUR CASESHELMUTH SPRINZ, ROBERT S. NELSONHELMUTH SPRINZ, ROBERT S. NELSONAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-41-5-952 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe more frequent use of needle biopsies in the diagnosis of liver disease has led to the clarification of the pathogenesis in many known clinical conditions. It might be expected that the widespread employment of this procedure would also uncover certain histologic findings which, together with the clinical picture, could not be readily explained under previous concepts. In a total of 630 needle biopsies of the liver performed on the Medical Service of the 98th General Hospital, Munich, Germany, two instances have been noted of unusual biopsy findings which, in our experience, showed no correlation with the initial clinical evaluation...Bibliography1. Dubin IN (a) : Personal communication to the authors. (b) Johnson, F. B., and Dubin, I. N.: Excessive lipochrome pigment in liver cells in constitutional hyperbilirubinemia, Am. J. Path. 29: 585, 1953. Google Scholar2. Gray CH: The bile pigments, in Methuen's Monographs on biochemical subjects, 1953, Methuen, London. Google Scholar3. Himsworth HP: The liver and its diseases, 2nd Ed., 1950, Harvard University Press, Cambridge. Google Scholar4. DibleMcMichaelSherlock JHJSV: Pathology of acute hepatitis, Lancet 2: 402, 1943. CrossrefGoogle Scholar5. SherlockWalshe SVV: The post-hepatitis syndrome, Lancet 2: 482, 1946. CrossrefMedlineGoogle Scholar6. Hult H: "Cholémie simple familiale" (Gilbert) and posthepatitic states without fibrosis of the liver, Acta med. Scandinav. 138: suppl. 244, 1950. Google Scholar7. NelsonSprinz RSH: The effect of physical activity on recovery from hepatitis: a follow-up study 2 to 3 years after onset of disease, Am. J. Med. 16: 780, 1954. CrossrefMedlineGoogle Scholar8. CurryGreenwaltTat JJTJRJ: Familial nonhemolytic jaundice: report of a case with liver biopsy, New England J. Med. 226: 909, 1942. CrossrefGoogle Scholar9. Comfort MW: Constitutional hepatic dysfunction, M. Clin. North America 29: 982, 1945. CrossrefGoogle Scholar10. DameshekSinger WK: Familial nonhemolytic jaundice, constitutional hepatic dysfunction with indirect van den Bergh reaction, Arch. Int. Med. 67: 259, 1941. CrossrefGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: *Received for publication March 16, 1954.From the Medical and Laboratory Services of the 98th General Hospital, Munich, Germany, and Walter Reed Army Hospital, Washington, D. C. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byEvaluation of Hepatic Functional Reserve and Perioperative Course after Hepatectomy in a Case of Dubin-Johnson SyndromeDisorders of Bilirubin MetabolismBile Pigment Metabolism and Its DisordersBilirubin Metabolism and Its DisordersDevelopmental and Inherited Liver DiseaseBilirubin Metabolism and JaundiceLinking organic anion transporting polypeptide 1B1 and 1B3 (OATP1B1 and OATP1B3) interaction profiles to hepatotoxicity - The hyperbilirubinemia use caseVariants of Hepatocellular CarcinomaInherited disorders of bilirubin clearanceVariants of Hepatocellular CarcinomaA Case of Low Anterior Resection and Hepatectomy for the Patient with Dubin-Johnson Syndrome—Report of a Case—Gene Replacement Therapy for Genetic Hepatocellular JaundiceInherited Disorders of Bilirubin Transport and Conjugation: New Insights Into Molecular Mechanisms and ConsequencesMRP2 (ABCC2) and MRP3 (ABCC3)Bile Pigment Metabolism and Its DisordersGenetic and metabolic liver diseaseBilirubin Metabolism and Its DisordersBilirubin Metabolism and JaundiceMultidrug Resistance Proteins (MRPs, ABCCs): Importance for Pathophysiology and Drug TherapyHyperbilirubinemia syndromes (Gilbert-Meulengracht, Crigler-Najjar, Dubin-Johnson, and Rotor syndrome)Approach to the Patient with Cholestasis and JaundiceInterplay of conjugating enzymes with OATP uptake transporters and ABCC/MRP efflux pumps in the elimination of drugsNeonatal Jaundice and Disorders of Bilirubin MetabolismAge estimates of ancestral mutations causing factor VII deficiency and Dubin–Johnson syndrome in Iranian and Moroccan Jews are consistent with ancient Jewish migrationsThe apical conjugate efflux pump ABCC2 (MRP2)A CASE OF HEPATOCELLULAR CARCINOMA ACCOMPANIED WITH DUBIN-JOHNSON SYNDROMETransmembrane Transport of Endo- and Xenobiotics by Mammalian ATP-Binding Cassette Multidrug Resistance ProteinsBilirubin Metabolism and its DisordersMolekulare Ursachen von Störungen des hepatogastroenterologischen Systems bei NeugeborenenTransport of Bilirubin Conjugates across Hepatocellular Membrane Domains and the Conjugated Hyperbilirubinemia of Dubin-Johnson SyndromeCanalicular multispecific organic anion transporter ABCC2Cholestase und IkterusMRP2, THE APICAL EXPORT PUMP FOR ANIONIC CONJUGATESA common Dubin-Johnson syndrome mutation impairs protein maturation and transport activity of MRP2 (ABCC2)Trafficking and functional defects by mutations of the ATP-binding domains in MRP2 in patients with Dubin-Johnson syndromeRadixin deficiency causes conjugated hyperbilirubinemia with loss of Mrp2 from bile canalicular membranesIdentification and Functional Analysis of Two Novel Mutations in the Multidrug Resistance Protein 2 Gene in Israeli Patients with Dubin-Johnson SyndromeRole of transport proteins in drug absorption, distribution and excretionImpaired protein maturation of the conjugate export pump multidrug resistance protein 2 as a consequence of a deletion mutation in dubin-johnson syndromeCholestaseGenetische HyperbilirubinämienConjugate export pumps of the multidrug resistance protein (MRP) family: localization, substrate specificity, and MRP2-mediated drug resistanceExon-intron organization of the human multidrug-resistance protein 2 (MRP2) gene mutated in Dubin–Johnson syndromeGenomic Structure of the Canalicular Multispecific Organic Anion–Transporter Gene (MRP2/cMOAT) and Mutations in the ATP-Binding–Cassette Region in Dubin-Johnson SyndromeThe canalicular multidrug resistance protein, cMRP/MRP2, a novel conjugate export pump expressed in the apical membrane of hepatocytesDefective hepatobiliary leukotriene elimination in patients with the Dubin-Johnson syndromeHepatobiliary secretion of organic compounds; molecular mechanisms of membrane transportBilirubinThe role of the canalicular multispecific organic anion transporter in the disposal of endo-and xenobioticsDefective biliary excretion of epinephrine metabolites in mutant (TR−) rats: Relation to the pathogenesis of black liver in the dubin-johnson syndrome and corriedale sheep with an analogous excretory defectErythrocyte membrane transport of glutathione conjugates and oxidized glutathione in the dubin-johnson syndrome and in rats with hereditary hyperbilirubinemiaDubin-Johnson Syndrome (Black Liver Disease): Report of two CasesPericanalicular microfilaments of hepatocytes in patients with familial non-hemolytic hyperbilirubinemiaHepatobiliary excretion of organic anions in double-mutant rats with a combination of defective canalicular transport and uridine 5′-diphosphate-glucuronyltransferase deficiencyHereditary chronic conjugated hyperbilirubinemia in mutant rats caused by defective hepatic anion transportClinicopathological studies of the Dubin-Johnson syndrome complicated with chronic hepatitisAltered coproporphyrin-isomer excretion in patients with the Dubin-Johnson syndromeDublin-Johnson syndrome with some unusual features in a Chinese family.On the Nature and Excretion of the Hepatic Pigment in the Dubin-Johnson SyndromeHistorical and Clinical Aspects of Bile PigmentsHereditary JaundiceCase 36-1978New insights into the classification and mechanisms of hereditary, chronic, non-haemolytic hyperbilirubinaemiasDie LeberBilirubin Metabolism: Review and Discussion of Inborn ErrorsCongenital Dyserythropoietic Anemia and the Dubin-Johnson SyndromeInheritance of the Dubin-Johnson SyndromePregnancy, Oral Contraceptives, and Chronic Familial Jaundice with Predominantly Conjugated Hyperbilirubinemia (Dubin-Johnson Syndrome)Workshop on comparative gastroenterologyA case of the Dubin-Johnson syndrome complicated by acute hepatitisDUBIN‐JOHNSON SYNDROMEChronic idiopathic jaundice in Papua and New Guinea: A report of nine patients with Dubin-Johnson's or rotor's syndromeJaundiceACUTE RENAL FAILURE COMPLICATING INTRAVENOUS CHOLANGIOGRAPHY IN A PATIENT WITH DUBIN‐JOHNSON SYNDROMEIsolement et propriétés d'une mélanine obtenue à partir de mélanogènes urinaires dans un cas de maladie de dubinjohnsonDubin-Johnson syndrome in immature sheepChronic Familial HyperbilirubinemiaStudies of Chronic Idiopathic Jaundice (Dubin-Johnson Syndrome)Chronic Idiopathic Jaundice (Dubin-Johnson's syndrome) in Three SistersSyndromes of Constitutional HyperbilirubinemiaChronic idiopathic jaundice—Rotor typeThe syndrome of benign recurrent cholestasisHepatitis with Resulting Mobilization of Hepatic Pigment in a Patient with Dubin-Johnson SyndromeIndications for Surgical Intervention in the Jaundiced PatientConstitutional hyperbilirubinemia with unconjugated bilirubin in the serum and pigment deposition in the liverPathology ProblemsInterpretation of the Liver BiopsyStudies of Chronic Idiopathic Jaundice (Dubin-Johnson Syndrome)BILIRUBIN METABOLISM IN DISEASEChronic Familial Nonhemolytic Jaundice with Conjugated Bilirubin in the SerumUntersuchungen �ber den Blutumsatz bei posthepatitischen und anderen funktionellen Hyperbilirubin�mienThree Cases of Chronic Idiopathic JaundiceSpätfolgen der Virus-HepatitisBeurteilung der Leberkrankheiten und ihrer Prognose auf Grund der LeberbiopsieBilirubin Metabolism with Special Reference to Neonatal JaundiceStudies of chronic familial non-hemolytic jaundice with conjugated bilirubin in the serum with and without an unidentified pigment in the liver cellsBLACK LIVER JAUNDICE (DUBIN‐SPRINZ SYNDROME)THE ACUTE CHOLESTATIC SYNDROMECurrent concepts of bilirubin metabolism in jaundiceChronic Idiopathic JaundiceCHRONIC IDIOPATHIC JAUNDICE WITH HEPATIC PIGMENTATION (DUBIN–JOHNSON SYNDROME)Familial Jaundice with Free and Conjugated Bilirubin in the Serum and Without Liver PigmentationChronic Hyperbilirubinemia with Normal Liver Biopsynew Type?Chronic idiopathic jaundiceSTUDIES IN A CASE OF CHRONIC IDIOPATHIC JAUNDICE*EDWARD R. BURKAFamilial Nonhemolytic Jaundice with Normal Liver Histology and Conjugated BilirubinChronic idiopathic jaundiceFamilial chronic idiopathic jaundice (Dubin-Sprinz disease), with a note on bromsulphalein metabolism in this diseaseCongenital Defects in Bilirubin Metabolism as Seen in the AdultBENIGN RECURRENT INTRAHEPATIC " OBSTRUCTIVE " JAUNDICEFamilial Nonhemolytic Jaundice with Conjugated Bilirubin in the SerumBLACK LIVER JAUNDICE DIAGNOSED BY NEEDLE BIOPSYChromatographic Separation of Different Bromsulphalein Metabolites in Urine and Bile1BENIGN FAMILIAL ICTERUS: A REPORT OF THREE CASES*S. ROSS FOX JR., M.D.The Cholecystogram in Chronic Idiopathic Jaundice.CHRONIC IDIOPATHIC JAUNDICEChronic idiopathic jaundiceJaundice Associated with Lipochrome Pigmentation of the Liver (Dubin -Johnson Syndrome)Mavero-Hepatic IcterusHepatitis und posthepatitische LebererkrankungenConstitutional non-hemolytic jaundice with "lipochrome" hepatosis (Dubin-Sprinz disease)Chronic idiopathic jaundicePersistent Nonhemolytic Hyperbilirubinemia with Unidentified Pigment in Liver Cells Report of Case Followed in PregnancyPROLONGED JAUNDICE WITH UNIDENTIFIED PIGMENT IN LIVER CELLS 1 November 1954Volume 41, Issue 5Page: 952-962KeywordsArmed forcesBiopsyCellsClinical pathologyLiverLiver diseasesMedical servicesPathogenesisResearch laboratories ePublished: 1 December 2008 Issue Published: 1 November 1954 PDF downloadLoading ...