BENIGN FAMILIAL ICTERUS: A REPORT OF THREE CASES*

doi:10.1059/0003-4819-50-1-115

BENIGN FAMILIAL ICTERUS: A REPORT OF THREE CASES*

S. ROSS FOX, JR., M.D.

Requests for reprints should be addressed to S. Ross Fox, Jr., M.D., Senior Assistant Surgeon, United States Public Health Service, U. S. Public Health Service Hospital,
Seattle, Washington
.

Excerpt

Benign familial icterus is a clinical entity, not infrequently encountered, that is often confused with hemolytic disease, biliary tract disease, and disease of the liver. For this reason it deserves discussion. The purpose of this paper is to substantiate further the observation that the disorder tends to be hereditary. Three patients, a father and his two sons, are discussed.

CASE REPORTS (Laboratory Results Table 1) Case 1. A 22 year old white Coast Guardsman was admitted on July 2, 1956, complaining of jaundice of two and one-half years' duration. Scleral icterus was first noted by the patient's mother after he

This 100-word excerpt has been provided in the absence of an abstract.

Acknowledgments

Dr. David D. Kliewer and Dr. James G. Telfer contributed valuable suggestions and encouragement, both appreciated by the author.

Summario in Interlingua

Benigne ictero familial es un entitate clinic que simula frequentemente morbo hemolytic, morbo del vias biliari, o morbo hepatic. Es presentate tres casos—de un patre e su duo filios—que demonstra un tendentia hereditari in iste disordine. Il pare que illo es transmittite como dominante mendelian.

Patientes qui suffre del condition hic discutite presenta un historia de episodios intermittente de jalnessa in que le exacerbationes es precipitate per stress emotional, excessos de fatiga, infectiones, dysfunction gastrointestinal, o ingestion de alcohol.

Le examine physic es usualmente intra le limites del norma, con le exception del ictero, sed isto non es necessarimente apparente ab le puncto de vista clinic.

In partitionar le bilirubina seral secundo van den Bergh, on trova que le fraction indirecte es elevate durante que le fraction directe es normal o minimalmente elevate. Le concentration urinari e fecal de urobilinogeno, le numeration reticulocytic, e studios del medulla ossee es usualmente normal. Le frottis de sanguine peripheric es normal, e le mesmo vale pro le fragilitate osmotic del erythrocytes e pro le tests de Coombs. Studios del vesica biliari es usualmente normal.

Studios del function hepatic es rarmente anormal, excepte le test de tolerantia pro bilirubina. Isto revela communmente un retention prolongate del bilirubina a reaction indirecte.

Studios laboratorial resulta in nulle indication de significative morbo hepatocellular, dysfunction del vias biliari, o de morbo hemolytic.

Multe theorias ha essite formulate con respecto al etiologia de benigne ictero familial. Recentemente il ha essite demonstrate que post que le molecula cyclic de heme se ha aperite, le cellula hepatic attacha duo radicales glucuronidic a ille composito. Isto augmenta le solubilitate del substantia, e le hepate es capace de excerner lo.

Benigne ictero familial es ben interpretabile como effecto de un reducite capacitate del parte del hepate de conjugar bilirubina a reaction indirecte con acido glucuronic, e iste reducite capacitate esserea le effecto de un specific carentia enzymatic. Il es possibile que le tendentia de disveloppar un tal carentia es transmittite geneticamente.

Le prognose in iste disordine es semper favorabile.