Dubin-Johnson Syndrome Seen in 2 Siblings Histopathological Examination

Year 1977, Volume: 30 Issue: 1, 193 - 206, 31.03.1977

Binnaz Ege

https://izlik.org/JA62AL35JD

Abstract

Ages 10 and 12 with Duhin-Johnson Syndrome Clinical, laboratory and histopathological findings of the liver in two sisters has been examined. Clinically, jaundice, fatigue, loss of appetite, occasional abdominal pain, laboratory examination hyperbilirubinemia, increase in the amount of direct bilirubin in the serum, bilirubinuria, light color of the stool, opaque substance administered Inability to see the gallbladder in cholecystography, liver biopsy In the histopathological examination of the material, liver Coarse granular dark brown-black color in parenchyma cells It was determined that a pigment accumulated. Other pathological findings not found.

Keywords

Dubin-Johnson Syndrome , Chronic jaundice , Hyperbilirubinemia

2 Kardeşte Görülen Dubin-Johnson Sendromunda Histopatolojik İnceleme

https://izlik.org/JA62AL35JD

Abstract

Duhin-Johnson Sendromu gösteren ve yaşları 10 ve 12 olan iki kız kardeşte klinik, laboratuvar ve karaciğerin histopatolojik incelenmesi yapılınıştır. Klinik olarak sarılık, halsizlik, iştahsızlık, zaman zaman gelen karın ağrısı, laboratuvar incelenmesinde hiperbilirubinemi, serumda direkt bilirubin miktarında, artma, bilirubinüri, gaitanın açık renk oluşu, opak madde verilerek yapılan kolesistografide safra kesesinin görülemeyişi, karaciğer biopsi materyelinin histopatolojik incelenmesinde ise, karaciğerin parankima hücrelerinde koyu kahverengi-siyah renkte kaba granüler bir pigmentin biriktiği tesbit edildi. Başka patolojik bulgu bulunamadı.

Keywords

Dubin-Johnson Sendromu , Kronik sarılık , Hiperbilirubinemi

References

• 1— AriasI.M.: Recent advances in bilirubin metabolism and their clinical implication. Med. Clin. North Amer. 44: 607, 1960. 2 — Ariasl.M, Gartner 1. M. Cohen M. etal.: Chronic nonhemolytic unconjugated hyperbilirubinemia with glucoronly trensferase defici- ency. Am. J. Med. 47: 395, 1969,
• 3 — Ariasl. M.: Studies of chronic familial nonhemolytic jaundice witli conjugated bilirubin in the serum with and without an unidentified pigment in the levercells. Am. J. Med. 31: 510, 1961.
• 4 — Beker S.. and Read A, E. ; Familial Dubin - Johnson Syndrome. Gas- troenterology, 35: 387, 1958,
• 5—BilingeB.H.: Chromatographic method for determination of 3 bile pigmenits in serum. J, Clin, Path. 8: 126, 1955.
• 6 — Castleman B. : Case records of the Massachusettes general hospital , N. England, J. Med. 258 : 335, 1955,
• 7 — Crigler J. F. and Najjar V. A. ; Congenital familial nonhemolytic jaundice with «kernicterus», Pediatrics 10: 169, 1952. 8 — Dameshek W. and Singer K. : Femilial non-hemolytic jaundice. Cons- titutional hepatic dysfunction with indirekt vandenbergh O reaction. Arch. Int. Med. 67: 258, 1941,
• 8 — Dameshek W. and Singer K. : Femilial non-hemolytic jaundice. Cons- titutional hepatic dysfunction with indirekt vandenbergh O reaction. Arch. Int. Med. 67: 258, 1941,
• 9 — Dubin -I.N. and Johnson E, B. : Chronic idiopathic jaundice with unidentified pigment in liver cells. A new clinico-pathologic Entity with a report of 12 cases. Med. 33: 155, 1954,
• 10 — Dubin 1. N. ; Chronic idiopathic isundice A review of fifty cases. Am. 11 — Ehrlich J.C,, Novikoff A, B.. PlattR., Essner E,; Hepatocellular - . Mpofuscin and the pigment df chronic idiopathiç jaundice. Bull. N, Y, Acad. Med. 38; 488, 1960.
• 12 — Essner E. and Novikoff A. B. : Human hepatocellular pigments and liposomes. J. Ultrastructure Res. 3: 374, 1960. J. Med. 24: 268, 1958.
• 13— Gilbert A., Castaigne J. and Leberoullet P. De Victere familial, contribution w İetude de la diathese biliare. Bull, et mâöm., Soc. Med. de Hop. de Paris 17: 948, 1900.
• 14 — Gilbert A. and Leberoullet P. : La cholemie simple familiale. Semaine Med. 21: 241, 1901.
• 15 — Gilbert A. Leberoullet P. and Herscher M. : Les trois cholemies Congenitales. Bull. et möm, Soc. Med. de Hop. de Paris. 24: 1203, 1907.
• 16 — Hamperi H. : Chronischer richt hamolytischer ikterus mit ablagerung einers eigentümlichen pigmenters in the leber. Klin. Wehnschr. 35: 177, 1957.
• 17 — Hurts P. E. and Walters M. N. 1. : Black - liver jaundice (Dubin - Sprinz Syndrome) Med. J. Austr. 48 : 698, 1961.
• 18 — John G.G. and Knudson K.P.: Chronic idiopathic jaundice, two cases occuring in siblings with histochemical studies. Am, J, Med. 21: 138, 1956.
• 19 — Karpisek J., L Hofka J., Tion F. and Vaneuk R. : Vleklea zloutenka S neuncenym pigmentum V jaternich bunkoch.. Dubinur Syndrom. Casor, Lek. Cesk. 96: 611, 1957.
• 20 — Klajman A. and Efreti P. : Prolonged jaundice with unidentified | pigment in liver celis. Lancet 1: 538, 1955.
• 21 — Kletsky A. L. and Huck R. F. : Dubin - Johnson Syndrome. U.S. Ar. Med. Forces M.J.8: 582, 1957.
• 22 — Lewi Z. and Wietschner S. : Chronische Gelbsucht mit nicht identifiziertem pigmenti in den Leber - Zellen (Dubin - JohnsonschleKrankheit) Schweiz Med. Wchnschr, 87: 341, 1957.
• 23 — Lichtman 5S. S. : Diseases of the liver, Gallbladder and bile ducts 2 nd ed. Les and Febiger Philadelphia p: 136, 1949.
• 24 — Mandema E. De Fraiture W. H., Nieweg H. O. and Arends A. Familiai chronic idiopathic jaundice (Dubin -Sprinz Disease) with anote on bromsulphalein metabolism in this disease. Am. J. Med. 28: 42, 1960.
• 25 — Mordechai S., Seligsohn U., Ezzer J. B. : Effect of phenobarbitai onliver funtions in patients with Dubin - Johnson Syndrome. Gastroenterology, 67:303, 1974.
• 26 — Masuda M. T., Nonomura Y. and Singer K. : Familial non - hemolytic jaundice, Constitutional hepatic dysfunction with indirekt van den bergh reaktion. Arch. Int. Med. 67: 259, 1941.
• 27 — Rotor A.B. Monahan L.and Florentin A.: Familial non - hemolytic jaundice. with direkt van den bergh reaction. Acta Med. Philipp. 5: 37, 1948.
• 28 — Schillinger H. : Dubin - Johnson Syndrome. Med. Klin. 62: 164, 1967.
• 29 — Shami M., Seligsohn U. and Adam A.: The inheritance of Dubin - Johnson Syndrome. Israel J. Med. Sci. 9: 1427, 1973. i
• 30 — Sprinz H. and Nelson R. S. : Persistant non-hemolytic hyperbiliru- ' binemia associated with lipochromelike pigment in liver celis. Reported 4 cases. Ann. Int. Med. 41: 952, 1954.
• 31 — Tamaki H. T. and Carfagno S. C. : Chronic idiopathic jJaundice withunidentitied pigment in liver cells, Arch. Int. Med. 99; 294, 1957.
• 32 — Toshijumi K. R. : Dubin - Johnson Syndrome in a neonate. New Engi. J. Med. 292: 1028, 1975.
• 33 — Vaughan V.C. McKayR.J, Nelson W.E.: Nelson Textbook of Pediatrics W. B. Saunders Company Philadelphia London, Toronto, Çi 1975, p: 882.
• 34 — Wittekind D. and Messner E. : Chronischer idiopathischer ikterus bei einer pigment ablagerung unbekannter herkut in den leberzellen.Deutsch. Arch. Klin. Med. 203: 135, 1956.
• 35 — Woltt R. L, Pizette M, Richman, A,, Dreiling D. Jacobs W., Fernandez C. and Popper H. : Chronic idiopathic jaundice. Am, J. Med. 28: 32, 1960