Neonatal and infantile Dubin-Johnson syndrome PDF20160214-9750-KE1KHL

Title	Neonatal and infantile Dubin-Johnson syndrome
Author	Jacob Amir
Pages	1
File Size	20.6 KB
File Type	PDF20160214-9750-KE1KHL
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Pediatr Radiol (1998) 28: 900 Ó Springer-Verlag 1998 L E TTE R S TO TH E E D ITO R Yishai Haimi-Cohen tool to the investigational armamentarium Toshiaki Shimizu Jacob Amir of neonatal and infantile cholestasis. Find- ings of marked liver attenuation on ab- Paul Merlob dominal CT, together with more ...

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Yishai Haimi-Cohen Jacob Amir Paul Merlob Neonatal and infantile Dubin-Johnson syndrome Received: 3 November 1997 Accepted: 17 April 1998 Sir, We read with great interest the article by Shimizu et al. [1] on Dubin-Johnson syn- drome (DJS). We recently described two non-identical twins with neonatal cholesta- sis who were later diagnosed as having DJS [2]. We would like to emphasize three im- portant aspects of this syndrome in the neonatal and infantile age group which were refected in the case described in your journal as well: 1. DJS may present as severe cholestasis in neonates and in infants, and despite its rarity [3±6] at this early age, it should be considered in the investigation of the cholestasis. 2. Delayed or complete absence of passage of contrast material may be noted on he- patobiliary scintigraphy in cases of DJS with severe cholestasis. This is apparent- ly a result of biliary hepatocyte conges- tion. 3. Measurement of urinary coproporphyrin I and II is the mainstay of the clinical di- agnosis of DJS. It should be included in every investigation of neonatal cholesta- sis for early detection of the syndrome, to prevent the needles performance of such diagnostic procedures as liver biopsy and exploratory laparotomy. We believe the fnding of high computer- ized tomography (CT) attenuation in the liver in infantile DJS may be of great im- portance because it adds a sensitive new tool to the investigational armamentarium of neonatal and infantile cholestasis. Find- ings of marked liver attenuation on ab- dominal CT, together with more than 80 % of the total urinary coproporphyrins being accounted for by coproporphyrin I, should clearly confrm the diagnosis of infantile DJS without additional invasive means. References 1. Shimizu T, Tawa T, Maruyama T, Oguchi S, Yamashiro Y, Yabuta K (1997) A case of infantile Dubin-Johnson syndrome with high CT attenuation in the liver. Pe- diatr Radiol 27: 345±347 2. Haimi-Cohen Y, Merlob P, Marcus Eid- lits T, Amir J (1998) Dubin-Johnson syn- drome as a cause of neonatal jaundice: the importance of coproporphyrins in- vestigation. Clin Pediatr 37: 511±514 3. Kondo T, Yagi R, Kuchiba K (1975) Du- bin-Johnson syndrome in a neonate. N Engl J Med 292: 1028±1029 4. Nakate F, Oyangi K, Fujiwara M, Soga- wa H, Minami R, Horino K (1979) Du- bin-Johnson syndrome in a neonate. Eur J Pediatr 132: 299±301 5. Shieh CC, Chang MH, Chen CL (1990) Dubin-Johnson syndrome presenting with neonatal cholestasis. Arch Dis Child 65: 898±899 6. Kimura A, Ushijima K, Kage M, Mahara R, Tohma M, Inokuchi T (1991) Neona- tal Dubin-Johnson syndrome with severe cholestasis: efective phenobarbital ther- apy. Acta Pediatr Scand 80: 381±385 Y. Haimi-Cohen ( ) ) × J. Amir Department of Day Care, Schneider Children's Medical Center of Israel, Beilinson Campus, 14 Kaplan Street, Petah Tikva 49202, Israel P. Merlob Department of Neonatology, Beilinson Medical Center, Petah Tikva, Israel Toshiaki Shimizu Reply Sir, We thank Haimi-Cohen et al. for the addi- tional comment which emphasized the im- portance of abdominal CT as well as an increase in urinary coproporphyrin I for the diagnosis of infantile Dubin-Johnson syn- drome (DJS). In order to confrm the im- portance of abdominal CT, as we described in our discussion, it may be very important to examine reports of abdominal CT fnd- ings in other infants with DJS and to ob- serve the follow-up CT fndings in our patient. Because CT attenuation of the li- ver in our patient is still high without any clinical symptoms at 3 years of age, the fnding of abdominal CT in other patients with infantile DJS may confrm the neces- sity of the examination, though we could not fnd the result of abdominal CT in their patients. T. Shimizu Department of Pediatrics, Juntendo University, 3-1-3, Hongo, Bunkyo-ku, Tokyo 113, Japan Pediatr Radiol (1998) 28: 900 Ó Springer-Verlag 1998 LETTERS TO THE EDITOR...