Yeşim ÖZTÜRK, Barış ERDUR, Hatice ARSLAN, Özhan ÖZDOĞAN, Handan ÇAKMAKÇI, Alper SOYLU

Konjuge hiperbilirubinemi ve intraabdominal lenfadenopati: Rotor sendromu, toksoplazmozis birlikteliği: Olgu sunumu

Rotor Sendromu kronik konjuge bilirubin yüksekliği ile seyreden otozomal resesif geçişli bir bozukluktur. Kendine özgü bir semptom veya fizik muayene bulgusu olmayan bu sendromda karaciğer fonksiyon testleri de normal bulunur. Eşlik eden başka hastalıkların varlığında, ayırıcı tanıda zorluklar yaşanmaktadır. Burada; konjuge bilirubin yüksekliği intraabdominal lenfadenopatilerin safra yollarına basısı ön tanısıyla kliniğimize yollanan, tanı zorluğu yaşanan ve sonuçta Rotor sendromu ile birlikte Toksoplazmozis tanısı almış olan bir vaka sunulmuştur.

Conjugated hyperbilirubinemia and intraabdominal lymphadenopathy: Coexistance rotor syndrome and toxoplasmosis: Case report

Rotor syndrome is an autosomal recessive disorder presenting with chronic elevation of the conjugated serum bilirubin fraction. There are no spesific symptoms and physical examination findings of this syndrome and liver function tests are normal. Differential diagnosis is difficult in the existence of concomitant diseases. Here; we report a child with conjugated hyperbilirubinemia though to result from biliary tract compression by intraabdominal lymphadenopathies. The diagnosis was established as Rotor Syndrome and Toxoplasma lymphadenitis eventually.

PDF

___

1. Wolkoff AW. Inheritable disorders manifested by conjugated hyperbilirubinemia. Semin Liver Dis 1983;3: 65-72.
2. Harb R, Thomas DW. Conjugated hyperbilirubinemia: screening and treatment in older infants and children. Pediatr Rev 2007;28:83-91.
3. Shimizu Y, Naruto H, Ida S, Kohakura M. Urinary coproporphyrin isomers in Rotor’s syndrome: a study in eight families. Hepatology 1981;1:173-178.
4. Fretzayas AM, Garoufi AI, Moutsouris CX, Karpathios TE. Cholescinthigraphy in the diagnosis of Rotor syndrome. J Nucl Med 1994;35:1048-1050.
5. Bar-Meir S, Baron J, Seligson U, Gottesfeld F, Levy R, Gilat T. 99mTc-HIDA cholescintigraphy in Dubin-Johnson and Rotor syndromes. Radiology 1982;142:743-746.
6. Pashankar D, Schreiber RA. Jaundice in older children and adolescents. Pediatr Rev 2001;22:219-226.
7. Durlach RA, Kaufer F, Carral L, Hirt J. Toxoplasmic lymphadenitis-clinical and serologic profile. Clin Microbiol Infect 2003;9:625-631.
8. Evans R, Ho-Yen DO. Evidence-based diagnosis of toxoplasma infection. Eur J Clin Microbiol Infect Dis 2000;19:829-833.
9. Montoya JG, Liesenfeld O. Toxoplasmosis. Lancet 2004; 363:1965-1976.
10. Contini C. Clinical and diagnostic management of toxoplasmosis in the immuncompromised patient. Parassitologia 2008;50:45-50.