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  • 1 Semmelweis Egyetem, Általános Orvostudományi Kar I. Gyermekgyógyászati Klinika Budapest Bókay u. 53. 1083
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Turner-szindrómában a cardiovascularis és renalis malformatiók gyakori előfordulása jól ismert. A ritkábban tapasztalt gastrointestinalis vérzés jellemző oka e betegségben az irodalmi adatok szerint intestinalis teleangiectasia, gyulladásos bélbetegség és portalis hypertensio. Hároméves, Turner-szindróma miatt gondozott gyermek súlyos, transzfúziót igénylő gastrointestinalis vérzéssel járó esetét ismertetik a szerzők. A haematochesia hátterében a képalkotó vizsgálat praehepaticus portalis hypertensiót igazolt, amelynek oka a vena portae veleszületett hiánya volt. A máj szövettani vizsgálata az intrahepaticus artériák és vénák anomáliáját írta le. Az eset kapcsán a szerzők a Turner-szindrómához társuló májelváltozásokat, valamint a vena portae elzáródásának gyermekkorban előforduló okait elemzik.

  • Lisser, H., Curtis, L. E., Escamilla, R. F. és mtsai: The syndrome of congenitally aplastic ovaries with sexual infantilism, high urinary gonadotropins, short stature, and other congenital abnormalities: Tabular presentation of 25 previously unpublished cases. J. Clin. Endocrinol., 1947, 7 , 665–687.

    Escamilla R. F. , 'The syndrome of congenitally aplastic ovaries with sexual infantilism, high urinary gonadotropins, short stature, and other congenital abnormalities: Tabular presentation of 25 previously unpublished cases ' (1947 ) 7 J. Clin. Endocrinol. : 665 -687.

    • Search Google Scholar
  • Schultz, L. S., Assimacopoulos, C. A., Lillehei, R. C.: Turner’s syndrome with associated gastrointestinal hemorrhage: a case report. Surgery, 1970, 68 , 485–488.

    Lillehei R. C. , 'Turner’s syndrome with associated gastrointestinal hemorrhage: a case report ' (1970 ) 68 Surgery : 485 -488.

    • Search Google Scholar
  • Eroglu, Y., Emerick, K. M., Chou, P. M. és mtsai: Gastrointestinal bleeding in Turner’s syndrome: A case report and literature review. J. Pediatr. Gastroenterol. Nutr., 2002, 35 , 84–87.

    Chou P. M. , 'Gastrointestinal bleeding in Turner’s syndrome: A case report and literature review ' (2002 ) 35 J. Pediatr. Gastroenterol. Nutr. : 84 -87.

    • Search Google Scholar
  • Elsheikh, M., Dunger, D. B., Conway, G. S. és mtsa: Turner’s syndrome in adulthood. Endocr. Rev., 2002, 23 , 120–140.

    Conway G. S. , 'Turner’s syndrome in adulthood ' (2002 ) 23 Endocr. Rev. : 120 -140.

  • Sokol, L., Stueben, E. T., Jaikishen, J. P. és mtsai: Turner syndrome associates with acquired von Willebrand disease, primary biliary chirrhosis and inflamatory bowel disease. Am. J. Hematol., 2002, 70 , 257–259.

    Jaikishen J. P. , 'Turner syndrome associates with acquired von Willebrand disease, primary biliary chirrhosis and inflamatory bowel disease ' (2002 ) 70 Am. J. Hematol. : 257 -259.

    • Search Google Scholar
  • Vermeire, S., Satsangi, J., Peeters, M. és mtsai: Evidence for inflammatory bowel disease of a susceptibility locus on the X chromosome. Gastroenterology, 2001, 120 , 834–840.

    Peeters M. , 'Evidence for inflammatory bowel disease of a susceptibility locus on the X chromosome ' (2001 ) 120 Gastroenterology : 834 -840.

    • Search Google Scholar
  • Jolley, C., Langham, M. R., Dillard, R. és mtsai: Intraoperative endoscopy in a child with Turner’s syndrome and gastrointestinal haemorrhage: a case report. J. Pediatr. Surg., 2001, 36 , 951–952.

    Dillard R. , 'Intraoperative endoscopy in a child with Turner’s syndrome and gastrointestinal haemorrhage: a case report ' (2001 ) 36 J. Pediatr. Surg. : 951 -952.

    • Search Google Scholar
  • Bondy, C. A.: Turner’s syndrome and X chromosome-based differences in disease susceptibility. Gender Med., 2006, 3 , 18–30.

    Bondy C. A. , 'Turner’s syndrome and X chromosome-based differences in disease susceptibility ' (2006 ) 3 Gender Med. : 18 -30.

    • Search Google Scholar
  • Bondy, C. A.: Care of girls and women with Turner syndrome: A Guideline of the Turner Syndrome Study Group. J. Clin. Endocrin. Metab., 2007, 92 , 10–25.

    Bondy C. A. , 'Care of girls and women with Turner syndrome: A Guideline of the Turner Syndrome Study Group ' (2007 ) 92 J. Clin. Endocrin. Metab. : 10 -25.

    • Search Google Scholar
  • Larizza, D., Locatelli, M., Vitali, L. és mtsai: Serum liver enzimes in Turner syndrome. Eur. J. Pediatr., 2000, 159 , 143–148.

    Vitali L. , 'Serum liver enzimes in Turner syndrome ' (2000 ) 159 Eur. J. Pediatr. : 143 -148.

    • Search Google Scholar
  • Gravholt, C. H., Naeraa, R. W., Fisker, S. és mtsai: Body composition and physical fitness are major determinants of the growth hormone-insulin-like growth factor axis aberrations in adult Turner’s syndrome, with important modulations by treatment with 17β-estradiol. J. Clin. Endocrinol. Metab., 1997, 82 , 2570–2577.

    Fisker S. , 'Body composition and physical fitness are major determinants of the growth hormone-insulin-like growth factor axis aberrations in adult Turner’s syndrome, with important modulations by treatment with 17β-estradiol ' (1997 ) 82 J. Clin. Endocrinol. Metab. : 2570 -2577.

    • Search Google Scholar
  • Elsheikh, M., Hudson, H. J. F., Wass, J. A. H. és mtsai: Hormone replacement therapy may improve hepatic function in women with Turner’s syndrome. Clin. Endocrinol., 2001, 55 , 227–231.

    Wass J. A. H. , 'Hormone replacement therapy may improve hepatic function in women with Turner’s syndrome ' (2001 ) 55 Clin. Endocrinol. : 227 -231.

    • Search Google Scholar
  • Wardi, J., Knobel, B., Shahmurov, M. és mtsai: Chronic cholestasis associated with Turner’s syndrome: 12 years of clinical and histopathological follow-up. Digestion, 2003, 67 , 96–99.

    Shahmurov M. , 'Chronic cholestasis associated with Turner’s syndrome: 12 years of clinical and histopathological follow-up ' (2003 ) 67 Digestion : 96 -99.

    • Search Google Scholar
  • Garavelli, L., Donadio, A., Banchini, G. és mtsai: Liver abnormalities and portal hypertension in Ullrich–Turner syndrome. Am. J. Med. Gen., 1998, 80 , 180–182.

    Banchini G. , 'Liver abnormalities and portal hypertension in Ullrich–Turner syndrome ' (1998 ) 80 Am. J. Med. Gen. : 180 -182.

    • Search Google Scholar
  • Idilman, R., Maria, N., Colantoni, A. és mtsai: Cirrhosis in Turner’s syndrome: case report and literature review. Eur. J. Gastroenterol. Hepatol., 2000, 12 , 707–709.

    Colantoni A. , 'Cirrhosis in Turner’s syndrome: case report and literature review ' (2000 ) 12 Eur. J. Gastroenterol. Hepatol. : 707 -709.

    • Search Google Scholar
  • Gravholt, C. H., Juul, S., Naeraa, R. W. és mtsai: Morbidity in Turner syndrome. J. Clin. Epidemiol., 1998, 51 , 147–158.

    Naeraa R. W. , 'Morbidity in Turner syndrome ' (1998 ) 51 J. Clin. Epidemiol. : 147 -158.

  • Roulot, D., Begott, C., Chazouilleres, O. és mtsai: Vascular involvement of the liver in Turner’s syndrome. Hepatology, 2004, 39 , 239–247.

    Chazouilleres O. , 'Vascular involvement of the liver in Turner’s syndrome ' (2004 ) 39 Hepatology : 239 -247.

    • Search Google Scholar
  • Kinjo, T., Auki, H., Sunagawa, H. és mtsai: Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital choledochal cyst: a case report. J. Pediatr. Surg., 2001, 36 , 622–625.

    Sunagawa H. , 'Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital choledochal cyst: a case report ' (2001 ) 36 J. Pediatr. Surg. : 622 -625.

    • Search Google Scholar
  • Prandstaller, D., Mazzanti, L., Picchio, F. M. és mtsai: Turner’s syndrome: Cardiologic profile according to the different chromosomal patterns and long-term clinical follow-up of 136 nonpreselected patients. Pediatr. Cardiol., 1999, 20 , 108–112.

    Picchio F. M. , 'Turner’s syndrome: Cardiologic profile according to the different chromosomal patterns and long-term clinical follow-up of 136 nonpreselected patients ' (1999 ) 20 Pediatr. Cardiol. : 108 -112.

    • Search Google Scholar
  • Arató A., Szőnyi J.: Gyermekgasztroenterológia. Medicina Kiadó, Budapest. 2003, 471.

    Szőnyi J. , '', in Gyermekgasztroenterológia , (2003 ) -.

  • Sarin, S. K., Agarwal, S. R.: Extrahepatic Portal Vein Obstruction. Semin. Liv. Dis., 2002, 22 , 43–58.

    Agarwal S. R. , 'Extrahepatic Portal Vein Obstruction ' (2002 ) 22 Semin. Liv. Dis. : 43 -58.

  • Schettino, G. C. M., Fagundes, E. D. T., Roquete, M. L. V.: Portal vein thrombosis in children and adolescents. J. Pediatr. (Rio. J.), 2006, 82 , 171–178.

    Roquete M. L. V. , 'Portal vein thrombosis in children and adolescents ' (2006 ) 82 J. Pediatr. (Rio. J.) : 171 -178.

    • Search Google Scholar
  • Noe, J. A., Pittman, H. C., Burton, E. M. és mtsai: Congenital absence of the portal vein in a child with Turner syndrome. Pediatr. Radiol., 2006, 36 , 566–568.

    Burton E. M. , 'Congenital absence of the portal vein in a child with Turner syndrome ' (2006 ) 36 Pediatr. Radiol. : 566 -568.

    • Search Google Scholar
  • Wang, J. T., Zhao, H. Y., Liu, Y. L.: Portal vein thrombosis. Hepatobiliary Pancreat. Dis. Int., 2005, 4 , 515–518.

    Liu Y. L. , 'Portal vein thrombosis ' (2005 ) 4 Hepatobiliary Pancreat. Dis. Int. : 515 -518.

  • Denninger, M. H., Chait, Y., Casadevall, N. és mtsai: Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors. Hepatology, 2000, 31 , 587–591.

    Casadevall N. , 'Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors ' (2000 ) 31 Hepatology : 587 -591.

    • Search Google Scholar
  • Valla, D. H., Condat, B.: Portal vein thrombosis in adults: pathophisiology, pathogenesis and management. J. Hepatol., 2000, 32 , 865–871.

    Condat B. , 'Portal vein thrombosis in adults: pathophisiology, pathogenesis and management ' (2000 ) 32 J. Hepatol. : 865 -871.

    • Search Google Scholar
  • Guimaraes, H., Castello, L., Guimaraes, J. és mtsai: Does umbilical vein catheterization to exchange transfusion lead to portal vein thrombosis? Eur. J. Pediatr., 1998, 157 , 461–463.

    Guimaraes J. , 'Does umbilical vein catheterization to exchange transfusion lead to portal vein thrombosis? ' (1998 ) 157 Eur. J. Pediatr. : 461 -463.

    • Search Google Scholar
  • Pinto, R. B., Silveira, T. R., Bandinelli, E. és mtsai: Portal vein thrombosis in children and adolescents: The low prevalence of hereditary thrombophilic disorders. J. Pediatr. Surg., 2004, 39 , 1356–1361.

    Bandinelli E. , 'Portal vein thrombosis in children and adolescents: The low prevalence of hereditary thrombophilic disorders ' (2004 ) 39 J. Pediatr. Surg. : 1356 -1361.

    • Search Google Scholar
  • Zilz, C. K., Brenner, J. K., Elnecave, R. H.: Portal vein thrombosis and high factor VIII in Turner syndrome. Horm. Res., 2006, 66 , 89–93.

    Elnecave R. H. , 'Portal vein thrombosis and high factor VIII in Turner syndrome ' (2006 ) 66 Horm. Res. : 89 -93.

    • Search Google Scholar
  • Kanamori, Y., Hashizume, K., Kitano, Y. és mtsai: Congenital extrahepatic portocaval shunt (Abernethy type II), huge liver mass, and patent ductus arteriosus – a case report of its rare clinical presentation in a young girl. J. Pediatr. Surg., 2003, 38 , E15.

    Kitano Y. , 'Congenital extrahepatic portocaval shunt (Abernethy type II), huge liver mass, and patent ductus arteriosus – a case report of its rare clinical presentation in a young girl ' (2003 ) 38 J. Pediatr. Surg. : E15 -.

    • Search Google Scholar
  • Gallego, C., Miralles, M., Marin, C.: Congenital hepatic shunts. Radiographics, 2004, 24 , 755–772.

    Marin C. , 'Congenital hepatic shunts ' (2004 ) 24 Radiographics : 755 -772.

  • Gallego, C., Velasco, M., Marcuello, P. és mtsai: Congenital and acquired anomalies of the portal venous system. Radiographics, 2002, 22 , 141–159.

    Marcuello P. , 'Congenital and acquired anomalies of the portal venous system ' (2002 ) 22 Radiographics : 141 -159.

    • Search Google Scholar
  • Takagaki, K., Kodaira, M., Kuriyama, S. és mtsai: Congenital absence of the portal vein complication hepatic tumors. Internal Med., 2004, 43 , 194–198.

    Kuriyama S. , 'Congenital absence of the portal vein complication hepatic tumors ' (2004 ) 43 Internal Med. : 194 -198.

    • Search Google Scholar
  • Nakasaki, H., Tanaka, Y., Ohta, M. és mtsai: Congenital absence of the portal vein. Ann. Surg., 1989, 210 , 190–193.

    Ohta M. , 'Congenital absence of the portal vein ' (1989 ) 210 Ann. Surg. : 190 -193.

  • Appela, H., Loddenkemperc, C., Schirmacherd, P. és mtsai: Congenital absence of the portal vein with splenomegaly and hypersplenism in a young woman. Digestion, 2003, 67 , 105–110.

    Schirmacherd P. , 'Congenital absence of the portal vein with splenomegaly and hypersplenism in a young woman ' (2003 ) 67 Digestion : 105 -110.

    • Search Google Scholar
  • Tanaka, Y., Takayanagi, M., Shiratori, Y. és mtsai: Congenital absence of portal vein with multiple hyperplastic nodular lesions in the liver. J. Gastroenterol., 2003, 38 , 288–294.

    Shiratori Y. , 'Congenital absence of portal vein with multiple hyperplastic nodular lesions in the liver ' (2003 ) 38 J. Gastroenterol. : 288 -294.

    • Search Google Scholar
  • Shinkai, M., Ohhama, Y., Nishi, T. és mtsai: Congenital absence of the portal vein and role of liver transplantation in children. J. Pediatr. Surg., 2001, 36 , 1026–1031.

    Nishi T. , 'Congenital absence of the portal vein and role of liver transplantation in children ' (2001 ) 36 J. Pediatr. Surg. : 1026 -1031.

    • Search Google Scholar
  • O’Hare, J. P., Hamilton, M., Davies, J. D. és mtsai: Oestrogen deficiency and bleeding from large bowel teleangiectasia in Turner’s syndrome. J. R. Soc. Med., 1986, 79 , 746–747.

    Davies J. D. , 'Oestrogen deficiency and bleeding from large bowel teleangiectasia in Turner’s syndrome ' (1986 ) 79 J. R. Soc. Med. : 746 -747.

    • Search Google Scholar
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