Long-term survival with growth hormone replacement after liver transplantation of pediatric nonalcoholic steatohepatitis complicating acquired hypopituitarism.
Nonalcoholic steatohepatitis (NASH) is the most severe form of nonalcoholic fatty liver disease (NAFLD). In adult patients, liver transplantation (LT) is the treatment of choice for end-stage liver disease secondary to NASH. However, little information is available regarding outcomes of LT in pediatric patients with NASH. We describe here a pediatric patient with NASH associated with hypopituitarism who underwent living donor liver transplantation (LDLT). An 11-year-old boy was diagnosed with a pituitary tumor, which was removed by trans-interhemispheric approach following bifrontal craniotomy. Histopathological examination revealed a mature teratoma. Eighteen months later, magnetic resonance imaging showed recurrence of the pituitary tumor, which was found to be a germinoma. He underwent 3 months of chemoradiotherapy, with a complete response. He gradually became obese, with elevated transaminase levels. At age 15 years, he developed fatigue and dyspnea and was found to have liver cirrhosis secondary to NASH with severe hepatopulmonary syndrome. He underwent LDLT using a right liver graft from his mother. Twelve months later, abdominal computed tomography showed recurrence of NAFLD. Five years after the LDLT, transaminases were slightly elevated. Growth hormone replacement therapy was started, reducing transaminase levels to their normal ranges. Ten years after LDLT, fatty liver remains stable, although his body mass index has not been reduced. Growth hormone replacement therapy may be effective in graft maintenance. This is the first case report of a patient with maintained stable liver function 10 years after LDLT for pediatric NASH.
Division of Transplantation, Reconstruction, and Endoscopic Surgery, Tohoku University Hospital.
Atsushi
A
Fujio
Naoki
N
Kawagishi
Taketora
T
Echizenya
Kazuaki
K
Tokodai
Chikashi
C
Nakanishi
Shigehito
S
Miyagi
Kazushige
K
Sato
Keisei
K
Fujimori
Noriaki
N
Ohuchi
D000293:Adolescent; D001706:Biopsy; D002648:Child; D005500:Follow-Up Studies; D013006:Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D007018:Hypopituitarism / Q000150:complications* / Q000188:drug therapy*; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000150:complications / Q000473:pathology; D016031:Liver Transplantation; D008279:Magnetic Resonance Imaging; D008297:Male; D065626:Non-alcoholic Fatty Liver Disease / Q000150:complications* / Q000628:therapy*; D010911:Pituitary Neoplasms / Q000150:complications / Q000473:pathology; D011182:Postoperative Care; D016019:Survival Analysis
D013006:Growth Hormone