title: Rapid height growth after liver transplantation in adulthood.
abstract: Glycogen storage disease Ib is a rare, inherited metabolic disorder caused by glucose-6-phosphatase translocase deficiency. Its main symptoms are hypoglycemia, hyperlipidemia, neutropenia, hepatomegaly, liver adenomas and short stature. The exact mechanism of short stature in this disease is unclear, the most feasible possibility is that it is caused by impairment of growth-hormone and insulin-like growth factor I axis. Here we report the case of a patient who showed typical symptoms of glycogen storage disease Ib since his infancy, his height being under 1 percentile since then. Later-developed hypothyroidism and hypogonadism have also contributed to his short stature. Hypothyroidism was treated but sexual steroid substitution was not started because of an increased risk of hepatic adenomas. Because he developed hepatic adenoma at the age of 23, he had to undergo orthotopic liver transplantation. At the time of the transplantation his height was 128cm. The transplantation was followed by rapid height growth; our patient's height reached 160.3cm 62months after transplantation. We observed that while his IGF-I level increased, his GH level remained unchanged. During the post-transplantation period we ensured adequate calcium and vitamin D supplementation, leaving hormonal substitution unchanged. According to our knowledge, this is the first report of a rapid height growth as big as 32cm, of an individual over the age of 20, not related to endocrine treatment but liver transplantation.
authors.affiliation: Semmelweis University, 1st Department of Internal Medicine, Korányi S. u. 2/A, H-1083 Budapest, Hungary. Electronic address: szilib@med.semmelweis-univ.hu.
authors.forename: Balázs
authors.initials: B
authors.lastname: Szili
authors.affiliation: Semmelweis University, Department of Transplantation Surgery, Baross u. 23-25, H-1082 Budapest, Hungary. Electronic address: gorogd@freemail.hu.
authors.forename: Dénes
authors.initials: D
authors.lastname: Görög
authors.affiliation: Semmelweis University, Department of Transplantation Surgery, Baross u. 23-25, H-1082 Budapest, Hungary. Electronic address: gerleizs@gmail.com.
authors.forename: Zsuzsanna
authors.initials: Z
authors.lastname: Gerlei
authors.affiliation: Semmelweis University, Department of Radiology and Oncotherapy, Üllői út 78/A, H-1083 Budapest, Hungary. Electronic address: gyoriga@gmail.com.
authors.forename: Gabriella
authors.initials: G
authors.lastname: Győri
authors.affiliation: Semmelweis University, 1st Department of Internal Medicine, Korányi S. u. 2/A, H-1083 Budapest, Hungary. Electronic address: lakatos.peter@med.semmelweis-univ.hu.
authors.forename: Péter
authors.initials: P
authors.lastname: Lakatos
authors.affiliation: Semmelweis University, 1st Department of Internal Medicine, Korányi S. u. 2/A, H-1083 Budapest, Hungary. Electronic address: takacs.istvan@med.semmelweis-univ.hu.
authors.forename: István
authors.initials: I
authors.lastname: Takács
mesh_terms: D000236:Adenoma / Q000150:complications / Q000601:surgery*; D001827:Body Height; D005953:Glycogen Storage Disease Type I / Q000150:complications / Q000378:metabolism*; D006130:Growth Disorders / Q000150:complications / Q000378:metabolism*; D006801:Humans; D007006:Hypogonadism / Q000150:complications / Q000378:metabolism; D007037:Hypothyroidism / Q000150:complications / Q000378:metabolism; D007334:Insulin-Like Growth Factor I / Q000378:metabolism*; D008113:Liver Neoplasms / Q000150:complications / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: C583557:IGF1 protein, human; D007334:Insulin-Like Growth Factor I
keywords: Glycogen storage disease type Ib; Height growth in adult; Liver transplantation; Short stature