Metabolic Effects of Growth Hormone Therapy in an Alström Syndrome Patient

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

Abstract

Aim: To investigate the metabolic effects of recombinant human growth hormone (rhGH) in an Alström syndrome patient with growth hormone deficiency. Methods: A 15-year-old Alström syndrome boy with growth hormone deficiency received rhGH therapy for 1 year. Biochemical parameters, including hepatic enzyme levels, lipid profiles, and insulin sensitivity, were measured. Body composition analysis and computed tomography scans of the liver were performed. Results: After 1 year of rhGH treatment, body fat mass, fat infiltration in the liver, and serum lipid profiles had all decreased. Insulin sensitivity and acanthosis nigricans improved. Conclusion: rhGH therapy might have beneficial effects on body composition, liver fat content, lipid profiles, and insulin resistance in Alström syndrome patients, with improvement of the glucose homeostasis.

Related collections

Most cited references 13

Record: found
Abstract: found
Article: not found

Noninvasive in vivo quantitative assessment of fat content in human liver.

C Ricci, R. Longo, E Gioulis … (1997)

Since the introduction of ultrasonography, liver steatosis has become an increasingly frequent diagnosis. Both ultrasonography (US) and computerized tomography (CT) provide qualitative rather than quantitative assessment of fatty infiltration. The objective of this study was to develop a noninvasive method for the quantification of the hepatic fat content in vivo. A test object containing solutions with CT scan density (CTD) similar to normal liver ("liver-equivalent") or "fat-equivalent material" in variable proportions was prepared to measure patients with variable degrees of steatosis in vivo. A linear correlation (r=0.99, p<0.001) linked CTD and the increasing percentage of fat-equivalent material. A CTD calibration curve was derived as a reference for the in vivo determinations. In 29 consecutive patients with steatosis diagnosed by histology, CTD was linearly correlated (r=0.83, p<0.001) with the hepatic fat content (HFC) expressed as percent of the whole liver, obtained by a computerized histomorphometric analysis. Based on the calibration curve obtained in 29 subjects who underwent liver biopsy, 38 additional consecutive steatotic patients were examined and the degree of hepatic fat content was calculated. The HFC was linearly correlated (r=-0.86, p<0.001) with the liver-to-spleen ratio. We conclude that the use of test objects allows an accurate and reproducible noninvasive quantitative assessment of hepatic fat infiltration in humans. This technique may prove useful in the evaluation of the natural course and treatment of hepatic steatosis as well as in the assessment of donor livers prior to transplantation.

0 comments Cited 43 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Genealogy, natural history, and phenotype of Alström syndrome in a large Acadian kindred and three additional families.

M D Ludman, Patsy M. Nishina, Barry S Shea … (1997)

We describe a large Acadian kindred including 8 Alstrom Syndrome (AS) patients, with an age range of 4 to 26 at the time of clinical assessment. The affected subjects come from 5 nuclear families within this kindred. The phenotype includes early childhood retinopathy, progressive sensorineural hearing loss, truncal obesity, and acanthosis nigricans. In addition, hyperinsulinemia and hypertriglyceridemia with normal cholesterol levels were observed in most affected individuals tested. Non-insulin dependent diabetes mellitus and growth retardation appear to be age-related manifestations that occur post-adolescence. Younger affected children are not overtly hyperglycemic and are normal or above average height for age. Although the AS patients in kindred 1 presumably carry the same mutation, many manifestations of the disease are variable. For example, of the 8 children in the Acadian kindred, 4 have scoliosis, 2 have had infantile cardiomyopathy, 2 are hypothyroid, 1 has had hepatic dysfunction and is hypertensive, and 4 have developed asthma. Seven subjects described in this kindred exhibit developmental delay. One additional manifestation not described widely in the literature, advanced bone age, was observed in all subjects tested. The clinical data from this large Acadian kindred, together with information obtained from 4 additional AS patients in 3 unrelated kindreds, confirm and extend clinical observations previously described. In addition, the Acadian kindred with multiple affected individuals, probably arising from a common founder, should allow for identification of the chromosomal localization of a gene causing AS.