[Problems typical for patients with hypopituitarism - a case presentation].
The authors present a 20-year follow-up of a typical case of hypopituitarism resulting from the Prop-1 gene mutation and diagnosed when the patient was 8 years of age based on growth deficit (-4 SD), delayed bone age (3.5 years), as well as proven deficit of GH and TSH, as well as LH and FSH detected in the peripubertal period. Following 8-year rhGH substitution treatment (0.75 IU (0.25) mg/kg/week and therapy with the levorotatory form of thyroxine and estrogens, the patient achieved an appropriate height (168 cm) and - after a 2-year preliminary period of treatment with a metabolic rhGH dose (0.5 IU/kg/week) - normalization of bone mineral density (z score -1.1 vs. -2.1 for lumbar spine) and improved quality of life. In both treatment periods the authors also observed normalization of the lipid profile without any detrimental effect on glucose and insulin concentration levels, what indicates a necessity for employing rhGH supplementation also after growth completion. The lack of progression in size of a Prop-1 mutation-dependent hypopituitary tumor (10x12x13 mm) demonstrated over the 20 years of follow-up emphasizes the importance of conservative management in such special cases.
Starzyk
Jerzy
J
Klinika Endokrynologii Dzieci i Młodziezy Katedry Pediatrii Polsko-Amerykańskiego Instytutu Pediatrii Collegium Medicum UJ w Krakowie. mistarzy@cyf-kr.edu.pl
Wójcik
Małgorzata
M
D015519:Bone Density / Q000187:drug effects; D002648:Child; D004967:Estrogens / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006130:Growth Disorders / Q000209:etiology* / Q000517:prevention & control*; D013006:Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D007018:Hypopituitarism / Q000188:drug therapy* / Q000209:etiology; D010911:Pituitary Neoplasms / Q000150:complications* / Q000175:diagnosis*; D011788:Quality of Life; D013974:Thyroxine / Q000627:therapeutic use
D004967:Estrogens; D013006:Growth Hormone; D013974:Thyroxine