Long-term evolution of endocrine disorders and effect of GH therapy in 35 patients with pituitary stalk interruption syndrome.
We report long-term evolution of endocrine functions and the results of GH treatment in 35 patients (26 male and 9 female) with pituitary stalk interruption. At diagnosis, mean chronological age was 4.8 +/- 2.7 years, mean SDS for height -3.1 +/- 0.8 with a bone age retardation of 2.3 +/- 1.3 years and a mean SDS for growth velocity of -0.5 +/- 1.1; 80% presented complete GH deficiency (GHD) and 20% partial GHD; thyroid deficiency was present in 47.1% of children with complete GHD but absent in all partial GHD. Diagnosis was made during the first months of life in only 2 patients while 23% presented with severe neonatal distress; neonatal signs were only observed in the group with pituitary height below 2 mm (45.7% of patients). GHD was isolated in 40.6% of patients below 10 years while multiple hormone deficiencies was consistent at completion of growth in all patients. Height gain was significantly higher in patients who started GH treatment before 4 years (p = 0.002). GH treatment is very effective: in 13 patients, final height was -0.4 +/- 1.0, total height gain 3.2 +/- 1.2 and distance to target height -0.3 +/- 1.6 SDS.
Tauber
Maïthé
M
Division of Endocrinology, Genetics, Gynecology and Bone Diseases, Hôpital des Enfants, Toulouse, France. crc@chu-toulouse.fr
Chevrel
Jean
J
Diene
Gwenaelle
G
Moulin
Pierre
P
Jouret
Béatrice
B
Oliver
Isabelle
I
Pienkowski
Catherine
C
Sevely
Annick
A
D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000172:deficiency; D002648:Child; D002675:Child, Preschool; D004700:Endocrine System Diseases / Q000209:etiology*; D005260:Female; D006065:Gonadotropins, Pituitary / Q000172:deficiency; D006128:Growth; D019382:Human Growth Hormone / Q000172:deficiency* / Q000627:therapeutic use*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D010900:Pituitary Diseases / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D010902:Pituitary Gland / Q000002:abnormalities*; D013972:Thyrotropin / Q000172:deficiency; D016896:Treatment Outcome
D006065:Gonadotropins, Pituitary; D019382:Human Growth Hormone; D000324:Adrenocorticotropic Hormone; D013972:Thyrotropin