Multiple pituitary hormone deficiency, empty sella and ectopic neurohypophysis in Turner syndrome

2015 ◽  
Vol 52 (9) ◽  
pp. 803-804
Author(s):  
Deep Dutta ◽  
Chitra Selvan ◽  
Satinath Mukhopadhyay
2018 ◽  
Vol 31 (5) ◽  
pp. 533-537 ◽  
Author(s):  
Hongbo Yang ◽  
Linjie Wang ◽  
Xiaonan Qiu ◽  
Kemin Yan ◽  
Fengying Gong ◽  
...  

Abstract Background: Recombinant human growth hormone (rhGH) replacement therapy is usually stopped after linear growth completion in patients with growth hormone deficiency. In patients with multiple pituitary hormone deficiency (MPHD), the long-term effects of discontinuation of rhGH replacement are unknown. Methods: In this study, the anthropometric and metabolic parameters of 24 male patients with adult growth hormone deficiency (AGHD) due to MPHD in childhood after cessation of rhGH therapy for a mean of 7.1 years were measured and compared with 35 age-matched controls. Body composition was evaluated by bioelectrical impedance analysis (BIA). Results: In the AGHD group, body mass index (BMI) was significantly increased and 29.2% had obesity. The AGHD group had a 17.7 cm increase in waist circumference (WC). The fat free mass (FFM) was significantly lower in the AGHD group. Both the fat mass (FM) and percentage of fat mass (FM%) were significantly increased in the AGHD group. Both the systolic blood pressure (BP) and diastolic pressure were significantly lower in AGHD group. The lipid profile was generally similar in both groups, except for a decrease of high density lipoprotein-cholesterol (HDL-C) in the AGHD group. There was significant hyperuricemia in the AGHD group. Conclusions: Cessation of rhGH leads to a significant increase of FM in early adulthood in male patients with childhood-onset MPHD (CO-MPHD).


1996 ◽  
Vol 5 (Supple8) ◽  
pp. 127-128 ◽  
Author(s):  
Ryuuzou Takaya ◽  
Masakuni Tokuda ◽  
Shuichi Shimakawa ◽  
Shuhei Suzuki ◽  
Kazutaka Konishi ◽  
...  

2019 ◽  
Vol 12 (4) ◽  
pp. e229016 ◽  
Author(s):  
Nese Akcan ◽  
Firdevs Bas ◽  
Sukran Poyrazoglu ◽  
Ruveyde Bundak

Joubert syndrome (JS) and JS-related disorders are a group of developmental delay, multiple congenital anomalies and complex midbrain–hindbrain malformations. A few cases of JS with multiple pituitary hormone deficiency (MPHD) have been reported in literature. Here, we presented an unusual presentation of JS in a newborn with MPHD. This case is intended to draw attention to the rare association of JS and MDPH by increasing the awareness of this syndrome.


Author(s):  
R. Stanhope ◽  
F. De Luca ◽  
H.A. Delemarre-Van de Waal ◽  
A. Liotta ◽  
E. Noijavaara ◽  
...  

AbstractThe overview in this paper focuses on ways of achieving optimal auxological results in puberty, principally in idiopathic and congenital multiple pituitary hormone deficiency (MPHD), suggested by the co-authors. We agreed that diagnosing gonadotrophin insufficiency/deficiency is difficult in young children and should be repeated in late prepuberty, but a firm diagnosis of MPHD helps avoid endocrine re-testing at the end of growth. The hypothalamic-pituitary axis must be reassessed periodically in evolving endocrinopathies, though current practice varies widely. Optimum age to induce puberty is 11-12 years in girls and 13-14 boys, and sex steroids are the preferred agents. Short-course testosterone to increase micropenis size is advantageous, but inducing early testicular maturation is not known to improve later fertility. There is also little evidence for increasing the dose of GH during puberty, though therapy should continue to final height, and possibly until peak bone mass is achieved. Delaying puberty is an option in septo-optic dysplasia, and minimising the dose of hydrocortisone is crucial in treating ACTH/ cortisol insufficiency. Many unresolved questions remain in this difficult area.


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