scholarly journals Joubert syndrome with multiple pituitary hormone deficiency

2019 ◽  
Vol 12 (4) ◽  
pp. e229016 ◽  
Author(s):  
Nese Akcan ◽  
Firdevs Bas ◽  
Sukran Poyrazoglu ◽  
Ruveyde Bundak
Keyword(s):  
Developmental Delay ◽  
Congenital Anomalies ◽  
Joubert Syndrome ◽  
Unusual Presentation ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Multiple Congenital Anomalies ◽  
Multiple Pituitary Hormone Deficiency ◽  
Pituitary Hormone Deficiency ◽  
Rare Association

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.

scholarly journals Multiple pituitary hormone deficiency: beware of combined hormones deficiency

2013 ◽  
Vol 2013 (S1) ◽  
Author(s):  
Nur Rochmah ◽  
Muhammad Faizi ◽  
Achmad Yuniari ◽  
Netty Harjantien
Keyword(s):  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Multiple Pituitary Hormone Deficiency ◽  
Pituitary Hormone Deficiency

Body composition and metabolic health of young male adults with childhood-onset multiple pituitary hormone deficiency after cessation of growth hormone treatment

2018 ◽  
Vol 31 (5) ◽  
pp. 533-537 ◽  
Author(s):  
Hongbo Yang ◽  
Linjie Wang ◽  
Xiaonan Qiu ◽  
Kemin Yan ◽  
Fengying Gong ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Body Composition ◽  
Growth Hormone Deficiency ◽  
Fat Mass ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Childhood Onset ◽  
Multiple Pituitary Hormone Deficiency ◽  
Pituitary Hormone Deficiency ◽  
Male Patients

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).

scholarly journals A Case of Multiple Pituitary Hormone Deficiency with Anomaly of Internal Carotid Artery

1996 ◽  
Vol 5 (Supple8) ◽  
pp. 127-128 ◽  
Author(s):  
Ryuuzou Takaya ◽  
Masakuni Tokuda ◽  
Shuichi Shimakawa ◽  
Shuhei Suzuki ◽  
Kazutaka Konishi ◽  
...  
Keyword(s):  
Carotid Artery ◽  
Internal Carotid Artery ◽  
Internal Carotid ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Multiple Pituitary Hormone Deficiency ◽  
Pituitary Hormone Deficiency

scholarly journals Laparoscopic appendicectomy in a child with multiple pituitary hormone deficiency

2013 ◽  
Vol 57 (1) ◽  
pp. 88
Author(s):  
SBala Bhaskar ◽  
BP Mallanna ◽  
Chetana Arun ◽  
D Srinivasalu
Keyword(s):  
Laparoscopic Appendicectomy ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Multiple Pituitary Hormone Deficiency ◽  
Pituitary Hormone Deficiency

Multiple Pituitary Hormone Deficiency: Management of Puberty for Optimal Auxological Results

2001 ◽  
Vol 14 (s2) ◽  
Author(s):  
R. Stanhope ◽  
F. De Luca ◽  
H.A. Delemarre-Van de Waal ◽  
A. Liotta ◽  
E. Noijavaara ◽  
...  
Keyword(s):  
Sex Steroids ◽  
Current Practice ◽  
Final Height ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Multiple Pituitary Hormone Deficiency ◽  
Pituitary Hormone Deficiency ◽  
Short Course ◽  
Hypothalamic Pituitary Axis ◽  
Difficult Area

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.

Elevation of serum creatine phosphokinase during growth hormone treatment in patients with multiple pituitary hormone deficiency

1995 ◽  
Vol 154 (11) ◽  
pp. 886-889 ◽  
Author(s):  
Toru Momoi ◽  
Chutaro Yamanaka ◽  
Rieko Tanaka ◽  
Akira Yoshida ◽  
Mitsuyoshi Okumura ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Treatment ◽  
Creatine Phosphokinase ◽  
Hormone Treatment ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Multiple Pituitary Hormone Deficiency ◽  
Pituitary Hormone Deficiency ◽  
Serum Creatine Phosphokinase

scholarly journals Multiple Pituitary Hormone Deficiency Due to Gunshot Injury in a 6-Year-Old Girl

2013 ◽  
Vol 5 (3) ◽  
pp. 209-211 ◽  
Author(s):  
Demirbilek Hüseyin ◽  
Özbek Mehmet Nuri ◽  
Baran Rıza Taner ◽  
Baran Ahmet
Keyword(s):  
Gunshot Injury ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Multiple Pituitary Hormone Deficiency ◽  
Pituitary Hormone Deficiency

Oral Manifestation Associated with Multiple Pituitary Hormone Deficiency and Ectopic Neurohypophysis

2011 ◽  
Vol 35 (4) ◽  
pp. 409-414 ◽  
Author(s):  
T Scaramucci ◽  
CAB Guglielmi ◽  
RD Fonoff ◽  
Zardetto CGD
Keyword(s):  
Dental Treatment ◽  
Medical Condition ◽  
Hormone Deficiency ◽  
Permanent Teeth ◽  
Pituitary Hormone ◽  
Deep Bite ◽  
Multiple Pituitary Hormone Deficiency ◽  
Pituitary Hormone Deficiency ◽  
Oral Manifestation ◽  
Prolonged Retention

Multiple pituitary hormone deficiency (MPHD) is the diminished secretion of all the hormones produced in the anterior lobe of the pituitary gland. The oral manifestation of this condition includes delayed eruption and prolonged retention of primary teeth, delayed formation and eruption of permanent teeth, delay in development and growth of the jaws, tendency towards development of deep bite and enamel disturbances. This paper reports the case of an adolescent patient with MPHD. Clinical examination revealed partial ankylosis and prolonged retention of primary second molars, primary maxillary canines and deep bite. Dental treatment included extraction of all molars with prolonged retention preceded by the necessary medical care with clinical and radiographic follow-up afterwards. The patient was also referred to an orthodontist for orthodontic treatment. Patients' medical condition should always be investigated by clinicians when faced with cases of delayed tooth eruption and bone development.

Sign in / Sign up

Export Citation Format

Share Document