Personality traits and sex-role schema in adult patients with childhood-onset combined pituitary hormone deficiency not treated with growth hormone

2017 ◽  
Vol 86 (4) ◽  
pp. 279
Author(s):  
Izabela Warmuz-Stangierska ◽  
Monika Gołąb ◽  
Adam Stangierski ◽  
Katarzyna Piątek ◽  
Natalie Musial ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Personality Traits ◽  
Minnesota Multiphasic Personality Inventory ◽  
Sex Role ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Childhood Onset ◽  
Pituitary Hormone Deficiency ◽  
Combined Pituitary Hormone Deficiency ◽  
Polish Version

Background. Patients with combined pituitary hormone deficiency have quantitative and qualitative abnormalities of pituitary hormone production that may trigger psychological consequences. Several studies have evidenced symptoms of social disturbances in these patients. Aim. The aim of this study was to evaluate personality traits and psychological sex-role schema influencing social adaptation in patients with childhood-onset combined pituitary hormone deficiency.Material and Methods. Study involved a unique group of 28 adult patients with childhood-onset combined pituitary hormone deficiencies that were never treated with growth hormone. To psychological assessment the short Polish version of Bem’s Sex Role Inventory and the Polish version of Minnesota Multiphasic Personality Inventory were used in the study. Results. The analysis of scores on the Polish version of Minnesota Multiphasic Personality Inventory showed significantly elevated results in the scales for lying, hysteria, psychopathic deviation, hypochondria, and schizophrenia as well as decreased scores in hypomania indicating a number of symptoms of maladjustment in many different areas of life. The short Polish version of Bem’s Sex Role Inventory scores indicated that most of combined pituitary hormone deficiency patients were sex-undifferentiated and no one was androgynous. Conclusions. The sex-role schema and certain personality traits seem to predispose childhood-onset combined pituitary hormone deficiency patients not treated with growth hormone to problems with social adaptation and greater susceptibility to situational stressors. Neurotic reactions, tendency for social alienation, and lack of flexibility have all been observed in these patients. Therefore, combined pituitary hormone deficiency patients may more often need special support when it comes to coping with disease.

scholarly journals Cognitive function in patients with childhood‑onset combined pituitary hormone deficiency not treated with growth hormone

2016 ◽  
Vol 85 (3) ◽  
pp. 178
Author(s):  
Izabela Warmuz-Stangierska ◽  
Monika Gołąb ◽  
Adam Stangierski ◽  
Małgorzata Kałużna ◽  
Elvira Rak ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Cognitive Function ◽  
Fluid Intelligence ◽  
Adult Patients ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Childhood Onset ◽  
Verbal Iq ◽  
Pituitary Hormone Deficiency ◽  
Combined Pituitary Hormone Deficiency

Introduction. Growth hormone deficiency is a known factor leading to impairment of psychological performance but there are very few studies on cognitive function in adult patients with childhood‑onset combined pituitary hormone deficiency (ChO‑CPHD). Therefore, the aim of our study was to assess cognitive skills in adult patients with this disorder. Material and Methods. The study was performed in a unique group of 28 adult ChO‑CPHD patients, never treated with growth hormone (mean age 42.5 +/- 16.3 years; 12 women and 16 men). The assessment of cognitive performance (WAIS‑R) comprised scores of verbal IQ, non‑verbal IQ and particular qualitative analysis of subtests. Results. The mean score of full scale (IQ = 81,4) was below the normal when compared to the population norm (IQ = 100, SD +/- 15), although their verbal score (IQ = 85,7) was a bit higher than their nonverbal score (IQ = 81,7). Conclusions. The obtained result of total intelligence quotient (IQ) had shown equable subnormal results and a cognitive level below average in the study group. Decreased subscales of WAIS‑R are related to fluid intelligence (this may reflect abnormal brain development or could be linked to the influence of hormonal disorders in early life) as well as, emotional traits of personality.

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 Identification of a Novel PROP1 Mutation in a Patient with Combined Pituitary Hormone Deficiency and Enlarged Pituitary

2019 ◽  
Vol 20 (8) ◽  
pp. 1875 ◽  
Author(s):  
Laura Penta ◽  
Carla Bizzarri ◽  
Michela Panichi ◽  
Antonio Novelli ◽  
Francesca Romana Lepri ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Novel Mutation ◽  
Pituitary Hormones ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Pituitary Hormone Deficiency ◽  
Combined Pituitary Hormone Deficiency ◽  
Familial Cases ◽  
Number Of Patients

Growth hormone deficiency (GHD) can be present from the neonatal period to adulthood and can be the result of congenital or acquired insults. In addition, GHD can be classified into two types: isolated growth hormone deficiency (IGHD) and combined pituitary hormone deficiency (CPHD). CPHD is a disorder characterized by impaired production of two or more anterior and/or posterior pituitary hormones. Many genes implicated in CPHD remain to be identified. Better genetic characterization will provide more information about the disorder and result in important genetic counselling because a number of patients with hypopituitarism represent familial cases. To date, PROP1 mutations represent the most common known genetic cause of CPHD both in sporadic and familial cases. We report a novel mutation in the PROP1 gene in an infant with CPHD and an enlarged pituitary gland. Close long-term follow-up will reveal other possible hormonal defects and pituitary involution.

scholarly journals LHX3 deficiency presenting in the United States with severe developmental delay in a child of Syrian refugee parents

2018 ◽  
Vol 2018 ◽  
Author(s):  
Susan Ahern ◽  
Mark Daniels ◽  
Amrit Bhangoo
Keyword(s):  
United States ◽  
Growth Hormone ◽  
Novel Mutation ◽  
Hormone Replacement ◽  
The United States ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Central Hypothyroidism ◽  
Pituitary Hormone Deficiency ◽  
Combined Pituitary Hormone Deficiency

Summary In this case report, we present a novel mutation in Lim-homeodomain (LIM-HD) transcription factor, LHX3, manifesting as combined pituitary hormone deficiency (CPHD). This female patient was originally diagnosed in Egypt during infancy with Diamond Blackfan Anemia (DBA) requiring several blood transfusions. Around 10 months of age, she was diagnosed and treated for central hypothyroidism. It was not until she came to the United States around two-and-a-half years of age that she was diagnosed and treated for growth hormone deficiency. Her response to growth hormone replacement on linear growth and muscle tone were impressive. She still suffers from severe global development delay likely due to delay in treatment of congenital central hypothyroidism followed by poor access to reliable thyroid medications. Her diagnosis of DBA was not confirmed after genetic testing in the United States and her hemoglobin normalized with hormone replacement therapies. We will review the patient’s clinical course as well as a review of LHX3 mutations and the associated phenotype. Learning points: Describe an unusual presentation of undertreated pituitary hormone deficiencies in early life Combined pituitary hormone deficiency due to a novel mutation in pituitary transcription factor, LHX3 Describe the clinical phenotype of combined pituitary hormone deficiency due to LHX3 mutations

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