scholarly journals Congenital Growth Hormone Deficiency – A Review with a Focus on Neuroimaging

2010 ◽  
Vol 9 (2) ◽  
pp. 136 ◽  
Author(s):  
Sarah L Tsai ◽  
Eoghan Laffan ◽  
◽  
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Medical Literature ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Resonance Imaging ◽  
Pituitary Dysfunction ◽  
Classic Triad ◽  
Frequent Finding ◽  
Magnetic Resonance Imaging Mri

Growth hormone deficiency is an important cause of short stature in childhood. It is characterised by low growth velocity in childhood and is diagnosed by stimulation testing. Individuals with growth hormone deficiency may have other pituitary hormone deficits in addition to growth hormone deficiency. When multiple pituitary hormone deficiencies are present, abnormal pituitary anatomy, as visualised on magnetic resonance imaging (MRI), is a frequent finding. The classic triad (ectopic posterior pituitary, hypoplastic or aplastic anterior pituitary and absent/thin pituitary stalk) or variants of the classic triad are commonly seen in these patients. Volumetric sequencing allows all three planes of visualisation to be reconstructed in post-processing, allowing the radiologist to more fully evaluate pituitary anatomy. The normal dimensions of the pituitary gland vary by age and precise definitions of what constitutes a hypoplastic gland are not clearly defined in the medical literature. Having an experienced neuroradiologist interpret the MRI in patients with pituitary dysfunction is very important.

scholarly journals Congenital Growth Hormone Deficiency—A Review with a Focus on Neuroimaging

2013 ◽  
Vol 09 (02) ◽  
pp. 166
Author(s):  
Sarah L Tsai ◽  
Eoghan Laffan ◽  
◽  
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Medical Literature ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Resonance Imaging ◽  
Pituitary Dysfunction ◽  
Classic Triad ◽  
Frequent Finding ◽  
Magnetic Resonance Imaging Mri

Growth hormone deficiency is an important cause of short stature in childhood. It is characterized by low growth velocity in childhood and is diagnosed by stimulation testing. Individuals with growth hormone deficiency may have other pituitary hormone deficits in addition to growth hormone deficiency. When multiple pituitary hormone deficiencies are present, abnormal pituitary anatomy, as visualized on magnetic resonance imaging (MRI), is a frequent finding. The classic triad (ectopic posterior pituitary, hypoplastic or aplastic anterior pituitary, and absent/thin pituitary stalk) or variants of the classic triad are commonly seen in these patients. Volumetric sequencing allows all three planes of visualization to be reconstructed in postprocessing, allowing the radiologist to more fully evaluate pituitary anatomy. The normal dimensions of the pituitary gland vary by age and precise definitions of what constitutes a hypoplastic gland are not clearly defined in the medical literature. Having an experienced neuroradiologist interpret the MRI in patients with pituitary dysfunction is very important.

scholarly journals Pituitary stalk transection syndrome

2020 ◽  
Vol 7 (12) ◽  
pp. 2397
Author(s):  
Gayathri Sajeevan ◽  
Sajitha Nair ◽  
Devika Geetha ◽  
Nisha Bhavani ◽  
C. Jayakumar ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Anterior Pituitary ◽  
Pituitary Stalk ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Posterior Pituitary ◽  
Resonance Imaging ◽  
Current Report

Growth hormone deficiency is one of the most common endocrinological causes for short stature. It can either be idiopathic or associated with organic causes like tumors or following surgery. One of the rare causes for growth hormone deficiency in children is pituitary stalk transection syndrome. It can be diagnosed by magnetic resonance imaging of the hypothalamus and pituitary gland which shows an ectopic or absent posterior pituitary, an absent or interrupted pituitary stalk, or small anterior pituitary in combination with growth hormone or other pituitary hormone deficiencies. Current report presents a child with pituitary stalk transection syndrome who was brought for evaluation of hypoglycemic seizures.

A novel diagnostic tool for the evaluation of hypothalamic-pituitary region and diagnosis of growth hormone deficiency: pons ratio

2020 ◽  
Vol 33 (6) ◽  
pp. 735-742
Author(s):  
Meliha Demiral ◽  
Mehmet Salih Karaca ◽  
Edip Unal ◽  
Birsen Baysal ◽  
Rıza Taner Baran ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Cost Benefit ◽  
Practical Method ◽  
Hormone Deficiency ◽  
Small Scale ◽  
Pituitary Hormone ◽  
Clinical Value ◽  
Pubertal Status ◽  
Sensitive Tool

AbstractBackgroundsLimitations in the evaluation of the pituitary size and changes according to pubertal status make its validity questionable. Recently, in a small-scale study, pons ratio (PR) has been suggested as a more sensitive tool for diagnosis and etiological evaluation of growth hormone deficiency (GHD). The aim of the study is to evaluate the diagnostic value of PR in the diagnosis of GHD.MethodsWe retrospectively evaluated the pituitary magnetic resonance imaging (MRI) of 133 patients with a diagnosis of GHD. Primary axis (PA) was assigned as a line crossing the mid-sagittal dorsum sella and fourth ventricle. PR was defined as the pons height above the PA divided by total pons height. The PR of patients with GHD was compared to subjects without GHD.ResultsStudy included 133 patients with GHD and 47 controls. In total, 121 (91%) patients had isolated GHD and 12 (9%) patients had multiple pituitary hormone deficiency. The PR of the patient group (mean: 0.32 ± 0.89; range: 0.14–0.63) was significantly higher than controls (mean: 0.26 ± 0.067; range 0.19–0.44) (p: 0.000). The optimal cut-off value of PR for GHD diagnosis was 0.27 (sensitivity 71% specificity 56%). There was a negative correlation between anterior pituitary height (APH)-SDS and PR (p: 0.002; r: −0.27). APH was increased, but PR remained unchanged in pubertal patients (p: 0.089).ConclusionsPR measurement is a noninvasive, practical method with a cost-benefit clinical value. As it is not affected by pubertal status, PR is potentially a more sensitive tool for evaluation of pituitary gland in GHD patients compared to APH.

Growth hormone and insulin-like growth factor regulate insulin-like growth factor-binding protein-1 in Laron type dwarfism, growth hormone deficiency and constitutional short stature

1992 ◽  
Vol 127 (4) ◽  
pp. 351-358 ◽  
Author(s):  
Zvi Laron ◽  
Anne-Maria Suikkari ◽  
Beatrice Klinger ◽  
Aviva Silbergeld ◽  
Athalia Pertzelan ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Growth Factor ◽  
Growth Hormone Deficiency ◽  
Short Stature ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Healthy Children ◽  
Insulin Like Growth Factor ◽  
Factor Binding ◽  
Igf I

Insulin-like growth factors (IGFs) mediate the effects of growth hormone (GH), and the insulin-like growth factor-binding proteins (IGFBPs) modulate the actions of IGFs in tissues. We studied the circulating levels of IGFBP-1 in 6 children and 9 adults with Laron type dwarfism (LTD), in 11 children and 21 adults with growth hormone deficiency (GHD), and in 8 children with constitutional short stature. Compared with the situation in healthy children, the basal serum IGFBP-1 concentration was 5.4-fold higher in LTD children, 4.1-fold higher in GHD children, and 3.8-fold higher in children with short stature (p<0.02 vs controls in all groups). In adult patients with multiple pituitary hormone deficiency (MPHD), the IGFBP-1 concentration was 2-fold elevated, but it was normal in adult LTD patients. Intravenous (N= 10) or subcutaneous (N=9) administration ofIGF-I (75 μg·kg−1 and 150 μg·kg−1, respectively) in LTD children resulted in a rapid 50–60% fall in serum insulin (p<0.02), a decline in blood glucose and a concomitant 40–60% rise of IGFBP-1 levels (p<0.05). Treatment for seven days with IGF-I (150 μg·kg−1·d−1) resulted in a decrease by 34% and 44% of serum IGFBP-1 level in two out of three children with LTD. After prolonged GH therapy, the IGFBP-1 level fell in GHD children by 29% (p<0.05), in GHD adults by 52% (p<0.02) and in children with constitutional short stature by 17% (p<0.02). IGFBP-1 and insulin concentrations were inversely related in patients with GHD (r= −0.66, p<0.001) or with LTD (r= −0.57, p<0.05). Our data suggest that: (a) increased IGFBP-1 concentration in LTD, GHD and constitutional short children may, at least in part, be accounted for by an IGF-I deficiency; (b) both the rise in IGF-I and a fall in insulin contributed to the rise in IGFBP-1 after acute IGF-I administration; (c) prolonged IGF-I or GH treatment causes a persistent decline in IGFBP-1 concentration. In conclusion, IGF-I and GH may regulate IGFBP-1 secretion either directly or via insulin.

Marked phenotypic variable expression among brothers with duplication of Xq27.1 involving the SOX3 gene

2020 ◽  
Vol 33 (3) ◽  
pp. 443-447 ◽  
Author(s):  
Elizabeth T. Rosolowsky ◽  
Robert Stein ◽  
Seth D. Marks ◽  
Norma Leonard
Keyword(s):  
Growth Hormone ◽  
Mental Retardation ◽  
Growth Hormone Deficiency ◽  
Hormone Deficiency ◽  
Pharyngeal Arch ◽  
Pituitary Hormone ◽  
Facial Dysmorphisms ◽  
Pituitary Hormone Deficiency ◽  
Variable Expression ◽  
Sox3 Gene

AbstractWe describe four phenotypically different brothers who share the same microduplication of Xq27.1, which contains the SOX3 gene. SOX3 mutations have been associated with growth hormone deficiency, variable degrees of additional pituitary hormone deficiencies, and mental retardation. SOX3 also appears to play an important role in pharyngeal arch segmentation that gives rise to craniofacial structures. While these four brothers have inherited the same mutation, they manifest a spectrum of phenotypes, ranging from complete, multiple pituitary hormone deficiencies to no apparent pituitary hormone deficiency with or without craniopharyngeal/facial dysmorphisms. We look to the literature to provide putative explanations for the variable expression of the brothers’ shared SOX3 mutation.

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).

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