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Best Practice & Research. Clinical... Dec 2023Growth hormone (GH) deficiency (GHD) is one of the most prevalent deficiencies in patients with hypopituitarism and several cohort studies have demonstrated an increased... (Meta-Analysis)
Meta-Analysis Review
Growth hormone (GH) deficiency (GHD) is one of the most prevalent deficiencies in patients with hypopituitarism and several cohort studies have demonstrated an increased mortality risk in hypopituitary patients with a presumed GHD. The cause of the excess mortality is most likely multifactorial, including the etiology of the hypopituitarism, non-physiological replacement therapies (mostly glucocorticoid), tumor treatment and its side effects as well as untreated GHD. Several years later, other cohort studies that investigated life expectancy in patients with hypopituitarism on GH replacement therapy (GHRT) that showed a normalized mortality. By comparison of the distribution of characteristics of interest between cohorts, we discuss the existing literature to answer the following question: does growth hormone replacement really improve mortality rates in adult patients with hypopituitarism and GHD? We also conducted a meta-analysis of these studies. Since the literature suffers from selection and time bias (improvement of tumor management and other pituitary hormone replacement therapies), there is no high-quality evidence that replacement therapy for GHD really improves mortality. However, the available data does suggest that GHRT plays a significant part in the normalization of the mortality in patients with hypopituitarism.
Topics: Adult; Humans; Hypopituitarism; Dwarfism, Pituitary; Human Growth Hormone; Growth Hormone; Pituitary Neoplasms; Hormone Replacement Therapy
PubMed: 37914564
DOI: 10.1016/j.beem.2023.101835 -
Translational Andrology and Urology Dec 2020Prostate biopsy is the gold standard for the diagnosis of prostate cancer. However, not all patients are suitable for prostate biopsy. For example, some patients have...
Prostate biopsy is the gold standard for the diagnosis of prostate cancer. However, not all patients are suitable for prostate biopsy. For example, some patients have anal stenosis, some patients are too old to withstand the pain caused by puncture, patients who are unwilling to undergo prostate biopsy. We found that there was currently no literature report on a specific solution to this problem. This is the first report of a laparoscopic radical prostatectomy (LRP) in a pituitary dwarfism who didn't have a prostate biopsy before LRP due to anal stenosis. And this report added a new method to diagnose prostate cancer. We present a case of a 61-year-old pituitary dwarfism who had a prostate specific antigen (PSA) of 32.13 ng/mL by physical examination and didn't perform prostate biopsy due to anal stenosis. Preoperative prostate MRI suggests a low-signal mass on the left side of the prostate and Ga PSMA-11 PET/CT demonstrated that Abnormally high PSMA and CHO uptake on the left side of the prostate. Therefore, combined with the patient's PSA, MRI and Ga PSMA-11 PET/CT, our clinical diagnosis was prostate cancer. Surgery was difficult due to narrow pelvic space, but achievable through LRP. Histological analysis revealed multifocal prostate cancer, with negative surgical margins and no extraprostatic extension. Postoperative patient had no serious complications and was discharged. Based on this case, For the first time, we proposed to make full use of the results of clinical tests and imaging examinations for the diagnosis and treatment of diseases without prostate biopsy.
PubMed: 33457254
DOI: 10.21037/tau-20-489 -
Endocrinologia Japonica Apr 1975To investigate hypothalamic-pituitary functions and the primary site of the lesion in idiopathic pituitary dwarfism, various pituitary function tests, especially the...
To investigate hypothalamic-pituitary functions and the primary site of the lesion in idiopathic pituitary dwarfism, various pituitary function tests, especially the pituitary hormone responses to the hypophysiotropic hormones were studied in 23 patients with idiopathic pituitary dwarfism. A few cases showed slight responses of GH to GH stimulation tests. Gonadotropin deficiencies were most frequently noted among pituitary hormones. The basal levels and the responses of plasma LH and FSH to LH-RH test were diminished markedly in all of the cases except in 5 cases with isolated GH deficiency. Responses of LH and FSH to LH-RH improved markedly after a long term administration of LH-RH for a period of one month in 2 patients with gonadotropin deficiency. As to TSH axis, half of the cases accompanied hypothyroidism. However, the responses of TSH to TRH were normal in all of the cases regardless of the thyroid function. The basal levels and the responses of plasma cortisol and 11-deoxycortisol to the rapid metopirone test were also impaired in about half of the cases. Basal levels of plasma prolactin were normal in all of the cases and the responses of prolactin to TRH were normal in cases with normal thyroid function, but slightly delayed in cases with hypothyroidism. It is concluded from the above observations that the incidences of various pituitary hormone deficiencies were quite high in this disorder and hypophysiotropic hormone deficiencies may cause pituitary hormone deficiencies. Therefore, it is suggested that the primary site of the lesion in this disorder might be at the hypothalamus.
Topics: Adolescent; Adult; Arginine; Child; Cortodoxone; Dwarfism, Pituitary; Follicle Stimulating Hormone; Glucagon; Gonadotropin-Releasing Hormone; Growth Hormone; Humans; Hydrocortisone; Hypothalamo-Hypophyseal System; Hypothyroidism; Insulin; Luteinizing Hormone; Metyrapone; Pituitary Function Tests; Prolactin; Propranolol; Thyroid Function Tests; Thyrotropin; Thyrotropin-Releasing Hormone
PubMed: 807471
DOI: 10.1507/endocrj1954.22.97 -
Journal of Korean Medical Science Mar 2022The study aimed to compare the growth responses to 3 years of growth hormone (GH) treatment in children and adolescents with GH deficiency (GHD) according to idiopathic,... (Comparative Study)
Comparative Study
Growth Responses During 3 Years of Growth Hormone Treatment in Children and Adolescents With Growth Hormone Deficiency: Comparison Between Idiopathic, Organic and Isolated Growth Hormone Deficiency, and Multiple Pituitary Hormone Deficiency.
BACKGROUND
The study aimed to compare the growth responses to 3 years of growth hormone (GH) treatment in children and adolescents with GH deficiency (GHD) according to idiopathic, organic, isolated (IGHD), and multiple pituitary hormone deficiency (MPHD).
METHODS
Total 163 patients aged 2-18 years (100 males and 63 females; 131 idiopathic and 32 organic GHD; 129 IGHD and 34 MPHD) were included from data obtained from the LG Growth Study. Parameters of growth responses and biochemical results were compared during the 3-year GH treatment.
RESULTS
The baseline age, bone age (BA), height (Ht) standard deviation score (SDS), weight SDS, mid-parental Ht SDS, predicted adult Ht (PAH) SDS, and insulin like growth factor-1 (IGF-1) SDS were significantly higher in the organic GHD patients than in the idiopathic GHD patients, but peak GH on the GH-stimulation test, baseline GH dose, and mean 3-year-GH dosage were higher in the idiopathic GHD patients than in the organic GHD patients. The prevalence of MPHD was higher in the organic GHD patients than in the idiopathic GHD patients. Idiopathic MPHD subgroup showed the largest increase for the ΔHt SDS and ΔPAH SDS during GH treatment, and organic MPHD subgroup had the smallest mean increase after GH treatment, depending on ΔIGF-1 SDS and ΔIGF binding protein-3 (IGFBP-3) SDS. The growth velocity and the parental-adjusted Ht gain were greater in the idiopathic GHD patients than the organic GHD patients during the 3-year GH treatment, which may have been related to the different GH dose, ΔIGF-1 SDS, and ΔIGFBP-3 SDS between two groups. Multiple linear regression analysis revealed that baseline IGF-1 SDS, BA, and MPH SDS in idiopathic group and baseline HT SDS in organic group are the most predictable parameters for favorable 3-year-GH treatment.
CONCLUSION
The 3-year-GH treatment was effective in both idiopathic and organic GHD patients regardless of the presence of MPHD or underlying causes, but their growth outcomes were not constant with each other. Close monitoring along with appropriate dosage of GH and annual growth responses, not specific at baseline, are more important in children and adolescents with GHD for long-term treatment.
TRIAL REGISTRATION
ClinicalTrials.gov Identifier: NCT01604395.
Topics: Adolescent; Body Height; Child; Child, Preschool; Congenital Hypothyroidism; Dwarfism, Pituitary; Female; Human Growth Hormone; Humans; Male
PubMed: 35315601
DOI: 10.3346/jkms.2022.37.e90 -
Hormone Research in Paediatrics 2022The phase 3 fliGHt Trial evaluated the safety and tolerability of once-weekly lonapegsomatropin, a long-acting prodrug, in children with growth hormone deficiency (GHD)...
INTRODUCTION
The phase 3 fliGHt Trial evaluated the safety and tolerability of once-weekly lonapegsomatropin, a long-acting prodrug, in children with growth hormone deficiency (GHD) who switched from daily somatropin therapy to lonapegsomatropin.
METHODS
This multicenter, open-label, 26-week phase 3 trial took place at 28 sites across 4 countries (Australia, Canada, New Zealand, and the USA). The trial enrolled 146 children with GHD, 143 of which were previously treated with daily somatropin. All subjects received once-weekly lonapegsomatropin 0.24 mg human growth hormone/kg/week. The primary outcome measure was safety and tolerability of lonapegsomatropin over 26 weeks. Secondary outcome measures assessed annualized height velocity (AHV), height standard deviation score (SDS), and IGF-1 SDS at 26 weeks.
RESULTS
Subjects had a mean prior daily somatropin dose of 0.29 mg/kg/week. Treatment-emergent adverse events (AEs) reported were similar to the published AE profile of daily somatropin therapies. After switching to lonapegsomatropin, the least-squares mean (LSM) AHV was 8.7 cm/year (95% CI: 8.2, 9.2) at Week 26 and LSM height SDS changed from baseline to Week 26 of +0.25 (95% CI: 0.21, 0.29). Among switch subjects, the LSM for average IGF-1 SDS was sustained at Weeks 13 and 26, representing an approximate 0.7 increase from baseline (prior to switching from daily somatropin therapy). Patient-reported outcomes indicated a preference for weekly lonapegsomatropin among both children and their parents.
CONCLUSIONS
Lonapegsomatropin treatment outcomes were as expected across a range of ages and treatment experiences. Switching to lonapegsomatropin resulted in a similar AE profile to daily somatropin therapy.
Topics: Body Height; Child; Drug Substitution; Dwarfism, Pituitary; Growth Disorders; Growth Hormone; Human Growth Hormone; Humans; Insulin-Like Growth Factor I
PubMed: 35263755
DOI: 10.1159/000524003 -
Pituitary Apr 2022Adult growth hormone deficiency (AGHD) is considered a rare endocrine disorder involving patients with childhood-onset and adult-onset growth hormone deficiency (AoGHD)... (Review)
Review
Adult growth hormone deficiency (AGHD) is considered a rare endocrine disorder involving patients with childhood-onset and adult-onset growth hormone deficiency (AoGHD) and characterized by adverse cardiometabolic risk profile. Besides traditional cardiovascular risk factors, endothelial dysfunction, low-grade inflammation, impaired adipokine profile, oxidative stress and hypovitaminosis D may also contribute to the development of premature atherosclerosis and higher cardiovascular risk in patients with AGHD. Growth hormone replacement has been proved to exert beneficial effects on several cardiovascular risk factors, but it is also apparent that hormone substitution in itself does not eliminate all cardiometabolic abnormalities associated with the disease. Novel biomarkers and diagnostic techniques discussed in this review may help to evaluate individual cardiovascular risk and identify patients with adverse cardiometabolic risk profile. In the absence of disease-specific guidelines detailing how to assess the cardiovascular status of these patients, we generally recommend close follow-up of the cardiovascular status as well as low threshold for a more detailed evaluation.
Topics: Adult; Atherosclerosis; Child; Dwarfism, Pituitary; Growth Hormone; Hormone Replacement Therapy; Human Growth Hormone; Humans
PubMed: 35106704
DOI: 10.1007/s11102-022-01207-1 -
Pediatric Endocrinology, Diabetes, and... 2022Hypopituitarism is a chronic disease characterized by partial or complete absence of secretion of one or more pituitary hormones. Delayed diagnosis can have serious...
INTRODUCTION
Hypopituitarism is a chronic disease characterized by partial or complete absence of secretion of one or more pituitary hormones. Delayed diagnosis can have serious consequences during the neonatal period and adulthood.
MATERIAL AND METHODS
A retrospective study was conducted on patients who had started treatment in the Children's Clinical University Hospital from 1 January 1995 to 31 December 2020. In total 243 patients were included; they were divided into 3 subgroups: isolated growth hormone deficiency, multiple pituitary hormone deficiency, and panhypopituitarism.
RESULTS
The prevalence of hypopituitarism in Latvia is about 45 cases per 100,000 live births. The average detection age of abnormal growth in Latvia is 8 years and 3 months. Most cases had isolated growth hormone deficiency, at 67.1% (n = 163), followed by cases of multiple pituitary hormone deficiency, at 26.3% (n = 64), and cases of panhypopituitarism, at 6.6% (n = 16). Abnormalities in MRI were found in 44.7% (n = 101) of patients. The most best therapeutic effect was achieved in the first year of treatment: with growth of an average of 9.3 cm (+0.1 SD) for isolated growth hormone deficiency, 9.0 cm (+0.6 SD) for multiple pituitary hormone deficiency, and 11.7 cm (+1SD) for patients with panhypopituitarism.
CONCLUSIONS
It is important to increase awareness and promote early diagnosis for hypopituitarism patients in Latvia and in Europe. More attention should be paid to education about growth restriction problems to parents, caregivers, and other specialists. The treatment should be adjusted individually with the focus not only on physical and mental health but also on safety and treatment costs.
Topics: Infant, Newborn; Child; Humans; Retrospective Studies; Dwarfism, Pituitary; Latvia; Hypopituitarism; Hormones
PubMed: 36047289
DOI: 10.5114/pedm.2022.118320 -
Growth Hormone & IGF Research :... 2019Adult growth hormone deficiency (AGHD) is a rare disease characterised by abnormal body composition, reduced strength and exercise capacity and impaired psychological... (Review)
Review
OBJECTIVES
Adult growth hormone deficiency (AGHD) is a rare disease characterised by abnormal body composition, reduced strength and exercise capacity and impaired psychological wellbeing. An advisory board of leading Central and Eastern European (CEE) endocrinologists was assembled to gain insights into the status of AGHD care in the CEE region. Topics of discussion included the position of adult hypopituitarism/AGHD in health system priorities, availability and affordability of treatments, awareness of AGHD, practice guidelines used in CEE countries and provisions for long-term care of patients.
DESIGN
Prior to the meeting, the advisors were asked to summarise, using an itemised survey questionnaire, the usual standards of care for patients with AGHD in their country. At the meeting, the panel of experts discussed the findings and thereby elucidated similarities and differences among CEE countries; these were compared with international guideline-recommended practices for AGHD.
RESULTS
All CEE countries involved reported having some type of infrastructure in place for care of patients with GHD transitioning from adolescence to adulthood. Most countries reported having at least one specialist centre for patients with AGHD. The main variations across the region included initial entry into healthcare systems, tests required to confirm AGHD diagnosis and medication reimbursement by health authorities. Most CEE countries relied on international society-led guidelines, while some countries have developed national guidelines.
CONCLUSION
The CEE Adult Endocrinology Advisory Board meeting recognised considerable diversity in the care and patient pathways for AGHD across CEE countries. Additional work is needed to optimise care of patients with AGHD in the CEE region.
Topics: Adult; Critical Pathways; Dwarfism, Pituitary; Human Growth Hormone; Humans; Hypopituitarism; Practice Guidelines as Topic; Standard of Care
PubMed: 31234055
DOI: 10.1016/j.ghir.2019.06.001 -
International Journal of Endocrinology... Jul 2015Pituitary stone or pituitary calculus is a scientific enigma characterized by a large calcification in the pituitary sella. It can be discovered incidentally or in a...
INTRODUCTION
Pituitary stone or pituitary calculus is a scientific enigma characterized by a large calcification in the pituitary sella. It can be discovered incidentally or in a patient with endocrine and/or neurological problems. Its mechanism is not understood. In this article, we described three patients harboring a large pituitary calcification.
CASE PRESENTATION
The first case was observed in a 27-year-old woman who consulted for secondary amenorrhea. The second case concerned a woman who consulted for infertility, and the third one was observed in an 11-year and nine-month-old girl who was sent to our department for short stature. Clinical examination was normal in both adults. The pediatric case had dwarfism with lack of pubertal development. Hormonal assessment showed hyperprolactinemia in both women and thyrotroph and somatotroph deficits in the child. Radiologic exploration discovered pituitary calcifications measuring 10, 11, and 45 mm without any cystic or solid mass.
CONCLUSIONS
Radiological findings pleaded for a pituitary stone, but calcified adenomas in women, and calcified craniopharyngioma in the pediatric case could not be excluded, as our three patients were not operated on.
PubMed: 26401144
DOI: 10.5812/ijem.28383v2 -
Current Opinion in Endocrinology,... Feb 2012The diagnosis of growth hormone deficiency (GHD) in childhood is challenging, in large part because of the lack of a true gold standard and the relatively poor... (Review)
Review
PURPOSE OF REVIEW
The diagnosis of growth hormone deficiency (GHD) in childhood is challenging, in large part because of the lack of a true gold standard and the relatively poor performance of available diagnostic testing. This review discusses the recent literature on this topic.
RECENT FINDINGS
Auxology and clinical judgment remain the foundation for the diagnosis of GHD. Provocative growth hormone testing is poorly reproducible, dependent on factors such as body composition and pubertal status, and further limited by significant variability among commercially available growth hormone assays. Measurement of insulin-like growth factor I and insulin-like growth factor-binding protein 3 is not diagnostically useful in isolation but is helpful in combination with other diagnostic measures. Neuroimaging is also useful to inform diagnosis, as pituitary abnormalities suggest a higher likelihood of GHD persisting into adulthood. Although genetic testing is not routinely performed in the diagnosis of GHD at the present time, multiple recent reports raise the possibility that it may play a more important role in diagnosing GHD in the future.
SUMMARY
Beyond physicians' integrated assessment of auxology, clinical presentation, and bone age, current tools to diagnose GHD are suboptimal. Recent literature emphasizes the need to reappraise our current practice and to consider new tools for diagnosis.
Topics: Adolescent; Age Determination by Skeleton; Child; Child, Preschool; Dwarfism, Pituitary; Female; Genetic Testing; Human Growth Hormone; Humans; Insulin-Like Growth Factor Binding Protein 3; Insulin-Like Growth Factor I; Male; Neuroimaging; Reproducibility of Results
PubMed: 22157400
DOI: 10.1097/MED.0b013e32834ec952