Despite being a classical growth disorder, pituitary gigantism has not been studied previously in a standardized way. We performed a retrospective, multicenter, international study to characterize a large series of pituitary gigantism patients. We included 208 patients (163 males; 78.4%) with growth hormone excess and a current/previous abnormal growth velocity for age or final height O2 S.D. above country normal means. The median onset of rapid growth was 13 years and occurred significantly earlier in females than in males; pituitary adenomas were diagnosed earlier in females than males (15.8 vs 21.5 years respectively). Adenomas were R10 mm (i.e., macroadenomas) in 84%, of which extrasellar extension occurred in 77% and invasion in 54%. GH/IGF1 control was achieved in 39% during long-term follow-up. Final height was greater in younger onset patients, with larger tumors and higher GH levels. Later disease control was associated with a greater difference from mid-parental height (rZ0.23, PZ0.02). AIP mutations occurred in 29%; microduplication at Xq26.3 -X-linked acrogigantism (X-LAG) -occurred in two familial isolated pituitary adenoma kindreds and in ten sporadic patients. Tumor size was not different in X-LAG, AIP mutated and genetically negative patient groups. AIP-mutated and X-LAG patients were significantly younger at onset and diagnosis, but disease control was worse in genetically negative cases. Pituitary gigantism patients are characterized by male predominance and large tumors that are difficult to control. Treatment delay increases final height and symptom burden. AIP mutations and X-LAG explain many cases, but no genetic etiology is seen in O50% of cases.
Традиционные представления о витамине D свя-заны прежде всего с его ключевой ролью в кальцие-во-фосфорном обмене и влиянии на минеральную плотность костной ткани [1]. Эти «классические» функции витамина D были известны достаточно давно, поэтому данный витамин широко применял-ся для профилактики рахита у детей, для профилак-тики и лечения остеопороза у взрослых. В последнее время представления о витамине D пересмотрены, поскольку стало известно, что витамин D является по сути стероидным гормоном, необходимым для обеспечения широкого спектра физиологических процессов во всех органах. Currently, the vitamin D deficiency is pandemic. The impact of vitamin D on reproductive function both in men and women is proven. In this regard, the modern correction of vitamin D deficiency plays a significant role in solving reproductive problems. The review is devoted to the problem of correction of vitamin D levels in different population groups. The results of a study in a large cohort of males and females performed at the "Clinic of Professor kalinchenko" (Moscow) confirmed a low vitamin D level in the adult population. Therefore, correction of vitamin D deficiency is not exclusively a pediatric problem. Many diseases in the middle and old age populations are directly associated with vitamin D deficiency. Метаболизм и основные эффекты витамина D
Abdominal obesity is a major component of metabolic syndrome (MS) considered to be the key factor contributing to the development of testosterone deficiency (hypogonadism) in men. Bearing in mind the etiopathogenetic relationship between MS and urolithiasis (UL), the present study was undertaken for the purpose of elucidating the role of the main biochemical risk factors of metabolic syndrome in men, evaluating the influence of correction of hypogonadism on these factors, and optimization of the treatment and prevention of MS in these patients. The study included 90 men presenting with hypogonadism in whom the serum levels and renal excretion of the main lithogenic substances (uric acid, calcium, phosphates, magnesium) and urine pH were measured. At the first stage of the study, the patients were allocated to two groups. Group 1 was comprised of 52 patients with UL, group 2 consisted of 38 patients without UL. The patients of group 1 were found to suffer hyperuricemia (the serum uric acid levels 430 mcmol/l and higher). The patients of both groups exhibited hyperuricosuria (daily uric acid excretion over 4.13 mmol/24 hr) with the urine pH value of 5.5 and lower. The serum uric acid concentration was shown to positively correlate with the insulin and C-peptide levels. At the second stage of the study, 65 randomly selected patients were divided into two groups in one of which (n=40) they were given testosterone therapy. Compensation of hypogonadism in these patients was associated with a significant reduction of the serum uric acid levels and its daily excretion, an increase and normalization of urine pH, and a rise in the serum magnesium concentration, Similar significant changes of the same parameters were absent in the control subjects (n=25). Moreover, testosterone therapy improved characteristics of lipid and carbohydrate metabolism, caused reduction in the body weight, BMI, and the severity of insulin resistance.
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