The Russian Registry of Chronic Hypoparathyroidism

Abstract

IntroductionChronic hypoparathyroidism is a relatively rare disease associated with multicomponent medical therapy and various complications. The analysis of large databases of patients with chronic hypoparathyroidism is a necessary tool to enhance quality of medical care, as well as to determine the optimal clinical and therapeutic approaches, and prognostic markers of the disease.The Aimof this study is to estimate the clinical and biochemical profile, long-term complications, medical therapy and disease control of the patients with chronic postsurgical and non-surgical hypoparathyroidism.Materials and Methodsthe cross-sectional, observational, continuous study was based on the Russian Registry of patients with hypoparathyroidism. 544 patients from 63 regions of the Russian Federation were included in this study.ResultsThe majority of cases had postsurgical etiology (88.4%). Postsurgical hypoparathyroidism prevailed in females (р<0.001). About a half of patients had blood calcium and phosphorus targets, 56 and 52% respectively. Nephrolithiasis was confirmed in 32.5%, nephrocalcinosis - in 12.3% of cases. The risk of nephrocalcinosis/nephrolithiasis increased by 1.85 times with disease duration more than 4.5 years. The cataract was found in 9.4%. The cut-off point for the development of cataracts was 9.5 years, with a 6.96-fold increased risk. The longer duration of hypoparathyroidism of any etiology was associated with more frequent cataract (p=0.0018).We found brain calcification in 4%, arrhythmias in 7.2% and neuropsychiatric symptoms in 5.15% of cases. Generally, the BMD in the studied group corresponded to age values, and there was no evidence for the phenomenon of high bone density. TBS was consistent with normal bone microarchitectonics. In our study, the majority of patients (83.5%) was treated with standard therapy of calcium and vitamin D supplements. 5 patients with severe disease course were treated with rhPTH (1–34).ConclusionsAnalysis of the presented database indicates insufficient diagnosis of the complications associated with chronic hypoparathyroidism. Overall, hypoparathyroidism is associated with higher risks of renal stone formation, decreased GFR, cataract especially in patients with longer duration of disease.

Biochemical Control of 78 Patients with Chronic Hypoparathyroidism Referred between 2006 and 2020 – Where do We Actually Stand?

“…Concerning possible diff erences between surgical and non-surgical cases we confi rmed the tendency for non-surgical patients to be younger and to present with deeper hypocalcemia and hypomagnesiemia. This fi nding has already been reported in previous studies by other authors [14,30]. The reasons behind these diff erences are not yet clearly elucidated but surely refer to the diff erent etiology of hypoPT (lack of parathyroid tissue versus autoimmune or invasive destruction of the parathyroid cells and secretion) [31].…”

“…At the same time hypercalciuria was relatively uncommon (in 11.8%). In other detailed studies the prevalence of renal stones had been reported as low as 32.5% [14] or even < 27% [15]. The detrimental impact of the hypoPT itself on the kidney has already been documented [23].…”

Dihydrotachysterol: a bad choice in the treatment of chronic hypoparathyroidism

“…По данным анализа всероссийского регистра пациентов с гипопаратиреозом (n=544) отмечено, что нарушение функции почек со снижением СКФ менее 60 мл/мин/1,73 м 2 происходит в 15,8% случаев, нефролитиаз выявляется у 32,5% пациентов, нефрокальциноз -у 12,3%. Установлено, что уровень кальциемии имеет отрицательную корреляцию с рСКФ, а риски структурных нарушений почек повышаются с увеличением длительности заболевания более 4,5 года [16].…”

Skeletal and nonskeletal consequences of hypoparathyroidism

“…The skeletal consequences of chronic HypoPT conventionally treated with calcium supplements and active vitamin D are described below and summarized in Table 1. Bone remodeling Reduced • Bone turnover markers in the lower half of the normal range (12,16).…”

“…The bone formation markers procollagen type 1 amino-terminal propeptide, osteocalcin, and bone-specific alkaline phosphatase are decreased, along with reduced levels of the bone resorption markers serum C-telopeptide and tartrate-resistant acid phosphatase 5b ( 1 ). Of note, while histomorphometry of bone biopsies shows a marked reduction in bone remodeling, bone turnover markers are not frankly low but are usually in the lower half of the normal range ( 12 , 16 ), which limits their use in clinical practice to delineate the status of bone turnover in patients with HypoPT.…”