Identifying Clinical Characteristics of Hypoparathyroidism in Turkey: HIPOPARATURK-NET Study

Hypoparathyroidism is an orphan disease with ill-defined epidemiology that is subject to geographic variability. We conducted this study to assess the demographics, etiologic distribution, treatment patterns and complication frequency of patients with chronic hypoparathyroidism in Turkey. This is a retrospective, cross-sectional database study, with collaboration of 30 endocrinology centers located in 20 cities across seven geographical regions of Turkey. A total of 830 adults (mean age 49.6 ± 13.5 years; female 81.2%) with hypoparathyroidism (mean duration 9.7 ± 9.0 years) were included in the final analysis. Hypoparathyroidism was predominantly surgery-induced (n = 686, 82.6%). The insulting surgeries was carried out mostly due to benign causes in postsurgical group (SG) (n = 504, 73.5%) while patients in nonsurgical group (NSG) was most frequently classified as idiopathic (n = 103, 71.5%). The treatment was highly dependent on calcium salts (n = 771, 92.9%), calcitriol (n = 786, 94.7%) and to a lower extent cholecalciferol use (n = 635, 76.5%) while the rate of parathyroid hormone (n = 2, 0.2%) use was low. Serum calcium levels were most frequently kept in the normal range (sCa 8.5–10.5 mg/dL, n = 383, 46.1%) which might be higher than desired for this patient group. NSG had a lower mean plasma PTH concentration (6.42 ± 5.53 vs. 9.09 ± 7.08 ng/l, p < 0.0001), higher daily intake of elementary calcium (2038 ± 1214 vs. 1846 ± 1355 mg/day, p = 0.0193) and calcitriol (0.78 ± 0.39 vs. 0.69 ± 0.38 mcg/day, p = 0.0057), a higher rate of chronic renal disease (9.7% vs. 3.6%, p = 0.0017), epilepsy (6.3% vs. 1.6%, p = 0.0009), intracranial calcifications (11.8% vs. 7.3%, p < 0.0001) and cataracts (22.2% vs. 13.7%, p = 0.0096) compared to SG. In conclusion, postsurgical hypoparathyroidism is the dominant etiology of hypoparathyroidism in Turkey while the nonsurgical patients have a higher disease burden with greater need for medications and increased risk of complications than the postsurgical patients. © 2021, The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature

The Relationship of Frequency and Intensity of Bleeding with Hemophilic Arthropathy in Hemophilia Patients, Radiological Assesment and Socio-Economic Impacts of the Arthropathy

Hemophilic arthropathy is the most common complication of hemophilia, and the most important element for follow-up and treatment of hemophilia. In this study, hemophilic arthropathy was evaluated for bleeding, radiological assessment, and physical examination. Besides, socio-economic outcomes of hemophilic arthropathy and its impacts on quality of life were also investigated. Forty male patients diagnosed with hemophilia in the Hematology Unit of Gaziantep University Medical Faculty were included in the study. Of the 40 patients, 34 had hemophilia A and 6 had hemophilia B; 20 (50%) had severe, 8 (20%) had moderate and 12(30%) had mild hemophilia. According to our data collected in this study, there was a close relationship between hemophilic arthropathy and factor level. It was detected that the factor level and frequency of bleeding (p < 0.05), functional assessment of joints (p < 0.05) and scores of radiological assessment (p < 0.05) was negatively correlated. Frequency of joint bleeding during one-year period had a significantly positive correlation with functional assessment of joints (p < 0.01) and scores of radiological assessment (p < 0.01). It was observed that quality of life impaired in advanced arthropathy patients whose physical examination and radiological assessment scores were high among those who had more frequent bleedings. In addition, with increased frequency of bleeding, factor consumption and treatment costs are also increasing. Hemophilic arthropathy is a serious disease that imposes a large economic burden on patients and society. The full treatment of the disease may only be possible if the treatment is started at an early age and during this period it is crucially important to prevent joint blpedings, rehabilitate disabilities, and to introduce patients into society