Di Maio Salvatore, Soliman Ashraf T, De Sanctis Vincenzo, Kattamis Christos C
Emeritus Director in Pediatrics, Santobono-Pausilipon Children's Hospital, Naples, Italy.
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Acta Biomed. 2018 Mar 27;89(1):122-131. doi: 10.23750/abm.v89i1.7118.
The diagnosis of hypoparathyroidism(HPT)is readily made in the presence of hypocalcemia with markedly reduced or absent parathormone (PTH) levels. Currently available treatments for HPT include high dose vitamin D (ergocalciferol, D2 and cholecalciferol, D3) or, the active metabolite dihydroxy vitamin D (calcitriol), in addition to calcium supplements.This regimen, if not well monitored, can lead to hypercalciuria, as PTH deficiency impairs renal calcium reabsorption. Thus the goal of treatment, is to maintain serum calcium at the low end of the normal range. Undertreatment can cause symptomatic hypocalcemia, while overtreatment hypercalciuria, which may lead to nephrolithiasis, nephrocalcinosis, and renal insufficiency. At present, there is no consensus on the management of HPT in children and adolescents and only few studies are available on the long term outcome of patients with recombinant HPT treatment. The purpose of this article is to review, in a comprehensive manner, the major aspects of HPT management in children and adolescents waiting for authoritative guidelines for the treatment of HPT in this group of patients. Further research, addressing specific questions for this population are urgently needed to improve long-term safety of patients. Educational interventions are also needed for professionals, parents and patients to enable them to improve knowledge, quality of life and effective management care at home.
甲状旁腺功能减退症(HPT)的诊断在血钙过低且甲状旁腺激素(PTH)水平显著降低或缺乏时很容易做出。目前用于治疗HPT的方法包括高剂量维生素D(麦角钙化醇,D2和胆钙化醇,D3),或者活性代谢物二羟基维生素D(骨化三醇),此外还需补充钙剂。如果对该治疗方案监测不当,可能会导致高钙尿症,因为甲状旁腺激素缺乏会损害肾脏对钙的重吸收。因此,治疗的目标是将血清钙维持在正常范围的下限。治疗不足会导致有症状的低钙血症,而治疗过度则会导致高钙尿症,这可能会引发肾结石、肾钙质沉着症和肾功能不全。目前,关于儿童和青少年HPT的管理尚无共识,关于重组HPT治疗患者长期预后的研究也很少。本文的目的是全面回顾儿童和青少年HPT管理的主要方面,等待针对该组患者HPT治疗的权威指南。迫切需要针对该人群的特定问题进行进一步研究,以提高患者的长期安全性。还需要对专业人员、家长和患者进行教育干预,以使他们能够提高知识水平、改善生活质量并在家中进行有效的管理护理。