儿童和青少年原发性甲状旁腺功能亢进的表现谱。

Q3 Medicine
Hiya Boro, Saurav Khatiwada, Sarah Alam, Suraj Kubihal, Vinay Dogra, Sundeep Malla, Chitresh Kumar
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引用次数: 3

摘要

简介:原发性甲状旁腺功能亢进(PHPT)是儿童和青少年中一种罕见的疾病。早期识别这种疾病对于预防显著的发病率和死亡率是重要的。材料和方法:我们纳入了10例年龄在14 - 19岁的PHPT患者,并在甲状旁腺切除术后进行了长达一年的前瞻性随访。结果:我们的队列包括6名女性和4名男性。患者平均年龄16.7±1.8岁。就诊时的症状为肌肉骨骼疼痛(90%)、骨畸形(50%)、骨折(30%)、近端肌病(40%)、肾结石(50%)、反流症状(40%)和胰腺炎(30%)。平均血清钙3.1±0.5 mmol/l,平均血清无机磷0.9±0.3 mmol/l,平均血清碱性磷酸酶(ALP) 1911.5 IU/l (IQR: 522.7-5702.3)。血清完整甲状旁腺激素中位数为133.5 pmol/l (IQR: 69.5 ~ 178.7),血清25(OH)D中位数为47.7 nmol/l (IQR: 23.7 ~ 72.7)。7例患者出现高钙尿。术后4例(40%)患者出现饥饿骨综合征。典型甲状旁腺瘤9例(90%),非典型腺瘤1例,有丝分裂指数高。手术一年后,所有患者的临床和生化指标均有显著改善,且骨畸形残余持续存在。结论:我们的研究显示了儿童和青少年PHPT的频谱表现和甲状旁腺切除术的结果,直到一年。需要更大规模的长期随访研究,以了解儿童和青少年疾病的真实性质。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

The spectrum of manifestations of primary hyperparathyroidism in children and adolescents.

The spectrum of manifestations of primary hyperparathyroidism in children and adolescents.

The spectrum of manifestations of primary hyperparathyroidism in children and adolescents.

The spectrum of manifestations of primary hyperparathyroidism in children and adolescents.

Introduction: Primary hyperparathyroidism (PHPT) is a rare disease in children and adolescents. Early recognition of this disease is important to prevent significant morbidity and mortality.

Material and methods: We included 10 consecutive patients with PHPT aged 14 to 19 years of age and followed-up prospectively upto one year after parathyroidectomy.

Results: Our cohort included 6 females and 4 males. The mean age of the patients was 16.7 ±1.8 years. The symptoms at presentation were musculoskeletal pain (90%), bone deformity (50%), fracture (30%), proximal myopathy (40%), renal stones (50%), reflux symptoms (40%), and pancreatitis (30%). The mean serum calcium was 3.1 ±0.5 mmol/l, mean serum inorganic phosphorus was 0.9 ±0.3 mmol/l and median serum alkaline phosphatase (ALP) was 1911.5 IU/l (IQR: 522.7-5702.3). The median serum intact parathyroid hormone was 133.5 pmol/l (IQR: 69.5 -178.7) while serum 25(OH)D was 47.7 nmol/l (IQR: 23.7-72.7). Hypercalciuria was observed in 7 patients. Hungry bone syndrome was observed in 4 (40%) patients after surgery. Typical parathyroid adenoma was found in 9 (90%) patients while one patient had atypical adenoma with high mitotic index. After one year of surgery, all patients had significant improvement in clinical and biochemical parameters with persistence of residual bone deformities.

Conclusions: Our study showed the spectrum of manifestations of PHPT in children and adolescents and outcomes of parathyroidectomy till one year. Long-term follow-up studies with bigger cohorts are required to understand the true nature of the disease in children and adolescents.

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来源期刊
Pediatric Endocrinology, Diabetes and Metabolism
Pediatric Endocrinology, Diabetes and Metabolism Medicine-Pediatrics, Perinatology and Child Health
CiteScore
2.00
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发文量
36
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