Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, 226 014, India.
Department of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
World J Surg. 2021 Feb;45(2):488-495. doi: 10.1007/s00268-020-05816-4. Epub 2020 Oct 12.
There is limited experience in managing pediatric primary hyperparathyroidism (PHPT). The aim of this study was to analyze the clinical presentation and outcome of surgery in children with PHPT managed at a tertiary referral center.
This retrospective study (September 1989-August 2019) consisted of 35 pediatric PHPT patients (< 18 years) who underwent parathyroidectomy. Clinico-pathologic profile and outcome were noted.
The mean age of cohort was 15.2±2.9 years and girls outnumbered boys (M:F = 1:1.9). Familial and symptomatic disease was noted in 8.5 and 94.3% cases, respectively. Skeletal manifestations (83%) were the commonest followed by renal (29%). Fifty-four percent children had skeletal fractures, and 23% were bed-ridden. Among rare manifestations, hypercalcemic crisis, recurrent pancreatitis and stigmata of rickets were observed in 2.8, 11.4 and 14.2% children, respectively. Mean calcium concentration was 12.1 ± 2.0 mg/dl and PTH 91.8 ± 66.5 pmol/L. The sensitivity of preoperative imaging in parathyroid localization was 91.4%. Minimally invasive parathyroidectomy (MIP) was performed in 40% cases. Parathyroid adenoma was observed in 91.4% patients, whereas remaining had hyperplasia. Thirty-four percent suffered from Hungry bone syndrome in postoperative period. The cure rate following primary surgery was 97%. One child with persistent PHPT had successful re-operation. Median follow-up was 5 (1-17) years, and no recurrence or familial disease was revealed during this period.
Majority of pediatric patients present with symptomatic PHPT. Despite relatively high incidence of familial disease select pediatric patients can undergo successful MIP.
小儿原发性甲状旁腺功能亢进症（PHPT）的治疗经验有限。本研究旨在分析在三级转诊中心接受治疗的儿童 PHPT 患者的临床表现和手术结局。
这是一项回顾性研究（1989 年 9 月至 2019 年 8 月），共纳入 35 例接受甲状旁腺切除术的小儿 PHPT 患者（<18 岁）。记录了临床病理特征和结局。
该队列的平均年龄为 15.2±2.9 岁，女孩多于男孩（男女比为 1:1.9）。分别有 8.5%和 94.3%的患者存在家族性和症状性疾病。骨骼表现（83%）最为常见，其次是肾脏（29%）。54%的患儿有骨骼骨折，23%卧床不起。在罕见表现中，分别有 2.8%、11.4%和 14.2%的患儿出现高钙血症危象、复发性胰腺炎和佝偻病体征。平均血钙浓度为 12.1±2.0mg/dl，甲状旁腺激素（PTH）91.8±66.5pmol/L。术前影像学对甲状旁腺定位的敏感性为 91.4%。40%的病例采用了微创甲状旁腺切除术（MIP）。91.4%的患者发现甲状旁腺瘤，其余患者为增生。34%的患者在术后发生饥饿骨综合征。初次手术后的治愈率为 97%。1 例持续性 PHPT 患儿再次手术成功。中位随访时间为 5（1-17）年，在此期间未发现复发或家族性疾病。
大多数小儿患者表现为有症状的 PHPT。尽管家族性疾病发病率相对较高，但部分小儿患者可成功行 MIP。
