Sadacharan Dhalapathy, Rao Smitha S, Mahadevan Shriraam, Shanmugasundar G, Murthy S, Chandrashekaran Shruti, Reddy Vijay Bhaskar, Ramji Bharath
Department of Endocrine Surgery, Rajiv Gandhi Government General Hospital, Madras Medical College, Chennai, Tamil Nadu, India.
Endocrine and Breast Surgery, Department of Oncology, K S Hegde Medical Academy, Mangalore, Karnataka, India.
Indian J Endocrinol Metab. 2024 Jan-Feb;28(1):22-28. doi: 10.4103/ijem.ijem_150_23. Epub 2024 Feb 26.
Primary hyperparathyroidism (PHPT) is a common endocrine condition but rare in the pediatric and adolescent populations. The presentations can be unique, accounting for significant morbidity in the case of untimely detection.
To study surgically treated pediatric PHPT retrospectively.
Surgically treated children of PHPT up to 20 years of age between 2010 to 2022 were analyzed. All of them were operated on by an endocrine surgeon and team.
There was a total of 712 parathyroidectomies over 12 years, out of which there were 52 children (7.3%) had PHPT at less than 20 years of age. This group included 32 male children. The mean age was 16.1 years, including 7 cases of neonatal severe HPT. Multiple Endocrine Neoplasia type 1 was confirmed in 12 children. Presentations were more severe like bone pain (35.13%), renal stones (27.02%), incidental asymptomatic detection (18.9%), failure to thrive (10.8%), and pancreatitis (8.1%) as compared to adults. Mean serum calcium was 12.9 mg/dl (highest-14.1, N-8.8-10.8 mg/dl), mean parathormone levels were 386.91 pg/ml (N-10-65) and vitamin D levels ranged from 2.9-22.8 ng/ml. Localization was done with ultrasound and Tc- SESTAMIBI scans. Mean serum calcium levels in NSPHPT were 28.6 mg/dl (N-8.8-10.8 mg/dl). There were a total of 45 cases (6.32%) of PHPT less than 20 years of age, excluding the cases of NSPHPT. All children underwent parathyroidectomy, with 14 cases having an additional thymectomy, 2 cases with thyroidectomy, and a single case of hemithyroidectomy. The cure rate was 97.3%, while one baby with NSPHPT had persistent disease (postop PTH-110 pg/ml). The uniglandular disease was seen in 54.05% and the rest had a multiglandular disease. Adults accounted for 559/660 cases with 80% uniglandular disease. All cases had a postoperative histopathological confirmation with an average follow-up of 1 year.
Childhood PHPT has a few features same as the adult population. Symptomatic presentations like adults, though pancreatitis and fatigue were more commonly seen as compared to bone pain. Calcium, phosphorus, and parathormone levels were comparable. Uniglandular involvement was seen just like the adult population. There are a few others that make them a distinct subtype like their symptoms of bone pain and being more common among boys. One-fourth of them had MEN1. Fewer cases in this age group make them unique.
原发性甲状旁腺功能亢进症（PHPT）是一种常见的内分泌疾病，但在儿童和青少年人群中较为罕见。其临床表现可能具有独特性，如果未能及时发现，会导致显著的发病率。
回顾性研究接受手术治疗的儿童PHPT。
分析2010年至2022年间接受手术治疗的20岁及以下的PHPT患儿。他们均由内分泌外科医生及其团队进行手术。
12年间共进行了712例甲状旁腺切除术，其中52例（7.3%）为20岁以下的PHPT患儿。该组包括32名男童。平均年龄为16.1岁，其中包括7例新生儿重症甲状旁腺功能亢进症。12名儿童确诊为多发性内分泌腺瘤1型。与成人相比，其临床表现更为严重，如骨痛（35.13%）、肾结石（27.02%）、偶然无症状发现（18.9%）、生长发育迟缓（10.8%）和胰腺炎（8.1%）。平均血清钙为12.9mg/dl（最高值为14.1，正常范围为8.8 - 10.8mg/dl），平均甲状旁腺激素水平为386.91pg/ml（正常范围为10 - 65），维生素D水平在2.9 - 22.8ng/ml之间。通过超声和锝-甲氧基异丁基异腈（Tc-SESTAMIBI）扫描进行定位。新生儿重症甲状旁腺功能亢进症（NSPHPT）的平均血清钙水平为28.6mg/dl（正常范围为8.8 - 10.8mg/dl）。排除NSPHPT病例后，共有45例（6.32%）20岁以下的PHPT患儿。所有患儿均接受了甲状旁腺切除术，其中14例还进行了胸腺切除术，2例进行了甲状腺切除术，1例进行了半甲状腺切除术。治愈率为97.3%，而1例NSPHPT患儿疾病持续存在（术后甲状旁腺激素为110pg/ml）。单腺体疾病占54.05%，其余为多腺体疾病。成人占559/660例，其中80%为单腺体疾病。所有病例术后均经组织病理学证实，平均随访1年。
儿童PHPT有一些与成人相同的特征。与成人一样有症状表现，不过与骨痛相比，胰腺炎和疲劳更为常见。钙、磷和甲状旁腺激素水平相当。与成人一样可见单腺体受累。还有一些特征使它们成为一个独特的亚型，如骨痛症状以及在男孩中更为常见。其中四分之一患有多发性内分泌腺瘤1型。该年龄组病例较少，使其具有独特性。
