Primary Hyperparathyroidism in Young and Adolescents: Alike or Unlike Adult Hyperparathyroidism? - A Series from South India.

Background: 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. Aim: To study surgically treated pediatric PHPT retrospectively. Methods: 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. Results: 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 99mTc- 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. Conclusion: 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.

Response of South Indian girls with central precocious puberty to gonadotrophin analogue (GnRHa) therapy - a single center experience.

AIM OF THE STUDY: To describe the clinical, auxological, biochemical and radiological response to GnRH analogue in female children with central precocious puberty (CPP). MATERIAL AND METHODS: The data on 22 female children presenting with the larche < 8 years, pubarche < 8 years or menarche < 9 years diagnosed as CPP was collected from the records over a four year period. Assessment included growth parameters, Tanners staging; bone age (BA) by Greulich and Pyle method, ultrasonography of abdomen to assess uterine length and ovarian size and z score derived; biochemical evaluation included serum luteinising hormone (LH), follicle stimulating hormone (FSH) and estradiol (E2); and MRI brain. The children were initiated on injection Leupride 0.9 mg/kg 3 monthly (body weight   30 kg received 22.5 mg). The predicted adult height (PAH) was calculated with Bayley Pinneau method. RESULTS: Treatment was started at the mean chronological age (CA) of 6.09 ±2.1 years and continued till 8.3 ±2.4 years. MRI brain was abnormal in 4 children. Duration of treatment was 2.1 ±0.4 years. The height z scores reduced from 0.5 ±2.4 to 0.18 ±2.4 (p < 0.05). A significant reduction in tanner's stage, uterine size and ovarian volume was observed in the study period. BA/CA ratio reduced from 1.27 ±0.4 to 1.07 ±0.3. PAH z score improved from 1.47 ±1.6 to 0.2 ±2.3 (p < 0.05). CONCLUSIONS: We observed a good clinical and radiological response to GnRHa therapy in girls with CPP and a significant improvement in PAH. Long term follow up is needed to assess the attainment of final height.

LDL-cholesterol: Friedewald calculated versus direct measurement-study from a large Indian laboratory database.

BACKGROUND: Validity of Friedewald formula (FF) in patients with serum triglycerides (TGs) <400 mg/dl is unclear. MATERIALS AND METHODS: We compared low-density lipoprotein (LDL)-cholesterol calculated by FF to directly measured LDL in a laboratory database of 14,620 lipid profile samples from south India. RESULTS: LDL by FF correlated with directly measured LDL with correlation coefficient of 0.89 with the best correlation seen in TG levels 100-150. Higher level of TG (>200) underestimates the LDL calculated by FF particularly at LDL values <70 mg/dl. On the other hand, LDL is overestimated by FF in more than 70% of cases at LDL levels >130 mg/dl. CONCLUSION: We suggest repeating the LDL by direct assay techniques particularly in patients with TG >200 and when LDL <70 or >130. This helps in correctly stratifying the coronary artery diseases' (CADs') risk and goals of treatment.