Hyperparathyroidism In Children Research Articles

Primary hyperparathyroidism in children and adolescents: Clinical features and treatment outcomes from an Italian multicenter study

Primary hyperparathyroidism in children and adolescents: Clinical features and treatment outcomes from an Italian multicenter study

Surgical treatment of secondary hyperparathyroidism in children with chronic kidney disease. Experience in 19 patients.

Secondary hyperparathyroidism (sHPT) is an important contributor to bone disease and cardiovascular calcifications in children with chronic kidney disease (CKD). When conservative measures are ineffective, parathyroidectomy is indicated. The aim of our study was to evaluate the efficacy and safety of subtotal parathyroidectomy (sPTX) in pediatric and adolescent patients, and to provide a rationale for considering this aggressive treatment in CKD patients with uncontrolled sHPT. We retrospectively analyzed the medical records of 19 pediatric CKD patients on dialysis with refractory sHPT who underwent sPTX at our institution between 2010 and 2020. All patients had clinical, radiological, and biochemical signs of renal osteodystrophy. One year after sPTX, parathyroid hormone (PTH) levels (median and interquartile range (IQR)) dropped from 2073 (1339-2484) to 164 (93-252) pg/mL (p=0.0001), alkaline phosphatase (ALP) levels from 1166 (764-2373) to 410 (126-421) IU/L (p=0.002), and the mean (±SDS) calcium-phosphate (Ca*P) product from 51±11 to 41±13mg2/dL2 (p=0.07). Postoperatively, all patients presented with severe hungry bone syndrome (HBS) and required intravenous and oral calcium and calcitriol supplementation. None of them had other postoperative complication. Histological findings had a good correlation with preoperative parathyroid ultrasound imaging (n: 15) in 100 % and with technetium-99m (99mTc) sestamibi scintigraphy (n: 15) in 86.6 %. Clinical and radiological signs of bone disease improved in all patients. Pediatric sPTX is effective and safe to control sHPT and calcium-phosphate metabolism in children with CKD on dialysis and may mitigate irreversible bone deformities and progression of cardiovascular disease.

Determinants of hyperparathyroidism in children after kidney transplantation.

Hyperparathyroidism (HPT) can contribute to metabolic bone disease following kidney transplantation. We evaluated post-transplant trends in intact parathyroid hormone (iPTH) and determined predictors of HPT in pediatric kidney transplant (KTx) recipients. In this single-center study, retrospective data were collected on 88 children from 2013 to 2019. Data collected included dialysis vintage, biochemical parameters, post-transplant trends in iPTH, 25(OH)Vitamin D levels and estimated glomerular filtration rate (eGFR ml/min/1.73 m2 ). Pre-transplant treatment for HPT was quantified with a Treatment Burden score (TB, score range: 0-100). After log-transforming skewed variables (iPTH and eGFR), multivariable linear regression was performed to determine predictors of log {iPTH} at 6 and 36 months (mo) post-transplant. Median age was 12.8 (range: 1.9-20.5) years, and dialysis vintage was 11.2 (range:0.0-112.9) months. The majority were of Hispanic and African Ancestry (77.3%). Median post-transplant iPTH was 69.5 (range: 1.8-306.8) pg/ml at 6mo with a gradual downward trend to 59.0 (range: 28.0-445.0) pg/ml at 36mo. Significant multivariable predictors of higher log {iPTH} post-transplant included longer dialysis vintage, higher TB, and lower log{eGFR} at 6mo, and higher TB, lower log{eGFR}, and deceased donor transplant at 36mo. Recognition of risk factors for HPT and monitoring iPTH post-transplant may facilitate timely interventions to mitigate cardiovascular and bone disease in pediatric KTx recipients. Describe serial trends in intact PTH after kidney transplantation. Pre- and post-transplant factors that contribute to persistence or re-occurrence of hyperparathyroidism after kidney transplantation in children include longer dialysis vintage, high pre-transplant treatment burden and decreased post-transplant GFR. Recognition of these factors, and monitoring intact PTH after kidney transplantation, could facilitate timely interventions to mitigate cardiovascular and bone disease in children.

"Primary hyperparathyroidism in children: assessment of the state of bone tissue before and after treatment"

Evaluating the effectiveness of treatment of the bone form of primary hyperparathyroidism in children

Primary hyperparathyroidism in children

Primary hyperparathyroidism (PHPT) is an endocrine disorder characterized by excessive secretion of parathyroid hormone (PTH) with upper-normal or elevated blood calcium levels due to primary thyroid gland pathology. PHPT is a rare pathology in children, with a prevalence of 2-5:100,000 children according to the literature. Due to the non-specificity of clinical manifestations at onset (nausea, vomiting, abdominal pain, emotional lability), the disease may remain undiagnosed for a long time. To study the features of the course and molecular genetic basis of primary hyperparathyroidism in children. Retrospective observational study of 49 patients diagnosed with primary hyperparathyroidism. All patients underwent a comprehensive laboratory-instrumental and molecular genetic study at the Institute of Pediatric Endocrinology, Endocrinology Research Center of Russia in the period 2014-2022. The first clinical symptoms of PHPT were noted at the age of 13.8 years [10.6; 1 5.2], among which fatigue, headaches, dyspepsia, lower limb pain, and fractures were the most common. The age of diagnosis was 15.81 years [13.1; 16.8], all children were found to have high levels of PTH, total and ionized calcium, with hypophosphatemia in 93.9% of patients (n=46) and hypercalciuria in 43% (n=21). Five out of 49 patients (10.2%) were found to have ectopy of the thyroid: 3 showed an intrathyroidal location, 2 in the mediastinal region. Molecular genetic study revealed mutations in 32.7% of patients (n=16, CI (21; 47)), mutations inMEN1being the most frequent (n=11). Pathogenic variants inCDC73were detected in 3patients,RET -in 2. Among the operated 39 patients, adenoma of the thyroid was detected in 84.6% of cases (n=33), hyperplasia in 7.7% (n=3), atypical adenoma in 5.1% (n=2), carcinoma in 5.1% of cases (n=2). The paper presents the peculiarities of the course and the results of molecular genetic study of pediatric PHPT. This sample is the largest among those published in the Russian Federation.

Load Tests of the Renal form of Primary Hyperparathyroidism in Children

After the introduction of calcium chloride (12.4 mg/kg) examined the levels in the blood at 20 and 120 minutes. The calcium content in a comparison group of children after 120 minutes returned to the baseline, in patients with a renal form of primary hyperparathyroidism its level was greater than 1.3 times. The test was sensitive and provided an opportunity to reveal hypercalcemia in 39 children who had been the normo[1]and hypocalcemia. The impaired renal function had no effect on the significance of the test. Osmotic hypertension develops under the influence of hypertonic sodium chloride. In osmotic diuresis, the excretions of calcium and sodium cations are interdependent. Increasing sodium in the loops of Henle and distal renal tubules stimulates sodium excretion, decreased reabsorption, which leads to increased levels of calcium in the daily urine in children with primary hyperparathyroidism. Test with sodium chloride made it possible to detect hypercalciuria in 12 (21.43%) children who have had normal levels of calcium in urine.

Features of the diagnosis of primary hyperparathyroidism in children

Background. Primary hyperparathyroidism (PHPT) is a rare pathology in pediatric and adolescent patients. Collection, analysis and generalization of the literature data and experience of the leading clinics allow to develop unified, statistically substantiated approaches to diagnostics and surgical treatment of this group of patients.Material and methods. The article presents a retrospective analysis of 17 cases of PHPT in children and adolescents aged from 6 to 18 years operated on in the department of Surgery of St.-Petersburg State Pediatric Medical University in the period from 1973 till 2021. Among those operated there were 10 girls and 7 boys, the M:F ratio was 1:1.4. The mean age of the patients was 12,9±0,71 years.Results and discussion. The main criteria of the disease diagnosis were elevated blood calcium and parathormone levels, excessive urinary calcium secretion. Manifest forms of the disease were diagnosed in 10 (58,8%) of 17 children. In 3 (17,6%) cases the parathyroid neoplasms were accompanied neither by clinical, nor laboratory manifestations of the disease and were regarded as incidentalomas. Another 4 (23.5%) patients had only laboratory changes (hypercalcemia and hyperparathyrinaemia) that manifested themselves preoperatively. These observations were referred to the asymptomatic form of PHPT.In 7 (41.2%) cases parathyroid adenomas were found and removed during surgeries for various thyroid diseases (thyroid cancer in 5 cases, diffuse toxic goiter in 1 case). An incidental finding of parathyroid adenoma was during prophylactic thyroidectomy for Sipple syndrome.A radioisotope method proved to be the most informative way to localize parathyroid tumors.Conclusion. The diagnosis of the disease in manifest sporadic cases does not differ from that in adults. Genetic study is indicated in the presence of a family history of multiple involvement of the parathyroid glands. Surgical treatment with removal of parathyroid tumor is the main method, which allows to achieve complete recovery.

Secondary Hyperparathyroidism in Children with Mucolipidosis Type II (I-Cell Disease): Irish Experience.

Mucolipidosis type II (ML II) is an autosomal recessive lysosomal targeting disorder that may present with features of hyperparathyroidism. The aim of this study was to describe in detail the clinical cases of ML II presenting to a tertiary referral centre with biochemical and/or radiological features of hyperparathyroidism. There were twenty-three children diagnosed with ML II in the Republic of Ireland from July 1998 to July 2021 inclusive (a 23-year period). The approximate incidence of ML II in the Republic of Ireland is, therefore, 1 per 64,000 live births. Medical records were available and were reviewed for 21 of the 23 children. Five of these had been identified as having biochemical and/or radiological features of hyperparathyroidism. Of these five, three children were born to Irish Traveller parents and two to non-Traveller Irish parents. All five children had radiological features of hyperparathyroidism (on skeletal survey), with evidence of antenatal fractures in three cases and an acute fracture in one. Four children had biochemical features of secondary hyperparathyroidism. Three children received treatment with high dose Vitamin D supplements and two who had antenatal/acute fractures were managed with minimal handling. We observed resolution of secondary hyperparathyroidism in all cases irrespective of treatment. Four of five children with ML II and hyperparathyroidism died as a result of cardiorespiratory failure at ages ranging from 10 months to 7 years. Biochemical and/or radiological evidence of hyperparathyroidism is commonly identified at presentation of ML II. Further studies are needed to establish the pathophysiology and optimal management of hyperparathyroidism in this cohort. Recognition of this association may improve diagnostic accuracy and management, facilitate family counseling and is also important for natural history data.

Ectopic parathyroid adenoma with acute pancreatitis in a pediatric patient

Abstract Introduction: Hypercalcemia due to primary hyperparathyroidism (PHPT) is uncommon in children with an incidence of 2 to 5 in 100,000 children and adolescents. Primary hyperparathyroidism in children has a bimodal distribution that reflects different etiologies in very young and older children. PHPT is typically caused by a single parathyroid adenoma. Ectopic parathyroid adenomas account for 20% to 22% of primary hyperparathyroidism cases and are rare in children but should be considered in cases that present with hypercalcemia. Patient concerns: We report the case of a 13-year-old girl with PHPT who presented with an unusual presentation of acute pancreatitis and persistent hypercalcemia. Diagnosis: Tc-99m-sestamibi scanning and chest computed tomography scan with contrast showed an anterior mediastinal lesion at the midline embedded into the thymus and was diagnosed as an ectopic anterior mediastinal parathyroid adenoma. Interventions: The genetic test results of whole-exome sequencing were negative. After thoracoscopic and thymus excision, the patient's serum calcium level immediately normalized. Outcomes: Intact parathyroid hormone, serum calcium, and alkaline phosphatase levels returned to normal within two months. Conclusion: PHPT may present with acute pancreatitis, generalized fatigue, and muscle pain as presented in this patient and need a high index of suspicion for early diagnosis. Delayed diagnosis of PHPT can cause end-organ damage; timely diagnosis is especially critical to preserving bone and renal function.

MO1020EFFICACY OF CALCITRIOL COMPARING TO ALFACALCIDOL FOR THE TREATMENT OF SECONDARY HYPERPARATHYROIDISM IN CHILDREN ON PERITONEAL DIALYSIS

Abstract Background and Aims Active vitamin D analogues are used routinely for the treatment of secondary hyperparathyroidism in children with end-stage renal disease (ESRD) on peritoneal dialysis (PD). Calcitriol is preferred active form of vitamin D, while data on efficacy of alfacalcidol in children on PD are limited. The aim of this study was to compare the efficacy of these two active vitamin D analogues for the treatment of secondary hyperparathyroidism in children on PD. Method This retrospective cohort study included data from 26 pediatric patients under 18 years of age with ESRD on PD who were treated at the National Research Center for Maternal and Child Health, Astana, Kazakhstan. Patients were allocated by initial treatment into two groups: oral alfacalcidol (n=21) and oral calcitriol (n=5). The first step of the study was to compare the efficacy of treatment between this two groups 3 and 6 months after. The second step was to compare the efficacy of calcitriol in subgroup of patients from alfacalcidol group who were switched to calcitriol later (n=12). The following characteristics were analyzed: parathyroid hormone (PTH), total plasma calcium, ionized plasma calcium, plasma phosphorus, initial dose of active vitamin D and corrected dose of vitamin D after 3 months. Independent t-test and repeated measures Anova were used for comparison between alfacalcidol and calcitriol groups. To compare data in subgroup between baseline and 3 months after conversion from alfacalcidol to calcitriol we used paired Student t-test. Results The mean age and baseline characteristics were no different between alfacalcidol and calcitriol groups (Table 1). The initial dose of alfacalcidol was 2.42±1.03 µg/week (0.157±0.097 µg/kg/week) and 2.09±0.74 µg/week (0.105±0.039 µg/kg/week) for calcitriol were not significantly different (P=0.5 and P=0.25 respectively). After 3 months of treatment there was statistically significant decrease of PTH level in calcitriol group 180±168 pmol/L comparing to alfacalcidol group 869±670 pmol/L (P=0.006). After 6 months, there was statistically significant decrease of PTH level in calcitriol group 261±259 pmol/L in contrast with alfacalcidol group 1080±716 pmol/L (P=0.047) and rise in total calcium in calcitriol group 2.46±0.17 mmol/L compared with alfacalcidol group 2.09±0.25mmol/L (P=0.035). The mean dose of calcitriol was also significantly lower as opposed to alfacalcidol dose: 0.04±0.039 µg/kg/week and 0.17±0.076 µg/kg/week respectively (P=0.002). The median age of children who were switched from alfacalcidol to calcitriol were significantly younger comparing with those who continued alfacalcidol (5±4.6 and 10±4.3 years of age respectively (P=0.022)). 3 months after conversion there was significant increase in ionized calcium level and decrease in PTH level comparing with baseline (Table 2). The dose of alfacalcidol before and dose of calcitriol after switch were differed (0.18±0.1 µg/kg/week and 0.13±0.05 µg/kg/week respectively, P=0.025) Conclusion Calcitriol showed superior efficacy for the treatment of secondary hyperparathyroidism in children on PD with the dose 4.25 times lower that of alfacalcidol. Alfacalcidol had limited efficacy for the treatment of secondary hyperparathyroidism in younger children when prescribed in recommended doses.

A Challenging Diagnosis of Primary Hyperparathyroidism in an Adolescent Female

Background: Hypercalcemia secondary to primary hyperparathyroidism (PHPT) is less common in children than adults. Single parathyroid adenoma is commonly the cause of primary hyperparathyroidism in children. Clinical Case: We present a 15-year-old female with one-week history of abdominal pain despite taking over the counter antacids. Her initial work up by her primary care provider revealed serum calcium of 11.8 mg/dL (9.0–11.5) and creatinine of 0.8 mg/dL (0.4–1.2). A week later, she presented to the emergency department with same complaint. In ED, she was found to have hypercalcemia (12.8 mg/dl) with elevated parathyroid hormone (PTH) at 78.5 pg/mL (15–65). Her random urine calcium creatinine ratio was high at 2.1. Her 25OHD was 25 ng/mL (30–100). She had negative urine pregnancy test but had trace ketones, leukocyte esterase, blood and bacteria. CBC and CMP were otherwise unremarkable. She continued to complain abdominal pain with nausea, decrease appetite, fatigue, and general muscle weakness. There was no known family history of calcium or metabolic bone disorders. Her vital signs and physical exam were normal. Subsequent labs showed mild improvement of calcium between (11–12.3 mg/dL), PTH between 54.5 and 77 pg/mL, normal thyroid function. Ionized calcium was mildly elevated 6.0 mg/dL (4.5–5.3) but her repeat 25OHD was low at18 ng/mL. Serum phosphorus levels were relatively normal with lowest level of 2.5 mg/dL (2.7–4.5). Gliadin Deamidated IgA was detectable 15 U/mL (< 15.0 U/). Ultrasound of abdomen was significant for nonspecific mild hepatomegaly; kidneys were normal in size and appearance. Ultrasound of thyroid was significant for probably intrathyroid parathyroid, measuring 6 x 8 x 8 mm. Tc-Sestamibi scan did not confirm a parathyroid adenoma. Genetic testing for MEN-1 was negative. FHH- related genes (i.e. CASR) was positive for p.R990G variant resulting in a mild gain of function of the calcium-sensing receptor. Although previous Tc-Sestamibi scan was unremarkable, an over read of it raised a concern for questionable uptake in the left superior lobe. SPEC-CT demonstrated possible abnormal parathyroid tissue in the upper pole of the left thyroid. FNA of the left thyroid nodule confirmed likely intrathyroidal parathyroid adenoma. Subsequent follow up and treatment, including parathyroidectomy, was done by another institution. She underwent a left parathyroidectomy with normalization of serum calcium and PTH levels post operatively (10.1 mg/dl and 8 pg/mL, respectively) and has complete resolution of her previous abdominal and gastrointestinal symptoms. Conclusion: PHPT is uncommon in children and adolescents and is typically associated with a single parathyroid adenoma. High index of suspicion is key for early diagnosis of PHPT despite a negative Tc-Sestamibi initially.

Cinacalcet for Severe Secondary Hyperparathyroidism in Children with End-stage Kidney Disease.

Advanced chronic kidney disease with mineral and bone disorder have a significant obstacles to control serum bone profile [serum intact parathyroid hormone (iPTH), calcium and phosphorus] which subsequently have major effect on optimal bone strength, final adult height, and cardiovascular health. A retrospective, observational study, including a total of 36 children with end-stage kidney disease (ESKD). Fourteen children who were prescribed cinacalcet had been compared with the remaining 22 children who were managed with standard care. We report the efficacy and safety of cinacalcet for treatment of refractory secondary hyperparathyroidism (SHPT) in children with ESKD. After 6 months of cinacalcet treatment, the mean level of iPTH serum level decreased by 56% from 202 pmol/L [95% confidence interval (CI): 150-253] to 88 pmol/L (95% CI: 41-136), compared to the change observed in the control group (P <0.001). None of our patients reported serious adverse effects or developed hypocalcemia. Cinacalcet could be an effective and safe alternative to treat severe SHPT in children with ESKD. Further long-term and large-scale studies are necessary to confirm its safety and efficacy.

Vitamin D revisited: Individualized Vitamin D normal values according to PTH levels; incidence and treatment of Normocalcemic Hyperparathyroidism in children

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Parathyroid carcinoma in a 13-year-old girl with a long-term survival

BackgroundParathyroid carcinoma as a cause of primary hyperparathyroidism in children is extremely rare. We report a case of parathyroid carcinoma which occurred in a 13-year-old girl who survived for more than 45 years after the first operation.Case presentationA woman was admitted to our hospital for the treatment of recurrent parathyroid carcinoma in the neck. She had been diagnosed with primary hyperparathyroidism from a fibula fracture and underwent parathyroidectomy at 13 years old. She had no family history of multiple endocrine neoplasia or jaw tumor syndrome. Genetic testing was not performed, and the histopathological diagnosis of the tumor had been parathyroid adenoma at the time. At 22 years old, she showed hypercalcemia after a femur fracture. Pulmonary metastases of parathyroid carcinoma in the bilateral lungs were found and surgically removed. Regarding the clinical course, her diagnosis was corrected from parathyroid adenoma to parathyroid carcinoma. At 33 years old, re-resection of the lung metastases was performed. For 10 years, her serum calcium level stayed within the normal range. However, her serum calcium level and intact parathyroid hormone eventually began to increase. Two masses suspected of being parathyroid carcinoma recurrence were found in the neck when she was 57 years old. En bloc resection was performed. Pathologically, the tumors were diagnosed as parathyroid adenoma. The serum calcium level and intact parathyroid hormone did not decrease after the operation. A 99mTc-methoxy-isobutyl-isonitrile- and 18F-fluorodeoxyglucose-negative, 11C-methionine-positive tumor was detected at the right side of the trachea in the neck. The tumor was removed, along with the thyroid, muscle, and trachea that were involved. The pathological diagnosis was parathyroid carcinoma recurrence. The serum calcium level and intact parathyroid hormone decreased temporarily but had increased again 8 months later. Methionine-positive tumors were found at the right side of the trachea and suspected of being a recurrence. Denosumab reduced her serum calcium level, and radiation successfully suppressed the growth of the recurrent tumors.ConclusionWe have reported a rare case of parathyroid carcinoma in a child who has survived for over 40 years. Positron emission tomography of 11C-methionine was useful for detecting local recurrence. This patient’s long-term survival has been attributed to multimodality treatment including repeated surgery, medication, and radiation.

Single Gland, Ectopic Location: Adenomas are Common Causes of Primary Hyperparathyroidism in Children and Adolescents.

Primary hyperparathyroidism (PHPT) in children and adolescents is uncommon. Data-driven guidelines for management in pediatric patients are limited. We performed a retrospective cohort analysis of all patients (1997-2017) with PHPT ≤ 21years of age who underwent parathyroidectomy at three institutions. Clinical and demographic variables were analyzed. Primary operative outcome was cure (normocalcemia > 6months after surgery); secondary outcome was operative success (intraoperative parathyroid hormone decrease of ≥ 50%). We identified 86 patients with a median age of 17years (IQR: 14, 19); 64% (n = 55) were female. The mean preoperative serum calcium was 11.7mg/dL, median parathyroid hormone (PTH) was 110pg/mL, and median urine calcium was 4.1mg/kg/24h. Preoperatively, sestamibi scan localized in 41/71 patients (58%); neck ultrasound localized in 19/44 (43%). The most common pathology at surgery was a single ectopic parathyroid adenoma in 71% (n = 61). A high incidence of ectopic adenomas (25%, n = 22) was observed, most commonly intrathymic (n = 13), followed by tracheoesophageal groove (n = 5), carotid sheath (n = 2), and intrathyroidal (n = 2). Of 56 patients with retrievable data > 6months postoperatively, cure was achieved in 55 of 56 patients (98%). One patient who presented to us with parathyromatosis require subsequent reoperation. In this multi-institutional series of PHPT in children and adolescents, the majority were sporadic PHPT and were due to a single adenoma. We observed a high incidence of ectopic parathyroid adenomas, most commonly intrathymic. Given the high risk for ectopic adenoma in pediatric patients, parathyroid surgery in children and adolescents should be performed by experienced surgeons.

MON-541 Successful Treatment of Normocalcemic Hyperparathyroidism in Children

Childhood and adolescence are the critical periods for the establishment of lifelong bone health. Normocalcemic primary hyperparathyroidism (NPHPT) has been recognized as a variant of primary hyperparathyroidism (PHPT) 15 years ago and it is characterized by elevated PTH level with persistently normal concentrations of albumin-adjusted total and ionized calcium, in the absence of secondary causes of hyperparathyroidism and it is related to increased risk in development of osteopenia or osteoporosis as well as development of parathyroid adenoma and hypercalcemia-hypercalciuria with renal consequences. In order to identify biochemical disorders of PTH in normocalcemic children we performed in all patients that visited our pediatric endocrine unit for two years (1-Nov 2016 until 31-Oct 2018), a complete calcium metabolism evaluation (Ca, P, ALP, 25OHD, intact PTH). A total of 3060 patients - excluding those that consulted for vitamin D deficiency, Ca metabolism abnormalities or known renal pathology (i.e. Bartter syndrome) - were included. We identified 157 patients (5.1%) with hyperparathyroidism (PTH > 45 pg/ml, Horm Res Paediatr 2015;84:124-129) and normal total serum calcium levels; adequate data on laboratory results and follow up were available in 114 patients; 67 (58.7%) of them were vitamin D replete (25OHVitD >30 ng/ml, group 1) and forty-seven were vitamin D deficient (41.3 %) (25OHVitD <30 ng/ml, group 2). All patients were treated with cholecalciferol (8000-16000 IU daily). All patients in groups 1 and 2 who did not normalize their PTH (< 45 pg/ml) within 6 months of monotherapy with vitamin D, received additional calcium supplementation (1000 mg/day). Evaluation of calcium metabolism (Ca, P, ALP, 25OHD, 1,25OHD, PTH) was performed every 3 months. In 6 patients (4 from group 1 and 2 from group 2) elevated PTH did not respond to 6 months of combined cholecalciferol/calcium therapy. These patients were switched to the non-calcemic synthetic 1-25(OH)2-vitamin D analogue, paricalcitol, at the dose of 2 mcg x 1-3/day. Evaluation of calcium metabolism (Ca, P, ALP, 25OHD, 1,25OHD, PTH, urine Ca/Cr) was performed every 3 months. Parathormone levels normalized in 5 patients by 3 months of treatment and in one by 10 months of treatment with calcium in serum and urine being within normal range for age during treatment in all patients. We propose that all normocalcemic children that are checked for vitamin D deficiency undergo concomitant measurement of PTH levels. Subclinical hyperparathyroidism (PTH > 45 pg/ml) should be treated with cholecalciferol (8000-16000 IU/day) and calcium supplementation (1000 mg/day) and if unresponsive after a minimum of 3 months, with paricalcitol 2-6 mcg/day in order to normalize PTH and protect their bone and general health. Further studies are needed to standardize this approach.

Risk factors for persistent hyperparathyroidism in children with stable renal function after kidney transplantation.

This study aimed to investigate the risk factors for PHPT in children with stable renal function who received KT. We retrospectively analyzed the clinical findings and laboratory results of patients who underwent KT below 19years of age, between 1996 and 2016 at our hospital. Patients were followed up for more than 1year after KT. We calculated the mean±standard deviation or median [minimum - maximum] for each parameter. We included a total of 46 patients (male:female=26:20). Twelve patients (26.1%) were included in the PHPT group, and 34 (73.9%) were in the nPTH group. The dialysis duration was 57.1±49.9, 44 [0-145]months in the PHPT group and 23.5±25.8, 15 [1-121]months in the nPTH group (P=.040). The post-KT total CO2 level was significantly higher in the PHPT group (P=.022). The pre- (P=.021) and post-KT (P=.005) and 3-month average (P=.018) iPTH levels were also significantly higher in PHPT group. The height z-score showed a negative correlation, and the pre-KT, 3-month average phosphorus and alkaline phosphate levels showed positive correlations with iPTH levels, at 1year after KT. Patients who undergo prolonged durations of dialysis, have increased iPTH levels before and after KT, and have low bicarbonate levels after KT are at risk of PHPT and should be monitored carefully.

Primary hyperparathyroidism in childhood

Primary hyperparathyroidism is a disease caused by excessive production of parathyroid hormone by the parathyroid glands. Most often, sporadic cases of the disease occur. In the family form of hyperparathyroidism, as well as in the syndrome of multiple endocrine neoplasia, the disease is caused by genetic changes. In manifest forms of the disease, hyperparathyroidism leads to destruction and deformation of bone tissue, osteoporosis, urolithiasis, cholelithiasis, pancreatitis, peptic ulcer disease, and disorders of mineral and electrolyte metabolism. Bone manifestations of the disease can lead to disability of the child. The article presents the experience of surgical treatment of 15 children, patients with primary hyperparathyroidism, aged from 6 to 18 years old, 10 of them girls and 5 boys operated in the period from 1973 to 2017. The main methods of diagnosis of the disease, the difficulties of timely diagnostic are discussed. The features of laboratory indicators of clinical manifestations of the disease are clearly shown. The clinical manifestations of primary hyperparathyroidism in children differed from adults more heavily damage of bone tissue with the development of gross deformities of the limbs. In 8 (57.3%) of 15 children, manifest forms of the disease were diagnosed. In 7 (46.7%) children, parathyroid adenomas were detected and removed during surgery for various thyroid diseases. In two patients, parathyroid adenomas were removed during operations performed for a genetically confirmed syndrome of multiple endocrine neoplasia of type IIa. In this study, the features of adenoma localization of the parathyroid gland, the possibility of surgical treatment and the dynamics of clinical manifestations after surgery are discussed. Proper treatment allowed to achieve full recovery.

Intraperitoneal Calcitriol for Treatment of Severe Hyperparathyroidism in Children with Chronic Kidney Disease: A Therapy Forgotten.

Active Vitamin D sterols such as calcitriol and alfacalcidol are quite effective in the treatment of mineral bone disease secondary to chronic kidney disease. However, some children on peritoneal dialysis (PD) are resistant to oral formulations of active Vitamin D, and use of an intravenous formulation in such patients is inconvenient. In these children, intraperitoneal (IP) calcitriol has been shown to be effective in the treatment of secondary hyperparathyroidism. However, its use has declined. We report 2 children, aged 1 and 9.5 years, on chronic cycler PD with severe secondary hyperparathyroidism refractory to oral active Vitamin D who were successfully treated with IP calcitriol for a period of 12 and 4 months, respectively. We also discuss the published literature on the efficacy of IP calcitriol for treatment of secondary hyperparathyroidism and specific considerations for its use in PD patients.

Primary hyperparathyroidism in children and adolescents

Searchable abstracts of presentations at key conferences on calcified tissues ISSN 2052-1219 (online)