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Neonatal severe primary hyperparathyroidism (NSPHPT) is an extremely rare autosomal recessive disorder, requiring a high index of suspicion. Infants affected with this disorder present with severe life-threatening hypercalcemia early in life, requiring adequate preoperative medical management followed by surgery. We report seven newborns with neonatal severe primary hyperparathyroidism (NSPHPT) who were managed over 12 twelve years. Demography, clinical presentation, treatment, and follow-up data were retrospectively studied with descriptive analysis to highlight the utility of long-term medical management, surgery, and genetic testing reported in the literature. The statistical analysis used was descriptive analysis. We had six baby boys and one baby girl infant with a mean age of diagnosis at 50.42 days, calcium 23.32 ± 6.9 mg/dl, and parathormone (PTH) 1072 ± 1440 pg/ml. All seven infants presented with failure to thrive, hypotonia, and respiratory distress. All infants were treated medically followed by total parathyroidectomy plus transcervical thymectomy, with an additional hemithyroidectomy in one of them. Imaging was negative in all seven cases. Six babies became hypocalcemic while the fourth infant had a drop in PTH and is on tab. cinacalcet 30 mg/day. CASR mutation was positive in six infants. Our article on NSPHPT reiterates the importance of early diagnosis and referral for definitive management. Follow-up of these cases highlights the importance of complete surgery at presentation and lifelong compliance for calcium and vitamin D supplementation. The role of preoperative medical management also is of utmost importance, to make the surgery successful. Parental counseling for neonatal surgery poses a real challenge. Surgery offers a cure for this unusual lethal hypercalcemia, presenting with crisis. We want to reemphasize the need for early intervention in the form of definitive surgery—total parathyroidectomy and thymectomy with no autotransplantation. Thymectomy ensures the removal of all parathyroids though our series had no evidence of ectopia in the thymus. [ABSTRACT FROM AUTHOR]

Background: Bone disease in posttransplantation of the kidney characterized by changes in bone quality and density as well as mineral metabolism is a major cause of morbidity in kidney transplant recipients, with a significantly higher risk of fractures as well as increased health-care costs. Several studies have shown that Vitamin D therapy prevents bone loss in nontransplant patients with corticosteroid-induced osteoporosis. However, because of the concern about the risk of hypercalcemia, the use of Vitamin D in this setting has been limited. In this open-labeled, randomized, and controlled prospective intervention trial, we compared the effect of calcium alone versus calcium plus intermittent calcitriol therapy on bone loss after renal transplant (RT). We reasoned that intermittent calcitriol might minimize the risk of hypercalcemia. Materials and Methods: This is an open-label prospective randomized control trial study, conducted in the Department of Nephrology, IPGME and R and SSKM Hospital. A total of 41 patients who were transplanted at the Department of Nephrology and attending outpatient department services constituted the study population from February 2017 to May 2018. Each patient was followed for at least 6 months. Patients were randomly assigned to two groups through GraphPad with 21 patients receiving calcitriol and 20 patients receiving calcium only. Results: Most of the patients were relatively young around 30 years in both groups (29.10 ± 7.06 and 31.95 ± 8.24), and were male. A statistically significant difference was found in serum creatinine in the 3rd month between calcium and calcitriol groups. However, no significant difference was found in the 6th month between the groups. There were, as expected, statistically significant falls in intact parathyroid hormone (PTH) in the 3rd and 6th months posttransplant in both calcium and calcitriol groups. Statistically significant improvement of Vitamin D level was seen in both the groups posttransplant in all time periods as expected. The decline was statistically significant in serum phosphorus level at the end of the 6th month between the groups (P < 0.01). The incidence of hypercalcemia is more in the calcitriol group. A statistically significant change in bone mineral density (BMD) in the lumbar spine was seen in the 3rd and 6th months (P < 0.001). However, the change in BMD in radius was not statistically significant between the two groups in the 3rd and 6th months (P > 0.01). A statistically significant change in BMD in the femur and trochanteric region was also seen between the groups in the 3rd and 6th months (P < 0.001). Conclusion: The study finds a safe treatment for bone health with low-dose calcium supplements and intermittent calcitriol after RT which lowers PTH levels more rapidly. It also shows evidence of prevention of bone loss at the neck femur, lumbar spine, and trochanteric region. Future studies on these patients are needed to assess whether the treatment is effective in decreasing the fracture rate. [ABSTRACT FROM AUTHOR]

Background: We studied the incidence of magnesium (Mg) disturbances in patients admitted to a multidisciplinary intensive care unit (ICU) and correlated serum magnesium levels with clinical outcomes. Materials and methods: The study was conducted on 280 critically ill patients aged above 18 years and admitted to the ICU. Serum magnesium levels at admission were correlated with mortality, need for and duration of mechanical ventilation, duration of ICU stay, presence of comorbid conditions, and electrolyte disturbances. Result: There was a high incidence of Mg disturbances at admission among patients admitted to the ICU. The incidence of hypomagnesemia and hypermagnesemia was 40.9 and 13.9% respectively. The mean Mg level among patients who expired was 1.55 ± 0.68 mg/dL, and the association with outcome was found to be statistically significant (p = 0.001). Hypomagnesemia (HypoMg) was associated with significantly higher mortality (51.3%) as compared to normomagnesemia (NormoMg) (29.3%) and hypermagnesemia (HyperMg) (23.1%) (HypoMg vs NormoMg, HypoMg vs HyperMg, p = 0.001, 0.002 respectively). The need for mechanical ventilation was significantly higher in hypomagnesemic as compared to hypermagnesemia patients (p = 0.012). The association of baseline APACHE II and SOFA scores with serum Mg levels was statistically significant (p = 0.001 and 0.002 respectively). The incidence of gastrointestinal disorders was significantly higher among hypomagnesemia patients (HypoMg vs NormoMg, p = 0.023), while chronic kidney disease was significantly higher in hypermagnesemic patients (HypoMg vs HyperMg, p = 0.0009, NormoMg vs HyperMg, p = 0.0004). On comparing the incidence of electrolyte disorders between HypoMg, NormoMg, and HyperMg groups, it was found that hypokalemia and hypocalcemia (p = 0.0003 and 0.039 respectively) were associated with hypomagnesemia and hyperkalemia and hypercalcemia (p = 0.001 and 0.005 respectively) were associated with hypermagnesemia. Conclusion: Our study highlights the role of Mg monitoring in critically ill patients admitted to the ICU and its value for a favorable outcome. We found that hypomagnesemia was significantly associated with adverse outcomes and higher mortality in critically ill patients. Intensivists should maintain a high index of suspicion for Mg disturbances and evaluate patients appropriately. [ABSTRACT FROM AUTHOR]

Background: The etiology of hypercalcemia varies according to the clinical setting. Hitherto, data on the prevalence and profile of hypercalcemia in hospitalized Asian–Indian patients are limited. Hence, we conducted a prospective observational study to determine the prevalence and etiological profile of hypercalcemia in hospitalized Asian–Indian patients and its association with 6‑month mortality. Materials and Methods: We conducted a prospective observational study wherein all the patients (aged >12 years) admitted to the general medicine wards of a tertiary care hospital in North India between January 1, 2016, and June 30, 2017, were screened. Finally, patients with sustained hypercalcemia (defined as corrected serum total calcium ≥10.4 mg/dl documented twice at least 24 h apart) were included in this study. These patients were followed up throughout the hospital course and thereafter till 6 months from the date of discharge. Results: Out of 9902 patients, 150 patients had sustained hypercalcemia (prevalence 1.5%). The most common cause of hypercalcemia was malignancy (41.3%), followed by primary hyperparathyroidism (PHPT, 32.7%). Vitamin D intoxication was responsible for hypercalcemia in 8.7% of patients; 2.7% of patients had hypercalcemia of advanced chronic liver disease. Nevertheless, a definite etiology could not be identified in 7.3% of the patients with hypercalcemia. At the end of 6 months of follow‑up, the cumulative mortality rate was 28%. Underlying malignancy and higher calcium levels were the significant determinants of mortality. Conclusions: The prevalence of hypercalcemia in Asian–Indian patients admitted to a tertiary care hospital was 1.5%. The most common etiology was malignancy, followed by PHPT. [ABSTRACT FROM AUTHOR]

Context: Neonatal severe primary hyperparathyroidism (NSPHPT) is an extremely rare autosomal recessive disorder, requiring a high index of suspicion. Infants affected with this disorder present with severe life-threatening hypercalcemia early in life, requiring adequate preoperative medical management followed by surgery. Aims: We report four newborns with NSPHPT who were managed over 10 years. Subjects and Methods: Demography, clinical presentation, treatment, and follow-up data were retrospectively studied with descriptive analysis to highlight the utility of long-term medical management, surgery, and genetic testing reported in the literature. Statistical Analysis Used: Descriptive Analysis. Results: We had three males and one female infant with a mean age of diagnosis at 28.7 days, calcium 29.2+/-2.8 mg/dL, and parathormone (PTH) 1963+/-270.4 pg/mL. All four infants presented with failure to thrive, hypotonia, and respiratory distress. All infants were treated medically followed by total parathyroidectomy plus transcervical thymectomy, with an additional hemithyroidectomy in one of them. Imaging was negative in all four cases. Three babies became hypocalcemic while the fourth infant had a drop in PTH and is on the tab. cinacalcet 30 mg/day. CaSR mutation was positive in three infants. Conclusions: Diagnosing NSPHPT needs expert clinical acumen. It requires emergency medical management to control calcium levels. The crisis may present later, necessitating parathyroidectomy in these cases once the child is fit for surgery. Surgery offers a cure for this unusual lethal hypercalcemia while the role of cinacalcet needs a special mention. Sound knowledge in endocrinology with parathyroid embryology and morphology is of paramount importance. Our case series might add a few insights into managing this unusual genetic disorder. [ABSTRACT FROM AUTHOR]

Among the multitudinous etiologies of pancreatitis, primary hyperparathyroidism (PHPT) is rarely associated with pancreatitis. However, the cause and effect relationship between the two still evokes controversy. We aimed to study and characterize the nature of pancreatic disease in PHPT. A retrospective single-center study was carried out in North India over a period of 1 year (June 2015 to May 2016). All patients with pancreatitis were included. In patients with high calcium levels, Intact Parathyroid Hormone (iPTH) by Radioimmunoassay (RIA) and an Technetium 99m Sestamibi scintigraphy (MIBI-Tc-99m) scan were performed. During the study period, 70 patients with pancreatitis were admitted to our hospital (53 with acute pancreatitis [AP] and 17 with chronic pancreatitis [CP]). Of them, 5 patients (9.4%) were detected to have PHPT. The mean age of patients was 30.4 years (20–49 years) with 3 males and 2 females, including 1 pregnant female (29th week of gestation). Contrast enhanced computed tomography (CECT) abdomen was performed in 4 cases (excluding 1 pregnant patient) with mean Computed tomography severity index (CTSI) of 4.7 (2–8). Four patients were detected to have increased uptake in one of the parathyroid glands, and the fifth patient had an ectopic parathyroid uptake in the mediastinum. All the resected samples were identified as parathyroid adenoma on histology. The patients were followed up for 1 year with no reported recurrence of symptoms. The data suggest an association between pancreatitis (both acute and chronic) and hypercalcemia due to PHPT. A high calcium value during AP or CP should always draw suspicion and warrants corresponding investigations in search of endocrine or malignant cause. [ABSTRACT FROM AUTHOR]

Introduction: Primary hyperparathyroidism is one of the most common endocrine disorders requiring surgical parathyroidectomy for its definitive treatment. Surgical exploration is traditionally performed through conventional open neck approach. A wide range of minimal access and minimally invasive endoscopic techniques (gas less and with gas) have been attempted in the past two decades. In this context, we evaluated the feasibility and safety of an innovative transoral endoscopic parathyroidectomy (EP) technique, which represents a paradigm shift in transluminal endocrine surgery. Materials and Methods: This is a prospective study conducted at a tertiary care Endocrine Surgery Department in South India between May 2016 and August 2017. We employed a novel transoral, lower vestibular route for EP. All the clinical, investigative, operative, pathological and post-operative data were collected from our prospectively filled database. Statistical analysis was performed with SPSS 20.0 version. Operative Technique: Under inhalational general anaesthesia, access to the neck was obtained with 3 ports (central frenulotomy and two lateral port sites), dissected in subplatysmal plane and insufflated with 6 mm Hg CO2 for working space. Rest of surgical steps is similar to conventional open parathyroidectomy. Results: Out of the 38 hyperparathyroidism cases operated during the study, 12 (32%) were operated by this technique. Mean operative time was 112 ± 15 min (95-160). The post-operative course was uneventful with no major morbidity, hypocalcemia or recurrent laryngeal nerve palsy. Cure and diagnosis were confirmed by >50% fall in intraoperative parathyroid hormone levels and histopathology (all were benign solitary adenomas). Conclusions: Through this study, we opine that this novel transoral vestibular route parathyroidectomy is a feasibly applicable approach for primary sporadic hyperparathyroidism, especially with solitary benign adenomas. [ABSTRACT FROM AUTHOR]

Background: Urinary stone constitutes one of the commonest diseases in our country. In India, approximately 5-7 million population suffer from stone disease and at least 7-10 per 1000 of Indian population needs hospitalization due to a kidney stone. It has been proposed that comorbidity with essential hypertension, overweight and Type 2 Diabetes Mellitus predispose to kidney stone disease. Few lithogenic risk factors like urinary calcium, oxalate and uric acid excretion, are known to be influenced by the rich animal protein diet, which in turn is frequently related to overweight. In a female patient with basal metabolic index (BMI), 40% higher than standard, there was an (89%) increase in the prevalence of kidney stone. The aim of the study: To diagnosis the different biochemical composition in women who were diagnosed with urolithiasis. Materials and methods: This observational study was done in 2018 at, Department of Urogynecology, Institute of Social Obstetrics, Government Kasturba Gandhi Hospital, Chennai. Chemical methods were used to perform stone analysis. Calculi were thoroughly washed with tap water to remove attached debris. Then they were rinsed with deionized water and air dried for two weeks in a plastic container. Once the calculi were dry, they were weighed and then grounded to a fine powder using mortar and pestle. These powdered calculi were used for qualitative and quantitative analysis. Results: According to the results, all calculi had oxalic acid and uric acid which were the commonest components in calculi. Calcium and phosphorous were the next common components followed by magnesium. Ammonium ion was detected in 59.5% renal calculi. None of the calculi contained carbonate or cysteine. Conclusion: Factors like diet and lifestyle plays an important role in the changing epidemiology of kidney stone. Changes in two of the most important environmental factors, diet, and climate, are the significant impact on these trends. Patients who had raised serum calcium and serum uric acid level had larger and multiple calculi bilaterally. There is strong evidence that diminished fluid and dietary calcium consumption is a risk factor and an increase in animal protein intake has an equal impact on kidney stone risk. [ABSTRACT FROM AUTHOR]

Vitamin D deficiency state is endemic to the Kashmir valley of the Indian subcontinent. Physicians often treat patients with high doses of vitamin D for various ailments and on occasion the prescribed doses far exceed the requirements of the patients. Ten cases of hypercalcemia due to vitamin D intoxication are presented with features of vomiting, polyuria, polydipsia, encephalopathy and renal dysfunction. All the patients had demonstrable hypercalcemia and vitamin D levels were high in nine of the 10 cases. The patients had received high doses of vitamin D and no other cause of hypercalcemia was identified. Treatment of hypercalcemia resulted in clinical recovery in nine cases. We conclude that hypervitaminosis D must be considered in the differential diagnosis of patients with hypercalcemia in endemically vitamin D deficient areas. A careful history and appropriate biochemical investigation will unravel the diagnosis in most of the cases. [ABSTRACT FROM AUTHOR]

Background: Primary hyperparathyroidism (PHPT) has a variable clinical presentation and symptomatic PHPT is still the predominant form of the disease in India. Data from western India is lacking. Aim: To present the clinical profile of PHPT from western India. Settings and Design: This retrospective study was conducted at a tertiary care referral center. Materials and Methods: We analyzed the clinical presentation, biochemical, radiological features, and operative findings in adult patients with PHPT (1986-2008) and compared with our published data of children and adolescent patients with PHPT. Statistical Analysis: was done with SPSS 16 software. Results: Seventy-nine patients (F: M-2:1) with age ranging from 21 to 55 years (mean 33.5±8.82) were analyzed. Skeletal manifestations (75.5%), renal calculi (40.5%) and proximal muscle weakness (45.5%) were the most common symptoms of presentation with mean duration of symptoms being 33.70 (median: 24, range 1-120) months. Biochemical features included hypercalcemia (total corrected calcium 12.55±1.77 mg/dl), low inorganic phosphorus (1.81±0.682 mg/dl), elevated total alkaline phosphatase (mean: 762.2; median: 559; range: 50-4930IU/L) and high parathyroid hormone (PTH) (mean±SD: 866.61±799.15; median: 639.5; range: 52-3820 pg/ml). Preoperative localization was achieved in 74 patients and single adenoma was found during surgery in 72 patients. Hungry bone disease was seen in 30.3% and transient hypoparathyroidism developed in 62% patients. In comparison to PHPT in children there were no significant differences with regard to clinical, laboratory and radiological features. Conclusions: PHPT in western India is symptomatic disorder with skeletal and renal mani-festations at a much younger age. Clinical profile of PHPT in children is similar to that of adults. [ABSTRACT FROM AUTHOR]

The article presents information on a study which analyzes the characteristics of primary hyperparathyroidism in adolescents in India. A brief overview is given on the incidence of primary hyperparathyroidism (PHPT) as the third most common endocrine disorder in the country. It summarizes the presenting complaints and associated conditions which include pathological fracture, bone pain, bone deformity, and muscle weakness. It describes the demography and symptoms of PHPT in different studies in children and adolescent.