Raiz Ahmad Misgar

Primary hyperparathyroidism presenting as recurrent acute pancreatitis: A case report and review of literature.

The association between pancreatitis and primary hyperparathyroidism (PHPT) is controversial. We report a 32-year-old man who presented with recurrent episodes of acute pancreatitis. Primary hyperparathyroidism was diagnosed after the fourth episode of pancreatitis. He had no additional risk factors for pancreatitis. Eighteen months after successful parathyroid surgery, there has been no recurrence of abdominal pain and his serum calcium is within the normal range.

Effect of Vitamin D supplementation on glycemic parameters and progression of prediabetes to diabetes: A 1-year, open-label randomized study

Background: Whether Vitamin D supplementation in prediabetes subjects prevents the development of diabetes is a matter of debate, and the results are inconsistent. This open-label, randomized study in subjects with prediabetes evaluated the effect of 12 months of Vitamin D supplementation on glycemic parameters and progression of prediabetes to diabetes in an ethnically homogeneous Kashmiri population. Materials and Methods: A total of 147 subjects were diagnosed as prediabetes out of which 137 subjects were randomized to receive in addition to standard lifestyle measures, either Vitamin D 60,000 IU weekly for 4 weeks and then 60,000 IU monthly (n = 69) or no Vitamin D (n = 68). Fasting plasma glucose (FPG), 2-h plasma glucose and A1C levels were estimated at 0, 6 and 12 months. Changes in FPG, 2-h plasma glucose, A1C level and the proportion of subjects developing diabetes were assessed among 129 subjects. Results: At 12 months, A1C levels were significantly lesser (5.7% ± 0.4%) in the Vitamin D supplemented group when compared with non-Vitamin D supplemented (6.0% ± 0.3%). Similarly, FPG (97 ± 7) and 2-h plasma glucose (132 ± 16) were significantly less in Vitamin D supplemented group as compared with non-Vitamin D supplemented group (FPG = 116 ± 6 and 2-h plasma glucose = 157 ± 25) at 12 months. Nine out of 65 in non-Vitamin D supplemented and seven out of 64 in the Vitamin D supplemented group developed diabetes. Conclusions: Vitamin D supplementation in prediabetes subjects significantly lowered FPG, 2-h plasma glucose and A1C levels.

Alteration of Lipid Parameters in Patients With Subclinical Hypothyroidism

Background: Overt hypothyroidism is associated with abnormalities of lipid metabolism, but conflicting results regarding the degree of lipid changes in subclinical hypothyroidism (SCH) exist. Objectives: The aim of this study was to assess differences in lipid profile parameters between subjects with and without SCH in a north Indian population. Patients and Methods: Serum lipid parameters of 70 patients with subclinical hypothyroidism and 100 age and sex matched euthyroid controls were evaluated in a cross-sectional study. Results: Mean serum total cholesterol (TC), triglycerides (TG) and very low-density cholesterol (VLDL) were significantly higher in patients with SCH than controls (P < 0.05). Mean TC, TG and low-density cholesterol (LDL) concentrations were higher in patients with serum thyroid stimulating hormone (TSH) greater than 10 mU/L than those with serum TSH equal to or less than 10 mU/L, but this difference was not statistically significant. No association was found between serum high-density cholesterol (HDL-C) concentration and serum TSH level. Conclusions: High TC, TG and VLDL were observed in our patients with SCH.

Emphysematous pyelonephritis: A 10-year experience with 26 cases

Background: Emphysematous pyelonephritis (EPN) is a necrotizing infection which results in gas within the renal parenchyma, collecting system, or perinephric tissue. A majority of cases occur in patients with diabetes mellitus (DM). In EPN, early aggressive medical treatment may avoid nephrectomy. Aims: The aim of this study was to analyze the characteristics of patients with EPN with respect to patient demographics, clinical presentation, diagnostic investigations, microbiological findings, treatment modality and outcome, and the influence of prognostic factors on the outcome. Materials and Methods: We reviewed the hospital records of 26 patients with EPN for clinical, laboratory, radiological, and microbiological findings, treatments given, and outcome. The severity of EPN was graded as per the Huang classification. We applied the reported prognostic factors to our patients to find out whether these factors correlated with failure of conservative treatment. Results: All the study subjects had DM and all but two of them were females. The majority of our patients (61.5%) had extensive EPN (class 3 or 4) and majority (76.9%) had two or more bad prognostic factors. Escherichia coli was the most common causative organism involved in 50% of our cases. Twenty-three (88.5%) of our patients responded to conservative treatment, two required nephrectomy, and one expired on conservative treatment. Conclusions: In this series of patients with EPN, all had DM, nearly all were women, and E. coli was the most frequently isolated pathogen. Nearly a third of our patients had bilateral disease. Despite the presence of two or more bad prognostic factors and extensive EPN (class 3 or 4) in a majority of our patients, conservative treatment afforded a striking success rate of 88.5%. We recommend early aggressive medical treatment and suggest that nephrectomy should be considered only if patients deteriorate or do not improve on conservative treatment.

Allgrove (Triple A) Syndrome: A Case Report from the Kashmir Valley.

Allgrove (Triple A) syndrome is a rare autosomal recessive disorder characterized by cardinal features of adrenal insufficiency due to adrenocorticotropic hormone (ACTH) resistance, achalasia, and alacrimia. It is frequently associated with neurological manifestations like polyneuropathy. Since its first description by Allgrove in 1978, approximately 100 cases have been reported in the literature. Here we report an 18-year-old boy diagnosed as having Allgrove syndrome, with ACTH resistant adrenal insufficiency, achalasia, alacrimia, and severe motor polyneuropathy. Alacrimia was the earliest feature evident at the age of 8 years. He presented with achalasia and adrenal insufficiency at 12 and 18 years respectively and developed neurological symptoms in the form of severe muscle wasting at the age of 15 years. Patients with Allgrove syndrome usually manifest adrenal insufficiency and achalasia during first decade of life. Our patient manifested adrenal insufficiency and achalasia in the second decade and manifested neurological dysfunction before adrenal dysfunction.

Successful Medical Management of Severe Bilateral Emphysematous Pyelonephritis: Case Studies

Infections remain a major cause of morbidity and mortality in patients with diabetes in developing countries (1). Many infections are more common in patients with diabetes, and some occur almost exclusively in these patients. Emphysematous pyelonephritis (EPN) is an uncommon infection of the kidney characterized by production of gas within the renal parenchyma, collecting system, or perinephric tissue. Mostly confined to patients with diabetes (>90% of all cases occur in patients with diabetes), this life-threatening infection carries a mortality rate as high as 80% (2,3). The first case of EPN was reported by Kelly and MacCullum in 1898 (4). The term “emphysematous pyelonephritis” was recommended by Schultz and Klorfein (5) because of its emphasis on the relationship between the gas formation and the nature of the infectious process. Bilateral EPN (10%) is a rare phenomenon (6). The management of EPN has traditionally been aggressive, and nephrectomy is considered the treatment of choice (7). Such an approach in bilateral EPN would entail life long renal replacement therapy. Successful nonsurgical management of bilateral EPN has been previously reported (8). This article describes two additional cases in which bilateral EPN was successfully managed medically. M.K. was a 20-year-old woman who had had type 1 diabetes for 12 years, complicated by azotemic nephropathy, peripheral neuropathy, and nonproliferative diabetic retinopathy. She had been contracting recurrent urinary tract infections for the past 2 years and presented with a high-grade fever (temperature 102°F), bilateral flank pain, and repeated vomiting for 1 week. Oral ciprofloxacin prescribed by her general practitioner had provided no relief. Clinical examination revealed a distressed, apparently ill patient with tachycardia, tachypnea, pallor, dehydration, hypotension (blood pressure …

Prevalence and pattern of growth abnormalities in children with extrahepatic portal vein obstruction: Response to shunt surgery

Objective: Growth retardation is common in children with extrahepatic portal vein obstruction (EHPVO) and growth hormone (GH) resistance may play a dominant role. The aim of this study was to ascertain growth parameters and growth-related hormones in children with EHPVO, comparing with controls and to study the response of shunt surgery on growth parameters. Materials and Methods: The auxological and growth-related hormone profile (GH; insulin-like growth factor binding protein-3 [IGFBP-3] and IGF-1) of thirty children with EHPVO were compared with controls. The effect of shunt surgery on growth parameters in 12 children was also studied. Results: The mean height standard deviation score (HSDS) of cases (−1.797 ± 1.146) was significantly lower than that of controls (−0.036 ± 0.796); the mean weight SDS of cases (−1.258 ± 0.743) was also lower than that of controls (−0.004 ± 0.533). The mean GH level of cases (5.00 ± 6.46 ng/ml) was significantly higher than that of controls (1.78 ± 2.04 ng/ml). The mean IGF-1 level of cases (100.25 ± 35.93 ng/ml) was significantly lower as compared to controls (233.53 ± 115.06 ng/ml) as was the mean IGFBP-3 level (2976.53 ± 1212.82 ng/ml in cases and 5183.28 ± 1531.28 ng/ml in controls). In 12 patients who underwent shunt surgery, growth parameters significantly improved. Conclusions: Marked decrease in weight and height SDSs associated with GH resistance is seen in children with EHPVO, which improves with shunt surgery.

Amelogenesis imperfecta with distal renal tubular acidosis: A novel syndrome?

Amelogenesis imperfecta (AI) is a heterogeneous group of inherited dental enamel defects. It has rarely been reported in association with multiorgan syndromes and metabolic disorders. The metabolic disorders that have been reported in association with AI include hypocalciuria, impaired urinary concentrating ability, and Bartter-like syndrome. In literature, only three cases of AI and distal renal tubular acidosis (dRTA) have been described: two cases in adults and a solitary case in the pediatric age group. Here, we report a child with AI presenting with dRTA; to the best of our knowledge, our reported case is the only second such case in pediatric age group. Our case highlights the importance of recognizing the possibility of renal abnormalities in patients with AI as it will affect the long-term prognosis.

Clinical and laboratory profile of primary hyperparathyroidism in Kashmir Valley: A single-center experience.

Background: Although primary hyperparathyroidism (PHPT) has become an asymptomatic disease in the West, in India, PHPT is still an uncommonly diagnosed, overtly symptomatic disease with skeletal, muscular, and renal manifestations. Aims: To describe the profile and surgical outcome of 78 consecutive PHPT patients over a period of two decades at a single center. Materials and Methods: All patients who underwent evaluation and surgery for PHPT from January 1996 to December 2015 were included. Evaluation included measurement of serum total calcium, inorganic phosphorus, alkaline phosphatase, intact parathyroid hormone, 25-hydroxy Vitamin D, 24 hour urinary calcium and radiological survey. Ultrasonography neck and technetium-99m sestamibi scan were used for preoperative localization. Results: A total of 78 patients were identified during the two decades of whom 29 patients were studied retrospectively and 49 patients prospectively. Mean age of patients was 44.72 ± 12.46, and male:female ratio was 1:6. The most common presenting features were nephrolithiasis and/or nephrocalcinosis (64.10%), bone pain (44.1%), abdominal pain (39%), constipation (26%), and myopathy (14.10%). Fractures were present only in 10.25%, and brown tumors in 6.41% patients. The cure rate in our series was 96.15%. The mean parathyroid gland weight was 2.05 ± 3.03 g. None of the 41 patients in whom long-term follow-up was available, had recurrence of PHPT. Conclusions: The profile of PHPT is changing with older age at presentation, and emergence of renal stone disease and decline in overt skeletal disease as common presentation. The parathyroid weight in our study resembles that reported from developed countries.

TUMOR-INDUCED OSTEOMALACIA DUE TO HITHERTO UNDIAGNOSED SUBCUTANEOUS PHOSPHATURIC MESENCHYMAL TUMOR

Objective: Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome caused by endocrine tumors that secrete fibroblast growth factor 23 (FGF-23), a phosphaturic hormone. Mesenchymal tumors causing TIO are usually deep seated and difficult to localize. We report a case of hitherto undiagnosed TIO because of an easily palpable unsuspecting cystic lesion in the right scapular region. Methods: The clinical, biochemical, and radiologic evaluation of a 50-year-old woman with TIO are presented. Results: A 50-year-old woman presented with a 2-year history of progressive fatigue, musculoskeletal pain, and muscle weakness. Despite multiple consultations, she remained undiagnosed. Our evaluation revealed low serum phosphorus, renal phosphate wasting, and elevated FGF-23 levels, suggesting a diagnosis of TIO. Clinical examination revealed an easily palpable soft and cystic swelling 2.8 × 2.2 cm in right scapular region. A whole-body scintigraphy with Ga-68 DOTATATE positron emission tomography/computed tomo...