Lean Cushing's.

This is a case report of 59 years old male with adrenocorticotropic hormone ACTH-independent macronodular adrenal hyperplasia (AIMAH), which is a rare cause of Cushing's syndrome. He was detected to have adrenal lesions incidentally on imaging. The biochemical evaluation was suggestive of endogenous ACTH-independent Cushing's syndrome. Contrast Enhanced CT of the abdomen done to characterize the lesion was suggestive of bilateral macro-nodular adrenal hyperplasia. There was no evidence of cushingoid features except for type 2 diabetes mellitus suggestive of subclinical Cushing's. He was not planned for any immediate medical/surgical management since metabolic control was achieved with oral anti-diabetics (OADs) and anti-hypertensive medications. He is on regular follow up with well-controlled sugars and blood pressure.

Schwannoma: An uncommon dweller in the adrenal gland.

An elderly adult woman presented with upper abdominal pain to the urology department. Imaging studies revealed a right adrenal lesion with central necrosis, abutting the large vessels and suggestive of malignancy. Histopathology showed an encapsulated spindle cell tumor with marked nuclear atypia, cystic change, and hemorrhage. S100 immunomarker was strongly and diffusely positive. Final diagnosis was ancient schwannoma with secondary change. Schwannomas are rare in the adrenal gland, with <50 cases reported in the literature. These incidentally detected nonfunctional adrenal tumors need confirmation with histopathology and ancillary studies.

Does Baseline PTH Influence Recovery of Bone Mineral Density, Trabecular Bone Score and Bone Turnover Markers? A Prospective Study Following Curative PArathyroidectomy in Primary Hyperparathyroidism.

OBJECTIVE: This prospective study was carried out to assess trabecular bone score, bone mineral density (BMD), and bone biochemistry in Indian subjects with symptomatic primary hyperparathyroidism (PHPT), and to study the influence of baseline parathyroid hormone (PTH) on recovery of these parameters following curative surgery. METHODS: This was a 2-year prospective study conducted at a tertiary care centre in southern India. Baseline assessment included demographic details, mode of presentation, bone mineral biochemistry, BMD, trabecular bone score (TBS), and bone turnover markers (BTMs). These parameters were reassessed at the end of the first and second years following curative parathyroid surgery. RESULTS: Fifty-one subjects (32 men and 19 women) with PHPT who had undergone curative parathyroidectomy were included in this study. The mean (SD) age was 44.6 (13.7) years. The TBS, BTMs, and BMD at lumbar spine and forearm were significantly worse at baseline in subjects with higher baseline PTH (≥250 pg/mL) when compared to the group with lower baseline PTH (<250 pg/mL). At the end of 2 years, the difference between high versus low PTH groups (mean ± SD) persisted only for forearm BMD (0.638±0.093 versus 0.698±0.041 g/cm2; P =.01). However, on follow-up visits in the first and second year after curative parathyroidectomy, there was no significant difference in BTMs, BMD at the femoral neck, lumbar spine, and TBS between the 2 groups stratified by baseline PTH. CONCLUSION: The BMD at the forearm remained significantly worse in individuals with high baseline PTH even at 2 years after surgery, while other parameters including TBS improved significantly from baseline. ABBREVIATIONS: 25(OH)D = 25-hydroxyvitamin D; BMD = bone mineral density; BMI = body mass index; BTMs = Bone turnover markers; CTX = C-terminal telopeptide of type 1 collagen; DXA = dual energy X-ray absorptiometry; P1NP = N-terminal propeptide of type 1 procollagen; PHPT = primary hyperparathyroidism; PTH = parathyroid hormone; TBS = trabecular bone score.

Clinico-radiological characteristics and not laboratory markers are useful in diagnosing diabetic myonecrosis in Asian Indian patients with type 2 diabetes mellitus: A 10-year experience from South India.

INTRODUCTION: Diabetic myonecrosis or muscle infarction is an unusual complication of Type 2 Diabetes, usually associated with longstanding disease. It commonly presents as an acute non-traumatic palpable swelling of the affected muscle with predilection for the quadriceps and thigh muscles, often accompanied by retinopathy and nephropathy. METHODOLOGY: A retrospective review of the medical records of patients admitted with diabetic myonecrosis under the Department of Endocrinology, Christian Medical College Vellore over a period of ten years(2006-2015) was done. Data pertaining to clinical, biochemical and radiological characteristics were obtained and treatment modalities and outcomes were recorded. RESULTS AND ANALYSIS: A total of n = 4 patients with diabetic myonecrosis and completed clinical data were included in the study. In our present series, the mean age at presentation was 45.5 years (±7.3 years), the mean duration of the diabetes was 9.0 years (±2.5 years)with an equal distribution of male and female subjects. The mean HbA1c (9.5 ± 0.6%) was suggestive of poor glycemic control at presentation with all (100%) the patients in our series having concomitant one or more microvascular complications. While laboratory parameters of elevated CPK or LDH were mostly normal, the findings of T1 hyperintense and T2 hypointense heterogenous lower limb lesions were present in all the subjects (n = 4). Conservative management with bed rest, analgesics and good glycemic control were effective in good clinical improvement over a period of 1-2 months. CONCLUSIONS: Our series of diabetic myonecrosis in Indian patients with Type 2 diabetes mellitus, elucidates the varied clinical presentations, with MRI findings rather than laboratory markers being the mainstay of diagnosis.

An unusual presentation of metastatic bone disease in a subject with Paget's disease of bone.

Solid organ malignancies involving breast, prostate, and lung frequently metastasize to the skeleton. However, the occurrence of Paget's disease and metastatic bone disease in the same patient is uncommon. We report a case of a 63-year-old man who presented with back pain and a lump in the right breast. He was earlier diagnosed to have Paget's disease of bone based on characteristic skeletal radiological features,99mTc methylene diphosphonate bone scan and elevated alkaline phosphatase, and treated with bisphosphonates, and his disease was in remission. Further evaluation revealed an underlying skeletal metastatic disease secondary to a breast malignancy. He underwent radical mastectomy with axillary node clearance, radiotherapy, and chemotherapy. In addition, he also received parenteral bisphosphonates for his skeletal metastatic bone disease.

Hajdu Cheney Syndrome.

Hajdu-Cheney Syndrome is a rare genetic disorder characterised by progressive focal bone destruction. It is known to be an autosomal dominant disorder but there have been reports of sporadic cases as well. Although the disease manifestation is found to begin from birth, it is most often not diagnosed until adolescence or adulthood. It could be due to the rarity of the condition and the variation of the disease manifestation at different age groups. We report a case of Hajdu-Cheney Syndrome in a 26-year-old male who presented with severe periodontitis and premature loss of teeth. The other characteristic features included craniofacial dysmorphism, abnormalities of the digits and dental anomalies. Patients with craniofacial dysmorphism along with dental abnormalities should be thoroughly examined for any underlying systemic disorder. A team of specialists may be able to diagnose this condition before the disease is advanced.