Pattern of Lipid Abnormalities Among South Asian Indians With Cushing's Syndrome and the Short Term Impact of Surgical Correction of Hypercortisolism.

Atherosclerotic cardiovascular events are one of the common causes of mortality in patients with Cushing's syndrome (CS). Atherogenic dyslipidemia is more common among South Asian Indians as compared to other ethnicities and is likely to worsen among patients with CS. This retrospective study was done over 5 years at a single institute to evaluate the pattern of lipid abnormalities in subjects with CS and the changes in lipid parameters after surgical control of hypercortisolemia. The study was done in two parts. In the first part, records of patients with CS diagnosed over 3 years were retrospectively reviewed. Hormonal and metabolic parameters including fasting plasma glucose (FPG), post prandial plasma glucose (PPPG), HbA1c, serum lipids, serum cortisol and plasma ACTH were recorded. In the second part, lipid parameters were rechecked among patients who underwent surgery and a median follow up of 4±2 months after remission. Out of the 126 patients diagnosed with endogenous CS over 3 years, 100 patients were eligible for inclusion in the study. At baseline, sixty five (65%) patients had dyslipidemia as defined by the NCEP-ATPIII criteria. 47 out of 63 (74.6%) subjects achieved remission after surgical management of CS. 32 (68.1%) of these patients had dyslipidemia prior to surgery. After excluding 1 death, 26 of 46 (56.5%) subjects had dyslipidemia after the follow up period. Lipid abnormalities are common among South Asian Indian subjects with endogenous CS and the pattern persists in most of them, 3 months after surgical correction of hypercortisolism.

Absence of vitamin D deficiency among common outdoor workers in Delhi.

BACKGROUND: There is reservation about accepting the notion of widespread vitamin D deficiency (VDD) in sunny countries because information base is largely urban indoors, and the cut-off serum 25(OH)D > 75.0 nmol/L to define sufficiency is perceived as high. OBJECTIVE: We assessed the vitamin D status of subjects engaged in six types of outdoor jobs with freedom to seek shade, when needed. DESIGN: Descriptive observational study. SUBJECTS AND METHODS: A total of 573 outdoors, (hawkers, n = 144; auto-rickshaw drivers, n = 113; manual rickshaw pullers, n = 49; fuel-station attendants, n = 84; gardeners, n = 96; traffic police personnel, n = 87) were assessed for serum 25(OH)D, iPTH and total calcium during summer and winter. Bank employees were indoor controls (n = 72). Serum 25(OH)D was defined as sufficient if ≥50.0 nmol/L and deficient when <30.0 nmol/L, as per 'Institute of Medicine'. RESULTS: Mean serum 25(OH)D of 573 outdoors was 44.8 ± 19.6 nmol/L and showed a physiological inverse relation with iPTH (P < 0.001). 77.5% of the outdoors did not have VDD. Hawkers, gardeners, fuel-station attendants and rickshaw pullers had sufficient or near sufficient serum 25(OH)D. The mean serum 25(OH)D (30.6 ± 23.2 nmol/L) of indoors though lower by 12.7 nmol/L than outdoors was above the cut-off of VDD. Proportions with supranormal iPTH were comparable between outdoors and indoors (14.0% vs 20.8%). Despite winter dip, the mean serum 25(OH)D (31.2 ± 14.3 nmol/l) of outdoors was not deficient. CONCLUSIONS: Vitamin D deficiency is not universal. Most urban outdoor workers do not have VDD.

Phenotypic presentation of adolescents with overt primary hypothyroidism.

BACKGROUND: The phenotypic presentation of overt hypothyroidism during adolescence is less well characterized. The aim of the study was to study the phenotypic presentation of patients with overt hypothyroidism presenting during adolescence (age 9-18 years). METHODS: Records of adolescent patients with overt hypothyroidism (thyroid stimulating hormone [TSH]>10 mIU/L) were retrospectively analyzed for presenting complaints, height and pubertal status. RESULTS: A total of 67 patients (40 females and 37 males, average age 13.2+2.3 years) with a mean TSH of 241.3±336.6 mIU/L were included. The commonest presentation was short stature in 46.2% of patients followed by neck swelling (16.4%) and weight gain (11.9%). The mean height standard deviation score (SDS) was -2.5+2.11, 43% of patients had less than 3 SDS. The height age and bone age were around 3 years less than the chronological age. The bone age significantly correlated with the height age but not with TSH levels. Three patients referred from neurology with primary complaints of headache had pituitary hyperplasia and one presented with hypokalemic periodic paralysis. Seven had delayed puberty and one patient had early periods due to huge ovarian cysts. CONCLUSIONS: In the present study, severe short stature and uncommon phenotypic presentations were a consequence of long-standing severe untreated hypothyroidism.

Peptide Receptor Radionuclide and Octreotide: A Novel Approach for Metastatic Tumor-Induced Osteomalacia.

KEY MESSAGES: Octreotide can be used as an adjunctive therapy to increase phosphorus levels in patients with tumor-induced osteomalacia. Malignant phosphaturic mesenchymal tumor (PMT) may benefit from treatment with peptide receptor radionucleotide therapy. CONTEXT: The success of treatment modalities for malignant PMT is limited. Octreotide has been used to treat hypophosphatemia in patients with tumor-induced osteomalacia with equivocal results. To our knowledge, there are no reports of octreotide or peptide receptor radionuclide therapy use for malignant PMT. CASE DESCRIPTION: We report a 40-year-old man having hypophosphatemia, phosphaturia (tubular maximum of phosphorus corrected for glomerular filtration rate of <2.5 mg/dL), and somatostatin avid lesions in the right foot region with metastasis to both lungs. The patient had been subjected to resection of the primary tumor from the foot with thoracoscopic removal of the lung secondaries. Histology from all three lesions showed a spindle cell soft tissue tumor with a high mitotic index and somatostatin receptor 2 and 5 positivity. A trial of subcutaneous octreotide therapy at a dose of 100 µg thrice daily resulted in an increase in serum phosphorus levels from an average of 1.44 mg/dL to an average of 2.3 mg/dL. Finally, the affected limb was amputated, and the hypophosphatemia persisted postoperatively. In view of persistent hypophosphatemia and transient response to octreotide, the patient was administered four cycles of peptide receptor radionuclide therapy using 177Lutetium, which showed moderate improvement of serum phosphorus levels. CONCLUSION: Although octreotide use has been reported in four patients with benign PMT, to our knowledge, this is the first case of malignant PMT that has used peptide receptor radionuclide therapy in the treatment of malignant PMT. This moderately beneficial evidence is likely to guide the future use of radionuclide treatments in such tumors.

A Rare Occurrence of Simultaneous Venous and Arterial Thromboembolic Events - Lower Limb Deep Venous Thrombosis and Pulmonary Thromboembolism as Initial Presentation in Acute Promyelocytic Leukemia.

The development of acute myeloid leukemia has been attributed to various factors, including hereditary, radiation, drugs, and certain occupational exposures. The association between malignancy and venous thromboembolism events is well established. Here, we present a case of a 70-year-old Indian man who had presented with arterial and venous thrombosis, and the patient was later diagnosed with acute promyelocytic leukemia (APL). In our case, the patient presented with right lower limb deep venous thrombosis and pulmonary thromboembolism four months prior to the diagnosis of APL. Although thromboembolic event subsequent to the diagnosis of malignancy, and especially during the chemotherapy has been widely reported, this prior presentation with simultaneous occurrence of both venous and arterial thromboembolism has rarely been reported. We take this opportunity to state the significance of a complete medical evaluation in cases of recurrent or unusual thrombotic events.

Bilateral femoral head avascular necrosis with a very low dose of oral corticosteroid used for panhypopituitarism.

Avascular necrosis (AVN) of the femoral head is a rare complication related to glucocorticoid administration and traditionally has been associated with high doses and/or prolonged therapy. Occurrence of osteonecrosis with a physiological replacement dose of glucocorticoids has not been reported previously. We report a 38-year-old man with non-secreting pituitary adenoma who developed bilateral AVN while on a very small dose of oral prednisolone for secondary adrenal insufficiency after surgery for pituitary adenoma. The patient was switched to hydrocortisone. Zolindronic acid was administered and the patient underwent bilateral core decompressive surgery resulting in a reduction of hip pain and improvement. When last evaluated, 2 years after diagnosis of AVN, the patient was functionally independent, and was able to do his routine activities with mild pain. The report intends to highlight the occurrence of AVN of the femur even with a very small dose of prednisolone used for treatment of panhypopituitarism. Glucocorticoids may have to be continued in the lowest possible dose using the most physiological preparation such as hydrocortisone when stoppage is not possible.

Bilateral breast lumps as a presentation of disseminated squamous cell carcinoma of lung.

We present the case of a 65-year-old female who presented to our hospital with nodular swelling in her breast that first appeared in the right upper quadrant 10 months earlier, followed by involvement of the left upper quadrant along with nodular swelling in the right inguinal region for the past six months. She was also complaining of breathlessness on exertion and right-sided pleuritic chest pain for the past one year. Her chest X-ray showed well defined consolidation in the right lower lobe of the lung with pleural effusion. Further pleural tap showed malignant cells with squamous differentiation. Fine needle aspiration cytology (FNAC) from breast lumps was suggestive of malignant cells with morphology of cells likely to be squamous. CT-guided biopsy of the lung mass showed moderately differentiated squamous cell carcinoma of the lung. She succumbed to her illness following severe respiratory distress. Breast lump secondary to lung malignancy is very rare. Squamous cell carcinoma presenting as breast metastasis is a very rare presentation and reported in few cases. No previous case reporting bilateral breast lumps as a presentation of squamous cell carcinoma of the lung could be found in the literature.

Recurrent symptomatic hypoglycaemia with pancytopenia as a delayed presentation of Sheehan's syndrome with complete recovery after hormone replacement.

A 38-year-old woman presented to our hospital emergency section in altered sensorium and with blood pressure of 80/60 mm Hg. She gave a history of recurrent episodes of loss of consciousness for 15-20 days, generalised body swelling and generalised weakness for the past 5-6 years. On further evaluation she was found to have severe hypoglycaemic episodes. In view of history of pregnancy and significant blood loss during surgery and long history of lethargy, asthaenia and generalised anasarca, the patient was evaluated for Sheehan's syndrome. Her serum cortisol levels were very low and she was found to have central hypothyroidism. MRI of the brain also revealed small sella turcica and small pituitary gland suggestive of hypopituitarism. The patient was started on high-concentration dextrose drips, steroids and thyroid hormone replacement. The patient showed a marked improvement within 1 week of treatment. And she was discharged with an advice to follow-up at our outpatient department.

Successful treatment of acquired pure red cell aplasia with oral corticosteroids in a patient with B-cell CLL.

We present the case of a 57-year-old male patient diagnosed with chronic lymphoid leukaemia (CLL) B-cell type along with moderate anaemia. On follow-up investigations the aetiology of anaemia turned out to be pure red cell aplasia (PRCA) on trephine bone biopsy with an elevated serum erythropoietin level. The patient received blood transfusion support. He showed remarkable improvement on oral corticosteroids (prednisolone 60 mg/daily dose) with no further requirement of blood transfusion over next 3 months. However, when the dose of steroid was tapered down to 10 mg/day, the anaemia reappeared. An increase in the dose of steroid brought the haemoglobin level back to normal. Anaemia in CLL can be due to many reasons, of which PRCA is an uncommon association occurring in only around 1% of patients with CLL and usually refractory to the conventional treatment with steroids. This PRCA secondary to CLL is considered to be immune in origin and a response to combination of immunosuppressive therapy such as steroids, cyclosporine, rituximab is anticipated. Our case responded completely to oral steroids alone.

MEN2B syndrome presenting as an acute respiratory emergency.

An 18-year-old girl presented to the emergency department with a history of noisy breathing and breathlessness progressively increasing for few days. The patient had stridor and tachypnoea. She was tall with a long thin face, wrist sign and high-arched palate suggestive of marfanoid features. X-ray of the neck revealed critical tracheal narrowing. Emergency tracheostomy was performed as a lifesaving procedure. Non-contrast CT neck revealed extratracheal compression by a mass surrounding it. Contrast-enhanced CT scan of the neck revealed heterogeneous mass arising from the right lobe of the thyroid and tracheal deviation with narrowing. Fine-needle aspiration cytology of the mass revealed medullary carcinoma of the thyroid, positive for calcitonin. Calcitonin levels were raised. Apart from the marfanoid features she had localised swellings over the lips, lower eyelid and the lateral aspects of the tongue, clinically suggestive of neuromas. A clinical diagnosis of multiple endocrine neoplasia type 2B syndrome was made. The patient underwent total thyroidectomy with central lymphnode dissection. This case highlights an unusual presentation of a rare disease.

Hand grip strength and its correlation with vitamin D in Indian patients with hip fracture.

UNLABELLED: This case-control study was performed to evaluate 25-hydroxyvitamin D [25(OH)D] deficiency and its correlation with hand grip strength in 95 Indian hip fracture subjects and 95 controls. 25(OH)D deficiency was found in 88.4 % of hip fracture subjects that was significantly higher as compared to controls. Hand grip strength as measured by hand held dynamometer was significantly lower in patients, and there was a significant positive correlation between 25(OH)D and hand grip strength. PURPOSE: The present study was conducted to assess correlation between 25(OH) D and hand grip strength in hip fracture subjects residing in North India. METHODS: Ninety-five patients with hip fracture and similar number of controls were enrolled in the study. Fasting venous samples were analyzed for 25(OH)D, intact parathyroid hormone (PTH), alkaline phosphatase, calcium, and phosphate. Hand grip strength of study subjects was measured using Jamar dynamometer. Correlation between vitamin D levels and hand grip strength was analyzed in study population. RESULTS: The mean age of hip fracture subjects was 61.4 ± 12.6 years which was comparable in men and women. Out of 95 subjects, 57 were men and 38 were women. Mean 25(OH)D levels were significantly lower whereas intact PTH levels were significantly higher in patient group compared controls (10.29 ± 6.53 vs 13.6 ± 4.01 ng/ml; 62.6 ± 59.3 vs 37.7 ± 28.8 pg/ml, respectively). The number of subjects with 25(OH)D deficiency and secondary hyperparathyroidism was significantly higher in hip fracture group. The mean hand grip strength among hip fracture subjects was significantly lower compared to that of controls (16.57 ± 5.74 vs 26.74 ± 5.23 kg). There was a significant positive correlation between 25(OH)D and hand grip strength ( r = 0.482, p value <0.01) in hip fracture population. CONCLUSION: Majority of hip fracture patients in India have vitamin D deficiency, secondary hyperparathyroidism, and lower hand grip strength compared to controls. Further, there is significant positive correlation between 25(OH)D and hand grip strength.

Vanishing large ovarian cyst with thyroxine therapy.

UNLABELLED: A 21-year-old female patient recently diagnosed with severe hypothyroidism was found to have a large ovarian cyst. In view of the large ovarian cyst, she was advised to undergo elective laparotomy in the gynaecology department. She was further evaluated in our medical out-patient department (OPD), and elective surgery was withheld. She was started on thyroxine replacement therapy, and within a period of 4 months, the size of the cyst regressed significantly, thereby improving the condition of the patient significantly. This case report highlights the rare and often missed association between hypothyroidism and ovarian cysts. Although very rare, profound hypothyroidism that can cause ovarian cysts in an adult should always be kept in the differential diagnosis to avoid unnecessary ovarian surgery. LEARNING POINTS: Hypothyroidism should be considered in the differential diagnosis of adult females presenting with multicystic ovarian tumours.Adequate thyroid hormone replacement therapy can prevent these patients from undergoing unnecessary and catastrophic ovarian resection.Surgical excision should be considered only when adequate thyroid replacement therapy fails to resolve ovarian enlargement.In younger women with ovarian cysts, it is also desirable to avoid unnecessary surgery so as to not compromise fertility in the future.