Acute respiratory distress syndrome in disseminated tuberculosis: an uncommon association.

OBJECTIVE: To analyse clinicopathological features of acute respiratory distress syndrome (ARDS) in disseminated tuberculosis (TB) at autopsy. METHODS: A retrospective analysis of an autopsy database of disseminated TB from 1990 to 2010 was conducted. ARDS cases were assessed for histological changes of diffuse alveolar damage (DAD) and other pathological pulmonary features. RESULTS: Disseminated TB was diagnosed in 196 cases. The clinical diagnosis of disseminated TB was made in 67% of cases. Of the 196 cases, 10 met the clinical criteria for ARDS, 60% of whom showed histological evidence of DAD. One case of DAD was diagnosed on histology alone. DAD was thus found in 7/196 cases of disseminated TB. Other pulmonary changes included necrotising granulomas (n = 10), tuberculous bronchopneumonia (n = 4), tuberculous vasculitis (n = 3), infarction (n = 1) and aspergilloma (n = 1). Histopathological diagnosis other than DAD was found in 4/10 cases and disseminated TB was presumed clinically in only 4/10 cases of ARDS. CONCLUSION: Disseminated TB may be clinically missed and diagnosed only post mortem. Disseminated TB is a relatively uncommon cause of ARDS; however, it should always be presumed clinically as it is a potentially treatable cause. DAD is a rare histological feature of disseminated TB and there may not always be a clinicopathological correlation between ARDS and DAD.

Adolescent acromegaly: clinical parameters and treatment outcome.

BACKGROUND: Adolescent acromegaly is a rare disorder and these patients present with tall stature/gigantism, tumor mass effects and menstrual irregularities. PATIENTS AND METHODS: 34 consecutive (26 males) patients having onset of disease prior to 21 years of age were included in this retrospective analysis. Their clinical features and treatment outcome were studied. RESULTS: Mean age and lag time at presentation were 21.6 +/- 3.9 years and 5.1 +/- 3.5 years respectively. Common presenting manifestations included acral enlargement, tumor mass effects and menstrual irregularities. Mean height at presentation was 174.6 +/- 13.7 cms (range: 150-210 cm) and one third had gigantism (height > or =97th percentile, WHO growth charts). Hypertension and glucose intolerance were seen in 15% and 23.5% respectively. Mean nadir GH after glucose load was 58.2 +/- 13.7 ng/ml and IGF -1 was 534.8 +/- 132.8 ng/ml. Half of the patients had concomitant hyperprolactinemia. Almost all (97%) had macroadenoma and anterior pituitary hormone deficiencies were frequent (75%). Patients with gigantism were younger (19.6 +/- 4.9 vs. 22.6 +/- 2.9 years; p = 0.001), had higher GH values (66.68 +/- 27.22 vs. 53.98 +/- 15.99 ng/ml; p = 0.04) and hypogonadism was more common (90.9% vs. 56.5%, p = 0.03) than those with normal stature. 32 patients (94.1%) were treated primarily with surgery, 7 (21.9%) received post operative radiotherapy. Mean duration of follow up was 33.1 +/- 10.1 months. Only 30% had nadir GH values of <1 ng/ml. CONCLUSION: One third of adolescent patients had acrogigantism. These patients were younger, had higher GH levels and concurrent hypogonadism was more common. Cure could be achieved only in about one third of the patients.

Hypophosphataemic rickets/osteomalacia: a descriptive analysis.

BACKGROUND & OBJECTIVES: Hypophosphataemic rickets/osteomalacia (HRO) is an uncommon metabolic bone disorder which affects all ages and either sex. It is characterized by low concentration of serum phosphate levels leading to impairment of mineralization of bone matrix with variable aetiology. We present clinical profile and treatment outcome of 17 patients of HRO. METHODS: Seventeen consecutive patients (8 were < 18 yr of age, with median age of presentation being 27.5 yr) of HRO who came to the department of Endocrinology in a tertiary care hospital in north India from January 2000 to December 2006 were included in the present study. Their aetiology, clinical features, biochemical parameters, radiographic features, treatment and outcome were analyzed. RESULTS: HRO was commoner in females (70.5%) with positive family history observed in 6 (35.3%) patients. Common presenting features were short stature (58.8%), backache (58.8%), bony deformities (58.8%), joint pain (52.9%), fractures (29.4%) and dental abnormalities (23.5%). Radiological abnormalities noted were generalized bony deformities (58.8%), fractures (29.4%), and pseudo fractures (17.6%). Mesenchymal tumours were localized in the pelvis in one patient and in the right jaw in another. The patients were treated with calcium (elemental calcium 1 g/d) and oral phosphate supplements (dose 30 - 50mg/kg/day in divided doses) along with active vitamin D supplements (dose 1 - 3 microg/day) and followed up for a mean of 2 yr. Two patients also received growth hormone (GH) therapy in the dose of 2U/day for 6 and 18 months respectively. Symptomatic well being was reported by all the patients and improvement was noted in the levels of phosphate (P<0.005) and alkaline phosphatase (P<0.05) after treatment. INTERPRETATION & CONCLUSIONS: A diagnosis of HRO should be considered in all patients presenting with short stature, deformities or musculoskeletal pains along with low serum phosphate with normal iPTH and 25--hydroxy vitamin D.

Very low or undetectable intact parathyroid hormone levels in patients with surgically verified parathyroid adenomas.

OBJECTIVES: To report and explore potential reasons for undetectable or low-normal serum intact PTH levels in patients with surgically verified primary hyperparathyroidism with parathyroid adenomas, review the relevant literature, and offer suggestions for management of such patients occasionally encountered in clinical practice. For future research, to help understand mechanisms underlying 'undetectable' or inappropriately low serum intact PTH levels. METHODS: Serum intact PTH levels were measured pre- and postoperatively by immunochemiluminescent assay (ICMA) and the results were confirmed by at least two repeated measurements on different occasions in each patient. PATIENTS: We encountered two unusual patients with primary hyperparathyroidism who had suggestive biochemical and/or clinical features of primary hyperparathyroidism. However, serum intact PTH levels were either very low or undetectable in the context of hypercalcaemia, with no other obvious cause. A (99m)Tc sestamibi scan showed increased uptake in one of the parathyroid glands, suggesting a single adenoma in each case that was confirmed at surgery. RESULTS: In the first patient, from India, mean +/- SD serum calcium was 2.6 +/- 0.32 mmol/l (reference range 2.12-2.74 mmol/l) with intact PTH of 0.11 pmol/l (reference range 1.1-7.59 pmol/l). In the second patient, from the USA, mean +/- SD serum calcium and intact PTH were 2.9 +/- 0.07 mmol/l (reference range 2.17-2.51 mmol/l) and 1.35 pmol/l (reference range 1.1-7.59 pmol/l), respectively. Following curative parathyroidectomy, serum calcium levels normalized in both patients. By contrast, serum intact PTH levels, which were either suppressed or very low before surgery, rose into the low-normal reference range in all patients. CONCLUSIONS: When the clinical suspicion is high, the diagnosis of primary hyperparathyroidism should be pursued despite suppressed or low-normal serum intact PTH levels after carefully excluding other causes of hypercalcaemia. Further research on various intact PTH molecular species secreted by parathyroid adenomas or post-translational changes in the intact PTH molecule that might interfere with in vitro measurements should be undertaken to understand the precise reason(s) for such anomalous findings.