Changes in clinical & biochemical presentations of primary hyperparathyroidism in India over a period of 20 years.

Background & objectives: With the advent of serum chemistry autoanalyzer and routine estimation of serum calcium as a part of annual physical examination, there has been a dramatic change in the presentation of primary hyperparathyroidism (PHPT) from symptomatic to asymptomatic disease in the United States. However, such trend has not been documented from India. We carried out this retrospective study to analyse the changes in clinical presentations of PHPT patients over a period of two decades in a tertiary care centre in north India. Methods: This retrospective study included patients with PHPT treated at a single centre of north India between March1990 and October 2010. Two decades were divided into four different time periods, i.e. 1990 to 1994, 1995 to 1999, 2000 to 2004 and 2005 to 2010. Clinical presentations, biochemical parameters and surgical outcomes were compared between different time periods using appropriate statistical methods. Results: Data of 202 patients with PHPT with male: female ratio of 3:7 were analyzed. There was a rise in the number of cases of PHPT diagnosed in the last decade compared to the previous decade (28 cases vs 174 cases, P<0.001). Change in the mean age, male: female ratio, lag time for the diagnosis of PHPT and clinical presentations of PHPT (predominance of bone and stone symptoms) did not differ across different time periods. Non-significant decrease in serum calcium levels at the time of diagnosis of PHPT and a significant, decline in the serum alkaline phosphatase levels (P<0.01) were found in the last decade, however, iPTH levels were higher in the last decade (pP<0.05). There was no change in the site and size of parathyroid adenoma in the two decades, however, postoperative symptomatic hypocalcemia was less frequent in the last decade. Interpretation & conclusions: The findings of this retrospective analysis show that the PHPT still remains symptomatic disease with increasing awareness over the last two decades in our center. There was not much change in the clinical presentation, in the past two decades.

Fibrous dysplasia & McCune-Albright syndrome: An experience from a tertiary care centre in north India.

Background & objectives Fibrous dysplasia (FD) is a rare metabolic bone disease and information available from India is limited to only anecdotal case reports. We describe the clinical profile and therapeutic outcome of 25 patients with FD observed over a period of 14 yr in a tertiary care centre from north India. Methods In this retrospective study patients (n = 25) with diagnosis of fibrous dysplasia based on either classical radiological features and/or histological evidence on bone biopsy, were analyzed. Associated endocrinopathies if any, were evaluated. The diagnosis of McCune Albright syndrome (MAS) was considered when fibrous dysplasia was accompanied by either café-au-lait macules and/or endocrinopathies. The clinical presentation, biochemical parameters and imaging were analysed. Seven patients received bisphosphonate therapy. The final outcome and side effects were noted. Results Age of the patients ranged from 7 to 48 yr (mean ± SD, 24.2 ± 11.4 yr) with a lag time between onset of symptoms and presentation ranging from 1 to 20 yr (mean ± SD, 6.6 ± 6.2 yr). The mean duration of follow up was 3.5 ± 2.1 yr. Eighteen (72%) patients had polyostotic disease while the remaining had monostotic FD. Eight patients had endocrinopathies: five had acromegaly, one each had gonadotropin independent precocious puberty (GIPP), hyperthyroidism and hypophosphatemic rickets. One child with GIPP later developed hyperthyroidism. McCune Albright syndrome was observed in 10 (40%) patients. A majority of the patients underwent various minor or major surgical procedures and seven patients received bisphosphonates for recurrent pathological fractures. Bone pain was reduced in all bisphosphonate treated patients with a decrease in subsequent fractures. Interpretation & conclusions This series of FD patients from north India shows the varying presentations of this rare disease. Medical treatment with bisphosphonates appears to be potentially rewarding.

Computed tomographic pulmonary angiography: Utility in acute pulmonary embolism in providing additional information and making alternative clinical diagnosis.

Objective: To evaluate the role of computed tomographic (CT) pulmonary angiography (CT-PA) in detecting additional information that may help in making an alternative clinical diagnosis in patients referred to CT for a suspected acute pulmonary embolism (PE). Materials and Methods: 50 patients (34 males, 16 females) in the age group of 18-72 years (mean 42.3 years), having high clinical suspicion of PE, underwent CTPA over a 2 year period. Chest x-ray, arterial blood gas (ABG) analysis, echocardiography were done in all patients. All patients underwent at least one other imaging examination besides CTPA: ventilation perfusion scan, Doppler ultrasound or compression ultrasound (for DVT). All patients were followed for 3 months after completion of the diagnostic work up at baseline. The final diagnosis was achieved by a combination of clinical, imaging, and laboratory analysis, after adequate imaging, laboratory tests, and follow up. Result: CTPA helped correctly identify 29 of 30 patients with PE. In the remaining 20 patients (with no evidence of PE), CT-PA provided additional information (that suggested or confirmed alternate clinical diagnosis) in 15 patients (75%): pleural effusion (n=8), mediastinal or hilar lymphadenopathy (6), pneumonia/airspace consolidation (5), atelectasis/collapse (2), aspergilloma (1), malignancy (1), and others (2). Conclusion: CT-PA is highly specific and sensitive for diagnosis of PE. In addition, in a majority of patients who do not have PE, it also provides important ancillary additional information and helps in making an alternative clinical diagnosis.

A pilot study on hypothalamo-pituitary-adrenocortical axis in primary hyperparathyroidism.

Background & objectives: Parathormone (PTH) and calcium, both have been shown to stimulate adrenal steroidogenesis in animal models and in vitro experiments. This is attributed to structural similarity between 15-25 amino acid region of the parathyroid hormone (PTH) and 1-11 amino acid region of adrenocorticotropin (ACTH). However, there are no in vivo human data regarding the effect of PTHcalcium axis on adrenocortical function. Materials: Ten patients with primary hyperparathyroidism underwent evaluation for cortisol dynamics including 0800 h and 2000 h plasma cortisol on day 1, cortisol response to insulin induced hypoglycaemia (IIH) on day 2, and 1 mg overnight dexamethasone suppression test (ONDST) on day 4. Serum aldosterone was also measured at 0800 h in fasting state on salt ad libitum for three days. These parameters were repeated 3 months after curative parathyroidectomy. Results: Basal plasma cortisol level at 0800 h and 2000 h were within upper normal range and loss of circadian rhythm in cortisol secretion was observed in half and forty per cent of patients had nonsuppressibility with ONDST. The defined peak cortisol response to insulin induced hypoglycaemia (>550 nmol/l) was achieved in all and nearly one third of patients had exaggerated response (>2000 nmol/l). After curative parathyroidectomy, the abnormalities in circadian rhythm and non-suppressibility with ONDST continued to prevail in 40 per cent of patients. The peak cortisol response to IIH showed a decrement but remained higher than normal. No correlation was observed between circulating parathyroid hormone and calcium with cortisol levels. Serum aldosterone was in upper normal range pre - and postoperatively, though it decreased postoperatively, but it could not attain a statistical significance (p = 0.5). Interpretation & conclusion: Abnormalities in hypothalamo-pituitary-adrenocortical axis in primary hyperparathyroidism do occur, however these are inconsistent and do not recover in majority of patients even after 3 months of curative parathyroidectomy.

Risk factors for renal injury in patients with meningomyelocele.

PATIENTS AND METHODS: Thirty operated patients of myelodysplasia were clinically evaluated for the age at presentation, the extent of lesion and neurological deficit. Urological assessment was done with urine cultures, serum creatinine, radiological (ultrasound of kidney, ureters and bladder, voiding cystourethrogram) and urodynamic (water cystometry) parameters. An objective scoring for bladder (Galloway, et al.) was applied. Dimercapto-succinic acid (DMSA) scan was done in all the patients for evidence of renal scars. The results of above investigations were correlated with presence or absence of renal scars (renal injury) on DMSA scan. None of the patients had received any prior bladder care. RESULTS: Twenty one patients had no renal scars and 9 patients had evidence of renal scarring. Patients with renal scars were older at presentation, they had greater degree of hydroureteronephrosis (P < or = 0.001) and vesicoureteric reflux (P < or = 0.005). The incidence of high leak pressures (>25 cm of water, P < or = 0.05), unacceptable bladder volumes (maximum cystometric capacity < 60% for age, P < or = 0.005) and high risk Galloway's score (> 5, P < or = 0.05) was high in patients with associated renal scarring as compared to their nonscarred counterparts. Three of these patients had serum creatinine >1 mg/dl (P < or = 0.005). The incidence of urinary complaints and positive urine cultures was also higher in these patients (NS). CONCLUSION: Increasing age, evidence of hydroureteronephrosis and vesicoureteric reflux, high leak pressures, low bladder volume and high combined Galloway score (>5) define a high risk bladder in our population and predispose to renal injury in patients of myelodysplasia. Early referral for bladder risk assessment and management of all myelodysplasia patients is recommended.

Clinical profile, diagnosis and management of patients presenting with symptomatic pulmonary embolism.

OBJECTIVE: To study the clinical profile and diagnostic methods in patients with symptomatic pulmonary embolism (PE). METHODS: Prospective assessment of clinical features, radiology and outcome of patients presenting with symptomatic PE over an 18-month period. RESULTS: During study period, 24 patients with a mean age of 39 +/- 12.1 years were diagnosed to have symptomatic pulmonary embolism. Dyspnoea (91.7%) and cough (58.3%) were the predominant complaints. Spiral computed tomographic pulmonary angiography (CTPA) was performed in 21 (87.5%) patients and perfusion scans in 14 (58.4%) patients. Echocardiography performed in all patients revealed evidence of pulmonary artery hypertension and right ventricular dyskinesia in 20 (83.3%) and 15 (62.5%) patients, respectively. Thrombolysis with streptokinase was performed in 14 (58.3%) patients. All patients received low molecular weight heparin followed by warfarin. Of the 24 patients, 20 (83.3%) were discharged and are under regular follow-up; four patients died. CONCLUSIONS: Pulmonary embolism is a common problem and can be easily diagnosed provided it is clinically suspected. Early diagnosis and aggressive management is the key to successful outcome.

Bone scan in diagnosis of lymphoma with extensive bone marrow necrosis--a case report.

Bone marrow necrosis (BMN) is a relatively uncommon clinicopathologic entity with diverse etiology. We describe a case of BMN in a 11 1/2 year old male child with an underlying non Hodgkin's lymphoma T-cell type. With the help of 99m Tc-MDP (methylene diphosphonate) bone scan we were able to find out the etiologic factor in this case.

Bronchial transection: delayed diagnosis and successful repair.

Traumatic bronchial transection is usually recognized and repaired immediately after injury. Bronchial transection has a variety of clinical presentations due to air leak into the pleural cavity and it is very rare to have total absence of air leak from the transected bronchus at presentation. We present one such case of main right bronchus injury with total absence of initial clinical signs and symptoms, leading to a delay in the diagnosis. However, the surgical repair eight months after injury showed excellent recovery of the chronically collapsed lung.

Effect of erythromycin and cisapride on emptying of the vagally denervated intrathoracic stomach.

BACKGROUND: Subsequent to esophagectomy and reconstruction among patients with esophageal cancers, the intrathoracic denervated stomach acts as a passive conduit without peristalsis. OBJECTIVE: The study was designed to assess the impact of two prokinetic drugs viz. erythromycin and cisapride on the emptying of vagally denervated intrathoracic stomach. METHODS: Twenty consecutive patients of carcinoma esophagus, who had undergone one stage transhiatal oesophagectomy with cervical esophagogastrostomy and were disease free at three months postoperative follow-up, were included in the study. These patients were randomised into two groups of ten each. The patients in group A received erythromycin, while patients in group B received cisapride. The gastric emptying was studied by scintigraphy, using a standard test meal containing 99m Tc sulphur colloid labelled 'IDLIS' [rice based radio labelled food] before and after the drug treatment. RESULTS: The pre and post treatment mean gastric half emptying time of the patients in the erythromycin group was 52.6 min and 49.7 min (p > 0.1) and in cisapride group it was 53.76 and 26.4 min respectively (p < 0.05). Intergroup comparison of the difference was not statistically significant. CONCLUSION: Cisapride is an effective prokinetic agent in the treatment of gastric stasis of the vagally denervated intrathoracic stomach.

Gastric emptying in patients with non ulcer dyspepsia (dysmotility type).

Non ulcer dyspepsia is a clinical entity characterized by chronic recurrent upper gastrointestinal symptoms in the absence of identifiable organic or systemic cause. Many such patients may have delayed gastric emptying which can easily be documented using radionuclide food markers. We evaluated gastric emptying patterns in 20 patients having non ulcer dyspepsia and 30 healthy controls. Gastric emptying was studied using indigenously prepared radiolabelled vegetarian solid food marker, the integrity of which had already been established and reported by us. Nine of the 20 (45%) non ulcer dyspepsia patients showed delayed gastric emptying. All these patients were administered cisapride and on reevaluation showed improvement in their upper gastrointestinal symptoms.

Role of biliary scintigraphy in management of bile leak.

BACKGROUND: Endoscopic biliary drainage is effective in the management of patients with postoperative bile leak. Evidently, it cannot be used in patients with completely tied-off common bile duct (CBD). AIM: To ascertain whether biliary excretion scintigraphy could reliably detect patients with tied-off CBD so that endoscopic retrograde cholangiography (ERC), an invasive test, could be avoided. METHODS: Twelve patients with active bile leak (11 postcholecystectomy, one posttraumatic), in whom the biliary system was imaged by both 99m technetium mebrofenin biliary scintigraphy and ERC, were evaluated. RESULTS: Bile leak was demonstrated in all the 12 patients by scintigraphy. In six of these, there was no visualization of radionuclide activity in the intestines; in three of these six patients, ERC showed a patent CBD potentially amenable to endoscopic drainage, whereas in the remaining three, there was complete obstruction presumably due to CBD tie-off during surgery. CONCLUSION: Biliary excretion scintigraphy is unreliable for detecting complete biliary obstruction in patients with bile leak.