Thoracoscopic and endovascular management of retained haemothoarx associated with an intercostal artery pseudoaneurysm.

Intercostal artery pseudoaneurysm (IAP) is a rare entity and may complicate a percutaneous intervention through an intercostal space or follow thoracic trauma. Its rupture into the pleural space can give rise to haemothorax, which if untreated may lead to a retained haemothorax (RH). Traditionally both the IAP and the RH are managed by a thoracotomy. We report a patient who developed an IAP with haemothorax following a trauma. The diagnosis was established by computed tomography. The patient was treated by endovascular embolisation of the IAP followed by thoracoscopic decortications of the RH.

Idiopathic pulmonary haemosiderosis: complete remission with steroids.

A 13-year-old boy was diagnosed as a case of idiopathic pulmonary haemosiderosis on the basis of clinical presentation, radiological assessment and open lung biopsy. The patient had a complete clinical and radiological remissions with oral corticosteroids.

Cryptogenic organizing pneumonia: clinical profile in a series of 34 admitted patients in a hospital in India.

OBJECTIVES: Cryptogenic Organizing Pneumonia (COP) is a relatively rare disorder which is gratifying to treat due to its prompt steroid responsiveness. There have been only 2 case reports on COP from India but no large series entity reported from this country. METHODS: The medical records of all patients with biopsy (histopathology) proven COP admitted in a tertiary care hospital in Mumbai (2000-2005) were retrospectively analyzed. We looked at clinical and radiographic profiles, initial diagnosis and treatment, lag period to starting definitive therapy and steroid responsiveness. RESULTS: When compared to other series of patients with COP, our series showed several similarities and some differences. Distinctive features were the striking female preponderance and the utility of transbronchial biopsies in establishing the diagnosis. Long delays in diagnosis with patients mislabeled as tuberculosis or pneumonia, lead to delays in starting steroids resulting in 21% of our patients continuing to deteriorate. CONCLUSIONS: This comprehensive review of COP, the first of its kind from India, reveals its varied clinical and radiographic spectrum. A high index of suspicion will lead to prompt steroid therapy which will result in better patient outcome.

Hypereosinophilic syndrome with isolated Loeffler's endocarditis: complete resolution with corticosteroids.

Hypereosinophilic syndrome (HES) is classically defined as prolonged, unexplained peripheral eosinophilia in a patient presenting with evidence of end-organ damage. The heart is involved in two forms; endomyocardial fibrosis (Davies disease) and eosinophilic endocarditis (Loffler's endocarditis). It was first reported in 1968 by Hard and Anderson. Chusid and co-workers formulated a definition with strict criteria for the diagnosis of HES as 1) peripheral blood eosinophilia more than 1500 cells/cu mm for at least six months duration 2)signs, symptoms of end-organ (heart, lungs, gastrointestinal tract, skin, bone-marrow, brain) involvement with eosinophil tissue infiltration/injury 3) exclusion of known secondary causes of eosinophilia. We report a case of hypereosinophilic syndrome with Loffler's endocarditis, in the absence of endomyocardial fibrosis. The patient presented with a eosinophilic vegetation over the posterior leaflet of the mitral valve. There was complete resolution of the vegetation after two months of corticosteroid therapy.

Pulmonary hyalinizing granuloma with ureteric fibrosis: A case report and review of relevant literature.

A 52-year-old, asymptomatic patient presented with bilateral lung nodules on chest radiograph. She was diagnosed to have "pulmonary hyalinizing granuloma" on an open lung biopsy. We review the clinical features of this rare disease.

Pneumocystis carinii pneumonia in HIV infected patients from Mumbai.

OBJECTIVE: Pneumocystis carinii pneumonia (PCP) is believed to be rare in the developing world and no large prospective Indian series have been reported to date. The present study was conducted to study the clinical profile and outcome of PCP in patients with HIV infection. METHODS: All HIV positive patients with PCP admitted over 4 years (2000-2003) to a tertiary referral centre in Mumbai were prospectively studied. RESULTS: There were 38 patients with proven PCP from 300 HIV admissions. The patients with PCP were predominantly male (M: F = 5.4:1), with a mean age of 40.1 years. The median CD4 count of the PCP group was 96 cells/microL. Bronchoscopy was needed to make a definitive diagnosis in 17 of the 38 patients. PCP co-existed with tuberculosis in 4 of the 38 patients. The mortality of the group was high at 15.8% with all patients needing ventilatory support dying. CONCLUSIONS: PCP is not an uncommon infection in Indians with advanced HIV. Lack of recognition has probably been responsible for the absence of any large series from this country. In our series of hospitalised HIV positive patients, PCP was the second commonest pulmonary disease after tuberculosis accounting for 32% of pulmonary admissions and 13 % of all HIV positive admissions.

Molecular diagnosis of cystic fibrosis in Indian patients--a preliminary report.

AIM: The aim of the study was to screen for the common deltaF508 mutation and the poly T polymorphism and to determine their frequency in the cystic fibrosis transmembrane conductance regulator (CFTR) gene among the suspected CF cases referred to our clinical care centre for sweat chloride tests. METHODOLOGY: Sweat and EDTA blood samples were obtained from 23 clinically suspected cystic fibrosis (CF) cases. Sweat was estimated by pilocarpine iontophoresis procedure. Poly T polymorphism was detected by the multiplex-PCR based on ARMSTM technique and deltaF508 mutation by PCR-mediated site-directed mutagenesis method. RESULTS: Five cases, mainly with respiratory abnormalities and followed by steatorrhea had elevated sweat chloride levels (> 60 mmol/l), three of them, each with nutritional, respiratory and pancreatic abnormalities were borderline (40-60 mmol/l) and the remaining 15 clinically suspected CF cases had normal sweat chloride levels (< 40 mmol/l). The 9T variant was frequently observed (75%) in cases with elevated sweat chloride, including those exhibiting borderline values; with no 5T variant. The 7T was the most common variant (77%) observed in the cases with normal sweat chloride, with only one 5T variant (33%). Of the five cases with high sweat chloride, four cases were homozygous for deltaF508, whereas one was heterozygous with borderline sweat chloride, thus showing an overall frequency of 56.25% in the CF chromosome. DeltaF508 was found to be present with the 9T variant in all the instances. CONCLUSION: The presence of the 9T variant along with elevated sweat chloride levels can be used to predict a high risk of the individual harboring the severe deltaF508 mutation. It would be advisable to test for to the deltaF508 mutation along with the sweat chloride estimation in all the critically suspected CF cases diagnose CF with a higher degree certainty.

Herpes simplex esophagitis in immunocompetent individuals.

Herpes simplex esophagitis commonly occurs in immune-compromised individuals. We report the condition in two immunocompetent individuals (one presenting with retrosternal pain and diarrhea and the other with dysphagia and fever) and in two patients with obstructive airway disease who had received corticosteroid therapy. The first two did not receive treatment, one was lost to follow up and the other is asymptomatic two years later. The latter two patients received acyclovir therapy.

Spontaneous resolution of diffuse alveolar septal amyloidosis.

Amyloidosis is a generic term for a heterogeneous group of disorders associated with deposition of protein in an abnormal fibrillar form. Amyloidosis can be hereditary or acquired, localised or systemic and potentially lethal or merely an incidental finding. We report a case of a male who was totally asymptomatic and was incidentally detected to have lung amyloidosis. He was not given any treatment but kept under observation., His chest radiograph (CXR) and high resolution computed tomograph (HRCT) of the chest cleared spontaneously after one year and the patient continues to remain asymptomatic even after two years.

Lymphocytic interstitial pneumonitis associated with autoimmune hepatitis.

A 49-year-old woman was diagnosed as autoimmune hepatitis and started on steroids and azathioprine. Subsequently, she developed fever; chest radiograph showed lower lobe nodular opacities. Bronchoalveolar lavage and transbronchial lung biopsy confirmed the diagnosis of lymphocytic interstitial pneumonitis.

Prader-Willi syndrome with sleep disordered breathing: effect of two years nocturnal CPAP.

We report a case of a 3-year-old child with Prader-Willi syndrome who had severe sleep disordered breathing with daytime hypersomnolence. His daytime blood gases showed type II respiratory failure. He was effectively treated with continuous positive airway pressure (CPAP) and has used this form of therapy for 2 years now with improvement in daytime somnolence, improved mental acuity and normalisation of daytime blood gases.

Hepatic sarcoidosis responding to chloroquine as steroid-sparing drug.

We report a 59-year-old lady who presented with exertional dyspnea and was diagnosed to have sarcoidosis. She responded to steroids, but one year later developed abdominal symptoms and was found to have hepatosplenomegaly. Liver biopsy showed non caseating granulomas. As she had developed steroid-induced diabetes she was started on chloroquine and responded well with regression of the liver and spleen during one year of treatment.