Interstitial Lung Disease due to Siderosis in a Lathe Machine Worker.

Since its first description in 1936, siderosis of lung has been considered a benign pneumoconiosis due to absence of significant clinical symptoms or respiratory impairment. Subsequently, authors have questioned the non-fibrogenic property of iron. However, siderosis causing interstitial lung disease with usual interstitial pneumonia (UIP) pattern has not been described in the past. We report a case of UIP on high resolution computed tomography, proven to be siderosis on transbronchial lung biopsy in a lathe machine worker.

Pulmonary choriostoma in a case of tuberous sclerosis complex.

A 52 years old lady was diagnosed to have Tuberous Sclerosis Complex (TSC) on the basis of 2 major and one minor criterion. She had family history of similar complaints in her sister and two sons. There was involvement of kidney in the form of angiomyolipoma, skin in the form of facial angiofibroma and teeth with a dental pit. She had an unusual lung involvement in the form of multiple small choristomas. Choristoma was diagnosed on transbronchial lung biopsy and was present in the form of disorganised striated muscles. The reported pulmonary manifestations of TCS i.e. lymphangioleiomyomatosis (LAM) and multifocal micronodular pneumocyte hyperplasia (MMPH) are types of hamartomas. Hamartomas and choristomas are both types of disorganized tissue. 'Choristoma'of lung in TSC however is not reported. Clinopathological correlation of pulmonary hamartoma and choristoma, and treatment in TSC has been discussed.

Pulmonary rehabilitation in resource poor settings.

Pulmonary rehabilitation is an important component in the management of chronic obstructive pulmonary disease (COPD) and other chronic respiratory diseases. The goal of rehabilitation is to evaluate various systems, treat optimally, improve dyspnoea and health-related quality of life. It is a multi-disciplinary approach and involves a physician, a psychiatrist, a dietician and a physiotherapist. However, in a resource-poor setting, even an experienced physician alone may suffice. Exercise training is the backbone of pulmonary rehabilitation, which may be hospital-based or home-based. Though, the previous trials have shown benefit with hospital-based rehabilitation, several recent studies have demonstrated significant improvement in the 6-minute walk test and quality of life even with unsupervised, home-based pulmonary rehabilitation. In the resource-poor settings, the goal of rehabilitation may be achieved by incorporating regular unsupervised exercise in daily routine. This is not only better accepted and more suitable but is also more feasible for the lifelong maintenance of rehabilitation.

Marfan syndrome with multiseptate pneumothorax and mandibular fibrous dysplasia.

We describe a rare case of pneumothorax due to Marfan syndrome associated with fibrous dysplasia of the mandible. Marfan syndrome and fibrous dysplasia were possibly due to a common etiological factor. The association between the two and other tumors described in literature related to Marfan syndrome is discussed.

25-year old male with pleural thickening.

The growth of some of the adenocarcinomas is virtually identical to that of malignant mesothelioma, also known as pseudomesotheliomatous adenocarcinoma of lung. Their differentiation on the basis of histopathology can pose diagnostic difficulties; hence immunohistochemistry and electron microscopy may be required for further differentiation.

Clinical profile of diseases causing chronic airflow limitation in a tertiary care centre in India.

SETTING: Pulmonary department of a tertiary health care centre in India. OBJECTIVES: To study the clinical profile of diseases causing chronic airflow limitation (CAL). DESIGN: Standard criteria were used for the diagnosis for various diseases causing CAL. Severity of CAL was graded using forced expiratory volume in one second (FEV1)% predicted. Pulmonary hypertension (PH) was confirmed by 2-dimensional echocardiography with colour Doppler. RESULTS: Two hundred sixty eight consecutive patients of CAL, age range 12-75 years, 172 men and 96 women were included in the study. Sixty three percent had asthma, 17% had chronic obstructive pulmonary disease (COPD), 6% had bronchiectasis, 13% had obliterative bronchiolitis (OB) and 1% had occupational airway disease. 98% of COPD was caused by tobacco smoking, of which 84% were bidi smokers. Ninety-two percent cases of OB were post infectious, 78% being post tuberculosis. 37% of COPD, 33% of bronchiectasis, 53% of OB and 22% of asthma had severe airflow limitation. PH was observed in 15%, 19% and 13% cases of COPD, OB and bronchiectasis, while none with asthma had PH. CONCLUSION: Although, asthma was the leading cause of CAL, it caused least functional impairment. CAL due to OB was as common as COPD. Bidi smoke was an important cause of COPD, while respiratory infection was common cause for OB.

Sleep apnea.

OBJECTIVE: To study clinical presentation of sleep disordered breathing (SDB) in children, their causative factors and response to treatment. METHODS: A retrospective study of clinical data nd results of overnight polysomnography done at baseline and after therapy were reviewed in 56 patients under 18 years of age. RESULTS: Of the 56 patients included in the study 23(41%) cases were positive for SDB. 12 (52.1 %) patients had craniofacial abnormalities, 4 (17.3%) had neuromuscular and skeletal disorders, 2 (8.6%) had adenotonsillar hypertrophy, 1(4.3%) had bilateral vocal cord palsy and 3 (13%) had sleep apnoea associated with multisystemic disorders. Post-operative data showed improvement in all 6 cases of craniofacial abnormalities and both cases of adenotonsillar hypertrophy. Positive airway pressure treatment was useful in cases with obstructive sleep apnea (OSA) due to vocal cord palsy, thoracic scoliosis, systemic disorders and central hypoventilation. CONCLUSION: 41% of suspected cases were detected to have SDB. Craniofacial abnormality was the leading cause of OSA in the present study. Surgical correction improved symptoms apnea-hypopnea index (AHI) and desaturation in cases of craniofacial disorders and adenotonsillar hypertrophy. Vocal cord palsy, thoracic scoliosis, hypoventilation and systemic disorders associated OSA responded to positive airway pressure ventilation.

Late onset hypoventilation syndrome: is there a spectrum of idiopathic hypoventilation syndromes?

We report a case of late onset central hypoventilation syndrome (LO-CHS) with hypothalamic dysfunction (HD) and ganglioneuroma presenting at the age of ten years. LOCHS-HD is now a well-established syndrome; the key is to investigate each child's history and presentation to expeditiously offer the most accurate diagnosis and optimal management.

Acromesomelic dysplasia with bronchiectasis.

We report a rare case of acromesomelic dysplasia with bilateral bronchiectasis and obstructive sleep apnoea. Diagnosis of acromesomelic dysplasia was based on radiographs of whole skeleton.

Clinical and laboratory observations of tuberculosis at a Mumbai (India) clinic.

OBJECTIVES: To study the positivity of sputum acid fast bacilli (AFB) smears in patients with pulmonary tuberculosis using 24 hour sputum collection. To detect HIV seropositivity in patients suffering from tuberculosis, and to analyse the pattern of tuberculosis disease in this subgroup. To determine the outcome of patients treated with directly observed therapy. SETTING: The tuberculosis referral unit of a tertiary care hospital. DESIGN: A total of 893 consecutive patients with tuberculosis, diagnosed between 1 November 2000 and 30 September 2002, were included in the study. An HIV test was performed in all patients, with adequate counselling and informed consent. Treatment was prescribed as per World Health Organisation treatment categories. RESULTS: Out of 893 patients with tuberculosis, 695 had pulmonary tuberculosis and 198 had extrapulmonary tuberculosis. Out of the 695 pulmonary tuberculosis patients, 673 (96.8%) were sputum smear AFB positive. Overall, 71 patients (8.0%) were HIV positive. The pattern of tuberculosis was the same in HIV seropositive and seronegative patients. Treatment outcome could be analysed in 112 out of 150 patients: 78 patients (70%) were declared cured or completed treatment. CONCLUSIONS: Sputum smear AFB could be a very sensitive test when a large quantity of sputum is used. The presence of HIV coinfection does not alter the clinical presentation. Only 70% of patients treated were cured/completed treatment, in spite of a strict directly observed therapy.

Hemorrhagic pleural effusion due to pleural hemangioma.

We report a rare case of isolated pleural hemangioma presenting as recurrent, haemorrhagic pleural effusion. Chest radiograph and computed tomography of chest was suggestive of left apical tumour. Diagnosis of hemangioma was revealed only after thoracotomy and excision of the tumour mass.