Scleroderma: a case report.

Scleroderma is systemic multi organ autoimmune disorder characterized by hardening of skin. Also known as systemic sclerosis. Estimated annual incidences of approximately 19 cases per million persons. The limited skin disease has a 10-year survival rate of 71%, whereas those with diffuse skin disease have a 10-year survival rate of just 21%. Risk is higher in women than men and peak in individuals aged 30-50 years. It has no definitive treatment. It may be limited or diffuse depending upon manifestations of symptoms or signs affecting internal organs especially lungs, heart, or kidney. We report a case of scleroderma with pulmonary hypertension and interstitial lung disease in our hospital who presented with tightening of skin, joint pain, dysphagia, and breathlessness. On examination skin appeared dark, shiny, and tight, with loss of hair, paraesthesia and digital ulceration. Patient also has history of Raynaud's phenomenon. On investigation, Scl-70 and ANA (antinuclear antibodies) by enzyme immunoassay came positive. HRCT thorax was suggestive of interstitial fibrosis and PFT revealed moderate restriction. On 2D echocardiography, mild pulmonary hypertension was present while barium swallow showed motility disorder involving oesophagus. On view of extensive systemic involvement like skin, respiratory system, gastrointestinal system and heart, we would like to present this rare disorder.

Effect of inhaled terbutaline on platelet activity in bronchial asthma.

Thirty five young subjects with long standing bronchial asthma were studied for the effects of terbutaline misthaler (500 micrograms) during acute asthma. Bronchodilators were omitted for a day before the test. Ten matched controls were also studied. In all subjects, basal IgE level (ELISA), pulmonary parameters (FVC, FEV, PEFR, FEF 25-75 and FEF 75-85) and platelet activity, (platelet clumping/150 WBC on smear, platelet adhesiveness, blood recalcification time and kaolin clotting time for platelet factor3) were compared. During acute asthma, the subjects displayed IgE levels > or = 500 IU/ml (n = 33), activation of clotting (n = 30) and eosinophilia > or = 450/cmm (n = 28). These changes were not seen in normal controls. Terbutaline resulted in a reversal of airway obstruction, mainly of small airways, while the platelet hyperactivity and blood hypercoagulability were rectified (P < 0.001). It is concluded that platelet activity increases in acute asthma and is corrected by inhaled terbutaline concomittant with the relief of bronchospasm.

Intensive respiratory care service. Organisation, orientation, system and future. Our experience of management of 288 cases.

Between Jan. 1983 and Dec. 1986, 288 patients with acute respiratory failure of varied aetiologies were admitted to tetanus and respiratory care ward. One hundred and twenty patients (41.66%) had primary respiratory diseases, 107 (37.15%) of poisoning, 24 (8.3%) had neuromuscular diseases and 37 (12.48%) had miscellaneous disorders. Ventilatory support was given for more than 6 hours to 118 patients. The overall survival was 61.81% and on ventilator 38.13%. The mortality was high with ARDS (100%), miscellaneous (100%) pneumonia with septicaemia (75%) and COAD (54.28%). Patient with COAD had high mortality with acidosis (pH less than 7.1, P less than 0.01), hypotension (systolic BP less than 90 mm of Hg, p less than 0.05) and oliguria (urine out put less than 400 ml/24 hours, p less than 0.05). Organophosphorus compound was the commonest poison (89.75%) and patients who had moderate to severe hypoxia (pO2 less than 60 mm of Hg), hypotension and an interval of more than 4 hours between the consumption of poison and admission (all P less than 0.05) expired; 68.18% expired within the first 72 hours. All the patients with primary neuromuscular paralysis and bronchial asthma survived. Hospital acquired infections (160 patients), retained secretions (108 patients) and hypotension (64 patients) were the commonest complications seen in the 288 patients. Staphylococcus aureus (32.14%) was the commonest organism isolated. Financial constraints, drug shortages and frequent failure of machines were other major problems in the intensive respiratory care unit.