Haemoglobin H Disease-Woodrow et al.
SummaryThe occurrence in an English family of two brothers with a mild chronic haemolytic anaemia associated with thalassaemia and the presence of haemoglobins H and Bart's is described. Splenectomy was performed in one of the brothers with symptomatic improvement.The clinically unaffected father and paternal grandmother showed haematological evidence of thalassaemia, but the mother was haematologically normal.We are grateful to Dr. E. T. Baker-Bates for his encouragement and for permission to report this family. Dr , 1964, 1, 38 This case is presented to illustrate a potential danger of noninflammable paint-strippers and to raise the question of safety.rcautions. Their active components are chlorinated hydrocarbons, which can oxidize to form phosgene.
CA&E HISTORYThe patient, an interior decorator aged 67, has had chronic bronchitis and a quiescent duodenal ulcer for several years. After ight hours in a small unventilated room heated by a paraffin stove he mperienced breathlessness, giddiness, a tight feeling across the chest, and headache. On admission to hospital next morning the ireathlessness was worse, and he had anorexia and retrostemal pain.He was cyanosed, sweating, and tachypnoeic, with negligible chest rXatnsion and extensive coarse rales in both lungs except the right }owa lobe; pulse 86, regular; B.P. 95/60. He had extreme right hypochondrial tenderness. Thorough examination revealed no other abnormality.Investigations showed: Hb 118% ; W.B.C. 20,000/c.mm. (P. 38%, L. 11%, M. 1%); E.S.R. 4 mm./hour; blood urea 56 mg./ 100 ml. Serum electrolytes and liver-function tests revealed nothing abnormal. Chest x-ray examination showed diffuse bronchiolitis in all areas except the R.L.L. Barium-meal examination showed a large hiatus hernia and duodenal bulb deformity.The alimentary symptoms were thought to be due to oesophagitis and reactivation of the duodenal ulcer. Methylene dichloride itself produces epigastric pain, and the hydrocortisone administered might have been a contributory factor; but as the symptoms began so soon after contact and as the patient had been using paint-strippers previously without symptoms, dissolved phosgene was their most likely cause. Improvement was slow. Dyspnoea and hypotension persisted for four days, during which time he required intermittent oxygen, intravenous saline, potassium, and glucose. Penicillin and streptomycin were given for 10 days, together with hydrocortisone 100 mg. b.d., which was gradually reduced and after a further week was stopped. Analgesics and antacids were administered symptomatically. Nausea and vomiting continued for one week and anorexia and intractable retrosternal and epigastric pain for four weeks after admission. His chest was clinically and radiologically normal after two weeks. He was discharged five weeks after admission, but lassitude, weakness, and hypochondriasis continued for three months.
COMMENTPhosgene caused 80% of the gas deaths in the 1914-18 war. Rare cases result from its use industriall...
