If gas infection is suspected, vigorous treatment should be instituted: (1) The wound should be widely opened and a swab taken for bacteriological examination. (2) Blood transfusion should be commenced as soon as possible as these patients all have some degree of haemolysis. (3) Penicillin should be given as above. (4) Hyperbaric oxygen can be life-saving (Brummelkamp, Boerema & Hoogendyk, 1963...

Benign intracranial hypertension is uncommon, with a prevalence of 0 9 per 100 000 and a noticeable female predominance (8:1). Several associations have been reported including with proliferative haematological disease such as polycythaemia rubra vera, essential thrombocythaemia, and paroxysmal nocturnal haemoglobinuria. In a series of 29 men with benign intracranial hypertension an empty sella...

Osteopetrosis is a rare inherited genetic disease characterized by sclerosis of the skeleton caused by the absence or malfunction of osteoclasts. Three distinct forms of the disease have been recognized, autosomal dominant osteopetrosis being the most common. Autosomal dominant osteopetrosis exhibits a heterogeneous trait with milder symptoms, often at later childhood or adulthood. The aim of t...

We have observed congenital hypophosphataemic rickets in two sons of a marriage between first cousins, their mother being clinically and biochemically normal. Both patients are now approaching middle age. In addition to severe childhood rickets and lifelong hypophosphataemia, their disease is characterised by gross osteosclerosis with extraskeletal ossification, clinically persistent osteomalac...

Osteopetrosis is an inherited bone disorder characterized by general skeletal sclerosis. The cortex and spongiosa are increased in thickness and density. and the bone marrow spaces are reduced in volume [1]. Histopathologically, excessive retention of chondroosseous tissue and failure of remodeling of bones are the result of retarded osteocytic osteolysis and osteocytic chondrolysis [2] . Signs...

pyknodysostosis is a rare bone disease that has autosomal dominant trait. it is characterized by short stature, diffuse osteosclerosis, acro-osteolysis, finger, and nail abnormalities. other features include open fontanelles and sutures, relative proptosis and obtuse mandibular gonial angle with relative mandibular prognathism. intraoral features such as dental abnormalities also have been repo...

Disorders of calcium metabolism in childhood (except rickets) were regarded as uncommon until the recognition by Lightwood (1952) of the syndrome of 'idiopathic hypercalcaemia of infants with failure to thrive'. Previously there had been occasional reports of children considered to have AlbersSchonberg disease, hypervitaminosis D, primary hyperparathyroidism (Pratt, Geren and Neuhauser, 1947; P...