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Biomedical subjects

M B Forman

Publications and source records attributed to M B Forman.

86 records · Page 5Linked to original sources
PubMed1982

Surgical correction of combined supravalvular and valvular aortic stenosis in homozygous familial hypercholesterolaemia.

Although premature coronary atherosclerosis is a well-recognized complication of homozygous familial hypercholesterolaemia, involvement of the aortic root and valve has not been recognized frequently during life. Two cases of supravalvular and valvular aortic stenosis due to familial hypercholesterolaemia are described. Both patients underwent successful surgical correction of these lesions. The importance of relieving left ventricular outflow tract obstruction by a technique of aortic root enhancement and oblique insertion of a prosthesis is stressed.

Aortic Valve↗
PubMed1982

Precocious puberty associated with craniopharyngioma.

Craniopharyngiomas in children are usually associated with growth retardation and hypogonadism. A patient with a craniopharyngioma treated with radiotherapy, who later developed isosexual precocious puberty, is described. She was treated with cyproterone acetate with a good clinical response. We suggest that craniopharyngiomas may become a more frequent cause of precocious puberty now that conservative therapy has been advocated for the condition.

Child, Preschool↗
PubMed1982

Treatment of homozygous familial hypercholesterolaemia with portacaval shunt.

Thirteen patients with homozygous familial hypercholesterolaemia (HFH) were treated with portacaval shunt operations. One patient died postoperatively and the others were followed for 2-6 years. The main beneficial effects were regression of xanthomas and sustained falls in serum total and LDL cholesterol levels of about 18% in the majority of cases, sustained rises in HDL cholesterol values in most patients, and the long period of survival after the operation without further deaths or serious cardiac complications. Other favourable effects were improvement of angina pectoris, ejection systolic múrmurs or electrocardiograms in some cases. Growth and development of patients after the operation were normal and there were no clinical or biochemical signs of liver damage or portal systemic encephalopathy. Apart from the patient who died, negative or adverse effects of the operation included shunt closure causing portal hypertension and bleeding varices in 1 case, unsatisfactory clinical responses in half the patients, small or unsustained falls in serum total cholesterol in one third of cases, and failure of total cholesterol to fall below 12.0 mmol/l in any patient. Enlargement of the spleen appeared to be an invariable consequence of the operation and possible reasons for this are discussed. Finally the place of portacaval shunts in the management of HFH is reviewed.

Adolescent↗
PubMed1981

Monoclonal gammopathy in a patient with chronic active hepatitis. A case report.

A case of chronic active hepatitis associated with monoclonal gammopathy (MG) is reported. In addition, the patient had Bence-Jones proteinuria, which is said to be an unusual finding in benign MG. Although a polyclonal increase in immunoglobulins is not uncommon in patients with liver disease, MG is relatively rare. No evidence of malignant disease was found in our patient, but it should be noted that numerous malignant disorders occur with increased frequency in patients with benign MG. A further reason for careful follow-up in these patients is that the paraproteinaemia itself may become malignant with time.

Chronic Disease↗
PubMed1981

Hepatic involvement in systemic lupus erythematosus. A case report.

The existence of hepatic involvement in systemic lupus erythematosus (SLE) is still contested in the literature. We report a patient with SLE and deranged liver function tests, especially marked elevation of alkaline phosphatase levels, an association not hitherto described. These changes have persisted for 3 1/2 years with no evidence of chronic active hepatitis (CAH) on liver biopsy or biliary obstruction. Current concepts of liver involvement in SLE, 'lupoid' hepatitis and aspirin hepatotoxicity are reviewed.

Adult↗
PubMed1981

Monoclonal gammopathy associated with idiopathic stenosing papillitis. A case report.

Stenosing papillitis is a recently recognized cause of obstructive jaundice. Although many liver disorders are associated with a polyclonal increase in serum immunoglobulin levels, a monoclonal increase is relatively rare. A patient with monoclonal gammopathy associated with idiopathic stenosing papillitis is reported. The gammopathy has persisted for 3 years after surgical relief of the obstructive jaundice.

Adult↗
PubMed1981

Yersinia arthritis mimicking acute rheumatic fever. A case report.

A reactive or post-infectious arthritis is a well-known complication of yersinia enterocolitica infection and tends to occur in patients with the HLA-B27 haplotype. We describe such a patient, who in addition satisfied the revised Jones criteria for a diagnosis of acute rheumatic fever. Our case illustrates that arthritis due to Yersinia may mimic acute rheumatic fever both clinically and serologically. In areas where Yersinia infection is known to occur, infection with this organism should be excluded in all patients who satisfy the revised Jones criteria.

Adult↗
PubMed1981

Eosinophilic fasciitis in South Africa. A report of 2 cases.

Eosinophilic fasciitis was first described by Shulman in 1974 and is characterized by oedema of the extremities, peripheral eosinophilia, hypergammaglobulinaemia and inflammation of the deep fascia. The condition has not been previously reported in South Africa. We describe 2 cases seen at the Rheumatology Clinic of the Johannesburg Hospital recently. The most striking clinical features were the marked palmar fascial thickening and oedema of the hands and feet. The latter is said to be uncommon in this syndrome and mimicked the early changes of scleroderma. Furthermore, blood eosinophilia may also occur in scleroderma. Our patients illustrated the extremely variable laboratory features in this disorder. In accordance with previous findings in the literature, visceral involvement was absent in our cases. We suggest that in patients without Raynaud's phenomenon who present with scleroderma-like changes of the distal extremities, a diagnosis of eosinophilic fasciitis should be excluded by a full-thickness biopsy.

Adult↗
PubMed1980

A host of hypercholesterolaemic homozygotes in South Africa.

From 1972 to 1979 34 patients with homozygous familial hypercholesterolaemia were seen in one clinic in Johannesburg. All were Afrikaners and most lived in Transvaal Province. Their epidemiological, genetic, clinical, and biochemical characteristics were studied. The course of the disease varied considerably among the 34 patients, with no fewer than six surviving into their fourth or fifth decades. In some patients arterial atheroma was severe while cutaneotendinous xanthomas were slight and vice versa. Coronary heart disease was common but peripheral and cerebral arterial disease was rare. Another prominent finding was high concentrations of low-density lipoprotein cholesterol coupled with low high-density lipoprotein cholesterol values. The prevalences of homozygotes and heterozygotes with familial hypercholesterolaemia in Transvaal Afrikaners, calculated from this group of patients, were 1 in 30,000 and 1 in 100 respectively. These figures are the highest ever reported and may help to explain why South African whites have the highest death rate from coronary heart disease in the Western world.

Adolescent↗
PubMed1980

Pulmonary haemorrhage following Swan-Ganz catheterization in a patient without severe pulmonary hypertension.

A case of haemoptysis following pulmonary artery rupture by a Swan-Ganz catheter is described. The most likely pathogenesis appears to be spontaneous migration of the catheter into a peripheral vessel due to a redundant loop in the right ventricle. This, in association with possible hyperreactivity of the vessel, caused a tight fit; on inflation of the balloon a shearing force ruptured the vessel, with subsequent haemoptysis. Severe pulmonary hypertension does not appear to be an absolute perequisite for the occurrence of this rare but potentially fatal complication. The most important preventive measures are the early removal of any redundant loop of the catheter in the right ventricle seen on the radiograph taken after insertion, and gradual inflation of the balloon.

Blood Pressure↗
PubMed1980

Basal ganglia calcification in postoperative hypoparathyroidism.

The presence of bilateral basal ganglia calcification was sought in nine patients with post-operative and two patients with primary hypoparathyroidism, using computerized axial tomography (CAT) and plain skull radiographs. Calcification was detected with CAT in seven patients; five with post-operative and both with primary hypoparathyroidism, but in only one was it demonstrable on plain X-ray films. The presence of calcification appeared to be related to the duration of the disease and possibly the degree of control of serum calcium. Six of the seven patients with cerebral calcification also had cataracts. The incidence of basal ganglia calcification in hypoparathyroidism is probably much greater than previously recognized, especially in post-operative hypoparathyroidism.

Adult↗