A case of renal cell carcinoma and angiomyolipoma in an adolescent girl.

We describe a case of renal cell carcinoma in the right kidney together with an angiomyolipoma in the left kidney, encountered in an adolescent girl at Potchefstroom Provincial Hospital, North West Province, South Africa.

Lone ventricular cardiomyopathy, 1993-1996.

OBJECTIVE: To identify subjects with univentricular idiopathic structural and/or functional myocardial disorders (as defined) and to describe the characteristic features. DESIGN: Over a period of 4 years, 1993-1996, all adult subjects were obtained consecutively from a centralised referral cardiological service. The subjects had to fulfil a set of formulated diagnostic criteria for each isolated type of univentricular disease-symptomatic or asymptomatic. The subjects were diagnosed on the basis of clinical features, supported by electrocardiographic, radiological and echocardiographic evidence of lone ventricular disease, with a further definition of abnormalities based on appropriately selected standard left and right heart assessments, inter alia: (i) cardiac catheterisation, including coronary arteriography and pulmonary angiography; (ii) radio-isotope studies-mibiscan; (iii) ventilation perfusion scan; and (iv) laboratory tests to identify likely cause(s) of diffuse myocardial damage as well as to recognise nonspecific effects of tissue damage and organ dysfunction. SETTING: A referral cardiological service of a tertiary academic hospital, which provides a consultative service for inpatients and ambulatory cases. All subjects were studied on admission to hospital. PARTICIPANTS: A set of criteria was formulated for each category of lone ventricular myopathy. A total of 30 patients were thus identified and included in the study-men and women ranging in age from 18 years to 84 years, with an average of 48 years. All were investigated after admission to hospital by means of a detailed set of investigations that rigorously excluded overt or occult causes of diffuse myocardial damage and any severe myocardial dysfunction secondary to haemodynamic conditions. Seven patients with significant coronary artery disease were excluded. Any subject with pulmonary or systemic hypertension was also excluded. MAIN OUTCOME MEASURES: Total number of patients, number of patients in each subgroup were analysed by age, sex, clinical features, and by special investigation. The mode of presentation and electrocardiographic features were analysed separately. RESULTS: Twenty-two left ventricular and 8 right ventricular cases of lone ventricular cardiomyopathy were diagnosed. All but 1 patient with right ventricular disease were symptomatic and 5 subjects with left ventricular myopathy were incidentally discovered. There were 17 men and 13 women in the series. Of the 8 patients with right ventricular disease, 6 were women, while of the 22 patients with left ventricular cardiomyopathy, 15 were men. CONCLUSION: The study supports the previously described existence of lone ventricular idiopathic cardiomyopathy. Further studies are, however, indicated in order to define its prevalence and nature more accurately, as well as to describe any relationship with univentricular cardiomyopathies, and define the characteristics of each category and the possible evolutionary patterns. Right ventricular cardiomyopathy is a new entity which may pose difficult diagnostic challenges, while left ventricular disease is generally accepted as a stage in the clinical spectrum of classic idiopathic dilated cardiomyopathy.

The Cardiothoracic Outreach Programme--a pilot project.

OBJECTIVE: To test the practicality, safety and benefits of major cardiothoracic surgery in two rural hospitals. DESIGN: Analysis of morbidity and mortality outcomes of a random collection of 35 patients, who underwent diverse surgical procedures. At each visit, the cardiothoracic team of Ga-Rankuwa Hospital-one surgeon, three registrars, two medical officers, six nurses, and four perfusionists-moved all equipment for major surgery, including bypass machines, to two small rural hospitals. Ga-Rankuwa Hospital, as a tertiary hospital attached to a medical school (Medical University of Southern Africa), mounted an outreach programme on a trial basis. The exercise was designed to render assistance, offer decentralised services, and test the skills of the cardiothoracic team in an environment where high-technology procedures have never been undertaken by the local health professionals. SETTING: Two rural hospitals, viz. Tintswalo and Mankweng, in the referral area of MEDUNSA, with no conventional ICU facilities. The support services for operative, pre-operative and postoperative care were very basic. The local personnel consisted only of general nurses and medical officers. None had experience of high-technology or complex theatre work. PARTICIPANTS: Thirty-five randomly selected patients of both sexes with ages ranging from 11 years to 64 years. Pre-operative diagnoses of diverse cardiac and lung conditions were made. The operations performed comprised 35 major procedures, including open heart operations and major lung procedures. The personnel comprised the MEDUNSA cardiothoracic team, who were assisted by local nurses. INTERVENTION AND OUTCOME: Thirty-five patients underwent 35 major procedures, all under general anaesthesia. Twenty cardiopulmonary bypasses were performed. There was 1 intra-operative death, due to low-output state. Intra-operative morbidity occurred in 2 of the 35 operations. These consisted of a cerebrovascular accident (CVA) (air embolism), and a temporary heart block. Late outcomes (after 1 week) were also analysed; the incidence was 1/35 operations. This was a CVA due to a left atrial appendage clot. Staff morale at the local hospital improved remarkably. The process of teaching the local professional nurses was surprisingly easy. Benefits included a decreased referral rate (100%), lower costs (transport, medicines, operations), excellent patient and community confidence in the work of the hospitals (increased hospital outpatient numbers), and political support (new tools, upgraded facilities and new equipment). RESULTS: The exercise was a resounding success in both measurable and general terms. All operations were successfully performed, with very low adverse outcomes (morbidity, mortality) and good quality of life for all the subjects. The safety and cost-effectiveness of cardiothoracic surgery under primitive conditions were demonstrated. The standard of patient care improved, and local staff acquired good basic skills in patient care. The referral patterns changed for the better and the confidence of the community in the services was enhanced. The risk/benefit ratio of the exercise was commendable. The feasibility of an extended service was encouraging.

Iron deficiency anaemia and pica.

Pica was a surprise finding in 8 cases of severe iron deficiency anaemia of unknown cause and 2 patients with pregnancy-related anaemia who had a mixed picture of iron deficiency and megaloblastic features owing to folic acid deficiency. The pica cases demonstrated an apparently consistent pattern: severe anaemia, young age, normal psychiatric assessment, good nutrition and lack of an overtly disadvantaged background. Pica was significantly absent in other forms of anaemia in a series of 53 subjects. The literature is briefly reviewed to highlight the unanswered questions about this disorder. The cases reported do not furnish any clues to the enigma of pica, but reveal an obscure form of idiopathic iron deficiency anaemia. Pica may be commoner than is thought.

Aspects of hypertension among blacks.

The universal prevalence of arterial hypertension among Blacks is no longer disputed, although there still remain unanswered questions. Hypertension among Blacks is nearly always primary (85% of cases), while the secondary form is synonymous with some type of renal disorder. A few 'associations' of hypertension exist. Diet may be significant, especially potassium deficiency as a result of high starch intake, low intake of potassium and other practices. Enigmatic clinical aspects include: (i) the absence of symptoms generally, especially in the presence of established severe clinical disease with and without target organ damage; (ii) the clinically apparent selective target organ damage; (iii) labile blood pressure readings at any stage; (iv) cerebral thrombosis and frequent blood pressure normalization; (v) cardiomyopathy in relation to hypertension; (vi) the discordance of morbid events; (vii) dramatic isolated morbid events, which suggest special vulnerability.

Can hypertension masquerade as congestive cardiomyopathy?

Cardiomyopathy and hypertension are both common in Black patients, and confusion may arise between them. A series of 19 patients admitted to hospital in congestive cardiac failure had been diagnosed as cases of cardiomyopathy but were shown to have persistent hypertension. The differential diagnosis is discussed.

Herb use and necrodegenerative hepatitis.

Twelve patients with herbally-induced hepatitis are described and the clinicopathological features of their illness, which seem to present a recognisable spectrum, are discussed. The nature and the severity of the histological changes seem to correlate with the clinical manifestations and the immediate prognosis. Laboratory tests, especially liver function studies, are of limited diagnostic and prognostic value. The plants which contain the responsible toxins, have been identified in this country and in other parts of Southern Africa. A short list is provided of, apparently, the commonest medicinal plants in Lesotho. Many more toxic plants, however, are used in food and in diverse traditional medications. Senecio species are the principal source of hepatotoxic alkoloids, especially pyrrolizidines. Experimental studies ann evidence of similar disorders in animals, have thrown some light on the pathogenic mechanisms of these hepatotoxic and possibly hepatocarcinogenic agents. The disease in humans probably results from a combination of factors.