A complication of mesenteric lymphangioma: case report and brief review of literature.

Mesenteric lymphangioma (ML) has only rarely been reported in the literature. These cases are rare enough to arouse interest when encountered. Usually, the cases are asymptomatic but may present with acute or chronic intestinal obstruction. We report an additional case of ML associated with intermittent intestinal volvulus in a 5 years old boy.

Pachydermatous eosinophilic dermatitis.

We report three South African black teenage girls with extensive pruritic papular lesions arising on a pachydermatous base, resembling severe atopic dermatitis or onchodermatitis. All three had peculiar hypertrophic genital lesions and peripheral blood eosinophilia. Histological studies showed an eosinophil-rich lymphohistiocytic infiltrate and variable fibrosis. Extensive fibrillar extracellular deposition of eosinophil granule major basic protein was demonstrated by an indirect immunofluorescence technique. A beneficial therapeutic effect was obtained using dapsone, prednisolone and cetirizine. The term pachydermatous eosinophilic dermatitis is proposed and its position among other conditions characterized by peripheral blood and skin tissue eosinophilia, is discussed.

Mid-pregnancy genetic terminations of pregnancy--postnatal assessment and management.

STUDY OBJECTIVE: To assess the need for and ability to apply a postnatal assessment protocol (PNAP), consisting of clinical examination, photographs, radiographs, chromosomal analysis and postmortems, of fetuses from mid-pregnancy genetic terminations of pregnancy. DESIGN: Prospective hospital-based study. SETTING: The maternity unit at the Pretoria Academic Hospital. MAIN RESULTS: Fifty consecutively delivered fetuses were assessed by means of the PNAP after genetic termination of pregnancy. A definitive prenatal diagnosis was available in 17 (34%) cases. In 33 (66%) cases the termination was undertaken on the basis of a provisional prenatal diagnosis which was confirmed postnatally in 12 (24%) cases; a definitive postnatal diagnosis could not be confirmed in 5 (10%) cases. In the remaining 16 (32%) cases a totally different postnatal diagnosis was obtained. The definitive postnatal diagnoses in the 28 cases with provisional prenatal diagnoses were confirmed by clinical examination in 13 (26%), by chromosomal analysis in 7 (14%), by postmortem in 5 (10%) and with radiographs in 3 (6%). On retrospective analysis, 22 of the 33 provisional prenatal diagnoses could have been confirmed using available radiographs, chromosome results and photographs only. CONCLUSIONS: Genetic terminations of pregnancy are a subgroup of stillbirths for which a PNAP is essential to ensure that appropriate postnatal genetic counselling can be given to the parent(s).

Retrospective determination of HIV-1 status by a PCR method on paraffin wax embedded sections.

AIM: To develop a simple but reliable polymerase chain reaction (PCR) method to determine the HIV-1 status of patients on formalin fixed, paraffin wax embedded lymph node tissue. METHODS: Fifty lymph node specimens, 20 from HIV-1 seropositive and 30 from HIV-1 seronegative patients, were analysed. Lymph nodes with a variety of disease conditions were included in the study. Tissue sections were treated with a DNA extraction buffer containing proteinase K and the crude cell lysate was used in PCR analysis. Nested primers were used to amplify HIV-1 DNA sequences coding for gag, pol and env proteins. PCR products were demonstrated by polyacrylamide gel electrophoresis. Results were then compared with HIV-1 serology of the patients from whom the tissue was obtained. RESULTS: The PCR method yielded a specificity of 100%, a sensitivity of 95%, a positive predictive value of 100%, and a negative predictive value of 97% when compared with HIV-1 serology. The kappa statistic (0.958) showed an excellent agreement between the PCR method and serology. Furthermore, HIV-1 DNA was demonstrated in lymph node tissue from a serologically unconfirmed acquired immunodeficiency syndrome case necropsied in 1982. CONCLUSION: This PCR method is a simple and reliable means of retrospectively determining the HIV-1 status of patients using formalin fixed, paraffin wax embedded lymph node tissue.

Human papillomavirus DNA in aerodigestive squamous carcinomas demonstrated by means of in situ hybridisation.

A series of 10 oesophageal and 10 laryngeal squamous carcinomas was examined by means of immunocytochemistry and in situ DNA hybridisation to demonstrate human papillomavirus (HPV) infection. Changes in the epithelium adjacent to the carcinoma were found in 5 of 10 oesophageal and 7 of 10 laryngeal carcinomas. Viral antigens could not be detected with immunocytochemistry in any of the specimens. HPV 6, 11 and 16 were detected in 3 oesophageal specimens. In one of these, HPV 16 was detected in normal as well as malignant cells. HPV 7 was detected for the first time in a laryngeal carcinoma. Our results confirm previous reports of possible HPV involvement in the pathogenesis of aerodigestive carcinomas.

Cardiovascular causes of death at Tshepong Hospital in 1 year, 1989-1990. A necropsy study.

Cardiovascular disease is the third most common cause of death in Tshepong Hospital in the western Transvaal, and the most common cause of death in patients older than 35 years. A prospective study was undertaken which included limited necropsies in 90 of the 167 cardiovascular disease deaths over 1 year. A reliable mortality pattern for cardiovascular deaths is described. Additionally, attention is paid to co-existing conditions. Conditions relating to cardiovascular disease, such as hypertension, benign hypertensive nephrosclerosis, atherosclerosis and obesity, were also evaluated. Cerebrovascular conditions were found in 32% of cardiovascular deaths. Intracerebral haemorrhage was found in 50% and cerebral infarction in 29% of cases. Fifty-seven per cent of cardiovascular deaths were due to cardiac conditions, the most common being pulmonary hypertension (31%), dilated cardiomyopathy and chronic rheumatic valvular disease (17% each) and hypertensive heart disease (14%). Forty-nine per cent of subjects were hypertensive, while 40% exhibited benign nephrosclerosis and only 3% of the examined vessels had signs of severe atherosclerosis. Tuberculosis was present in 13% of cases. The clinical diagnosis was the same as the final necropsy diagnosis in 38% of cases. These results emphasise the importance of performing necropsies to obtain reliable mortality statistics.

A randomized phase II study of acivicin and 4'deoxydoxorubicin in patients with hepatocellular carcinoma in an Eastern Cooperative Oncology Group study.

We analyzed 56 of 75 previously untreated patients with hepatocellular carcinoma who entered on a prospectively randomized trial of acivicin versus 4'deoxydoxorubicin (esorubicin). At least one episode of severe toxicity was documented in 23% of the patients on acivicin and 45% of those on 4'deoxydoxorubicin. Two patients responded to 4'deoxydoxorubicin. One response was partial, lasting 58 weeks, and one was complete, lasting more than 4 years. The 90% confidence interval for response is 1-20%. In view of a 45% rate of severe or worse toxicity with 4'deoxydoxorubicin, this drug cannot be recommended as treatment. There were no responses on acivicin.

Bizarre parosteal osteochondromatous proliferation of a phalanx.

A rare proliferative osteochondromatous malformation of a proximal phalanx is described. This malformation may mimic the appearance of a sessile osteochondroma or parosteal osteosarcoma on X-rays and may be mistaken for a low-grade osteosarcoma on histology, with disastrous therapeutic consequences. We found that cryotherapy followed by local excision was adequate to remove the tumour.

[The incidence of ischemic heart disease in white South Africans cremated in Pretoria, 1978-1985]. / Die insidensie van iskemiese hartsiekte onder blanke Suid-Afrikaners wat in Pretoria veras is, 1978-1985.

The prevalence of ischaemic heart disease was studied in a population selected on the basis of an application for cremation by the next of kin. In the age group 35-44 years 15.2% of the deaths were ascribed to ischaemic heart disease (IHD), while the corresponding figure in the general population was 27.2%. In females in the age group 45-54 years IHD was responsible for 9.3% of the deaths; the corresponding prevalence in the general population was 18.3%. The figures for all age groups and both sexes were 22% for the cremation group and 24.5% for the general population. In the economically active group (20-65) years, the figures were 20.0% for the cremation group and 30% for the general population. There was no significant difference in the IHD deaths between Afrikaans-speakers and other language groups. These findings are not necessarily applicable to the general population, as the cremation group is not truly representative, but the consistently lower prevalence of IHD suggests that there is over-reporting of this disease in unmonitored death certification.

Causes of death at Tshepong, a rural hospital, 1986-1987.

A prospective study was undertaken to determine causes of death at Tshepong Hospital in the western Transvaal over 1 year. Specific attention was paid to the accurate coding of causes of death; this entailed discussions with the attending doctor as well as 38 autopsies. Only 2.4% of deaths were coded under 'symptoms, signs and ill-defined conditions'. The 1046 deaths followed a typical Third-World pattern when classified according to the International Classification of Diseases (1979). Deaths during the perinatal period were additionally classified according to the recommendations of the Third Conference on Priorities in Perinatal Care in South Africa. The commonest cause of death was conditions originating in the perinatal period (19.2%), followed by infections and parasitic diseases (15.9%), diseases of the circulatory system (14.8%), endocrine, nutritional and metabolic diseases and immune-disorders (12.1%), and neoplasms (8.8%). The other main groups accounted for under 8% each. The commonest cause of death in all age groups above 35 years was diseases of the cardiovascular system, and the commonest cause of death among females aged 15 - 35 years was complications of pregnancy, childbirth and the puerperium. The hospital maternal mortality rate was 4.7/1,000 deliveries and the hospital perinatal mortality rate was 53/1,000 deliveries.

Normalization of skin appearance in a patient with scleromyxoedema after intensive chemotherapy for Hodgkin's disease.

A patient with scleromyxoedema was treated for 6 years with cytostatic drugs. During this time the skin lesions followed a fluctuating but progressive course. After 6 years she developed Hodgkin's lymphoma of the mixed cellularity type. Intensive cytostatic treatment given for Hodgkin's disease resulted in virtually complete disappearance of the scleromyxoedema lesions. The development of Hodgkin's disease is considered fortuitous and not due to the previous cytostatic drugs.

Malignant atrophic papulosis (Degos' disease)--diffuse involvement of brain and bowel in an African patient.

We report pathognomonic skin lesions of malignant atrophic papulosis, accompanied by lethal central nervous system and gastrointestinal disease in a 22-year-old black man. Histopathological studies of the brain and colon showed areas of infarction and an underlying vasculitis with leukocytoclasis.

A random phase II study of mitoxantrone and cisplatin in patients with hepatocellular carcinoma. An ECOG study.

Of 86 patients entered in an Eastern Cooperative Oncology Group (ECOG) random Phase II study of mitoxantrone (DHAD) and cisplatin (DDP) in primary liver cancer, 69 were eligible. Nine of the 13 ineligible patients were excluded after a pathology review. Sixty-one percent of the patients were North American, and 39% were South African. The most common severe or the worst toxicity on DHAD was hematologic; and to DDP, hematologic and vomiting. Of the 69 eligible patients, 21 experienced severe, life-threatening or fatal toxic reactions. Two patients treated with DDP had partial responses. With a 95% confidence interval, the true response rate to DHAD was less than 8%, and to DDP, less than 17%. The median survival time was 14 weeks on both drugs. Assuming a proportional hazards model, factors that are significantly associated with survival are patient performance status, the presence of the symptoms, raised bilirubin and hepatomegaly, and clinical evidence of cirrhosis. Any differences between survival rates for South African and North American patients were largely explainable by these factors.

The causes and consequences of chronic hepatic venous outflow obstruction.

The causes of chronic hepatic venous outflow obstruction are membranous obstruction of the inferior vena cava (group 1), hepatic venous ostial obstruction (group 2) and partial hepatic venous ostial obstruction (group 3). At the H. F. Verwoerd and Kalafong Hospitals, Pretoria, 155 cases have been diagnosed, almost all in the first group. Over 50% of the patients in this series had hepatocellular carcinoma; this association appears to be a constant finding world-wide. The most likely cause is a chronic hepatocaval thrombosis with organisation, leading to fibrous stenosis or membranous occlusion of the involved veins.

Granulocytic sarcoma of the kidney in a patient with acute myelomonocytic leukaemia. A case report.

A 26-year-old man with acute myelomonocytic leukaemia had been in remission for 16 months when he developed a painful swelling of the right testis. Intravenous pyelography showed a non-functioning right kidney. Surgical exploration revealed a granulocytic sarcoma of the right kidney and a right nephrectomy was performed. The patient refused further treatment and relapsed 3 months after the nephrectomy. Autopsy revealed a large greyish-white tumour mass occupying the pelvis and retroperitoneal tissue. Special techniques with immunoperoxidase and naphthol-ASD chloro-acetate esterase staining confirmed the histological diagnosis of granulocytic sarcoma. The literature is reviewed and the rarity of this case is discussed.