Bullous pemphigoid in association with cutaneous lesions specific to a myelodysplastic syndrome

Specific cutaneous lesions are a rare occurrence in myelodysplastic syndromes (MDS). The concurrent association of blistering skin lesions similar to those in bullous pemphigoid (BP), even though a rare event, suggests that BP may be a paraneoplastic syndrome. We report an 86-year-old man who had a refractory anaemia with excess bone marrow blasts in transformation, who developed a generalized pruritic blistering eruption. Immunohistopathological tests showed subepidermal blisters with linear deposits of IgG and C3 along the basement membrane zone of the epidermis surrounding a tumoral dermal infiltrate of CD13+ and CD15+ cells. Immunoblotting studies using epidermal extracts revealed circulating IgG antibodies against three protein bands: a 210–215 kDa band. a 180kDa band which co-migrated with the BP 180 antigen, and a 190kDa band. The tumour infiltrate may have revealed antigenic determinants which led to the onset of BP. The concept of paraneoplastic pemphigoid remains to be either confirmed or invalidated by further epidemiological studies.     

MRI in hemiballismus due to subthalamic nucleus hemorrhage: An unusual complication of liver transplantation

A 60-year-old man developed hemiballismus due to an intracranial hemorrhage involving the subthalamic nucleus 8 weeks after orthotopic liver transplantation. The hemorrhage was thought to be due to alterations in cerebral blood flow following a period of hypotensive shock due to sepsis, in the presence of anticoagulant therapy and thrombocytopenia. This represents a rare neurologic complication of liver transplantation.     

Anomalies of the position of the spleen in the child Case report and review of the literature from 1896 to 1990

We report on the case of a 6-year-old girl admitted as an emergency with an acute abdomen with the diagnosis of peritonitis; clinical examination under anesthesia revealed the presence of an abdomino-pelvic mass. Laparotomy confirmed the diagnosis of torsion of an ectopic spleen. Splenectomy was required because the spleen was gangrenous. A review of the literature from 1896 to 1990 revealed reports of 74 cases. Epidemiology, etiology, pathophysiology, the diagnostic and therapeutic problems are discussed.

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Ektopische Milz beim Kind: Fallbericht und Literaturübersicht 1896–1990

Zusammenfassung Anhand eines Fallberichtes und einer Literaturübersicht über 74 Fälle werden Epidemiologie, Ätiologie, Pathophysiologie, Diagnostik und Therapie der im Kindesalter sehr seltenen ektopen Milz besprochen. Die klinischen Zeichen reichen von unspezifischen abdominellen Beschwerden bis hin zum Vollbild eines akuten Abdomen (50%). Die praeoperative Diagnostik richtet sich nach Klinik, Abdomenleeraufnahme und Sonographie, evtl. Computertomographie. Aufgrund der hohen Torsionsraten besteht generell eine Operationsindikation, wobei die Entscheidung über Splenopexie oder Splenektomie sich an dem Lokalbefund orientieren sollte. Zur Splenopexie wurden mehrere Techniken mit gleich guten Resultaten angegeben.

     

Electro-encephalographic disturbances due to chronic toxin abuse in young people, with special reference to glue-sniffing

A study was carried out in order to document any abnormalities in the electro-encephalogram (EEG) that might appear in young adolescents who have deliberately inhaled the range of volatile substances loosely referred to as 'glue'. The EEGs of a group of 'street children' being assisted in a Johannesburg shelter were examined. The records were analysed for any clinical abnormalities and also subjected to spectral analysis in order to examine the overall characteristics of frequency, power and spatial distribution. The EEGs clearly revealed that, although at the time of the examination the subjects were ostensibly abstinent, both clinical and normative evidence of continuing brain disturbance was present. It was concluded that glue sniffing is likely to have long term electrocerebral sequelae.     

The in vitro fertilisation programme at Tygerberg Hospital and the University of Stellenbosch. Five years' experience, April 1983-January 1988

The results of the in vitro fertilisation programme at Tygerberg Hospital for the period April 1983 to January 1988 are presented. Of the 1117 laparoscopies performed, 825 patients reached the transfer stage. A live-birth rate of 9.3% was achieved. The pregnancy rate after transfer of 4 embryos was 25.9% compared with 15.4% after 2 embryos and 10.8% after 3 embryos (P = less than 0.0001). The multiple pregnancy rate was 2.8% in the group receiving 2 embryos and 11.7% and 10.4% in those receiving 3 and 4 embryos, respectively. Of the 77 successful pregnancies (90 babies), 1 baby died at 34 weeks' gestation as the result of abruptio placentae due to preeclampsia and 1 cot death occurred. The only congenital abnormality encountered was a cleft palate.     

The nature and extent of aggressive behaviour amongst people with learning difficulties (mental handicap) in a single health district

ABSTRACT. This paper describes the method and results of a survey of aggressive behaviour amongst people with learning difficulties. The study was confined to a single health district in the South Western Region of the UK with a general population of about 370000. At the time of the survey, the Handicap Register for the district recorded 1362 people as having a learning difFiculty. The overall prevalence of aggressive behaviour amongst people with learning difficulties for whom base population data was available was 17.6%. The lowest rate was identified amongst day facilities (9.7 %) and the highest in hospitals within the district (38.2%). The prevalence rate amongst those attending schools for children with severe learning difSculties was 12.6%. Proportionately more males than females were reported to present problems of aggressive behaviour. The gender difference appeared to be largely accounted for by the disproportionate number of men identified within day facilities. There was no significant evidence of an association between the person's gender and the presence aggressive behaviour within schools or hospitals. The results indicated that, although physical and verbal aggression were the most frequently reponed behaviours, many of the sample were also said to engage in other forms of challenging behaviour, particularly self-injurious, ritualistic, stereotypical and withdrawn behaviour. The risk of a serious or very serious injury to another person was very low; 0.7% or six people with learning difiSculties firom the district were reponed to be currently presenting such a risk. Similarly, only 2% of the base population (n=18) were reponed to be extremely difficult to manage. The implications of the findings are discussed.     

Technetium-99-labelled methylene diphosphonate uptake scans in patients with dialysis arthropathy

Patients on long-term haemodialysis suffer from dialysis arthropathy due to the deposition of dialysis amyloid. We investigated the use of 99Tc-labelled methylene diphosphonate bone scans in 17 patients as a possible in vivo diagnostic technique. In most clinically affected joints, with the exception of shoulders and hands, there was increased radioisotope uptake consistent with uptake by periarticular bone. In addition, we describe intense soft-tissue uptake around some clinically affected large joints. In contrast, control groups of patients on haemodialysis without arthropathy and patients without renal failure did not have increased uptake. A semi-quantitative scale of uptake was devised, and the following correlations were significant: pain perception and isotope uptake score in the ankles and feet, and the number of radiological lesions and isotope uptake scores in the wrists and knees. The following sites where the radioisotope might bind in the affected joints are proposed: amyloid deposits, areas of soft-tissue calcification, or areas of increased bone turnover. It is concluded that whereas the scanning technique cannot make a definite diagnosis of amyloid and, therefore, cannot be expected to supersede histological diagnosis, it is a useful adjuvant investigation, of particular importance in those patients unable or unwilling to undergo biopsy.