Case report. Transient nonketotic hyperglycinaemia: ultrasound, CT and MRI

We report a case of transient nonketotic hyperglycinaemia in which radiography correlated closely with clinical and biochemical findings. Only 5 patients have been previously described with this transient form of nonketotic hyperglycinaemia. Among the radiographic findings, thinning of the corpus callosum is the most characteristic. Received: 26 June 1996 Accepted: 29 November 1996     

Severe hypoxemia due to a right-left shunt at the level of the foramen ovale. A complication of right ventricular infarct]

A 76-year-old woman with an inferior wall myocardial infarction, with right ventricular involvement, developed severe arterial hypoxemia with neurological involvement. Pulmonary edema or embolism and chronic obstructive pulmonary disease were ruled-out, and a right-to-left shunt was demonstrated by contrast echocardiography at the level of the foramen ovale. After inotropic support and oxygen supplementation, the patient recovered, although with significant neurological sequelae. No focal lesions were detected in the central nervous system by computerized tomography. Hypoxemia improved, coinciding with the disappearance of right-to-left shunt by contrast echocardiography.     

Low serum selenium concentration in a healthy population resident in Catalunya: a preliminary report

Serum selenium levels were determined in 92 healthy subjects [40 men, 52 women; mean age 33.5 +/- 1.6 (s.e.m.) years, range 16-71 years] living in the province of Barcelona, Catalunya, Spain. Only well-nourished individuals with unremarkable clinical history, normal blood chemistry and haematological tests were selected. The subjects were divided into 6 age groups for each sex. Serum samples were analysed using a modification of the standard electrothermal graphite furnace atomic absorption spectrophotometry method to shorten the procedure while maintaining its accuracy. A significant correlation (y = -1.31 + 1.51 x; r = 0.9967, P less than 0.001) was observed between our method and the standard assay method. The mean serum selenium concentration was 60.39 micrograms/l, 95 per cent CI 53.35-67.45 micrograms/l. There were age- but no sex-group differences (P less than 0.001). This result is similar to that found in countries whose low selenium levels have been related to an increased risk of some disease states. Clinical and health implications of this suboptimal selenium status are discussed.     

Adenocarcinoma at an ileostomy site after colectomy for ulcerative colitis

In this article we present the tenth case, according to the literature, of adenocarcinoma occurring at a long standing ileostomy originally performed for ulcerative colitis. Study of this and previous cases demonstrates common factors such as a previous history of ulcerative colitis, a long interval until the appearance of symptoms, together with a similar clinical and pathological characterisation with invasion of neighbouring skin layers close to the ileostomy. Several pathogenic hypotheses are considered. We believe that local excision in oder to obtain the diagnosis is a better option than a sampling biopsy and that surgery should include a wide resection of the abdominal wall and intestine in order to comply with therapeutic criteria in malignant disease.     

Surgical results of performing R4 gastrectomy for gastric cancer located in the upper third of the stomach

Because gastric cancers located in the upper third of the stomach are difficult to detect at an early stage, the surgical results remain poor. We performed R4 gastrectomy as a radical procedure for 25 patients, involving complete resection of the latero-aortic and interaorticovenous lymph modes above and below the left renal vein, in combination with the ordinary R2 or R3 gastrectomy (the R4 group). These patients were compared with 156 others who underwent R2 gastrectomy alone (the R2 group). There were no significant differences in operation time, blood loss, or the incidence of complications between the two groups; however, when the survival rates of the patients with tumors invading beyond the subserosa were compared, the 5-year survival rate was found to be significantly higher in the R4 group than in the R2 group. Furthermore, in patients with para-aortic nodal involvement, a significant survival advantage was observed in the R4 group, as compared with the R2 group. These results suggest that the R4 gastrectomy is a rational approach for patients with advanced gastric cancer located in the upper third of the stomach.     

Porphyria cutanea tarda associated with autoimmune hypothyroidism, vitiligo and alopecia universalis.

The aetiology of porphyria cutanea tarda (PCT) has not been elucidated, but the possibility of an autoimmune mechanism has been proposed. We report a case of an unknown clinical combination of PCT with autoimmune hypothyroidism, alopecia universalis and vitiligo with thyroid and parietal cell circulating antibodies. This is highly suggestive of underlying autoimmune damage in this patient.     

Intraoperative radiotherapy in the multidisciplinary treatment of bone sarcomas in children and adolescents

From September 1984 to December 1989, 38 patients of pediatric age with localized bone sarcomas received intraoperative radiotherapy (IORT) as part of a multidisci plinary treatment program. The age ranged from 6 to 21 years. The tumor histologies were 22 osteosarcomas and 16 Ewing's sarcomas. Thirty-four had initial primary disease (90%) and 4 were treated for local recurrence (10%). IORT was used on 32 untreated patients and in 6 previously treated with external beam radiotherapy (EBR). The IORT field included the surgically exposed tumor bed area. Single radiation doses ranging from 10 to 20 Gy were delivered, using 6–20 MeV electron beams. The median follow-up time for the entire group is 25 months (2–65+ months). The projected 5-year disease-free and overall survival rates are 65% and 69%, respectively. One patient developed a local recurrence in each histological group: one chondroblastic osteosarcoma and one cervical Ewing's sarcoma. Six patients died from metastatic progression: 3 initially recurrent tumors and three primary disease cases. Severe neuropathy and soft tissue necrosis were seen in some patients as IORT related complications. IORT is a feasible technique to be integrated in multidisciplinary programs that may promote local control in pediatric and adolescent patients with bone sarcomas. Peripheral nerves are dose-limiting tissue structures for IORT.     

In vivo 23Na NMR studies of myotonic dystrophy

Myotonic dystrophy is an inherited multi-system disease. Its pathophysiology leading to muscle malfunction and damage is not well understood. ²³Na NMR spectroscopy was applied here for an in vivo comparative study of the calf muscles of 7 myotonic dystrophy patients at various stages of the disease and 11 healthy volunteers. Both the total sodium content, expressed as the ratio of the ²³Na and ¹H water signals, and the fast transverse relaxation time, T₂₁, determined from the triple quantum-filtered spectra, increased in correlation with the severity of the disease. The results demonstrate that ²³Na NMR enables the quantitation of myotonic dystrophy progression.