Gastric remnant recurrence--detection and implications for the management of gastric cancer

The ability of endoscopy to detect remnant recurrence of gastric cancer has been evaluated in patients, either as a follow-up procedure (17 patients) or after symptomatic referral (7 patients). Overall 15 out of 24 (62.5%) patients developed some form of recurrence. Fourteen out of 15 (94%) had local disease either alone or in combination with distant metastases. Remnant recurrence was observed in 11 of these and after excluding those referred with symptoms, the true incidence of remnant recurrence was 41.2%. Patients in the follow-up group had 'early' remnant lesions which were diagnosed sooner after surgery than in the symptomatic group who had 'advanced' remnant recurrences. The detection of premalignant histology occurred earlier in those who developed remnant recurrence and this short time interval could be used to predict recurrence. Despite the early diagnosis of remnant recurrence, resection of the remnant at second laparotomy was only possible in one patient as the majority had other sites of extensive recurrence. The implications for the management of 'early' and 'advanced' gastric cancer are discussed.     

Mortality from asthma in Western Australia

From a cohort of all 5760 male and 4979 female patients who were admitted to WA hospitals and were discharged with a diagnosis of asthma between 1976 and 1980, 265 deaths in men and 189 deaths in women were identified by the end of 1982. The standardized mortality ratio (SMR) for all causes of death for this cohort was 1.6 for men (P less than 0.001) and 1.7 for women (P less than 0.001). Both sexes showed a significant increase in deaths that were attributable to asthma (SMR, 57.9), chronic airflow obstruction (SMR, 9.3) and ischaemic heart disease (SMR, 1.3). The excess death rates for asthma were observed in all age groups, but those for chronic airflow obstruction and ischaemic heart disease were present in older age groups only. These findings indicate that asthma remains a potentially fatal disease in the Australian community. The excess mortality ratios for chronic airflow obstruction that were observed in patients who were admitted to hospital with asthma also suggest that asthma may result in irreversible airflow obstruction.     

Angelman's syndrome: a neuropathological study

Summary We report the neuropathological findings of a 3-year-old boy with Angelman's syndrome. The main abnormalities were macroscopic consisting of small temporal and frontal lobes which also showed disorganised and irregular gyri. The occipital lobes appeared flattened but were otherwise normal as were the parietal lobes. The brain stem and cerebellum appeared normal externally. Microscopically there was irregular distribution of neurons in layer 3 and in the cerebral white matter a few subcortical ectopic neurons were present in the temporal and frontal lobes. A single Purkinje cell heterotopia was seen but otherwise the cerebellum appeared normal.     

Brain temperature alterations and the retention of visual pattern discriminations in pigeons

Pigeons were taught visual discriminations. Immediately after they had reached criterion their forebrain was either left untreated or heated (by ≈1°C) or cooled (by≈2°C) for 30 min, with water flowing through a chronically implanted cannula system. The effects of the treatments on retention were assessed in retraining sessions. Both brain temperature alterations led to slight saving scores and marked, significant response latency advantages, compared with the control treatment.     

Simultaneous trisomy 9q3 and monosomy 5p in two children with der(5),t(5;9)(p15.1;q34.13): report of an extended family.

We present a family segregating for t(5;9)(p15.1;q34.13). Two cases with der(5),t(5;9), resulting in a partial duplication 9q34.13----qter and partial deletion of 5p15.12----pter, were ascertained. The phenotypes were consistent with features of both the cri du chat and trisomy 9q3 syndromes.     

Variable penetrance of familial pheochromocytoma associated with the von Hipple Lindau gene mutation, S68W. Mutations in brief no. 150. Online

Van Hippel-Lindau disease (VHL) is an autosomal dominantly inherited disorder, characterised by the development of clear cell renal carcinomas, CNS hemangioblastomas, retinal angiomas, pancreatic tumors, pheochromocytomas and hepatic cysts. Recently a number of families with dominant familial pheochromocytoma as the only clinical manifestation have been reported to carry mutations in the HVL gene. We describe a family in which a novel VHL S68W mutation was segregating and carrier individuals manifested with variable penetrance of isolated pheochromocytomas. Investigation of this kindred confirmed that a mutation in the VHL gene could produce isolated pheochromocytomas as the only clinical feature and was variably penetrant.     

X-linked intellectual handicap and precocious puberty with obesity in carrier females

Precocious puberty (PP) and intellectual handicap were present in 3 males in 2 generations of a Western Australian family. The 2 obligate carrier women were of normal intelligence but were grossly obese; one died of a cardiomyopathy at 55 years. The youngest affected male was found to have PP at 3 years. His mother was then pregnant, and because of her family history, was aware of the risk to have an affected male. Her mother had given birth to 2 sons with PP. By school age both of these males were mildly intellectually handicapped and this later became moderate. Apart from these 3 affected males, the 2 carriers lost 6 of their other 8 offspring, leaving only one normal woman and her unaffected son. The Fragile X test was negative on all males. This appears to be a new X-linked mental retardation syndrome, and a condition different from true idiopathic PP, or familial PP affecting both sexes, or PP with X-linked dominant inheritance.