PubMed Health⌕ Search

Biomedical subjects

G J Cullity

Publications and source records attributed to G J Cullity.

15 recordsLinked to original sources
PubMed1988

Fatal hepatic necrosis associated with multiple anticonvulsant therapy.

We report six fatal cases of hepatic necrosis in children associated with multiple anticonvulsant therapy. There was insufficient evidence to incriminate any single drug or combination of drugs, but it is noteworthy that sodium valproate was involved in only one case. We consider that an idiosyncratic metabolic reaction to the anticonvulsants was most likely. In five of the six cases hepatic failure was immediately preceded by a prolonged fit and we speculate that metabolic change from excessive neuronal activity may unmask the hepatotoxicity of anticonvulsants.

Anticonvulsants↗
PubMed1987

Cystic hamartomata of lung and kidney: a spectrum of developmental abnormalities.

We report on a developmental malformation of the lung and kidney which has not been previously described and which we have chosen to call "cystic hamartomata of the lung and kidney" to emphasize the non-malignant nature of these lesions. We also confirm a previous case report by Weinberg and Zumwalt [1977] as a different distinct disorder that results in a multifocal cystic hamartomata of the lung with associated marked parenchymal overgrowth of the kidney (the Weinberg-Zumwalt syndrome). These cases represent a spectrum of abnormal morphogenesis affecting both kidney and lung. Patients 1 and 2 presented during infancy with abdominal masses and hypertension due to bilateral multilocular cysts of the kidney with associated hamartomatous pulmonary cysts; patient 2 also had one area of cellular mesoblastic nephroma. Patient 3 demonstrated markedly hyperplastic renomegaly with medullary dysplasia in association with bilateral cystic hamartomata of the lungs. During the fifth week of gestation, the ureteric bud invades the unsegmented mesoderm that becomes the metanephric system, and the lung bud invades the splanchic mesoderm, which provides the stimulus for its growth. We suggest that the predominant pattern of a congenital kidney or lung hamartoma might reflect the timing of a prenatal neoplastic event affecting these developmental processes.

Hamartoma Syndrome, Multiple↗
PubMed1987

Atelosteogenesis: evidence for heterogeneity.

Four cases of neonatal death dwarfism resembling atelosteogenesis but with some distinctive radiographic and characteristic histopathologic features are reported. The name atelosteogenesis II is proposed for this entity.

Bone and Bones↗
PubMed1985

Medulloblastoma: a clinicopathological study of 42 cases.

Clinicopathological data have been collected for 42 patients with cerebellar medulloblastoma diagnosed and treated in Western Australia between the years 1961 and 1984. Thirty-one patients were male and 12 were over 14 years of age. In 31 patients the neoplasms were localized to the midline, while in 10 they were placed laterally in the cerebellar hemispheres. The remaining patient had a diffusely spreading neoplasm in the meninges of the posterior fossa. Thirty-nine underwent surgical treatment followed by postoperative radiotherapy. Three patients died in the early postoperative period. Twenty-four patients survived for one year or longer, 15 for two or more years, 10 for five years, and five survived longer than 10 years. The survival proportions estimated by the life-table method were 66% at one year, 48% at two years and 32% at 5 and 10 years. In one patient who survived for 23 years a meningioma developed, possibly due to radiotherapy.

Adolescent↗
PubMed1983

Inter-observer variation in the histopathological reporting of Hodgkin's disease: an analysis of diagnostic subcomponents using kappa statistics.

Levels of agreement between nine pathologists on the Rye classification of Hodgkin's disease and on diagnostic subcomponents used in applying the classification, were analysed by kappa statistics. Pathologists experienced comparatively little difficulty in agreeing on the presence of nodules and lacunar cells and hence best agreement was achieved on the nodular sclerosis category. Poorer agreement levels on the lymphocytic predominance, mixed cellularity and lymphocytic depletion categories were explained mainly by problems in the assessment of numbers of lymphocytes and abnormal reticulum cells other than Reed-Sternberg cells. Identification of the Reed-Sternberg cell, although of paramount importance to a diagnosis of Hodgkin's disease, appeared to have no great practical relevance to use of the Rye classification in this series of cases.

Cell Count↗
PubMed1981

The Rye classification of a population based series of Hodgkin's disease patients in Western Australia.

Data are presented on the incidence in Western Australia of subtypes of Hodgkin's disease which were obtained by review of all cases of the disease notified between 1960 and 1974. Observations were also made on within- and between-observer agreement on the use of the Rye classification by a group of 9 surgical pathologists, all in the day-to-day practice of pathology. Although many consensus diagnoses were made only after spirited discussion by the Group, the results suggested that general surgical pathologists can perform nearly as well as lymphoma experts. A comparison of the incidence of Hodgkin's disease and distribution of Rye subtypes in Western Australia with 4 other populations showed a similar overall incidence but appreciable variation in the relative frequency of the subtypes--mainly in the proportions of nodular sclerosing and mixed cellularity.

Adolescent↗
PubMed1981

A fatal case of meningoencephalitis due to a free-living amoeba of uncertain identity--probably acanthamoeba sp.

There are 2 main types of meningoencephalitis caused by free-living amoebae. The first is a well-defined acutely fatal disease resembling fulminating bacterial meningitis. It is caused by the single species Naegleria fowleri. The second is a more poorly defined disease that runs a subacute or chronic course and is characterized by focal granulomatous lesions in the brain. The causative organisms are probably Acanthamoeba sp. in most cases, but it is possible that other genera may be involved. The first case of the subacute form of the disease to be recognized in Australia is described. A 2 1/2-yr-old, previously well girl presented with ataxia and lower motor neurone paralyses. The cerebrospinal fluid was pleocytic and she was thought to be suffering from a relatively minor viral brain-stem encephalitis. Her symptoms persisted in a peculiarly fluctuating way for 30 d when she suddenly collapsed and died from an intracranial haemorrhage. Necropsy showed focal granulomatous lesions associated with necrotizing vasculitis in the basal regions of the brain. The lesions contained well preserved free-living amoebae which were morphologically different from N. fowleri and most closely resembled Acanthamoeba sp. The ultrastructure of the organisms was particularly well preserved and is described in some detail. Immunohistological studies also excluded N. fowleri but were inconclusive for Acanthamoeba or other genera of free-living amoebae. Difficulties with the diagnosis and treatment of this disease are discussed and some practical suggestions are made.

Amoeba↗
PubMed1977

The stomach in malnutrition.

Basal gastric acid output was reduced in 9 out of 14 infants and young children with malnutrition compared with 21 age-matched controls. In all the patients the response of the gastric mucosa to stimulation by pentagastrin was impaired, and gastritis of variable severity was present in 8 out of the 9 patients in who biopsies were performed. Impaired gastric acid secretion probably contributes towards bacterial overgrowth and diarrhoeal diseases in malnourished children.

Child, Preschool↗
PubMed1975

Ulceration and necrosis of vocal cords in hospital and unexpected child deaths.

Lesions are described in the vocal cords of an unselected group of 91 infants dying as "cot deaths", 11 stillbirths and 107 infants and children dying from conventional diseases. The lesions have been classified into six types. After allowances for the effects of intubation, the same incidence and type of change was present in all but the stillbirths. This argues against them being a specific pathogenic mechanism confined to the cot death situation. The aetiology and pathogenesis are unexplained but indicate the existence of an unsuspected laryngeal disorder that merits further study in mechanism leading to child death.

Autopsy↗