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Biomedical subjects

A A Gibson

Publications and source records attributed to A A Gibson.

At least 37 records · Page 2Linked to original sources

Proteinase inhibitors in the kidney and its tumours.

The proteinase inhibitors alpha-1-antitrypsin and alpha-1-antichymotrypsin are found in a variety of tissues including the kidney. We have used the immunoperoxidase technique to study the distribution of these glycoproteins in 10 fetal and five adult kidneys and in 44 renal tumours. Proteinase inhibitors were found in fetal kidneys in the cells of the metanephric blastema, primitive mesenchyme and in proximal tubules. They were found in proximal tubules of adult kidneys. These substances were also found in the mesenchyme of nephroblastomas and in the tumour cells of clear cell, oncocytic and sarcomatoid variants of renal cell carcinoma. These observations are significant in the understanding of the patterns of differentiation of which renal cell carcinoma is capable.

Adult↗

Perinatally related wastage--a proposed classification of primary obstetric factors.

Adapting Sir Dugald Baird's concept of primary obstetric causes of perinatal mortality, a revised clinico-pathological classification has been evolved to take account of new knowledge and developments, and to direct attention to potentially avoidable deaths and to where intensified efforts and investigation are needed. Categories highlighting the importance of intrauterine growth retardation, unexplained intrauterine death and spontaneous premature labour have been introduced, intrapartum hypoxia is separated from birth trauma, and infection again has its own category. Regular perinatal audit at one obstetric hospital, since 1979, has shown that the new system provides a workable and useful means for classifying not only perinatal deaths, but also late abortions, late neonatal deaths and perinatally related infant deaths. The rate of total perinatally related wastage, defined in this way, was almost twice that for perinatal mortality (22.8 compared with 11.9 per 1000 births). The former is advocated as a more realistic index for the audit of perinatal care. The revised and extended system is put forward as a contribution to the current debate on classifying and reporting such wastage, in the hope that it may be tested as a model for regional as well as hospital surveys.

Birth Injuries↗

National Post-Perinatal Infant Mortality and Cot Death Study, Scotland 1981-82.

Throughout 1981 and 1982 all deaths of infants aged 8-365 days (post-perinatal infant mortality, PPIM) in Scotland were studied. During this period there were 135 250 live births and 1533 infant deaths (infant mortality rate 11.3), including 763 PPIM deaths (5.6 per 1000 live births). These 763 deaths fell into three main categories: birth-determined (329), accident or acquired disease (65), and cot deaths (369). Birth-determined deaths included 109 preterm, 199 congenital disorders, and 21 miscellaneous deaths. 61 of the cot deaths were fully explained on necropsy, in 141 an associated finding which might or might not be relevant was found, and in the remaining 167 no explanation was found. The cot death rate was 2.7 per 1000 live births overall (3.3 for boys, 2.1 for girls), and more second-born than first-born children died (approximately 3:2). Excluding "explained" cot deaths the rate was 2.3 per 1000 live births.

Accidents↗

Lethal neonatal chondrodysplasias in the West of Scotland 1970-1983 with a description of a thanatophoric, dysplasialike, autosomal recessive disorder, Glasgow variant.

Complete ascertainment of lethal neonatal short-limb chondrodysplasias was attempted in the West of Scotland for the period 1970-1983. Forty-three cases were identified, representing a minimum incidence of 1 in 8,900. The differential diagnosis included 11 well-delineated skeletal dysplasias, one case of warfarin embryopathy, and one apparently new condition with presumed autosomal recessive inheritance that has radiographic similarities to those of thanatophoric dysplasia (TD). In this series TD had an incidence of 1 in 42,221, which is consistent with new dominant mutation at a rate of 11.8 +/- 4.1 X 10(-6) mutations per gene per generation. Ultrasonic measurement of fetal long bone length was performed in eight subsequent pregnancies at risk. Five unaffected fetuses were predicted correctly and three affected fetuses were detected during the second trimester (one with rhizomelic chondrodysplasia punctata-second trimester prenatal diagnosis not previously reported; one with achondrogenesis type II; and one with the new lethal condition).

Chondrodysplasia Punctata↗

The distribution of epithelial membrane antigen in the kidney and its tumours.

The distribution of epithelial membrane antigen (EMA) in the kidney and its tumours has been studied using a polyclonal anti-EMA antiserum and the immunoperoxidase-antiperoxidase technique (PAP). Twelve fetal and 10 adult kidneys examined showed EMA to be confined to the distal tubular or collecting duct epithelium or their embryological precursors. The examination of 55 primary renal tumours showed EMA to be present on the epithelial tumours, on tubular epithelium in nephroblastoma, but not on mesenchymal tumour cells nor on undifferentiated areas of nephroblastoma.

Adenoma↗

Immunocytochemical localisation of renin in nephroblastoma.

An antibody to pure human renin and an immunoperoxidase technique were used to stain immunoreactive renin in nephroblastomas. In an unselected series of surgically removed nephroblastomas immunoreactive renin was found in 10 of 19 tumours (53%). Tissue obtained at necropsy from 12 cases of disseminated nephroblastoma was also studied, and immunoreactive renin in metastatic tumours was found in three cases. Patients' blood pressures and serum potassium concentrations showed no evidence of excess renin activity. It is therefore suggested that if nephroblastomas contain renin as commonly as this study suggests, then any hormone secreted may be biologically inactive.

Blood Pressure↗

Post-perinatal infant mortality in Glasgow 1979-81.

Post-perinatal infant mortality (PPIM; deaths from the 8th day to the end of the 1st year of life) was studied in Glasgow over the 3-year period 1979-81. The 244 deaths were divided into three main categories--those determined at birth, those due to accidents and acquired disease, and cot deaths. 50% of deaths were determined at birth, and of these 46% were due to prematurity and 49% to congenital disorder. Cot deaths accounted for 44% of the total (88% of deaths not determined at birth) and a definite cause could be identified in only 10% of these. The PPIM rate was 6.1 per 1000 livebirths, a significant part of the infant mortality rate of 12.6. The significance of these findings is discussed in relation to the possible reduction of these figures.

Birth Injuries↗

Establishment and characterization of human neuroblastoma and ganglioneuroblastoma cell lines.

The establishment in culture and characterization of 4 human neuroblastoma (NB) cell lines and 1 human ganglioneuroblastoma cell line are described. Each cell line fulfilled at least 2 of 4 criteria for malignant or transformed cells: viz., subcultured more than 70 times, high saturation density with absence of contact inhibition, population-doubling time within a range of 10-40 h, and tumour formation in nu/nu mice. Each cell line also fulfilled at least 2 of 3 criteria for neuroblastoma cells: viz., humoral and cell-mediated immune reactivity toward NB-associated cell-surface antigen, intracellular storage and extra-cellular secretion of catecholamines, and characteristic neuroblast and ganglion-cell morphology.

Animals↗

Detection by ultrasound of abnormality in twin pregnancies during the second trimester.

Five cases of abnormality in twin pregnancies, detected by ultrasound examination during the second trimester, are reported. In four pregnancies there was a fetal malformation: discordant anencephaly, a monozygotic, heterokaryotypic twin pregnancy with coexistant Turner's syndrome and intrauterine death, concordant body stalk syndrome, and a dicephalus monster. In the other pregnancy one fetus dies in utero to become a fetus papyraceous. Four of the pregnancies were associated with raised levels of maternal serum alpha-fetoprotein, by singleton standards. Ultrasound examination proved of value in each case. Three pregnancies were terminated.

Adult↗

Epidermolysis bullosa in association with aplasia cutis congenita and pyloric atresia.

A female infant born to a mother who had an elevated serum alpha-fetoprotein during early pregnancy, presented a combination of epidermolysis bullosa and aplasia cutis congenita. She developed evidence of upper gastrointestinal obstruction and died at the age of 43 hours. Post-mortem examination showed the presence of pyloric atresia and electron microscopy of skin biopsies showed epidermolysis bullosa simplex. Examination of the placenta revealed a unique abnormality of the membranes, indicating the existence of two sacs. This case and the previously reported cases, which are reviewed, suggest an autosomal recessive inheritance. Serum alphafetoprotein estimation, ultrasonography and fetoscopy with skin biopsy are suggested as a means of pre-natal diagnosis in future pregnancies.

Abnormalities, Multiple↗

Recurring digital fibroma.

Seven cases of recurring digital fibroma were seen over a 35-year period. All demonstrated the classical clinical, macroscopic, and microscopic features of this distinct tumour, including the pathognomonic round, eosinophilic cytoplasmic inclusion bodies. Ultrastructurally, all seven cases were confirmed to be myofibroblastic in nature, and the morphology and intracellular topography of the inclusion bodies suggested their derivation from contractile protein. These findings establish recurring digital fibroma as a neoplastic lesion of the myofibroblast.

Child↗

Monarticular juvenile rheumatoid arthritis.

Seven out of 22 children with monarticular juvenile rheumatoid arthritis (MJRA) developed involvement of other joints between six months and three and a half years from the onset. In the other 15 patients the disease has remained monarticular for between one and 16 years (mean six years). Chronic iridocyclitis was seen in three of the five boys, two with antinuclear antibodies. Children with MJRA and antinuclear antibodies should have periodic ophthalmic assessment. Synovial biopsy was of value primarily in excluding other cases of arthritis, but there was only limited correlation between the histological findings and the subsequent course of the disease.

Arthritis, Juvenile↗

Diagnostic ultrasound: early detection of fetal neural tube defects.

In a series of 366 patients identified as at risk for a fetal neural tube defect (NTD) before the 24th week of pregnancy, 64 had an abnormal fetus. The abnormalities included anencephaly (39), open spinal defect (17), closed spinal defect (2), encephalocele (1), and a miscellany of other abnormalities (5). An ultrasound examinaton prior to diagnostic anmiocentesis positively identified all anencephalic fetuses, the fetus with the encephalocele, and 15 of the 19 fetuses with spina bifida. The spinal defects in 3 of the remaining 4 fetuses were demonstrated at a second examination. Since both amniotic fluid alpha-fetoprotein (AFP) assays and ultrasound examination have been shown to give false results in the diagnosis of NTDs, the importance of using 2 independent diagnostic techniques is stressed. In patients with elevated levels of maternal serum AFP, a careful ultrasound examination, in addition to identifying the majority of cases associated with an abnormal fetus, provided a good explanation for the elevation in over half of the remainder. In this series more than half the patients (40/69) who underwent amniocentesis because of raised maternal serum AFP levels were shown to have an abnormal fetus.

Amniotic Fluid↗

Prenatal diagnosis of fetal lymphatic system abnormalities by ultrasound.

Antenatal ultrasound diagnoses of gross lymphatic system abnormalities were made in three fetuses. Although the diagnosis was made in only 1 fetus at a stage early enough in pregnancy to allow selective termination, knowledge of the abnormality in the remaining 2 fetuses proved to be valuable for subsequent management of those pregnancies. It is stressed that the extent of the abnormality must be carefully assessed because of the possibility of corrective surgery should the lesion be small and that the parents must be given detailed counseling before any definitive measures are taken.

Abortion, Therapeutic↗

Avoidance of anencephalic and spina bifida births by maternal serum-alphafetoprotein screening.

Screening of 11 585 pregnant women between 16 and 20 completed weeks' gestation for raised serum-alphafetoprotein (A.F.P.) levels showed that the birth of 81.4% of babies with open neural-tube defects could be avoided. The screening test was sensitive enough to detect 93% of those affected and serum-A.F.P. levels above the point at which intervention shouldbe considered were found in 1.7% of pregnancies. After 75.2% of false-positives had been excluded by ultrasonography or by a repeat of the serum test, only 0.63% of pregnancies proceeded to amniocentesis, 46.6% of amniocenteses showed raised amniotic A.F.P. levels due to fetal abnormality. Fetal loss by abortion or perinatal death after amniocentesis occurred in 0.034% of pregnancies screeded, 75% being associated with threatened abortion before amniocentesis. There were no terminations of normal pregnancies due to false-positive amniotic A.F.P. results. It is concluded that voluntary maternal serum-A.F.P. screening has a valuable role in antenatal care.

Amniocentesis↗

Chest wall sarcoma of childhood with a good prognosis.

Four cases of a locally invasive sarcoma of the chest wall are described in children aged between 8 and 14 years. Although its morphology resembles a Ewing's sarcoma of bone there are light and electron microscopical features that are distinctive. The sarcoma is thought to represent an entity which has not previously been defined. Its clinical importance lies in the fact that the prognosis with appropriate treatment appears to be good. One child died after 2 1/2 years, but the others are alive 6, 8, and 16 years after presentation.

Adolescent↗