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Biomedical subjects

S Knowles

Publications and source records attributed to S Knowles.

At least 55 records · Page 3Linked to original sources
PubMed1991

Dandy-Walker malformation (variant), cystic dysplastic kidneys, and hepatic fibrosis: a distinct entity or Meckel syndrome?

Dandy-Walker malformation is an unusual finding in Meckel syndrome, which characteristically presents with encephalocele, polydactyly, and cystic renal disease. We report on a family in which three nonviable brothers had Dandy-Walker malformations (variant) with associated enlarged cystic dysplastic kidneys and hepatic fibrosis. The consistent segregation of these abnormalities in all three sibs in the absence of polydactyly and encephalocele suggested the possibility of a distinct syndrome. The clinicopathological findings of the disorder are described and discussed in the context of the phenotypic spectrum of Meckel syndrome.

Adult↗
PubMed1991

Blood component treatment: a retrospective audit in five major London hospitals.

A retrospective audit of 200 transfusion episodes involving the use of platelets or fresh frozen plasma (FFP) was performed in five hospitals in London. It examined the currently used practices of transfusion and assessed the appropriateness of blood component treatment. It was necessary to search for an excess of case notes to provide a sufficient number of patients for review. In 61.5% of cases the reason for using the components was not stated. Inadequate documentation of the use of blood components occurred in 66% of cases. An accepted clinical indication for the use of components was evident in only 36% of the total; inappropriate use of FFP was particularly apparent. It is concluded that many aspects of transfusion practice need to be improved.

Blood Transfusion↗
PubMed1990

Ablation of endometriotic implants in rabbits by hematoporphyrin derivative photoradiation therapy using the gold vapor laser.

In a rabbit model, endometriotic lesions were utilized to study hematoporphyrin derivative (HPD) uptake and the effect of photoradiation using the gold vapor laser. Morphological studies confirmed successful induction of ectopic endometrial growth and the lesions displayed preferential HPD uptake compared to normal intraperitoneal structures. Gold vapor laser irradiation of the lesions in HPD pretreated animals produced necrosis of the endometriotic lesions leaving surrounding tissues healthy. Results from this study suggest that HPD-mediated photodynamic therapy may be a suitable means of treating endometriosis in the human.

Animals↗
PubMed1989

Osteodysplastic primordial dwarfism: report of a further case with manifestations similar to those of types I and III.

We describe a male infant with microcephalic osteodysplastic primordial dwarfism. The clinical and radiological manifestations most closely resemble those of the patient described by Winter et al. to have manifestations overlapping with both osteodysplastic primordial dwarfism types I and III. The classification of the patient within the spectrum of osteodysplastic primordial dwarfism is discussed and the distinctive neuropathology documented.

Abnormalities, Multiple↗
PubMed1989

Umbilical cord sclerosis as an indicator of congenital syphilis.

In two cases of congenital syphilis examination of the umbilical cord showed a distinctive picture of perivascular sclerosis and inflammation. Large numbers of spirochaetes were shown in the sclerotic zone in both cases. No organisms were demonstrable in the placental disc and there were no morphological changes suggestive of syphilis. It is concluded that the finding of sclerosing funisitis, together with the clinical circumstances, should raise the possibility of congenital syphilis.

Adolescent↗
PubMed1989

Ultrasound in the perinatal necropsy.

Although plain and contrast radiology have become a well established part of the perinatal and fetal necropsy, the role of ultrasound has been less comprehensively explored. We have found a variety of ultrasonographic approaches to be of value in diagnosis, quality control, and teaching. Ultrasound is of particular help where consent for formal necropsy has been declined.

Autolysis↗
PubMed1989

Dietary restriction, tyramine, and the use of monoamine oxidase inhibitors.

The aim of this study is to provide clearer guidelines for rational, safe, and practical dietary restriction for use with monoamine oxidase inhibitors. Tyramine levels were assayed in over 100 of the controversial foods that have been associated with hypertensive reactions or reported to contain high levels of tyramine. Only a very limited number of foods appear to require absolute restriction. These include all aged cheeses, concentrated yeast extracts (e.g., Marmite), sauerkraut, and broad bean pods. Alcoholic beverages, including Chianti wine consumed in moderation, appear to be safe. Some aged meats contain relatively high levels of tyramine and require closer investigation.

Food Analysis↗
PubMed1988

Pulmonary agenesis as part of the VACTERL sequence.

Pulmonary agenesis is an uncommon anomaly that has been reported in isolation and in association with other congenital defects. Such defects include oesophageal atresia, cardiac malformation, horseshoe kidney, and anal atresia. Over a period of three years we have seen five neonates or fetuses with unilateral agenesis of the lung. All the cases had three or more anomalies seen in the VACTERL sequence in addition to the pulmonary atresia. None had a tracheo-oesophageal fistula. None were the products of consanguineous marriages. There had been no recurrence of this range of defects in any of the families at the time of writing. We suggest that pulmonary agenesis may occur as an alternative to tracheo-oesophageal fistula in the VACTERL sequence.

Abnormalities, Multiple↗
PubMed1986

A new category of lethal short-limbed dwarfism.

We describe a first cousin marriage between Asians which resulted in 13 pregnancies; 4 of the offspring were normal, 5 were stillborn dwarves, 3 pregnancies resulted in miscarriage, and one was terminated for dwarfism detected prenatally. The radiology and histology are unique and the pedigree suggests autosomal recessive inheritance.

Consanguinity↗
PubMed1986

Examination of products of conception terminated after prenatal investigation.

A large number of district general hospitals have access to diagnostic ultrasonography and other methods of prenatal diagnosis, resulting in an increased supply of freshly terminated malformed fetuses to general histopathology departments, and there is now more open discussion of malformation and greater concern over fetal wastage. General pathologists are therefore under greater pressure to produce complete and detailed descriptions of a wide range of often complex anomalies. The dismissal of specimens as "multiple congenital anomalies" is becoming increasingly unacceptable to couples who wish to embark on further pregnancies and to their medical attendants. As in other fields an understanding of the methods and terminology in clinical use and a consistent diagnostic approach should help pathologists to extract sufficient information for accurate counselling.

Abnormalities, Multiple↗
PubMed1985

Cephalosporin-induced immune neutropenia.

Neutropenia is an occasional complication of treatment with cephalosporin antibiotics. This report describes two patients who had neutropenia while receiving high doses of cephalosporins. The neutrophil counts returned to normal after stopping the drug, and cephalosporin-dependent neutrophil antibodies were demonstrated in both cases, using the granulocyte immunofluorescence test. In one patient, the immune neutropenia appeared to be due to a drug adsorption mechanism similar to penicillin-induced haemolytic anaemia, while an immune complex mechanism may have been involved in the second patient.

Adult↗
PubMed1984

Giant lymph node hyperplasia (Castleman's disease) of the mesentery. Observations on the associated anemia.

A mesenteric mass, histologically characterized as giant lymph node hyperplasia was found in an 18-yr-old man with at least 11 yr of growth retardation and anemia. The anemia was characterized by iron deficiency from selective malabsorption of iron, and by features of the anemia of chronic disorders. In contrast with a previous report, no inhibitor of erythropoietin was found and there was no abnormality of erythropoietin secretion. Resection of the mass was followed by rapid correction of the anemia and catch-up growth. The mass had histologic features of the hyaline vascular and plasma cell types of Castleman's disease with multinucleate giant cells probably of macrophage origin. Immunologic studies of the mass showed that the B lymphocytes were polyclonal and the T lymphocyte helper/suppressor cell ratio was normal, suggesting that giant lymph node hyperplasia is a local inflammatory reaction.

Adolescent↗