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Biomedical subjects

F W Wiglesworth

Publications and source records attributed to F W Wiglesworth.

At least 19 recordsLinked to original sources
PubMed1984

Maude E. Abbott.

Explore the source record for details and available documents.

History, 19th Century↗
PubMed1983

An oculocerebral hypopigmentation syndrome.

An oculocerebral hypopigmentation syndrome consisting of growth retardation, dolichocephaly, cataracts, high arched palate, small, widely spaced teeth, generalized hypopigmentation, psychomotor retardation, progressive neurological manifestations and hypochromic anemia is described in sibs. The finding of parental consanguinity supports autosomal recessive inheritance. The syndrome resembles the Cross syndrome (1,2).

Abnormalities, Multiple↗
PubMed1980

Congenital hypothalamic hamartoblastoma, hypopituitarism, imperforate anus and postaxial polydactyly--a new syndrome? Part I: clinical, causal, and pathogenetic considerations.

We report on six infants with a neonatally lethal malformation syndrome of hypothalamic hamartoblastoma, postaxial polydactyly, and imperforate anus. Some, but not all, patients had laryngeal cleft, abnormal lung lobulation, renal agenesis and/or renal dysplasia, short 4th metacarpals, nail dysplasia, multiple buccal frenula, hypoadrenalism, microphallus, congenital heart defect, and intrauterine growth retardation. The infants also had hypopituitarism and hypoadrenalism. All were sporadic cases, parents were not consanguineous, chromosomes were apparently normal. Family histories were unremarkable. There was insecticide and/or herbicide exposure in several of the cases, but no exposures were common to all 6 mothers. Five of the patients were born within an 8-month period, but all in different geographic locations. It is postulated that this is a previously apparently unreported syndrome of presently unknown cause.

Anus, Imperforate↗
PubMed1978

Congenital absence of the heart: observation of human funiculopagous twinning with insertio funiculi furcata, fusion, forking, and interpositio velamentosa.

An anatomic and radiologic study of a normal and acardius twinning with a single placenta is presented with literature review. The fetus was unusually well-developed with an almost normal skeletal and brain formation, a normal genito-urinary tract, and an absence of liver, spleen, lungs, and pancreas. The heart was absent but the great arteries, single aorta, and veins were present. The placentation is described as funiculopagous with insertio funiculi furcata, fusion, forking and interpositio velamentosa, and the latter may account for the fusion of the two circulations in this instance with the possibility that the heart was never present. The present and other observations of funiculopagous twins with two amniotic sacs suggest that the forked cord twin anomaly may occur earlier than generally recognized.

Abnormalities, Multiple↗
PubMed1976

Wilson disease. Comparative ultrastructure in a sibship of nine.

Comparative electron microscopy was done on liver tissue from a family of nine siblings to determine whether presymptomatic, affected patients with Wilson disease could be differentiated from heterozygous, normal carriers. Two of the nine had developed the neurologic manifestations of the disease; three others were considered to be homozygous but normal, two were heterozygous and normal, and two were classified as genotypically uncertain because of borderline biochemical abnormalities. Alterations of the mitochondria and endoplasmic reticulum suggestive of copper toxicity were present in both the heterozygous and the genotypically uncertain siblings, and a clear distinction could not be made on this basis. The severity of the abnormalities appeared to correlate with liver copper level, and organelle changes were found to precede lipid accumulation.

Adolescent↗
PubMed1973

Reye's syndrome in a newborn infant.

A case of Reye's syndrome in a newborn infant, believed to be the first recorded in the perinatal period, is reported. The clinical, biochemical and histological findings are those previously recognized in older infants. The intractable acidosis, hypoglycemia and extreme degree of lactic acidosis reflect an advanced degree of hepatic involvement, probably initiated in utero, with resultant impairment of glycogenolysis and intermediary metabolism.

Acidosis↗
PubMed1973

An outbreak of adenovirus type 7 infection in children in Montreal.

Thirteen infants and children with adenovirus type 7 infection proved by virus isolation are described. High fever, cough and dyspnea were the most frequent findings; in infants under 1 year of age wheezing was common. Four patients required artificial ventilation. Lobar collapse, consolidation and hyperinflation were frequent radiologic findings. None of the symptoms responded to antibiotic therapy or bronchodilator drugs. Three patients died (mortality rate of 23%). Pathologic findings were compatible with adenovirus type 7 pneumonia, and were characterized by a necrotizing bronchitis and bronchiolitis, patchy alveolar fibrinopurulent exudate and hyaline membrane formation. Some intra-alveolar epithelial cells showed strikingly abnormal nuclei and rare typical halo-outlined intranuclear inclusions were seen. Only one of eight survivors had evidence of significant chronic chest disease.

Adenoviridae Infections↗
PubMed1971

Familial holoprosencephaly.

The authors report a family in which several individuals were born with various anomalies of the face and brain. An analysis of the pedigree supports the hypothesis that a single gene defect with variable expressivity and reduced penetrance is responsible for the familial faciocerebral syndrome described which may range from fusion of the eyelids to various combinations of bilateral or median cleft lip and palate, absent nose and single brain ventricle.

Abnormalities, Multiple↗