Search PubMed⌕ Search

Biomedical subjects

J Kidd

Publications and source records attributed to J Kidd.

40 records · Page 3Linked to original sources
PubMed1976

Neuropathological considerations in cerebro-hepato-renal syndrome (Zellweger's syndrome).

The nervous system in patients with cerebro-hepato-renal syndrome appeared to be affected at various tissue levels. There was evidence of a migrational disorder manifested by polymicrogyria and lack of normal neuronal maturation. There was dysmyelination of the white matter associated with accumulation of neutral fat in astrocytes. Within the peripheral nerves, masses of tangled neurofilaments producing dystrophic axons were demonstrated by electron microscopy. These findings could be explained on the basis of a genetic metabolic defect, one that involved particularly the amino acids. The defect may have interfered with the normal intercellular reaction during embryogenesis resulting in the malformation of multiple organs. The same metabolic abnormality could have caused the hepatic damage and disturbance in normal myelination during the neonatal period.

Abnormalities, Multiple↗
PubMed1976

Ependymomas of childhood. I. Histological survey and clinicopathological correlation.

We have conducted a correlative histological and clinical study on 34 cases of childhood ependymomas encountered at the Children's Memorial Hospital from 1953 to 1974. The cases were classified into grades A, B and C according to the extent of cytological differentiation and the correlation between the histological grades and the clinical outcome was obtained. It is concluded that the biological behavior of an ependymoma could be predicted to a large extent by the degree of cytological differentiation and by its anatomical location. A large share of the infratentorial ependymomas are well differentiated (grade A) and prognosis in this group is good when complete surgical removal could be accomplished. Most of the supratentorial ependymomas belong to the intermediate grade B and the malignant grade C; prognosis in these two grades is less predictable, approximately two thirds of these patients died within 3 years. The most malignant form is the ependymoblastoma. This is a highly primitive neoplasm histologically similar to medulloblastoma but shows evidence of ependymal differentiation. Most of the ependymoblastomas are located in the supratentorial region and the average length of survival is 20 months.

Adolescent↗
PubMedDate not supplied

Apparent monomorphism of ALDH2 in seven American Indian populations.

Deficiency of mitochondrial aldehyde dehydrogenase (ALDH2) has been previously reported in South American Indians. We therefore assayed five individuals from each of five South American Indian populations (Quechua, Karitiana, Ticuna, Surui, Guahiba), and two North American populations (Maya and Moskoke) for the presence of the Oriental ALDH2(2) variant. These samples were also surveyed for other alleles altering ALDH2 function. Allele-specific amplification assay (ASA) did not detect the ALDH2(2) allele in any of the New World populations studied. The entire coding sequence of the ALDH2 cDNA was enzymatically amplified in partially overlapping fragments. Each fragment was digested using restriction endonucleases and subfragments 148-285 b.p. in length were analyzed by the single-stranded conformation polymorphism (SSCP) technique. No variants were detected within the coding region of the ALDH2 gene in any of the seven American Indian populations. Three potentially correct explanations for these results are suggested. First, an ALDH2 polymorphism is present but undetectable by SSCP; second, none of the studied individuals were ALDH2 negative; third, the polymorphism occurs beyond the coding region of ALDH2 gene.

Aldehyde Dehydrogenase↗