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Biomedical subjects

J Scotto

Publications and source records attributed to J Scotto.

At least 19 recordsLinked to original sources
PubMed1991

Indications of future decreasing trends in skin-melanoma mortality among whites in the United States.

Trends in skin melanoma death rates during a 35-year period, 1950-84, were analyzed according to age, sex, and birth cohort for whites in the United States. In contrast to upward trends observed for older men and women (i.e., over 40), downward trends were noted for younger age groups. The risk of dying from skin melanoma appears to have peaked for male cohorts born during the 1950s and for female cohorts born during the 1930s. Assuming no future environmental or lifestyle changes, the upward trend in age-adjusted mortality rates, which averaged 2 to 3% per annum since 1950, is projected to discontinue and bend downward by the second decade of the 21st century. Skin melanoma incidence data, which was limited to a series of 12 years (1973-84) and inadequate for cohort analyses, were included to demonstrate that trends in age-specific rates were comparable with those observed for mortality during the overlapping time period. Incidence trends according to anatomical site are also described. These results indicate that baseline data necessary for assessing the potential effects on this disease from future depletions of the ozone layer, and predicted increases of solar ultra-violet radiation exposure, would be improved with the inclusion of cohort data and age-specific trend analyses.

Age Factors↗
PubMed1991

Incidence of nonmelanoma skin cancer in New Hampshire and Vermont.

A survey of skin cancer occurrence between June 1979 and May 1980 among residents of New Hampshire and Vermont identified 277 cases of squamous cell carcinoma and 1761 cases of basal cell carcinoma. The age-adjusted incidence rates for squamous cell carcinoma (32 per 100,000 in men, 8 per 100,000 in women) and for basal cell carcinoma (159 per 100,000 in men, 87 per 100,000 in women) were similar to those reported in other populations in the northern United States. Skin cancer incidence was particularly high among men more than 70 years of age and a large proportion (greater than 30%) of patients 55 years or older had a history of at least one previous skin cancer.

Adolescent↗
PubMed1989

Molecular analysis of peroxisomal beta-oxidation enzymes in infants with peroxisomal disorders indicates heterogeneity of the primary defect.

Immunoblot analysis of peroxisomal beta-oxidation enzymes proteins was carried on liver samples from 15 patients with peroxisomal disorders in which accumulation of very long chain fatty acids was always observed in plasma. In 11 cases including 4 cerebro-hepatorenal syndrome (CHRS), 4 neonatal adrenoleukodystrophy (NALD) and 3 infantile Refsum's disease, the liver peroxisomes could not be detected by electron microscopy. Immunoblot analysis revealed the absence, or presence in weak amounts, of the 72-kDa subunit of acyl-CoA oxidase, and the complete absence of the 52-kDa and 21-kDa subunits which are processed from the 72-kDa. The bifunctional protein (78-kDa) was absent or very reduced, as was the mature form of peroxisomal 3-ketoacyl-CoA thiolase (41-kDa). Multiple defects of peroxisomal beta-oxidation enzymes may be caused by an absence of synthesis or an inability to import proteins into peroxisomes in these patients. One patient, diagnosed as NALD, had no detectable liver peroxisomes but the presence, in normal amounts, of the three peroxisomal beta-oxidation enzyme proteins suggests that the transport of these enzymes into "peroxisomal ghosts" was still intact. The last 3 patients, clinically diagnosed as NALD, had normal liver peroxisomes. One patient had an isolated deficiency of the bifunctional protein and the 2 others had normal amounts of the 3 peroxisomal beta-oxidation enzymes, as shown by immunoblotting. This suggests that import and translocation of some peroxisomal proteins had occurred and that a mechanism is therefore required to explain the defect in these patients.

3-Hydroxyacyl CoA Dehydrogenases↗
PubMed1989

Sanfilippo disease, type C: three cases in the same family.

Six siblings were followed, three of them suffering from a Sanfilippo disease, type C, as demonstrated by a deficient glucosamine acetyltransferase activity in cultured skin fibroblasts. Clinical and radiological findings were similar in the three affected children and the phenotypical expression of the disease allowed no distinction between the different types of Sanfilippo disease. Ultrastructural studies of the liver demonstrated characteristic intra-vacuolar inclusions.

Acetyltransferases↗
PubMed1986

Hepatic peroxisomes are deficient in infantile refsum disease: a cytochemical study of 4 cases.

We examined liver biopsies from 4 patients with the infantile form of Refsum disease. No peroxisomes were visualized by light microscopy after cytochemical staining for catalase, a marker enzyme for this organelle. Absence of peroxisomes was confirmed by electron microscopy in 3 patients; in the 4th patient we observed organelles of peculiar size and structure and with minimal catalase activity. Light microscopy also showed birefringent macrophages containing P.A.S.-positive material; they were abundant in the 3 older children, and rare in the youngest (8 months). Peroxisomes and birefringent macrophages were absent in 2 patients with the cerebrohepatorenal syndrome of Zellweger. The simultaneous presence of these unique light microscopical characteristics may be of diagnostic value.

Birefringence↗
PubMed1986

Neonatal adrenoleukodystrophy.

Nine cases of neonatal adrenoleukodystrophy are described. All patients had abnormal facial features, moderate to severe hypotonia, hepatomegaly, and retinitis pigmentosa. The clinical course was rapidly progressive in six cases and more protracted in three others. Biological signs of adrenal insufficiency were present in five cases. CT scan showed a demyelinating process in four patients. Trilamellar inclusions were found in the liver of four cases and dark and complex lipidic inclusions in three other cases. In the three necropsied patients there was severe alteration of the white matter involving particularly the cerebellum in two cases. Gyral and cytoarchitectonic disturbances were absent in all three cases. Increased plasma levels of very long chain fatty acids (8/8), phytanic acid (7/8) and bile fluid trihydroxycoprostanic acid (2/4) confirmed the deficiency of multiple peroxisomal enzymes. Clinical, histopathological and biochemical findings of these nine cases are compared to those reported in other neonatal adrenoleukodystrophy cases and to those of other neonatal peroxisomal disorders, that is cerebro-hepato-renal syndrome of Zellweger and infantile Refsum's disease.

Adrenal Cortex↗
PubMed1978

Myelin deficiency in experimental phenylketonuria: contribution of the aromatic acid metabolites of phenylalanine.

Retarded body and brain growth and a deficit of myelin in the cerebral hemispheres and the cerebellum were observed in an animal model of phenylketonuria, the p-chlorophenylalanine and L-phenylalanine treated preweanling rat. These manifestations of phenylketonuria were reproduced in rats treated with phenylacetate in amounts approximating those likely to be produced in phenylketonuria. Young rats treated with equivalent amounts of other metabolites of phenylalanine, namely, phenylpyruvate, phenyllactate, and mandelate, which also accumulate in the brain during hyperphenylalaninemia, did not exhibit any toxic effects. Phenylpyruvate did not give rise to phenylacetate in the brain, but a small percentage was converted to phenyllactate. The gross composition of myelin isolated from the brains of saline and phenylacetate treated animals was similar. At various time intervals after subcutaneous injection, phenylacetate in the brain reached levels thirty times those of phenylpyruvate and phenyllactate, although animals received equivalent amounts of the three metabolites. The retarded growth of the body and brain of the young animal treated with phenylacetate may be attributed to the formation of phenylacetylcoenzyme A in the tissues. The site of action is very likely linked to acylcoenzyme A metabolism, i.e., the synthesis and utilization of acetylCoA and acetoacetylCoA, which are involved in reactions generating ATP and energy and in the synthesis of cholesterol and fatty acids. Results of this investigation indicate that growth retardation induced by phenylacetate during the period of very rapid development of the brain is responsible for the mental retardation in phenylketonuria.

Animals↗
PubMed1978

Skin cancer epidemiology: research needs.

The basis data currently being used to estimate and evaluate the dose-response relationship of UV-B and skin cancer are from a 6-month survey for four areas that participated in the TNCS, 1971-1972. Although most investigators from various fields of interest outside of cancer research, i.e. aviation, environmental ecology, physics, chemistry, and photobiology, etc., may admit an association between nonmelanoma skin cancer and UV-B exists, they point out that 1) the epidemiologic data currently available are too sparse and lack certain detail, such as exposure patterns and skin types, and 2) more data of this type are needed over a broad geographical range to allow for more precise measurements of the effects of stratospheric ozone depletion. They argue that the present relationships could change drastically with the addition even of a few more points (geographical locations) and that location-specific and demographic factors should be evaluated. Therefore, these data need to be updated and expanded to include more locations over a longer study period. The National Cancer Institute and the Environmental Protection Agency undertook a special skin cancer study from June 1, 1977 to May 31, 1978. The objectives of this study were: 1) to determine the incidence of nonmelanoma skin cancer (basal cell and squamous cell carcinomas) in various population groups within the United States, and 2) to ascertain and measure epidemiologic factors that may contribute toward the excess risk of nonmelanoma skin cancer in specific population groups.

Adolescent↗
PubMed1977

Pathological study of alpha-chain disease, with special emphasis on evolution.

The pathology of six cases of alpha-chain disease (alpha-CD), four of which were followed until complete remission or death, was studied by histologic, immunofluorescence and ultrastructural techniques. The lesions could be classified in three histopographical stages. The late stage C is an immunoblastic sarcoma probably deriving from the same clone as the initial plasmacytic stage A, stage B being a transitional one between A and C. The asynchronism of the lesions in different organs in the same patient requires a laparotomy for an accurate staging which determines the prognosis and the treatment. Complete and prolonged remissions have been observed at stage A only, sometimes with oral antibiotic treatment alone. At all stages, alpha-CD and the "Mediterranean lymphoma" share identical aetiological, clinical and pathological features. Accurate immunological studies will determine the precise-frequency of alpha-CD protein synthesis in the latter syndrome.

Adult↗
PubMed1977

Methotrexate compared with placebo in lung cancer.

Two hundred thirty-nine patients with microscopically proven, inoperable bronchogenic carcinoma were allocated at random to receive twice weekly I.M. injections of either methotrexate at "high dose" of 0.06 mg/kg/dose or methotrexate at "low dose" of 0.2 mg/kg or visually indistinguishable placebo in the same volume of 0.1 ml/kg for four months. Twelve patients were invalidated for procedural reasons. Objective response (greater than or equal to 50% tumor regression) was dose-related with 21% of 48 patients with measurable disease on high -ose, 11% of 37 patients on low dose, and 6% of 32 patients on placebo. Corresponding response rates for epidermoid carcinoma were 35% of 23 patients, 9% of 11 patients, and 0 of 13 patients. Responders in the two treatment groups had a three to four fold increase of median survival (p less than .05). Non-responders on high and low dose methotrexate lived as long as patients on placebo. Leukopenic patients in all three treatment groups lived substantially longer than patients without leukopenia less than 4,500/mm3, irrespective of presence or absence of objective response. All three regimens were well tolerated. None of the patients had life-threatening toxicity. It is concluded that methotrexate at "high dose" is a potentially useful drug for temporary palliation of epidermoid carcinoma of the lung.

Adenocarcinoma↗
PubMed1977

Mathematical models of age and ultraviolet effects on the incidence of skin cancer among whites in the United States.

That sunlight leads to skin cancer has been generally accepted for nearly a century. Physical data are, for the first time, available which support this hypothesis. The authors have found that a simple power relationship can be used to describe the data and that the form of this power function suggests that the risk of nonmelanoma skin cancer is related to cumulative lifetime ultraviolet (UV) exposure and that the risk of melanoma skin cancer is related to annual UV exposure. The authors emphasize that skin cancer risk also depends on location-specific demographic variables other than ultraviolet radiation.

Adult↗
PubMed1977

Immunofluorescence on resin-embedded material.

Some antigenic determinants can be preserved in tissues after plastic embedding. In the present study liver tissue was fixed with glutaraldehyde or paraformaldehyde, dehydrated with ethanol and toluene and embedded in araldite which was then polymerized at 37 degrees C. Immunofluorescence was performed on semi-thin sections etched with hydrogen peroxide. This procedure allows correlation with light microscopy (on the same stained semi-thin section) and with electron microscopy (on adjacent ultra-thin section). Good results were obtained with anti-nuclear, anti-mitochondrial, anti-microsomal and anti-alpha1-antitrypsin sera.

Antibodies, Antinuclear↗
PubMed1977

Children sea-blue histiocytosis (2 cases) compared with phospholipidosis induced by 4-4' DET (4-4' p (diethylamino-2-ethoxy phenyl) 3-4 hexane) in rat.

Two cases of children with liver and spleen enlargement are reported. Sea-blue histiocytes and Pick cells were found in both cases in liver, spleen, bone marrow and blood. Further more, lysobisphodphatidic acids were identified in phospholipid analysis of liver biopsies and cultived liver cells. Absence of neurologic involvement at 14 and 18 years fo age suggest a Crocker type C of Niemann-Pick disease, i.e. a not yet well defined entity. Resemblance of these morphological and biochemical abnormalities with certain cases of drug poisoning (especially the well-known intoxication by 4-4' DET) is discussed on the basis of results from experimental studies with this drug in the rat.

Adolescent↗