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Biomedical subjects

C Scheiner

Publications and source records attributed to C Scheiner.

At least 37 records · Page 2Linked to original sources
PubMed1993

Childhood cancer incidence in the south-east of France. A report of the Provence-Alpes-Côte d'Azur and Corsica Regions Pediatric Cancer Registry, 1984-1991.

A prospective registration of incident cancers in childhood in two south-east regions of France since 1 January 1984 allows us to collect pertinent data on 875 cases throughout a period of 8 years. World age-standardised overall incidence rate is 137.63 cases/million/year. It is close to that reported in other white European. North American and Oceanian populations. The age-adjusted (age-standardised) relative frequency of each pathological group is: leukaemias 29.71%; central nervous system tumours 20.61%; lymphomas 12.75%; sympathetic tumours 9.03%; soft tissues tumours 7.37%; bone tumours 5.89%; kidney tumours 4.82%; epithelial tumours 3.83%; germinal and gonadal tumours 3.24%; retinoblastomas 2.11%; liver tumours 0.45% and others 0.14%. The comparison of these results with international available data shows that we record the world highest adjusted incidence rates for neuroblastomas (15.46) and rhabdomyosarcomas (7.04) and a high rate for Ewing's sarcomas (3.30); this fact will need to be confirmed by a longer period of observation, but even now the total number of cases (particularly for neuroblastoma) is high when compared with the data of other children registries which give rates for longer periods and for similar or larger populations.

Adolescent↗
PubMed1993

Ocular growth in the fetus. 1. Comparative study of axial length and biometric parameters in the fetus.

The knowledge of ocular growth during fetal life, when compared with other fetal biometric parameters, could not only provide a better definition of malformation syndromes but could also give a better understanding of certain pathological processes in premature babies and in newborns. As the literature concerning prenatal ocular dimensions contains few data, the aim of this study was to measure the axial length of the globe (AL) in fetuses and compare this measurement with their gestational age, weight, height, head circumference (HC) and thoracic circumference (TC) in order to compile a reference table. In the present study, 76 globes from 38 fetuses (18-41 weeks gestational age) from the Department of Pathology (Timone University Hospital, Marseille) were examined. Ultrasonography A and B were used to measure the AL, and a pathological examination determined fetal weight, HC, TC and height. We were interested to find out which of the parameters studied would give the best correlation with ocular growth. Statistical analysis showed that HC remained the most discriminant factor and correlated best with ocular growth. We thus obtained an equation for ocular size according to HC that could serve as a basis for detecting pre- or postnatal ocular defects.

Anthropometry↗
PubMed1993

Ocular growth in the fetus. 2. Comparative study of the growth of the globe and the orbit and the parameters of fetal growth.

In a previous study, we showed that the growth of the eye during fetal development compared with the other parameters of the fetus was above all connected to the head circumference, which appeared to be the most discriminant measurement. In a second study, we measured the growth of the orbit of the fetus in order to compare it with the growth of the globe and the cranial circumference. Another 60 eyeballs of 30 fetuses of 18-41 weeks gestational age were examined. The corneal diameter (CD) of the cornea was a measurement added to those used in the method in the previous study, and orbital radiography was carried out: the parameters used were the same as in the previous study measured in relation to the CD, orbital diameter (OD) and the biparietal diameter (BPD). The aim of this study was to show the links between the variations in the measurements and to estimate them in relation to fetal growth. The results confirm those of the previous study, and this study also shows that, in estimating the head circumference, the OD and CD are the most accurate indicators. This study was also concerned with the BPD: the best correlation is obtained with the OD, which can be estimated accordingly from the BPD. Although the OD is not in an area studied by ultrasonography (highly dependent on the sectional plane), this study shows that it should perhaps be reconsidered in the prenatal diagnosis of orbital malformation. It would then be necessary to take into account not only the BPD but also the relationship between the OD and the BPD.

Anthropometry↗
PubMed1993

[Aneurysmal cyst of the bones in children].

The authors have analysed a series of aneurysmal bone cysts (A.B.C.) in children and adolescents which were reviewed at an average follow-up of 4 years 2 months (6 months-15 years). The average age at diagnosis was 8 years (3-19 years). Pathological fractures were the main reason for consultation, probably due to the predominance of central lesions. If conventional radiology remains indispensable to diagnosis, magnetic resonance imaging is nevertheless the most important examination in determining the extent of the affection. The diagnosis remains anatomopathological, even if this may be difficult due to associated lesions (A.B.C. illness and A.B.C. symptoms). In long bones lesions, we observed recurrence after curettage in 3 out of 7 cases. For this reason the recommend simple resection or reconstructive resection rather than curettage whenever possible. 5 lesions in contact with growth plate were observed. In such cases we recommend its conservation by careful curettage, especially in young children. We feel that subsequent recurrence is easier to treat than an epiphysiodesis bridge. The surgical techniques employed to conserve the plate are described along with methods of bone reconstruction after surgery.

Age Factors↗
PubMed1992

[Acute necrotizing eosinophilic myocarditis. Favorable clinical course after heart transplantation].

A case of acute eosinophilic myocarditis without hypereosinophilia, presenting as hypokinetic dilated cardiomyopathy in a 24-year old man is reported. Sudden worsening of subacute cardiac failure required heart transplantation 3 months after the onset of the disease. Only pathological examination provided the diagnosis of acute necrotizing eosinophilic myocarditis of undetermined origin. Two years after transplantation, the patient had no clinical or histological sign of recurrence. Seldom described in the literature, acute eosinophilic myocarditis is a dangerous form of eosinophilic heart disease which often follows a fulminant course beyond all therapeutic resources. This case, which is particular in its clinical presentation, in the lack of hypereosinophilia and above all in its cure after heart transplantation, enables the authors to discuss the mechanisms and various manifestations of the cardiotoxicity of eosinophils.

Acute Disease↗
PubMed1992

Prenatal diagnosis of Fryns' syndrome.

We report on a new case of ultrasonographic prenatal diagnosis of Fryns' syndrome during the second pregnancy of a young woman whose first child died 90 min after birth and was diagnosed as having this autosomal recessive condition. The feasibility of diagnosis in utero and timing in the phenotypic expression of this multimalformation syndrome are discussed.

Abnormalities, Multiple↗
PubMed1992

Splenic hemangioma--report of three pediatric cases with pathologic correlation.

Splenic hemangioma is a very rare neoplasm in children. We report three cases of splenic hemangioma, two of solitary hemangioma and one of hemangiomatosis. Two patients had hematologic disorders related to hemangioma. Imaging findings of this entity are presented and discussed with emphasis on pathologic correlation.

Child, Preschool↗
PubMed1992

Defects of the mitochondrial respiratory chain complexes in three pediatric cases with hypotonia and cardiac involvement.

Three children displaying hypotonia, cardiac involvement and defects of the mitochondrial respiratory chain complexes are reported. The first case showed severe neonatal hypotonia, failure to thrive, hepatomegaly, dilation of the right cardiac cavities, profound lactic acidosis and amino aciduria. The boy died at the age of 7 weeks. In the second case hypotonia, severe cardiomyopathy, cyclic neutropenia, lactic acidosis and 3-methylglutaconic aciduria occurred. The boy died at the age of 27 months. The third case presented at the age of 16 months as an acute hypokinetic hypertrophic cardiomyopathy with transient hypotonia and mild lactic acidosis. Spontaneous clinical remission occurred. In all cases muscle biopsy was performed. Morphological studies failed to show ragged-red fibers but there was lipid storage myopathy and decreased cytochrome c oxidase activity. Biochemical studies confirmed the cytochrome c oxidase deficiency in muscle in all cases. It was associated with complex I III deficiency in case 1 and with severe deficits of all respiratory chain complexes in case 2. Post-mortem studies in case 1 indicated that complex IV was reduced in the liver but not in the heart and quantitative analysis of mtDNA revealed a depletion in muscle. Cases 1 and 2 shared some clinical features with fatal infantile myopathy associated with cytochrome c oxidase deficiency, while case 3 displayed a very unusual clinical presentation. The histochemical enzyme reaction of cytochrome c oxidase is useful for the diagnosis of mitochondrial myopathy because ragged-red fibers may be lacking. Finally, biochemical measurement of the different mitochondrial respiratory chain complexes is required because multiple defects are frequent and occasionally related to mtDNA depletion.

Abnormalities, Multiple↗
PubMed1992

Twenty-four-hour hypothermic preservation of rat liver with Euro-Collins and UW solutions. A comparative evaluation by 31P NMR spectroscopy, biochemical assays, and light microscopy.

A comparative study of 24 hr preservation at 4 degrees C of excised rat livers with Euro-Collins and hydroxyethyl starch-free University of Wisconsin (UWm) solutions has been conducted based on the assessment of (1) the cellular energy status determined by 31P NMR spectroscopy and (2) cellular injury estimated from the loss of purine compounds (inosine, hypoxanthine, xanthine, and uric acid) during cold ischemia and reperfusion measured by HPLC, the leakage of intracellular enzymes, and the modifications of parenchyma established by light microscopy. Recovery of nucleosides di- and triphosphate was greater in the UWm group (80 +/- 6% vs. 58 +/- 6%) while inorganic phosphate formation was comparatively reduced. During hypothermic storage, the UWm groups generated a higher amount of inosine and hypoxanthine (in relation to the presence of adenosine in the protective solution) while no xanthine or uric acid was detected due to the inhibitory effect of allopurinol. Conversely, large quantities of xanthine and uric acid were found in the reperfusate of the EC group, pinpointing the cytotoxic role of oxygen-derived free radicals in the generation of cellular damage, as also illustrated by a higher aspartate aminotransferase leakage in the EC group (devoid of allopurinol and glutathione. Light microscopy indicated no histological alterations in the UWm group and mild alterations in the EC group that showed ballooning of hepatocytes (no lactobionate and raffinose in EC) and an alternation of clarifications and eosinophilic condensations. This study clearly confirms and illustrates the overall superiority of UWm solution in liver transplant preservation.

Adenosine↗
PubMed1992

[Rhabdoid tumor of the neck].

Three pediatric cases of malignant rhabdoid tumor of the neck are described. Clinical data and imaging findings (US, CT and MRI) are stressed. The mass was well defined, containing punctate calcifications in two cases and encasing the vessels in two other cases. Two patients were treated with a chemotherapy regimen according to MMT 89 SIOP protocol, one had chemotherapy and radiotherapy. Two children died of progressive disease; the remaining child who had complete surgical removal of the tumor is on remission 17 months after diagnosis.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1992

[Drash syndrome].

The authors report one case of Drash syndrome: association of Wilms' tumor, nephropathy and genital abnormalities. The definition, prognosis and origin of this triad are discussed.

Cadaver↗
PubMed1991

Myelofibrosis in a child suffering from a hypereosinophilic syndrome with trisomy 8: response to corticotherapy.

The idiopathic hypereosinophilic syndrome (IHS) is extremely rare in childhood and relationships of this syndrome with myeloproliferative diseases are controversial. We reported the observation of a 7-year-old girl suffering from an IHS with myelofibrosis. A clonal cytogenetic abnormality, trisomy 8, was detected in the bone marrow cells of this child. This is the decisive proof of a myeloproliferative disorder. IHS with myelofibrosis is usually considered as unresponsive to corticotherapy. In our case, corticotherapy resulted in a rapid, complete, and lasting disappearance of myelofibrosis. Complete remission of the disease, however, was not achieved and the trisomy 8 persisted after treatment.

Child↗
PubMed1990

Hepatic hypervitaminosis A: a familial observation.

Four siblings with hepatic fibrosis are described. The liver damage in these patients was secondary to chronic ingestion of massive doses of vitamin A for congenital ichthyosis. Although the extrahepatic manifestations were helpful in the diagnosis of hypervitaminosis A, the distinctive features of hepatic histopathology were confirmatory. The plasma concentrations of vitamin A and retinol-binding protein were misleading. The recovery from the liver damage in these patients was slow despite a complete withdrawal of the vitamin A intake. These cases show the importance of hepatic vitamin A assessment in the diagnosis of hepatic fibrosis.

Adult↗
PubMed1990

[Melanotic neuroectodermal tumor of childhood or melanotic progonoma. Apropos of a case which recurred as an osteogenic tumor].

We are yielding a case of recurring melanotic neuroectodermal tumor of infancy situated in the premaxilla, with a very quick evolution, haemorrhage, and which has precociously recurred after surgical removal. This tumor is studied on the paraclinical (CT scan, M.I.R.) and on the histological hand (immunohistochemical and ultra-microscopy). This observation is then compared with those of literature in which we find about 200 cases. The neurocristopathic histogenesis is actually doing the unanimity. The prognosis must now be quite reserved because of the recurrences (one case for six) and the possibility of malignant forms, some with metastasis.

Connective Tissue↗