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Biomedical subjects

C Scheiner

Publications and source records attributed to C Scheiner.

At least 19 recordsLinked to original sources

Nitric oxide synthase distribution in the cat superior colliculus and co-localization with choline acetyltransferase.

Nitric oxide and acetylcholine are important neuromodulators implicated in brain plasticity and disease. We have examined the cellular and fiber localization of nitric oxide in the cat superior colliculus (SC) and its degree of co-localization with ACh using nicotinamide adenine dinucleotide phosphate diaphorase (NADPHd) histochemistry and an antibody to neuronal nitric oxide synthase. ACh was localized using an antibody against choline acetyltransferase. We also made injections of biocytin into the region of the parabrachial brainstem to confirm that this region is a source of nitric oxide containing fibers in SC. NADPHd labeled neurons within the superficial layers of the superior colliculus included pyriform, vertical fusiform, and horizontal morphologies. Labeled neurons in the intermediate gray layer were small to medium in size, and mostly of stellate morphology. Neurons in the deepest layers had mostly vertical or stellate morphologies. NADPHd labeled fibers formed dense patches of terminal boutons within the intermediate gray layer and streams of fibers within the deepest layers of SC. Choline acetyltransferase antibody labeling in adjacent sections indicated that many fibers must contain both labels. Over 94% of neurons in the pedunculopontine tegmental and lateral dorsal tegmental nuclei were also labeled by both NADPHd and choline acetyltransferase. In addition, biocytin labeled fibers from this region were localized in the NADPHd labeled patches. We conclude that nitric oxide is contained in a variety of cell types in SC and that both nitric oxide and ACh likely serve as co-modulators in this midbrain structure.

Animals↗

Giant-cell fibroblastoma and dermato fibro sarcoma protuberans: the same tumoral spectrum? Report of two cases of association in children.

We describe two cases of giant-cell fibroblastoma (GCF) with dermato fibro sarcoma protuberans (DFSP) component, occurring in two children in a chest wall localization. One case recurred 1 year later. The two patients were tumor-free 12 and 8 years later. GCF is a rare mesenchymal cutaneous and subcutaneous tumor reported mostly in the first two decades of life. Dermato fibro sarcoma protuberans, occurring preferentially in adults, is a rare skin tumor with a pronounced tendency to local recurrence. Some cases of association of recurrence of GFC under the form of DFSP have been reported, raising the question of a continuum between the two tumors. The treatment of choice of the two tumors is a wide local excision.

Cell Transformation, Neoplastic↗

[Accessory retrograde aneurysmal aortic lumen supplying the inferior mesenteric artery].

We report the case of a patient with infrarenal abdominal aortic aneurysm with mural thrombus covering the ostium of a patent inferior mesenteric artery (IMA). The IMA was supplied via flow from an accessory aneurysmal lumen within the mural thrombus that filled retrogradely from the aorta. This unusual pattern, associated with calcifications within the thrombus, raised the possibility of chronic aortic dissection.

Aged↗

Myositis ossificans: report of seven cases in children.

The clinical features of seven children with myositis ossificans (circumscripta and progressiva) and radiographic signs of the disease are described. We recommend systematic radiological examination to seek other skeletal malformations for congenital hallux valgus in young children, for it may be the first sign of a myositis ossificans progressiva. The "zone phenomenon" observed on histology, along with differential diagnosis and evolution, is documented. The necessity of a biopsy and different forms of treatment are discussed.

Adolescent↗

i(18q) in amniotic and fetal cells with a normal karyotype in direct chorionic villus sampling: cytogenetics and pathology.

A case of false-negative discrepancy between results of chorionic villi (direct preparation) and those of fetal tissue with an isochromosome 18q [i(18q)] in amniotic cells and fetal blood is reported. Fluorescence in situ hybridization (FISH) confirmed this uncommon chromosomal rearrangement. The fetus showed cyclopia and multiple congenital anomalies which have never been reported in cases of i(18q).

Abnormalities, Multiple↗

CD24, a glycosylphosphatidylinositol-anchored molecules is transiently expressed during the development of human central nervous system and is a marker of human neural cell lineage tumors.

CD24 is a glycoprotein with an unusual structure consisting of a small protein core extensively glycosylated and linked to the outer surface of the plasma membrane by a glycosylphosphatidylinositol (GPI) lipid anchor. Its murine homolog mCD24 is transiently expressed during the development and differentiation of the hematopoietic and neural cell lineages. We have searched for the expression of CD24 in the developing and in the mature human brain as well as in a wide range of neuroectodermal tumors. Neuroblastomas, a subgroup of tumors able to maturate from undifferentiated features towards mature ganglioneuromas, were more extensively studied. Immunohistochemical studies demonstrated that CD24 is transiently expressed by neurons during human brain development. In neuroectodermal tumors, CD24 is a marker of neuronal tumors. Furthermore, in neuroblastomas, CD24 expression decreases as tumors differentiate. In non-neuronal neuroectodermal tumors, CD24 expression is mostly absent. When present, it correlates with the emergence of anaplastic histological features. Reverse transcriptase -polymerase chain reaction (RT-PCR) demonstrated the presence of an unique transcript identical in both hematopoietic, developing and tumoral nervous tissue. RT-PCR and in situ hydridization techniques showed that CD24 expression is transcriptionally regulated. Interestingly, Western blot analysis demonstrated differential CD24 isoforms according to the tissue (hematopoietic versus nervous), the differentiation status, and the origin of neuroblastomas likely reflecting variations in the extent of glycosylation. This indicates an additional level of regulation of CD24 involving post-translational modifications.

Adult↗

[Pneumoblastoma in children. A clinical case and review of the literature].

Pulmonary blastoma is a rare malignant tumor. A new case is reported in a 3 years 6 month-old girl. An apparent clinical remission was first obtained after a surgical treatment followed by a conventional chemotherapy during six months. Afterwards the persistence of a microscopic residual pulmonary disease lead us to deliver successfully an intensive chemotherapy followed by autologous peripheral blood stem cells reinjection. The child remains disease free 12 months after graft. Problems set by the histogenesis of this tumor, its unspecific clinical and paraclinical features and the role of conventional and intensive chemotherapy followed by autologous bone marrow transplantation are discussed on the basis of a review of 50 cases in the literature.

Antineoplastic Combined Chemotherapy Protocols↗

Measurement of facial growth in the human fetus.

BACKGROUND: The fetal face is clearly seen by ultrasonography: we considered measurement of certain orbitofacial parameters of interest in the human fetus in order to establish norms for facial development. METHODS: We included 108 "normal" fetuses ranging in age from 16.5 to 41 weeks of amenorrhea. The orbitofacial parameters studied were outer canthal distance, inner canthal distance, oropalpebral distance right side and left side, and palpebral fissure length side and left side. The ocular parameters studied were corneal horizontal diameter and axial length. The traditional anthropometric parameters of the fetus were determined by pathological examination: age, weight head circumference and height. A statistical study analyzed the different correlations and established linear regression equations for orbitofacial parameters as a function of age. Polynomial regression models were tested to the third degree as a function of age and the head circumference/II ratio. RESULTS: Results are given in six different age groups. We find excellent correlation between the different parameters. Statistically valid linear regression equations were established for orbitofacial parameters. Polynomial regression equations were compared to linear equations their correlation coefficient and standard error, but showed no greater validity. Skull growth is more rapid than facial growth, which itself is more rapid vertically than horizontally. CONCLUSION: This study establishes norms for the different orbitofacial parameters, in particular the oropalpebral distance, for which we found no bibliographic references. The general interest of these measures lies in the description of malformation syndromes.

Anthropometry↗

[Melanotic neuroectodermal tumor in an infant].

BACKGROUND: Melanotic neuroectodermal tumor is a mostly benign tumor, rare in childhood, essentially located in the head and neck region. CASE REPORT: A two-month-old girl was seen for a rapidly increasing odontogenic tumor which appeared cystic at the CT scan. After enucleation, this premaxillar tumor recurred one month later with an osteogenic aspect at the CT scan. Urine catecholamine excretion was normal; Methyl Iodo Benzyl Guanidin scintigraphy failed to show any fixation and electron microscopy examination of the biopsy showed several varieties of melanocytes. A partial maxillectomy was performed by oral approach. The patient is well 4 1/2 years later. CONCLUSION: This observation confirms the recurrence potential of this tumor and the cosmetic interest of the oral surgical approach.

Female↗

Impact of prenatal diagnosis by ultrasound on the prevalence of congenital anomalies at birth in southern France.

STUDY OBJECTIVE: The aims were (1) to assess whether termination of pregnancy after prenatal screening by ultrasound affected the prevalence of congenital anomalies at birth, and (2) to examine the trend of this pattern over time. DESIGN: This study deals with congenital anomalies, possibly detectable prenatally or at birth, which were classified as isolated and multiple anomalies; chromosomal anomalies were not included. The prevalence rates of congenital anomalies at birth were determined from case registration data in the Marseille district, France, from the registry of congenital malformations (Eurocat no 22), which covers 23,500 births a year. The chi 2 test for homogeneity in proportions was used to test whether the differences in the total prevalence rates were significant over time. SETTING: The population was defined as all children born to parents living in the Marseille district between January 1 1984 and December 31 1990. PATIENTS: Among the 164,509 pregnancy outcomes monitored during the study, 1795 children with a single congenital anomaly and 288 with multiple congenital anomalies detectable at birth were assessed. MEASUREMENTS AND MAIN RESULTS: The percentage of pregnancy terminations was higher in the case of multiple anomalies (16%) than with single ones (7.5%). Leaving aside the lethal birth defects, this percentage became 7.9% in the case of multiple anomalies and 4.3% with isolated ones. A significant increase (p < 0.001) occurred over the seven year study period in the total percentage of terminations because of isolated anomalies but not in that involving multiple ones. The increase observed in the former case was found to be mainly attributable to an increase in the number of terminations of pregnancy undertaken because of anomalies which were either lethal or associated with very low survival rates (p < 0.001). CONCLUSIONS: Termination of pregnancy after prenatal ultrasound examination was found to have a definite impact on the prevalence at birth of lethal and congenital anomalies with a low survival rate, and this impact tended to increase over time. No such impact was observed in the case of congenital anomalies associated with high survival rates.

Abnormalities, Multiple↗

Cyclosporin A has divergent effects on plasma LDL cholesterol (LDL-C) and lipoprotein(a) [Lp(a)] levels in renal transplant recipients. Evidence for renal involvement in the maintenance of LDL-C and the elevation of Lp(a) concentrations in hemodialysis patients.

Cardiovascular disease is the major cause of mortality in renal transplant recipients. Plasma levels of low-density lipoprotein cholesterol (LDL-C) are often elevated following renal transplantation, and the immunosuppressant cyclosporin A has been implicated as a predisposing factor for posttransplantation hyperlipidemia. Lipoprotein(a) [Lp(a)] is an LDL-like lipoprotein particle; elevated levels of Lp(a) provide an independent and significant risk factor for cardiovascular disease. Plasma concentrations of Lp(a) vary greatly among individuals, and the mechanisms that govern changes in their levels in transplant patients are unknown. The effect(s) of cyclosporin A on Lp(a) was studied in two groups of renal transplantation patients. In group I plasma lipoproteins including Lp(a) were measured before and after successful renal transplantation; this group received both prednisone and cyclosporin A for immunosuppression. Group II patients were studied after renal transplantation and received prednisone alone for immunosuppression. Following surgery, group I patients demonstrated increased plasma concentrations of LDL-C (mean +/- SEM range, 111 +/- 6 to 142 +/- 17 mg/dL; P < .005). In contrast, plasma Lp(a) levels for this group were markedly decreased after renal transplantation (median, 34.3 to 19.7 mg/dL). Patients not treated with cyclosporin A (group II) exhibited mean LDL-C and median Lp(a) levels (118 +/- 42 and 33.1 mg/dL, respectively) that were remarkably similar to those observed before renal transplantation (group I). These data confirm that hyperlipidemia following renal transplantation is associated with cyclosporin A therapy and show that this drug has opposing effects on plasma Lp(a) and LDL-C accumulations.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Lung toxoplasmosis after HLA mismatched bone marrow transplantation.

We report a clinically isolated toxoplasma pneumonitis in a child treated by HLA haplo-mismatched BMT. Conditioning consisted of TBI, cytarabine and melphalan. The BM graft was T-depleted and the boy received iv moAb antiLFA1 and antiCD2. The clinical course of pneumonitis was characterised by an early onset (day 28) and a rapidly overwhelming course. Donor and recipient had pre-graft IgG Ab against toxoplasma without IgM. These Abs had completely disappeared from the serum of the patient at the time of pneumonitis. PCR amplification detected the B1 gene of Toxoplasma gondii in the patient's PBMC from day 28.

Animals↗

Hydroxyapatite in experimental laryngotracheal reconstruction.

Current concepts in laryngotracheal reconstruction include lumen augmentation with interposition of autogenous costal cartilage. In an effort to reduce potential increased morbidity at the donor site, the use of porous hydroxyapatite was compared with autogenous costal cartilage in an experimental study. Ninety-nine New Zealand rabbits were randomly operated on to receive autogenous costal cartilage (n = 32), hydroxyapatite covered by a perichondrium graft (n = 33), or hydroxyapatite alone (n = 34). Rabbits were killed at 3 months after the surgery. Clinically (n = 85), no difference was found among the groups. Histologically (n = 81), grafts (n = 54) in the group receiving autogenous costal cartilage were greater than in those receiving the hydroxyapatite. Concerning the value of epithelial layer covering the graft, the rate of inflammation, the cricoid-graft interface, and the graft viability, no difference was noted among the groups. In the group in which no graft was found (n = 27), a fibrous bridge maintaining a cricoid distraction was noted in 23 cases. This study shows the possible value of hydroxyapatite in the animal model, but before clinical application, the natural course of the fibrous bridge must be documented.

Animals↗

Physeal and epiphyseal extent of primary malignant bone tumors in childhood. Correlation of preoperative MRI and the pathologic examination.

Twenty-two patients with metaphyseal primary malignant bone tumors (17 osteosarcomas, 5 Ewing's tumors) occurring before closure of the growth plate were examined with plain radiographs and MRI in order to determine the physeal or epiphyseal extent of the tumor. Results were correlated with the pathologic examination. Transphyseal spread was pathologically proven in 13 cases (59%): 12 cases of osteosarcoma and 1 case of Ewing's tumor (70% and 20%, respectively). There was no significant relation between epiphyseal invasion, age of patient, length of tumor or, in the cases of osteosarcoma, response to chemotherapy. Plain radiographs showed epiphyseal involvement in 4 cases and there were 10 false negatives. MRI revealed epiphyseal involvement in all cases; there were no false positives or false negatives. T1-weighted images in coronal or sagittal planes appeared to be sufficient. These findings are very useful in planning surgical limb salvage procedures and stress the ineffectiveness of the "barrier effect" of the growth plate against tumor spread.

Adolescent↗

Cervical neuroblastoma and multiple endocrine neoplasia type 2a.

The authors report the case of a female infant who was referred at the age of 18 months for a left latero-cervical mass. This child belongs to a family with a history of multiple endocrine neoplasia (MEN) type 2a (10 persons in 4 generations had medullary thyroid carcinoma and/or pheochromocytoma). Surgery was performed with complete excision of an encapsulated tumor and pathological examination reported a typical ganglioneuroblastoma. No amplification of N-myc oncogene was observed. The probability of the child carrying a MEN-2a gene is estimated 2 to 4%, by DNA analysis. The patient has received no further treatment and is still free of disease 4 years later. The diagnostic problems of this tumor after basic tests were between neuroblastoma and medullary thyroid carcinoma. The link between neuroblastoma and MEN-2a syndrome is discussed because both diseases belong to the group of neural crest tumors.

Female↗