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Biomedical subjects

C Russo

Publications and source records attributed to C Russo.

At least 199 records · Page 11Linked to original sources
PubMedDate not supplied

Resistance to multiple steroids in two sisters.

A 14-year-old Native American girl from the Iroquois Nation was referred as a potential patient with the syndrome of Apparent Mineralocorticoid Excess. Instead, her evaluation revealed resistance to glucocorticoids, mineralocorticoids, and androgens. She lacked Cushingoid features in spite of significantly high cortisol levels. Menstruation was regular and there was no clinical evidence of masculinization despite high serum androgen levels in the male range. The patient's sister had similar clinical features. Partial resistance to exogenous glucocorticoid and mineralocorticoid administration was well demonstrated in both patients. It is proposed that these patients represent the first cases of partial resistance to multiple steroids, possibly owing to a coactivator defect.

Adolescent↗
PubMedDate not supplied

A multi-institutional retrospective study of intracranial ependymoma in children: identification of risk factors.

PURPOSE: The goal of this multi-institutional retrospective study of children with intracranial ependymoma was to identify risk factors associated with unfavorable overall survival (OS) and event-free survival (EFS). PATIENTS AND METHODS: Clinical data, including demographics, tumor location, spread, histology, details of surgery, radiation treatment, and chemotherapy were collected. Clinical characteristics and univariate and multivariate analyses of risk factors for OS and EFS are presented. RESULTS: Eleven U.S. institutions contributed 83 patients treated from 1987 to 1991. The OS at 5 and 7 years was 57% and 46%, and EFS at 5 and 7 years was 42% and 33%. Patients 3 years of age or younger differed from the older group by more common infratentorial location, less common gross total resection (GTR), and postoperative use of chemotherapy rather than radiation. This younger group of patients had worse survival (P < 0.01) than the older age group. Other than young age, less than GTR and World Health Organization (WHO) II grade 3 histology were significant adverse risk factors for EFS in univariate and multivariate analyses. OS shared the same adverse risk factors except for histology in multivariate analysis, which was only of borderline significance (P = 0.05). Progression at the original tumor location, present in 89% of patients, was the major pattern of tumor recurrence. Adjuvant chemotherapy in the group older than 3 years or craniospinal radiation in M0 patients did not significantly change EFS. CONCLUSIONS: Adverse outcome in childhood intracranial ependymoma is related to age (3 years or younger), histology (grade 3), and degree of surgical resection (less than GTR). New approaches, particularly for local tumor control in younger patients, are needed to improve survival.

Brain Neoplasms↗
PubMedDate not supplied

SIVrcm infection of macaques.

In a prior report, we described the isolation and characterization of SIVrcm, a distinct primate lentivirus found in a household pet Red-Capped Mangabey (RCM) in Gabon. SIVrcm is divergent from HIV-1 and HIV-2/SIV families of primate lentiviruses. In this report, additional in vitro replication studies and the results of SIVrcm infection in macaques are presented. SIVrcm causes little cytopathic effedct in Molt 4 Clone 8 cells and in rhesus and human PBMCs. In vivo, SIVrcm is non-pathogenic after 200 days in rhesus macaques and after one year in cynomolgous macaques, but does cause a chronic infection in both macaques.

Acquired Immunodeficiency Syndrome↗
PubMedDate not supplied

Conformation dependent pro-apoptotic activity of the recombinant human prion protein fragment 90-231.

The transition of prion protein from a mainly alpha-structured isoform (PrPC) to a beta sheet-containing protein (PrPSc) represents a major pathogenetic mechanism in prion diseases. To study the role of PrP structural conformation in prion-dependent neurodegeneration, we analysed the neurotoxicity of PrP in alpha and beta conformations, using a recombinant protein encompassing amino acids 90-231 of the human PrP (hPrP90-231). Using controlled thermal denaturation (53 degrees C, 1h) we converted hPrP90-231 in a structural isoform displaying PrPSc-related characteristics: high beta sheet content, increased aggregability and a slight increase in the resistance to protease K. In virtue of these structural changes, hPrP90-231 powerfully affected the survival of SH-SY5Y cells, inducing a caspase-3 and p38- dependent apoptosis. Conversely, in the native alpha-helix-rich conformation, hPrP90-231 did not show significant cell toxicity. The relationship between the structural state of hPrP90-231 and its neurotoxicity was demonstrated, inducing the thermal denaturation of the peptide in the presence of Congo red that prevented both the transition of hPrP90-231 into a beta-rich isoform and the acquisition of toxic properties. In conclusion, we report that the toxicity of hPrP90-231 is dependent on its three-dimensional structure, as is supposed to occur for the pathogen PrP during TSE.

Amyloid↗
PubMedDate not supplied

The problematic issue of Kufs disease diagnosis as performed on rectal biopsies: a case report.

Kufs disease, the late-onset form of a group of neurodegenerative disorders, known as the neuronal ceroid-lipofuscinoses, is characterized by intraneuronal/extraneuronal accumulation of proteins that are visible as fingerprint inclusions and granular osmiophilic deposits (GRODs) at the ultrastructural level. A problematic case of Kufs disease in a 53-year-old female affected by progressive gait disturbances, myoclonus, epilepsy, and profound dementia is presented. Laboratory, biochemical, and molecular genetic tests were unremarkable. A magnetic resonance imaging of the brain revealed a moderate atrophy over both hemispheres with no white matter changes. Ultrastructural examination of rectal mucosa biopsies showed fingerprint inclusions in perivascular smooth muscle cells. Pathological examination of autoptic tissues showed numerous intraneuronal PAS-positive, diastase-resistant inclusions corresponding to GRODs at the ultrastructural examination, but no fingerprint inclusions. Cerebellum, skeletal, and cardiac muscles, skin, and liver were unaffected. The present case illustrates the diagnostic difficulties encountered while examining Kufs disease pathological samples. Main problematic issues include (1) specificity and diagnostic value of fingerprint inclusions when found exclusively in perivascular smooth muscle cells; (2) safe distinction of GRODs from lipofuscin inclusions in cerebral tissue; and (3) reliability in using extraneural tissues and, in particular, rectal mucosa biopsies for diagnostic purposes.

Biopsy↗
PubMedDate not supplied

[Anti-BHc determination in blood donors in São Paulo: should this test be adopted in Brazil?].

PURPOSE: to study the incidence of anti-HBc (core) as a surrogate marker for post-transfusion Non-A, Non-B Hepatitis (HNANB-PT) among blood donors in São Paulo, Brazil. TYPE: prospective, screening all blood donors from September to December, 1989 (nr. 2,773 donors). PLACE: Sírio-Libanês Hospital and 9 de Julho Hospital (São Paulo). PATIENTS: a total of 2,773 donors, 84% male and 16% female. METHOD: the tests used were competitive ELISA for Anti-HBc. MEASUREMENTS AND RESULTS: the repeated rah reactivity (RR) was 10.2% among all donors, with a higher incidence in males than in females (10.9% x 6.8% p less than 0.05 6y X2). Only 4.5% were borderlines, and 94.5% showed an absorbance/cut-off ratio less than 0.9. CONCLUSIONS: despite the lack of prospective studies correlating HNANB-PT to surrogate markers (e.g. ALT and anti-HBc) in this country, the high incidence of anti-HBc in donors allows to conclude that it might be as high as reported in other countries. Although the costs related to the adoption of this test, its indication in other countries and its association with the newly-developed specific test (anti-HCV) supports the idea of anti-HBc as a screening test for HNANB-PT in Brazil, at least in the most developed blood centers in the country.

Adolescent↗
PubMedDate not supplied

[Treatment of pathological obesity biliopancreatic bypass operations: one-year follow-up of 10 cases (author's transl)].

A biliopancreatic bypass operation has been used to treat obese patients since June 1978, and 10 women have been followed-up for over a year. Biliopancreatic shunts reduce food absorption by eliminating enzymatic and biliary secretion activity in the intestinal lumen. Bodyweight loss was satisfactory, was proportional to excess weight, and was evaluated as being a mean of 33.1 kg. The constant and sometimes dramatic diarrhea provoked by conventional jejuno-ileal bypass operations was not observed. Hepatic steatosis, frequently present to varying degrees, was never made worse : in one case it even disappeared completely, while in another case biological tests conducted one year after operation showed considerable regression. Neither severe complications nor mortality were reported in this series.

Adult↗