Sensory neuronopathy is a specific and disabling neurological manifestation of autoimmune hepatitis.

BACKGROUND AND PURPOSE: Neurological manifestations have been identified in the context of autoimmune hepatitis (AIH). Previous case reports highlighted the association between AIH and sensory neuronopathy (SN). Despite that, little is known about the frequency of AIH-related SN and its clinical/neurophysiological profile. Moreover, it is not clear whether SN is an AIH-specific manifestation or related to chronic liver damage. METHODS: Seventy consecutive AIH patients were enrolled and their characteristics were compared with 52 consecutive patients with chronic active hepatitis B. All subjects underwent clinical and neurophysiological evaluation. Further comparisons were performed between AIH SN and AIH non-SN patients. RESULTS: Mean ages and male:female proportions in the AIH and chronic active hepatitis B groups were 42.2 ± 16.3/51.7 ± 13.6 years and 14:56/29:23, respectively. The frequencies of carpal tunnel syndrome, radiculopathy and polyneuropathy were similar between groups. In contrast, SN was identified only in AIH patients (5/70 vs. 0/52, P = 0.04); the overall prevalence of AIH-related SN was 7% with an average profile of a woman in her 40s with asymmetric onset of sensory deficits that chronically evolved to disabling proprioceptive ataxia associated with marked dysautonomia. Neurological disability and hepatocellular damage did not follow in parallel. Anti-fibroblast growth factor receptor type 3 antibodies were found in 3/5 (60%) of the patients with AIH-related SN. Clinical or demographic predictors of SN in the context of AIH could not be identified. CONCLUSION: Sensory neuronopathy, but not other peripheral nervous system diseases, is a specific AIH neurological manifestation. It is often disabling and, in contrast to hepatocellular injury, does not respond to immunosuppression.

Developmental and neurodegenerative damage in Friedreich's ataxia.

BACKGROUND AND PURPOSE: Friedreich's ataxia (FRDA) is the most common autosomal-recessive ataxia worldwide. It is characterized by early onset, sensory abnormalities and slowly progressive ataxia. All magnetic resonance imaging (MRI)-based studies have focused on the evaluation of adult patients. Therefore, we designed a cross-sectional multimodal MRI-based study to investigate the anatomical substrates involved in the early stages of FRDA. METHODS: We enrolled 37 patients (12 children) and 38 controls. All subjects underwent MRI in a 3T device to assess gray and white matter. We used measures from FreeSurfer and CERES to evaluate the cerebral and cerebellar cortices. The T1 multiatlas assessed deep gray matter. The diffusion tensor imaging multiatlas was used to investigate microstructural abnormalities in brain white matter and SpineSeg was used to assess the cervical spinal cord. All analyses were corrected for multiple comparisons. RESULTS: Comparison with age-matched controls showed that pediatric patients have spinal cord, inferior cerebellar peduncle and red nucleus damage. In contrast, adult patients showed more widespread white matter damage than pediatric patients. With regard to gray matter, we found cortical thinning at the left central sulcus and volumetric reduction in the thalami and hippocampi only in adult patients. Finally, values of fractional anisotropy in adult patients and radial diffusivity in pediatric patients from the inferior cerebellar peduncle correlated with disease duration and ataxia severity, respectively. CONCLUSIONS: Structural damage in FRDA begins in the spinal cord and inferior cerebellar peduncle as well as the red nucleus, and progresses to cerebral areas in adulthood. These results shed some light on the early stages of FRDA and highlight potential neuroimaging markers for therapeutic trials.

Registered designation of origin areas of fermented food products defined by microbial phenotypes and artificial neural networks.

Cheese produced from raw ewes' milk and chouriço, a Portuguese dry fermented sausage, are still produced in a traditional way in certain regions of Portugal by relying on colonization by microbial populations associated with the raw materials, equipment, and local environments. For the purpose of describing the product origins and types of these fermented foods, metabolic phenotypes can be used as descriptors of the product as well as to determine the presence of compounds with organoleptic value. The application of artificial neural networks to the metabolic profiles of bacterial isolates was assayed and allowed the separation of products from different regions. This method could then be used for the Registered Designation of Origin certification process of food products. Therefore, besides test panel results for these traditionally produced food products, another tool for validating products for the marketplace is available to the producers. The method can be improved for the detection of counterfeit products.

An international perspective on the treatment of pediatric shock: the Brazilian experience.

Shock in the pediatric population has many preventable causes. Treatment of children in shock will depend on access to health services, training of health personnel, availability of diagnostic procedures, monitoring, and therapeutic measures. Countries will differ among themselves and within themselves in the care provided to children developing shock. In Brazil, the majority of children are cared for in public hospitals, which often lack resources for basic care. Many children in shock do not even reach healthcare services. Investment in training healthcare personnel in a simplified and systematic approach to shock and access to equipped health services are basic to improved outcomes in the treatment of pediatric shock. The Brazilian experience in the treatment of children in shock outside hospital facilities, in the emergency department, and in the ICU is described.

Silicosis in Brazilian pit diggers: relationship between dust exposure and radiologic findings.

The incidental diagnosis of two cases of silicosis at Messejana Hospital, Fortaleza, capital of Ceará state, triggered a field research conducted with the objective of investigating the incidence of silicosis in pit diggers in four towns in the Ibiapaba Mountain Range, at the state's northwestern area, northeastern Brazil. Out of a universe of 1,050 pit diggers in the area, 366 participated in the study; they were all males ranging in age between 16 and 76 years old (average 39 years). The excavation and the sickening process were described and a dust exposure index in pit diggers (DEIPD) was defined. The study sample indicated a rate of 121 (33.06%) silicotics and possibly silicotics. The radiologic findings were related to the DEIPD and indicated significant prevalence of disease. Furthermore, during the research period, from 1986 to 1989, 30 (34.9%) silicotics died of respiratory failure and wasting of body tissues. The data suggested that these men were afflicted with a severe occupational health problem, possibly scattered throughout a large area in northeastern Brazil, where manual pit excavations occur during the whole year and increase during drought periods.

Sexual development of male Wistar rats.

1. Sexual development was investigated in male Wistar rats from 22 to 97 days of age by morphometric, biochemical and radioimmunological methods. 2. The first significant increase of plasma testosterone (T) occurred from 40 to 50 days of age and a progressive enhancement was observed thereafter to a maximum at 76 days (5.4 +/- 0.9 ng/ml). From that time onward, plasma T was gradually depressed to adult levels at 97 days of age (2.0 +/- 0.3 ng/ml). Plasma prolactin increased in parallel to T, reaching a maximum at 76 days (9.2 +/- 0.9 ng/ml) and attaining a lower plateau by 83 to 97 days of age (5.0 +/- 0.5 ng/ml). A small but significant increase was observed in plasma luteinizing hormone from 22 to 83 days of age. Plasma follicle stimulating hormone was high at 22 days, increased to a maximum at 40 days (15.4 +/- 0.6 ng/ml) and fell slowly to a lower plateau by 76 to 97 days of age. 3. Fructose content in the ventral prostate increased abruptly from 50 to 63 days of age (148.8 +/- 19.8 micrograms) and no significant change was observed thereafter. A progressive increase in the seminal vesicle fructose content was observed from 40 to 63 days (45.6 +/- 2.8 micrograms) when a plateau was reached. 4. The evolution of the germinal epithelium was investigated in cross-sections of seminiferous tubules analyzed at random for the presence of the most advanced germ cell and also for sperm production (estimated by the number of spermatids in stages 15 to 18 of spermiogenesis).(ABSTRACT TRUNCATED AT 250 WORDS)

Sexual development of male Wistar rats

1. Sexual development was investigated in male Wistar rats from 22 to 97 days of age by morphometric, biochemical and radioimmunological methods. 2. The first significant increase of plasma testosterone (T) occurred from 40 to 50 days of age and a progressive enhancement was observed thereafter to a maximum at 76 days (5.4 +/- 0.9 ng/ml). From that time onward, plasma T was gradually depressed to adult levels at 97 days of age (2.0 +/- 0.3 ng/ml). Plasma prolactin increased in parallel to T, reaching a maximum at 76 days (9.2 +/- 0.9 ng/ml) and attaining a lower plateau by 83 to 97 days of age (5.0 +/- 0.5 ng/ml). A small but significant increase was observed in plasma luteinizing hormone from 22 to 83 days of age. Plasma follicle stimulating hormone was high at 22 days, increased to a maximum at 40 days (15.4 +/- 0.6 ng/ml) and fell slowly to a lower plateau by 76 to 97 days of age. 3. Fructose content in the ventral prostate increased abruptly from 50 to 63 days of age (148.8 +/- 19.8 micrograms) and no significant change was observed thereafter. A progressive increase in the seminal vesicle fructose content was observed from 40 to 63 days (45.6 +/- 2.8 micrograms) when a plateau was reached. 4. The evolution of the germinal epithelium was investigated in cross-sections of seminiferous tubules analyzed at random for the presence of the most advanced germ cell and also for sperm production (estimated by the number of spermatids in stages 15 to 18 of spermiogenesis).(ABSTRACT TRUNCATED AT 250 WORDS)