Tree Nuts Anaphylaxis in Preschool Age Children.

Summary: Introduction. The incidence of food-induced anaphylaxis (FIA) is increasing in young children. Although the commonest culprits are cow's milk and egg, FIA to tree nuts (TNs) have been increasing. Objective. Characterization of children referred to our allergy department due to TNs-induced anaphylaxis (TNs-FIA) during preschool age. Materials and methods. We have retrospectively included 25 children with clinical history of preschool TNs-FIA, proven by allergological work-up. TNs sensitization was assessed by skin prick tests and/or specific IgE. Results. The mean age of the first anaphylactic episode was 3.1±1.2 years. The majority (92%) had an allergic disease (52% asthma). The implicated TNs were cashew (11 children), walnut (8), pine nut (5), hazelnut (2) and almond (1). The reaction occurred after the first known ingestion in 68%. In 92%, symptoms appeared within 30 minutes after exposure. The most frequent clinical symptoms were mucocutaneous (96%), respiratory (80%) and gastrointestinal (52%). Twenty-one children were admitted to the emergency department, although only 48% were treated with epinephrine. An underneath IgE-mediated mechanism was proven in all cases. Immunologic cross-reactivity with other TNs was identified in 84%, and with peanut in 36%. Overall, in our center, TNs-FIA represents 18% of all causes of FIA. Conclusions. In preschool age children with TNs-FIA, cashew and walnut were the commonest implicated nuts. Most reactions occurred briefly after exposure to minimal amounts of TNs, demonstrating the high potency of these allergens. About one-third also had peanut sensitization. Potentially life-threatening TNs allergy can occur early in childhood and adequate management should be undertaken.

The Future of Neurocritical Care Research: Proceedings and Recommendations from the Fifth Neurocritical Care Research Network Conference.

The Fifth Neurocritical Care Research Network (NCRN) Conference held in Boca Raton, Florida, in September of 2018 was devoted to challenging the current status quo and examining the role of the Neurocritical Care Society (NCS) in driving the science and research of neurocritical care. The aim of this in-person meeting was to set the agenda for the NCS's Neurocritical Care Research Central, which is the overall research arm of the society. Prior to the meeting, all 103 participants received educational content (book and seminar) on the 'Blue Ocean Strategy®,' a concept from the business world which aims to identify undiscovered and uncontested market space, and to brainstorm innovative ideas and methods with which to address current challenges in neurocritical care research. Three five-member working groups met at least four times by teleconference prior to the in-person meeting to prepare answers to a set of questions using the Blue Ocean Strategy concept as a platform. At the Fifth NCRN Conference, these groups presented to a five-member jury and all attendees for open discussion. The jury then developed a set of recommendations for NCS to consider in order to move neurocritical care research forward. We have summarized the topics discussed at the conference and put forward recommendations for the future direction of the NCRN and neurocritical care research in general.

Evaluation of the effects of quercetin on brain lesions secondary to experimental hydrocephalus in rats.

INTRODUCTION: Hydrocephaly is a disease that affects not only the dynamics of the cerebrospinal fluid, but also other structures of the central nervous system. Although shunt is effective in reducing ventriculomegaly, many neurological damages are not reversed with surgery. Several studies demonstrate that oxidative stress is involved in the genesis of hydrocephalus lesions. OBJECTIVE: Evaluate the neuroprotective response of quercetin in hydrocephalus. MATERIALS AND METHODS: Male newborns rats were used, which received the 15% kaolin injection in the cisterna magna for induction of hydrocephalus. They were divided into control group (C), untreated hydrocephalic (HN), shunted hydrocephalic (HD), hydrocephalic treated with distilled water (HA), hydrocephalic treated with distilled water and shunt (HDA), hydrocephalic treated with quercetin peritoneal (HQp), hydrocephalic treated with quercetin peritoneal and shunt (HDQp), hydrocephalic treated with quercetin by gavage (HQg), and hydrocephalus treated with quercetin by gavage and shunt (HDQg). RESULTS: Quercetin significantly improved the immunohistochemical markers, mainly caspase and GFAP. There were no significant changes in clinical/behavioral assessment. The use of isolated quercetin does not alter the volume and ventricular size, and the realization of ventriculo-subcutaneous shunt in newborn rats with hydrocephalus presents a high morbi-mortality. CONCLUSION: The use of quercetin shows laboratory improvement of the effects of glial lesion and corpus callosum fibers and is therefore not justified by the use of the routine substance as neuroprotective.

Polyphenols from guaraná after in vitro digestion: Evaluation of bioacessibility and inhibition of activity of carbohydrate-hydrolyzing enzymes.

Guaraná (Paullinia cupana) is a typical product from Amazon biota that exerts antioxidant capacity due to the presence of phenolic compounds, such as catechin, epicatechin and proanthocyanidins. The objective of this study was to evaluate the potential inhibitory activity of guaraná extracts, after digestion in vitro, on carbohydrates-metabolism enzymes and to assess the bioacessibility of guaraná polyphenols. The guaraná samples before and after enzymatic digestion, were compared for total phenolic content and phenolic profile. Furthermore, we investigated the uptake of polyphenols from guarana, using Caco-2 cells, and the effect of digested guaraná on carbohydrate metabolism enzymes. The amount of total phenolic compounds extracted from guaraná decreased after digestion in vitro, and native phenolics were not identified after cell permeation. On the other hand, polyphenols from guaraná were able to inhibit α-glucosidase and α-amylase activities. In conclusion, guaraná can be considered as a dietary source with anti-hyperglycemic potential.

Nitric oxide and immune response in infection control of Caseous Lymphadenitis / Óxido nítrico e resposta imune no controle de infecção da Linfadenite Caseosa

This study aimed to evaluate aspects of host immune response using an experimental infection model of Corynebacterium pseudotuberculosis (CP) in C57/Black6 wild-type and knockout for nitric oxide (KO-NO) mice. 28 mice were evaluated: 4 wild-type controls; 10 wild-type infected with CP; 4 KO-NO controls; 10 KO-NO infected with CP. Infection procedures were carried out by intraperitoneal inoculation using 107. Infected C57/Black6 KO-NO mice began to die after the 5° day post-inoculation, up until the 14º day. Neutrophils were found in increased numbers in the infiltrate of KO-NO murine peritoneal cavities. Examination of splenic tissue revealed an accumulation of lymphocytes, predominantly CD8 T-cells, in experimental animal groups. KO-NO animals were found to have a predominance of granulomas 7 days post-inoculation, primarily in the lymph nodes. In addition, greater amounts of bacteria were recovered from the mesenteric lymph nodes of KO-NO mice. There was no statistically significant difference in the levels of total IgG and its subclasses 14 days post-inoculation between KO-NO and wild groups. The results suggest the importance of nitric oxide in the process of controlling CP infection, as KO-NO animals were observed to be markedly more affected by infection with this bacterium.(AU)

O objetivo deste estudo foi avaliar os aspectos da resposta imune do hospedeiro, mediante o uso de um modelo experimental de infecção de Corynebacterium pseudotuberculosis (CP) em camundongos C57/Black6 do tipo selvagem e em C57/Black6 knockout para o óxido nítrico (KO-NO). Foram avaliados 28 camundongos: quatro controles de tipo selvagem; 10 do tipo selvagem infectados com CP; quatro controles KO-NO; e 10 KO-NO infectados com CP. A infecção foi realizada via intraperitoneal, usando-se 107. Os animais C57/Black6 KO-NO infectados começaram a vir a óbito no quinto dia pós-inoculação, o que aconteceu até o 14º dia. Um número maior de neutrófilos foi encontrado na sua cavidade peritoneal. O exame do baço revelou um acúmulo de linfócitos, predominantemente células T CD8, nos grupos de animais experimentais. Nos animais KO-NO, foi observada a presença de granulomas, sete dias pós-inoculação, principalmente nos gânglios linfáticos. Além disso, uma maior quantidade de bactérias foi detectada dos linfonodos mesentéricos desses animais. Não houve diferença estatisticamente significante nos níveis séricos IgG total e em suas subclasses aos 14 dias pós-inoculação nos grupos KO-NO e selvagem. Os resultados obtidos sugerem a importância do óxido nítrico no processo de controle da infecção por CP.(AU)

One-year survey of paediatric anaphylaxis in an allergy department.

AIM: To determine the frequency of anaphylaxis in an allergy outpatient department, allowing a better understanding regarding aetiology, clinical manifestations and management, in children and adolescents. METHODS: From among 3646 patients up to 18 years old observed during one-year period, we included those with history of anaphylaxis reported by allergists. RESULTS: Sixty-four children had history of anaphylaxis (prevalence of 1.8%), with mean age 8.1±5.5 years, 61% being male. Median age of the first anaphylactic episode was 3 years (1 month-17 years). The majority of patients had food-induced anaphylaxis (84%): milk 22, egg 7, peanut 6, tree nuts 6, fresh fruits 6, crustaceans 4, fish 4 and wheat 2. Food-associated exercise-induced anaphylaxis was reported in 2 adolescents. Drug-induced anaphylaxis occurred in 8%: 4 non-steroidal anti-inflammatory drugs and 1 amoxicillin. Three children had cold-induced anaphylaxis, one adolescent had anaphylaxis to latex and one child had anaphylaxis to insect sting. The majority (73%) had no previous diagnosis of the etiologic factor. Symptoms reported were mainly mucocutaneous (94%) and respiratory (84%), followed by gastrointestinal (42%) and cardiovascular (25%). Fifty-one patients were admitted to the emergency department, although only 33% were treated with epinephrine. Recurrence of anaphylaxis occurred in 26 patients (3 or more episodes in 14). CONCLUSIONS: In our paediatric population, the main triggering agent of anaphylaxis was IgE-mediated food allergy. Epinephrine is underused, as reported by others. Often, children have several episodes before being assessed by an allergist. We stress the importance of systematic notification and improvement of educational programmes in order to achieve a better preventive and therapeutic management of this life-threatening entity.

Toxicidade e repelência de extratos de plantas da caatinga sobre Tetranychus bastosi Tutler, Baker & Sales (Acari: Tetranychidae) em pinhão-manso / Toxicity and repellency of caatinga plant extracts on the Tetranychus bastosi Tutler, Baker & Sales in physic nut

RESUMO O objetivo deste trabalho foi avaliar o efeito repelente e a toxicidade dos extratos aquosos de Myracrodruon urundeuva Fr. All (Anacardiaceae), Croton blanchetianus Baill (Euphorbiaceae) e Ziziphus joazeiro Mart. (Rhamnaceae,) sobre o ácaro Tetranychusbastosi Tutler, Baker & Sales associado à cultura do pinhão- manso Jatropha curcas L. Para cada extrato as concentrações utilizadas foram 0, 5%, 10%, 15%, 20% e 25%. Avaliou-se, em teste sem chance de escolha, a mortalidade de fêmeas adultas de T. bastosi submetidas às diferentes concentrações de cada extrato. O delineamento estatístico foi o inteiramente casualizado, com seis tratamentos (testemunha e concentrações dos extratos) e 10 repetições. Os dados foram submetidos à análise de regressão. Também foi avaliado o efeito repelente dos referidos extratos sobre T. bastosi, nas concentrações supracitadas. Foi calculado o índice de repelência, percentual de repelência, classificação e índice de segurança. Os dados de percentual de repelência de adultos no tratamento e testemunha foram analisados pelo teste T de Student a 5% de probabilidade. De uma forma geral os extratos demonstraram efeito tóxico para adultos de T. bastosi nas concentrações testadas. O extrato de Z. joazeiro apresentou as maiores taxas de mortalidade (90%) média dos indivíduos. No que se refere à repelência destes extratos, todos os tratamentos se mostraram repelentes para fêmeas de T. bastosi, classificados como tratamentos repelentes, exceto para a dosagem de 5% do extrato de M. unrundeuva.

ABSTRACT The aim of this study was to evaluate the repellent effect and toxicity of aqueous extracts of M. urundeuva All Br. (Anacardiaceae), Crotonblanchetianus Baill( Euphorbiaceae ) and Ziziphus joazeiro Mart. (Rhamnaceae) on the mite Tetranychus bastosi Tutler, Baker & Sales associated with Jatropha curcas. For each extract, the concentrations used were 0, 5%, 10 %, 15 %, 20% and 25%. It was evaluated, at a no-choice test, the mortality of adult females of T. bastosi submitted to different concentrations of each extract. The experimental design was completely randomized with six treatments (control and concentrations of the extracts) and 10 repetitions. The data were subjected to regression analysis. The repellent effect of the extracts over the T. bastosi, in the concentrations already mentioned was also evaluated. The repellency index, percentage repellency, classification and safety index were assessed. The data of percentage repellency of adults in treatment and control were analyzed by the T test Student a 5% probability. In general, the extracts showed toxic effect on adults of T. bastosi for the concentrations tested. The extract of Z. joazeiro indicated d the highest average mortality rates (90 %) of individuals. Regarding the repellency of these extracts, all treatments have proved to be repellants for the females of T. bastosiclassified as repellent treatment, except for the 5% dose of the extract of M. unrundeuva.

Efeitos da parede de levedura em dieta úmida na microbiota fecal, na produção de gás e na morfologia intestinal de gatos adultos / Effects of the yeast cell wall in a moist diet in fecal microbial, gas production and intestinal morphology of adult cats

O objetivo deste estudo é avaliar os efeitos do extrato de levedura (EPL) em dietas úmidas sobre a microbiota fecal, a produção de gás e a morfologia intestinal de gatos adultos. Foram utilizados 20 gatos adultos, de ambos os sexos, distribuídos ao acaso em quatro tratamentos: 1) dieta comercial úmida (controle); 2) controle + 0,2% de extrato de levedura em matéria seca; 3) controle + 0,4%; e 4) controle + 0,6%. Foram realizadas a microbiologia fecal, a avaliação da morfologia intestinal por meio de exames radiográficos, ultrassonográficos e de colonoscopia, bem como a biópsia para histologia intestinal. Não foram observadas diferenças significativas (p>0,05) para contagem de bactérias do ácido lático e de clostrídio sulfito redutor, área de gás em alças intestinais (avaliação radiográfica), espessura da parede do cólon (ultrassonografia intestinal) e contagem de colonócitos/células caliciformes (histologia). Por meio da colonoscopia, notaram-se alterações em características de mucosa em animais submetidos ao tratamento 4. Conclui-se que a adição de até 0,6% de EPL não teve efeito sobre os parâmetros avaliados, mas novos estudos são necessários para compreender os mecanismos de ação e os efeitos desse aditivo para gatos domésticos.

The aim of this study is to evaluate the effects of yeast extract (EPL) in the moist diet on the fecal microbiotal, gas production and intestinal morphology of adult cats. Twenty adult cats from both sexes were randomly assigned to four treatments: 1) moist commercial diet (control); 2) control + 0,2% yeast extract dry matter; 3) control + 0,4%; and 4) control + 0,6%. Fecal microbiology and intestinal morphology were performed by radiographic, ultrasound, colonoscopy and intestinal biopsy exams for histology. There were no significant differences (P>0,05) for lactic acid bacteria counts and clostridium-reductor, gas area in the bowel (radiographic), wall thickness of the colon (ultrasound) and colonocytes count/globet cells (histology). Through colonoscopy, changes in characteristics of the intestinal mucosa in animals receiving treatment 4 were noticed. It is concluded that the addition of up to 0.6% EPL had no effect on the parameters evaluated, but further studies are needed to understand the action mechanisms and additive effects for domestic cats.

Effect of natural antioxidant combinations on lipid oxidation in cooked chicken meat during refrigerated storage.

The effect of combinations of sage, oregano and honey on lipid oxidation in cooked chicken meat during refrigeration at 4°C for 96h was determined. Chicken samples (thigh and breast) were then separated into five groups: control; butylated hydroxytoluene; oregano+sage; oregano+sage+5%honey and oregano+sage+10%honey. Quantitative measurements of thiobarbituric acid reactive substances, conjugated dienes, hexanal, fatty acids, cholesterol and cholesterol oxides were used as indicators of lipid oxidation. Acceptability and preference were also evaluated. The effectiveness of the natural antioxidants for reducing the velocity of lipid oxidation in cooked chicken thigh and breast was demonstrated after 48 and 96h of refrigeration at 4°C. The treatments that presented the lowest hexanal values after 96h of refrigeration were oregano+sage+5%honey and oregano+sage+10%honey. Only traces of free cholesterol oxides were found (25-OH, 7-k, 7α-OH and 7ß-OH). The natural antioxidants protected cooked chicken meat from oxidation processes and resulted in great acceptability.

Aspirin desensitization in a woman with inherited thrombophilia and recurrent miscarriage.

Women with inherited thrombophilia and recurrent miscarriage might benefit from preconceptional antiagreggation with low-dose acetylsalicylic acid (ASA), but concerns about severe adverse reactions may prevent physicians from performing this treatment in patients with ASA hypersensitivity. We report the first known case of ASA desensitization in a 41-year-old woman with inherited thrombophilia, who had homozygosity (4G/4G polymorphism) of the plasminogen activator inhibitor-1 (PAI-1) gene and first trimester recurrent miscarriage, and had previously presented with anaphylaxis to ASA. Desensitization was completed despite one self-limited adverse reaction, and the patient has maintained a daily ASA intake of 100 mg with good tolerance.

Técnicas radiográficas intra e extrabucal na avaliação dentária de cães com doença periodontal / Comparison between intra e extraoral radiologic procedures on dental evaluation of dogs with periodontal disease

Foram avaliadas as técnicas radiográficas dentárias intra (TIB) e extrabucal (TEB) em 50 cães com doença periodontal, no intuito de padronizar os procedimentos de diagnóstico dessa síndrome. A TIB revelou que 16 animais não apresentaram lesões ósseas visíveis, enquanto a TEB apontou que 39 pacientes foram negativos para as mesmas lesões. Em resumo, a TIB foi mais eficaz na detecção de sinais radiográficos, especialmente as chamadas lesões finas, que a TEB, sendo a técnica de escolha na síndrome periodontal.

The intra (IOT) and extraoral (EOT) dental radiographic techniques in 50 dogs with periodontal disease were compared to standardize the most appropriate procedure for accurate diagnosis of abnormalities that occur in this syndrome. For IOT, 16 animals showed no visible radiographic lesions, whereas in EOT, 39 patients were negative for the same radiographic lesions. For incisor and canine teeth, IOT presented the highest sensitivity of lesion detection, while there was no significant difference for the premolar and molar teeth group. It is concluded that IOT is more effective in detecting radiographic signs (especially the so-called thin lesions) than the EOT and should therefore be the technique of choice in the periodontal syndrome.

Antiproliferative, proapoptotic and morphogenic effects of the flavonoid rutin on human glioblastoma cells.

In this study, we investigated the effects of the flavonoid rutin (3,3',4',5,7-pentahydroxyflavone-3-rutinoside) on glioma cells, using the highly proliferative human cell line GL-15 as a model. We observed that rutin (50-100µM) reduced proliferation and viability of GL-15 cells, leading to decreased levels of ERK1/2 phosphorylation (P-ERK1/2) and accumulation of cells in the G2 phase of the cell cycle. On the other hand, 87.4% of GL-15 cells exposed to 100µM rutin entered apoptosis, as revealed by flow cytometry after AnnexinV/PI staining. Nuclear condensation and DNA fragmentation were also observed, further confirming that apoptosis had occurred. Moreover, the remaining cells that were treated with 50µM rutin presented a morphological pattern of astroglial differentiation in culture, characterised by a condensed cell body and thin processes with overexpression of GFAP. Because of its capacity to induce differentiation and apoptosis in cultured human glioblastoma cells, rutin could be considered as a potential candidate for malignant gliomas treatment.

Avaliação por eletrocardiografia contínua (holter) em cães da raça Pastor Alemão praticantes de atividade física regular / Ambulatory electrocardiography (Holter) evaluation in physically active German shepherd dogs

The pattern for ambulatory electrocardiography was evaluated in 25 healthy German Shepherd dogs. The influences of gender, age, and physical activity in HR maximum, mean, and minimum (HRmx, HRme, and HRmin, respectively) were studied. The physically active dogs (PA) showed lower HRme than sedentary animals (S) (P=0.03), whereas HRmx and HRmin were not altered (P=0.06 and P=0.65, respectively). The HRme was 80.89±13.85 for PA and 112.94±35.71 for S. No effect of gender and age on HR was observed. It is possible to state that the physical activity can modulate the sinus node of the dogs.

Reference range for T lymphocytes populations in blood donors from two different regions in Brazil

This study defined the normal variation range for different subsets of T-lymphocyte cells count in two different Brazilian regions. We analysed the T-lymphocytes subpopulations (CD3+, CD4+, CD8+) in blood donors of two Brazilian cities, located in North (Belem, capital state of Para, indian background) and Northeast (Salvador, capital state od Bahia, African background) regions of Brazil. Results were compared according to gender, stress level (sleep time lower than 8 hours/day), smoking, and alcohol intake. Lymphocytes subpopulations were measured by flow cytometry. Five hundred twenty-six blood donors from two Brazilians cities participated in the study: 450 samples from Bahia and 76 samples from Pará. Most (60 percent) were men, 59 percent reported alcohol intake, 12 percent were smokers, and 80 percent slept at least 8 h/day. Donors from Bahia presented with significantly higher counts for all parameters, compared with Para. Women had higher lymphocytes levels, in both states, but only CD4+ cells count was significantly higher than men's values. Smokers had higher CD4+ counts, but sleep time had effect on lymphocytes levels only for Para's donors (higher CD3+ and CD4+ counts). That state had also, a higher proportion of donors reporting sleep time <8 h/day. The values for CD3, CD4 and CD8+ cells count were significantly higher in blood donors from Bahia than among those from Pará. Female gender, alcohol intake, stress level, and smoking were associated with higher lymphocyte counts. The use of a single reference range for normal lymphocytes count is not appropriate for a country with such diversity, like Brazil is.

Reference range for T lymphocytes populations in blood donors from two different regions in Brazil.

This study defined the normal variation range for different subsets of T-lymphocyte cells count in two different Brazilian regions. We analysed the T-lymphocytes subpopulations (CD3+, CD4+, CD8+) in blood donors of two Brazilian cities, located in North (Belem, capital state of Para, indian background) and Northeast (Salvador, capital state od Bahia, African background) regions of Brazil. Results were compared according to gender, stress level (sleep time lower than 8 hours/day), smoking, and alcohol intake. Lymphocytes subpopulations were measured by flow cytometry. Five hundred twenty-six blood donors from two Brazilians cities participated in the study: 450 samples from Bahia and 76 samples from Pará. Most (60%) were men, 59% reported alcohol intake, 12% were smokers, and 80% slept at least 8 h/day. Donors from Bahia presented with significantly higher counts for all parameters, compared with Para. Women had higher lymphocytes levels, in both states, but only CD4+ cells count was significantly higher than men's values. Smokers had higher CD4+ counts, but sleep time had effect on lymphocytes levels only for Para's donors (higher CD3+ and CD4+ counts). That state had also, a higher proportion of donors reporting sleep time <8 h/day. The values for CD3, CD4 and CD8+ cells count were significantly higher in blood donors from Bahia than among those from Pará. Female gender, alcohol intake, stress level, and smoking were associated with higher lymphocyte counts. The use of a single reference range for normal lymphocytes count is not appropriate for a country with such diversity, like Brazil is.

Fixação esquelética externa em fratura tarsometatársica de seriema (Cariama cristata): relato de caso / External skeletal fixation in tarsumetatarsus fracture of red-legged seriema (Cariama cristata): a case report

Uma seriema (Cariama cristata) adulta foi atendida com histórico de traumatismo por tentativa de captura. A ave apresentava dificuldade de apoio do membro pélvico direito, dor à manipulação e fratura exposta do osso tarsometatarso. Optou-se pelo tratamento cirúrgico com redução fechada, utilizando-se fixador esquelético externo tipo II, com barra de conexão acrílica. A técnica cirúrgica utilizada foi satisfatória para o tratamento da fratura, possibilitando reparação óssea e retorno funcional do membro 60 dias após a cirurgia.

An adult red-legged seriema (Cariama cristata) was referred for examination with history of trauma by capture. The physical examination revealed lameness in the right pelvic limb, sensibility to touch and open fracture of tarsumetatarsus. The treatment was done with surgical closed reduction using a external skeletal fixator type II with acrylic connecting bar. The surgical technique applied was satisfactory for the treatment of the fracture of tarsumetatarsus, since there was bone healing and functional return of the limb at 60 days after surgery.

[Holoprosencephaly: morphological aspects of twelve cases of autopsy]. / Holoprosencefalia: análise do seu espectro morfológico em doze casos de autópsia.

The term holoprosencephaly (HPC) is used to indicate the group of hemispheric deformities caused by a failure in the development of the prosencephalic vesicle. The purpose of this study is to explain the morphologic spector of twelve cases of HPC, qualify them, and compare them to the literature. It was evaluated 5837 pediatrics necropsies, and there were 12 cases of HPC. Data like gender, age and the presence of another associated malformations were evaluated and compared to another malformations and to the total number of necropsies. The majority of the cases was male (66.66%), and stillborns (75%). The most frequently type of HPC found was the lobar type (58.3%), and the most frequently type of facial alteration was the ciclopy (25%). There is a statistic tendency to HPC affect the male sex, in comparison with other neurologic malformations. Besides, the stillborns are more frequently observed in HPC than in other neurologic malformations.

An inherited p53 mutation that contributes in a tissue-specific manner to pediatric adrenal cortical carcinoma.

The incidence of pediatric adrenal cortical carcinoma (ACC) in southern Brazil is 10-15 times higher than that of pediatric ACC worldwide. Because childhood ACC is associated with Li-Fraumeni syndrome, we examined the cancer history and p53 status of 36 Brazilian patients and their families. Remarkably, 35 of 36 patients had an identical germ-line point mutation of p53 encoding an R337H amino acid substitution. Differences within intragenic polymorphic markers demonstrated that at least some mutant alleles arose independently, thus eliminating a founder effect. In tumor cells, the wild-type allele was deleted, and mutant p53 protein accumulated within the nuclei. Although these features are consistent with Li-Fraumeni syndrome-associated adrenal tumors, there was no history of increased cancer incidence among family members. Therefore, this inherited R337H p53 mutation represents a low-penetrance p53 allele that contributes in a tissue-specific manner to the development of pediatric ACC.

[The neuropathology of neonatal period: analysis of 1616 autopsies]. / A neuropatologia no período neonatal: análise de 1616 casos de necropsia.

The lesions of the central nervous system represent an important cause of morbid-mortality in the neonatal period. This is due to the vulnerability of the brain to several adverse conditions during gestation and after birth. This study analyses the prevalence and pattern of central nervous system lesions in neonates autopsied at Hospital de Clínicas - Curitiba. There were 5743 pediatric autopsies performed in the Sector of Anatomic Pathology from 1960 to 1995 with 2049 cases corresponding to death during neonatal period. These later autopsies were reviewed and all cases with central nervous system lesions were selected and classified according to sex, age and pattern of central nervous system lesion. The central nervous system was affected in 1616 (78,87%) of neonatal autopsies and there was predominance of intracerebral hemorrhages (73,39%), congenital malformations (4,27%) and infections (3,59%). The hypoxic hemorrhages are the most prevalent central nervous system lesions in the neonatal period, affecting mainly premature babies. There was predominance of central nervous system malformations in the female neonates.