Baseline characteristics and evolution of Brazilian patients with atypical hemolytic uremic syndrome: first report of the Brazilian aHUS Registry.

Background: Atypical hemolytic uremic syndrome (aHUS) is an ultra-rare disease. Therefore, studies involving large samples are scarce, making registries powerful tools to evaluate cases. We present herein the first analysis of the Brazilian aHUS Registry (BRaHUS). Methods: Analysis of clinical, laboratory, genetic and treatment data from patients inserted in the BRaHUS, from 2017 to 2020, as an initiative of the Rare Diseases Committee of the Brazilian Society of Nephrology. Results: The cohort consisted of 75 patients (40 adults and 35 pediatric). There was a predominance of women (56%), median age at diagnosis of 20.7 years and a positive family history in 8% of cases. Renal involvement was observed in all cases and 37% had low C3 levels. In the <2 years of age group, males were predominant. Children presented lower levels of hemoglobin (P = .01) and platelets (P = .003), and higher levels of lactate dehydrogenase (LDH) (P = .004) than adults. Genetic analysis performed in 44% of patients revealed pathogenic variants in 66.6% of them, mainly in CFH and the CFHR1-3 deletion. Plasmapheresis was performed more often in adults (P = .005) and 97.3% of patients were treated with eculizumab and its earlier administration was associated with dialysis-free after 3 months (P = .08). Conclusions: The cohort of BRaHUS was predominantly composed of female young adults, with renal involvement in all cases. Pediatric patients had lower hemoglobin and platelet levels and higher LDH levels than adults, and the most common genetic variants were identified in CFH and the CFHR1-3 deletion with no preference of age, a peculiar pattern of Brazilian patients.

Inflammatory cytokines and lipid profile in children and adolescents with nephrotic syndrome receiving L. Plantarum: a randomized, controlled feasibility trial.

METHODS: This study aimed to evaluate the efficacy of the action of the Lactobacillus Plantarum probiotic as a immunomodulatory and hypolipidemic agent in dyslipidemic nephrotic children and adolescents. This is a randomized, double-blind, placebo-controlled clinical trial in pediatric, compensated or partially compensated nephrotic syndrome and dyslipidemic subjects undergoing regular outpatient follow-up. Serum lipid and TNF-α (proinflammatory) and IL-10 (anti-inflammatory) cytokine variations were evaluated. Cytokines were analyzed by enzyme-linked immunosorbent assay (ELISA). RESULTS: In the probiotic group there was a tendency to reduce TNF-α levels and increase IL-10 levels when compared to controls. Regarding the lipid profile, there was a decrease in serum triglyceride (6.0 mg / dL) and total cholesterol (41.5 mg / dL) levels in the probiotic group when compared to baseline levels, while in the control group there was an increase in serum triglyceride (49.5 mg / dL) and total cholesterol (8.0 mg / dL) levels, respectively. CONCLUSION: Preliminary results suggest that L. Plantarum showed an immunomodulatory and hypolipidemic effect in nephrotic and dyslipidemic pediatric subjects.

Inflammatory cytokines and lipid profile in children and adolescents with nephrotic syndrome receiving L. Plantarum: a randomized, controlled feasibility trial

SUMMARY This study aimed to evaluate the efficacy of the action of the Lactobacillus Plantarum probiotic as a immunomodulatory and hypolipidemic agent in dyslipidemic nephrotic children and adolescents. METHODS: This is a randomized, double-blind, placebo-controlled clinical trial in pediatric, compensated or partially compensated nephrotic syndrome and dyslipidemic subjects undergoing regular outpatient follow-up. Serum lipid and TNF-α (proinflammatory) and IL-10 (anti-inflammatory) cytokine variations were evaluated. Cytokines were analyzed by enzyme-linked immunosorbent assay (ELISA). RESULTS: In the probiotic group there was a tendency to reduce TNF-α levels and increase IL-10 levels when compared to controls. Regarding the lipid profile, there was a decrease in serum triglyceride (6.0 mg / dL) and total cholesterol (41.5 mg / dL) levels in the probiotic group when compared to baseline levels, while in the control group there was an increase in serum triglyceride (49.5 mg / dL) and total cholesterol (8.0 mg / dL) levels, respectively. CONCLUSION: Preliminary results suggest that L. Plantarum showed an immunomodulatory and hypolipidemic effect in nephrotic and dyslipidemic pediatric subjects.

RESUMO: O objetivo deste estudo foi avaliar a eficácia da ação do probiótico Lactobacillus Plantarum como um agente imunomodulador e hipolipemiante em crianças e adolescentes dislipidêmicos com síndrome nefrótica. MÉTODOS: Este é um estudo randomizado, duplo-cego, controlado com placebo e realizado em pacientes pediátricos com síndrome nefrótica, compensados ou parcialmente compensados e dislipidêmicos passando por acompanhamento ambulatorial. Variações no lipídio sérico e nas citocinas TNF-α (pró-inflamatória) e IL-10 (anti-inflamatória) foram avaliadas. As citocinas foram analisadas por ensaio imunoenzimático (ELISA). RESULTADOS: No grupo probiótico, houve uma tendência de redução dos níveis de TNF-α e de aumento dos níveis de IL-10, quando comparado ao controle. Em relação ao perfil lipídico, houve uma diminuição nos níveis séricos de triglicérides (6,0 mg/dL) e colesterol total (41,5 mg/dL) no grupo probiótico em comparação aos níveis basais, enquanto no grupo de controle houve um aumento nos níveis séricos de triglicérides (49,5 mg/dL) e colesterol total (8,0 mg/dL). CONCLUSÃO: Os resultados preliminares sugerem que o L. Plantarum tem um efeito imunomodulador e hipolipemiante em pacientes pediátricos dislipidêmicos e com síndrome nefrótica.

[Evaluation of risk and protection factors associated with high blood pressure in children]. / Avaliação dos fatores de risco e proteção associados à elevação da pressão arterial em crianças.

BACKGROUND: Epidemiological studies have shown an increased prevalence of high blood pressure in pediatric patients. Today we know that risk factors can be detected during childhood and may help in preventing the disease. OBJECTIVE: To evaluate risk and protection factors related to high blood pressure in childhood. METHODS: We evaluated children aged 3 to 10 years, residing in the east and southwest sanitary districts of the City of Goiânia, Goiás, and obtained the following data: birth weight, breastfeeding, family history of high blood pressure and obesity, weight, height, body mass index (BMI), and blood pressure. We applied the Mann-Whitney U-test to these variables in order to compare pressure variation. RESULTS: In this sample, 519 children were evaluated, and 246 (47.4%) of them were male. The BMI assessment identified 109 (21%) overweight children, of which 53 (10.3%) were obese. Predominant and/or exclusive breastfeeding for less than 6 months was found in 242 (51.2%). The mean systolic pressure was significantly higher in children with exclusive and/or predominant breastfeeding for less than 6 months (p = 0.04), and in children with family history of high blood pressure (p = 0.05), and in overweight children (p <0.0001). These data were confirmed in multivariate analysis. CONCLUSION: In this sample, we observed that overweight and hereditary factors may be associated with elevated blood pressure, and that breastfeeding for more than 6 months seems to offer a protective effect.

Avaliação dos fatores de risco e proteção associados à elevação da pressão arterial em crianças / Evaluation of risk and protection factors associated with high blood pressure in children

FUNDAMENTO: Estudos epidemiológicos têm mostrado um aumento da prevalência da hipertensão arterial na faixa etária pediátrica. Hoje se sabe que os fatores de risco poderiam ter sido detectados na infância, o que auxiliaria na prevenção da doença. OBJETIVO: Avaliar fatores de risco e de proteção relacionados à elevação da pressão arterial na infância. MÉTODOS: Foram avaliadas crianças de 3 a 10 anos moradoras dos distritos sanitários leste e sudoeste de Goiânia, Goiás. Obtiveram-se os seguintes dados: peso ao nascer, aleitamento materno, história familiar de hipertensão e obesidade, peso, estatura, índice de massa corporal (IMC) e pressão arterial. Utilizaram-se os testes de U de Mann-Whitney para comparar a variação da pressão arterial quanto às variáveis descritas. RESULTADOS: Na amostra estudada, 519 crianças foram avaliadas, 246 (47,4 por cento) do sexo masculino. Avaliação do IMC identificou 109 (21 por cento) com excesso de peso, das quais 53 (10,3 por cento) eram obesas. O aleitamento materno predominante e/ou exclusivo por tempo inferior a 6 meses foi encontrado em 242 (51,2 por cento). As médias da pressão sistólica se encontraram significativamente mais elevadas naquelas crianças com aleitamento materno exclusivo e/ou predominante por tempo inferior a 6 meses (p = 0,04), história familiar positiva para hipertensão (p = 0,05) e excesso de peso (p < 0,0001). Esses dados foram confirmados na análise multivariada. CONCLUSÃO: Na amostra estudada, excesso de peso e fatores hereditários podem estar associados à elevação da pressão arterial, e o tempo em aleitamento materno superior a 6 meses parece conferir um efeito protetor.

BACKGROUND: Epidemiological studies have shown an increased prevalence of high blood pressure in pediatric patients. Today we know that risk factors can be detected during childhood and may help in preventing the disease. OBJECTIVE: To evaluate risk and protection factors related to high blood pressure in childhood. METHODS: We evaluated children aged 3 to10 years, residing in the east and southwest sanitary districts of the City of Goiânia, Goiás, and obtained the following data: birth weight, breastfeeding, family history of high blood pressure and obesity, weight, height, body mass index (BMI), and blood pressure. We applied the Mann-Whitney U-test to these variables in order to compare pressure variation. RESULTS: In this sample, 519 children were evaluated, and 246 (47.4 percent) of them were male. The BMI assessment identified 109 (21 percent) overweight children, of which 53 (10.3 percent) were obese. Predominant and/or exclusive breastfeeding for less than 6 months was found in 242 (51.2 percent). The mean systolic pressure was significantly higher in children with exclusive and/or predominant breastfeeding for less than 6 months (p = 0.04), and in children with family history of high blood pressure (p = 0.05), and in overweight children (p <0.0001). These data were confirmed in multivariate analysis. CONCLUSION: In this sample, we observed that overweight and hereditary factors may be associated with elevated blood pressure, and that breastfeeding for more than 6 months seems to offer a protective effect.

Anatomia dos ramos do nervo lingual em suíno / Anatomy of branches of nerve lingual in pigs

Neste trabalho, foram estudadas as ramificações e a distribuição do nervo lingual em 18 línguas de suínos adultos de ambos os sexos, sem especificação de raça. O material, colhido a fresco em rotina de abate, foi resfriado (4°C) para transporte, fixado em solução aquosa de formaldeído (7 por cento, 72 horas), imerso em solução aquosa de ácido nítrico (15 por cento, 72 horas), e dissecado sob lupa (RANSOR - II-20). Cada antímero dissecado possibilitou o registro dos ramos nervosos linguais primários, secundários e terciários presentes no interior das estruturas miofasciais do órgão, exibindo diferentes arranjos: o antímero esquerdo apresentou quatro a 10 ramos primários, quatro a 12 ramos secundários e zero a 11 ramos terciários. Já o antímero direito apresentou três a 8 ramos primários, zero a 11 ramos secundários e zero a 10 ramos terciários. Em 27,6 por cento das análises, os ramos linguais estabelecem associações com as fibras do nervo hipoglosso no antímero correspondente.

The distribution and ramifications of the lingual nerve of 18 tongues of adult pigs from both genders with no strain specification were studied. The material freshly collected in slaughter routine was cooled (4°C) for transportation, fixed in formaldehyde aqueous solution (7 percent, 72h), immerse in nitric acid aqueous solution (15 percent, 72h) and dissected with magnifying glass (RASOR, II-20). Each dissected antimere enabled the recording of primary, secondary and tertiary lingual nervous branches present inside the myofascial structure of the organ, exhibiting different arrangements: left antimere: 4 to 10 primary branches, 4 to 12 secondary antimeres and 0 to 11 tertiary branches; right antimere: 3 to 8 primary branches, 0 to 11 secondary antimeres and 0 to 10 tertiary branches. In 27.6 percent of analyses, the lingual branches established associations with the fibers of the hypoglossal nerve in the corresponding antimere.

O uso do corticóide altera o crescimento estatural na síndrome nefrótica primária? / Can steroid use change linear growth of children with primary nephrotic syndrome?

Objetivo: Avaliar, a médio prazo, o efeito do uso de corticóides sobre o crescimento linear de crianças portadoras de síndrome nefrótica primária (SNP).Métodos: Análise retrospectiva de crianças entre 2 e 8 anos portadoras de SNP em tratamento no serviço de Nefrologia Pediátrica do HC-FM-UFGo, que estavam em uso de prednisona por um período mínimo de um ano. Estudaram-se as variações anuais da velocidade do crescimento durante cinco anos de acompanhamento, individualizando-se os períodos com e sem uso de corticóide. O teste t-Student foi utilizado para avaliar as correlações das variáveis com o uso do corticóide. Resultados: Foram avaliadas 29 crianças, com média de idade de 63,4 + 36,4 meses. A velocidade de crescimento anual no período do uso de corticóide variou de 2,5 -18,1 cm, com média de 7,0+ 3,2, e na ausência do uso de corticóide variou de 1,31 - 13,9 cm, com média de 6,8 + 2,8. A comparação dos grupos pelo teste T-student não demonstrou diferença estatisticamente significante (p=0,81). Conclusão: O uso da prednisona em doses terapêuticas não demonstrou alterações na velocidade de crescimento em médio prazo nesse grupo de crianças.