Hemolytic-uremic syndrome: 24 years' experience of a pediatric nephrology unit / Síndrome hemolítico-urêmica: 24 anos de experiência de uma unidade de nefrologia pediátrica

Abstract Introduction: A better understanding of hemolytic-uremic syndrome (HUS) pathophysiology significantly changed its treatment and prognosis. The aim of this study is to characterize the clinical features, severity, management, and outcomes of HUS patients. Materials and Methods: Retrospective study of HUS patients admitted to a Pediatric Nephrology Unit between 1996 and 2020. Demographic and clinical data regarding etiology, severity, treatment strategies, and patient outcome were collected. Results: Twenty-nine patients with HUS were admitted to our unit, but four were excluded. Median age at diagnosis was two years (2 months - 17 years). Clinical manifestations included diarrhea, vomiting, oliguria, hypertension, and fever. During the acute phase, 14 patients (56%) required renal replacement therapy. Infectious etiology was identified in seven patients (five Escherichia coli and two Streptococcus pneumoniae). Since 2015, 2/7 patients were diagnosed with complement pathway dysregulation HUS and there were no cases of infectious etiology detected. Six of these patients received eculizumab. The global median follow-up was 6.5 years [3 months-19.8 years]. One patient died, seven had chronic kidney disease, four of whom underwent kidney transplantation, one relapsed, and seven had no sequelae. Conclusion: These results reflect the lack of infectious outbreaks in Portugal and the improvement on etiological identification since genetic testing was introduced. The majority of patients developed sequels and mortality was similar to that of other countries. HUS patients should be managed in centers with intensive care and pediatric nephrology with capacity for diagnosis, etiological investigation, and adequate treatment. Long-term follow-up is essential.

Resumo Introdução: Um melhor entendimento da fisiopatologia da síndrome hemolítico-urêmica (SHU) mudou significativamente seu tratamento e prognóstico. Este estudo teve como objetivo caracterizar condições clínicas, gravidade, manejo e desfechos de pacientes com SHU. Materiais e Métodos: Estudo retrospectivo de pacientes com SHU admitidos numa Unidade de Nefrologia Pediátrica entre 1996-2020. Foram coletados dados demográficos e clínicos sobre etiologia, gravidade, estratégias de tratamento, desfechos de pacientes. Resultados: 29 pacientes com SHU foram admitidos em nossa unidade, mas quatro foram excluídos. A idade mediana ao diagnóstico foi dois anos (2 meses-17 anos). Manifestações clínicas incluíram diarreia, vômitos, oligúria, hipertensão e febre. Durante a fase aguda, 14 pacientes (56%) necessitaram de terapia renal substitutiva. Identificou-se a etiologia infecciosa em sete pacientes (cinco Escherichia coli; dois Streptococcus pneumoniae). Desde 2015, 2/7 pacientes foram diagnosticados com SHU por desregulação da via do complemento e não foram detectados casos de etiologia infecciosa. Seis desses pacientes receberam eculizumab. A mediana global de acompanhamento foi 6,5 anos [3 meses-19,8 anos]. Um paciente faleceu, sete apresentaram doença renal crônica, sendo quatro submetidos a transplante renal, uma recidiva e sete sem sequelas. Conclusão: Estes resultados refletem a ausência de surtos infecciosos em Portugal e a melhoria na identificação etiológica desde que os testes genéticos foram introduzidos. A maioria dos pacientes desenvolveu sequelas e a mortalidade foi semelhante à de outros países. Pacientes com SHU devem ser manejados em centros com cuidados intensivos e nefrologia pediátrica com capacidade para diagnóstico, investigação etiológica e tratamento adequado. O acompanhamento alongo prazo é essencial.

[Breastfeeding: prevalence and determinant factors]. / Aleitamento materno: prevalência e factores condicionantes.

Breastfeeding is the best way of feeding the baby for the first six months of life. However, in Portugal the abandonment rate of breastfeeding is very high during the baby first's months of life. The aim of this study was to assess prevalence of breastfeeding and to identify related factors during the six months after delivery, as socio demographic variables and life styles. We conducted a cohort study at the Maternity of the Hospital Santa Maria. A standard questionnaire was applied to 475 women after delivery, at three and six months postpartum. We studied socio demographics aspects, life styles and the way of feeding during the six months after delivery. Multivariate analysis was performed. The women studied (mean age of 29.8 +/- 5,4 years), 52.2% were primiparous, 86.1% were Caucasian, 40% had a high school degree and 33% had a University degree. Four hundred and sixty (96.8%) received prenatal care. The mean gestational age was 38.8 +/- 2 weeks and the birth weight was 3198.3 +/- 545.3 g. At the discharge 91% were breastfeeding (77% exclusively), 54.7% at third month and 34.1% at sixth month. The main causes pointed for abandoning breastfeeding were insufficient milk production, bad sucking and return to work. The milk formula introduction was in 68.6% cases by medical recommendation. The decision in maintenance breastfeeding at third and sixth months was correlated with a previous positive breastfeed experience, high educational level, healthy lifestyles, as non-smoking, regular physical activity, and information about advantage of breastfeed for mother health. Information about breastfeeding was received by media, friends, family and only 9% by health professionals. Fifty (13%) women had no information about breastfeeding. Although breastfeeding rate at discharge was high, there was an important rate of abandonment at third and sixth month. Healthy lifestyles, high educational level, a previous positive breastfeed experience had a positive influence in breastfeeding. Understanding attitudes towards pregnancy and breastfeeding can lead to new strategies for its promotion and maintenance.

[Pregnancy and smoking: an opportunity to change behaviours]. / Gravidez e tabagismo: uma oportunidade para mudar comportamentos.

INTRODUCTION: Maternal smoking during pregnancy is related not only to perinatal adverse events but also to important postnatal problems. Smoking is very prevalent in women with several socio-demographic factors playing an important role. AIMS: To assess the frequency of smoking as well as mothers' change in behaviours during pregnancy. To identify socio-demographic factors associated to smoking and to assess women's knowledge and sources of information about adverse effects of smoking during pregnancy. METHODS: We started a cohort study between March and October 2003 at the Maternity, Hospital Santa Maria. After an informed consent the mothers in the post natal ward were asked to fill in a questionnaire. RESULTS: Four hundred and seventy five mothers responded to the questionnaire and among them 30% were smokers before pregnancy. Thirty five percent stopped smoking during pregnancy and those who continued significantly reduced the number of cigarettes per day. Smoking was less prevalent in married women, with higher educational level and with stable employment (statistically significant correlations). Forty five percent of the mothers were misinformed or had no information about the adverse effects of smoking during pregnancy. Their main source of information were the media. DISCUSSION: Smoking prevalence was high (30%) among the respondents but the pregnancy lowered the numbers of cigarettes per day. The percentage of misinformed mothers concerned about the adverse effects of smoking during pregnancy was high (45%), which also showed that doctors' role as a source of information was surpassed by the media. The study also brought to light the need for the health professionals to improve their competences in their role on prevention of smoking during pregnancy and the postnatal period.

Orbital cysticercosis: diagnosis and treatment controversies.

Orbital cysticercosis is a rare condition and its management is controversial. We report 2 cases of orbital cellulitis associated with cysticercosis in which the treatment with antihelminthics was withheld. The 2 children had good evolution with spontaneous progressive resolution. The current literature is reviewed.