Neonatal meningitis is a serious disease with significant mortality and morbidity. Its signs and symptoms are subtle, non-specific, atypical or absent. Cephalohaematoma is frequent in newborns and complications are uncommon, including local infection after haematogenous spread in the setting of bacteraemia or meningitis with a possibility of osteomyelitis, epidural abscess and subdural empyema. We report the case of a late preterm newborn, with an unremarkable pregnancy, born by vacuum-assisted vaginal delivery that presented in the fifth day of life with irritability, fever and grunting. Cerebrospinal fluid and blood cultures were positive for Escherichia coli. The patient had neurological deterioration despite adequate antibiotic therapy and brain MRI showed a right parietal epidural empyema, subcutaneous abscess, osteomyelitis and supratentorial hydrocephalus. The culture of the cephalohaematoma's abscess material was positive for E coli. Antibiotic therapy was continued for 8 weeks. The child, now 2 years old, has spastic tetraparesis with global development delay.

Hyperreninemic hypoaldosteronism due to aldosterone synthase (AS) deficiency is a rare condition typically presenting as salt-wasting syndrome in the neonatal period. A one-month-old Portuguese boy born to non-consanguineous parents was examined for feeding difficulties and poor weight gain. A laboratory workup revealed severe hyponatremia, hyperkaliaemia and high plasma renin with unappropriated normal plasma aldosterone levels, raising the suspicion of AS deficiency. Genetic analysis showed double homozygous of two different mutations in the CYP11B2 gene: p.Glu198Asp in exon 3 and p.Val386Ala in exon 7. The patient maintains regular follow-up visits in endocrinology clinics and has demonstrated a favourable clinical and laboratory response to mineralocorticoid therapy. To our knowledge, this is the first Portuguese case of AS deficiency reported with confirmed genetic analysis.

Premature Pubarche (PP), or the development of pubic hair before the age of 8 years in girls or 9 years in boys, is most commonly caused by Idiopathic Premature Adrenarche (IPA). There is an increased production of Dehydroepiandrosterone (DHEA) and DHEA Sulfate (DHEAS) by the adrenal zona reticularis without a concomitant rise in Cortisol. IPA is a diagnosis of exclusion and differential diagnosis must include milder and nonclassic variants of Congenital Adrenal Hyperplasia (CAH), that can account for 0 to 40% of cases of PP. Early morning basal 17-Hydroxyprogesterone (17OHP) above 200 ng/dL is 100% sensitive and 99% specific for CAH.

Introduction Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive disorders characterized by impaired cortisol synthesis with an incidence of 1:15 000 births. The most common form is attributable to mutations in CYP21A2, the gene encoding the adrenal steroid 21-hydroxilase enzyme (P450c21). Because of that, cortisol synthesis is impaired which leads to a corticotropin stimulation of the adrenal cortex, with accumulation of cortisol precursors that are derived to sex hormone biosynthesis. Aldosterone production may also be decreased (salt wasting forms). The final height of children with these disorders seems to be compromised. That can happen because of the disease itself or because an excessive dose of corticoids used during treatment. This work evaluates the final stature SDS of a group of patients with CAH and correlates it with 17-hydroxyprogesterone levels (17OHP) and corticoid dose at 10 years of age and at puberty onset. References Conclusion In this group of children the final height SDS was inferior to their target familial stature, because of the poor growth after puberty onset: their height SDS when they were 10 years old and on their puberty onset were better than the final one. There wasn't any correlation with 17OHP and corticoid dose used during treatment. When the growth after puberty onset was analysed, it was positively correlated with corticoid dose and negatively correlated with 17OHP levels on puberty onset. There are many studies reporting that high doses of corticoids used in these children may impair their growth. But, in this study, to achieve better levels of 17OHP, higher doses of corticoids were used. So, maybe that could have helped to achieve a better growth before puberty and could have prejudiced the growth after puberty onset.

O experimento foi realizado com o objetivo de avaliar o efeito aditivo de um prebiótico a base de mananoligossacarídeos e β-glucanos sobre desempenho e morfometria intestinal de frangos de corte. Foram utilizados 180 pintos de corte machos distribuídos em delineamento experimental inteiramente casualizado com três tratamentos, seis repetições e dez aves por unidade experimental. Os tratamentos consistiram em: T1 – dieta controle (DC) sem adição de prebiótico, T2 – DC + 0,1% de prebiótico e T3 – DC + 0,15% de prebiótico. Aos 42 dias de idade, a suplementação de 0,1% de prebiótico na dieta melhorou o peso final, o ganho de peso e a conversão alimentar das aves, bem como promoveu maior relação vilo:cripta, maior largura dos vilos, menor distância entre os vilos e maior área de absorção (P = 0,0016) no duodeno das aves, em comparação as aves que receberam a dieta controle. Conclui-se que a suplementação de 0,1% do prebiótico a base de mananoligossacarídeos e β-glucanos melhora o desempenho e a qualidade intestinal de frangos de corte.