Changes in Microbiota Profile in the Proximal Remnant Intestine in Infants Undergoing Surgery Requiring Enterostomy.

Early-life gut dysbiosis has been associated with an increased risk of inflammatory, metabolic, and immune diseases later in life. Data on gut microbiota changes in infants undergoing intestinal surgery requiring enterostomy are scarce. This prospective cohort study examined the enterostomy effluent of 29 infants who underwent intestinal surgery due to congenital malformations of the gastrointestinal tract, necrotizing enterocolitis, or spontaneous intestinal perforation.

Congenital Glucagon-like Peptide-1 Deficiency in the Pathogenesis of Protracted Diarrhea in Mitchell-Riley Syndrome.

Context: Mitchell-Riley syndrome due to RFX6 gene mutations is characterized by neonatal diabetes and protracted diarrhea. The RFX6 gene encodes a transcription factor involved in enteroendocrine cell differentiation required for beta-cell maturation. In contrast to the pathway by which RFX6 mutations leads to diabetes, the mechanisms underlying protracted diarrhea are unknown.

Evolution of Resting Energy Expenditure, Respiratory Quotient, and Adiposity in Infants Recovering from Corrective Surgery of Major Congenital Gastrointestinal Tract Anomalies: A Cohort Study.

This cohort study describes the evolution of resting energy expenditure (REE), respiratory quotient (RQ), and adiposity in infants recovering from corrective surgery of major congenital gastrointestinal tract anomalies. Energy and macronutrient intakes were assessed. The REE and RQ were assessed by indirect calorimetry, and fat mass index (FMI) was assessed by air displacement plethysmography.

Colonisation of the proximal intestinal remnant in newborn infants with enterostomy: a longitudinal study protocol.

Introduction: The gut microbiota plays a main role in the maintenance of host's health. Exposure to different conditions in early life contributes to distinct 'pioneer' bacterial communities in the intestine, which shape the newborn infant development. Newborn infants with congenital malformations of the gastrointestinal tract (CMGIT), necrotising enterocolitis (NEC) and spontaneous intestinal perforation (SIP) commonly require abdominal surgery and enterostomy.

Parenteral nutrition-associated cholestasis and triglyceridemia in surgical term and near-term neonates: A pilot randomized controlled trial of two mixed intravenous lipid emulsions.

Background: Cholestasis is a common complication in infants receiving prolonged parenteral nutrition (PN). We studied the effects of two intravenous lipid emulsions composed with either 30% soybean oil, 30% medium-chain triglycerides (MCT), 25% olive oil, and 15% fish oil (SMOF) or with 50% MCT and 50% soybean oil n-6 (MCT/SOY) on the incidence of cholestasis in surgical term and near-term neonates.

Methods: A single-center, double-blinded, randomized controlled trial compared the incidence of cholestasis using either SMOF or MCT/SOY in neonates born at gestational age ≥34 weeks undergoing major surgery.

Fat mass index performs best in monitoring management of obesity in prepubertal children.

Objective: An early and accurate recognition of success in treating obesity may increase the compliance of obese children and their families to intervention programs. This observational, prospective study aimed to evaluate the ability and the time to detect a significant reduction of adiposity estimated by body mass index (BMI), percentage of fat mass (%FM), and fat mass index (FMI) during weight management in prepubertal obese children.

Methods: In a cohort of 60 prepubertal obese children aged 3-9 years included in an outpatient weight management program, BMI, %FM, and FMI were monitored monthly; the last two measurements were assessed using air displacement plethysmography.

The adjusted effect of maternal body mass index, energy and macronutrient intakes during pregnancy, and gestational weight gain on body composition of full-term neonates.

Objective: To evaluate the effect of prepregnancy body mass index (BMI), energy and macronutrient intakes during pregnancy, and gestational weight gain (GWG) on the body composition of full-term appropriate-for-gestational age neonates.

Study Design: This is a cross-sectional study of a systematically recruited convenience sample of mother-infant pairs. Food intake during pregnancy was assessed by food frequency questionnaire and its nutritional value by the Food Processor Plus (ESHA Research Inc, Salem, OR).

Mosaic trisomy 18 in a five-month-old infant.

Individuals with mosaic trisomy 18, only approximately 5% of all trisomy 18 cases, carry both a trisomy 18 and an euploid cell line. Their clinical findings are highly variable, from the absence of dysmorphic features to the complete trisomy 18 syndrome. A five-month-old daughter of a 38-year-old mother, with vomiting and feeding problems, was referred to our department.

[Iatrogeny in paediatrics: reconsider attitudes].

Iatrogeny is a pathologic condition caused in a patient in the different stages of a medical act. In the United States, it is the fourth cause of death but in Portugal, data are not yet available. Though different from medical error, it is still a taboo subject for health care providers.

Severe megaloblastic anaemia in an infant.

Vitamin B(12) or cobalamin deficiency, a rare clinical entity in pediatric age, is found most exclusively in breastfed infants, whose mothers are strictly vegetarian non-supplemented or with pernicious anaemia. In this article, the authors describe a 10-month-old infant admitted for vomiting, refusal to eat and prostration. The infant was exclusively breastfed and difficulties in introduction of new foods were reported.

[Anticonvulsant hypersensitivity syndrome in children].

Anticonvulsant hypersensitivity syndrome, or DRESS (Drug Rash with Eosinophilia and Systemic Symptoms), is a rare multisystem disorder, potentially fatal, that occurs after exposure to antiepileptic drugs, mainly aromatic ones. Clinically, this condition is recognized by the classic triad of fever, rash and internal organ involvement that usually develops 1 to 12 weeks after initiation of therapy. We report a case of a child treated with sodium valproate for epilepsy that showed a febrile rash 4 weeks after being medicated with phenobarbital.

Kawasaki disease.

Introduction: Kawasaki disease (KD) is the leading cause of acquired heart disease among children in developed nations. Its incidence has risen in recent years and 20% of untreated patients develop coronary artery abnormalities.

Objectives: To analyze the epidemiological, clinical, laboratory and echocardiographic data of cases diagnosed in Hospital Fernando Fonseca and to identify factors that may influence prognosis.

[Echovirus type 13 meningitis: admissions to a paediatric ward at a Lisbon hospital].

Infections by echovirus 13 are rare. In our country, it had not been previously reported any infection of this sort until 2000, when eleven echovirus 13 meningitis were observed in the Department of Pediatrics of the Hospital Fernando Fonseca. In England and Spain outbreaks of echovirus 13 meningitis during 2000 was also reported.