[Home and Ambulatory Artificial Nutrition (NADYA) Group report: home parenteral nutrition in Spain, 2019]. / Nutrición parenteral domiciliaria en España, 2019: informe del Grupo de Nutrición Artificial Domiciliaria y Ambulatoria NADYA.

INTRODUCTION: Aim: to communicate the home parenteral nutrition (HPN) data obtained from the HPN registry of the NADYA-SENPE group (www.nadya-senpe.com) for the year 2019. Material and methods: a descriptive analysis of the data collected from adult and pediatric patients with HPN in the NADYA-SENPE group registry from January 1 to December 31, 2019. Results: a total of 283 patients (51.9 % women), 31 children, and 252 adults from 47 Spanish hospitals were registered, which represents a prevalence rate of 6.01 patients per million inhabitants for year 2019. The most frequent diagnosis in adults was "palliative oncological" and "others" (21.0 %). In children, it was Hirschsprung's disease together with necrotizing enterocolitis, alterations in intestinal motility, and chronic intestinal pseudo-obstruction, with 4 cases each (12.9 %). The first reason for the indication was short-bowel syndrome in both children (51.6 %) and adults (37.3 %). The most used type of catheter was tunnelled both in children (75.9 %) and in adults (40.8 %). Sixty-eight episodes ended, all in adults, and the most frequent cause was death (54.4 %); 38.2 % were switched to oral. Conclusions: the number of collaborating centers and professionals in the NADYA registry is increasing. The main indications and reasons for HPN termination remain stable.

INTRODUCCIÓN: Objetivo: comunicar los datos de nutrición parenteral domiciliaria (NPD) obtenidos del registro del grupo NADYA-SENPE (www.nadya-senpe.com) del año 2019. Material y métodos: análisis descriptivo de los datos recogidos de pacientes adultos y pediátricos con NPD en el registro NADYA-SENPE desde el 1 de enero al 31 de diciembre de 2019. Resultados: se registraron 283 pacientes (51,9 %, mujeres), 31 niños y 252 adultos procedentes de 47 hospitales españoles, lo que representa una tasa de prevalencia de 6,01 pacientes/millón de habitantes/año 2019. El diagnóstico más frecuente en los adultos fue "oncológico paliativo" y "otros" (21,0 %). En los niños fue la enfermedad de Hirschsprung junto a la enterocolitis necrotizante, las alteraciones de la motilidad intestinal y la pseudoobstrucción intestinal crónica, con 4 casos cada uno (12,9 %). El primer motivo de indicación fue el síndrome del intestino corto tanto en los niños (51,6 %) como en los adultos (37,3 %). El tipo de catéter más utilizado fue el tunelizado tanto en los niños (75,9 %) como en los adultos (40,8 %). Finalizaron 68 episodios, todos en adultos: la causa más frecuente fue el fallecimiento (54,4 %). Pasaron a la vía oral el 38,2 %. Conclusiones: el número de centros y profesionales colaboradores con el registro NADYA va incrementándose. Se mantienen estables las principales indicaciones y los motivos de finalización de la NPD.

Early oral feeding versus traditional feeding after transanal endorectal pull-through procedure in Hirschsprung's disease.

Our study questioned whether the outcome of postoperative early oral feeding is different from traditional postoperative feeding in children with Hirschsprung's disease who underwent transanal endorectal pull-through.This was an observational and comparative study. Patients were allocated into 2 groups. Age, gender, fever, surgery-related infectious, abdominal distension, bowel obstruction, need for reoperation, peritonitis, anastomosis leak, and abscess formation were assessed. IV fluids and antibiotics usage were recorded. A Chi-square test, independent sample unpaired Student t test and Mann-Whitney test were used. P-value < .05 was considered statistically significant.Infections occurred in no patient in group 1 and 1 patient in group 2. Stenosis occurred in 3 patients in group 1 and 2 patients in group 2. Abdominal distension occurred in 4 patients in group 1 and 3 patients in group 2. Fever occurred in 2 patients in group 1 and 1 patient in group 2 within the first 24 hours and it occurred in 13 and 17 patients, respectively, within 48 hours. All patients of group 1 (n = 15) were treated with antibiotics and intravenous fluid administration; 1 patient for 24 hours, 12 patients for 48 hours, and 1 for 72 hours, respectively. All patients of group 2 (n = 18) were treated with antibiotics and intravenous fluid administration for 5 days. We noted a significant difference regarding the duration of antibiotic treatment and intravenous fluid administration after 72 hours.This study showed that there was no difference between the outcomes of early and traditional postoperative feeding. Due to a significant difference in the antibiotics and IV fluid administration intervals between these 2 groups which cause a prolonged hospital stay and higher costs, it seems that early postoperative feeding is superior to traditional strategy.

A rare case of severe lactic acidosis in a preterm infant: lack of thiamine during total parenteral nutrition.

Total parenteral nutrition (TPN) is a revolution in neonatal intensive care unit (NICU) care, but this therapy is not without problems. A 35-week-old, 1300 g female infant was transferred to our NICU because of bilious vomiting and feeding problems. When enteral feeding was started again, a severe condition similar to the previous one developed. On the 24th day, the patient underwent surgery with a diagnosis of Hirschprung's disease. One week before surgery, the parenteral solutions were composed without vitamins because intravenous vitamin supplements suitable for infants were not available. Thereafter, the patient suffered from severe hypoglycaemia, and sepsis started to develop, accompanied by a large anion gap and metabolic acidosis which is severe lactic acidosis refractory to massive doses of bicarbonate. The acidosis improved significantly when the patient was treated with thiamin. Although TPN is life saving in the NICU, meticulous attention must be paid while treating a patient with TPN, and all possible nutrients should be provided. In this report, a case of a preterm newborn requiring a prolonged period of TPN and complicated by serious lactic acidosis is presented and discussed.

New perspective for the management of near-total or total intestinal aganglionosis in infants.

BACKGROUND/PURPOSE: Extensive intestinal aganglionosis is rare. The diagnosis and treatment are known to be difficult and it had been considered to be fatal. The aim of this study was to review our experience with children with extensive intestinal aganglionosis. METHODS: Retrospective analysis was conducted in patients referred to the intestinal transplantation unit since 1993. Presentation and outcome were analysed looking at 2 groups who had either undergone previous subtotal intestinal resection (group I) or no or limited resection (group II). RESULTS: Eight children were selected (3 patients in group I and 5 in group II). Group I was remarkable in that patients all were referred early in age with progressing liver failure. Parents of one patient refused to accept transplantation as treatment, and he died one month later. Two noncirrhotic patients were maintained in the parenteral nutrition programme and currently progress well with enteral feedings. The other 5 patients underwent transplant, and 4 of 5 are alive after transplantation with a mean follow-up of 22.2 months (range 0.4 to 63.6). CONCLUSIONS: Subtotal resection of intestine at the time of diagnosis must be avoided. Conservative management with parenteral nutrition may be associated with long-term good outcome. Small bowel transplant may open new perspective in the management of patients with end-stage liver disease.

Management of Hirschsprung's disease in the adolescent.

Occasionally Hirschsprung's disease is misdiagnosed until the patient reaches adolescence. The disease of these young people may present technical problems not encountered in the neonate. I describe three such patients, ages 10, 13, and 19 years, who were managed successfully by slightly different surgical methods.