Is neurodevelopment impaired in Brazilian children with intestinal failure on prolonged parenteral nutrition? A single center study.

PURPOSE: To assess the neurodevelopment outcomes of children younger than 42 months of age with intestinal failure (IF) using prolonged parenteral nutrition (PN) followed by a Pediatric Multidisciplinary Intestinal Rehabilitation Program from a public tertiary hospital in Brazil. METHODS: Bayley III scale was administered in children aged 2 to 42 months with IF and receiving PN for more than 60 days. Composite scores in cognitive, motor, and language domains were analyzed. Developmental delay was defined as a performance 2 standard deviations (SD) below the average at the 3 domains. Association between Bayley III composite scores and clinical variables related to IF were tested. RESULTS: Twenty-four children with median (IQR) age of 17.5 months (9-28.5) were studied, 58.3% were male. Developmental delay was found in 34%, 33% and 27% of the patients in cognitive, motor, and language domains, respectively. There was no significant association between the Bayley-III composite scores and length of hospitalization, prematurity, and number of surgical procedures with anesthesia. CONCLUSION: The study demonstrated impairments in the cognitive, motor and language domains in approximately one-third of young patients with IF on prolonged PN.

Outcomes of the First 54 Pediatric Patients on Long-Term Home Parenteral Nutrition from a Single Brazilian Center.

OBJECTIVES: Data on multidisciplinary programs dedicated to home parenteral nutrition (HPN) in Latin America are limited. This study describes the results of the first multidisciplinary pediatric intestinal rehabilitation program for HPN at a public tertiary hospital in Brazil. METHODS: We retrospectively reviewed patients aged 0-18 years with intestinal failure (IF) who required parenteral nutrition (PN) for >60 days between January/2014 and December/2020. RESULTS: Fifty-four patients were discharged on HPN (15 achieved enteral autonomy, 34 continued on HPN at the end of the study, 1 underwent intestinal transplantation, and 4 died). The median (IQR) age at the study endpoint of patients who achieved enteral autonomy was 14.1 (9.7-19) versus 34.7 (20.4-53.9) months in those who did not achieve enteral autonomy. Overall prevalence of catheter-related thrombosis was 66.7% and catheter-related bloodstream infection rate was 0.39/1000 catheter-days. Intestinal failure-associated liver disease (IFALD) was present in 24% of all patients; none of the patients who achieved enteral autonomy had IFALD. All patients showed significant improvement in anthropometric parameters during the HPN period. The sociodemographic characteristics of the patients' family members were mothers less than 20 years old (7.5%), schooling time more than 10 years (55.5%), and household income between 1 and 3 times the minimum wage (64.8%). The 5-year survival rate for HPN is 90%, and 27.7% of patients achieve enteral autonomy. CONCLUSION: The treatment of pediatric patients with IF followed by a multidisciplinary pediatric intestinal rehabilitation program with HPN is feasible and safe in the Brazilian public health system.

Prevalence and characteristics of hepatopulmonary syndrome in children with cirrhosis in southern Brazil.

BACKGROUND: Hepatopulmonary syndrome (HPS) is defined as a triad characterized by arterial deoxygenation, intrapulmonary vascular dilatations (IPVDs), and liver disorder. The aims of this study were to assess the prevalence of HPS in children with cirrhosis, the clinical characteristics of patients with HPS, and the tests used for the diagnosis of IPVD. PATIENTS AND METHODS: This was a prospective, cross-sectional study of 40 children with cirrhosis (median age: 44 months). Investigations of HPS included arterial blood gas analysis, contrast-enhanced transthoracic echocardiography (CE-TTE), and perfusion lung scanning using technetium-99m-labeled macroaggregated albumin (Tc-MMA). Patients' clinical characteristics (age, etiology of cirrhosis, and severity of hepatopathy) were assessed. HPS was defined as liver disease; alveolar-arterial oxygen gradient of at least 15 mmHg and/or partial pressure of arterial oxygen less than 80 mmHg; and detection of IPVD by CE-TTE or Tc-MMA scanning. Statistical significance was indicated by a P value less than 0.05. RESULTS: The prevalence of HPS was 42.5% (17/40). Eight patients had moderate HPS (47%) and two patients had severe HPS (12%). In bivariate analysis, biliary atresia (P=0.033) and median age (10 months; P=0.005) were associated with HPS. In multivariate analysis, only age remained statistically significant (prevalence ratio=0.99; 95% confidence interval=0.98-0.99; P=0.010). Sixteen patients with HPS had IPVD detected by CE-TTE (94.1%) and six patients had IPVD detected by Tc-MMA scanning (35.3%), with no significant agreement between these methods (κ=-0.12; P=0.163). CONCLUSION: HPS is a common complication of cirrhosis in children. A combination of clinical and imaging criteria should be used to diagnose HPS.