Análise de características clínicas e laboratoriais dos pacientes com colestase neonatal atendidos em um serviço universitário do Rio de Janeiro

Abstract

Neonatal cholestasis is a typical and early form of manifestation of liver disease in children. Several causes can be attributed to neonatal cholestasis. Biliary atresia (BA) is the main disease that causes neonatal cholestasis. Furthermore, the group of intrahepatic diseases is the majority. The objective of this study was to describe and analyze the clinical and laboratory characteristics of patients with neonatal cholestasis. Retrospective and observational study carried out at the service of Gastroenterology of the Instituto de Puericultura e Pediatria Martagão Gesteira. It included patients diagnosed with neonatal cholestasis (direct bilirubin level greater than 1mg/dl) admitted between January 2011 and December 2018. Patients older than 180 days of age or with surgical procedures outside the institute or with inadequate data recording were excluded. Of the 102 patients, 62.7% were male. The most frequent diagnoses were: multifactorial neonatal cholestasis (28.4%) and BA (26.4%). Followed by the groups: congenital infection (16.7%); undetermined cause (16.7%); genetic-endocrine-metabolic-inflammatory causes (7.9%); other extrahepatic causes, except BA (3.9 %). The medians for the age at onset of jaundice and the first visit were 5 days and 57 days, respectively. The medians of birth weight and gestational age were 2887.5 g and 267 days, respectively. 96.1% (n=98) had jaundice, 46.1% (n=47) acholia and 41.2% (n=42) choluria. The diagnosis of BA was associated with: female sex (p<0,01), acholia (p<0,0001) and choluria (p<0,0001). The diagnosis of multifactorial neonatal cholestasis was totally linked to the sepsis variable (100%). Prematurity <34 weeks (p<0,01), small for age gestational (p<0,01), fasting (p<0,001), parenteral nutrition (p<0,001), asphyxia (p<0,01) and thoracoabdominal surgery (p<0,01) were positively associated with the multifactorial diagnosis. The variables female gender (p<0,05), acholia (p<0.001) and choluria (p<0,05) were negatively associated with the diagnosis of multifactorial cholestasis. Only the gamma-glutamyl transferase (GGT) level showed differences in the comparison between the diagnostic groups: BA group and other groups and group of other extrahepatic causes, except BA and other groups (p<0,05). In the comparison of BA and other extrahepatic causes, there was no statistical difference. Differences in GGT level were found between BA and non-BA groups, with strong statistical significance (p<0,1). Although the clinical presentation is similar in cases of neonatal cholestasis, this study found differences between groups of diseases. The multifactorial group was the most frequent and had the most clinical differences to the other groups, with associated perinatal factors and lower anthropometric measurements. The BA group was the group with the most striking cholestasis.