Retrospective investigation of cases diagnosed with neonatal cholestasis and admitted to İnönü University Medical Faculty Neonatal Care Unit between 2009 and 2014

Introduction: Neonatal cholestasis is a condition that begins in the neonatal period due to the intrahepatic and the extrahepatic causes. It is characterized by jaundice, conjugated hyperbilirubinemia and elevated ALP and GGT. The early differential diagnosis and early treatment are important in the prevention of morbidity and mortality in both the intrahepatic and the extrahepatic causes. The objective of our study was to evaluate the ethiological factors, symptom and physical examination findings, laboratory results and follow up results for cases with neonatal cholastase and to compare these data with those of other studies. Patients and Method: 94 newborn patients; 40 girls and 54 boys with neonatal cholestasis that followed up in İnönü University Department of Pediatrics Neonatal Unit between January 2009 and December 2014 were included in this study. We analyzed the clinical and the laboratory test findings and retrospectively evaluated the diagnoses, the physical examinations, the clinical courses and the prognoses in two groups; younger than 32 weeks (Group 1) and older than 32 weeks (Group 2). Results: The etiologies of the 94 patients in this study were TPN associated cholestasis (n=21), sepsis (n=14), galactosemia (n=8), Rh haemolytic disease (n=7), biliary atresia(n=5), congenital heart disease (n=4), perinatal hypoxia (n=3), hemochromatosis (n=2), cytomegalovirus infection (n=2), PFIC-1 (n=2), PFIC-3 (n=1), Down Syndrome (n=2), nonimmune hydrops fetalis ( n=2), congenital adrenal hyperplasia (n=1), hypothyroidis (n=1), tyrosinemia (n=1), portal vein thrombosis (n=1), ARC syndrome (n=1), fatty acid oxidation disorder (n=1), mitochondrial disease (n=1) and unknown (n=1). The two most common causes of the etiology were more frequent in the infants younger than 32 weeks. The most common causes of sepsis were Klebsiella pneumoniae and Escherichia coli in the bacteriological evaluation. The mean age for all patients was 34 weeks. In admission the age of the patients ranged from 1 day to 33 days .The admission age was younger and it was observed that the age of application in Group 1 was lower at a statistically significant level (p=0,000). The mean birth weights were respectively 1117±380 gr and 2259±664 gr in Group 1 and Group 2. This difference was statistically significant (p=0.000). The durations for the diagnosis from the admission were respectively 19.9±15 days and 9.7±7.6 days in Group 1 and Group 2 and the diagnosis of the patients were earlier in Group 2 (p=0.001). In the means of inhabitancy, we observed that the Group 2 patients were more frequently from the upstate (p=0.001). The first degree consanguinity rate was high and when compared in groups, it was significantly higher in Group 1 (p=0.004). When the prenatal features of the mothers examined, the rates of eclampsia and preeclampsia were higher in the mothers of the infants who were younger than 32 weeks (p= 0.007). The most common complaints in admission were icterus (%55.3) and malnutrition (%33). The most common physical examination finding was icterus (%80). The most common etiological disorder was SGA (%50). The other common factors such as NEC, RDS, PDA, ROP, BDP and pneumoniae were more frequent in the infants younger than 32 weeks. In comparison of the laboratory test findings between the groups, white blood cell, Hb, Hct, INR, total bilirubin, total protein and albumin levels were significantly different. Among 94 patients, 25 patients (%26.6) got well and 33 patients (%35) died. Conclusion: We consider that the early diagnosis is important. During the search of the etiological cause, the features such as the gestational age and the birth weight of the patient should be taken into consideration. We also consider that when the extrahepatic causes are relevant, the surgical treatment should not be delayed.