We report two infants with neonatal cholestasis and hepatosplenomegaly secondary to congenital syphilis. The onset of jaundice of the first infant was at six weeks of life and the second case on the 28th hour of life with associated neurologic and bone involvement. The diagnosis was suspected based on a maternal history of untreated syphilis, clinical findings, and a reactive rapid plasma reagin. Early recognition and treatment can lead to clinical improvement but prevention by mandatory testing and treatment of maternal syphilis is a more effective strategy
Congenital syphilis ; neonatal cholestasis

BACKGROUND: The direct/total (d/t) bilirubin ratio can be used to distinguish the causes of jaundice in many patients who have increased levels of direct and indirect bilirubin. However, the reference range of the d/t ratio has not been established, hindering its clinical usefulness. This study assessed the clinical usefulness of the d/t ratio. METHODS: Paired total bilirubin and direct bilirubin tests (N=4,357) of cholestasis, hemolytic anemia, and neonatal jaundice were evaluated. Regression analyses were performed between total bilirubin and direct bilirubin, and between total bilirubin and the d/t ratio for each disease. Theoretical correlation models were established and used to compare the regression analyses data. RESULTS: The theoretical model and regression equation between total bilirubin and direct bilirubin displayed linear correlations for all three cholestatic diseases. The model and regression equation between total bilirubin and the d/t ratio showed reciprocal curve correlations for the cholestatic diseases. When the total bilirubin concentration exceeded approximately 10 mg/dL, the rate of change of the d/t ratio decreased and converged to a constant value between 0.7 and 0.9. CONCLUSIONS: If the total bilirubin concentration exceeds 10 mg/dL, cholestatic diseases can be diagnosed if the d/t ratio is more than 0.7. However, if the total bilirubin concentration is lower than 10 mg/dL, cholestatic diseases should be considered even if the d/t ratio is lower than 0.7. Therefore, use of the d/t ratio with total bilirubin could prove to be valuable in clinical settings.
Anemia, Hemolytic ; Bilirubin* ; Cholestasis ; Humans ; Hyperbilirubinemia ; Infant, Newborn ; Jaundice ; Jaundice, Neonatal ; Models, Theoretical ; Reference Values

PURPOSE: Parenteral nutrition (PN)-associated cholestasis (PNAC) is one of the most common complications in very low birth weight infants (VLBWIs). The aim of this study is to evaluate the risk factors of PNAC in VBLWIs. METHODS: We retrospectively reviewed the medical records of 322 VLBWIs admitted to the neonatal intensive care unit of our hospital from July 1, 2009 to December 31, 2013. We excluded 72 dead infants; 6 infants were transferred to another hospital, and 57 infants were transferred to our hospital at 2 weeks after birth. The infants were divided into the cholestasis and the non-cholestasis groups. PNAC was defined as a direct bilirubin level of ≥2.0 mg/dL in infants administered with PN for ≥2 weeks. RESULTS: A total of 187 VLBWI were enrolled in this study; of these, 46 infants developed PNAC. Multivariate logistic regression analysis showed that the risk factors of PNAC in VLBWI were longer duration of antimicrobial use (odds ratio [OR] 4.49, 95% confidence interval [95% CI] 4.42-4.58), longer duration of PN (OR 2.68, 95% CI 2.41-3.00), long-term lack of enteral nutrition (OR 2.89, 95% CI 2.43-3.37), occurrence of necrotizing enterocolitis (OR 2.40, 95% CI 2.16-2.83), and gastrointestinal operation (OR 2.19, 95% CI 2.03-2.58). CONCLUSION: The results of this study suggest that shorter PN, aggressive enteral nutrition, and appropriate antimicrobial use are important strategies in preventing PNAC.
Bilirubin ; Cholestasis* ; Enteral Nutrition ; Enterocolitis, Necrotizing ; Humans ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Intensive Care, Neonatal ; Logistic Models ; Medical Records ; Parenteral Nutrition ; Parturition ; Retrospective Studies ; Risk Factors

PURPOSE: To evaluate the causes of nil per os (NPO) before reaching full enteral feeding and compare the clinical outcomes of extremely low birth weight infant (ELBWI) by NPO duration. METHODS: We retrospectively reviewed the medical records of 92 ELBWI who were born and admitted to Neonatal intensive care unit (NICU) of Seoul National University Children's Hospital from January 2009 to December 2011. We analyzed the perinatal factors and causes of NPO. To compare neurodevelopmental outcomes and growth, we used K-ASQ (Korean ages & stages questionnaires) and growth Z-score. RESULTS: There were total 163 fasting episodes before reaching full enteral feeding. Mean NPO time was 6.7+/-5.6 days and mean frequency of NPO was 1.8 episodes. Most common cause of NPO was the medication for patent ductus arteriosus (PDA) closure (47.5%) and the next was the feeding intolerance (25.3%). Longer NPO group (more than 7 days) showed longer time to full enteral feeding and hospital day. Incidence of necrotizing enterocolitis was significantly higher in the longer NPO group. But there was no difference between two groups in the incidence of sepsis, cholestasis, and osteopenia. Changes in height Z-score from birth to postmenstrual age 35 weeks were significantly higher in the longer NPO group. In longer NPO group, catch-up of weight Z-score at CA 8 months was poor. And number of patients with score under cutoff level in K-ASQ was higher. CONCLUSION: NPO duration seems to be related with long term growth and neurodevelopment. Effort to minimize fasting time is needed by keeping enteral feeding during PDA medication and active management for feeding intolerance.
Bone Diseases, Metabolic ; Cholestasis ; Ductus Arteriosus, Patent ; Enteral Nutrition* ; Enterocolitis, Necrotizing ; Fasting ; Humans ; Incidence ; Infant* ; Infant, Extremely Low Birth Weight ; Infant, Low Birth Weight* ; Infant, Newborn ; Intensive Care, Neonatal ; Medical Records ; Parturition ; Retrospective Studies ; Seoul ; Sepsis

PURPOSE: This study investigated the effects of early enteral feeding on the morbidities of extremely low birth weight infants (ELBWI) weighing less than 1,000 g. METHODS: We conducted a retrospective review of the medical records of sixty one ELBWI who were admitted to the neonatal intensive care unit of Inje University Busan Paik Hospital from January 2007 to October 2009. ELBWI were divided into two groups; the control group included ELBWI from January 2007 to March 2008, for whom enteral feeding was started beyond 3 days and the early feeding group included ELBWI from April 2008 to October 2009, for whom enteral feeding was started within 3 days. RESULTS: Gestational age and birth weight did not differ between the two groups. In the early feeding group, start day of enteral feeding (control group vs. early feeding group; 7+/-2days vs. 2+/-1days), time to achieve full enteral feeding (68+/-6 days vs. 22+/-2 days), and the duration of parenteral nutrition (58+/-6 days vs. 22+/-2 days) were significantly shorter, and weight gain at postnatal day 28 was significantly higher than that of the control group (P<0.001). No differences were observed in the incidence of sepsis and necrotizing enterocolitis and duration of hospitalization; however, the incidence of total parenteral nutrition induced cholestasis (44% vs. 7%) and bronchopulmonary dysplsia (78% vs. 24%) was significantly lower in the early feeding group. CONCLUSION: Early enteral feeding in ELBWI shortened the time to achieve full enteral feeding, improved weight gain, and decreased the incidence of brochopulmonay dysplasia and cholestasis.
Birth Weight ; Cholestasis ; Enteral Nutrition ; Enterocolitis, Necrotizing ; Gestational Age ; Humans ; Incidence ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Intensive Care, Neonatal ; Medical Records ; Parenteral Nutrition ; Parenteral Nutrition, Total ; Retrospective Studies ; Sepsis ; Weight Gain

INTRODUCTIONThis study determined any clinical features which may help to differentiate biliary atresia (BA) from other causes of neonatal cholestasis (NC).

MATERIALS AND METHODSA prospective and observational study was conducted on consecutive infants with NC referred to the University of Malaya Medical Centre, Malaysia, between November 1996 and May 2004.

RESULTSThe 3 most common causes of cholestasis among the 146 infants with NC studied were idiopathic neonatal hepatitis (n = 63, 43%), BA (n = 35, 24%) and congenital cytomegalovirus hepatitis (n = 13, 9%). Common clinical features at presentation were jaundice (100%), hepatomegaly (95%), splenomegaly (52%) and pale stools (47%). Three clinical features noted to be sensitive for BA were the presence of acholic or variably acholic stools on admission, a liver which was firm/hard in consistency and a palpable liver of ≥4 cm (sensitivity of 77%, 80% and 94%, respectively), but the corresponding specificity was poor (51%, 65% and 39%, respectively). The stools of 2 children with BA were pigmented initially but became acholic subsequently.

CONCLUSIONSWe did not find any single clinical feature with sufficient sensitivity and specificity to differentiate BA from other causes of NC. Repeated inspection of stools colour is necessary as occasionally, patients with BA may have initial pigmented stools. Biochemical assessment and imaging studies are important in the assessment of any infant with NC.

Adult ; Biliary Atresia ; diagnosis ; Cholestasis ; diagnosis ; etiology ; Cytomegalovirus ; Cytomegalovirus Infections ; diagnosis ; etiology ; Diagnosis, Differential ; Female ; Hepatitis ; diagnosis ; etiology ; Hepatomegaly ; diagnosis ; etiology ; Humans ; Infant, Newborn ; Jaundice, Neonatal ; diagnosis ; Logistic Models ; Malaysia ; Male ; Prospective Studies

PURPOSE: Intestinal atresia is a common cause of neonatal intestinal obstruction. Recently, the survival rate has been increasing from development of prenatal diagnosis, total parenteral nutrition (TPN) and neonatal intensive care. We evaluated the complication rate and cause of mortality after operative management for jejunoileal atresia. METHODS: We reviewed 62 patients (36 males, 26 females) with jejuno-ileal atresia who underwent operation from 1998 to 2007. RESULTS: There were 37 patients with jejunal atresia and 25 with ileal atresia. The average gestational age was 256+/-16.6 days and birth weight was 2,824+/-620 g. Prenatal diagnosis was performed in 45 patients (72.6%) around gestational age 27 weeks. Within 2nd day after birth, 44 patients (71%) underwent operation. Half of the jejunoileal atresia was type IIIa and type I was in 8, type II was in 3, type IIIb was in 12, and type IV was in 8. The operative treatment was resection & anastomosis in 59 patients and enterotomy & web excision in 3. They started feeding at 12.4+/-11.5 days after operation on average. The average duration of TPN was 26.7+/-23.5 days, and the incidence of cholestasis was 30.6%. Hospital days averaged 36.8+/-26 days. Early complication occurred in 14 patients (intestinal obstruction in 5, sepsis in 4, wound problem in 3, anastomosis leakage in 1, and intraabdominal abscess in 1). Late complication occurred in 7 patients (anastomosis stricture in 4 and intestinal obstruction in 3). There was only one case of mortality due to short bowel syndrome after re-operation for adhesive ileus. CONCLUSION: The operation for intestinal atresia was successful and aggressive management contributed to a low mortality rate.
Abscess ; Adhesives ; Birth Weight ; Cholestasis ; Constriction, Pathologic ; Gestational Age ; Humans ; Ileus ; Incidence ; Infant, Newborn ; Intensive Care, Neonatal ; Intestinal Atresia ; Intestinal Obstruction ; Male ; Parenteral Nutrition, Total ; Parturition ; Prenatal Diagnosis ; Sepsis ; Short Bowel Syndrome ; Survival Rate

PURPOSE: To assess the validity of individual and combined prognostic effects of severe bronchopulmonary dysplasia (BPD), brain injury, retinopathy of prematurity (ROP), and parenteral nutrition associated cholestasis (PNAC). METHODS: We retrospectively analyzed the medical records of 80 extremely low birthweight (ELBW) infants admitted to the neonatal intensive care unit (NICU) of the Severance Children's Hospital, and who survived to a postmenstrual age of 36 weeks. We analyzed the relationship between 4 neonatal morbidities (severe BPD, severe brain injury, severe ROP, and severe PNAC) and poor outcome. Poor outcome indicated death after a postmenstrual age of 36 weeks or survival with neurosensory impairment (cerebral palsy, delayed development, hearing loss, or blindness) between 18 and 24 months of corrected age. RESULTS: Each neonatal morbidity correlated with poor outcome on univariate analysis. Multiple logistic regression analysis revealed that the odds ratios (OR) were 4.9 (95% confidence interval [CI], 1.0-22.6; P=0.044) for severe BPD, 13.2 (3.0-57.3; P<.001) for severe brain injury, 5.3 (1.6-18.1; P=0.007) for severe ROP, and 3.4 (0.5-22.7; P=0.215) for severe PNAC. Severe BPD, brain injury, and ROP were significantly correlated with poor outcome, but not severe PNAC. By increasing the morbidity count, the rate of poor outcome was significantly increased (OR 5.2; 95% CI, 2.2-11.9; P<.001). In infants free of the above-mentioned morbidities, the rate of poor outcome was 9%, while the corresponding rates in infants with 1, 2, and more than 3 neonatal morbidities were 46%, 69%, and 100%, respectively. CONCLUSION: In ELBW infants 3 common neonatal mornidifies, severe BPD, brain injury and ROP, strongly predicts the risk of poor outcome.
Brain Injuries ; Bronchopulmonary Dysplasia ; Cholestasis ; Diterpenes ; Hearing Loss ; Humans ; Infant ; Infant, Extremely Low Birth Weight ; Infant, Newborn ; Intensive Care, Neonatal ; Logistic Models ; Medical Records ; Odds Ratio ; Paralysis ; Parenteral Nutrition ; Retinopathy of Prematurity ; Retrospective Studies

PURPOSE: To study the safety of more aggressive parenteral amino acid supplementation on liver functions during parenteral nutrition in preterm infants. METHODS: Medical records of preterm infants (n=58) treated in the neonatal intensive care unit from 2004 through 2008 with birth weight less than 2,000 g and exposure to parenteral nutrition (PN) for more than 7 days were reviewed. Patients were divided into two groups; low amino acid intake group (LAA, n=32) admitted from January 2004 to May 2006 and high amino acid intake group (HAA, n=26) admitted from June 2006 to July 2008. The incidence of cholestasis was compared between the two groups and the liver functions in infants with cholestasis were evaluated. RESULTS: Mean protein intake was significantly higher in HAA (1.0+/-0.2 g/kg/day vs 2.1+/-0.6 g/kg/day, P=0.001). But there were no differences in the incidence and duration of cholestasis between the groups. And peak total bilirubin (T.bil), peak direct bilirubin (D.bil), peak alanine aminotransferase (ALT), peak asparate aminotransferase (AST), and peak alkaline phosphatase (ALP) in infants with cholestasis were not significantly different. CONCLUSION: Aggressive parenteral amino acid supplementation in preterm infants were not associated significantly with the liver functions in this study.
Alanine Transaminase ; Alkaline Phosphatase ; Bilirubin ; Birth Weight ; Cholestasis ; Humans ; Incidence ; Infant ; Infant, Newborn ; Infant, Premature ; Intensive Care, Neonatal ; Liver ; Medical Records ; Parenteral Nutrition

PURPOSE: To analyze and compare various cases in which peritoneal drainage was used as the primary treatment method in preterm infants with intestinal perforation. METHODS: Among the preterm infants of less than 28 weeks of gestation who were admitted to the neonatal intensive care unit (NICU) at the Gangnam Severance Hospital from April 2006 to April 2009, 7 who had developed intestinal perforation were studied retrospectively. We investigated the clinical characteristics, secondary operation performances, morbidities, complications, and mortalities. RESULTS: Among the 7 infants, 5 survived. Of the 5 cases, 3 received laparotomy, of which 2 were confirmed as having necrotizing enterocolitis. Of the 2 infants who died, 1 had received laparotomy before 48 h of peritoneal drainage, while the other had not received any subsequent treatment. Of the 7 children, 4 had patent ductus arteriosus (PDA), of which 3 had received indomethacin injection. Five infants had begun enteral feeding before they developed intestinal perforation. Of the 5 infants who survived, 4 were diagnosed with cholestasis. Of the 7 infants, 4 developed periventricular leukomalacia (PVL) and 3 developed rickets. CONCLUSION: Although the use of peritoneal drainage as the primary management of intestinal perforation in preterm infants is controversial, we suggest that it can be used for treating extreme premature neonates. Further randomized controlled study will be required to determine the feasibility of using this method.
Child ; Cholestasis ; Drainage ; Ductus Arteriosus, Patent ; Enteral Nutrition ; Enterocolitis, Necrotizing ; Humans ; Indomethacin ; Infant ; Infant, Newborn ; Infant, Premature ; Intensive Care, Neonatal ; Intestinal Perforation ; Laparotomy ; Leukomalacia, Periventricular ; Pregnancy ; Retrospective Studies ; Rickets