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: The purpose of this study was to evaluate the effect of amino acid mixtures on incidence and severity of total parenteral nutrition associated-cholestasis(PNAC) in very low birth weight infants. METHODS: Retrospective review of 63 very low birth weight infants(birth weight < or =1,500 g) who received total parenteral nutrition(TPN) in our neonatal intensive care unit from January 2000 to December 2004 was performed. Patients were divided into 2 groups : Group I(n=32, Jan 2000-Jun 2002) and Group II(n=31, Jul 2002-Dec 2004), where infants in Group II received taurine and glutamic acid-rich amino acid mixtures. PNAC was defined as serum direct bilirubin(DB) level greater than 2.0 mg/dL. The incidence and severity of PNAC were compared between these groups. RESULTS: The incidence of PNAC was significantly lower in Group II than in Group I(21.9% vs 6.5%, P<0.148). Maximum and mean DB levels were also significantly lower in Group II(P<0.05). CONCLUSION: The incidence and severity of PNAC in very low birth weight infants may be reduced with different composition of amino acid mixtures in TPN. Further prospective randomized controlled studies are needed to determine an ideal composition of acid mixtures to prevent the development of PNAC.
Cholestasis* ; Humans ; Incidence ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Intensive Care, Neonatal ; Parenteral Nutrition, Total ; Retrospective Studies ; Taurine

PURPOSE: The aim of this study is to determine the feasibility and effectiveness of performing an ultrasound-guided percutaneous cholecysto-cholangiogram (PCC) for excluding biliary atresia as the cause of neonatal jaundice. MATERIALS AND METHODS: Between Oct. 2003 and Feb. 2005, six ultrasound-guided PCC procedures were performed to five jaundiced infants (4 females and 1 male; mean age: 60 days old) for whom possibility of biliary atresia could not be ruled out by the DISIDA scan as the cause of their neonatal jaundice. Gallbladder puncture was performed under ultrasound guidance with a 23-gauge needle. Contrast material injection during fluoroscopic examination was performed after dilatation of the gallbladder lumen with normal saline under ultrasound guidance. The criteria used for excluding biliary atresia were complete visualization of the extrahepatic biliary trees and/or contrast excretion into the duodenum. The complications and final diagnosis was assessed according to the clinical and laboratory findings. RESULTS: The procedures were successful in all the patients without any complication. Biliary atresia could be ruled out in all the patients. The final diagnosis was neonatal cytomegalovirus hepatitis in two patients, total parenteral nutrition-associated cholestasis in two patients, and combined cytomegalovirus hepatitis and total parenteral nutrition-associated cholestasis in one patient. CONCLUSION: Ultrasound-guided PCC is a feasible and effective method for the early definitive exclusion of biliary atresia as the cause of neonatal jaundice. By the technique of injecting normal saline before contrast injection, PCC can be done even in a totally collapsed or very small gallbladder.
Biliary Atresia* ; Cholangiography ; Cholecystography ; Cholestasis ; Cytomegalovirus ; Diagnosis ; Dilatation ; Duodenum ; Female ; Gallbladder ; Gastrointestinal Tract ; Hepatitis ; Humans ; Infant* ; Infant, Newborn ; Jaundice, Neonatal ; Male ; Needles ; Punctures ; Ultrasonography

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 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: This study was aimed to investigate the risk factors of cholestasis in very low birth weight infants (VLBWI). METHODS: The study includes 466 VLBWI admitted to the neonatal intensive care unit at Samsung medical center from November 1994 to January 2003 and the data were collected retrospectively from their medical records. They were divided into two groups; cholestatic group (group 1) and control group (group 2) by level of direct bilirubin at 2.0 mg/dL. RESULTS: The mean gestational age and birth weight were lower in group 1. In group 1, enteral feeding was started later, time to achieve full and near full enteral feeding were delayed and duration of parenteral nutrition was longer than group 2. The incidence of bronchopulmonary dysplasia was significantly higher in group 1, but the incidences of sepsis and necrotizing enterocolitis were not different between the two groups. After adjusting for birth weight and gestational age, logistic regression analysis of the above factors revealed that the factors significantly related to cholestasis were longer duration of parenteral nutrition and longer time to achieve near full enteral feeding. CONCLUSION: Earlier near full enteral feeding and shorter duration of parenteral nutrition decreased the incidence of cholestasis.
Bilirubin ; Birth Weight ; Bronchopulmonary Dysplasia ; Cholestasis* ; Enteral Nutrition ; Enterocolitis, Necrotizing ; Gestational Age ; Humans ; Incidence ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Intensive Care, Neonatal ; Logistic Models ; Medical Records ; Parenteral Nutrition ; Parenteral Nutrition, Total ; Retrospective Studies ; Risk Factors* ; Sepsis

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 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

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