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: 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: 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: 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: Cholestatic liver disease is a frequent complication of prolonged parenteral nutrition, especially in premature infants. The purpose of present study is to evaluate the clinical and biochemical efficacy of ursodeoxycholic acid (UDCA) in premature infants with total parenteral nutrition (TPN) cholestasis. METHODS: Retrospective chart review of 31 prematures with TPN cholestasis in Neonatal Intensive Care Unit of Chonnam University Hospital from January 1995 and December 2002 was done. Prematures were divided into two groups based on UDCA treatment: the study group (n=22, with UDCA treatment) and the control group (n=9, without UDCA treatment). Treatment efficacy of UDCA was evaluated by monitoring the changes of serum direct bilirubin level for more than one to two months. RESULTS: In study group, cholestasis appeared at a mean age of 23+/-18.8 days after a mean of 21+/-19.7 days of TPN. UDCA was initiated (ranged 15-30 mg/kg/day) at a mean age of 54+/-22.3 days for a mean of 32+/-19.0 days. There was no significant difference in decrease of direct bilirubin level between the study and control group. However, the day of initial enteral feeding and full enteral feeding were earlier in control group compared with study group. CONCLUSIONS: This study shows that UDCA treatment is not effective in the treatment of TPN cholestasis among premature infants unlike that of results reported in children and adults. To reduce the incidence of TPN cholestasis in premature infants, early introduction of enteral feeding as soon as possible is most important.
Adult ; Bilirubin ; Child ; Cholestasis* ; Enteral Nutrition ; Humans ; Incidence ; Infant, Newborn ; Infant, Premature* ; Intensive Care, Neonatal ; Jeollanam-do ; Liver Diseases ; Parenteral Nutrition ; Parenteral Nutrition, Total* ; Retrospective Studies ; Treatment Outcome ; Ursodeoxycholic Acid*

PURPOSE: To investigate the effect of early enteral trophic feeding within 24 hours after birth compared with being started within 7 days after birth in extremely low birth weight infants (ELBWIs). METHODS: We retrospectively analyzed the medical records of all ELBWIs with gestational age of 26 weeks and less, and birth weight below 1,000 g, admitted within 24 hours after birth to neonatal intensive care unit of Samsung Medical Center from January 2000 to June 2006 who were alive at the time of discharge. Data for nutritional status and morbidities were compared between Era 1 (n=76), in which early enteral trophic feeding was started within 7 days after birth and Era 2 (n=46), started within 24 hours after birth. RESULTS: Compared to era 1, despite that gestational age and enteral feeding strarting time of ELBWIs was significantly earlier (25(+4)+/-0(+6) vs. 24(+6)+/-1(+5) weeks, 4.5+/-5.1 vs. 0.7+/-1.3 days), the time to achieve full enteral feeding was significantly shorter, and the weight gain at corrected age of 36 weeks was significantly higher in era 2. Also the incidence of early and total confirmed sepsis, total parenteral nutrition induced cholestasis, was lower and the duration of hospitalization was significantly shorter in era 2 compared to era 1, without differences in the incidence of NEC, moderate to severe BPD, and severe IVH (> or =Gr III) between two eras. CONCLUSION: Early enteral trophic feeding in ELBWI within 24 hours after birth was safe and beneficial.
Birth Weight* ; Cholestasis ; Enteral Nutrition ; Gestational Age ; Hospitalization ; Humans ; Incidence ; Infant* ; Infant, Low Birth Weight* ; Infant, Newborn ; Intensive Care, Neonatal ; Medical Records ; Nutritional Status ; Parenteral Nutrition, Total ; Parturition* ; Retrospective Studies ; Sepsis ; Weight Gain

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: With the recent improved survival of extremely low birth weight infants (ELBWI), enteral feeding has become a major issue. This study investigates the effects of early enteral feeding in ELBWI on their morbidity, duration of hospitalization, and mortality. METHODS: ELBWI admitted to the neonatal intensive care unit at Samsung Medical Center from November 1994 to April 2004 who survived more than 14 days were enrolled. ELBWI were divided into two groups: an early feeding group (EF), in which enteral feeding was started within 3 days after birth; and a late feeding group (LF), in which enteral feeding was started beyond 3 days after birth. 80 ELBWI came under EF, and 131 ELBWI under LF. RESULTS: Birth weight and gestational age did not differ between the two groups. In EF, the time to achieve full enteral feeding and the duration of parenteral nutrition were significantly shorter than in LF. The incidence of bronchopulmonary dysplasia was significantly lower in EF, but the incidences of sepsis, necrotizing enterocolitis, and cholestasis were not different between the two groups. There was no difference in the survival rate between the two groups, but the duration of hospitalization was significantly shorter in EF. CONCLUSION: Early enteral feeding in ELBWI did not increase the incidence of necrotizing enterocolitis and sepsis, but rather decreased the incidence of bronchopulmonary dysplasia and shortened the duration of hospitalization.
Birth Weight ; Bronchopulmonary Dysplasia ; Cholestasis ; Enteral Nutrition* ; Enterocolitis, Necrotizing ; Gestational Age ; Hospitalization ; Humans ; Incidence ; Infant* ; Infant, Extremely Low Birth Weight ; Infant, Low Birth Weight ; Infant, Newborn ; Intensive Care, Neonatal ; Mortality ; Parenteral Nutrition ; Parturition ; Sepsis ; Survival Rate