Case Report: A case of Dubin-Johnson syndrome in a newborn.

Background: Dubin-Johnson Syndrome (DJS) is a rare autosomal recessive genetic disorder, with most cases presenting in adolescence, but rare in newborns. Objective: To investigate the clinical characteristics and treatment outcomes of DJS in a newborn. Methods: We present the clinical features of a newborn diagnosed with DJS through molecular genetic testing. Results: The patient was a male newborn who developed jaundice and scleral icterus on the 6th day of life. Both direct and indirect bilirubin levels were elevated. After treatment with phototherapy, indirect bilirubin levels decreased, but direct bilirubin remained unchanged, and the stool color gradually lightened. At 56 days of age, the patient underwent laparoscopic cholecystostomy, which revealed viscous bile plugs in the bile ducts. Following the surgery, the patient received oral ursodeoxycholic acid, compound glycyrrhizin, and methylprednisolone. Follow-up until one year post-surgery showed a gradual reduction in direct bilirubin levels to the normal range. Molecular genetic testing revealed three heterozygous mutations in the ABCC2 gene on chromosome 10, with one pathogenic variant inherited from the father and two from the mother, confirming the diagnosis of DJS. Conclusion: DJS is a benign condition with a favorable prognosis. In newborns, it should be differentiated from other causes of cholestasis, and compared to cholestasis, jaundice in newborns with DJS responds more slowly to treatment.

Graft-to-recipient weight ratio and risk of systemic inflammatory response syndrome early after liver transplantation in children.

BACKGROUND: Systemic inflammatory responses soon after liver transplantation in children can lead to complications and poor outcomes, so here we examined potential risk factors of such responses. METHODS: Data were retrospectively analyzed for 69 children who underwent liver transplantation at a single center between July 2017 and November 2019 through follow-up lasting up to one years. Numerous clinicodemographic factors were compared between those who suffered early systemic inflammatory response syndrome (SIRS) or not. RESULTS: Of the 69 patients in our analysis, early SIRS occurred in 35 [50.7%, 95% confidence interval (CI), 38.6-62.8%]. Those patients showed significantly higher graft-to-recipient weight ratio (3.69 ± 1.26 vs. 3.12 ± 0.99%, P = 0.042) and lower survival rate at one year (85.7% vs. 100%, P = 0.023). Multivariate analysis found graft-to-recipient weight ratio > 4% to be an independent risk factor for early SIRS [odds ratio (OR) 3.8, 95% CI 1.08-13.371, P = 0.037], and a cut-off value of 4.04% predicted the syndrome in our patients, and area under the receiver operating characteristic curve of 0.656 (95% CI 0.525-0.788, P = 0.026). CONCLUSIONS: Graft-to-recipient weight ratio > 4% may predict higher risk of SIRS soon after liver transplantation in children.

Pediatric perforated appendicitis diagnosis based on the C-reactive protein/prealbumin ratio.

Pediatric perforated appendicitis, prone to multiple complications, necessitates identifying potential serum biomarkers for early diagnosis and intervention. A cross-sectional study was conducted on patients under 16 with acute appendicitis, admitted to Hainan Women and Children's Medical Center from January 2019 to July 2023. The patients were categorized into perforated and non-perforated groups. Among the 313 included patients, 106 (33.87%, 95% CI 28.59-39.14%) developed perforation. The C-reactive protein to prealbumin ratio (CPA) showed a significant difference between the perforated and non-perforated groups [6.63 (2.9-13.02) vs. 0.7 (0.11-2.18), p < 0.001]. The AUC of CPA on the ROC curve was 0.691 (95% CI 0.513-0.869, p = 0.084) in patients under 4. In patients aged 4-9, the sensitivity of CPA > 3 predicting perforation was 76.2%, with a specificity of 81.6%, and an AUC of 0.816 (95% CI 0.747-0.886, p < 0.001). For patients aged 9-16, the sensitivity of CPA > 2.2 predicting perforation was 85%, with a specificity of 85.7%, and an AUC of 0.919 (95% CI 0.859-0.979, p < 0.001). CPA > 3 and CPA > 2.2 can predict perforated appendicitis in patients aged 4-9 and 9-16, respectively.

Factors associated with in-hospital recurrence of intestinal intussusception in children.

BACKGROUND: A minority of children experience in-hospital recurrence of intestinal intussusception after treatment. This study investigated the factors associated with in-hospital recurrence of intussusception in pediatric patients in China. METHODS: This retrospective study included patients aged 0-18 years-old with intestinal intussusception treated at Hainan Women and Children's Medical Center between January 2019 and December 2019. Demographic and clinical characteristics were extracted from the medical records. Factors associated with in-hospital recurrence of intussusception were identified by logistic regression analysis. RESULTS: The analysis included 624 children (400 boys) with a median age of 1.8 years (range, 2 months and 6 days to 9 years). Seventy-three children (11.7%) had in-hospital recurrence of intussusception after successful reduction with air enema. Multivariate logistic regression analysis identified age > 1 year-old (odds ratio [OR]: 7.65; 95% confidence interval [95%CI]: 2.70-21.71; P < 0.001), secondary intestinal intussusception (OR: 14.40; 95%CI: 4.31-48.14; P < 0.001) and mesenteric lymph node enlargement (OR: 1.90; 95%CI: 1.13-3.18; P = 0.015) as factors independently associated with in-hospital recurrence of intussusception. CONCLUSIONS: Age > 1 year-old, secondary intussusception and mesenteric lymph node enlargement were independently associated with increased odds of in-hospital recurrence of intussusception after successful reduction with air enema.