BackgroundChildren represent 10-20% of all systemic lupus erythematosus (SLE) patients. Their clinical manifestations and outcomes vary with age. We aim to clarify the relationship between pubescent status and the clinical manifestations of pediatric SLE.MethodsIn this study, pediatric SLE patients were divided into three groups, based on age at disease onset (≦8, 8–13 & 13–18 years), defined as prepubescent, pubescent and postpubescent, respectively. Initial clinical manifestations and laboratory characteristics at diagnosis were analyzed.ResultsNinety-six patients were entered into the study: 8 had disease onset before age 8, while 49 were between 8–13 and 39 of them were 13–18. Female predominance was noted in all three groups (2.5-7.0:1). Postpubescents showed significantly more renal involvement and lymphopenia, along with lower levels of C3 and C4, when compared with prepubescents. They also showed significantly more lymphopenia when compared with pubescents. Pubescents showed significantly more renal involvement, leukopenia and lupus anticoagulant (LAC) positivity, along with lower C3 and C4 levels, when compared with prepubescents. Pubescents also showed significantly higher anti-Sm antibody positivity when compared with postpubescents. Prepubescents showed significantly more splenomegaly and anti-Jo-1 antibody positivity when compared with those of pubescents. The results showed that the disease activity (SLEDAI-2K score) correlated positively with age at disease onset and negatively with disease duration before diagnosis (p = 0.011).ConclusionsAge at disease onset is related to initial manifestations in pediatric SLE patients at our center. Certain parameters such as renal involvement, splenomegaly, low C3 level, low C4 level, lymphopenia, leukopenia, and anti-Sm & anti-Jo-1 antibody were found to be significantly different among the age groups. Renal involvement might be the key symptom that varies with age.

Colonic perforation is an extremely rare complication following ventriculoperitoneal (VP) shunting. Laparotomy to repair the perforation site is usually required for patients with peritonitis. Here we report colonic perforation due to VP shunt in a 4-year-old girl, presenting with a distal catheter protruding out of the anus as well as symptoms and signs of peritonitis. The distal catheter was removed and the perforation site was repaired successfully via the anus. Postoperative course was uneventful. Trans-anal repair of the colonic perforation after removal of the distal shunt may be considered an alternative choice for managing this kind of complication.

These results summarized clinical manifestations of primary antibody deficiency in pediatric group in Taiwan. Clinicians should strive to shorten delays in diagnosis and maintain higher trough IgG levels to decrease subsequent mortality and morbidity.

Lung agenesis, a rare congenital anomaly, is sometimes associated with multiple congenital anomalies. 1 Young infants with this condition may face a catastrophic clinical course. We report an unusual case of lung agenesis with complex cardiac anomalies and crossed ectopic lung complicating diagnosis in a 6-month-old child. CLINICAL SUMMARYA boy was born uneventfully to a healthy mother at a gestational age of 37 weeks. Low birth weight (1700 g) and multiple congenital anomalies were found at birth, including large anterior fontanel and syndactyly over the second and third toes. Because tachypnea and heart murmur were heard 3 days after birth, serial workup was arranged. Chest radiography showed left lung haziness (Figure 1), and echocardiography showed tricuspid atresia, small ventricular septal defect, atrial septal defect, large patent ductus arteriosus, and suspected left pulmonary artery and pulmonary vein agenesis (Figure 2, A and B). Because of the suspected left lung agenesis, electrocardiographically gated chest computed tomography and 3-dimensional reconstruction were performed (Figure 2, C and D, and Video 1). Crossed ectopic right lower lobe in the left hemithorax was found incidentally, in addition to left lung agenesis and tricuspid atresia. The arteries and veins supplying not only this lobe but also the bronchus of this lobe were from the right lung. The patient's respiratory distress and heart failure symptoms improved after anticongestive medication, and he was then discharged at 2 weeks old.

Hepatitis B virus (HBV) infection is one of the main concerns in blood and marrow transplantation (BMT) patients for possible breakthrough hepatitis. Active recipient immunization against HBV was found to be ineffective and many studies had showed that the adoptive transfer of immunity against hepatitis B virus would be possible by BMT with unknown duration and mechanism. A 46-year-old female patient with chronic hepatitis B had persistent detectable HBV DNA and positive serum hepatitis B e antigen (HBeAg), even while on long-term lamivudine and adefovir therapy. She received allogeneic matched unrelated donor peripheral blood stem cell transplantation (allo-MUD-PBSCT) for her refractory acute myeloid leukemia (AML). The HBV DNA became undetectable and she developed HBeAg seroconversion after PBSCT. Her hepatitis B surface antigen (HBsAg) remained positive, which disappeared later, along with the development of antibody to HBsAg after one shot of donor lymphocyte infusion (DLI) as a boost against her AML. In summary, BMT from an immunized donor would probably bring adoptive immunity against HBV. This adoptive immunity might be further enhanced by the subsequent DLI.