RESUMO
BACKGROUND: Aplasia cutis congenita (ACC) in newborns is a condition in which congenital defects or hypoplasia is present in part of the epidermis, dermis and even subcutaneous tissue (including muscle and bones). First reported by Cordon in 1767, ACC is a rare disease with a low incidence of 1/100000 to 3/10000. Currently, there are 500 cases reported worldwide. ACC can be accompanied by other malformations. The onset mechanism of the disease remains unknown but is thought to be correlated to factors such as genetics, narrow uterus, foetal skin and amniotic membrane adhesion, use of teratogenic drugs in early pregnancy and viral infection. CASE SUMMARY: In August 2018, we treated a newborn with ACC on the left lower limbs using a combination of ionic silver dressing and moist exposed burn ointment (MEBO) and achieved a satisfactory treatment outcome. The skin defects were observed on the external genitals and on areas from the left foot to 3/4 of the upper left side. Subcutaneous tissue and blood vessels were observed in the regions with skin defects. The following treatments were provided. First, the wound was rinsed with 0.9% sodium chloride solution followed by disinfection with povidone-iodine twice. And then MEBO was applied to the wound at a thickness of approximately 1 mm. After applying ionic silver dressing, the wound was covered with sterile gauze. The wound dressing was replaced every 2-3 d. At the 4-mo follow-up, the treatment outcome was satisfactory. There was minimal scar tissue formation, and limb function was not impaired. CONCLUSION: The combination of ionic silver dressing and MEBO to ACC is helpful.
RESUMO
OBJECTIVE: This study was conducted to compare serum cytosolic ß-glucosidase (CBG) levels of age-matched control patients with those of infants with necrotizing enterocolitis (NEC) thereby to determine the eventual association between serum CBG levels with extensive disease in infants with NEC. METHOD: A total of 96 premature infants were divided into the early NEC group (n = 25), confirmed NEC group (n = 23) and the control group (n = 48). Serum CBG concentration, C-reactive protein (CRP) and peripheral blood white blood cells (WBC) were measured at the onset of the disease in patients in early NEC or confirmed NEC groups and at weeks 2-3 in control infants. Data were analyzed using descriptive statistics, non-parametric tests, Student's t-test, linear correlation, Spearman correlation analysis, receiver operating characteristic (ROC) curve were used for statistical analysis. RESULTS: The median birth weights (mean ± SE) in the three groups were not statistically significant (P > 0.05). Serum CBG concentration in the 3 groups were (112.369 ± 108.539) nmol/L, (693.013 ± 211.614) nmol/L and (36.478 ± 28.31) nmol/L, respectively. The infants in the confirmed NEC group had highest CBG levels, compared with the other 2 groups (P < 0.05). When the levels of CBG ≥ 65 ng/ml, CRP ≥ 2 mg/L and WBC < 5 × 10(9)/L within 3 days after birth or > 20 × 10(9)/L 3 days after birth were considered as positive parameters, the sensitivity of CBG and CRP was higher than that of WBC (P < 0.05). Among these indices, CBG had the highest specificity (87.4%), positive predictive (95.6%) and Youden's index (81.3%). CBG is correlated with CRP (the Spearman correlation coefficient was 0.379, P < 0.01). CONCLUSION: Serum CBG concentration increases early in NEC. Serum CBG level was associated with extensive disease in infants with NEC. Therefore CBG can be used as a marker in the early diagnosis of NEC.