DATA SOURCES: A PubMed search was conducted using Clinical Queries with the key terms "Gianotti-Crosti syndrome" OR "papular acrodermatitis". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. This paper is based on, but not limited to, the search results.
RESULTS: The eruption of Gianotti-Crosti syndrome is found predominantly on the cheeks, extensor surfaces of the extremities, and buttocks. There is a sparing of antecubital and popliteal fossae as well as palms, soles, and mucosal surfaces. Although often asymptomatic, the lesions may be mildly to moderately pruritic. Gianotti-Crosti syndrome is most common in children between 1 and 6 years of age. The Epstein-Barr virus and the hepatitis B virus are the most common pathogens associated with Gianotti-Crosti syndrome. No treatment for Gianotti-Crosti syndrome is necessary because it is self-limited. In an era of vaccine hesitancy and refusal, Gianotti-Crosti syndrome may be important to mention to parents, because it can occur and trigger alarmism.
CONCLUSIONS: Gianotti-Crosti syndrome is mainly a disease of early childhood, characterized by an acute onset of a papular or papulovesicular eruption with a symmetrical distribution. With the advent of more universal vaccination against hepatitis B virus, Epstein-Barr virus has become the most common etiologic agent of Gianotti-Crosti syndrome. Few cases of post-vaccination Gianotti-Crosti syndrome have been reported. Currently, the emphasis should be placed on its self-limiting attribution.
DATA SOURCES: A PubMed search was completed in Clinical Queries using the key terms "Staphylococcal scalded skin syndrome" and "Ritter disease".
RESULTS: SSSS is caused by toxigenic strains of Staphylococcus aureus. Hydrolysis of the amino-terminal extracellular domain of desmoglein 1 by staphylococcal exfoliative toxins results in disruption of keratinocytes adhesion and cleavage within the stratum granulosum which leads to bulla formation. The diagnosis is mainly clinical, based on the findings of tender erythroderma, bullae, and desquamation with a scalded appearance especially in friction zones, periorificial scabs/crusting, positive Nikolsky sign, and absence of mucosal involvement. Prompt empiric treatment with intravenous anti-staphylococcal antibiotic such as nafcillin, oxacillin, or flucloxacillin is essential until cultures are available to guide therapy. Clarithromycin or cefuroxime may be used should the patient have penicillin allergy. If the patient is not improving, critically ill, or in communities where the prevalence of methicillin-resistant S. aureus is high, vancomycin should be used.
CONCLUSION: A high index of suspicion is essential for an accurate diagnosis to be made and treatment promptly initiated.
METHODS: A web-based survey was conducted between February 2019 and August 2021. Quantifying skin injuries and describing skincare practices in extremely preterm infants were the main outcomes. The association between skin injuries and skincare practices was established using binary multivariable logistic regression adjusted for regions.
RESULTS: Responses from 848 neonatal intensive care units, representing all geographic regions and income status groups were received. Diaper dermatitis (331/840, 39%) and medical adhesive-related skin injuries (319/838, 38%) were the most common injuries. Following a local skincare guideline reduced skin injuries [medical adhesive-related injuries: adjusted odds ratios (aOR) = 0.63, 95% confidence interval (CI) = 0.45-0.88; perineal injuries: aOR = 0.66, 95% CI = 0.45-0.96; local skin infections: OR = 0.41, 95% CI = 0.26-0.65; chemical burns: OR = 0.46, 95% CI = 0.26-0.83; thermal burns: OR = 0.51, 95% CI = 0.27-0.96]. Performing skin assessments at least every four hours reduced skin injuries (abrasion: aOR = 0.48, 95% CI = 0.33-0.67; pressure: aOR = 0.51, 95% CI = 0.34-0.78; diaper dermatitis: aOR = 0.71, 95% CI = 0.51-0.99; perineal: aOR = 0.52, 95% CI = 0.36-0.75). Regional and resource settings-based variations in skin injuries and skincare practices were observed.
CONCLUSIONS: Skin injuries were common in extremely preterm infants. Consistency in practice and improved surveillance appears to reduce the occurrence of these injuries. Better evidence regarding optimal practices is needed to reduce skin injuries and minimize practice variations.
METHODS: All cases of IO-IBD, defined as onset of disease before 12 mo of age, seen at University Malaya Medical Center, Malaysia were reviewed. We performed mutational analysis for IL10 and IL10R genes in patients with presenting clinical features of Crohn's disease (CD).
RESULTS: Six [13%; CD = 3, ulcerative colitis (UC) = 2, IBD-unclassified (IBD-U) = 1] of the 48 children (CD = 25; UC = 23) with IBD have IO-IBD. At final review [median (range) duration of follow-up: 6.5 (3.0-20) years], three patients were in remission without immunosuppression [one each for post-colostomy (IBD-U), after standard immunosuppression (CD), and after total colectomy (UC)]. Three patients were on immunosuppression: one (UC) was in remission while two (both CD) had persistent disease. As compared with later-onset disease, IO-IBD were more likely to present with bloody diarrhea (100% vs 55%, P = 0.039) but were similar in terms of an associated autoimmune liver disease (0% vs 19%, P = 0.31), requiring biologics therapy (50% vs 36%, P = 0.40), surgery (50% vs 29%, P = 0.27), or achieving remission (50% vs 64%, P = 0.40). No mutations in either IL10 or IL10R in the three patients with CD and the only patient with IBD-U were identified.
CONCLUSION: The clinical features of IO-IBD in this Asian cohort of children who were negative for IL-10 or IL-10R mutations were variable. As compared to childhood IBD with onset of disease after 12 mo of age, IO-IBD achieved remission at a similar rate.
METHODS: This was a descriptive, retrospective analysis, conducted at the Department of Paediatrics, University Malaya Medical Centre, Malaysia. All children who had esophagogastroduodenoscopy (EGD) and colonoscopy from January 2008 to June 2011 were included. An endoscopy was considered appropriate when its indication complied with the NASPGHAN and ASGE guideline. All endoscopic findings were classified as either positive (presence of any endoscopic or histologic abnormality) or negative (no or minor abnormality, normal histology); effecting a positive contributive (a change in therapeutic decisions or prognostic consequences) or non-contributive yield (no therapeutic or prognostic consequences).
RESULTS: Overall, 76% of the 345 procedures (231 EGD alone, 26 colonoscopy alone, 44 combined EGD and colonoscopy) performed in 301 children (median age 7.0 years, range 3 months to 18 years) had a positive endoscopic finding. Based on the NASPGHAN and ASGE guideline, 99.7% of the procedures performed were considered as appropriate. The only inappropriate procedure (0.3%) was in a child who had EGD for assessment of the healing of gastric ulcer following therapy in the absence of any symptoms. The overall positive contributive yield for a change in diagnosis and/or management was 44%. The presence of a positive endoscopic finding was more likely to effect a change in the therapeutic plan than an alteration of the initial diagnosis. A total of 20 (5.8%) adverse events were noted, most were minor and none was fatal.
CONCLUSION: The NASPGHAN and ASGE guideline is more likely to predict a positive endoscopic finding but is less sensitive to effect a change in the initial clinical diagnosis or the subsequent therapeutic plan.
METHODS: A retrospective analysis of 154 patients who underwent mitral valve repair using various chordal reconstructive procedures from 1992 to 2012. Patients were divided into group A and group B based on use of artificial chords and autologous in situ chords, respectively, for the repair. There were 102 (66.2%) patients in group A and 52 (33.8%) patients in group B. The mean age at repair was 11.1 ± 4.5 years. Associated cardiac anomalies were found in 94 (61%) patients.
RESULTS: The median follow-up period was 4.2 years (Interquartile range: 2.0-9.9). There were two (1.3%) early deaths and five (3.2%) late deaths. There was no significant difference in survival at 15 years between the two groups (group A: 91.8% vs group B: 95.1%; P = .66). There was no significant difference in the freedom from reoperation at 15 years between group A (79.4%) and group B (97.2%; P = .06). However, there was significant difference in freedom from valve failure between group A (56.5%) and group B (74.1%; P = .03). Carpentier functional class III and postoperative residual mitral regurgitation (2+ MR, ie, mild-moderate MR) were the risk factors for valve failure.
CONCLUSIONS: Severity of the disease and its progression has profound effect on the valve repair than the technique itself. Both chordal reconstructive procedures can be used to produce satisfactory results in children.
METHODS: Between January 2013 and June 2015, a total of 116 patients underwent arterial switch operation. Of the 116 patients, 26 with TGA-IVS underwent primary arterial switch operation at more than 30 days of age.
RESULTS: The age and body weight (mean ± SD) at the operation were 120.4 ± 93.8 days and 4.1 ±1.0 kg, respectively. There was no hospital mortality. The thickness of posterior LV wall (preoperation vs postoperation; mm) was 4.04 ± 0.71 versus 5.90 ± 1.3; P < .0001; interval: 11.8 ± 6.5 days. The left atrial pressure (mm Hg; postoperative day 0 vs 3) was 20.0 ± 3.2 versus 10.0 ± 2.0; P < .0001; and the maximum blood lactate level (mmol/dL) was 4.7 ± 1.4 versus 1.4 ± 0.3; P < .0001, which showed significant improvement in the postoperative course. All cases had delayed sternal closure. The patients who belonged to the thin LV posterior wall group (<4 mm [preoperative echo]: n = 13) had significantly longer ventilation time (days; 10.6 ± 4.8 vs 4.8 ± 1.7, P = .0039), and the intensive care unit stay (days) was 14 ± 9.2 versus 7.5 ± 3.5; P = .025, compared with thick LV wall group (≥4.0 mm: n = 13).
CONCLUSIONS: The children older than 30 days with TGA-IVS can benefit from primary arterial switch operation with acceptable results under our indication. However, we need further investigation for LV function.
METHODS: A total of 16,040 primary procedures were identified over a two-year period. Centers that submitted procedures were dichotomized to low/middle income (LMI) and high income (HI) by the Gross National Income per capita categorization. Mortality was defined as any death following the primary procedure to discharge or 90 days inpatient. Multiple logistic regression models were utilized to identify independent predictors of mortality.
RESULTS: Of the total number of procedures analyzed, 83% (n = 13,294) were from LMI centers. Among all centers, the mean age at operation was 2.2 years, with 36% (n = 5,743) less than six months; 85% (n = 11,307) of procedures were STAT I/II for LMI centers compared with 77% (n = 2127) for HI centers (P