Current Pediatric Reviews - Volume 18, Issue 4, 2022

Takayasu arteritis (TA) is the third most common vasculitis of childhood and is extremely rare. It is mainly characterized by chronic, autoimmune, and granulomatous inflammation of the aorta and its major branches. Women under 40 years of age are mostly affected. It occurs for the first time in childhood in about 30% of affected individuals. Initially, it presents nonspecific constitutional findings. Since there is no specific laboratory finding, diagnosis is challenging. The gold standard imaging method for diagnosis is conventional angiography. Delay in diagnosis can cause devastating consequences. Therefore, in cases presenting with nonspecific findings, with hypertension and high acute phase reactants, the diagnosis should be suspected and confirmed with appropriate imaging method, and treatment should be started immediately. Immunosuppressive agents are the mainstay of the treatment. Biological agents are successful in refractory cases, and endovascular revascularization methods are used in the treatment of complications.

Background: Idiopathic or Primary Nephrotic Syndrome (INS) is a common glomerular disease in pediatric population, characterized by proteinuria, edema and hypoalbuminemia with variable findings in renal histopathology. Objective: This review aims to summarize current data on the etiopathogenesis diagnosis, protocols of treatment and potential therapeutic advances in INS. Methods: This narrative review searched for articles on histopathology, physiopathology, genetic causes, diagnosis and treatment of INS in pediatric patients. The databases evaluated were PubMed and Scopus. Results: INS is caused by an alteration in the permeability of the glomerular filtration barrier with unknown etiology. There are several gaps in the etiopathogenesis, response to treatment and clinical course of INS that justify further investigation. Novel advances include the recent understanding of the role of podocytes in INS and the identification of genes associated with the disease. The role of immune system cells and molecules has also been investigated. The diagnosis relies on clinical findings, laboratory exams and renal histology for selected cases. The treatment is primarily based on steroids administration. In case of failure, other medications should be tried. Recent studies have also searched for novel biomarkers for diagnosis and alternative therapeutic approaches. Conclusion: The therapeutic response to corticosteroids still remains the main predictive factor for the prognosis of the disease. Genetic and pharmacogenomics tools may allow the identification of cases not responsive to immunosuppressive medications.

Sweet’s syndrome is a serious dermatological disorder characterized by a rapid onset of tender plaques or nodules, fever, joint pain, headache, and oral and genital lesions. According to the clinical features and underlying causes, Sweet’s syndrome is divided into three categories, i.e., classical (or idiopathic), malignancy-associated Sweet's syndrome, and drug-induced Sweet's syndrome. It is multifactorial in etiology, and the exact cause is still undetermined. The diagnosis can be confirmed by the routine histopathologic evaluation of skin biopsy from the lesions. The first-line treatment options are topical and systemic steroids. Multiple databases, like Medline/PubMed, Scopus, and Google, were used to identify resources for this literature review. The relevant information was collected from various case reports, case series, reviews, meta-analyses, and large clinical trials reporting clinical description, etiology, diagnosis, and management of Sweet’s syndrome. This narrative review aimed to discuss recent understandings related to Sweet's syndrome, both in terms of clinical presentation and management approach.

Inborn errors of metabolism (IEMs) are rare hereditary or acquired disorders resulting from an enzymatic deformity in biochemical and metabolic pathways influencing proteins, fats, carbohydrate metabolism, or hampered some organelle function. Even though individual IEMs are uncommon, together, they represent a diverse class of genetic diseases, with new issues and disease mechanisms being portrayed consistently. IEM includes the extraordinary multifaceted nature of the fundamental pathophysiology, biochemical diagnosis, molecular level investigation, and complex therapeutic choices. However, due to the molecular, biochemical, and clinical heterogeneity of IEM, screening alone will not detect and diagnose all illnesses included in newborn screening programs. Early diagnosis prevents the emergence of severe clinical symptoms in the majority of IEM cases, lowering morbidity and death. The appearance of IEM disease can vary from neonates to adult people, with the more serious conditions showing up in juvenile stages along with significant morbidity as well as mortality. Advances in understanding the physiological, biochemical, and molecular etiologies of numerous IEMs by means of modalities, for instance, the latest molecular-genetic technologies, genome engineering knowledge, entire exome sequencing, and metabolomics, have prompted remarkable advancement in detection and treatment in modern times. In this review, we analyze the biochemical basis of IEMs, clinical manifestations, the present status of screening, ongoing advances, and efficiency of diagnosis in treatment for IEMs, along with prospects for further exploration as well as innovation.

Background: Surfactant application by a thin catheter represented by the term less invasive surfactant administration (LISA) for respiratory distress syndrome in spontaneously breathing preterm infants was developed as an alternative to endotracheal intubation. Methods: We conducted a meta-analysis to assess the effects of LISA when compared to the socalled intubation-surfactant-extubation (INSURE) and the standard endotracheal intubation and mechanical ventilation (MV). The primary outcome was the composite incidence of death or bronchopulmonary dysplasia at a postmenstrual age of 36 weeks. The secondary outcome was the composite incidence of seven other severe adverse events. On 06 October 2021, we searched randomized clinical trials (RCTs) in PubMed, the Cochrane Library, ClinicalTrials.gov, and the ICTRP Registry. Results: We included 18 RCTs. The pooled data on the primary outcome favored LISA when compared to either INSURE (risk ratio 0.67; 95% CI, 0.51 to 0.88) or MV (risk ratio 0.78; 95% CI, 0.61 to 0.99). The pooled data on the second outcome also favored LISA when compared to INSURE (risk ratio 0.75; 95% CI, 0.60 to 0.94) and MV (risk ratio 0.73; 95% CI, 0.55 to 0.96). Conclusion: The findings showed that surfactant application by non-intubation respiratory support and the use of a thin catheter may decrease the composite risk of death or bronchopulmonary dysplasia. The included data support the view that LISA should be considered the preferred treatment option in eligible infants.

Background: Near-infrared spectroscopy (NIRS) has been used for monitoring cerebral oxygen saturation (rSO2) in neonates. There is a lack of data from low-middle income countries (LMIC) setting of cerebral rSO2 in neonates with encephalopathy of diverse etiologies. This study aimed to monitor cerebral rSO2 using NIRS in encephalopathic neonates to maintain the rSO2 between 55 to 85 % in the first 72 hours of admission to improve short-term neurodevelopmental outcomes (NDO). Materials and Methods: This prospective cohort study enrolled encephalopathic neonates with hypoxic- ischemic encephalopathy (HIE) and non-HIE etiologies into 8 clinical categories. The cerebral rSO2 was monitored and targeted to be between 55 to 85 %, with predefined actions and management alterations over 72 hours. The neurodevelopmental assessment was conducted at 3, 6, and 9-12 months corrected age. Moreover, the motor and mental developmental quotients (MoDQ) (MeDQ) were recorded and compared to historical control. Results: A total of 120 neonates were enrolled and assessed for NDO. The MoDQ (mean ± SD) was 92.55 ± 14.85, 93.80 ± 13.20, 91.02 ± 12.69 and MeDQ (mean ± SD) was 91.80 ± 12.98, 91.80 ± 13.69, 88.41 ± 11.60 at 3, 6 and 9-12 months. The MoDQ and MeDQ scores of the historic cohort at 12 months were 86.35 ± 20.34 and 86.58 ± 18.27. The mean difference [MD (95 %CI)] for MoDQ was - 4.670 (- 8.48 to - 0.85) (p=0.0165) and for MeDQ was - 1.83 (- 5.26 to 1.6) (p=0.29). There was a negative correlation between the composite developmental quotient (CoDQ) with mean rSO2 and a positive correlation with cerebral fractional tissue oxygen extraction (CFTOE). Neonates with HIE and neonatal encephalopathy (NE) (n=37/120) had the lowest motor and mental DQ on neurodevelopmental assessment. Clinical categories, neonatal meningitis (NM), and intraventricular hemorrhage (IVH) improved in DQ scores over the study period. Conclusion: Monitoring and maintaining cerebral rSO2 between 55-85 % through appropriate management changes improved neurodevelopmental scores at the 12-month follow-up in neonates with encephalopathy caused by varied etiologies.

Background: Vancomycin is a glycopeptide antibiotic that is used to treat serious grampositive infections. However, therapeutic drug monitoring for vancomycin is not performed routinely in Vietnam in clinical practices. Monitoring of serum vancomycin concentration or trough levels is necessary to ensure the efficacy and safety of vancomycin therapy. Objective: This study aims to determine the impact of initial vancomycin dose and creatinine clearance on target trough attainment in hospitalized Vietnamese children. Methods: A prospective study with patients who received vancomycin for at least three days was conducted. Subsequently, demographic data, clinical diagnosis, vancomycin dosage, and serum creatinine levels were recorded. The vancomycin trough level was collected and creatinine clearance and adjusted vancomycin doses were calculated. Results: A total of 40 eligible patients were enrolled. Patients’ mean age, body weight, and height were 1.4 years old, 9.8 kg, and 75.5 cm, respectively. The mean vancomycin dose was 55.83 ± 19.34 mg/kg/day. The mean creatinine clearance was 80.18 ± 29.14 ml/min. The median trough level was 11.09 (7.84 - 16.46) μg/ml. There was no significant difference in the mean initial and the adjusted vancomycin doses (p = 0.062). However, there were statistically significant differences of initial (p = 0.004) or adjusted doses (p = 0.016) between groups of creatinine clearance. The trough vancomycin concentration was not statistically significant (p = 0.406) between these groups. Conclusion: Target trough vancomycin level may be associated with creatinine clearance but did not proportionally correspond to the vancomycin dose. Therefore, monitoring vancomycin trough levels is necessary to achieve the target trough and to ensure vancomycin efficacy and safety in treating severely infected Vietnamese children.