Autoimmune Hemolytic Anemia in the Pediatric Setting.

Autoimmune hemolytic anemia (AIHA) is a rare disease in children, presenting with variable severity. Most commonly, warm-reactive IgG antibodies bind erythrocytes at 37 °C and induce opsonization and phagocytosis mainly by the splenic macrophages, causing warm AIHA (w-AIHA). Post-infectious cold-reactive antibodies can also lead to hemolysis following the patient's exposure to cold temperatures, causing cold agglutinin syndrome (CAS) due to IgM autoantibodies, or paroxysmal cold hemoglobinuria (PCH) due to atypical IgG autoantibodies which bind their target RBC antigen and fix complement at 4 °C. Cold-reactive antibodies mainly induce intravascular hemolysis after complement activation. Direct antiglobulin test (DAT) is the gold standard for AIHA diagnosis; however, DAT negative results are seen in up to 11% of warm AIHA, highlighting the need to pursue further evaluation in cases with a phenotype compatible with immune-mediated hemolytic anemia despite negative DAT. Prompt supportive care, initiation of treatment with steroids for w-AIHA, and transfusion if necessary for symptomatic or fast-evolving anemia is crucial for a positive outcome. w-AIHA in children is often secondary to underlying immune dysregulation syndromes and thus, screening for such disorders is recommended at presentation, before initiating treatment with immunosuppressants, to determine prognosis and optimize long-term management potentially with novel targeted medications.

Can we predict the behavior of the scoliotic curve after bracing in adolescent idiopathic scoliosis? Τhe prognostic value of apical vertebra rotation.

PURPOSE: We aimed to recognize radiographic and clinical prognostic factors of scoliotic curve behaviour after bracing. METHODS: Our prognostic study was based on the 25 years outcomes of a Boston braced AIS cohort between 1978 and 1993 that were previously reported. All patients were followed-up during bracing, at short term and 25 years post-bracing. We evaluated the impact of socio-demographic, clinical and radiological parameters on the loss of curve correction after bracing. RESULTS: Seventy-seven patients were reevaluated at 25 years post-brace. The mean scoliotic curve was significantly increased after bracing until the 25 years follow-up (p < 0.001). The mean loss of curve correction between the end of bracing and long-term follow-up was independent on the curve type, apical vertebra, premenarcheal status at bracing, time and duration of bracing, Cobb angle before or after bracing. The mean apical vertebral rotation after bracing was significantly related to the loss of curve correction (Spearman ρ = 0.2, p = 0.049). Apical vertebral rotation (Perdriolle method) greater than 20° post-bracing had a three times higher chance of progression > 5° compared with lesser apical vertebral rotation (OR 3.071, CI 0.99-9.51). The rotation of the apical vertebra, type and magnitude of the scoliotic curve after bracing explained 27.4% of the variance in the loss of curve correction post-bracing (R square = 0.274, p < 0.001). CONCLUSION: A scoliotic curve is expected to lose some correction after bracing. The apical vertebral rotation post-bracing mainly affected the long-term curve behaviour. Adolescents with apical vertebral rotation greater than 20° after bracing may need further attention. LEVEL OF EVIDENCE: Prognostic study, Level II.

Pulmonary Infectious Complications in Children with Hematologic Malignancies and Chemotherapy-Induced Neutropenia.

Infections frequently complicate the treatment course in children with hematologic malignancies undergoing chemotherapy. Febrile neutropenia (FN) remains a major cause of hospital admissions in this population, and respiratory tract is often proven to be the site of infection even without respiratory signs and symptoms. Clinical presentation may be subtle due to impaired inflammatory response. Common respiratory viruses and bacteria are widely identified in these patients, while fungi and, less commonly, bacteria are the causative agents in more severe cases. A detailed history, thorough clinical and basic laboratory examination along with a chest radiograph are the first steps in the evaluation of a child presenting signs of a pulmonary infection. After stratifying patient's risk, prompt initiation of the appropriate empirical antimicrobial treatment is crucial and efficient for the majority of the patients. High-risk children should be treated with an intravenous antipseudomonal beta lactam agent, unless there is suspicion of multi-drug resistance when an antibiotic combination should be used. In unresponsive cases, more invasive procedures, including bronchoalveolar lavage (BAL), computed tomography (CT)-guided fine-needle aspiration or open lung biopsy (OLB), are recommended. Overall mortality rate can reach 20% with higher rates seen in cases unresponsive to initial therapy and those under mechanical ventilation.

Permanent pacemaker implantation in pediatric heart transplant recipients: A systematic review and evidence quality assessment.

Bradyarrhythmias are a common complication following pediatric OHT and may require permanent pacemaker implantation (PPM). The purpose of this study was to investigate the incidence, predictors, and outcomes of children undergoing PPM implantation following OHT. A PRISMA-compliant systematic literature review was performed using the PubMed database and the Cochrane Library (end-of-search date: January 27, 2019). The Newcastle-Ottawa scale and the Joanna Briggs Institute tool were used to assess the quality of cohort studies and case reports, respectively. We analyzed data from a total of 11 studies recruiting 7198 pediatric patients who underwent heart transplant. PPM implantation was performed in 1.9% (n = 137/7,198; 95% CI: 1.6-2.2) of the patients. Most patients underwent dual-chamber pacing (46%, 95% CI: 32.6-59.7). Male-to-female ratio was 1.3:1. Mean patient age at the time of OHT was 10.1 ± 6.3. Overall, biatrial anastomosis was used in 62.2% (95% CI: 52.8-70.6) of the patients. The bicaval technique was performed in the remaining 37.8% (95% CI: 29.4-47.1). Sinus node dysfunction was the most frequent indication for PPM implantation (54.4%; 95% CI: 42.6-65.7) followed by AV block (45.6%; 95% CI: 34.3-57.3). The median time interval between OHT and PPM implantation ranged from 17 days to 12.5 years. All-cause mortality was 27.9% (95% CI: 18.6-39.6) during a median follow-up of 5 years. PPM implantation is rarely required after pediatric OHT. The most common indication for pacing is sinus node dysfunction, and patients undergoing biatrial anastomosis may be more likely to require PPM.