Risk Factors for Infection and Mortality Associated With Stenotrophomonas maltophilia Bloodstream Infections in Children; Comparison With Pseudomonas aeruginosa Bloodstream Infections.

INTRODUCTION: The increasing incidence of Stenotrophomonas maltophilia ( S. maltophilia ) infections raises concern because of the high fatality/case ratio. This study aimed to evaluate the risk factors for infection and mortality associated with S. maltophilia bloodstream infections (BSIs) in children and compare them with Pseudomonas aeruginosa BSIs. METHODS: All BSIs caused by S. maltophilia (n:73) and P. aeruginosa (n:80) were enrolled in this study between January 2014 and December 2021 at the Medical School of Ege University. RESULTS: Previous Pediatric Intensive Care Unit (PICU) admission, prior glycopeptide, and carbapenem use were significantly more common in patients with S. maltophilia BSIs ( P = 0.044, P = 0.009, and P = 0.001, respectively) than with P. aeruginosa BSIs. C-reactive protein (CRP) levels were significantly higher in S. maltophilia BSIs ( P = 0.002). Multivariate analysis showed that prior carbapenem use was associated with S. maltophilia BSIs ( P = 0.014, adjusted odds ratio [AOR]: 2.710; 95% confidence interval [CI]: 1.225-5.992). PICU admission because of BSI, prior carbapenem and glycopeptide use, neutropenia, and thrombocytopenia were significantly more common in patients with mortality because of S. maltophilia BSIs ( P < 0.001, P = 0.010, P = 0.007, P = 0.008, P = 0.004, respectively), while only PICU admission because of BSI, and prior glycopeptide use were significant in multivariate analysis (AOR, 19.155; 95% CI: 2.337-157.018; P = 0.006 and AOR, 9.629; 95% CI: 1.053-88.013; P = 0.045, respectively). CONCLUSION: Prior carbapenem use is a significant risk factor for developing S. maltophilia BSIs. PICU admission because of BSI and prior glycopeptide use are risk factors associated with the mortality rate in patients with S. maltophilia BSIs. Therefore, S. maltophilia should be considered in patients with these risk factors, and empirical treatment should include antibiotics for S. maltophilia .

Clinical Utility of Respiratory Scores at Admission for Estimating the Definitive Microbiological Diagnosis in Lower Respiratory Tract Infections in Infants.

We aimed at determining the clinical utility of respiratory scores and the durations of wheezing or respiratory distress during hospitalization in infants with lower respiratory tract infections (LRTI) at admission for estimating the definitive microbiological diagnosis. We obtained data from a study population of 201 patients, 79 girls and 122 boys. There was a significant divide in the causative agents of LRTI among patients younger and older than 6 months of age (P = .002), and significantly different respiratory score findings were determined in infants with viral LRTI: a low respiratory score in a younger-than-6 month infant suggests Adenovirus as the causative agent and a high respiratory score suggests Parainfluenza 1 or 2; as for infants of 6 months of age or older, a low respiratory score indicates Influenza A or B or a mixed infection, whereas a high respiratory score is likely an indication of Parainfluenza 3 or RSV.

Effect of Breastfeeding and Preterm Births on the Severity of Lower Respiratory Tract Infections and Associated Risk of Hospitalization in Infants and Toddlers.

We studied the effect of duration of breastfeeding and history of prematurity on the duration of hospitalization in infants with lower respiratory tract infections (LRTI) because these may reflect the severity of illness as well as sizable direct and indirect healthcare costs. One hundred twenty-five patients (49 girls, 76 boys; aged 1-24 months) were hospitalized for LRTI during a period of 102 days and studied prospectively. We found a significant difference (P = .045) between the durations of hospitalization of the 92 patients breastfed for at least six months, compared to the other group of 33 patients who were breastfed for less than six months. The durations of hospitalization among the groups with and without a history of prematurity were not statistically different (P = .78). A history of breastfeeding for more than six months had significant effect on the duration of hospitalization, but this was not true for children with a history of preterm birth.

Aplastic Anemia Frequency and Management in Pediatric Liver Transplantations Due to Non-A-E Hepatitis.

BACKGROUND: Hepatitis-associated aplastic anemia (HAAA) is a rare complication that presented with bone marrow failure after acute hepatitis. HAAA usually occurs in adolescent men within 1-6 months following hepatitis. Most of HAAA's etiology has non-A-E viral hepatitis. METHODS: Our retrospective study included patients with acute fulminant hepatitis who had been treated in Ege University Pediatric Gastroenterology, Hepatology and Nutrition Department and Izmir Kent Hospital Clinical, laboratory, and epidemiological data of the patients were collected from the files. RESULTS: In this study, 499 children underwent liver transplantation (LT) in two pediatric transplantation centers. Sixty-eight (13.6%) out of 499 patients, underwent liver transplantation due to fulminant hepatic failure (FHF). Therefore, a total of 64 patients (34 girls, 30 boys) with a diagnosis of FHF have included in the study. Thirty-two (50.0%) of 64 FHF were due to non-A-E hepatitis and 4 out of the 64 patients (6.2%) with FHF developed HAAA. All of the patients received prednisolone as immunosuppression treatment after LT. Three patients were also given Tacrolimus and 1 received an additional mycophenolate mofetil. One of the patients was given prednisolone and cyclosporine treatment without tacrolimus. Bone marrow transplantation was performed in 1 patient (25.0%). Two of the patients received immunosuppressive treatment including rabbit-derived anti-thymocyte globulin, cyclosporine, and initially prednisolone. CONCLUSION: In children who underwent liver transplantation for non-A-E FHF are at high risk to develop aplastic anemia. The clinicians should be alert after orthotropic liver transplantation patient could develop aplastic anemia and early treatment with immunosuppressive therapies result in a more successful outcome.

Homozygous c.130-131 ins A (pW44X) mutation in the HAX1 gene as the most common cause of congenital neutropenia in Turkey: Report from the Turkish Severe Congenital Neutropenia Registry.

BACKGROUND: Severe congenital neutropenia is a rare disease, and autosomal dominantly inherited ELANE mutation is the most frequently observed genetic defect in the registries from North America and Western Europe. However, in eastern countries where consanguineous marriages are common, autosomal recessive forms might be more frequent. METHOD: Two hundred and sixteen patients with severe congenital neutropenia from 28 different pediatric centers in Turkey were registered. RESULTS: The most frequently observed mutation was HAX1 mutation (n = 78, 36.1%). A heterozygous ELANE mutation was detected in 29 patients (13.4%) in our cohort. Biallelic mutations of G6PC3 (n = 9, 4.3%), CSF3R (n = 6, 2.9%), and JAGN1 (n = 2, 1%) were also observed. Granulocyte colony-stimulating factor treatment was given to 174 patients (80.6%). Two patients died with infectious complications, and five patients developed myelodysplastic syndrome/acute myeloblastic leukemia. The mean (± mean standard error) follow-up period was 129.7 ± 76.3 months, and overall survival was 96.8% (CI, 94.4-99.1%) at the age of 15 years. In Turkey, severe congenital neutropenia mostly resulted from the p W44X mutation in the HAX1 gene. CONCLUSION: In Turkey, mutation analysis should be started with HAX1, and if this is negative, ELANE and G6PC3 should be checked. Because of the very high percentage of consanguineous marriage, rare mutations should be tested in patients with a negative mutation screen.

Fertility in Patients with Thalassemia and Outcome of Pregnancies: A Turkish Experience

Objective: In recent years, the rates of marriage and pregnancy are increasing in patients with thalassemia major. The aim of the present study was to investigate the fertility rate of thalassemic patients and the course of pregnancies in terms of mother and infant health. Materials and Methods: In this observational study patients with major hemoglobinopathy were evaluated regarding marital status, the need for assisted reproductive techniques, fertility rate, iron status, and pregnancy complications. Results: Seventeen female patients gave birth to 21 healthy infants. About one-third of the patients needed assisted reproductive techniques. Thalassemia major patients showed increased serum ferritin levels from 1203±1206 µg/L at baseline to 1880±1174 µg/L at the end of pregnancy. All babies are still alive and healthy. Conclusion: Pregnancy in patients with thalassemia can be safe for the mother and newborn with close monitoring and a multidisciplinary approach.

A National Registry of Thalassemia in Turkey: Demographic and Disease Characteristics of Patients, Achievements, and Challenges in Prevention.

OBJECTIVE: The Turkish Society of Pediatric Hematology set up a National Hemoglobinopathy Registry to demonstrate the demographic and disease characteristics of patients and assess the efficacy of a hemoglobinopathy control program (HCP) over 10 years in Turkey. MATERIALS AND METHODS: A total of 2046 patients from 27 thalassemia centers were registered, of which 1988 were eligible for analysis. This cohort mainly comprised patients with ß-thalassemia major (n=1658, 83.4%) and intermedia (n=215, 10.8%). RESULTS: The majority of patients were from the coastal areas of Turkey. The high number of patients in Southeastern Anatolia was due to that area having the highest rates of consanguineous marriage and fertility. The most common 11 mutations represented 90% of all ß-thalassemia alleles and 47% of those were IVS1-110(G->A) mutations. The probability of undergoing splenectomy within the first 10 years of life was 20%, a rate unchanged since the 1980s. Iron chelators were administered as monotherapy regimens in 95% of patients and deferasirox was prescribed in 81.3% of those cases. Deferasirox administration was the highest (93.6%) in patients aged <10 years. Of the thalassemia major patients, 5.8% had match-related hemopoietic stem cell transplantation with a success rate of 77%. Cardiac disease was detected as a major cause of death and did not show a decreasing trend in 5-year cohorts since 1999. CONCLUSION: While the HCP has been implemented since 2003, the affected births have shown a consistent decrease only after 2009, being at lowest 34 cases per year. This program failure resulted from a lack of premarital screening in the majority of cases. Additional problems were unawareness of the risk and misinformation of the at-risk couples. In addition, prenatal diagnosis was either not offered to or was not accepted by the at-risk families. This study indicated that a continuous effort is needed for optimizing the management of thalassemia and the development of strategies is essential for further achievements in the HCP in Turkey.

Brain natriuretic peptide: the reason of respiratory distress is heart disease or lung disease?

OBJECTIVES: The aim of this study was to determine whether plasma levels of amino-terminal brain natriuretic peptide (BNP) could differentiate between heart failure and lung disease among infants with acute bronchiolitis. METHODS: Sixty-eight infants (age range, 1-26 months; median age, 5.9 ± 5.0 months) who presented with respiratory distress underwent physical examination, plasma BNP measurement, and echocardiography within 24 hours after admission. Nineteen (28%) patients had congenital heart disease. The control group was consisted of 30 healthy infants. RESULTS: Although mean plasma BNP levels were 118.9 ± 219.5 pg/mL in patients with isolated bronchiolitis (n = 49), it was 841.2 ± 1475.8 pg/mL in patients with congenital heart disease (n = 19). Plasma BNP levels were significantly higher in infants with congenital heart disease (P = .001). CONCLUSION: It was shown that plasma BNP levels were affected much more in cardiac disease rather than lung disease. Among infants with respiratory distress, plasma BNP measurements can differentiate congenital heart disease and lung disease and can be used to monitor the effects of treatment for infants with heart failure. RESPONSE TO REVIEWERS: The comments were taken for consideration. The patient groups control BNP levels were attached to the results. As it was a clinical study and multiple factors (respiratory score, respiratory rate, treatment, etc) may effect on BNP levels, the tables could not be decreased to 1 table.