Emerging importance of multidrug-resistant Stenotrophomonas maltophilia infections in neonatal intensive care unit in a tertiary center in Turkey.

OBJECTIVE: Stenotrophomonas maltophilia is an emerging multi-drug resistant, opportunistic pathogen in the neonatal intensive care unit (NICU). In this study, we aimed to assess the incidence, clinical features, antibiotic susceptibility, and treatment options of S. maltophilia infection among the healthcare-associated infections (HAIs) in the neonatal unit. METHODS: In this study, the patients who were hospitalized in the NICU between January 2020 and December 2021 with S. maltophilia isolated from clinical samples were included. Demographic, clinic features, and microbiological findings of the patients were retrospectively evaluated by using the medical records. The samples (lower respiratory tract, urine, peritoneal fluid) were first examined microscopically by gram preparation and cultured. Antibiotic susceptibility tests were performed according to the recommendations of The European Committee on Antimicrobial Susceptibility Testing (EUCAST) for TMP-SMX. RESULTS: S. maltophilia was isolated in 38 clinical samples of the 20 patients who were hospitalized at the NICU between January 2020 and December 2021. A total of 40 % (n = 8) of samples from different patients were accepted as colonization. Ventilator-associated pneumonia was determined in 55 % (n = 11), and urinary tract infection in 5 % (n = 1). S. maltophilia-associated bacteremia was not detected in any of the cases. The TMP-SMX susceptibilities of the strains were as it follows: 3 (15 %) were resistant (R), 7 (28 %) were susceptible (S), and 10 (47 %) were susceptible-increased exposure (I). Three of these patients were given dual antibiotics therapy (levofloxacin plus TMP-SMX) and nine of them were given only TMP-SMX. The most common hospital-acquired infectious agents are Gram negative microorganisms (51 %), followed by coagulase negative staphylococci (CNS), Staphylococcus aureus (24 %) and S. maltophilia (24 %). CONCLUSION: Increasing TMP-SMX resistance and specific drug and dosage-related problems in the neonatal unit are important problems in treatment management.

A snapshot of pediatric inpatients and outpatients with COVID-19: a point prevalence study from Turkey.

This multi-center point prevalence study evaluated children who were diagnosed as having coronavirus disease 2019 (COVID-19). On February 2nd, 2022, inpatients and outpatients infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) were included in the study from 12 cities and 24 centers in Turkey. Of 8605 patients on February 2nd, 2022, in participating centers, 706 (8.2%) had COVID-19. The median age of the 706 patients was 92.50 months, 53.4% were female, and 76.7% were inpatients. The three most common symptoms of the patients with COVID-19 were fever (56.6%), cough (41.3%), and fatigue (27.5%). The three most common underlying chronic diseases (UCDs) were asthma (3.4%), neurologic disorders (3.3%), and obesity (2.6%). The SARS-CoV-2-related pneumoniae rate was 10.7%. The COVID-19 vaccination rate was 12.5% in all patients. Among patients aged over 12 years with access to the vaccine given by the Republic of Turkey Ministry of Health, the vaccination rate was 38.7%. Patients with UCDs presented with dyspnea and pneumoniae more frequently than those without UCDs (p < 0.001 for both). The rates of fever, diarrhea, and pneumoniae were higher in patients without COVID-19 vaccinations (p = 0.001, p = 0.012, and p = 0.027).  Conclusion: To lessen the effects of the disease, all eligible children should receive the COVID-19 vaccine. The illness may specifically endanger children with UCDs. What is Known: • Children with COVID-19 mainly present with fever and cough, as in adults. • COVID-19 may specifically threaten children with underlying chronic diseases. What is New: • Children with obesity have a higher vaccination rate against COVID-19 than children without obesity. • Among unvaccinated children, fever and pneumoniae might be seen at a higher ratio than among vaccinated children.

[Long COVID in Children: A Pediatric Center Experience]. / COVID-19 Nedeniyle Hastanede Yatan Çocuk Hastalarda Uzun Dönem Etkilerin ve Uzamis COVID Sikliginin Degerlendirilmesi.

Although COVID-19 (Coronavirus disease-2019) is observed to be milder in children, it has been observed that the symptoms continue for a long time in many people after the acute period of the disease, especially the multisystemic inflammatory syndrome (MISC) that developed after COVID-19 with the progression of the pandemic. Although it was first defined by different names such as long COVID and post COVID in adults, it has been observed in studies that similar complaints such as cough, fatigue and difficulty in concentrating continue for a long time in children, just as in adults. In our study, we aimed to evaluate the status of long COVID in childhood. Our study included patients aged from one month to 18 years with moderate and severe symptoms who were hospitalized and discharged for SARS-CoV-2 infection in Istanbul University Faculty of Medicine between November 1, 2020 and November 1, 2021. A questionnaire form was created to learn about the complaints of the patients and their ongoing complaints. The patients/parents were called by phone and their complaints were recorded in the questionnaire. The patients were classified according to the definitions in the guidelines published by NICE, RCGP and SIGN. In total, 116 patients were included; 57.8% (n= 64) male, 42.5% (n= 49) female; 53.4% (n= 62) 0-9, 46.6% (n= 54) 10-18 years old. Comorbid conditions were found in 64 (55.2%) patients. The mean follow-up period was 5.90 ± 3.61 [min-max (1-12)] months; longest symptom durations: decrease in effort loss/fatigue 19.25 ± 74.56 (0-365) days, concentration difficulties 11.12 ± 49.75 (0-365) days, fatigue 9.61 ± 34.96 (0-365) days and cough were 8.34 ± 35.37 (0-365) days. The percentage of the patients who met the definition of subacute/ongoing symptomatic COVID-19 was 37.9% (n= 44). The most common symptoms were the decrease of effort capacity/fatigue 12.1% (n= 14) and the concentration difficulties 10.3% (n= 12) in subacute symptomatic patients. The percentage of patients matching the definition of chronic/post COVID-19 was 11.2% (n= 13). In the first year of the disease, ongoing complaints such as fatigue and concentration difficulties were observed in eight patients. The rate of concentration difficulties in the 10-18 age group was statistically significantly higher than the 0-9 age group (p= 0.037). In terms of other symptoms, no significant difference was found according to age, gender and concomitant disease status. Out of these, one patient was diagnosed with Type 2 diabetes mellitus during the acute illness, and two patients were diagnosed with allergic rhinitis after COVID-19. A statistically significant difference was found in the rates of concentration disorders according to age groups with subacute/ongoing symptoms. Although only the hospitalized patients were included, fatigue and difficulty in concentration were among the most common ongoing symptoms in our study, similar to the literature, and they were seen to be more common in older children. It is important both for early diagnosis and awareness to follow up children with COVID-19 in terms of symptoms, not only in terms of prolonged symptoms but also in terms of new diseases.

Mutation identification and prediction for severe cardiomyopathy in Alström syndrome, and review of the literature for cardiomyopathy.

OBJECTIVE: Alström syndrome (ALMS) is a rare autosomal recessive genetic disorder that is caused by homozygous or compound heterozygous mutation in the ALMS1 gene. Dilated cardiomyopathy (DCM) is one of the well-recognized features of the syndrome ranging from sudden-onset infantile DCM to adult-onset cardiomyopathy, sometimes of the restrictive hypertrophic form with a poor prognosis. We aimed to evaluate severe cardiomyopathy in Alström syndrome in infancy and display susceptible specific mutations of the disease, which may be linked to severe DCM. Secondarily we reviewed published mutations in ALMS1 with cardiomyopathies in the literature. METHOD: We represent new mutagenic alleles related to severe cardiomyopathy and cardiac outcome in this patient cohort. We evaluated echocardiographic studies of nine Turkish patients diagnosed with Alström syndrome (between 2014 and 2020, at age two weeks to twenty years). Thus, we examined the cardiac manifestations of a single-centre prospective series of nine children with specific ALMS mutations and multisystem involvement. All patients underwent genetic and biochemical testing, electrocardiograms, and echocardiographic imaging to evaluate systolic strain with speckle tracking. RESULTS: Four of the patients died from cardiomyopathy. Three patients (including three of the four fatalities) with the same mutation (c.7911dupC [p.Asn2638Glnfs*24]) had cardiomyopathy with intra-familial variability in the severity of cardiomyopathy. Global longitudinal strain, a measure of systolic contractile function, was abnormal in all patients that can be measured. CONCLUSION: Cardiac function in ALMS patients with infantile cardiomyopathy appears to have different clinical spectrums depending on the mutagenic allele. The c.7911dupC (p. Asn2638Glnfs*24) mutation can be related to severe cardiomyopathy. Parents can be informed and consulted about the progression of severe cardiomyopathy in a child carrying this mutagenic allele.

Impact of sodium phenylbutyrate treatment in acute management of maple syrup urine disease attacks: a single-center experience.

OBJECTIVES: Accurate management of metabolic decompensation in maple syrup urine disease (MSUD) has a crucial role, as acute attacks can cause neurological sequels and can be life threatening. Here, we aimed to evaluate effect of sodium phenylbutyrate (NaPBA) in acute management of MSUD attacks. METHODS: Episodes with an initial plasma leucine (Leu) level above 750 µmoL/L and that require hospitalization due to clinical findings of Leu neurotoxicity and/or feeding difficulties were included to the study. Patients who had no molecular diagnosis and a regular follow-up were excluded. Clinical findings, laboratory results and therapy responses were reviewed, retrospectively. RESULTS: Ten patients who experienced 19 distinct episodes of MSUD attacks were enrolled. Initial median Leu level was 901.67 (range 756-1989.11) and 33.9 µmoL/L (range 7.91-347.3 µmoL/L) at the end of therapy. None of our patients underwent extracorporeal toxin removal during the course of attack. In patients with serial plasma quantitative amino acid sampling, mean Leu reduction rate was calculated to be 529.68 ± 250.08 µmoL/L/day at the 24th h of treatment and 318.72 ± 191.52 µmoL/L/day at the 48th h of treatment. CONCLUSIONS: This study is the first original study that investigates the effect of NaPBA in management of acute attacks of MSUD patients from Turkey. We suggest that NaPBA treatment in MSUD attacks can ameliorate clinical and biochemical findings. This therapeutic option should be considered especially in smaller centers without the toxin removal chance and for patients who were not appropriate for extracorporeal toxin removal like hemodynamic instability.

Comparison of the efficacy of physical examination and radiological imaging in detecting sacroiliitis in patients with juvenile spondyloarthropathies.

OBJECTIVES: To determine and compare the effectiveness of history, physical examination, conventional radiography and magnetic resonance imaging (MRI) in the detection of sacroiliitis in juvenile spondyloarthropathies. METHODS: One hundred and one patients with JSpA, 33 patients with other diseases and 24 children without rheumatologic complaints were included in the study. Subjects were evaluated using physical examination, laboratory findings, pelvic radiography and MRI. Abdominal or pelvic MRIs of 24 control patients who were obtained in the last 6 months were reevaluated and multivariate logistic regression analyses were used to calculate probability ratios of variables. RESULTS: In our study, the rate of active sacroiliitis was 52.4% and in most of them, erosive and sclerotic changes indicating destruction of the sacroiliac joints were recorded. The presence of sacroiliitis on direct x-ray, high JSPADAI score, and hip involvement on MRI were independent risk factors with high predictive potential for active sacroiliitis. Inflammatory lumbar pain, sacroiliac tenderness, modified Schober's limitation, acute phase elevation, HLA-B27 positivity and presence of uveitis failed to predict sacroiliitis. The best specificity was 100% with a high BASFI score (>5), then 94% with a high JSPADAI score (>4). None of the patients in the control group showed active sacroiliitis. CONCLUSIONS: All patients with possible JSpA should undergo sacroiliac MRI whether HLA-B27 positive or not. In this way, early diagnosis and treatment of axial joint involvement could be possible and it prevents unnecessary examination and loss of time.