Turkish adaptation of the maternal cognition about infant sleep questionnaire.

Background: Exploring early childhood sleep problems requires a detailed understanding of parental beliefs and cognitions related to infant sleep. There is a need for validated measures to investigate the cognitions of Turkish mothers about infant sleep however no scale measuring parental perceptions related to infant sleep behaviors in Turkish is available. We aimed to culturally adapt the Maternal Cognitions about Infant Sleep Questionnaire (MCISQ) in Turkish. Methods: Subjects were recruited from an internet sample through social media. Internal consistency was evaluated by Cronbach's alpha, and test-retest reliability was determined by Pearson's correlation test and paired t-test. For factorial validity, the principal component factor analysis was performed for the components of MCISQ. Results: A total of 417 mothers, most aged between 25 and 29 years (47.8 %), participated in the study. Infants' age ranged between 6 and 18 months, with a mean of 10.5 ± 3.9 months. Factor analysis revealed four factors after removal of item 11: Anger, doubt, safety, limit setting. Cronbach's alpha was 0.85. A subgroup of 32 mothers completed MCISQ three weeks after the initial administration. Total mean scores showed a significantly strong correlation (p:<0.01, r:0.82). Higher scores were noted in both total and subscale scores in infants with maternally reported sleep problems (p:<0.01). Conclusion: Findings suggest a four-factor solution for MCISQ in Turkish mothers with infants aged 6-18 months. The adapted Turkish version is composed of 19 items with good reliability. Factor structure and items included in the subscales differed from the original study, highlighting the cultural factors related to maternal perceptions about infant sleep.

Single-Center Experience in Vaccination of Children in Special Risk Groups: A Multidisciplinary Institutional Consensus Protocol.

OBJECTIVE: Despite marked improvements in the accessibility of childhood vaccines, knowledge gaps remain about the vaccination of children in special risk groups (SRG). This study aimed to analyze the clinical data of children vaccinated in SRG in a single-center unit to contribute to the clinical evidence for the specific planning of immunization of children in SRG. The second- ary aim is to present institutional consensus on the vaccination of children in SRG. MATERIALS AND METHODS: This retrospective study was conducted at a single-center pediatric vaccination clinic. Patient charts between 2018 and 2021 were retrospectively reviewed, and clinical and laboratory data were extracted. Serial joint meetings with multiple healthcare pro- fessionals were performed to develop an institutional protocol for vaccination. RESULTS: There were 479 children vaccinated between 2018 and 2021 for reasons such as post- chemotherapy, after hematopoietic stem cell transplantation, before/after solid organ trans- plantation, allergies, and chronic diseases. Of these, 298 (62.2%) children vaccinated in the unit due to a history of food or vaccine allergies were excluded. One hundred eighty-one children were vaccinated at a median age of 11 [7-15] years. Most children were vaccinated after treat- ment for malignancies. Solid tumors were the most frequent malignancy (67%), followed by acute lymphoblastic leukemia (29.0%) and acute myeloid leukemia (4.0%). Institutional vacci- nation protocols for cancer survivors, hematopoietic stem cells, and solid organ recipient chil- dren were developed and presented. CONCLUSION: There is a need to prepare national guidelines for vaccinating children with altered immunocompetence. Sharing vaccination practices by multidisciplinary vaccination units might increase and provide knowledge to develop national policies.

Effects of caffeine therapy for apnea of prematurity on sleep and neurodevelopment of preterm infants at 6 months of corrected age.

STUDY OBJECTIVES: To determine the differences in sleep patterns between preterm infants who received caffeine and those who did not and to evaluate the effects of caffeine therapy on early neurodevelopment. Secondarily, actigraphy and polysomnography were compared to evaluate the sleep of preterm infants. METHODS: Twenty-eight preterm infants ages 28-34 weeks admitted to a single-center Level III neonatal intensive care unit between May 2020 and May 2021 were included. Sleep was assessed by actigraphy for 72 hours with Respironics Mini-Mitter® Actiwatch-2 and Brief Infant Sleep Questionnaire at 6 months corrected age. On the first day of actigraphy, infants underwent polysomnography between 10:00 am and 3:00 pm. Neurodevelopment was evaluated by the Bayley Scales of Infant and Toddler Development-III, the Ages & Stages Questionnaire, and the Hammersmith Infant Neurological Examination. RESULTS: There were no significant differences in sleep parameters measured by actigraphy, the Brief Infant Sleep Questionnaire, and polysomnography between infants in the caffeine group (n = 12) and no-caffeine group (n = 16). Sensitivity (91.07%) and agreement rate (77.21%) for the actigraphy against polysomnography were highest at the automatic threshold. No significant differences were observed in the neurodevelopment of infants in the caffeine group compared to the no-caffeine group. CONCLUSIONS: Sleep parameters and neurodevelopmental outcomes were not different in infants at 6 months of corrected age with regard to caffeine therapy. Actigraphy at the automatic threshold can be used in infants for sleep pattern assessment. CLINICAL TRIAL REGISTRATION: Registry: ClinicalTrials.gov; Name: Influence of Caffeine Therapy in Preterm Infants; URL: https://www.clinicaltrials.gov/ct2/show/NCT04376749; Identifier: NCT04376749. CITATION: Atalah YEY, Baris HE, Akdere SK, et al. Effects of caffeine therapy for apnea of prematurity on sleep and neurodevelopment of preterm infants at 6 months of corrected age. J Clin Sleep Med. 2023;19(12):2075-2085.

Associations between sleep characteristics and glycemic variability in youth with type 1 diabetes.

OBJECTIVE: This study aimed to determine sleep characteristics and their associations with glycemic variability in youth with type 1 diabetes (T1D). MATERIAL AND METHODS: This cross-sectional study conducted at two pediatric diabetes centers in Istanbul, Turkey, included 84 children with T1D (mean age 10.5 years). Sleep characteristics and glycemic variability were determined by actigraphy, DSM-5 Level 2-Sleep Disturbance Scale Short Form and continuous glucose monitoring. Circadian preference was evaluated by the Children's Chronotype Questionnaire. Sleep disturbances were assessed by the. The sleep quality was determined by actigraphy-derived sleep measures. RESULTS: Eighty-eight percent of participants had insufficient age-appropriate total sleep time (TST) (<9 h for 6-13-year-olds and <8 h for 14-17-year-olds). Chronotype was classified as intermediate in 50%, evening in 45.2%, and morning in 4.8%. A higher chronotype score indicating a stronger eveningness preference was associated with more time spent in hypoglycemia (ß = 0.433, p = 0.002). On nights when participants had lower sleep efficiency and longer sleep onset latency, they had significantly higher overnight glycemic variability (ß = -0.343, p = 0.016, ß = 0.129, p = 0.017, respectively). Prolonged nocturnal wake duration was significantly associated with more time spent in daytime hypoglycemia (ß = 0.037, p = 0.046) and higher overnight glycemic variability (J index, ß = 0.300, p = 0.015). The associations between TST and glycemic variability indices were not significant. CONCLUSIONS: Sleep quality rather than TST was significantly associated with glycemic variability in children with T1D. Eveningness preference might contribute to an increased risk of hypoglycemia. Addressing sleep patterns and chronotypes can be crucial in management plans for youth with T1D.

Sleep assessment in preterm infants: Use of actigraphy and aEEG.

OBJECTIVE: Objective methods to monitor the sleep of preterm infants at the neonatal intensive care unit (NICU) are required to prevent potentially adverse neurodevelopmental outcomes. This study aimed to determine the concordance of actigraphy and amplitude-integrated electroencephalogram (aEEG) against gold standard direct observation (DO) in assessing sleep/wake states of typically developing preterm infants. METHODS: This prospective observational study was conducted in a single center level III NICU. Sleep variables were measured using Philips Respironics Mini-Mitter® Actiwatch-2 for 24 h and compared with 8-h matched data of aEEG and DO. Sensitivity-specificity analysis, Cohen's kappa, prevalence-adjusted and bias-adjusted kappa (PABAK), and Bland Altman plots were generated. RESULTS: Seventeen preterm infants were recruited. A total of 11252 epochs were studied. Sensitivity (86.4%), agreement rate (67.9%), and predictive value for wake (47.9%) for the actigraphy were highest at the automatic activity threshold whereas specificity (54.5%) and predictive value for sleep (75.5%) were highest at low threshold. The sensitivity of aEEG was 79.3% and the specificity was 54.3%. At all thresholds, the agreement was largely equivalent with low kappas (0.14-0.17) and PABAK coefficients (0.22-0.35) for actigraphy and DO. Moderate agreement was observed between aEEG and DO according to the PABAK coefficient (0.44). Mean differences in sleep parameters were not different between DO and aEEG as well as DO/aEEG and actigraphy at medium threshold (p > 0.05). CONCLUSIONS: Actigraphy at medium threshold can be used in depicting sleep in typically developing preterm infants at NICU. aEEG may be an alternative adjunctive method to actigraphy for the evaluation of sleep/wake states in the NICU setting. CLINICAL TRIAL REGISTRATION NUMBER: NCT04145362.

Rates of emergency room visits and hospitalizations among refugee and resident children in a tertiary hospital in Turkey.

Evaluation of emergency department (ED) presentation by Syrian refugee children might provide important information about their health care needs. For this purpose, we compared ED presentation of refugee and resident children in a tertiary university hospital in Istanbul, Turkey.Electronic medical records of Syrian refugee children ≤ 18 years old presenting to the ED between January 2013 and July 2019 were retrospectively reviewed and compared with resident children.The study population consisted of 7299 refugees and 690,127 resident children admitted to the ED. High-acuity cases were more frequent in Syrian refugees (2.2% vs 1% p < 0.001). One-third of Syrian children were under 12 months of age (31% vs 17%, p < 0.001). Syrian children were more commonly hospitalized (7.9% vs 3.1% p < 0.001). The median age (and interquartile range - IQR) was lower in hospitalized refugee than in resident children [12 (0-83) months vs 41 (8-111) months, p < 0.001]. Rate of intensive care unit hospitalization (13% vs 9.4%, p = 0.001) and neonatal hospitalization was higher in Syrians compared to resident children (29% vs 12%, p < 0.001). The median NICU stay was longer in refugees [6 (IQR 4-17) days vs 3 (IQR 1-9) days, p < 0.001]. CONCLUSION: Refugee children, as compared to resident children, are more likely to present to the ED with high acuity conditions and at a younger age resulting in higher rates of inpatient admissions. Strategies to increase access to preventive health care services for young refugee children should be explored to decrease ED and hospital services and improve health outcomes. WHAT IS KNOWN: • Children are the most affected victims of armed conflicts in terms of health outcomes. • Refugees prefer to access healthcare through the emergency department. WHAT IS NEW: • Refugee children were more likely to present as urgent when compared to resident children. • Admission to neonatal and intensive care units was more frequent among refugee than resident children.

The differences in perinatal outcomes between refugee and resident mother-infant dyads: A retrospective study in Turkey between 2013 and 2018.

In this study, we aimed to examine and compare the perinatal outcomes between refugee and resident mother-infant dyads. Data of refugee women who had given birth in a university hospital (n = 924) and matched resident mother-infant dyads (n = 957) were included. Analysis revealed higher adolescent pregnancy rates and lower rates of antenatal care attendance among refugee mothers compared to residents. No significant differences in neonatal outcomes were found, except for a significantly higher number of preterm births among refugee infants. Interventions should be made to ensure antenatal care for all pregnant women, which can also prevent preterm birth.

Immunization practices in children with a history of allergies.

AIM: In the presence of food allergies, especially egg allergies, primary physicians in Turkey avoid vaccine administration and refer children to a hospital setting. We aimed to evaluate children who had allergies or suspected allergies and were referred to our Well Child Clinic in a university hospital for vaccination. MATERIAL AND METHODS: Charts of all children referred to our clinic due to concerns for allergies in the last two years, were reviewed. Demographic data, laboratory evaluation and reactions after immunization were recorded. RESULTS: A total of 122 children with or without a confirmed diagnosis of allergies were referred by primary physicians. In the history, 50 children (43.5%) had reactions with egg, 42 (36.5%) had reactions with multiple foods, nine (7.8%) had reactions with milk and seven (6.1%) had reactions with a previous vaccination. The most common reaction was rash (n=89, 86.4%). Nine children reported anaphylaxis. Skin testing or serum allergen specific IgE measurement revealed that 66 (54.1%) children had sensitization to egg white and 25 (20.5%) had sensitization to egg yolk. Most children (n=87, 71.9%) were referred for all the 12th-month vaccines, and 21 children were referred only for the measles-mumps-rubella vaccine (n=21, 17.4%). The median delay time in the administration of the measles-mumps-rubella vaccine was 20.0 (interquartile range: 8.7-41.2) days. No reaction was observed except for one child reporting a slight rash several hours after vaccination. CONCLUSION: Egg allergy was the most common barrier of vaccine administration in children referred from family physicians. Given the absence of any reactions, we support the administration of the measles-mumps-rubella vaccine in primary care settings to prevent delays in national vaccine schedule.

Diagnostic Modalities Based on Flow Cytometry for Chronic Granulomatous Disease: A Multicenter Study in a Well-Defined Cohort.

BACKGROUND: Chronic granulomatous disease (CGD) is characterized by defective microbial killing due to mutations affecting subunits of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. Definitive genetic identification of disease subtypes may be delayed or not readily available. OBJECTIVE: Sought to investigate the role of intracellular staining of NADPH oxidase enzyme subunits in predicting the respective genetic defects in patients with CGD and carriers. METHODS: Thirty-four patients with genetically inherited CGD, including 12 patients with X-linked CGD (gp91phagocyte oxidase (phox) deficiency due to cytochrome b-245, beta polypeptide [CYBB] mutations) and 22 patients with autosomal-recessive CGD (p22phox, p47phox, and p67phox deficiency due to cytochrome b-245, alpha polypeptide [CYBA], neutrophil cytosolic factor 1 [NCF1] and NCF2 mutations, respectively) were recruited from different immunology centers and followed up prospectively. Dihydrorhodamine testing and NADPH oxidase subunit expression in white blood cells were determined by flow cytometry. RESULTS: gp91phox and p22phox defects, which result in simultaneous loss of both proteins due to their complex formation, were differentiated only by comparative analysis of patients' and mothers' intracellular staining. p47phox and p67phox protein expression was almost undetectable in patients compared with carrier mothers and healthy controls. The expression values of the respective subunits were found to be significantly higher in all controls as compared with carrier mothers, which in turn were higher than those of patients. CONCLUSIONS: Analysis of NADPH oxidase enzyme subunits by flow cytometry in patients and carriers is useful in the rapid prediction of the genetic defect of patients with CGD, thus guiding targeted sequencing and aiding in their early diagnosis.

The impact of vitamin B12 deficiency on infant gut microbiota.

Although physiologic and neurologic consequences of micronutrient deficiencies have been addressed extensively, less is known about their impact on developing gut microbiota. Vitamin B12 deficiency is a common micronutrient deficiency in infants. We aimed to analyze the gut microbial composition of exclusively breastfed infants aged between 4 and 6 months with and without vitamin B12 deficiency by 16S rRNA gene sequencing. In a subgroup of infants with vitamin B12 deficiency, stool samples are recollected and reanalyzed after vitamin B12 supplementation. A total of 88 infants' stool samples (median age 4 months [IQR 4-5], 50% males) were analyzed, of which 28 (31.8%) were vitamin B12 sufficient and 60 (68.2%) were vitamin B12 insufficient. Comparisons between vitamin B12-sufficient and vitamin B12-insufficient infants revealed no evidence of differences in the microbiota. Proteobacteria, Firmicutes, Actinobacteria, and Bacteroidetes were the most abundant phyla in all groups. There was no difference between the pre- and post-treatment composition of gut microbiota.Conclusion: Vitamin B12-deficient infants have similar gut microbial composition as vitamin B12-sufficient infants. Since the samples were collected at an early period of life and the exposure to deficiency was relatively short, it may be possible that the effects were not fully established.What is Known: • Vitamin B12 is an essential vitamin for humans and also a crucial compound for human gut microbiota. • Vitamin B12 deficiency is common in exclusively breastfed infants. • In contrast to the adult gut microbiota, infant gut microbiota has been shown to have decreased capacity for de novo synthesis of vitamin B12 and depend on dietary source of vitamin B12.What is New: • There is no difference in the gut microbial composition of vitamin B12-deficient and vitamin B12-sufficient infants.

Impact of biologics on disease course in systemic onset juvenile idiopathic arthritis.

To analyze our cohort of patients with systemic onset juvenile idiopathic arthritis (SoJIA) and investigate the impact of biologic disease-modifying antirheumatic drugs (BDMARDs) on disease course. Children who were diagnosed with SoJIA according to International League of Associations for Rheumatology (ILAR) criteria in Boston Children's Hospital between January 1996 and December 2015 were included. Data were collected from patients' charts retrospectively. Demographic features, disease course, and medication usage were identified. There were 76 patients who met ILAR criteria. Most common presenting features were fever (100%), arthralgia (92%), rash (87%), and arthritis (83%). Median follow-up was 69 months. At last visit, 18% still had active disease. Disease course was monophasic in 18 patients (24%), persistent in 24 patients (32%), and polycyclic in 34 patients (45%). Thirty-three percent (n, 6) of children with monophasic disease was diagnosed before 2004 and 67% (n, 12) was diagnosed after 2004 (p = 0.08). Sixty-six percent was treated with a BDMARD. Anakinra (37%) was the most common prescribed BDMARD. Monophasic disease was less common in patients treated with a BDMARD (n, 6, 12%) compared to children not treated with a BDMARD (n, 12, 46%) (p = 0.01). BDMARDs are started earlier (rs, - 0.67; p < 0.001) and diagnosis of SoJIA is made sooner after symptom onset in recent years (rs, - 0.37; p = 0.001). Most patients in our cohort were able to achieve remission. Proportion of monophasic disease tends to increase after 2004 although not statistically significant. In recent years, physicians tend to diagnose SoJIA earlier and treat more aggressively early in the course of the disease with BMARDs. Future prospective research in larger cohorts investigating the effects of BDMARDs on disease course and predictive factors for outcome is needed.

Clinical and polysomnographic features of children evaluated with polysomnography in pediatric sleep laboratory.

AIM: Sleep disordered breathing is a common problem in childhood that encompasses a spectrum of disorders extending from primary snoring to obstructive sleep apnea. This study aims to investigate the results of children undergoing evaluation with polysomnography in the sleep laboratory of a tertiary care hospital. MATERIAL AND METHODS: Demographic and clinical features as well as sleep associated symptoms, scores of pediatric sleep questionnaire and Pittsburgh sleep quality index and polysomnography results are retrospectively evaluated. RESULTS: Totally 131 patients were evaluated, of which 47.3% (n=62) were females and 52.7% (n=69) were males. Mean age was 101.85±59.15 months at the time of the study. Fifty percent (n=59) of patients complained of snoring and 43.7% (n=52) of patients complained of apnea during sleep. Mean obstructive hypopnea-apnea index was 5.12±11.72. Mean obstructive hypopnea-apnea index of snorers (6.93±13.53) was significantly higher than the mean obstructive hypopnea-apnea index of nonsnorers (2.32±5.43) (p=0.011). Mean obstructive hypopnea-apnea index of patients experiencing apnea during sleep (7.52±14.25) was significantly higher than the mean obstructive hypopnea-apnea index of the children who do not experience apnea (2.61±5.84) (p=0.008). No significant correlation was observed between obstructive hypopnea-apnea index and scores of pediatric sleep questionnaire and Pittsburgh sleep quality index. The prevalence of obstructive sleep apnea was 33.6% (n=44). Forty nine patients (39.8%) were treated after polysomnography. Frequently suggested treatment options were noninvasive mechanical ventilation (n=23, 46.9%), intranasal steroid (n=15, 30.6%), montelukast (n=11, 22.4%) and adenotonsillectomy (n=9, 18.4%). CONCLUSIONS: Polysomnography is the gold standard in the diagnosis of sleep disordered breathing in children. Pediatricians should be able to recognize early signs and symptoms of sleep disordered breathing and refer the patients in risk to centers where evaluation with polysomnography is available.

The effect of systemic corticosteroids on the innate and adaptive immune system in children with steroid responsive nephrotic syndrome.

UNLABELLED: The severity and duration of immunosuppression caused by corticosteroids (CSs) usage have not been extensively studied. We aimed to investigate the effects of CSs on the various compartments of immune system in relation to timing of initiation and persistence of therapy. Pediatric patients with idiopathic nephrotic syndrome (NS) treated with 2 mg/kg/day prednisolone and healthy control (HC) were enrolled. Blood samples were drawn for immunologic analyses at baseline and at the first and second weeks and first, second, and third months of CS therapy in addition to first and second weeks and first, second, and third months of discontinuation. Fourteen patients (M/F, 7/7) between 1 and 8 years old were evaluated. Untreated NS exhibited high absolute lymphocyte count (ALC)(p = 0.010), absolute CD3(+) T cells (p = 0.020) and absolute CD8(+) T cells (p = 0.006) compared to HC. Suppression in ALC was observed and nadir value was noted at first month of therapy compared to baseline (p = 0.002). The CD4(+) (p = 0.036) and CD8(+) T cell (p = 0.013) counts decreased significantly at the first week of treatment compared to baseline. While baseline B cell counts was indifferent from HC, gradually increased in 2 weeks of CS initiation and decreased during the treatment with a statistical significance compared to HC (p = 0.010). However, after cessation of CS, B cell counts continued to decline and found to be significantly different than baseline at first week (p = 0.008) and at third month (p = 0.040). CONCLUSION: Apart from baseline lymphocyte subset changing observed in untreated NS patients, our data implies that T cells were suppressed very early in the CS treatment. Interestingly, depressed B cell counts were detected later but persisted even after CS cessation. Due to early decrease in T cells, it would be beneficial to assume the patients as immunosuppressed at the very beginning of CS treatment to avoid infections. WHAT IS KNOWN: • Corticosteroids (CSs) are widely used for a variety of diseases including nephrotic syndrome, which is related with complex immune disturbance including T and B cells dysfunctions. • CSs induce neutrophilic leukocytosis concomitant with lymphopenia and eosinopenia leading to immunosupression. What is New: • T cell subsets and proliferation are susceptible to CSs more than B cells; however, the reversibility is faster with dose reduction in CS. • The change of B cells and B cell subtypes (CD27 (+) memory) shows prolonged effect of CSs on B cells which may alter antibody production even after 3 months of CSs cessation.

The plethora, clinical manifestations and treatment options of autoimmunity in patients with primary immunodeficiency.

AIM: Although the association between primary immunodeficiency and autoimmunity is already well-known, it has once again become a topic of debate with the discovery of newly-defined immunodeficiencies. Thus, investigation of the mechanisms of development of autoimmunity in primary immunodefficiency and new target-specific therapeutic options has come to the fore. In this study, we aimed to examine the clinical findings of autoimmunity, autoimmunity varieties, and treatment responses in patients who were genetically diagnosed as having primary immunodeficiency. MATERIAL AND METHODS: The files of patients with primary immunodeficiency who had clinical findings of autoimmunity, who were diagnosed genetically, and followed up in our clinic were investigated. The demographic and clinical features of the patients and their medical treatments were evaluated. RESULTS: Findings of autoimmunity were found in 30 patients whose genetic mutations were identified. The mean age at the time of the first symptoms was 8.96±14.64 months, and the mean age of receiving a genetic diagnosis was 82.55±84.71 months. The most common diseases showing findings of autoimmunity included immune dysregulation, polyendocrinopathy, enteropathy X-linked syndrome (16.7%); autoimmune lymphoproliferative syndrome (10%); lipopolysaccharide-responsive beige-like anchor protein deficiency (10%); and DiGeorge syndrome (10%). Twelve (40%) patients showed findings of autoimmunity at the time of first presentation. The most common findings of autoimmunity included inflammatory bowel disease, inflammatory bowel disease-like findings (n=14, 46.7%), immune thrombocytopenic purpura (n=11, 36.7%), and autoimmune hemolytic anemia (n=9, 30.0%). A response to immunosupressive agents was observed in 15 (50%) patients. Ten patients underwent hematopoietic stem cell transplantation. Six patients were lost to follow-up due to a variety of complications. CONCLUSION: Autoimmunity is frequently observed in patients with primary immunodeficiency. The possibility of primary immunodeficiency should be considered in patients with early-onset manifestations of autoimmunity, and these patients should be carefully monitored in terms of immunodeficiency development. Early diagnosis of primary immunodeficiency may provide favorable outcomes in terms of survival.