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1.
Respir Med ; : 107654, 2024 May 10.
Artigo em Inglês | MEDLINE | ID: mdl-38735372

RESUMO

BACKGROUND: Quality of life and survival in Cystic Fibrosis (CF) have improved dramatically, making family planning a feasible option. Maternal and perinatal outcomes in women with CF (wwCF) are similar to those seen in the general population. However, the effect of undergoing multiple pregnancies is unknown. METHODS: A multinational-multicenter retrospective cohort study. Data was obtained from 18 centers worldwide, anonymously, on wwCF 18-45 years old, including isease severity and outcome, as well as obstetric and newborn complications. Data was analyzed, within each individual patient to compare the outcomes of an initial pregnancy (1st or 2nd) with a multigravid pregnancy (≥3) as well as secondary analysis of grouped data to identify risk factors for disease progression or adverse neonatal outcomes. Three time periods were assessed - before, during, and after pregnancy. RESULTS: The study population included 141 wwCF of whom 41 (29%) had ≥3 pregnancies, "multiparous". Data was collected on 246 pregnancies, between 1973 and 2020, 69 (28%) were multiparous. A greater decline in ppFEV1 was seen with multiparous women, primarily in pancreatic insufficient (PI) wwCF and those with two severe (class I-III) mutations. Multigravid pregnancies were shorter, especially in wwCF over 30 years old, who had high rates of prematurity and newborn complications. There was no effect on pulmonary exacerbations or disease-related complications. CONCLUSIONS: Multiple pregnancies in wwCF are associated with accelerated respiratory deterioration and higher rates of preterm births. Therefore, strict follow-up by a multidisciplinary CF and obstetric team is needed in women who desire to carry multiple pregnancies.

2.
Acta Derm Venereol ; 104: adv23932, 2024 Apr 04.
Artigo em Inglês | MEDLINE | ID: mdl-38576090

RESUMO

Chronic spontaneous urticaria (CSU) is a disturbing skin condition often severely detrimental to quality of life. Haematological markers of inflammation such as neutrophil-to-lymphocyte and platelet-to-lymphocyte may be used in the assessment of inflammatory skin diseases. Their usefulness in urticaria is unknown. Neutrophil- to-lymphocyte, platelet-to-lymphocyte, and total serum IgE were investigated in urticaria patients: acute spontaneous urticaria (ASU) versus CSU, children versus adults with CSU, and patients with mild-to-moderate versus severe CSU. This retrospective cohort study included patients of all ages diagnosed with urticaria between 2005 and 2020 and blood counts within 30 days of diagnosis. Patients with comorbidities influencing blood cells (infection, surgery, malignancy) were excluded. Neutrophil-to-lymphocyte and platelet-to-lymphocyte were evaluated in patients with ASU vs CSU and mild-to-moderate CSU vs severe CSU (defined by the use of systemic medications or hospitalizations). A total of 13,541 urticaria patients were included in the study. CSU patients (n = 5,021) had higher neutrophil-to-lymphocyte and platelet-to-lymphocyte, as well as serum IgE levels compared with ASU patients (n = 8,520). Adults had higher neutrophil-to-lymphocyte and platelet-to-lymphocyte than children. Severely affected patients (n = 53) had higher neutrophil-to-lymphocyte and platelet-to-lymphocyte compared with mild-to-moderately affected patients (n = 4,968). Patients with higher neutrophil-to-lymphocyte and platelet-to-lymphocyte had higher odds of having CSU rather than ASU and severe urticaria rather mild-to-moderate. In conclusion, neutrophil-to-lymphocyte and platelet-to-lymphocyte are simple and available markers that can be used to predict and assess severe and chronic urticaria.


Assuntos
Urticária Crônica , Transtornos Leucocíticos , Urticária , Adulto , Criança , Humanos , Estudos Retrospectivos , Neutrófilos , Qualidade de Vida , Doença Crônica , Urticária/tratamento farmacológico , Urticária Crônica/diagnóstico , Linfócitos , Imunoglobulina E
3.
Eur J Pediatr ; 2024 Apr 29.
Artigo em Inglês | MEDLINE | ID: mdl-38679661

RESUMO

Though PCD usually presents after birth in term neonates, diagnosing PCD during the neonatal and infancy stages is uncommon, particularly in children who do not exhibit laterality defects. We report our recent experience with the diagnosis of PCD in the neonatal and early infantile period in a highly consanguine population. This was achieved by implementing a novel genetic-based diagnostic approach based on direct testing for recognized regional genetic variants. We conducted a retrospective analysis of children diagnosed with PCD at Soroka University Medical Center during the neonatal or early infantile period between 2020 and 2023. We included children under 3 months of age who had a genetic confirmation of PCD, as evidenced by the presence of two pathogenic variants in recognized genes. Genetic testing targeted regional genetic variants in previously identified PCD genes. Eight patients were included. The median age at diagnosis was 12.5 days. Three (38%) were born prematurely < 34 weeks gestational age. All patients were presented with respiratory distress and hypoxemia after birth. The median duration of oxygen support was 23 days, and upper lobe atelectasis was present in five patients (63%). Congenital cardiac malformation was present in four patients. Organ laterality defects were present in four patients. Genetic mutations identified were in the DNAAF5, DNAL1, DNAAF3, and DNAH1 genes.     Conclusion: Neonatal diagnosis of PCD is uncommon, especially in atypical presentations such as children without laterality defects or preterms. Focusing on a genetic diagnosis of the local tribal pathogenic variants promotes a potential cost-efficient test leading to earlier diagnosis. There is a need for a standardized protocol for earlier diagnosis of PCD in high-consanguinity areas. What is Known: • Primary ciliary dyskinesia (PCD) typically presents after birth in term neonates. • Diagnosing PCD during neonatal and infancy stages is challenging, particularly in children without laterality defects. What is New: • A novel genetic-based diagnostic approach was implemented on the neonatal population in a highly consanguine community, focusing on direct testing for regional genetic variants, leading to early and rapid diagnosis of PCD.

4.
Eur J Pediatr ; 2024 Apr 13.
Artigo em Inglês | MEDLINE | ID: mdl-38613577

RESUMO

Thermal epiglottitis, a non-infectious cause of epiglottitis, is a rare entity that shares some clinical features with infectious epiglottitis. This study presents 16 years of experience in diagnosing and managing thermal epiglottitis. A retrospective descriptive study in a tertiary center in southern Israel included confirmed cases of thermal epiglottitis in children (0-18 years) between 2004 and 2020 by endoscopy. Of approximately 600,000 pediatric ER admissions between 2004 and 2020, seven children were diagnosed by endoscopy with thermal epiglottitis (mean age 24 months, 71% males). Clinical presentation included stridor, respiratory distress, and drooling. Four children had fever and elevated inflammatory markers at presentation and were treated with systemic antibiotics. All were treated with systemic steroids. The median length of stay in the PICU was five days, and four patients required intubations. All fully recovered without experiencing any sequelae.  Conclusion: Thermal epiglottitis stands as a potential contributor to acute upper airway obstruction. Although it's rarity, it should be discussed in any child with acute upper airway obstruction. It is essential to inquire directly about the accidental intake of hot beverages, particularly in cases lacking fever or elevated inflammatory markers. What is Known: • Thermal epiglottitis is a rare, non-infectious condition sharing clinical features with infectious epiglottitis. • Common presentations include stridor, respiratory distress, and drooling. What is New: • Thermal epiglottitis is a potential contributor to acute upper airway obstruction, urging consideration even in the absence of fever or elevated markers. • Direct inquiry about hot beverage intake for diagnosis is essential for diagnosis.

5.
Eur J Pediatr ; 2024 Mar 20.
Artigo em Inglês | MEDLINE | ID: mdl-38507064

RESUMO

Acute bronchiolitis is among the most common causes of hospitalizations in infants worldwide. Associations between weight and severity of respiratory syncytial virus (RSV) bronchiolitis remain unclear. The aim of this study was to evaluate this association. A single-center, retrospective cohort study of infants aged under 24 months, who were hospitalized between 2018 and 2022 for RSV bronchiolitis. Data from computerized medical records were extracted using the MDclone platform. Participants were divided into three groups according to weight percentiles: underweight (below 5th percentile), normal-weight, and overweight (above 85th percentile). A total of 1936 infants (mean age 6.3 months, 55% males) were included, comprising 274 infants who were underweight, 1470 with normal weight, and 192 with overweight. Underweight infants had a higher rate of admission to the pediatric intensive care unit (PICU) (9.1% vs. 3.5%, P < 0.005) and prolonged length of stay (LOS) in the hospital (3.13 vs. 2.79 days P < 0.001) compared to those with normal weight. Hyponatremia was also more common in the underweight group (23% vs. 15%, P < 0.001). A multivariable model accounting for prematurity and birthweight predicted a relative risk of 2.01 (95% CI 1.13-3.48, P = 0.015) for PICU admission and 1.42 (95% CI 1.17-1.7, P < 0.001) for a prolonged LOS. Being overweight was not associated with a more severe disease.  Conclusion: Underweight infants, hospitalized for RSV bronchiolitis, had a more severe disease course with a higher complication rate, including PICU admission and prolonged LOS. Thus, careful attention and supervision should be given to this subgroup of infants. What is Known: • Established risk factors for severe bronchiolitis include prematurity, BPD, CHD, and compromised immunity. • Abnormal weight status has been associated with an increased risk for morbidity and mortality from infectious diseases, proposedly due to the effects on endocrine and immunologic systems. What is New: • Underweight infants hospitalized with RSV bronchiolitis face an independent risk of PICU admission and prolonged hospital stay. • Conversely, overweight infants did not display associations with severity measures in our study.

6.
Int J Dermatol ; 2024 Mar 08.
Artigo em Inglês | MEDLINE | ID: mdl-38459652

RESUMO

BACKGROUND: Psoriasis is a common skin disorder linked to systemic inflammation and immune dysregulation. It is believed to involve activated T cells and neutrophils. Recent research has highlighted the potential role of hematological ratios, such as neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), eosinophil-to-lymphocyte ratio (ELR), eosinophil-to-neutrophil ratio (ENR), and eosinophil-to-monocyte ratio (EMR), as markers for inflammatory skin diseases, including psoriasis. OBJECTIVES: We aimed to investigate hematological ratios between children and adults, patients and controls, and patients with moderate-to-severe and mild psoriasis. MATERIALS AND METHODS: This national retrospective cohort study included over 16,000 psoriasis patients in Israel. Patients with comorbidities influencing blood counts were excluded. Ratios were calculated from blood counts taken within 30 days of diagnosis. Multivariable logistic regression, including age, gender, ethnicity, smoking status, and socioeconomic status, was performed. RESULTS: Findings revealed age-specific variations in blood counts, hematological ratios, and differences between mild and moderate-severe patients and patients versus controls. Moderate-severe psoriasis patients had elevated neutrophil and eosinophil counts (4.57 vs. 4.25, P < 0.001, and 0.24 vs. 0.22, P = 0.047, respectively), as well as increased NLR (2.46 vs. 2.29, P < 0.001). Multivariable logistic regression analysis confirmed the significance of neutrophil and platelet counts as well as NLR and PLR in predicting psoriasis severity. LIMITATIONS: This was a retrospective study without subjective data on disease severity. CONCLUSION: This study highlights hematologic ratios' diagnostic and prognostic potential in psoriasis.

7.
PLoS One ; 19(2): e0296626, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38346060

RESUMO

BACKGROUND: Hematological markers such as eosinophil-to-lymphocyte (ELR), eosinophil-to-neutrophil (ENR), and eosinophil-to-monocyte (EMR) ratios may be used in the assessment of skin diseases. However, the relationship with vitiligo remains unclear. OBJECTIVES: We investigated ELR, ENR, and EMR in vitiligo patients and the association with severe vitiligo. METHODS: This study included patients of all ages diagnosed with vitiligo between 2005-2020. ELR, EMR, and ENR were calculated from complete blood counts within 30 days of the first recorded vitiligo diagnosis and 12-18 months before the vitiligo diagnosis. We evaluated the associations between ELR, ENR, and EMR and vitiligo. RESULTS: 9,826 vitiligo patients (8,398 adults and 1,428 children) and 8,951 age and sex-matched controls were included in this study. We found that ELR, EMR, and ENR were higher in children than in adults. Patients with vitiligo had higher ENR and EMR than their matched controls. Patients with severe vitiligo had higher ELR, ENR, and EMR compared to patients with mild vitiligo. Patients with increased ENR and EMR 12-18 months before the vitiligo diagnosis had a higher chance of having severe vitiligo (ENR: OR = 7.40, p<0.001, EMR: OR = 3.17, p<0.001). CONCLUSION: ELR, ENR, and EMR may be used in the assessment and prognosis of vitiligo, especially in severe cases.


Assuntos
Eosinófilos , Vitiligo , Adulto , Criança , Humanos , Neutrófilos , Monócitos , Estudos Retrospectivos , Linfócitos
8.
Arch Dis Child ; 109(5): 428-431, 2024 Apr 18.
Artigo em Inglês | MEDLINE | ID: mdl-38296613

RESUMO

BACKGROUND: The American Thoracic Society guidelines for the diagnosis of primary ciliary dyskinesia (PCD) consider the presence of a bi-allelic pathogenic variant confirmatory for the diagnosis of PCD, with genetic testing recommended when other confirmatory diagnostic tests are less accessible. We present our experience with genetic testing as first line with a proposed algorithm for high consanguinity populations. METHODS: Patients with a suspected diagnosis of PCD underwent genetic testing according to a diagnostic algorithm composed of three steps: (1) patients with a previously known causative familial/Bedouin tribal pathogenic variant completed direct testing for a single variant; (2) if the initial test was negative or there was no known pathogenic variant, a PCD genetic panel was completed; (3) if the panel was negative, whole exome sequencing (WES) was completed. RESULTS: Since the implementation of the protocol, diagnosis was confirmed by genetic testing in 21 patients. The majority of them were of Bedouin origin (81%) and had a positive history of consanguinity (65%). Nine patients (43%) had a sibling with a confirmed diagnosis. Most patients (15/21, 71%) were diagnosed by direct pathogenic variant testing and the remainder by genetic panel (19%) and WES (10%). Disease-causing variants were found in nine genes, with DNAL1 (24%) and DNAAF3, DNAAF5, ZMYND10 (14% each) as the most prevalent ones. CONCLUSIONS: In highly consanguineous regions, a stepwise genetic testing approach is recommended. This approach may be particularly useful in areas where the ability to obtain confirmatory diagnostic tests through other modalities is less accessible.


Assuntos
Transtornos da Motilidade Ciliar , Testes Genéticos , Humanos , Consanguinidade , Transtornos da Motilidade Ciliar/diagnóstico , Transtornos da Motilidade Ciliar/genética , Mutação
9.
J Cyst Fibros ; 23(1): 41-49, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37173154

RESUMO

BACKGROUND: The prevalence of nontuberculous mycobacteria (NTM) infections is rising in people with cystic fibrosis (pwCF). NTM infection, especially infection with Mycobacterium abscessus complex (MABC), is commonly associated with severe lung deterioration. The current treatment modalities, including multiple intravenous antibiotics, frequently fail to achieve airway eradication. Although treatment with elexacaftor/tezacaftor/ivacaftor (ETI) has been shown to modulate the lung microbiome, data regarding its role in eradicating NTM in pwCF is lacking. Our aim was to evaluate the impact of ETI on the rate of NTM eradication in pwCF. METHODS: This retrospective multicenter cohort study included pwCF from five CF centers in Israel. PwCF aged older than 6 who had at least one positive NTM airway culture in the past two years and were treated with ETI for at least one year were included. The annual NTM and bacterial isolations, pulmonary function tests, and body mass index were analyzed before and after ETI treatment. RESULTS: Fifteen pwCF were included (median age 20.9 years, 73.3% females, 80% pancreatic insufficient). In nine patients (66%) NTM isolations were eradicated following treatment with ETI. Seven of them had MABC. The median time between the first NTM isolation and treatment with ETI was 2.71 years (0.27-10.35 years). Eradication of NTM was associated with improved pulmonary function tests (p<0.05). CONCLUSIONS: For the first time, we report successful eradication of NTM, including MABC, following treatment with ETI in pwCF. Additional studies are needed to assess whether treatment with ETI can result in the long-term eradication of NTM.


Assuntos
Aminofenóis , Benzodioxóis , Fibrose Cística , Indóis , Infecções por Mycobacterium não Tuberculosas , Mycobacterium abscessus , Pirazóis , Piridinas , Pirrolidinas , Quinolonas , Feminino , Humanos , Idoso , Adulto Jovem , Adulto , Masculino , Micobactérias não Tuberculosas , Fibrose Cística/complicações , Fibrose Cística/tratamento farmacológico , Fibrose Cística/epidemiologia , Estudos de Coortes , Infecções por Mycobacterium não Tuberculosas/diagnóstico , Infecções por Mycobacterium não Tuberculosas/tratamento farmacológico , Infecções por Mycobacterium não Tuberculosas/epidemiologia , Regulador de Condutância Transmembrana em Fibrose Cística
10.
Eur J Pediatr ; 183(2): 815-825, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38017338

RESUMO

Foreign body aspiration (FBA) is a common cause of pediatric morbidity, but a standardized protocol to guide decision-making about bronchoscopy is lacking. We aimed to validate a new Foreign body aspiration score (FOBAS) for the pediatric emergency department (ED). Patients aged 0-18 years referred to the ED for suspected FBA were prospectively enrolled. FOBAS was calculated according to clinical features of a choking episode, sudden cough, exposure to nuts, absence of fever and rhinitis, stridor, and unilateral auscultatory and radiological findings. FBA risk was evaluated based on the total score (low, 1-3; moderate, 4-6; high, 7-10). Low-risk children were discharged from the ED and followed clinically. Moderate-risk children were hospitalized and evaluated by a pediatric pulmonologist, and high-risk children were referred directly for therapeutic bronchoscopy. Among the 100 enrolled children (59% males; median age 20 [interquartile range 11-39] months), a foreign body was diagnosed in 1/49 (2%), 14/41 (34.1%), and 9/10 (90%) with low, moderate, and high FOBAS, respectively (P < .001). Logistic regression indicated a higher risk for FBA with higher scores. The odds ratio for each additional point was 2.75 (95% confidence interval 1.78-4.24), and FOBAS showed a high predictive value for FBA (area under the curve 0.89). FOBAS implementation significantly reduced the rate of negative bronchoscopies, from 67.4% annually during 2016-2019 to 50% in 2020 (P = .042). CONCLUSION: FOBAS reliably predicts FBA in cases of suspected FBA and improves management and in-hospital decision-making. WHAT IS KNOWN: • Foreign body aspiration is a major cause of pediatric morbidity and mortality. • Currently, there is no unified protocol for children referred to the emergency department for suspected FBA, therefore, a well-defined algorithm is needed to improve the decision-making process. WHAT IS NEW: • The pediatric Foreign body aspiration score (FOBAS) is a new, prospectively validated clinical score that shows high sensitivity and specificity for the presence of FBA in children. • FOBAS reduces unnecessary admissions and invasive procedures and leads to better clinical outcomes.


Assuntos
Obstrução das Vias Respiratórias , Corpos Estranhos , Masculino , Criança , Humanos , Lactente , Pré-Escolar , Feminino , Estudos Retrospectivos , Aspiração Respiratória/diagnóstico , Aspiração Respiratória/etiologia , Aspiração Respiratória/terapia , Broncoscopia/efeitos adversos , Broncoscopia/métodos , Obstrução das Vias Respiratórias/diagnóstico , Obstrução das Vias Respiratórias/etiologia , Obstrução das Vias Respiratórias/terapia , Algoritmos , Corpos Estranhos/diagnóstico , Corpos Estranhos/terapia , Corpos Estranhos/complicações
11.
Pediatr Pulmonol ; 59(3): 707-714, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38131521

RESUMO

BACKGROUND: Lung maldevelopment due to in-utero events may potentially cause respiratory morbidity during childhood. Maternal nutritional status during pregnancy is critical for lung development. This study is contributing to the understanding of the interplay between maternal nutrition status during pregnancy, fetal lung development and the risk for respiratory diseases in early life. RESEARCH QUESTION: To investigate the association between maternal hyperemesis gravidarum (HG) during pregnancy and respiratory morbidity in the offspring's early childhood. STUDY DESIGN AND METHODS: This is a retrospective population-based cohort study that included all singleton term deliveries at Soroka University Medical Center (SUMC) between 1991 and 2021. Preterm deliveries (<37 gestational week), perinatal deaths, multiple gestations, and children with congenital malformations or chromosomal abnormalities were excluded. The main outcomes measured were offspring's hospitalizations due to pneumonia, acute bronchiolitis, asthma, or wheezing. RESULTS: Overall 232,476 deliveries were included in the study, of which 3227 women (1.4%) were diagnosed with HG. Offspring in the HG group exhibited significantly higher rates of respiratory morbidity, including asthma (OR = 1.36, 95% CI 1.22-1.36, p < .001), acute bronchiolitis (OR = 1.38, 95% CI 1.21-1.59, p < .001), and pneumonia (OR = 1.2, 95% CI 1.12-1.48, p < .001). An inverse correlation between multivariate adjusted-hazard ratios for asthma and pneumonia with offspring's age was noted. INTERPRETATION: This study provides evidence of a potential association between maternal HG during pregnancy and increased risk of respiratory morbidity in offspring's early childhood. Maternal nutritional status during pregnancy plays a crucial role in lung development, affecting respiratory health in childhood.


Assuntos
Asma , Bronquiolite , Hiperêmese Gravídica , Pneumonia , Gravidez , Recém-Nascido , Criança , Humanos , Pré-Escolar , Feminino , Hiperêmese Gravídica/complicações , Hiperêmese Gravídica/epidemiologia , Estudos Retrospectivos , Estudos de Coortes , Asma/epidemiologia , Morbidade
12.
Transl Pediatr ; 12(10): 1823-1834, 2023 Oct 30.
Artigo em Inglês | MEDLINE | ID: mdl-37969117

RESUMO

Background: Atopic dermatitis (AD) is a chronic dermatological condition, often diagnosed and managed by pediatricians. However, pediatricians have difficulties with adhering to guidelines, which recommend the use of topical corticosteroids (TCS) as a first-line treatment and oral corticosteroids (OCS) for resistant cases. Our aim was to assess pediatricians' self-confidence in using steroids in the management of pediatric AD, and investigate which characteristics are related to high self-confidence in prescribing corticosteroids (CS). Methods: We conducted a cross-sectional questionnaire study among Israeli pediatricians between April 2022 and June 2022. Participants were asked to answer questions dealing with self-assessment of prescribing CS in the management of AD. Results: A total of 171 residents and pediatricians participated in the survey; 86.6% and 28.1% admitted feeling either average or below-average confidence in the prescription of OCS and TCS, respectively. Physicians who were exposed to higher AD patients (P=0.048) and worked at the clinics (88.2% vs. 60.4%, P<0.001) had high self-confidence in treating AD with TCS. Males (20.3% vs. 8%, P=0.03), and having gone to medical school outside Israel (22.2% vs. 10.4%, P=0.09) were all related to high self-efficacy in prescribing OCS. In total, 11.7% of participants confessed to refraining from prescribing steroids because of fear of side effects. Conclusions: Most pediatricians have below-average confidence in prescribing OCS for the treatment of AD. Males, working in a community setting, and previous exposure improve the confidence level and can be easily considered in future pediatric training programs.

13.
J Cyst Fibros ; 2023 Nov 16.
Artigo em Inglês | MEDLINE | ID: mdl-37980178

RESUMO

BACKGROUND: Population genetic carrier screening (PGCS) for cystic fibrosis (CF) has been offered to couples in Israel since 1999 and was included in a fully subsidized national program in 2008. We evaluated the impact of PGCS on CF incidence, genetic and clinical features. METHODS: This was a retrospective national study. Demographic and clinical characteristics of children with CF born in Israel between 2008 and 2018 were obtained from the national CF registry and from patients' medical records. Data on CF births, preimplantation genetic testing (PGT), pregnancy termination and de-identified data from the PGCS program were collected. RESULTS: CF births per 100,000 live births decreased from 8.29 in 2008 to 0.54 in 2018 (IRR = 0.84, p < 0.001). The CF pregnancy termination rate did not change (IRR = 1, p=  0.9) while the CF-related PGT rate increased markedly (IRR = 1.33, p < 0.001). One hundred and two children were born with CF between 2008 and 2018 with a median age at diagnosis of 4.8 months, range 0-111 months. Unlike the generally high uptake nationally, 65/102 had not performed PGCS. Even if all had utilized PGCS, only 51 would have been detected by the existing genetic screening panel. Clinically, 34 % of children were pancreatic sufficient compared to 23 % before 2008 (p = 0.04). CONCLUSIONS: Since institution of a nationwide PGCS program, the birth of children with CF decreased markedly. Residual function variants and pancreatic sufficiency were more common. A broader genetic screening panel and increased PGCS utilization may further decrease the birth of children with CF.

14.
Front Med (Lausanne) ; 10: 1250271, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37799592

RESUMO

Background: Pediatricians daily see large numbers of patients with skin disorders. However, they encounter limited guidance as a result of a marked deficiency in pediatric dermatologists. Hence, reevaluation of training opportunities during pediatric residency has become essential. Our aim was to evaluate the confidence level of pediatric residents and specialists in diagnosing and treating skin disorders in children and to determine career and training-related characteristics that influence it. Methods: Conducted as a cross-sectional study, we administered a questionnaire to 171 pediatricians across Israel. We assessed respondents' self-efficacy about their ability to diagnose and treat skin disorders and collected data regarding their previous dermatology training and preferred training methods. Results: 77.8% of respondents reported below or average self-efficacy scores in diagnosing and managing children with skin disorders. Older age (>40 years old; OR = 5.51, p = 0.019), treating a higher number of patients with skin disorders (OR = 2.96, p = 0.032), and having any training in dermatology, either during medical school or residency (OR = 7.16, p = 0.031, OR = 11.14, p = 0.003 respectively), were all significant parameters involved in pediatricians reporting high self-efficacy in skin disorder management. Conclusion: Most pediatric residents and pediatricians have average or below-average confidence in managing pediatric skin disorders. We suggest incorporating dermatology rotations during pediatric residency to improve young pediatricians' self-efficacy in managing skin disorders and ultimately help pediatricians provide better care for patients presenting with dermatological conditions. These findings can ultimately help refine a pilot program in dermatology that might be implemented during pediatric residency.

15.
Eur J Pediatr ; 182(11): 5223-5230, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37718387

RESUMO

Atopic dermatitis (AD) is one of childhood's most common skin conditions. Although pediatricians often diagnose and manage it, more than half refer even mild cases to dermatologists. In this study, we aimed to evaluate pediatric residents' and pediatricians' self-confidence regarding their ability to diagnose and manage AD. A cross-sectional questionnaire study was conducted with Israeli pediatric residents and pediatricians in 2022. The questionnaire was designed to distinguish participants with high vs. low self-confidence characteristics regarding their ability to diagnose and treat AD. In total, 171 participants completed the questionnaire (59.4% women; age, 41.1 ± 10.6 years); 39.1% of the participants were residents, while 60.9% were board-certified pediatricians. Overall, 64.4% of the responders reported below or average confidence (score ≤ 3, on a scale of 1-5) in diagnosing and treating AD in children. The group with higher self-confidence was significantly older (44.39 vs. 39.14 years, P = 0.003), had more years of experience in evaluating pediatric AD (P = 0.004), had trained in dermatology during their residency (P = 0.02) with a longer training period (P = 0.01), and with more than three training methods (P = 0.009). Multivariable logistic regression analysis showed that high self-confidence was associated with age older than 40 years and training in dermatology during residency (odds ratios = 5.63 [P = 0.04] and 3.36 [P = 0.05], respectively).  Conclusion: Most pediatric residents and pediatricians were not particularly confident in treating children with AD. Those with high self-confidence were older, had been exposed to more patients, and had been trained in dermatology during their residency with various methods and for longer periods. Therefore, we encourage the implementation of a training program in dermatology during pediatric residency programs. What is Known: • Atopic dermatitis (AD) is one of childhood's most common skin conditions and often presents to pediatricians for diagnosis and management. • Many pediatricians refer children with even mild cases of AD to dermatologists. What is New: • Most pediatric residents and pediatricians report low confidence in diagnosing and treating pediatric AD. • Physicians with high self- confidence were older, exposed to more AD patients, and had been trained in dermatology during their residency with various methods and for longer periods. Therefore, the implementation of a training program in dermatology during pediatric residency programs is warranted.


Assuntos
Dermatite Atópica , Dermatologia , Internato e Residência , Humanos , Criança , Feminino , Adulto , Pessoa de Meia-Idade , Masculino , Israel , Autoeficácia , Dermatite Atópica/diagnóstico , Dermatite Atópica/terapia , Estudos Transversais , Pediatras , Inquéritos e Questionários
16.
Pediatr Pulmonol ; 58(12): 3530-3541, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37728225

RESUMO

OBJECTIVE: Acute bronchiolitis is the most common cause of hospitalization in young children. Data on monocyte-to-lymphocyte-ratio (MLR) and neutrophil-to-lymphocyte-ratio (NLR) as biomarkers are limited. We aim to evaluate these ratios in children hospitalized with respiratory syncytial virus (RSV) bronchiolitis and their value as biomarkers for severe clinical outcomes. STUDY DESIGN: A single-center retrospective cohort study of children aged <2 years hospitalized due to RSV bronchiolitis, between January 2018 and March 2022, with a complete blood count upon admission. We divided the cohort into quartiles based on MLR and NLR values. We examined associations between quartiles and four clinical severity outcomes. RESULTS: A total of 2038 children (median age: 4.4 months, IQR: 1.9-9.8) were included in the study. The median MLR and NLR values for quartiles 1-4 were 0.14, 0.22, 0.30, 0.47, and 0.37, 0.70, 1.16, 2.29, respectively. Children with higher MLR had higher hospitalization rates to the pediatric intensive care unit (PICU) (Q1 2.4%, Q4 9.4%, p < .001), extended hospital stays (Q1 19.4%, Q4 32%, p < .001), and lower minimal oxygen saturation (Q1 90%, Q4 87%, p < .001). Cut-off values of 0.34 for MLR and 0.67 for NLR optimally identified PICU admissions. In a model accounting for age and sex, the fourth MLR quartile had an RR of 3.4 (95% CI: 1.76-7.22) and successfully predicted PICU admissions (area under the curve = 0.73; 95% CI: 0.681-0.789). CONCLUSIONS: MLR and NLR are potential biomarkers for identifying children with RSV bronchiolitis at a higher risk for severe outcomes, specifically PICU admission.


Assuntos
Bronquiolite , Infecções por Vírus Respiratório Sincicial , Criança , Humanos , Pré-Escolar , Lactente , Neutrófilos , Monócitos , Estudos Retrospectivos , Criança Hospitalizada , Linfócitos , Biomarcadores
17.
Eur J Pediatr ; 182(9): 4205-4212, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37450025

RESUMO

Foreign Body Aspiration (FBA) is a common medical emergency among young children, but the evaluation and management of a suspected FBA case can vary across physicians and centers. We aimed to identify which clinical, laboratory, and radiological findings can predict FBA in children and to evaluate a clinical score to improve FBA prediction. This is a retrospective cohort study of patients aged 0-18 years admitted to Soroka University Medical Center between 2010 and 2020 with suspected FBA. All patients underwent flexible bronchoscopy and were divided into positive and negative FBA groups. A newly developed foreign body aspiration score (FOBAS), based on medical history, physical examination, and chest X-ray findings, was evaluated for its predictability. The study included 412 children (median age 21 months, 56.8% females), of whom 154 (37.4%) had FBA and 258 (62.6%) did not. Multivariate regression analysis showed exposure to nuts/seeds, unilateral wheezing or decreased breath sounds, stridor, and suggestive findings on chest X-ray were significant risk factors for FBA (OR [95%CI] -1.994[1.290-3.082], 1.487[1.206-1.832], 1.883 [1.011-3.509] and 2.386[1.917-2.970], respectively). However, a choking episode, acute cough, and absence of fever and rhinorrhea did not predict FBA. FOBAS showed an increased risk of FBA for each additional point of the score, with an odds ratio of 1.572 (95% CI-1.389-1.799).  Conclusion: FOBAS is a good predictor for the presence of FBA in children. Once prospectively validated, FOBAS could aid in decision-making at the emergency department, enabling more standardized care, reducing unnecessary procedures, and leading to better clinical outcomes. What is Known: • The evaluation and management of a child with suspected foreign body aspiration (FBA) vary across physicians and centers, without a consensus regarding the indications and criteria for performing bronchoscopy. • Flexible bronchoscopy is the standard procedure for the diagnosis and sometimes treatment of FBA in children, but it may hold potential complications. What is New: • We propose a newly developed foreign body aspiration score (FOBAS), based on medical history, physical examination, and chest X-ray findings, for the prediction of FBA in children at the emergency department. • The FOBAS is a good predictor of FBA in children. The score enables more standardized care and may reduce unnecessary procedures.


Assuntos
Corpos Estranhos , Feminino , Criança , Humanos , Lactente , Pré-Escolar , Masculino , Estudos Retrospectivos , Corpos Estranhos/diagnóstico , Corpos Estranhos/complicações , Broncoscopia/efeitos adversos , Broncoscopia/métodos , Radiografia , Tosse/etiologia , Sons Respiratórios/etiologia
20.
J Clin Med ; 12(9)2023 May 06.
Artigo em Inglês | MEDLINE | ID: mdl-37176752

RESUMO

Asthma is the most common chronic respiratory disease in children. The neutrophil-to-lymphocyte ratio (NLR) is a marker of a chronic inflammatory state; however, data on the association of NLR with acute asthma exacerbations in children is lacking. In this cross-sectional study, between 2016 and 2021, children aged 2-18 years who were referred to the emergency department (ED) due to asthma exacerbation, were included. NLR, calculated from complete blood count upon arrival, was assessed as a continuous variable and was classified into four groups according to quartiles. The association between severity parameters and NLR quartiles was examined. A total of 831 ED visits for asthma exacerbation were included in the study. The median NLR was 1.6, 3.8, 6.7, and 12.9 in quartiles 1-4, respectively (p < 0.001). Demographic parameters, background diseases, and chronic medications were similar between the quartiles. Higher heart rate, body temperature, systolic blood pressure, and respiratory rate were observed in the higher NLR quartiles, as well as lower oxygen saturation. Higher urgency scale and higher rates of intravenous magnesium sulfate were observed in the higher NLR quartiles, with higher admission rates and prolonged hospitalizations. In summary, NLR upon admission is associated with the severity of asthma exacerbation and higher chances of hospitalization among children in the ED.

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