Function Abnormalities In Children Research Articles

719 Elexacaftor-tezacaftor-ivacaftor therapy improves early signs of lung function abnormalities in children with cystic fibrosis aged 6 to 11

719 Elexacaftor-tezacaftor-ivacaftor therapy improves early signs of lung function abnormalities in children with cystic fibrosis aged 6 to 11

Association of lung function by spirometry with serum ferritin in transfusion-dependent thalassemia patients in a tertiary care centre

Background: β-thalassemia major is an inherited, transfusion-dependent chronic anemia which is caused by decreased production of β-globin chains required for formation of hemoglobin. Regular blood transfusion is most important factors that help in improving the survival of patients with TM; however, it leads to iron deposition in many organs such as lung. Most studied concerned about effect of iron on lung functions, revealed abnormalities, but limited data were observed. The aim of our study was to determine pulmonary function abnormalities in children with thalassemia major and assess the relation between these abnormalities and iron overload. Methods: This study had included 51 children in each group, aged between 6 and 18 years (28 males and 23 females) with TM. All included children were subjected to full clinical examination and laboratory investigations including complete blood counts and serum ferritin. Pulmonary function tests (PFTs) were assessed in all included children using spirometry. Results: 31.4% patients had restrictive lung functions. Spirometer parameters i.e., FEF25-75% (p value≤0.0001) and PEFR (%pred) (p value≤0.0001) had significant difference in both groups. FEF25-75% and PEFR had no effect of serum ferritin. Conclusions: The lung may be considered a site for organ damage, and alteration of pulmonary function may be expected in transfusion-dependent patients in spite of no pulmonary symptoms or normal chest X-ray.

The neurophysiology aspect of congenital craniofacial deformities in children

Congenital craniofacial deformities in children are conditions with varying degrees of severity between minor and severe, they can be present at birth, but they can also be detected during the child's development. Material and methods. In the period 2014-2021, 57 children (29 boys and 28 girls), average age 9,1 ± 2,4 months, with craniofacial deformities from different medical centers in the Republic of Moldova were examined polysystemically. In this sense, we evaluated the state of the masseter, temporal and tongue muscles by electromyography with surface electrodes to determine the quality of their muscle activity. Recordings of the bioelectrical activity of muscle activity during exercise were performed using a Nihon Kohden portable electromyograph (Japan). Results. The balance dysfunction of the neuromuscular system in these children, as a neuro-physiological sign of pathological muscular weakness in the sucking reflex activity, is highlighted in 87.7% of cases by a marked asymmetry (p<0.001) of the tongue and masseter muscles activity and, respectively less highlighted (p<0.05) of the anterior temporal. Discussion. Thus, the EMG pattern evaluation highlights a decrease in the average amplitude of the action potentials (mV) mainly for the tongue, mm. masseters and temporalis on the left side, associated with an increase in the potential (ms) average duration from both the affected and the contralateral muscles, thus reflecting the reduction in their contraction capacity and the quality decrease in the act of sucking and mastication, predominantly on the left, in the child with congenital craniofacial deformities and disorders of the sucking reflex in infants. Conclusions. The neurophysiological examination highlights various forms of muscle function abnormalities in children with craniofacial deformities, which require multifunctional and multidisciplinary recovery.

The effect of iron overload on pulmonary function tests in children with thalassemia major

Objective The aim was to determine pulmonary function abnormalities in children with thalassemia major (TM) and assess the relation between these abnormalities and iron overload. Introduction Regular blood transfusion program is among the most important factors that help in improving the survival of patients with TM; however, it leads to iron deposition in many organs such as lung. Most studied concerned about effect of iron on lung functions, revealed abnormalities, but limited data were observed. Patients and methods This cross-sectional study had included 50 children aged between 6 and 18 years (34 males and 16 females) with TM. All included children were subjected to full clinical examination and laboratory investigations including complete blood counts and serum ferritin. Pulmonary function tests (PFTs) were assessed in all included children using spirometry. Results Overall, 82% of the study group had abnormal pattern of PFTs. The majority of them were restrictive and of mild severity. Moreover, serum ferritin levels were found to be significantly higher in patients with thalassemia with abnormal PFTs (1456.65 ± 650.39) than patients with thalassemia with normal PFTs (744.34 ± 541.27) (P = 0.03). Conclusion Most children involved in the study had abnormal pattern of PFTs, and most frequent abnormality is restrictive pattern. In addition, there was a strong relationship between serum ferritin level and lung affection.

Secondhand Smoke Is an Important Modifiable Risk Factor in Sickle Cell Disease: A Review of the Current Literature and Areas for Future Research.

Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy that causes significant morbidity and mortality related to chronic hemolytic anemia, vaso-occlusion, and resultant end-organ damage. Tobacco smoke exposure (TSE) through secondhand smoke exposure in people with SCD of all ages and through primary smoking in adolescents and adults is associated with significantly increased morbidity, with increased rates of emergency department visits and hospitalizations for painful vaso-occlusive crises and acute chest syndrome (ACS). Secondhand smoke is also associated with pulmonary function abnormalities in children with SCD who are already at risk for pulmonary function abnormalities on the basis of SCD. TSE is emerging as one of the few modifiable risk factors of SCD. This review discusses the current state of the evidence with respect to TSE and SCD morbidity, discusses potential mechanisms, and highlights current gaps in the evidence and future research directions.

Pulmonary Function Tests in Sickle Cell Disease.

To determine pulmonary function abnormalities in children with Sickle Cell Disease (SCD) from Western India. In this cross sectional study conducted at Surat, Gujarat, India; equal number of age and gender matched children i.e., 99 in the age group of 6-18y was recruited in case (children with SCD) and control (non-SCD healthy children) groups respectively. Weight, height, body mass index (BMI) and hemoglobin (Hb) were assessed as baseline characteristics and spirometry was performed to assess the pulmonary function. The two groups of children were comparable in the baseline characteristics such as weight, height and BMI, however mean hemoglobin was significantly low in SCD as compared to healthy controls [9.1 ± 1.52 vs. 11.4 ± 1.04 (p=0.001)]. Mean (% predicted) Forced expiratory volume in 1s (FEV1) (86.79 ± 11.6 vs. 94.3 ± 16.1) and FVC (84.4 ± 11.5 vs. 91.75 ± 15.2) values were significantly low (p < 0.001) in cases. The present study revealed that the difference of pulmonary function tests between sickle cell patients and normal age matched controls were statistically significant but this difference was not clinically significant.

Abnormalities in lung volumes and airflow in children with newly diagnosed connective tissue disease.

Connective tissue diseases (CTDs) of childhood are rare inflammatory disorders, involving various organs and tissues including respiratory system. Pulmonary involvement in patients with CTDs is uncommon but may cause functional impairment. Data on prevalence and type of lung function abnormalities in children with CTDs are scarce. Thus, the aim of this study was to asses pulmonary functional status in children with newly diagnosed CTD and follow the results after two years of the disease course. There were 98 children (mean age: 13 ± 3; 76 girls), treated in Department of Pediatric Rheumatology, Institute of Rheumatology, Warsaw and 80 aged-matched, healthy controls (mean age 12.7 ± 2.4; 50 girls) included into the study. Study procedures included medical history, physical examination, chest radiograph and PFT (spirometry and whole body-plethysmography). Then, the assessment of PFT was performed after 24 months. FEV₁, FEV₁/FVC and MEF50 were significantly lower in CTD as compared to control group, there was no difference in FVC and TLC. The proportion of patients with abnormal lung function was significantly higher in the study group, 41 (42%) vs 9 (11%). 24-months observation didn't reveal progression in lung function impairment. Lung function impairment is relatively common in children with CTDs. Although restrictive ventilatory pattern is considered typical feature of lung involvement in CTDs, airflow limitation could also be an initial abnormality.

Pulmonary Functions in Children With Thalassemia Major.

Thalassemia major (TM) is a chronic disease requiring regular transfusions that may result in generalized iron loading, such as in the heart, the liver, endocrine organs, and the lungs. We aimed to determine pulmonary function abnormalities in children with TM in our center. In this study, pulmonary function tests (PFTs) of 49 patients with TM who received regular blood transfusion and had no history of chronic respiratory disease were evaluated. The relationship between PFTs and the age, the body surface area, pretransfusional hemoglobin, and serum ferritin was evaluated. Among the β-TM patients included in this study, 61% were male and 39% were female, with a mean age of 10.8±3 years (range, 5 to 17 y). The patients' mean level of ferritin was 3873±2011 ng/dL (range, 676 to 9476 ng/dL). A reduced forced vital capacity (FVC) was found in 33 patients (67%). A reduced forced expiratory volume in 1 second (FEV1) was found in 15 patients (30%). A forced expiratory volume in 1 second to forced vital capacity (FEV1/FVC) ratio of >80% was found in all patients. The peak expiratory flow (PEF) was decreased in 23 patients (46.9%). The forced mid-expiratory flow between 25% and 75% of the exhaled vital capacity (MEF25%-75%) was decreased in 5 patients (10%). FVC and FEV1 values in patients with a high ferritin level (>2500 ng/dL) were decreased compared with patients with a low ferritin level (<2500 ng/dL) (P=0.04, 0.03). FVC, FEV1, and PEF parameters were negatively correlated with the age and the body surface area. Age was a predictor of FVC (β=-0.450, P<0.001), FEV1 (β=-0.419, P<0.001), and PEF (β=-0.505, P<0.001), and hemoglobin was a predictor of FEV1/FVC (β=0.366, P=0.01) and MEF25%-75% (β=0.323, P=0.003). Our results concluded that the respiratory system should be evaluated by PFTs even in asymptomatic patients with high serum ferritin levels during the adolescent period annually to prevent the squeal of pulmonary disease in TM. Patients who have abnormal PFTs should be reevaluated for compliance with chelation therapy and the transfusion program.

AB0892 Evaluation of Thyroid Function in Children with Familial Mediterranean Fever

BackgroundFamilial Mediterranean fever (FMF) is the most frequent hereditary autoinflammatory disease, characterized by recurrent self-limited attacks of fever and polyserositis. The development of renal amyloidosis type AA is the most...

Pulmonary function abnormalities in childhood cancer survivors treated with bleomycin.

Bleomycin is associated with pulmonary toxic side effects including pneumonitis and pulmonary fibrosis. We evaluated the prevalence of long-term pulmonary function abnormalities in children receiving bleomycin therapy in the context of current chemotherapeutic regimens. A retrospective review of patients who received bleomycin between January 1999 and December 2011 was conducted. Abnormalities in the most recent pulmonary function test (PFT) at least 1 year after diagnosis were analyzed. Two-hundred and seven patients had received bleomycin. The results of PFT performed at least 1 year from diagnosis were available for 80 patients. Median time of follow up was 3.9 years (range 1.1-11.76 years). Median cumulative dose of bleomycin was 65 IU/m(2) (range 10-120). The most common diagnoses were Hodgkin lymphoma and germ cell tumor. At least one pulmonary function abnormality was present in 42 (52.5%) patients. When classified in groups, 22.5% patients had obstructive lung disease, 7.5% had restrictive lung disease, 28.8% had hyperinflation and 14% of patients had non-uniform distribution of ventilation. Non-Hispanic patients (OR 2.81) and children younger than 8 years (OR 4.14) had higher odds of having an abnormal PFT parameter. Very few patients had pulmonary symptoms. More than half the patients who received bleomycin had subclinical pulmonary dysfunction as evidenced by abnormalities in pulmonary function tests, although the incidence of clinical symptoms was low.

Pulmonary Function Abnormalities in Children with Type 1 Diabetes Mellitus

Pulmonary Function Abnormalities in Children with Type 1 Diabetes Mellitus

Pulmonary Function Abnormalities in Children with Active Crohn’s Disease

Pulmonary Function Abnormalities in Children with Active Crohn’s Disease

666 Pulmonary Abnormalities in Children and Adolescents with Type 1 Diabetes Mellitus

Background and AimsFew studies are available on pulmonary function abnormalities in children with diabetes with controversial results. Spirometric abnormalities and reduction of lung diffusing capacity for carbon monoxide (DLCO) have...

Evaluation of Thyroid Gland Function in Children with Obstructive Apnea Hypopnea Syndrome

Patients with obstructive sleep apnea hypopnea syndrome (OSAHS) and thyroid function abnormalities, such as hypothyroidism and Hashimoto's thyroiditis, usually have closely resembling clinical features. Differentiation between these disorders is made more difficult because hypothyroid patients are also at risk for secondary sleep-disordered breathing. The aim of our study is to evaluate the prevalence of thyroid function abnormalities in children with OSAHS. Forty-four children (15 females: 29 males), 2.5-14.5 (7.43±2.98) years old were studied with overnight polysomnography. Biochemical screening of thyroid gland function was also carried out. Patients were judged to have OSAHS based mainly on the evaluation of Apnea Hypopnea index per hour of sleep (AHI). 15/44 (34.1%) children had mild OSAHS, 17/44 (38.6%) moderate and 12/44 (27.3%) severe OSAHS. Hypothyroidism was recorded only in 5/44 (11.4%) and Hashimoto's thyroiditis in 3/44 (6.8%) of OSAHS patients. Two patients with hypothyroidism showed mild and three severe OSAHS, while from the 3 children with Hashimoto's thyroiditis one presented mild, one moderate and one severe degree of OSAHS. Although the majority of studies in bibliography worldwide do not consider necessary the systemic evaluation of thyroid gland function in patients with breathing disorders during sleep, it seems that in children this type of screening is required for the differential diagnosis between primary sleep apnea and hypothyroid sleep-disordered breathing in order to differentiate these two conditions. Therefore, the laboratory investigation of thyroid gland function could be considered necessary.

Alteraciones de la función pulmonar tras el trasplante de progenitores hematopoyéticos

To evaluate lung function abnormalities in children who underwent haematopoietic stem cell transplantation (HSCT) and to compare these abnormalities between autologous and allogenic transplantation. Prospective observational study from 1996 to 2005. Ninety-three children receiving HSCT, 47 autologous and 46 allogenic, were included. Lung function tests were performed before transplantation and at 2, 6, 12 and 24 months afterwards. The following indices were determined: forced expiratory volume in 1 s (FEV1), FEV1/forced vital capacity (FVC), total lung capacity (TLC), and carbon monoxide diffusing capacity (DLCO). Paired Student's t-test was used for statistical analysis of data. Before HSCT, 6.8% of the children had FEV1<80%, 1% FEV1/FVC<80%, 7.8% TLC<80% and 13.5% DLCO<70%. At 2 months, FEV1/FVC, TLC and DLCO were significantly reduced, when compared to pre-transplantation values (p=0.05, 0.011 and p<0.001, respectively). Lung function gradually improved from 6 months post-transplantation, but did not reach pre-transplantation values at 24 months. No significant differences were found when comparing allogenic and autologous transplantation, apart from a lower FEV1/FVC value at 6 months (p=0.02) in the first group. An important proportion of children who undergo HSCT have early pulmonary abnormalities (at 2 and 6 months after transplantation) with partial recovery at 24 months.

194 – Brain function abnormalities in children experiencing putative antecedents of schizophrenia

194 – Brain function abnormalities in children experiencing putative antecedents of schizophrenia

Pulmonary function abnormalities in children treated with whole lung irradiation

We sought to determine the prevalence of abnormal pulmonary function tests (PFTs) in a cohort of children who had received whole lung irradiation (WLI) for treatment of metastatic disease. This was a retrospective (1988-2003) chart review that included all children treated at our institution with WLI who had undergone PFT. Data abstracted included oncologic diagnosis, radiation dose and fractionation, spirometry (FVC, FEV1, FEV1/FVC, FEF25%-75%), plethysmography (TLC, FRC, RV, RV/TLC), diffusing capacity (DLCO), and respiratory muscle strength (MIP, MEP). PFTs were normalized according to standard deviation (Z) scores. Thirty patients were identified who had one or more PFT. The incidence of mild, moderate, or severe reductions in FEV1 was 30%, 10%, and 10%, respectively, with 50% having normal FEV1. Seventeen percent of patients had mild reduction in total lung capacity (TLC), while 13% and 30% had moderate or severe reductions. Thirty-eight percent had mild reductions in diffusing capacity, while 29% and 14% had moderate or severe reductions. Pulmonary function abnormalities were common in this cohort of children treated with WLI, and may be progressive in nature. Further studies are warranted to identify patients at highest risk.

Pulmonary function impairment in children following hematopoietic stem cell transplantation

Deterioration of pulmonary function after hematopoietic stem cell transplantation (SCT) is a well-known late effect of this treatment, but the course of pulmonary function over time is less clear. The aim of our study was to establish both the prevalence and course of pulmonary function abnormalities in children following SCT. Thirty-nine of 106 patients, who visited a post-SCT late effects clinic and who underwent a pulmonary function test (PFT) both before and at least twice after SCT were included in this study. Forced expiratory volume in 1 sec (FEV1), forced vital capacity (FVC), total lung capacity (TLC), and total lung diffusion capacity (TLCO) were determined and recorded as percentage predicted for age, sex, and length matched controls. Values of less than 80% of predicted were considered abnormal. Change in PFT parameters over time was determined by comparing the mean PFT parameter in our group at three different time points: pre-SCT, < or =1 year post-SCT (SCTpost1) and >1 year post-SCT (SCTpost2). After SCT restrictive and/or diffusion abnormalities are most prevalent (45% and 76% at SCTpost1, respectively). A significant decrease of TLC (-9.7%) and TLCO (-20.3%) was observed during the first year after SCT, with improvement over time, but no normalization. Obstructive lung disease was less common (6% at SCTpost1). Clinical signs of lung function impairment were rare. Restrictive and diffusion lung function disorders are common after SCT. They improve over time but do not normalize. As only a few patients with pulmonary function abnormalities had clinical signs of lung function impairment, the clinical relevance of performing long-term follow-up of PFT is questionable.

Lung function abnormalities in children with type 1 diabetes

Recent developments in intrabronchial administration of insulin raise lung function in patients with type 1 diabetes as important issue. Several studies in adults report abnormalities of lung function of these patients. The aim of this study was to investigate lung function in children with type 1 diabetes. Twenty-seven children with type 1 diabetes performed measurement of airway obstruction (forced flow-volume curves), lung volumes and airway resistance (bodyplethysmography) and of pulmonary carbon monoxide diffusion capacity. Mean age (± SD) of the children was 12.8±5 years. Mean time between the detection of type 1 diabetes and the lung function tests was 5.5 years with a variation from 1 to 17 years. The total airway resistance (Raw) was significantly higher compared to the reference values ( P<0.001). The other lung function parameters were not significantly different from reference values ( P>0.05). In this relatively small study no relationship between lung function abnormalities and age, the duration of disease or level of HbA 1c was observed. Our data show that increase of airway resistance do occur in children with type 1 diabetes. Progressive abnormalities in lung function might interfere with the promising results of treatment with intrabronchial administration of insulin.

Visual function in children with hemiplegia in the first years of life

The aim of this study was to evaluate the incidence of visual function abnormalities in children with infantile hemiplegia, and the relation between visual abnormalities and type of lesion, as shown by brain MRI. Visual function was tested (grating acuity, visual field size, binocular optokinetic nystagmus [OKN], and ocular movements) in a group of 47 children with congenital or early acquired hemiplegic cerebral palsy (mean age 25 months, range 8 to 52 months). The cohort was subdivided into four groups according to MRI findings: brain malformations (n=5), abnormalities of the periventricular white matter (n=20), cortical-subcortical lesions (n=16), and non-progressive postnatal brain injuries (n=6). More than 80% of the children showed abnormal results in at least one visual test: acuity was the least impaired function, while visual field and OKN were abnormal in more than 50% of the cohort. No specific correlation could be identified between the type and timing of the lesions and visual function. Unlike adults with stroke, visual field defects were not always related to contralateral damage in the optic radiations or in the visual cortex. These results indicate that visual abnormalities are common in children with hemiplegia, and that they cannot always be predicted by MRI. All children with hemiplegia need a detailed assessment of visual function.