Assessment of Right Heart Functions in Children with Mild Cystic Fibrosis.

BACKGROUND: Cystic fibrosis (CF) is a multisystemic disease that prevalently involves the lungs. Hypoxemia occurs due to the existing of progressive damage to the pulmonary parenchyma and pulmonary vessels. The condition may cause systolic and diastolic dysfunction to the right ventricle due to the effects of high pulmonary artery systolic pressure (PASP). The study aimed to determine echocardiographic alterations in PASP, right ventricle (RV) anatomy, and functions in mild CF children. MATERIALS AND METHODS: RV anatomy, systolic, and diastolic functions were evaluated with conventional echocardiographic measurements. Estimated PASP was used measured with new echocardiographic modalities, including pulmonary artery acceleration time (PAAT), right ventricular ejection time (RVET), and their ratio (PAAT/RVET). The obtained echocardiographic data were statistically compared between the patient group and the control group. RESULTS: The study consisted of 30 pediatric patients with mild CF and 30 healthy children with similar demographics. In patient group, conventional parameters disclosed differences in RV anatomy, both systolic and diastolic functions of RV compared with the healthy group. We did not compare the patient group with published standard data because of the wide range variability. However, new echocardiographic parameters showed notable increase in pulmonary artery pressure compared with values of control group and published standard data (p<0.001). CONCLUSION: Elevated PASP, RV failure, and Cor pulmonale usually begin early in children with mild CF. In addition to routine echocardiographic measurements to evaluate RV, we recommend the use of new echocardiographic modalities for routine examinations and in the follow up of children with mild CF.

Post-intubation subglottic stenosis in children: Analysis of clinical features and risk factors.

BACKGROUND: Subglottic stenosis (SGS) is a complication that develops after intubation and is characterized by respiratory distress. The aim was to evaluate patients with post-intubation SGS and to discover the factors contributing to its development. METHODS: A total of 112 patients who had a history of intubation were included. The case group consisted of 50 patients with post-extubation persistent respiratory symptoms for which flexible bronchoscopy (FOB) was conducted and showed SGS. The control group consisted of 62 patient with no post-extubation persistent respiratory symptoms, for whom FOB was not done (n = 54), and who had post-extubation persistent respiratory symptoms and underwent FOB, which did not show subglottic stenosis (n = 8). RESULTS: No significant differences were detected related to age, gender, and gestational age. The median number of recurrent intubations was 2.5 and 3 in the case group and in control group, respectively (P = 0.14). The median duration of intubation was 20.5 days in the case group, and 6 days in the control group (P < 0.001). The Myer-Cotton classification indicated a degree of obstruction of grade 1 (mild) in 30% (n = 15), grade 2 in 16% (n = 8), grade 3 in 48% (n = 24), and grade 4 in 6% (n = 3) of the case group. CONCLUSION: The duration of intubation was found to be a significant risk factor for SGS development. Age at intubation, gender, gestational age, indication of intubation, and the number of recurrent intubations were found to have no significant association. Patients with post-extubation persistent respiratory problems, especially those with prolonged intubations, should be evaluated for SGS.

Electrocardiographic Changes in Jordanian Patients With Becker Muscular Dystrophy.

Background and aim Becker muscular dystrophy (BMD) is an X-linked disease caused by an in-frame mutation in the dystrophin gene, which is considered an allelic disorder to the most severe form of dystrophinopahies, Duchenne muscular dystrophy, which leads to skeletal and cardiac muscle involvement and results in dilated cardiomyopathy (DCM). The aim of this study is to present our ECG data and the significance of this data in the early detection of DCM in these patients. Methods This is a retrospective study. All patients known to the clinical Genetic Clinic and Queen Alia Heart Center in Jordan with a diagnosis of Becker muscular dystrophy from the year 2011-2022 are offered cardiac evaluation according to the guidelines, which included clinical assessment, electrocardiograph, and 2-D echocardiograph (echo) at the time of diagnosis and every five years thereafter once the initial assessment was normal. All the records were retrieved and analyzed. Results Fifty-three patients of all ages with genetically confirmed BMD were identified. Twelve had no record as they didn't attend any cardiac evaluation. Forty-one were under regular clinical follow-up. Two were excluded as they died, and another four had no recorded data in our center. Ultimately, 35 patients were included and studied. The mean age was 30.5 years ± 22.1, ranging from two to seventy-seven years of age. Twenty-seven (77%) had abnormal ECG. High voltage R wave in V2 and V1 was the most common finding, followed by repolarisation abnormalities and Q wave (43%, 17%, 13%, and 11% respectively). Incomplete right bundle branch block in 4% as well as R/S ratio >1.2. U wave abnormalities in 3% and sinus tachycardia were found in only one patient. Conclusion Cardiac surveillance for patients with Becker muscular dystrophy is mandatory after the age of 16. Q wave and repolarisation changes should be taken seriously as early signs of dilated cardiomyopathy, even if the echo is normal.

Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosis.

BACKGROUND: Pseudo-Bartter syndrome (PBS) is a rare complication of cystic fibrosis (CF) and there are limited data in the literature about it. We aimed to compare clinical features and accompanying findings of patients with PBS in a large patient population. METHODS: The data were collected from the Cystic Fibrosis Registry of Turkey where 1170 CF patients were recorded in 2017. Clinical features, diagnostic test results, colonization status, complications, and genetic test results were compared in patients with and without PBS. RESULTS: Totally 1170 patients were recorded into the registry in 2017 and 120 (10%) of them had PBS. The mean age of diagnosis and current age of patients were significantly younger and newborn screening positivity was lower in patients with PBS (P < .001). There were no differences between the groups in terms of colonization status, mean z-scores of weight, height, BMI, and mean FEV1 percentage. Types of genetic mutations did not differ between the two groups. Accompanying complications were more frequent in patients without PBS. CONCLUSION: PBS was detected as the most common complication in the registry. It could be due to warm weather conditions of our country. It is usually seen in younger ages regardless of mutation phenotype and it could be a clue for early diagnosis of CF.