Echocardiographic assessment of cardiovascular involvements in children with cystic fibrosis.

BACKGROUND: Over the past four decades, numerous case reports and clinical studies have highlighted the presence of heart disease in individuals with cystic fibrosis. Given the limited information in this field and the imperative to identify early changes during childhood, our study aims to explore cardiac dysfunction in patients with cystic fibrosis using echocardiography. METHODS: In this case-control study, we examined echocardiographic findings from thirty-three patients with cystic fibrosis and sixty healthy children. Demographic information for both groups was recorded, and the disease severity in patients was assessed using the Schawachman criterion. M-mode, Doppler flow velocity, and Tissue Doppler Imaging echocardiography were performed for all participants, with subsequent data analysis using SPSS 24. RESULTS: Our study encompassed thirty-three CF patients and sixty healthy children. The estimated pulmonary artery blood pressure (systolic and mean) in patients with cystic fibrosis was significantly higher than in the control group (P < 0.05). Additionally, the mean trans-tricuspid peak early to late diastolic flow velocity (E/A) was significantly lower in the case group than the control group (P < 0.05), along with a significantly lower mean tricuspid valve deceleration time (DT) (P < 0.05). Similarly, the mean TAPSE in the case group was notably lower than in the control group (P < 0.05). No significant difference in Mean left ventricular Ejection Fraction (EF) and Fractional Shortening (FS) existed between the two groups (P > 0.05). Furthermore, Trans-mitral peak early to late diastolic flow velocity (E/A) in the case group was significantly lower than in the control group (P < 0.05), and the mean mitral valve DT in the case group was also significantly lower (P < 0.05). CONCLUSION: Our study findings indicate the presence of some degree of right ventricular dysfunction in children with cystic fibrosis. This finding may have implications for the development or modification of clinical guidelines for managing cystic fibrosis in children. Further investigations are recommended to elucidate the underlying mechanisms and contributing factors, providing valuable insights for clinical management.

Prevalence of Dysglycemia, Dyslipidemia, and Metabolic Syndrome among Patients with HIV Infection: a Cross-sectional Study from Iran.

Background: Human immunodeficiency virus (HIV) resulted in considerable morbidity and mortality. Following antiretroviral therapy (ART), the life expectancy of HIV-infected patients increased; however, they were more at risk of developing chronic diseases such as endocrinopathies. This study aimed to determine the prevalence of dysglycemia, dyslipidemia, and metabolic syndrome among patients with HIV infection. Methods: This cross-sectional study was conducted on HIV-infected patients referring to Loghman Hakim Hospital (Tehran, Iran) between April 2020 and April 2021. We examined demographic features, medical history, and laboratory tests indicating the metabolic status of the patients. Eventually, collected data were processed using SPSS version 23. Results: The mean age of 68 confirmed HIV patients was 39.85±10.54 years and 64.7% were male. BMI (MD = 2.57, 95% CI = [0.25, 4.88], P = 0.035), cholesterol (MD = 22.73, 95% CI = [4.70, 40.76], P = 0.014), HDL (MD = 8.54, 95% CI = [2.06, 15.02], P = 0.011), and LDL of women was significantly higher than men (MD = 22.43, 95% CI = [7.60, 37.27], P = 0.004). Additionally, 30 patients (44.1%) suffered from metabolic syndrome. The prevalence of metabolic syndrome differed significantly between men (34.1%) and women (62.50%) (P = 0.024). Conclusion: Dysglycemia, dyslipidemia, and metabolic syndrome are common among HIV-infected patients. Thus, periodic evaluation of the patients can be advantageous in early diagnosis and timely treatment.

Arterial tortuosity syndrome presented as nonvisualization of thoracic aorta in preoperative transthoracic echocardiogram: A case report.

Arterial tortuosity syndrome is a rare genetic disorder characterized by dilation, elongation, and significant tortuosity of major arteries. Approximately 100 cases of this disorder have been reported worldwide, including 3 reports in Iran. We describe a case of arterial tortuosity syndrome suspected during the preoperative evaluation for hypertrophic pyloric stenosis, where the thoracic aorta was not visualized appropriately in transthoracic echocardiography. Our report focuses on identifying the disease through diagnostic imaging.

Pulmonary Involvement in Neuromuscular Diseases: A Review.

Neuromuscular diseases (NMDs) affect muscle function directly or indirectly by affecting nerves or neuromuscular junctions. One of the leading causes of death in patients with NMD is respiratory muscle weakness (RMW). Respiratory involvement in patients with NMD can manifest widely, from mild failure that may initially affect only sleep to severe failure that can be life-threatening. Care approaches include arranged and precise clinical follow-ups of signs of sleep-disordered breathing, daytime hypoventilation, coughing, and swallowing disturbances. This manuscript will review the mechanisms and abnormalities of respiratory function in patients with NMD and help optimize NMD management.

A novel gene mutation for multicentric osteolysis nodulosis and arthropathy: Case report and review of literature.

Frank-Ter Haar syndrome (FTHS), Winchester syndrome (WS), Torg syndrome (TS) and Multicentric Osteolysis Nodulosis and Arthropathy (MONA) are progressive skeletal dysplasia consisting of acro-osteolysis. Mutation in Matrix Metalloproteinase 2 (MMP2), Matrix Metalloproteinase 14 (MMP14) and SH3PXD2B are known genetic defects in these disorders. We hereby report a 5 years and 9 months old girl suffering from progressive limb deformity. She is the first child of a relative couple, who was referred to metabolic disorders' clinic due to poor growth and bone pain. On physical examination, minor facial dysmorphism, hypertrichosis, severe hand deformity with limitation in range of motion in carpal, metacarpal and phalangeal joints, hallux valgus deformity of feet, soft tissue hypertrophy and nodule formation in palmoplantar areas were detected. Her past history indicated a cardiac defect resulting in open heart surgery at 8 months of age. Genetic study revealed a new homozygote nonsense mutation in MMP2 gene explaining her clinical manifestations. We recommend careful evaluation and follow-up of patients with congenital heart disease, as it may be the first presentation of a genetic multisystem disorder. Early differentiation of the disease from other skeletal dysplasia and rheumatologic disorders could prevent unnecessary management.

Defects in Very Long-Chain Fatty Acid Oxidation Presenting as Different Types of Cardiomyopathy.

Cardiac involvement may accompany various inborn errors of metabolism (IEM) including fatty acid oxidation (FAO) disorders, presenting as rhythm disturbances, conduction abnormalities, cardiomyopathies, pericardial effusion, and sudden cardiac death. FAO disorders are rare mitochondrial diseases with variable organ involvements and clinical presentations. Very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) is a FAO disorder with diverse clinical presentations. We report two VLCADD patients with cardiac involvement and diverse presentations. The first patient represents with cardiogenic shock and dilated cardiomyopathy (DCM) at childhood. The second patient represents with suspicious sepsis at early infancy and hypertrophic cardiomyopathy (HCM) at further evaluation. IEM should be thought of in every individual case with suspicious sepsis or cardiac failure regardless of age or previous history.

Echocardiographic Indices in Pediatric Chronic Kidney Disease.

INTRODUCTION: Cardiovascular disease (CVD) may accompany chronic kidney disease (CKD), resulting in additional complications and increased death rate. This study was performed to evaluate cardiac structure and function and several risk factors in hospitalized CKD children. METHODS: Seventy-four children with CKD were enrolled in this cross-sectional descriptive study. Two-dimensional and M-mode ultrasonography, Doppler flow velocity and Tissue Doppler Imaging (TDI) were used to evaluate cardiac chamber size, left ventricular mass (LVM) and echocardiographic indices of ventricular function. RESULTS: Advanced stages of CKD showed statistically insignificant increased LVM and LVM indexed to height2.7 (LVMI), and mildly reduced diastolic function. Hypertensive patients had an insignificant increase in the incidence of left ventricular hypertrophy (LVH) defined as LVMI greater than 95th percentile for age and sex and LVH2 as LVMI2 more than 95 gr/m2 for girls and more than 115gr/ m2 for boys older than 8 years. Patients with LVH had lower left ventricular ejection fraction (LVEF) and abnormal right ventricular (RV) function based on the tricuspid valve systolic velocity (TV S') survey. LVH2 cases, however, revealed decreased LV systolic function according to ejection fraction (EF) and abnormal mitral valve systolic velocity (MV S'). CONCLUSION: LVH related to hypertension and mild systolic and diastolic dysfunction were more prevalent in advanced CKD cases, however TDI showed no statistically significant difference in the prevalence of MV S' and TV S'. We recommend strict blood pressure control and prevention of renal function deterioration as effective tools for cardiac protection in CKD children.  DOI: 10.52547/ijkd.6643.

Intravascular Ultrasound for Assessment of Residual Coarctation of the Aorta after Balloon Angioplasty in Infants.

Intravascular ultrasound (IVUS) has been introduced as an accurate and minimally invasive diagnostic technique for the assessment of vascular anatomy and its abnormalities. We believe that IVUS can be used for clarifying the reasons for failure of balloon angiography in infantile coarctation of the aorta (CoA), because post-balloon angioplasty tearing, intimal flap, thrombosis and pseudoaneurysm of the aorta can be evaluated by IVUS with greater sensitivity and specificity. We aimed to assess the outcome of balloon angioplasty of CoA using angiography as the gold standard and IVUS as a new method in infants, comparing the two techniques for the evaluation of the diameter and area of CoA segment pre- and post-procedure. This cross-sectional study was performed on 18 infants hospitalized with a final diagnosis of CoA. All the infants underwent angiography and were also assessed by IVUS to measure the preoperative and postoperative diameter of the narrow segment in the two anterior-posterior and lateral views. In assessment by IVUS, the mean diameter of the coarctation site increased from 2.10 ± 0.30 mm to 4.50 ± 0.94 mm (P < 0.001). Similarly, the average minimum area of the coarctation level increased from 5.26 ± 1.50 mm2 to 13.77 ± 3.48 mm2 after angioplasty (P < 0.001). Comparing these findings, angiography and IVUS showed a high level of agreement. In the assessment of a dissection flap, there was a high level of agreement between angioplasty and IVUS before the procedure, but IVUS had higher accuracy after the procedure. Our study showed that IVUS was more reliable than angiography in the assessment of residual coarctation. IVUS yielded high sensitivity (58.3%) and specificity (100%) for discriminating the presence and absence of residual coarctation as well as the need for repeating the procedure. The assessment of coarctation before and after angioplasty procedures in children is possible using the IVUS method, with high accuracy. IVUS can offer greater accuracy than angiography in the evaluation of the coarctation area, detecting tears, dissection and flaps, and assessment of residual coarctation.

Vitamin D deficiency and its impact on asthma severity in asthmatic children.

BACKGROUND: Despite obtaining evidences on association between vitamin D and development of lung in fetus, little is known about vitamin D level and its impact on severity of asthma in children. The present study aimed to assess the relationship between the asthma severity and vitamin D deficiency in asthmatic children. METHODS: This case-control study was conducted on 106 individuals including asthmatic (n = 53) and healthy children (n = 53) who referred to Mofid hospital in Tehran in 2013. The level of serum vitamin D in both groups was measured by radioimmunoassay method at the reference lab and was categorized as sufficient (> 30 ng/ml), insufficient (20 to 30 ng/ml), or deficient (< 20 ng/ml). The control status of asthma in patients group was classified as controlled, partially controlled, and uncontrolled. RESULTS: In the groups with and without asthma, the prevalence of vitamin D deficiency was 73.6 and 49.1%, and the prevalence of vitamin D insufficiency was 18.9 and 18.9%, while normal vitamin D level was revealed in 7.5 and 32.1%, respectively with a significant difference (p = 0.005). Using the multivariate logistic regression analysis, the presence of asthma was associated with reduced level of vitamin D (OR = 1.068, 95% CI: 1.027-1.110, P = 0.001). In this context, the risk for asthma in the children with vitamin D deficiency was 6.3 times of those with normal vitamin D level. Although the presence of asthma was strongly associated with reduced level of vitamin D in serum, neither severity of asthma nor control status of asthma were associated with vitamin D deficiency. CONCLUSION: The presence of vitamin D deficiency effectively predict increased risk for childhood asthma; however the severity or control status of this event may not be predicted by confirming vitamin D deficiency.

Postoperative outcome for hyperglycemic pediatric cardiac surgery patients.

This prospective cohort study aimed to determine the morbidity and mortality among hyperglycemic pediatric patients after cardiac surgery. The study was conducted in a pediatric intensive care unit (PICU) for cardiac surgery patients at a university-affiliated, referral, heart hospital. A total of 379 postcardiac surgery pediatric patients participated in the study. No interventions were performed. Measurements of blood glucose level together with other clinical and laboratory data were collected on postoperative days 1, 2, 3, and 7. Mean blood glucose level exceeding 126 mg/dl was considered hyperglycemia, and a level exceeding 200 mg/dl determined severe hyperglycemia. These measurements were analyzed for association with major complications and death. Hyperglycemia was common (86%) in this cohort study. There was no statistical correlation between hyperglycemia and death or major complications, but patients with severe hyperglycemia showed a significantly higher mortality rate (16/64 deaths [25%] vs. 13/315 deaths [4.12%]; P < 0.001]) and more morbidities (16/64 [25%] vs. 43/315 [13.65%]; P = 0.022). Severe hyperglycemia was independently associated with mortality according to multivariate logistic regression. Hyperglycemia is quite prevalent among pediatric patients after cardiac surgeries. Severe hyperglycemia is associated with higher morbidity and mortality rates in this patient population.

Balloon Angioplasty versus Surgical Repair of Coarctation of Aorta in Infants.

BACKGROUND: Coarctation of the aorta is a discrete stenosis of the proximal thoracic aorta. The common clinical pattern is congestive heart failure in infancy. Treatment methods include balloon angioplasty and surgical repair in this age group. Percutaneous balloon angioplasty is a less invasive method for the repair of discrete coarctation but remains controversial as a primary treatment strategy for a native coarctation. This study aimed to compare the effectiveness and outcome of balloon angioplasty and surgical repair in coarctation infants younger than 1 year old. METHODS: This retrospective study evaluated the results of the two methods in 167 patients younger than one year old admitted into a tertiary heart center pediatric ward with the diagnosis of coarctation of the aorta: Balloon angioplasty was done for 55 and surgical repair for 112 infants. Patients with previous interventions were not included in this study. RESULTS: Primary results revealed no significant difference in the effectiveness of the two methods (p value = 0.0601). While the rate of recurrent coarctation was significantly lower in the surgery group [19 (17%) vs. 11 (20%), p value = 0.0470], the mortality rate was lower in the balloon angioplasty method [5 (5.5%) vs. 13 (11.6%), p value = 0.039]. Our multivariate logistic regression model, however, showed no statistically significant difference (p value = 0.120). CONCLUSION: Because of the incidence of re-coarctation, balloon angioplasty compared with surgical repair did not confer an improved outcome for our infants' coarctation.

Morning report: a survey of Iranian senior faculty attitudes.

Morning report (MR) is a universal component of internal medicine training. Despite its omnipresence, little is known about senior faculty attitudes toward MR. We aimed to survey expert attitudes towards MR. Using an 85-item self-administered questionnaire, we made a survey of medical education-trained faculty members (FMs) who participated in and presented articles at the Seventh National Iranian Congress on Medical Education, 12-15 November 2005, Tabriz, Iran, on the processes of teaching and learning during MR (n=175). Among the 111 FMs (63.4%) who completed the survey, education was the most frequently cited goal of the MR. Clinical wisdom and the ability to expand a resident's differential diagnosis skills were the attributes most often proposed as essential for the FMs. Respondents believed the FM in cooperation with the senior resident of the last duty (41%) or the senior resident of the last duty alone (40%) should select the presenting cases. The study participants stated that 41% of the MR should be spent discussing common cases, 33% in the area of expertise of the FM and 26% in discussing rare or interesting cases. A common opinion was that treatment recommendations should be based on the medical literature. In terms of the educational impact of activities during the MR, listing differential diagnoses and bedside visits to examine unique findings scored the highest. Education as the main goal of the MR can best be achieved by expanding residents' skills in differential diagnosis and in making decisions using an evidence-based, self-directed method.