Short-term azithromycin use is associated with QTc interval prolongation in children with cystic fibrosis.

BACKGROUND: Azithromycin is used for children with cystic fibrosis (CF) for its immunomodulatory and anti-inflammatory action. This study investigated the short-term alterations in QTc interval associated with azithromycin prophylaxis in pediatric patients with CF. METHODS: This study included 121 patients with mild CF, of whom 76 received azithromycin (patient group) and 45 did not receive azithromycin (control group). The patient and control groups were categorized according to age as under 12 years of age and over 12 years of age. The first presentation measured all the patient and control groups at basic QTc time intervals. The QTc intervals of all patients were then remeasured systemically at 1, 3, and 6 months. Age categories and QTc intervals that were calculated at each month in the patient and control groups were compared statistically. RESULTS: A statistically significant difference was detected in the patient group between the initial QTc interval time and the electrocardiogram (ECG) findings in the first and third months after prophylaxis treatment (p < 0.001; p = 0.01). However, no statistically significant difference was detected in the sixth month (p > 0.05) in all groups. Almost all of the children's QTc intervals were within normal range and within the safety zone (under 0.44 s). No statistically significant difference was detected in the control group between the initial ECG and the QTc intervals measured at 1, 3, and 6 months. CONCLUSION: Short-term use of azithromycin prophylaxis in pediatric patients with mild CF slightly increased the QTc interval in the first and third months of follow-up. Nevertheless, all QTc interval changes fell within the safety zone. Notably, 1 month of follow-up treatment should be performed to check for any alteration in the QTc interval. If increased QTc interval duration is not detected in the first month, azithromycin prophylaxis can be safely prescribed.

Diagnostic role of gray-scale and shear-wave elastography in pediatric patients with undescended testes: a prospective controlled study.

Aim: Ultrasound elastography is a simple non-invasive method for measuring tissue elasticity in relation to tissue fibrosis. The aim of this study was to compare echogenicity, volume and shear wave velocities of undescended vs normally descended testes. Material and methods: Sixty-six boys with undescended testes were included in this study. The median age range was 35.5 (10-118) months old. The cases included in this prospective study consisted of 66 patients with non-operated undescended testes, with 51 of them being affected unilaterally and 15 affected bilaterally, as diagnosed by physical examination. The control group consisted of 31 healthy boys without any particular health problems. This prospective study was performed by gray-scale ultrasonography and shear wave elastography in boys with undescended testes and healthy testes. The testicular volumes were established by ultrasound measurement, the echogenicity and shear wave elastography values were measured in boys with unilateral and bilateral undescended testes, and the results were compared with healthy boys' testes and their contralateral testes. The stiffness values were recorded for speed (m/s) and elasticity (kPa), and the stiffness values of undescended testes were compared with the healthy control group. Results: Echogenicity values were lower in the bilateral undescended testes group than in the healthy group, and the healthy group's echogenicity was normal (p <0.001). The ROC curve was used to identify a cut-off shear wave elastography value for predicting decreased testicular echogenicity by using average shear wave elastography values. The area under the curve for the undescended testes was 0.78 (95% CI: 0.70-0.85, sensitivity 83.7%, specificity 68.7%, p <0.001), with an average shear wave elastography value of 2.32 (m/s) for above the cut-off point indicates. This was found to be significantly associated with reduced echogenicity on gray-scale ultrasonography, suggesting that it may be correlated with fibrosis developing in patients with undescended testes. Conclusion: The study provides interesting findings in that it proposes an alternative non-invasive method for the assessment of testicular tissue in undescended testes. We used shear wave elastography to compare the stiffness of normal testes in both heathy patients and in the contralateral healthy testes of boys with undescended testes, with the values obtained for the undescended testes reflecting the level of fibrosis of the parenchyma. Another outcome of this study was observed in patients with unilateral undescended testes, where the normally descended testes showed increased shear wave elastography values, which could be an early indication of parenchymal change.

Prenatal Detection and Postnatal Outcome of Persistent Left Superior Vena Cava and Agenesis of Ductus Venosus Associated with Postnatal Bovine Aortic Arch.

BACKGROUND: Isolated agenesis of ductus venosus (ADV) is usually a benign condition, but it may be associated with cardiovascular defects, hydrops, growth restriction, and chromosomal abnormalities. Additionally, persistent left superior vena cava (PLSVC) and bovine aortic arch are relatively common fetal anomalies. To the author's knowledge, this is the first report of prenatal detection of DV agenesis and PLSVC associated with the postnatal bovine aortic arch with a hypoplastic transverse aortic arch. CASE: A 25-year-old, G2P1 woman was referred to our department at 31 weeks due to fetal growth restriction and short femur. On fetal echocardiography, DV could not be viewed via two-dimensional (2D) and Doppler ultrasound (US) imaging; there was also evidence of the co-occurrence of PLSVC and an aortic arch anomaly. We revealed the intrahepatic continuation of the umbilical vein. A weekly follow-up program was scheduled for the patient and the rest of the pregnancy was uneventful. Postnatal, thorax computer tomography and transthoracic echocardiography (TTE) demonstrated PLSVC and bovine aortic arch associated with hypoplastic transverse aortic arch. Routine echocardiographic examinations revealed that the blood flow of the aortic arch had increased gradually, and the male infant's aortic arch had significantly widened and reached the normal range until the baby was discharged from the hospital. CONCLUSION: DV agenesis and PLSVC are usually benign conditions but underlying serious heart diseases may accompany them. Therefore, in situations like ours, a prenatal aortic arch evaluation is of capital importance. Postnatal hemodynamic changes should be taken into consideration in the management of these cases. This is the first example in the literature that these abnormalities co-existed in one case.

The Effects of Maternal Smoking on Thyroid Function: Findings from Routine First-Trimester Sonographic Anomaly Screening.

AIM: This study aimed to assess the effect of tobacco exposure on maternal thyroid function and investigate its relationship to subclinical hypothyroidism in pregnant women during the first trimester. SUBJECTS AND METHOD: A comparison of maternal thyroid function was made on 45 smokers, who composed the study group, and 72 non-smokers, pregnant women, who constituted the control group. After determining smokers by questionnaire, carbon monoxide (CO) levels in the expiratory air of the participants in both groups were measured and recorded, and the smokers' exposure was objectively confirmed. RESULTS: Smoking and non-smoking pregnant women were similar regarding body mass index (BMI). While the TSH and fT4 levels were respectively 1.48 mlU/L and 11.43 pmol/L in pregnant women who smoked, that ratio changed to 1.72 mlU/L and 11.17 pmol/L in the non-smokers' group. But the differences between the groups were not statistically significant (p=0.239, p=0.179). Even though the rate of subclinical hypothyroidism was 8.9% in the smoking group, it was approximately 19.4% in the non-smoker group; the difference was not statistically significant (p=0.187). CONCLUSION: This study proved that there is no statistically significant difference between maternal serum TSH and fT4 levels and the rate of subclinical hypothyroidism in smokers during pregnancy in the first trimester.

MRI evaluation of right heart functions in children with mild cystic fibrosis.

BACKGROUND: This study aimed to assess the ventricular anatomy, function of the right ventricle, and the haemodynamic findings of pulmonary artery in children with cystic fibrosis using cardiac MRI. PATIENTS: This prospective study consisted of 32 children with mild cystic fibrosis and 30 age-matched healthy control participants. METHODS: Cardiac MRI was used to assess right ventricular volumes, anatomy, and function and to assessment of haemodynamic findings of pulmonary artery in the control and study groups. Haemodynamic findings of pulmonary arteries were determined using pulmonary arteries peak velocity (cm/s), and pulmonary arteries time-to-peak velocity (ms) and pulmonary artery systolic pressure. All data of children with mild cystic fibrosis were compared with those of 30 age-matched healthy control group participants. RESULTS: Our patients and their age-matched controls were aged from 6 to 17 years and from 7 to 15 years, respectively. We found that ejection fraction (%), cardiac output (L/ml), cardiac output (L/ml/m2), and systolic volume (ml/m2) were significantly lower in children with cystic fibrosis (p < 0.01). Right ventricular anterior wall thickness (mm) was significantly higher in children with cystic fibrosis (p = 0.01). No significant difference was observed between the haemodynamic parameters of pulmonary artery in the patient group. CONCLUSION: In our study, cardiac MRI was used to investigate whether the right ventricle was affected functionally and anatomically in children with mild cystic fibrosis. We detected a significant decrease in right ventricular systolic functions and notable alterations in the right ventricular geometry of children with mild cystic fibrosis. These alterations usually manifest themselves as hypertrophy of the right ventricle. Our study's results demonstrate no relationship between the development of pulmonary hypertension in mild cystic fibrosis children.

Development of Antiarrhythmic Therapy-Resistant Ventricular Tachycardia, Ventricular Fibrillation, and Premature Ventricular Contractions in a 15-Year-Old Patient.

Sudden cardiac arrest (SCA) is the sudden cessation of regular cardiac activity so that the victim becomes unresponsive, with no signs of circulation and no normal breathing. Asystole, ventricular tachycardia (VT), ventricular fibrillation (VF), and pulseless electrical activity are the underlying rhythm disturbances in the pediatric age group. If appropriate interventions (cardiopulmonary resuscitation-CPR and/or defibrillation or cardioversion) are not performed rapidly, this condition progresses to sudden death. There have not been many reported cases of the approach and treatment of cardiac arrhythmias after SCA. Herein, we would like to report a case of a 15-year-old female patient with dilated cardiomyopathy (DCM) who was admitted to our clinic a year ago, and while her left ventricular systolic functions were improved, SCA suddenly occurred. Since the SCA event occurred in another city, intravenous treatment of amiodarone was done immediately and was switch to continuous infusion dose of amiodarone until the patient arrived at our institution's pediatric intensive care unit (PICU) 3 hours later. During the patient's 20-day PICU hospitalization, she developed pulseless VT and VF from time to time. The patient's pulseless VT and VF attacks were brought under control by the use of a defibrillator and added antiarrhythmic drugs (amiodarone, flecainide, esmolol, and propafenone). Intriguingly, therapy-resistance bigeminy with premature ventricular contractions (PVCs) continued despite all these treatments. The patient did not have adequate blood pressure measured by invasive arterial blood pressure monitoring while having bigeminy PVCs. The intermittent bigeminy PVCs ameliorated rapidly after intermittent boluses of lidocaine. In the end, multiple antiarrhythmic therapies and intermittent bolus lidocaine doses were enough to bring her cardiac arrhythmias after SCA under control. This case illustrates that malign PVC's should be taken very seriously, since they may predispose to the development of VT or VF. Also, this case highlights the importance of close vigilance of arterial pressure tracings of patients with bigeminy PVCs which develop after SCA and should not be accepted as normal.

Evaluation of executive functions in children with rheumatic heart diseases.

BACKGROUND: Acute rheumatic fever (ARF) is a multisystemic inflammatory disease in children and young adults. The most notable complications of ARF are rheumatic heart disease (RHD) and Sydenham's chorea (SC). There have been many reports about executive dysfunctions with children who have SC. "Executive function" is an umbrella term that is used to describe higher level cognitive functions. The aim of this study is to determine the executive functions of children with RHD. We evaluated executive functions in healthy children with the same sociodemographic characteristics as children with RHD. METHODS: Our study was designed as a cross-sectional randomized study, including children with RHD aged between 12 and 18, and healthy controls. The difference between the patient and control group participants in terms of age, gender, education level, education level of the parents, family income level, and executive functions were investigated. Executive functions composed of Digit Sequence Test, Verbal Fluency Test, Trail-Making Test, Stroop Test, Wisconsin Card Sorting Test. RESULTS: In our study, a total of 30 children with RHD were followed up at the pediatric cardiology outpatient clinic of Bezmialem Vakif University Hospital composed the patient group. The control group was made up of 30 healthy children of the same sex and age group as the patient group. The mean age of the case group was 14.73 ± 1.84 years. The Digit Span Test, Verbal Fluency Test, Trail-Making Test, Wisconsin Card Sorting Test, and Stroop Test produced no statistically significant differences between the RHD patients and the controls. CONCLUSIONS: No statistically significant difference was found between the RHD patients and control patients in any executive function test. It was suggested that executive dysfunction might not develop in RHD patients before developing SC.

Comparison of ultrasonographic and mammographic features of extremely rare papillary carcinoma and invasive ductal carcinoma.

Introduction: This study aimed to investigate and compare ultrasonographic and mammographic findings of papillary breast carcinoma and invasive ductal carcinoma in breast masses that were diagnosed as pathological. Material and methods: This retrospective study included 88 patients with breast lesions, who underwent ultrasonography, mediolateral oblique-craniocaudal, and tomosynthesis imaging in the Picture Archiving and Communication System between January 2010 and March 2019. Results: 44 histopathologically diagnosed papillary carcinoma patients and 44 invasive ductal carcinoma patients were divided into groups according to contour, shape, internal structure, calcific-cystic component, echogenicity, posterior acoustic change, skin orientation, and environmental echogenic halo. There was a statistically significant difference between the groups in mammography contour, U/S contour, U/S shape, U/S posterior acoustics, and U/S internal structure. Logistic regression analysis showed that the presence of homogenous appearance (p < 0.001) and absence of shading in the posterior acoustic U/S (p = 0.001) were the most pertinent findings for determining papillary carcinoma. In the U/S, the likelihood of a homogenous tumour being a papillary carcinoma was 16.869 times higher than that of invasive ductal carcinoma, whereas the same probability was 0.1101 times less for a tumour with posterior acoustic shadowing. Conclusions: It is challenging to differentiate between invasive ductal carcinoma and papillary carcinoma of the breast without histopathological diagnosis both on ultrasound and mammography. The results of our study demonstrated that the ultrasonographic and mammographic findings of invasive ductal carcinoma and papillary carcinoma were like each other. Therefore, it is still not possible to distinguish between these 2 types of cancer only in accordance with these 2 criteria.

Lifesaving Treatment of Aortic Valve Staphylococcus aureus Endocarditis: Daptomycin and Early Surgical Therapy.

Infective endocarditis (IE) is an uncommon infection in children. The recommended treatment for native valve endocarditis secondary to methicillin-susceptible Staphylococcus aureus infection is antistaphylococcal penicillins such as nafcillin or oxacillin. If the initial therapy fails in IE, it can lead to catastrophic results. Nowadays, daptomycin is the best alternative antimicrobial agent to treat children with severe infections, when standard antimicrobial therapy does not yield a result. Herein, in this article, we described a case of a 16-year-old boy who had aortic valve S. aureus endocarditis with septic embolization and stroke. The patient was successfully treated only with daptomycin as well as surgical therapy in the early phase of the infection.

A fatal interstitial lung disease in an anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody negative patient with juvenile dermatomyositis.

BACKGROUND: Juvenile dermatomyositis associated interstitial lung disease, rarely seen in pediatric age groups, has adverse effects on survival. Anti-melanoma differentiation associated gene 5, one of the identified autoantibodies in juvenile dermatomyositis, preferentially affects the lung tissue and may cause rapidly progressive interstitial lung disease. It is a major cause of mortality in juvenile dermatomyositis. In this case report, we present a pediatric patient diagnosed with juvenile dermatomyositis without anti-melanoma differentiation associated gene 5 antibody positivity. CASE: A six-year-old male patient admitted to the Pediatric Intensive Care Unit with symptoms of respiratory failure, 1.5 months after the diagnosis of juvenile dermatomyositis. Thorax computed tomography examination revealed pneumomediastinum, a trace of left-sided pneumothorax, atelectasis on the left posterior lung region, ground-glass opacity, minimal subpleural patchy consolidation, and subcutaneous emphysema especially on the sides of the chest wall. Broad-spectrum antibiotics were started. His nasal swab sample was positive in terms of influenza B; therefore, oseltamivir was added to the treatment. Autoimmune myositis antibodies panel was examined but all of them including anti-melanoma differentiation associated gene 5 antibody resulted as negative. There was no notable reduction in lung infiltrations with the patient`s current treatment regimen. On the 12 < sup > th < /sup > day of Pediatric Intensive Care Unit admission, thorax computed tomography scan revealed progressed radiological lung findings compatible with rapidly progressive interstitial lung disease secondary to juvenile dermatomyositis. Despite intensive medical and extracorporeal treatments such as pulse steroid, intravenous immunoglobulin, methotrexate, cyclophosphamide, rituximab, therapeutic plasma exchange and, extracorporeal membrane oxygenation, the patient died on the 35 < sup > th < /sup > day. CONCLUSIONS: Juvenile dermatomyositis patients should be carefully monitored for the development of interstitial lung disease. Rapidly progressive interstitial lung disease with a high mortality may develop shortly after diagnosis, even if the anti-melanoma differentiation associated gene 5 antibody is negative.

Role of soluble triggering receptor expressed in myeloid cells-1 in distinguishing SIRS, sepsis, and septic shock in the pediatric intensive care unit.

BACKGROUND: Research into new markers has been intensified for early diagnosis, prognosis, and differentiation of SIRS, sepsis, and septic shock in recent years. This study aimed to investigate the role of soluble triggering receptor expressed in myeloid cells-1 (sTREM-1) and interleukin (IL)-6 in distinguishing between systemic inflammatory response syndrome (SIRS), sepsis, and septic shock in pediatric intensive care unit (PICU) patients. METHODS: Between June 2014 and July 2015, 90 consecutive patients who were treated in the PICU were included in this prospective observational study. Patients were divided into four groups: control (n = 23), SIRS (n = 22), sepsis (n = 23), and septic shock (n = 22). All patients were evaluated for white blood cell (WBC), serum C-reactive protein (CRP), procalcitonin (PCT), IL-6, and sTREM-1 levels at 0, 24, and 72 h of admission. The prognostic evaluations were made using the Pediatric Risk of Mortality III (PRISM III) and Pediatric Logistic Organ Dysfunction (PELOD) scores. Patients were evaluated in terms of age, gender, prognosis, pathogen growth in culture, PRISM III and PELOD score, WBC, CRP, PCT, IL-6, and sTREM-1 levels and a comparison was made between groups. RESULTS: There was no significant difference between all groups in terms of the 0-, 24-, and 72-h sTREM-1 values (p = 0.761, p = 0.360, and p = 0.822, respectively). CRP and PCT values did not differ between the septic shock, sepsis, and SIRS groups at 0, 24, and 72 h. In the septic shock group, the 0-h IL-6 value was significantly higher than that of the SIRS group (p = 0.025). The 24-h IL-6 value in the septic shock group was significantly higher than the values of the sepsis and SIRS groups (p = 0.048 and p = 0.043, respectively). No significant difference was detected between the septic shock, sepsis, and SIRS groups in terms of IL-6 values at 72 h. CONCLUSION: sTREM-1 is not useful for the diagnosis of infection and for distinguishing between sepsis, septic shock, and SIRS since it does not offer a clear diagnostic value for PICU patients, unlike other reliable markers such as WBC, CRP, and PCT. Elevated IL-6 levels may indicate septic shock in PICU patients. More research on sTREM-1 is needed in this setting.

Acute Myocarditis and Eculizumab Caused Severe Cholestasis in a 17-Month-Old Child Who Has Hemolytic Uremic Syndrome Associated with Shiga Toxin-Producing Escherichia coli.

Cardiovascular involvement is uncommon in pediatric patients with hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli (STEC-HUS). In this case report we presented a case of 17-month-old toddler who had a sporadic type of STEC-HUS complicated by acute myocarditis. The patient was successfully treated by a single dose of eculizumab after six doses of therapeutic plasma exchange (TPE) were inefficient to prevent the cardiac complication. Hepatotoxicity was observed after a single dose of eculizumab. Hepatic and cholestatic enzyme levels slowly returned to normal within 6 months. To the best of our knowledge, this is the first case of myocarditis/cardiomyopathy treated with eculizumab in STEC-HUS. This case illustrates the need for vigilance regarding myocardial involvement and eculizumab-induced hepatotoxicity in STEC-HUS.

The Possible Role of IVIG in the Treatment of Atrial Fibrillation Accompanied by Fulminant Myocarditis in a 12-Year-Old Pediatric Patient.

BACKGROUND: Fulminant myocarditis (FM) is a potentially lethal condition in children due to rapid progressive hemodynamic instability and cardiogenic shock. Patients with FM might show different clinical manifestations on emergency department admission. CASE: Herein, we describe the case of a 12-year-old girl who was admitted to our institution's emergency department due to complaints of abdominal pain and incessant vomiting. However, we detected an early onset of atrial fibrillation (AF) accompanied by FM. The patient's condition of AF and severe hemodynamic disorder was successfully treated in our institution's pediatric intensive care unit. CONCLUSION: To the best of our knowledge, this is the first report of the co-occurrence of FM and AF successfully treated in childhood. This case report will serve as a guide for the treatment of cases with FM accompanied by AF.

Subdural empyema, brain abscess, and superior sagittal sinus venous thrombosis secondary to Streptococcus anginosus.

Streptococcus anginosus can be frequently isolated from brain abscesses, but is a rare cause of the liver, lung, and deep tissue abscesses. In this report, we present a patient with subdural empyema, brain abscess, and superior sagittal cerebral venous thrombosis as complications of rhinosinusitis whose purulent empyema sample yielded S. anginosus. A 13-year-old female patient was referred to our pediatric intensive care unit with altered mental status, aphasia, and behavioral change. On a brain computed tomography scan, subdural empyema extending from the left frontal sinus to the frontal interhemispheric area and left hemispheric dura was detected. Intravenous ceftriaxone, vancomycin, and metronidazole treatments were started. Subdural empyema was surgically drained. Postoperative brain magnetic resonance venography imaging showed superior sagittal sinus thrombosis. Cultures obtained from purulent empyema sample revealed S. anginosus. On the third day of hospitalization, a brain computed tomography scan showed brain edema, especially in the left hemisphere and significantly increased subdural empyema that had been previously drained. She was reoperated and decompressive craniectomy was performed. On the fifth day, partial epileptic seizures occurred. Brain magnetic resonance imaging showed a brain abscess on the interhemispheric area. The magnetic resonance imaging findings of abscess formation improved on 30th day and she was discharged on the 45th day after the completion of antibiotic therapy.

Balanced Double Aortic Arch Causing Persistent Respiratory Symptoms Mimicking Asthma in an Infant.

Double aortic arch (DAA) is a common form of complete vascular ring. The condition leads to airway obstruction and compression of the esophagus. A balanced type of DAA is an extremely rare anomaly. The anatomical aberration cannot be easily distinguished by transthoracic echocardiography when an infant has chronic respiratory distress. Herein we presented a case of an 11-month-old infant who had chronic respiratory distress and a balanced type of DAA.

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.