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.

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.

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.

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.

A Rare Presentation of Neurobrucellosis in a 6-Year-Old Pediatric Patient with Sagittal Sinus Thrombosis.

Brucellosis is one of the most common zoonosis worldwide. It is still endemic in many regions of the world. A 6-year-old female was admitted to the emergency department (ED) due to a sudden change in consciousness, urinary incontinence, vomiting, and difficulty in walking. Neurological examination demonstrated abducens nerve paralysis, mild-to-moderate motor deficit in hemiparesis in the left arm. Brain magnetic resonance imaging showed a hemorrhagic focus at the right frontal lobe and thrombosis in the superior sagittal sinus of the brain. The diagnosis of neurobrucellosis was confirmed by identifying Brucella spp. in the blood culture on the day 6 of pediatric intensive care unit admission; thus, trimethoprim-sulfamethoxazole and rifampicin, and ceftriaxone were promptly initiated. Despite neuroprotective management and acetazolamide, the patient's neurological problems and high intracranial pressure (ICP) persisted. An external ventricular drainage tube and a Codman ICP monitor were placed to be on the consent vigilance of the patient's neurological condition. The patient's ICP continued to increase despite the current treatment regimen; therefore, a decompressive bitemporal craniectomy was performed. The ICP level of the patient returned to its normal range immediately after the craniectomy. The patient did not have any notable neurologic sequelae at the first-year follow-up. Neurobrucellosis is a rare complication of systemic brucellosis and may present as meningitis, encephalitis, myelitis, radiculitis, and/or neuritis. Herein, we describe a six-year-old girl with brucellosis complicated with cerebral vein thrombosis. This case illustrates the need for close monitoring of patients with unexplained neurological signs or symptoms for brucellosis in endemic areas.

A Snapshot of Pediatric Patients with COVID-19 in a Pandemic Hospital.

AIM: Reports describing coronavirus disease 2019 (Covid-19) in children are fewer than adult studies due to milder clinical picture. We aimed to share our experience at a single center with an emphasis on collective decision making. MATERIALS AND METHODS: A suspected case was defined as the presence of symptoms suggestive of COVID-19 and/or positive contact history. SARS-CoV-2 PCR positive patients were defined as confirmed COVID-19. Between March 12, 2020, and May 15, 2020, all children presenting with fever, cough, or respiratory difficulty were investigated for COVID-19. A total of 719 children were examined at outpatient clinics, and 495 were tested with polymerase chain reaction (PCR) for suspicion of COVID-19. A team was organized for monitoring and treating patients either as outpatients or hospitalization. Patients were evaluated in terms of age, gender, travel history, epidemiological history, clinical symptoms and signs, laboratory and radiological findings, treatment, and outcome. RESULTS: Sixty patients were hospitalized for suspicion of COVID-19. Forty-three patients were diagnosed as probable or confirmed COVID-19. 21 of 43 patients (48.8%) were PCR confirmed. The remaining 22 were diagnosed by epidemiologic history, clinical assessment, and computerized thorax tomography (CT) findings. The median age was 126 and 78.5 months in PCR positives and PCR negatives, respectively and the youngest patient was a 28 days old baby. Nineteen of the patients had an upper respiratory infection (44.1%). Although five patients had no clinical signs, chest X-ray, or CT revealed pneumonia. CONCLUSIONS: As previously reported, the clinical manifestations of COVID-19 in children are mostly mild. Even very young kids can become infected following exposure to sick family members. International and local guidelines are valuable for decision making since it is a new disease. A combination of chest disease, infectious diseases, and emergency care physicians approach will aid the appropriate management of cases.

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.

Persistent pleural effusion in an infant with an unusual diagnosis: congenital alveolar rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is a malignant form of neoplasm that originates from skeletal muscle. RMSs can exist anywhere in the human body but are more commonly detected in the neck region and extremities. The alveolar type is one of the subtypes of RMS that has a poor prognosis. Because the clinical manifestation of a tumour can be a painless mass, symptoms might be non-contributary to the diagnosis. Herein, a four-month-old girl was admitted to the emergency department with complaints of respiratory distress without a runny nose, cough, and fever. Recurrent effusions subsided with subsequent tube thoracostomy. Video-assisted thoracoscopic surgery (VATS) was performed to determine the aetiology of the recurrent effusion. The Tru-Cut biopsy obtained during VATS resulted in the diagnosis of alveolar rhabdomyosarcoma. Pleural effusion decreased, and the tube drainage was stopped rapidly after first vincristine, actinomycin-D, and cyclophosphamide chemotherapy cycle. Persistent and recurrent pleural effusions should alert physicians to rule out unusual diagnoses like that of our case.

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.