Interrelationship between liver T2*-weighted magnetic resonance imaging and acoustic radiation force impulse elastography measurement results and plasma ferritin levels in children with ß-thalassemia major.

PURPOSE: To evaluate correlation and agreement between T2*-weighted magnetic resonance imaging (T2*-wMRI), acoustic radiation force impulse elastography (ARFI-e) measurement results of liver and plasma ferritin levels (PFLs) in children with ß-thalassemia major (ß-TM). METHODS: The study included 40 pediatric patients (aged 64-216 months; 14 girls, 26 boys) receiving blood transfusion and chelation therapy. To detect the severity of liver iron overload (LIO) and concomitant parenchymal fibrosis, T2*-wMRI and ARFI-e measurements were performed from the right lobe segments. Student's t-test, Mann-Whitney U, ANOVA, Spearman's test and ICC were used for statistical analysis. RESULTS: After the measurements of T2*-wMRI, patients were grouped as normal in 4 (10%), mild in 11 (27.5%), moderate in 21 (52.5%), and severe in 4 (10%) cases in terms of LIO. Combined moderate and severe groups had significantly higher ARFI-e and PFL values than the combination of other groups (p = .001, p = .040). The ARFI-e measurements of boys were found to be significantly higher than those of girls (p = .023). A strong negative correlation between T2*-wMRI and ARFI-e and a moderate negative correlation between T2*-wMRI and PFL were detected (p;r = 0.001;-0.606, p;r = 0.009; -0.407). A strong positive correlation was found between ARFI-e values and PFL (p;r = 0.001; 0.659). The optimal cut-off value of ARFI-e to predict liver fibrosis because of moderate&severe LIO was determined to be 1.29 M/s (80% sensitivity and 88% specificity). A moderate agreement was observed between the T2*-wMRI and ARFI-e methods [ICC: 0.680, 95% CI: (0.470 to 0.817)]. CONCLUSION: Given the strong correlation and moderate agreement between ARFI-e and T2*-wMRI, ARFI -e could be used to monitor LIO in children with ß-TM.

A Mathematical Model to Predict Human Microcephaly.

BACKGROUND: Microcephaly (MiC) is defined as head circumference (HC) dimensions smaller than the normal standards. OBJECTIVE: To detect MiC with a mathematical formula. MATERIALS AND METHODS: The 0 to 5 years head HC percentile data for girls and boys reported by the World Health Organization were used. To assess early childhood, these growth standards are available on its website for international use. Mathematical formulas best estimating the 3rd percentile curves were defined using basic regression analysis methods. RESULTS: The mathematical models obtained as a result of logarithmic regression analysis with the highest coefficient of determination values (R2 = 0.991 for girls; R2 = 0.991 for boys) were identified as the best model. The formulas of HC = 34.025 + 3.283 ∗ ln (age as months), and HC = 35.475 + 3.14 ∗ ln (age as months) were determined for girls and boys, respectively. A limitation of these formulas is that they do not provide the HC at birth (ln (0) = undetermined). CONCLUSION: Microcephaly can be estimated using the mathematical formulas with a calculator without using percentile scales, mobile applications, software, or the Internet.

Posterior fossa horns; a new calvarial finding of mucopolysaccharidoses with well-known cranial MRI features

Background/aim: Mucopolysaccharidoses (MPS) are a group of hereditary metabolic diseases. The aim of this study was to share the previously unreported calvarial finding of internal hypertrophy of the occipitomastoid sutures (IHOMS) together with some other well-known cranial MRI findings in this patient series. Materials and methods: A retrospective evaluation was conducted of 80 cranial MRIs of patients who had been diagnosed and followed up with MPS from 2008 to 2019 in our center. Of these patients, 11 had Hurler, 14 had Hunter, 24 had Sanfilippo, 15 had Morquio, 14 had Maroteaux­Lamy, and 2 had Sly disease. The cranial MRIs were assessed in two main groups as parenchymal intradural cranial MRI findings and extradural calvarial findings. Results: The most common parenchymal intradural cranial MRI findings were white matter signal alterations (n = 51, 63%) and perivascular space enlargements (n = 39, 48%). The most common extradural calvarial findings were J-shaped sella (n = 45, 56%) and tympanic effusion (n = 44, 55%). Although IHOMS was defined in a relatively small number of the patients (n = 12, 15%), the prevalence rate was high in MPS type I (n = 6, 54%). Conclusion: The abnormal cranial MRI findings of the MPS patients, including the newly identified IHOMS, may provide diagnostic clues to differentiate the type of the disease in radiological imaging.

Hepatic Complications of Umbilical Venous Catheters in the Neonatal Period: The Ultrasound Spectrum.

OBJECTIVES: Umbilical venous catheterization is commonly used in the neonatal period; however, it has some complications. In this study, we evaluated neonates who underwent umbilical venous catheterization and developed hepatic complications. Furthermore, we aimed to define all of the possible lesions and to clarify the imaging findings of umbilical venous catheter-induced hepatic injury. METHODS: Two hundred forty-four neonates who underwent umbilical venous catheterization between March 2013 and September 2015 in a single tertiary care referral center were included in this study. To determine whether they had any hepatic complications, all patients underwent abdominal grayscale and Doppler ultrasound examinations, and their clinical data were recorded. RESULTS: The frequency of liver-related complications from umbilical venous catheterization was 33.6% (82 of 244). Air in the portal venous system was the most frequent complication (20.1% [49 of 244]). Left portal venous thrombosis was noted in 6.1% (15 of 244). Parenchymal lesions in the liver related to umbilical venous catheterization were seen in 7.4% of patients (18 of 244) as follows: single nodular echogenic lesions (4.1% [10 of 244]), branching small nodular echogenic lesions (2.1% [5 of 244]), and large irregular heterogeneous lesions with laceration and perihepatic fluid (1.2% [3 of 244]). There was no statistical significance for any type of complication according to the gestational age (P > .05). CONCLUSIONS: Hepatic complications due to umbilical venous catheters are not uncommon in the neonatal period. Ultrasound is the best imaging modality for confirming the diagnosis and for follow-up.

A Newborn with an Alternative Porto-Caval Shunt.

BACKGROUND: Absent ductus venosus (ADV) is a rare condition, but it should be known that this embryonic anomaly may be detected by fetal echocardiographic or newborn ultrasound examinations. CASE REPORT: We present a baby with an ADV and an accompanying alternative porto-caval shunt between the right portal vein and inferior vena cava detected on postnatal ultrasound examination. CONCLUSIONS: Variations in the fetal umbilical or porto-systemic circulations should be detected by fetal or newborn ultrasound examinations and kept in mind before common interventions such as UV catheterizations.

The effect of alfuzosin on renal resistive index, urinary electrolytes and ß2 microglobulin levels and TGF ß-1 levels of kidney tissue in rats with unilateral ureteropelvic junction obstruction.

BACKGROUND: In this study, it was aimed to determine the effects of alfuzosin on experimentally generated unilateral partial ureteropelvic junction obstruction (UPO) in rats. MATERIALS AND METHODS: Thirty Long-Evans rats were randomly allocated into five groups. In control group (C), nothing was performed; in group Sham (S) only laparotomy was done; in Alfuzosin group (A) only alfuzosin was administered for two weeks (10 mg/kg/day p.o.) without any surgery; in UPO group, unilateral UP junction obstruction was produced; and in the Group UPT (ureteropelvic obstruction + treatment), alfuzosin was administered for two weeks (10 mg/kg/day p.o.) in addition to UPO production. Renal pelvic anteroposterior diameters were determined with ultrasonography (USG) and renal arterial resistivity indexes by color Doppler USG. Urine was collected both at the beginning and at the end of the experiment for 24 h in all the groups and at the end of the experiment, blood samples were obtained. Blood and urine electrolytes and TGF-ß1, urine density, urine ß2 microglobulin levels were determined. Renal tissue samples harvested from all of the rats were histopathologically evaluated. Results were determined using one-way ANOVA t-test; p < 0.05 was accepted as significant. RESULTS: Urine density in the UPT group was lower with respect to UPO group and blood electrolytes were preserved as close to normal (p < 0.05). In the UPT group, urine TGF-ß1 and blood TGF-ß1, blood ß2 microglobulin levels and histopathologic damage scores were lower compared to the UPO group (p < 0.05). CONCLUSION: It is shown in this experimental unilateral partial UPO model that alfuzosin treatment prevents obstructive renal damage.

Radiologic findings of Patterson-Lowry rhizomelic dysplasia in two sisters.

We report two sisters who have a rare skeletal abnormality termed Patterson-Lowry rhizomelic dysplasia. The typical findings of these cases on bone survey are isolated shortening and proximal metaphyseal enlargement and cupping of the bilateral humeri. The elder sister also has coxa vara deformity and dysplastic proximal femoral epiphyses on both sides. The younger sister has normal hip joint bones bilaterally, but her proximal femoral epiphyses are smaller than normal. All other bones of the sisters are of normal size and configuration. Our patients are two siblings, and their parents are first degree relatives, suggesting autosomal-recessive (AR) inheritance. The present patients help us to understand the genetic relationships and skeletal variabilities of this rare entity.

Evaluating Postoperative Outcomes and Investigating the Usefulness of EU-TIRADS Scoring in Managing Pediatric Thyroid Nodules Bethesda 3 and 4

Objective: The aim was to assess postoperative outcomes in pediatric thyroid nodules with atypia of undetermined significance (AUS/FLUS) or suspicious for a follicular neoplasm (SFN) and their respective the European-Thyroid Imaging Reporting and Data System (EU-TIRADS) scores. Methods: Forty-four pediatric patients at a single center with thyroid nodules classified as AUS/FLUS or SFN from August 2019 to December 2022 were retrospectively reviewed. Data on demographics, thyroid function, nodule size, and ultrasonographic features were collected. Postoperative pathologies were categorized into benign, low-risk, and malignant neoplasms according to the World Health Organization 2022 criteria, and EU-TIRADS was used for retrospective radiological scoring. Results: Among 21 (47.7%) of patients who had surgical intervention, 72% had Bethesda 3 and 28% had Bethesda 4 thyroid nodules. Post-surgical histopathological classifications were 43% benign, 19% low-risk, and 38% malignant. Of note, EU-TIRADS 3 and 5 scores were present in 44% and 56% of the benign cases, respectively. Malignant cases tended to produce higher EU-TIRADS scores, with 64% rated as EU-TIRADS 5. Bethesda category 4 nodules had a 66% malignancy rate, significantly higher than the 27% in category 3. Conclusion: A substantial proportion of histologically benign cases were classified as EU-TIRADS 5, suggesting that EU-TIRADS may lead to unnecessary biopsies in benign cases. Malignant cases were more likely to have a higher EU-TIRADS score, indicating a positive correlation with malignancy risk, particularly in Bethesda 4 cases. However, the EU-TIRADS system's predictive value for malignancy in Bethesda 3 cases was poorer.

Detection of prostate cancer with magnetic resonance imaging: optimization of T1-weighted, T2-weighted, dynamic-enhanced T1-weighted, diffusion-weighted imaging apparent diffusion coefficient mapping sequences and MR spectroscopy, correlated with biopsy and histopathological findings.

PURPOSE: The aim of this study was to optimize and predict the most efficient magnetic resonance imaging (MRI) sequences; T1-weighted (T1W), T2-weighted (T2W), dynamic contrast-enhanced (DCE) T1W, diffusion-weighted imaging (DWI) apparent diffusion coefficient (ADC) mapping sequences and proton MR spectroscopy (H-MRS) for the detection of prostate cancer. MATERIALS AND METHODS: After institutional review board approval and informed consent taken from all the patients, 40 patients with prostate cancer were included in this research. Two readers independently evaluated the results of T1W, T2W, DCE T1W, and DWI-ADC mapping sequences and proton H-MRS for the depiction of prostate cancer. Reference standard was the transrectal ultrasonography-guided biopsy and the surgical histopathological results. Statistical analysis was assessed by the Fisher exact t test, Wilcoxon signed rank test, variance analysis test with kappa (κ) values and receiver operating characteristics (ROC) curve for ADC values, choline (Cho)/citrate (Cit) and Cho+creatine (Cre)/Cit ratios for each observer. RESULTS: Based on both readers' results, sensitivity declined to 31% and specificity to 75% for the T1W sequence, sensitivity declined to 43% and specificity to 67% for the DCE T1W sequence, sensitivity declined to 46% and specificity to 68% for the T2W sequence, sensitivity declined to 29% and specificity to 82% for the DWI-ADC mapping; and specificity was 49% for the Cho/Cit and Cho+Cre/Cit ratios, sensitivity was 69% for the Cho/Cit ratio, and sensitivity was 70% for the Cho+Cre/Cit ratio for H-MRS. The T2W sequence and H-MRS presented significant statistical differences for the depiction of prostatic cancer (P < 0.05), the most efficient sequence to detect prostatic cancer was H-MRS: Cho+Cre/Cit and Cho/Cit ratios. CONCLUSION: Instead of using either sequences alone owing to low sensitivity and specificity rates, combined use of MRI techniques could easily improve the detection and staging of prostate cancer.

Evaluation of aortic arch morphologies by computed tomographic angiography in Turkish population.

Background: The aim of this study was to evaluate the aortic arch morphologies in the Turkish population using the computed tomography angiography technique. Methods: Between August 2009 and August 2019, a total of 2,037 (1,003 males, 1,034 females; mean age: 52.8±20.3 years; range, 3 months to 100 years) thoracic computed tomography angiography scans were retrospectively analyzed. The findings were classified as described previously in the literature. The prevalence of aortic arch morphologies and possible relationship with sex were analyzed. The prevalence of variations reported in previous studies was compared with the current study. Results: The normal aortic arch pattern (type A), observed in 1,562 cases (76.7%), was determined statistically significantly more in males than females (p<0.05). The most common variation, bovine aortic arch (type B1) which observed in a total of 315 cases (15.5%), was determined statistically significantly more in females than males (p<0.05). The second most frequent variation, in which the left vertebral artery originates directly from the aortic arch (type C1) was detected in 97 cases (4.7%). There was also observed to be aberrant right subclavian artery in 21 cases (1%), right-sided aortic arch variation in seven cases (0.4%), and double aortic arch anomaly in four cases (0.1%). In terms of the reported frequency of type B variation, a significant difference was determined between the current and previous studies in Türkiye (p<0.05). Conclusion: With the largest sample size to date, this study provides comparative information about the prevalence of aortic arch patterns in the Turkish population.

Relationship between Quantitative Sonographic Measurements and Serum Biochemical Parameters in Childhood Obesity.

PURPOSE: We investigated the relationship between sonographic measurements of fatty liver and body mass index standard deviation score (BMI-Z score), abdominal wall fat thickness (AWFT), and serum biochemical parameters in childhood obesity. METHODS: Anthropometric, laboratory, and ultrasonography data were obtained from 174 children with BMI-Z score >1. After the qualitative grading of hepatosteatosis (grades 0-3), the quantitative liver-kidney echogenicity ratio (LKER) was calculated using a software tool. Groups according to sex, age (AG-I to AG-III), BMI-Z score (BMG-I to BMG-III), and hepatosteatosis degree (HS-I and HS-II) were formed. The differences and distributions of the variables were statistically analyzed and compared among the groups. RESULTS: Serum transaminase and glucose levels showed a positive correlation with LKER, whereas the HDL level showed a negative correlation. BMI-Z score and AWFT showed a positive correlation with fasting insulin level and HOMA-IR value. LKER was significantly higher in girls than in boys (p=0.008). In the AG-I group (age 3-8.9 years), the BMI-Z score was significantly higher, whereas AWFT was significantly lower than in the other age groups (p<0.001). The cutoff point of LKER for predicting grade 2 or higher steatosis (HS-II group) was determined to be 1.83. Cardiovascular disease risk was significantly higher in the HS-II group (p=0.035). CONCLUSION: As a valuable quantitative measurement tool, LKER can be used for the sonographic screening of fatty liver. AWFT, on the basis of its correlation with fasting insulin level and HOMA-IR value, may be a useful sonographic parameter in the management of childhood obesity.

Overview of the Pulmonary Manifestations in Patients with Autosomal Recessive Cutis Laxa Type IC.

Background: Autosomal recessive cutis laxa type IC (ARCL1C) is characterized by cutis laxa accompanied by pulmonary, gastrointestinal, urinary, musculoskeletal involvement caused by biallelic mutations in latent transforming growth factor-beta binding protein 4 (LTBP4) gene. The overall prognosis is poor, and most patients die in infancy because of severe pulmonary emphysema (PE). Aim: We aimed to evaluate 3 ARCL1C patients, 2 of whom are still alive and in their childhood period, from 2 unrelated families with novel LTBP4 mutations, to demonstrate the clinical variability of pulmonary involvement. Materials and Methods: Three children who were molecularly confirmed by LTBP4 sequencing analysis were comprehensively reviewed in terms of pulmonary manifestations through chest examination, lung function tests (LFTs), chest X-ray, and thorax computed tomography. Results: Family 1 (c.3740A>G LTBP4 mutation): A 5-year-old male patient with pulmonary artery stenosis (PAS) presented with persistent cough and exhibited mild restriction on LFT. Family 2 (c.2T>G LTBP4 mutation): Radiographic examinations revealed PE in a 7-year-old female patient who was operated for diaphragmatic hernia. She had recurrent bronchiolitis and pulmonary infections. LFT revealed both obstructive and restrictive pattern. Her cousin also had respiratory distress with the onset of the newborn period and died due to bilateral pneumothorax in early infancy. Conclusion: The variable severity of pulmonary findings was shown in these patients. It should also be kept in mind that there could be intrafamilial variability of systemic manifestations. Although obstructive lung disease is expected to be seen in ARLC1C patients, restrictive LFT patterns may also be detected as a result of comorbidities such as diaphragmatic hernia and PAS.

Esophageal Atresia and Thenar Hypoplasia Associated with Asymmetric Crying Face.

Asymmetric crying face (ACF) is a minor congenital anomaly that is often associated with a high rate of major malformations and may be considered an indication of a syndromic clinical presentation. Here, we report a 21-month-old male presenting with left- sided ACF, thenar hypoplasia, and esophageal atresia. Ultrasonographic images of the volar surface of the left hand evidenced the absence of muscle tissue around the thenar prominence at the level of the first metacarpal bone. No pathogenic copy number variation was detected on array-comparative genomic hybridization analysis (CGH). The association of esophageal atresia, thenar hypoplasia, and ACF has not been reported before. We discuss the possibility of a distinct association or of a sequence of anomalies associated with ACF.

An adolescent case of extensive Behçet`s disease successfully treated with Infliximab.

Isiyel E, Bakkaloglu S, Oguz D, Yenicesu I, Boyunaga Ö, Özdemir Y, Damar Ç, Kandur Y, Akçaboy M, Aslan AT, Sismanlar T, Hasanoglan E, Buyan N. An adolescent case of extensive Behçet`s disease successfully treated with Infliximab. Turk J Pediatr 2019; 61: 585-588. Cardiac involvement is an uncommon and life-threatening complication of Behçet`s Disease. We present a 14-year-old boy, admitted to our hospital for recurrent hemoptysis. In his radiologic evaluation, a right ventricular thrombus and pulmonary arterial aneurysm were identified. He was diagnosed with Behçet`s Disease, and then he received prednisone and cyclophosphamide. However, his cardiac thrombus enlargened. After his treatment was replaced with infliximab, the pulmonary aneurysms regressed, and the cardiac thrombus disappeared. In conclusion, infliximab should be considered as a reliable option for vascular Behçet`s Disease resistant to conventional treatment.

Association between deep neck space abscesses and internal carotid artery narrowing in pediatric patients

Background/aim: Our aim was to interpret the effects of deep neck space abscesses on the adjacent carotid artery according to abscess location, as well as to determine narrowing by calculating the mean stenosis ratios.Materials and methods: Neck computed tomography scans and clinical data of 45 children with neck abscesses were evaluated retrospectively for abscess location and internal carotid artery narrowing. The lumen areas of the carotid arteries were measured from standard levels, and stenosis ratios were calculated with two different techniques. The mean stenosis ratios of each group according to abscess location were then compared with the control group.Results: Among the 45 abscesses included in the study, 51.1% (n = 23/45) were located in the peritonsillar region, 37.8% (n = 17/45) were located in the parapharyngeal-lateral retropharyngeal space, and 11.1% (n = 5/45) were in the midline retropharyngeal space. We found a statistically significant difference between the mean stenosis ratios of the ipsilateral side of the parapharyngeal-lateral retropharyngeal abscesses and the control group (P < 0.01).Conclusion: The children with parapharyngeal-lateral retropharyngeal abscesses all had narrowing in the adjacent carotid lumen to some degree. Although most of the patients had no clinical symptoms, radiologists have to be aware of this arterial complication to prevent further progress and fatal complications.

Imaging features of Burkitt lymphoma in pediatric patients.

Burkitt lymphoma is an aggressive and rapidly growing tumor that is curable and highly sensitive to chemotherapy. It can affect almost every tissue in the body, producing various clinical presentations and imaging appearances, according to the predilection of the different subtypes for certain sites. Awareness of its diagnostically specific imaging appearances plays an important role in rapid detection and treatment. In this pictorial review, we aimed to identify the most common imaging features of Burkitt lymphoma in pediatric patients.

Juvenile Nasopharyngeal Angiofibroma: Magnetic Resonance Imaging Findings.

PURPOSE: Juvenile nasopharyngeal angiofibroma (JNA) is a rare tumor that exhibits a predictable spreading pattern. Radiologist's prior knowledge on the tumor's characteristics aids in establishing a diagnosis. We aimed to report the characteristic Magnetic Resonance Imaging (MRI) findings and the spread patterns of JNA. MATERIALS AND METHODS: We retrospectively evaluated the MRI findings and extension pathways of 6 cases of JNA. RESULTS: The patients' age ranged from 8 to 16 years and all patients were male. The tumors were classified according to the Onerci system. Tumors were largely isointense to muscle on T1-weighted images and hyperintense on T2-weighted images. All lesions had internal signal-void regions and all exhibited intense enhancement after IV contrast injection. Diffusion restriction was not an associated feature. ADC values for these tumors were high. The evaluation of the available MR angiography studies of three patients showed the blood supply to the tumor to be mainly from the internal maxillary branch of the external carotid artery. In all patients, the diagnosis was based on MR images and a surgical excision was planned. CONCLUSION: The diagnosis can be established based on the characteristic imaging findings and the clinical history without performing a biopsy.

Multiple liver masses in a patient with breast cancer, metastasis or not? An unexpected diagnosis: hepatic fascioliasis. A case report and overview of the literature from Turkey.

A patient who underwent mastectomy of the left breast owing to breast cancer was referred to our department for abdominal ultrasonography during her routine check-up. Radiological examinations demonstrated multiple masses that tended to form clusters in the liver parenchyma, and the lesions were initially thought to represent metastases from the breast cancer. Multisite biopsies and serological tests confirmed the diagnosis of Fasciola hepatica infestation. To our knowledge, this is a unique case report of a patient with a known malignant neoplasm. We also present an overview of the literature about human fascioliasis in Turkey.