Effect of cervical and lumbosacral spina bifida cystica on volumes of intracranial structures in children.

PURPOSE: Spina bifida is a major disorder that occurs when the membranes of the spinal cord and medulla fail to close during the embryonic period and affects the individual for the rest of life. Some physical, mental, and social difficulties can be observed in the lives of children with spina bifida after surgery. The aim of this study is to determine what kind of volumetric changes occur in the brain when spina bifida occurs in different regions of the cord. METHODS: The volume of intracranial structures of 14 children aged 1 to 9 years (7 cervical, 7 lumbosacral) with different levels of spina bifida compared with vol2Brain. RESULTS: Spina bifida occurring in the cervical region was found to cause a greater volumetric reduction in subcortical structures, cortex and gyrus than spina bifida occurring in the lumbosacral region. CONCLUSION: We believe that our study will help clinicians involved in the management of this disorder.

Evaluation of the Relationship Between Preoperative and Postoperative Platelet Values and Amount of Blood Transfusion in Cases with Craniosinocytosis.

AIM: To demonstrate that decreased platelet count in patients with craniosynostosis increases the requirement for blood replacement, thus providing guidance to clinicians by revealing the time at which the platelet count decreases. Additionally, the relationship between the amount of blood transfusion and preoperative and postoperative platelet counts was evaluated. MATERIAL AND METHODS: This study included 38 patients with craniosynostosis who underwent surgery between July 2017 and March 2019. The patients exhibited no cranial pathologies except craniosynostosis. All surgeries were performed by a single surgeon. The demographic data, anesthesia and surgical durations, preoperative complete blood count and bleeding time, intraoperative blood transfusion amount, and postoperative complete blood count and total blood transfusion amount of the patients were recorded. RESULTS: The preoperative and postoperative changes and the timing of changes in hemoglobin and platelet counts, amount and timing of postoperative blood transfusion, and relationship between the amount and timing of blood replacement and preoperative and postoperative platelet counts were evaluated. The postoperative platelet counts tended to decrease after 12, 18, 24, and 36 hours (h), and began increasing after 48 h. Although decreased platelet count did not lead to platelet replacement, it influenced the erythrocyte replacement requirement in the postoperative period. CONCLUSION: Platelet count was associated with the amount of blood replacement. The platelet counts decreased within the first 48 h following surgery and tended to elevate thereafter; thus, clinicians should closely monitor these platelet counts within 48 h after surgery.

A new supportive approach in the diagnosis of Chiari malformation type 1 in pediatric patients.

PURPOSE: Chiari malformation type 1 (CM-1) is a posterior fossa anomaly characterized by herniation of the cerebellar tonsils from the foramen magnum (FM). This study compares FM, medulla spinalis (MS), and herniated cerebellar tonsils ratios by making area measurements from axial plane MRI in CM-1 patients and the control group. METHODS: Our study evaluated 30 pediatric patients with CM-1 and 30 people in the control group. The lengths of the McRae line, twining line, and clivus line were measured on the posterior cranial fossa evaluation. The areas of FM (AFM), MS (AMS), and herniated cerebellar tonsils (ATONSILS) were measured by axial images. RESULTS: As a result of area measurements obtained from axial cross-sectional MRI, a statistically significant difference was found between CM-1 patients and the control group. According to the results of the ROC analysis, if an individual's AMS/AFM value is above 17.9% or the ATONSILS/AFM value is above 18.4%, it can be interpreted as a CM-1 patient. CONCLUSION: It will be easier to diagnose the patient with the new approach we obtained from axial MR images in addition to sagittal MR images. This method can be a guide in some cases when the surgeons are undecided.

Evaluation of liver elasticity with shear-wave elastography in juvenile idiopathic arthritis patients receiving methotrexate.

BACKGROUND: Methotrexate (MTX) is the first-choice disease-modifying drug in juvenile idiopathic arthritis (JIA) treatment. Methotrexate is metabolized in the liver and can cause liver toxicity and fibrosis with long-term use. Ultrasound shear wave elastography (SWE) is a non-invasive method and can detect liver fibrosis by evaluating the liver elasticity. The aim of this study was to assess liver stiffness and detect if there is an increase in liver stiffness or fibrosis findings with the non-invasive SWE method in JIA patients under MTX treatment. METHOD: The study included 49 JIA patients under MTX treatment and 48 healthy controls, matched for age and sex with a body mass index below the 95th percentile. The demographic data and clinical characteristics of patients were obtained from medical records. Liver function tests were evaluated, and liver tissue stiffness measurements were performed with SWE. RESULTS: Of the 49 patients, 67.35% were girls and the mean age was 10.69 (±4.33) years. The duration of MTX treatment was 23.00 (1-80) months, and the cumulative dose of MTX was 1,280.867 mg (±934.2) in the patient group. There was no statistically significant difference in liver stiffness between patients receiving MTX and healthy controls (P = 0.313). There was no relationship between MTX duration, cumulative dose, route of administration, and liver stiffness. Only gamma glutamyl transferase values were weakly correlated with liver stiffness (P = 0.029). CONCLUSIONS: We did not detect an increase in liver tissue stiffness in JIA patients using methotrexate in comparison with controls.

Identifying the effects of excess weight, metabolic syndrome and insulin resistance on liver stiffness using ultrasound elastography in children.

BACKGROUND: Metabolic syndrome (MetS) and insulin resistance (IR) are known predictors of nonalcoholic fatty liver disease (NAFLD) which is one of the significant comorbidities of obesity. Obese children with MetS and IR are reported to be more likely to have advanced liver fibrosis compared to those without MetS or IR. The aim of this study is to determine the effects of excess weight, MetS and IR on liver fibrosis assessing liver stiffness in children using ultrasound elastography and compare gray scale ultrasonographic findings of hepatic steatosis (HS) with liver fibrosis. METHODS: The study group involved 131 overweight/obese children. The control group involved 50 healthy lean children. Groups were adjusted according to body mass index (BMI) and BMI-standard deviation scores (SDS). Liver stiffness measurements which are expressed by shear wave velocity (SWV) were performed for each individual. The study group was further subgrouped as children with MetS and without MetS, with IR and without IR. RESULTS: The mean SWV of liver was 1,07 ± 0,12 m/s in the control group and 1,15 ± 0,51 m/s in the study group. The difference was significant (p=0,047). SWV of liver was weakly correlated with age, BMI, BMI-SDS, Homeostatic Model Assessment-Insulin Resistance and high-density lipoprotein cholesterol. The mean SWV of the liver in the study group for children without MetS was 1,1 ± 0,44 m/s, with MetS was 1,23 ± 0,70 m/s. The difference was not significant (p=0,719). The mean SWV of the liver in the study group for children without IR was 1,02 ± 0,29 m/s, with IR was 1,24 ± 0,61 m/s. The difference was not significant (p=0,101). In multivariate regression analysis, the only independent factor affecting liver stiffness was BMI-SDS (OR:2,584, 95% CI: 1,255- 5,318, p=0,010). CONCLUSIONS: Obesity itself, regardless of MetS or IR seems to be the major problem affecting liver stiffness in this study. However, large scale longitudinal studies might clarify this issue.

Assessment of clinical, biochemical, and radiological outcomes following intra-articular injection of Wharton jelly-derived mesenchymal stromal cells in patients with knee osteoarthritis: A prospective clinical study.

The aim of the present study was to perform clinical, biochemical, and radiological evaluation of the efficacy of mesenchymal stem cells derived from Wharton jelly (WJ) present within the human umbilical cord in the treatment of knee osteoarthritis. Between 2018 and 2019, 10 patients with knee osteoarthritis for whom the conservative treatment was not beneficial were included in the study. Patients were clinically, radiologically, and biochemically evaluated before treatment initiation. Thereafter, the patients were intra-articularly injected using a solution containing 1 × 108 WJ-derived MSCs. Evaluations were performed on day 21 (V1) and 42 (V2) and month 3 (V3), 6 (V4), and 12 (V5) after the procedure. At 1-year post-injection, visual analogue scale, Western Ontario and McMaster Universities Osteoarthritis Index, and Lequesne scores of patients were lower than those observed during the initial evaluation, whereas the mean 36-Item Short Form Health Survey score was higher. Cartilage thicknesses were found to be increased in all regions except in the medial femur, medial posterior femur, lateral posterior femur, and lateral posterior tibia regions in magnetic resonance imaging. A significant increase was observed in tumor necrosis factor-alpha, interleukin-1ß, adiponectin, resistin, and interleukin-6 levels compared with pre-injection values. The leptin levels at 6-month and 1-year controls were lower than the pre-injection levels, and the decrease observed at 6 months was significant. In patients with knee osteoarthritis, intra-articular WJ-derived MSC injection causes significant pain reduction, satisfactory functional improvement, and increased patient satisfaction following a 1-year follow-up. These clinical improvements were supported by magnetic resonance images, along with changes in adiponectin and leptin levels in synovial fluid. Level of evidence: IV.

Circulating fibrocyte level in children with cystic fibrosis.

BACKGROUND: This study aimed to evaluate circulating fibrocyte levels in cystic fibrosis (CF) patients during stable and exacerbation periods of the condition. METHODS: The study group consisted of 39 patients diagnosed with CF and 20 healthy controls. Individuals included in the study were divided into three groups: CF, CF exacerbated, and a healthy control group. Their circulating fibrocyte levels were compared. Findings from a pulmonary function test and high-resolution computed tomography of the lung were evaluated and compared. RESULTS: The circulating fibrocyte count was found to be significantly higher in patients with CF compared with the exacerbated and control groups. No correlation was found between the forced expiratory volume in 1 s and forced vital capacity values in the pulmonary function test and the circulating fibrocyte count. The circulating fibrocyte count in patients (in the CF group) with positive findings in the high-resolution computed tomography was statistically significantly lower. CONCLUSIONS: The circulating fibrocyte level in the peripheral blood of the patients with CF was increased.

Determining the effects of excess weight on renal cortical stiffness in children and adolescents with point Shear Wave Elastography.

AIM: To determine the early effects of excess weight on renal cortical stiffness in children and adolescents using point shear wave elastography (pSWE). MATERIALS AND METHODS: One hundred and forty-six overweight and obese children (43.2% male; mean age, 12.6±2.9 years: range 4.3-18) and 48 lean children (27.1% male: mean age, 12.4±3.4: range 4.8-18.9) were included in the study and control group, respectively. pSWE measurements of the two kidneys were performed. The mean value of shear wave velocity was compared between groups. RESULTS: The mean shear wave velocity was 2.79±0.53 m/s for the control subjects and 3.09±0.59 m/s for the overweight-obese subjects. The differences between the two groups were sta-tistically significant (p=0.001). There was no correlation between shear wave velocity and age or depth. A positive correlation was found between shear wave velocity and body mass index, body mass index-standard deviation score. CONCLUSION: Renal cortical stiffness was higher in children with excess weight than in lean children. This study is the first attempt at applying pSWE to investigate the early adverse effects of excess weight.

Encephalocraniocutaneous lipomatosis (Haberland syndrome) in a newborn baby: a case report with review of literature.

Encephalocraniocutaneous lipomatosis (ECCL) is an extremely uncommon, neurocutaneous disease, with a classical triad of ocular, skin lesions and central nervous system anomalies. We here report a case of ECCL in a newborn baby, characterized with naevus psiloliparus, choristoma, lipodermoids, cervical subcutaneous soft tissue mass, lowset ear, porencephalic cyst, polymicrogyria, arachnoid cyst, leptomeningeal angiomatosis and spinal lipomas. We here stress on the importance of early diagnosis to prevent misdiagnosis and employ a multidisciplinary approach in the management of these patients.

Relationship between Serum YKL-40 Level and Forearm Arterial Patency after Repair.

BACKGROUND: The study aimed to assess the effects of serum YKL-40 level on patency at the repair site in patients who underwent arterial repair at the level of the forearm. METHODS: The study included 58 subjects, including 29 patients (aged 18-50 years) who had ulnar or radial artery injury secondary to cut injury to wrist between June 2015 and November 2019 and no comorbid disease and 29 age- and sex-matched healthy controls. The vascular patency was assessed using Doppler sonography in patients who underwent arterial repair at the level of the forearm. The patients were defined as flow failure if the blood flow was ≤50%, and sufficient flow if the blood flow was >50% of those in the synonymous artery on the intact extremity. The YKL-40 level differences in the patient and control groups were compared to those in the sufficient and insufficient flow groups. RESULTS: The patients were stratified into 2 groups based on the presence of sufficient flow. The mean YKL level was 11.96 ± 8.87 in the sufficient flow groups, whereas it was 32.22 ± 15.43 in the insufficient flow groups (p= 0.038). Besides, it was found that each unit of increase in the YKL-40 level increased the likelihood of having flow failure by 1.128. CONCLUSION: Based on our results, it was observed that over-expression of the YKL-40 level has adverse effects on patency following arterial repair.

The Possible Relationship Between Familial Mediterranean Fever and Chronic Nonbacterial Osteomyelitis: Coincidence or Coexistence?

BACKGROUND/OBJECTIVES: Chronic nonbacterial osteomyelitis (CNO) is an inflammatory disease characterized by recurrent attacks and remissions due to sterile bone inflammation. The CNO may be accompanied by various inflammatory diseases. The aims of our study were to determine the clinical, laboratory, and radiological characteristics of children with CNO, and to investigate the possible effect of concomitant diseases on the course of CNO. METHODS: Twenty-three patients who were diagnosed with CNO between 2012 and 2019 were analyzed. Demographic characteristics, clinical courses, laboratory and imaging findings, and concomitant diseases were recorded. The characteristics of the CNO patients with and without concomitant diseases were compared. RESULTS: The mean ± SD age of patients at the time of diagnosis and the last follow-up was 10.46 ± 4.1 and 12.47 ± 4.47 years, respectively. The median (range) time interval between disease onset and diagnosis was 5.33 (1-55) months. The mean ± SD duration of disease was 24.71 ± 16.76 months. Twelve patients (52.2%) were male. The most commonly affected areas were femur (74%), tibia/fibula (74%), and pelvis (52.2%). Age at symptom onset, age at diagnosis, mean number of lesions, presence of sacroiliitis, acute phase reactants at the start of disease, clinical and radiological remission rates, and treatment responses were not significantly different between the 13 patients with concomitant diseases and those without. Eight patients (34.8%) had familial Mediterranean fever (FMF), and all of them had exon 10 mutations. Four patients (17.4%) had juvenile spondylarthritis, one had inflammatory bowel disease, and one had psoriatic arthritis as concomitant diseases. Clinical remission was achieved in 19 patients (82.6%) and complete remission in 11 patients (47.8%) at the time of follow-up. CONCLUSIONS: In our cohort, half of the patients with CNO had concomitant diseases, with FMF being the most common. We think that the coexistence of FMF and CNO is not a coincidental one and that both may result due to an abnormality of a common pathogenetic pathway.

Is there an association of hematopoietic stem cell and endothelial progenitor cell markers with maturation in forearm arterial repair?

OBJECTIVES: This study aims to assess the effects of the hematopoietic stem cells and endothelial progenitor cells in peripheral blood and monocyte cluster of differentiation (CD) 34, CD133 and CD309 expression levels on maturation at the repair site in patients who underwent forearm arterial repair. PATIENTS AND METHODS: This prospective study included 30 patients (23 males, 7 females; mean age 28.9±1.8 years; range, 18 to 49 years) with a well-defined cut at the wrist due to a stabbing injury but no comorbid condition who presented to the emergency department of our hospital between November 2014 and November 2017. Vascular patency was assessed by Doppler sonography in patients who underwent forearm arterial repair via micro-vascular techniques. The relationships between patency and hematopoietic stem and endothelial progenitor cell markers such as CD34, CD133 and CD309 were assessed by flow cytometry. RESULTS: The patients were divided into two groups according to presence of sufficient flow in the arteries repaired. The mean CD34 expression level was 72.09±3.00 in the group with maturation whereas it was 54.64±7.34 in the group without maturation, indicating a statistically significant difference (p<0.05). In addition, the likelihood of sufficient flow was increased by 1.075 per one unit increase in CD34 level. Resistive index values were significantly lower in the group with maturation and CD34 level was predictive for maturation of arterial repair. CONCLUSION: In the present study, the findings demonstrated that high CD34 expression level has favorable effects on maturation after arterial repair.

Diffusion tensor imaging of the median nerve healing. Preliminary results.

AIMS: This study aimed to examine the correlation between DTI, clinical assessment, and electromyography results in patients who underwent primary median nerve repair. METHODS: Ten patients who underwent primary repair of the complete median nerve transection were included. Study assessments were performed on both the traumatized and non-traumatized extremities and patients were followed up for a minimum duration of 11 months. Clinical assessments, (Tinnel test, static 2-point discrimination test, motor and quality of life assessments), electromyography and DTI were performed. RESULTS: None of the clinical or electromyographic parameters correlated significantly with any of the diffusion tensor imaging parameters, i.e. fractional anisotropy (FA) or apparent diffusion coefficient (ADC) (p>0.05 for all). In addition, The Disabilities of the Arm, Shoulder and Hand (DASH) scores did not correlate with either FA (r=0.55, p=0.098) or ADC (r=0.40, p=0.260) values. However, Tinnel positive cases (n=3) had lower relative FA when compared to Tinnel negative cases (n=7) (-0.11±0.19 vs. 0.05±0.04, p=0.033). CONCLUSION: Our findings do not support the presence of relations between DTI parameters and electromyographic or most of the clinical parameters. Further MRI studies with larger numbers of patients with complete transection of the median nerve using the novel imaging parameters are warranted. KEY WORDS: Diffusion Tensor Imaging (DTI), Electromyography (EMG), Median nerve, Nerve injury, Nerve repair.

The effects of locally applied simvastatin on an experimental mouse femur nonunion model.

OBJECTIVE: The aim of this study was to assess the effects of locally applied simvastatin on femur nonunions in a mouse model. METHODS: The study included 32 male Wistar albino mice randomly allocated to one of four groups: two control groups (control-4 week [C4w] and control-8 week (C8w)] and two treatment groups (simvastatin-4 week [S4w] and simvastatin-8 week [S8w]). The control groups received dimethylsulfoxide locally injected at a dose of 10 mg/kg/day after surgical intervention for 1 week. Treatment groups received a liquefied form of simvastatin locally to the osteotomy field by injection at a dose of 10 mg/kg/day, starting from the first postoperative day for 1 week. The C4w and S4w groups were sacrificed 4 weeks and the C8w and S8w groups 8 weeks after the end of local treatment. Before sacrifice, intracardiac blood samples were retrieved for biochemical analysis and radiographies were taken. The right femurs of mice were then removed for histopathological evaluation. RESULTS: There were significant differences between the control and treatment groups when evaluated radiologically. Significantly higher levels of bone-specific alkaline phosphatase and osteocalcin values were found in the treatment groups than in the controls (p<0.05). CONCLUSION: According to biochemical, radiological and histopathological results, local application of simvastatin appears to produce beneficial effects on the mouse femur nonunion model.