Is Ultrasonography a Reliable Approach for the Evaluation of Carpal Tunnel Syndrome in Patients With Mucopolysaccharidosis?

BACKGROUND: One of the most common causes of carpal tunnel syndrome (CTS) in childhood is mucopolysaccharidosis (MPS). While ultrasonography (US) can aid in the diagnosis of CTS in adult patients, there is limited experience of this in the pediatric group. We aimed to investigate the results of wrist ultrasonography, which may be a candidate alternative to electrophysiological examination. METHODS: The participants were evaluated for symptoms, physical examination findings, electrophysiological tests and grayscale US. CTS was diagnosed in accordance with the American Academy of Orthopedic Surgeons Management of Carpal Tunnel Syndrome: Evidence-Based Clinical Practice Guideline. RESULTS: Included in the study were 27 MPS patients aged 4.5-32 years and 30 healthy control subjects aged 4.3-26 years. Of the 54 wrists in the MPS group, 30 were diagnosed with CTS. The median cross-sectional area (CSA) at the proximal carpal tunnel, the CSA at the forearm, and the wrist-forearm ratio (WFR) were higher in the wrists of the MPS with CTS group than in those without CTS and the healthy control subjects. The WFR cutoff of ≥1.35, 56.6% (95% CI: 437.4-74.5) sensitivity, and 89.8% (95% CI: 81.0-95.5) specificity were consistent with a diagnosis of CTS (receiver operating characteristics analysis, area under the curve = 0.775, 95% CI: 0.673-0.877). CONCLUSION: Although the US provides results with unsatisfactory specificity and sensitivity, it is a candidate for further investigation for the diagnosis of CTS because it is an innovative, noninvasive, and more accessible method. WFR value may produce more meaningful results than wrist or forearm nerve area measurements.

Correlation between early computed tomography findings and neurological outcome in pediatric traumatic brain injury patients.

Traumatic brain injury (TBI) is a leading cause of morbidity and mortality in children. Head computed tomography (CT) is frequently utilized for evaluating trauma-related characteristics, selecting treatment options, and monitoring complications in the early stages. This study assessed the relationship between cranial CT findings and early and late neurological outcomes in pediatric TBI patients admitted to the pediatric intensive care unit (PICU). The study included children aged 1 month to 18 years who were admitted to the PICU due to TBI between 2014 and 2020. Sociodemographic data, clinical characteristics, and cranial CT findings were analyzed. Patients were categorized based on their Glasgow Coma Scale (GCS) score. Of the 129 patients, 83 (64%) were male, and 46 (36%) were female, with a mean age of 6.8 years. Falls (n = 51, 39.5%) and in-vehicle traffic accidents (n = 35, 27.1%) were the most common trauma types observed. Normal brain imaging findings were found in 62.7% of the patients, while 37.3% exhibited intracranial pathology. Hemorrhage was the most frequent CT finding. Severe TBI (n = 26, p = 0.032) and mortality (n = 9, p = 0.017) were more prevalent in traffic accidents. The overall mortality rate in the study population was 10.1%. In children with TBI, cranial CT imaging serves as an essential initial method for patients with neurological manifestations. Particularly, a GCS score of ≤ 8, multiple hemorrhages, diffuse cerebral edema, and intraventricular bleeding are associated with sequelae and mortality.

Nutcracker syndrome in pediatrics: initial findings and long-term follow-up results.

BACKGROUND: Nutcracker syndrome (NCS) describes a set of symptoms and signs resulting from compression of the left renal vein (LRV). There is a lack of knowledge about its natural course, diagnosis, and management, especially in children. Herein, we present our single-center experience with a large number of patients who have long-term follow-up results. METHODS: All patients with NCS diagnosed between January 2011 and March 2021 were included and their data were obtained retrospectively. RESULTS: A total of 123 NCS patients (85 females) were included. The median age at the time of diagnosis was 12 (IQR 10-14) years, and BMI percentiles were below 5% in 38% of the cases. At the time of diagnosis, two-thirds of the patients were asymptomatic. The most common laboratory finding was nephritic proteinuria (98%), followed by microscopic hematuria (16%). Signs of LRV compression were significantly more evident in upright position Doppler ultrasonography (DUS) examination. All patients have been followed conservatively; hematuria and/or proteinuria resolved in 43 of the 108 patients (40%) within 35.8 ± 25.8 months of follow-up. Control DUS was performed in 52 patients after a mean period of 39.1 ± 21.3 months. The median peak velocity and diameter ratios of the LRV in the upright position were found to be decreased significantly when compared to the initial assessment (p < 0.05). Normal DUS findings were noted in 13 patients at the final evaluation. CONCLUSIONS: In unexplained proteinuria and/or hematuria, NCS should be considered, especially in asthenic adolescents. Our results support conservative management in children as the first-line treatment approach.

Early period intensive care follow-up after liver transplantation in children: a single center experience.

BACKGROUND: Liver transplantation (LT) is a well-established, life-saving treatment for children with irreversible acute and chronic liver failure (LF). We aimed to evaluate the factors associated with morbidity and mortality in the early period of LT in children by reviewing our pediatric intensive care unit (PICU) experience. METHODS: We reviewed children`s medical records followed in the PICU after LT between May 2015-August 2021, including demographic parameters, indications for LT, operative variables, respiratory and circulatory support requirements, LT-related complications and survival. RESULTS: During this period, 40 pediatric patients who underwent LT were evaluated. LT was performed in 35 (87.5%) cases of chronic liver disease and 5 (12.5%) cases of acute liver failure. Twenty-four patients had chronic liver failure due to cholestatic liver disease. The patients` Pediatric Risk of Mortality (PRISM) III score was 18.82±SD (2-58) at PICU admission. 1-year survival was 87.5%, and overall survival was 85%. Younger age, low body weight, preoperative pediatric end-stage liver disease (PELD), and model for end-stage liver disease (MELD) values of 20 and higher were important risk factors for unfavorable outcomes after living donor liver transplantation (LDLT). These risk factors are both associated with technically more challenging vascular and bile duct reconstruction and higher complication rates, and increased mortality during the early period after LT. CONCLUSIONS: The early period of optimum PICU management in pediatric LT recipients is crucial for successful outcomes, which is also related to the patients` characteristics, disease severity scores, and surgical procedures.

Childhood-Onset Sacroiliitis: Causes and Correlation Between Clinical Findings and Magnetic Resonance Imaging.

OBJECTIVE: The aims of this study were to describe disease associations of magnetic resonance imaging (MRI)-confirmed and clinically symptomatic sacroiliitis in pediatric patients with rheumatic diseases and to examine the relationship between patient characteristics and MRI findings of the sacroiliac joint (SIJ). METHODS: Demographic and clinical data were extracted from the electronic medical records of the patients with sacroiliitis followed in the last 5 years. Active inflammatory and structural damage lesions of the SIJ-MRI were examined by the modified Spondyloarthritis Research Consortium of Canada scoring system, and correlation analysis of these results with clinical characteristics was evaluated. RESULTS: A total of 46 symptomatic patients were found to have MRI-proven sacroiliitis of 3 different etiologies: juvenile idiopathic arthritis (JIA) (n = 17), familial Mediterranean fever (FMF) (n = 14), and chronic nonbacterial osteomyelitis (CNO) (n = 8). Seven patients, FMF and JIA (n = 6) and FMF and CNO (n = 1), had a co-diagnosis that might cause sacroiliitis. Although inflammation scores and structural damage lesions did not statistically differ between the groups, capsulitis and enthesitis on the MRI were more frequently detected in the CNO group. There was a negative correlation between symptom onset and inflammation scores of bone marrow edema. Disease composite scores and acute phase reactants were correlated with MRI inflammation scores. CONCLUSIONS: We demonstrated that JIA, FMF, and CNO were the major rheumatic causes of sacroiliitis in children originating from the Mediterranean region. Quantitative MRI scoring tools can be used to assess the inflammation and damage of the SIJ in rheumatic diseases, show discrepancies between them, and have an important correlation with various clinical and laboratory features.

[Evaluation of the Epidemiological, Clinical and Radiological Features of Pneumonia Cases Caused by Mycoplasma pneumoniae in Childhood]. / Çocukluk Çaginda Mycoplasma pneumoniae'nin Etken Olarak Saptandigi Pnömoni Olgularinin Epidemiyolojik, Klinik ve Radyolojik Özelliklerinin Degerlendirilmesi.

The current study aimed to investigate the clinical, laboratory and radiological findings of the pneumonia cases in children that were confirmed as M.pneumoniae by polymerase chain reaction (PCR) testing and to reveal the factors that can be decisive in the diagnosis. Seventy-seven children were included in this study. The median age of the patients was 31 months (1 month-17 years 4 months). The 63.6% of the patients were younger than five years of age, 53.2% were girls and 46.8% were boys. During the eight-year research period, the frequency of M.pneumoniae in the patients hospitalized with the diagnosis of pneumonia was found to be 3.1%. The rate of M.pneumoniae as the underlying factor of pneumonia was found to be statistically significantly lower in patients aged 0-60 months compared to the patients aged 61-216 months. In patients with M.pneumoniae accompanied by viruses, the age group was more likely to between 0-60 months. The most common symptoms were cough (96.1%) and fever (74%). Physical examinations revealed that 70.1% of the patients had rales, 63.6% had tachypnea, 45.5% had oropharyngeal hyperaemia, 35.1% had subcostal-intercostal retraction, 31.2% had long expiration period, 26% had rhonchus, 24.7% had decrease in breath sounds, 15.6% had cervical lymphadenopathy, 13% had tachycardia, 3.9% had otitis media, 3.9% had tonsil hypertrophy and 2.6% had a maculopapular rash. The rate of hypoxemia was found to be 42.2%. When the physical examination findings of patients with only M.pneumoniae detected in multiplex PCR analysis and those with accompanying viruses in M.pneumoniae were compared, tachypnea, oropharyngeal hyperemia and decreased breath sounds were found to be statistically significantly higher in patients with M.pneumoniae only. Retraction was detected more frequently in patients with accompanying viruses. When the laboratory results of the patients were evaluated according to age, leukocytosis was detected in only 18.2% of the patients, while the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were found to be high in 75% and 85.7% of the patients, respectively. In the multiplex PCR analysis, the CRP values of the patients with only M.pneumoniae were found to be higher than the patients with accompanying viruses. M.pneumoniae was accompanied by viruses at the rate of 40.3%. The most common accompanying viruses were rhinovirus, adenovirus, bocavirus and metapneumovirus. The 55.8% of the patients had lobar-segmental consolidation, 46.8% had parahilar-peribronchial thickening, 18.2% had atelectasis, 11.7% had pleural effusion, 9.1% had increase in reticulonodular density, 6.5% had lymphadenopathy whereas no abnormality was observed in 5.2% of them. No diffuse interstitial involvement was recorded. The CRP value of the patients who had lobar segmental consolidation which was detected through chest X-rays were statistically higher than those without consolidation. In multiplex PCR analysis, the rate of parahilar-peribronchial thickening detected in chest X-ray findings was found to be higher in patients with M.pneumoniae accompanied by viruses compared to those with only M.pneumoniae. The rate of the patients who were given empirical antibiotics against atypical agents was 45.5%. The rate of empirically administered antibiotic treatment for atypical agents after being hospitalization was higher in patients diagnosed with only M.pneumoniae compared to patients with M.pneumoniae and viruses. One patient (1.3%) died. As there are no typical clinical, laboratory or radiological findings specific to M.pneumoniae pneumonia, all of the findings should be assessed as a whole to establish a diagnosis. Besides, for the detection of M.pneumoniae, diagnostic tests which are cost effective, with rapid results and are capable of distinguishing colonisation from active infection should be developed.

The unique features of middle cranial fossa and Sylvian fissure arachnoid cysts in children: MRI evaluation.

PURPOSE: The purpose of the study is to evaluate MRI findings of middle fossa arachnoid cysts in children according to Galassi classification and determine the differences between types and to assess the morphological changes in cysts during follow-up imaging. METHODS: MR images of 266 middle fossa arachnoid cysts of 255 pediatric patients were evaluated by two experienced radiologists retrospectively. MRI features including the sidedness of the cyst, Galassi type, parenchymal compression findings (cortical flattening and white matter compression), bone remodeling, and midline shift were evaluated on axial T2- and T1-weighted images. Follow-up MRI and available CSF flow MR imaging data were evaluated for change in cyst size and cisternal connections, respectively. RESULTS: The most common type was type 1 according to Galassi classification. The accompanying bone remodeling and white matter compression had a higher incidence in Galassi type 2 and 3 groups than Galassi type 1. Mean age of patients with bone remodeling and white matter compression was significantly higher in patients with Galassi type 1. All patients with cyst enlargement were younger than 2 years of age, and all of them were Galassi type 1. Cisternal connection was demonstrated in 7 patients. CONCLUSION: While parenchymal compression and bone remodeling are expected findings in Galassi type 2 and 3 cysts, these features can also be seen in smaller Galassi type 1 cysts, regardless of size. Most of the middle fossa arachnoid cysts remain stable on follow-up imaging, and the increase in size is not an expected finding, especially in older children.

Pediatric Invasive Aspergillosis: a Retrospective Review of 59 Cases.

Invasive aspergillosis (IA) is a major cause of morbidity and mortality. This study aimed to present our 10-year IA experience at a single center. Fifty-nine pediatric patients with IA were included in this study. The male-to-female ratio was 42/17. The median age was 8.75 years. Hematologic malignancy was present in the majority of the patients (40/59, 68%). The mean neutropenia duration was 18.5 days. Cytosine arabinoside was the most common immunosuppressive therapy directed at T cells during IA diagnosis. IA cases were categorized as proven (27%), probable (51%), or possible (22%) according to the 2008 European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group (EORTC/MSG) criteria. The lungs (78%) were the most common site of IA, and nodules were the most frequent radiological findings (75.5%). In 38 patients (64.4%) receiving antifungal prophylaxis, prophylactic agents included fluconazole (30.5%), liposomal amphotericin B (23.7%), posaconazole (8.5%), and voriconazole (1.7%). Initial treatment was most commonly administered as monotherapy (69.5%). The median antifungal treatment duration was 67 days. Eleven deaths (18.6%) were due to aspergillosis. With the increased use of corticosteroids, biological agents, and intensive immunosuppressive chemotherapy, IA will most likely continue to occur frequently in pediatric patients.

Clinical features and outcomes of children admitted to the pediatric intensive care unit due to posterior reversible encephalopathy syndrome.

OBJECTIVE: The aim of this study is to analyze the clinical features, neuroimaging findings and outcomes of the children admitted to our tertiary pediatric intensive care unit (PICU) due to posterior reversible encephalopathy syndrome (PRES). METHODS: This was a retrospective study where the hospital records of children admitted to PICU due to PRES between January 1, 2011 and January 1, 2021 were reviewed. RESULTS: We enrolled 14 patients with a median age of 8 years (IQR 2.2-14.2) to study. Eight (57 %) patients were male. All patients had comorbid illnesses such as hemophagocytic lymphohistiocytosis in 3, Β-cell acute lymphoblastic leukemia in 2, and different diagnosis in other patients as one one. Three patients had cardiac arrest, 9 patients had seizures, 5 patients had SE, 12 patients had altered mental status, 8 patients had hypertensive crisis, 1 patient had visual impairment. Thirteen patients had occipital involvement, 11 had parietal involvement, 4 had temporal involvement, 1 had thalamic involvement, 2 had cerebellar involvement, 1 had involvement of the corpus callosum, 1 had brainstem involvement, 1 had hippocampus involvement and 1 had involvement of the basal ganglia. Fourteen patients had supratentorial involvement while 3 had infratentorial involvement. Electroencephalogram was performed for 7 patients, out of which 6 revealed encephalopathy. Median PICU LOS was 19.5 days (IQR 13.2-49.2, minimum 2 - maximum 84 days). Five patients had neurologic sequelae. Four (28.5 %) patients died and ten patients survived. CONCLUSION: Co-occurence of hypertension and seizures should prompt consideration of PRES and urgent neuroimaging, particularly in patients on immunosuppressants or chemotherapeutics. Hypertension should be addressed aggressively in patients with PRES. Electroencephalographic monitoring should be performed if there is suspicion of SE or nonconvulsive SE. Despite its usually good prognosis, PRES can cause serious morbidity and mortality with delay in diagnosis or treatment.

Early neuroimaging findings of infants diagnosed with inherited metabolic disorders in neonatal period: A case-control study.

OBJECTIVE: Most IMDs are diagnosed in the neonatal period and have severe neurological findings. Neuroimaging plays an essential role in the diagnosis. We aim to investigate early cranial MRI findings of newborns suspected with IMDs to determine IMD-related neuroimaging patterns in the early infant period. METHODS: The medical records of a total of 195 infants with suspected IMDs were screened, and 56 patients who underwent a cranial MRI within the first three months of life were included in the study. The 56 patients were categorized into those diagnosed (Group I) and those not diagnosed (Group II) with IMDs. The patient's clinical findings and radiological imaging reports were extracted to a database. RESULTS: The most common IMDs were mitochondrial diseases, urea cycle disorders, and organic acidemias. In the cranial MRI evaluations, the T2-hyperintensity of white matter and the T2-hyperintensity of basal ganglia were higher in Group I. It was found that high lactate/lipid peaks on 1H-MRS (10.68 times), T2-hyperintensity of white matter (5.75 times), and T2-hyperintensity of the basal ganglia (5.71 times) were more likely to be identified in Group I. Furthermore, no difference was noted between the groups in terms of the diffusion restriction of white matter, basal ganglia, cerebellum, and brainstem, and no statistically significant difference was noted in the T2-hyperintensity of the cerebellum and the brainstem. CONCLUSION: Early neuroimaging findings are essential in evaluations of IMDs, so familiarity with neuroimaging findings is essential for diagnosis, especially in countries that lack an expanded neonatal screening program.

SARS-CoV-2 infection showing signs of cerebral sinus vein thrombosis in the infantile period.

The clinical manifestations of SARS-CoV-2 infection mainly involve the respiratory system. However, there is increasing evidence that this virus can affect other organs, causing a wide range of clinical symptoms. This is the report of a 40-day-old patient who presented with sepsis and had no risk factors other than SARS-CoV-2 infection, whose radiological findings were compatible with cerebral sinus vein thrombosis.

Orbital structures in the pediatric age group: A normative assessment using magnetic resonance imaging.

PURPOSE: Many diseases of traumatic, infectious, endocrinologic and neoplastic origin can lead to orbital involvement and related morphological changes. In the present study, we aimed to determine the age-based normal orbital measurements using magnetic resonance imaging (MRI) in pediatric patients. METHODS: Our retrospective study included 186 patients with normal orbital MRI findings. The patients were divided into the following five groups based on their age. Group 1: 1-24 months; Group 2: 25-60 months; Group 3: 61-120 months; Group 4: 121-168 months; and Group 5: 169-216 months. Globe position relative to interzygomatic line (IZL) and orbital rim, optic nerve-sheath complex, extraocular muscle diameters, were measured. RESULTS: The differences among the age groups for the distances between cornea and sclera in relation to IZL, for the distances between cornea and sclera relative to orbital rim, and for the extraocular muscle diameters were statistically significant. In terms of the extraocular muscle diameter, it was found that the thinnest muscle of all groups was the lateral rectus muscle while the thickest was the medial rectus muscle. CONCLUSION: In pediatric patients who were divided into five groups based on their age, the positions of bulbus oculi relative to IZL or orbital rim and the normal diameter ranges of extraocular muscles and of the optic nerve-sheath complex in orbital MRI were determined. We are of the opinion that the presence of such a scale could be a guide in the evaluation of orbital MRI in pediatric patients.

Could urinary nerve growth factor and bladder wall thickness predict the treatment outcome of children with overactive bladder?

ABSTRACT Objective: Bladder wall thickness (BWTh) measurements and Nerve Growth Factor (NGF) /creatinine (Cr) values, as noninvasive tools, were found to predict daytime voiding problems in children with overactive bladder (OAB). The goal of this research was to examine if bladder wall thickness together with urine NGF/Cr could be a clinical utility in treatment outcome of OAB in children. Patients and Methods: A total of 60 children with OAB, (Group 1; n=40) and healthy normal controls (Group 2; n=20), aged 6-14 years old were involved in this prospective study. Children were evaluated with detailed history and physical examination, including neurologic examination, and were asked to complete a self-reported questionnaire and a 3-day bladder diary with the aid of their parents. Uroflowmetry was performed in all cases. Urinary nerve growth factor levels were measured by the ELISA and BWTh was measured trans-abdominally by one uro-radiologist specialized in pediatric ultrasonography. Urinary NGF levels were normalized by urinary creatinine levels and compared among all subgroups. Children with OAB received urotherapy as first line treatment at least for three months. 18 children refractory to urotherapy received anticholinergic therapy defined as group 3. Results: The median age of the study group was 10 (range 6 to 16). After urotherapy, 22 children had similar BWTh and NGF/Cr values compared to controls. (2.75 ± 1.15; 2.40 ± 1.00 mm; p=0.86 and 1.02 ± 0.10; 0.78 ± 0.15; p=0.12, respectively). After anticholinergic treatment, BWTh levels (2.25 ± 0.90; 2.40 ± 1.00 mm; p=0.94) and NGF/Cr values (0.95 ± 0.10; 0.78 ± 0.15; p=0.42, respectively) had no significantly difference compared to controls (Group 2). In receiver operating characteristic analysis, bladder wall thickness was found to have sensitivity of 85% and specificity of 84.2% (3,20 AUC, 913; 95 %) and NGF/Cr had sensitivity of 90% and specificity of 92.1% (1,595; AUC, 947; 95 %) in predicting treatment outcome in children with OAB. Conclusions: Bladder wall thickness measurements and NGF/Cr values, as noninvasive tools, could guide outcomes in the treatment of children with overactive bladder.

Could urinary nerve growth factor and bladder wall thickness predict the treatment outcome of children with overactive bladder?

OBJECTIVE: Bladder wall thickness (BWTh) measurements and Nerve Growth Factor (NGF) /creatinine (Cr) values, as noninvasive tools, were found to predict daytime voiding problems in children with overactive bladder (OAB). The goal of this research was to examine if bladder wall thickness together with urine NGF/Cr could be a clinical utility in treatment outcome of OAB in children. PATIENTS AND METHODS: A total of 60 children with OAB, (Group 1; n=40) and healthy normal controls (Group 2; n=20), aged 6-14 years old were involved in this prospective study. Children were evaluated with detailed history and physical examination, including neurologic examination, and were asked to complete a self-reported questionnaire and a 3-day bladder diary with the aid of their parents. Uroflowmetry was performed in all cases. Urinary nerve growth factor levels were measured by the ELISA and BWTh was measured trans-abdominally by one uro-radiologist specialized in pediatric ultrasonography. Urinary NGF levels were normalized by urinary creatinine levels and compared among all subgroups. Children with OAB received urotherapy as first line treatment at least for three months. 18 children refractory to urotherapy received anticholinergic therapy defined as group 3. RESULTS: The median age of the study group was 10 (range 6 to 16). After urotherapy, 22 children had similar BWTh and NGF/Cr values compared to controls. (2.75 ± 1.15; 2.40 ± 1.00 mm; p=0.86 and 1.02 ± 0.10; 0.78 ± 0.15; p=0.12, respectively). After anticholinergic treatment, BWTh levels (2.25 ± 0.90; 2.40 ± 1.00 mm; p=0.94) and NGF/Cr values (0.95 ± 0.10; 0.78 ± 0.15; p=0.42, respectively) had no significantly difference compared to controls (Group 2). In receiver operating characteristic analysis, bladder wall thickness was found to have sensitivity of 85% and specificity of 84.2% (3,20 AUC ,913; 95 %) and NGF/Cr had sensitivity of 90% and specificity of 92.1% (1,595; AUC ,947; 95 %) in predicting treatment outcome in children with OAB. CONCLUSIONS: Bladder wall thickness measurements and NGF/Cr values, as noninvasive tools, could guide outcomes in the treatment of children with overactive bladder.

The role of galactomannan test results in the diagnosis of pediatric invasive aspergillosis.

BACKGROUND: Invasive aspergillosis (IA) is an important cause of morbidity and mortality in immunosuppressed children. Early detection of the infection can improve prognosis in this patient population. OBJECTIVES: To investigate the utility of Aspergillus galactomannan antigen assay (GM-EIA) as a diagnostic tool for IA in at-risk paediatric patients. PATIENTS/METHODS: For the study, 659 GM-EIA results from 59 patients diagnosed with IA and 3368 GM-EIA results from 351 subjects without evidence for IA (controls) were reviewed retrospectively. Three cut-off values (i.e. ≥0.5, ≥1, ≥1.5) were specified to determine GM-EIA positivity. RESULTS: The median age was 6.3 years for boys and 14.5 years for girls. There was a significant difference between the girls and boys in terms of age (p < 0.01). For proven/probable/possible IA patients, sensitivity of 67.8% and specificity of 59.8% were detected when the ≥0.5 cut-off value was used for GM-EIA-positivity. The specificity increased to 80% at the cut-off of ≥1 and to 88% at the cut-off of ≥1.5. False positivity rates were 9.14, 3, and 1.45% at the ≥0.5, ≥1 and ≥1.5 cut-offs respectively. In the proven/probable IA group, sensitivity and negative predictive values were 86.9 and 97.2% at the ≥0.5 cut-off, 85.7 and 97.9%, at the ≥1 cut-off and 84.2 and 98.1% at ≥1.5 cut-off respectively. The positive likelihood ratio was 7.57 and the odds ratio was 42.67 at ≥1.5 cut-off. CONCLUSION: The GM-EIA may be used for both screening and diagnostic purposes in paediatric patients using a cut-off value of ≥1.5 for GM-EIA positivity.

Nutcracker syndrome: a potentially underdiagnosed cause of proteinuria in children with familial Mediterranean fever.

INTRODUCTION: Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory disease with an increased risk for secondary amyloidosis. Since lifelong colchicine has been the treatment of choice that prevents renal amyloidosis, non-amyloid kidney diseases are more frequently considered in the differential diagnosis of proteinuria. Nutcracker syndrome (NCS) can be one of the confounding causes. This long-term retrospective study aimed to evaluate the causes of proteinuria in a pediatric cohort of patients with FMF and discuss changing trends in recent years . METHODS: Demographic, clinic, and laboratory data were extracted from electronic medical records of patients with FMF. All urine tests of the study population were reviewed. Patients were evaluated for persistent proteinuria and grouped according to the etiology of proteinuria. RESULTS: A total of 576 patients with FMF were identified with a mean follow-up of 6.3 years in the last 10 years; 8% had persistent proteinuria. The etiology was NCS in 67.5% of the patients with proteinuria, and renal amyloidosis was less commonly encountered (15%) without any new diagnosis for the last 8 years. Non-amyloid kidney diseases were also diagnosed in 17.5% of the patients. Patients with NCS had significantly lower BMI than other patients in the cohort and less subclinical inflammation, higher hemoglobin concentration, and milder levels of proteinuria with normal serum albumin and eGFR than other patients with proteinuria. CONCLUSION: Nutcracker syndrome is the leading cause of proteinuria in children with FMF nowadays, and it should be kept in mind during the evaluation of proteinuria in these patients. A higher resolution version of the Graphical abstract is available as Supplementary information.

3D-reconstruction and heterotopic implantation of reduced size monosegment or left lateral segment grafts in small infants: A new technique in pediatric living donor liver transplantation to overcome large-for-size syndrome.

BACKGROUND: Monosegmental grafts and reduced left lateral segment grafts have been introduced to overcome the problems of large-for-size grafts in pediatric living donor liver transplantation. Here, we introduce a new method of reduced size monosegment or left lateral segment grafts transplanted in the right diaphragmatic fossa heterotopically in small infants. METHODS: There were 4 infants who underwent living donor liver transplantation with heterotopically implanted reduced monosegmental or left lateral segment grafts at our center. The demographic, operative, postoperative, and follow-up data of these infants were collected from our prospectively designed database and reviewed. Technical details of the donor and recipient operation are shared and a supplemental provided. RESULTS: The mean recipient age was 7.5 ± 0.9 months (range: 5-10 months), and body weight was 5.9 ± 0.7 kg (range: 4.6-7.8). Primary diagnoses of the recipients were biliary atresia (n:3) and progressive familial intrahepatic cholestasis (n:1). Mean graft-recipient weight ratio was 3.3 ± 0.2. Reduced monosegment III grafts were used in 2 cases, and reduced left lateral segment grafts were used in the other 2 patients. Bile duct reconstruction was done by Roux-en-Y hepaticojejunostomy in 3 patients and duct-to-duct anastomosis in the remaining patient. All patients recovered from the liver transplantation operation and are doing well at a mean follow-up of 8 months. CONCLUSION: Living donor liver transplantation with heterotopically implanted reduced monosegmental or left lateral segment seems feasible for the treatment of neonates and extremely small infants. Further accumulation of cases and long-term follow-up are necessary to collect data for the establishment of this treatment modality.