The remarkable characteristics of the children with colchicine-resistant familial Mediterranean fever in Turkey.

OBJECTIVES: Colchicine is the fundamental treatment of familial Mediterranean fever (FMF). Still, 5-10% of patients are not in remission with colchicine treatment. A consensus could not be established for the definition of colchicine resistance in FMF. This study aimed to determine factors that help to predict colchicine resistance in pediatric FMF patients. METHODS: Patients with FMF that age of diagnosis was under 18 years old were included in our study. Fifty colchicine responsive and 33 colchicine-resistant patients were stratified as groups 1 and 2, respectively. Patients' clinical and laboratory findings were evaluated. Logistic regression analysis was used to determine the risk factors of colchicine-resistant FMF. Receiver operating characteristic (ROC) curve analysis was used to identify and compare the predictive performances of colchicine-resistant FMF models. RESULTS: Homozygous exon 10 MEFV mutations were frequent in group 2 (Group 1: 34 (68%), group 2: 32 (97%), p = .013). Univariate analysis showed that the age of onset of symptoms, age of diagnosis, chronic arthritis, myalgia and diarrhea during attacks, and the number of attacks, high ISSF and Pras score, high C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) values under colchicine treatment were risk factors for colchicine-resistant FMF. With multivariate analysis, the number of attacks (OR 1.418, CI (95%) 1.149-1.750, p = .001) and high ESR values (OR 1.129, CI (95%) 1.059-1.204, p<.001) were detected as independent risk factors for colchicine-resistant FMF. CONCLUSION: The predictive factors were determined for pediatric colchicine-resistant FMF in our study. The results will help to early diagnosis and treatment of chronic inflammation in FMF.

Unexpected condition in a rare disease: encephalopathy in early-onset sarcoidosis.

BACKGROUND: Granulomatous autoinflammatory diseases are monogenic syndromes caused by mutations in the region encoding the nucleotide-binding domain of the nucleotide-binding oligomerization domain-containing 2 gene. Blau syndrome and early-onset sarcoidosis are familial and sporadic forms of the same disease and are very rare. Many organ systems may be involved; however, neurologic involvement is infrequent. We reported a case of encephalitis in a 12-year-old girl followed with a diagnosis of early-onset sarcoidosis. CASE: The patient was diagnosed with juvenile idiopathic arthritis at 3 years of age. We considered druginduced sarcoidosis at 6 years of age with granulomatous inflammation of liver and kidney. Small joint involvement and camptodactyly developed during follow-up. M315T mutation was detected in the NOD2 gene supporting the diagnosis of early-onset sarcoidosis. The patient suffered from encephalopathy when she was under methotrexate, infliximab, and systemic steroid treatment at 12 years of age. Cerebrospinal fluid limbic encephalitis antibody panel was negative. CONCLUSION: Encephalopathy is not common in Blau syndrome and early-onset sarcoidosis. The cause of encephalopathy in our patient was interpreted as autoimmune encephalitis.

Magnesium levels in children with chronic kidney disease stages 1-3 / Niveles de magnesio en niños con enfermedad renal crónica estadios 1-3

ABSTRACT Objective: The objective of this study was to assess serum and urinary magnesium levels in children who have chronic kidney disease stages 1-3. Methods: Eighty-seven patients who were followed at pediatric nephrology department for chronic kidney disease were included in the study. Age, gender, magnesium, dietary magnesium, and creatinine levels, and fractionated magnesium excretion for all cases were recorded. Patients with chronic kidney disease and control groups were compared in terms of these data. Results: Thirty-nine cases with chronic kidney disease were stage 1, 26 were stage 2, and 22 were stage 3. Average age was 9.9 ± 2.8 years in the control group and 10.2 ± 2.6 years in the chronic kidney disease group. The serum magnesium levels were significantly higher in the stage 3 group than in the control group (P<0.001). Also, in stage 3, fractionated magnesium excretion levels were higher compared to the control group (P<0.001). Conclusion: In chronic kidney disease with advancing renal failure, hypermagnesemia is frequently seen. Serum magnesium levels should be measured periodically in all the children with chronic kidney disease stage 3 to investigate magnesium abnormalities and assess clinical results.

RESUMEN Objetivo: El objetivo de este estudio fue evaluar los niveles de magnesio sérico y urinario en niños con enfermedad renal crónica en estadios 1-3. Material y métodos: Se incluyeron en el estudio 87 pacientes que tuvieron seguimiento en el servicio de nefrología pediátrica por enfermedad renal crónica. Se registraron los siguientes datos: edad, sexo, niveles de magnesio, ingesta de alimentos con magnesio, y creatinina, así como también la excreción fraccionada de magnesio para todos estos casos. Sobre la base de dichos datos, se compararon los pacientes con enfermedad renal crónica y los grupos de control. Resultados: De los 87 casos de enfermedad renal crónica, 39 se hallaban en estadio 1; 26, en estadio 2, y 22, en estadio 3. La edad promedio fue de 9,9 ± 2,8 años en el grupo control y de 10,2 ± 2,6 años en el grupo de enfermedad renal crónica. Los niveles de magnesio en suero fueron significativamente más altos en el grupo del estadio 3 que en el grupo control (p <0,001). Además, en el estadio 3, los niveles de excreción fraccionada de magnesio fueron más altos en comparación con el grupo control (p <0,001). Conclusión: En la enfermedad renal crónica con insuficiencia renal avanzada, se observa con frecuencia una hipermagnesemia. Los niveles séricos de magnesio deben medirse periódicamente en todos los niños con enfermedad renal crónica en estadio 3 para investigar las anomalías del magnesio y evaluar los resultados clínicos.

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.

Evaluation of familial Mediterranean fever patients concomitant with juvenile spondyloarthropathy.

OBJECTIVES: Familial Mediterranean fever (FMF) may present with various concomitant diseases. This study aims to evaluate the clinical characteristics of patients with FMF with Juvenile Spondyloarthropathy (jSpA). METHOD: Thirty-two patients diagnosed with FMF/jSpA, sixty-four with FMF, and fifty-four with jSpA were included in this retrospective study. Three patient groups were compared in terms of clinical and laboratory features. RESULTS: The mean ages of patients in the FMF/jSpA, FMF and jSpA groups were 15.75(11.50-19.83), 15,41(6.83-21.50), and 16(9-22) years, respectively. Chronic arthritis (OR: 0.11, p = .049), erythrocyte sedimentation rate values (OR:1.07, p = .011), and C-reactive protein values (OR:1,08, p: .039) of the patients in remission period were found higher, the international severity scores for FMF (ISSF) before and after colchicine treatment (OR: 1.16, p: .021, OR: 2,21, p: .012) were higher in the FMF/jSpA group compared to FMF. Plantar fasciitis was more common and HLA-B27 positivity rate was lower in the FMF/jSpA group (OR:0.08, p = .024), (OR:4.71, p = .002) compared to jSpA. FMF/jSpA patients were divided as previous diagnosed FMF and jSpA.The diagnosis of jSpA was at a younger age(p = .002), Juvenile arthritis damage index-articular(p = 0.022) and extraarticular(p = .026), and the rate of biologic drug usage(p = .015) were higher in the previous jSpA group. The number of FMF attacks before colchicine was lower in the previous jSpA group(p = .02). CONCLUSION: Our findings suggest that both classical FMF and jSpA findings were lower in patients with FMF/jSpA. Patients who were diagnosed with jSpA at an early age and who had enthesitis and plantar fasciitis should also be evaluated in terms of FMF.

Evaluation of the thicknesses of cartilage and enthesis in familial Mediterranean fever and enthesitis-related arthritis.

OBJECTIVE: Subclinical inflammation is still a controversial issue in inflammatory diseases. There is no reliable, easy, and cheap inflammation marker in daily clinical practices currently. This study aims to predict clinical remission using cartilage and tendon thicknesses. METHODS: Eleven patients with Familial Mediterranean Fever (FMF) who had musculoskeletal involvement before and 11 patients with Enthesitis-Related Arthritis (ERA) were included in this study. They were on remission with clinical and laboratory evaluations for at least three months. Demographic and clinical features of the subjects, including age, sex, body mass index, disease duration, age at onset, medical treatment, and laboratory evaluations, were all noted. Healthy children of the same age were included as the control group. The thicknesses of the bilateral knee, second metacarpophalangeal and ankle joints cartilages, quadriceps, superior and inferior patellar, and the Achilles tendons were measured with a linear probe. A total of 198 joint and 264 tendon thicknesses were measured. RESULTS: The thicknesses of metacarpophalangeal, knee, and ankle cartilages were higher in the FMF group than in the others. In the FMF group, the quadriceps tendon thickness was higher than in the ERA group, and the superior patellar tendon thickness was higher than in the control group (p<0.05). CONCLUSION: According to our preliminary findings, an increased thickness of the cartilage and tendon in FMF patients may be an indicator of subclinical inflammation. Increased thickness of the enthesis in FMF patients may also indicate that enthesitis-related arthritis will also develop in the future.

Blau Syndrome and Early-Onset Sarcoidosis: A Six Case Series and Review of the Literature.

OBJECTIVES: This study aims to discuss the clinical, laboratory and genetic findings, and treatment options for six patients who were diagnosed with Blau syndrome (BS)/early-onset sarcoidosis (EOS). PATIENTS AND METHODS: The study included four patients (2 males,2 females; mean age 7 years; range 4 to 10 years) with EOS and two siblings (1 male, 1 female; mean age 10 years; range, 9 to 11 years) with BS. Age, age of initial symptoms, age of diagnosis; articular involvement, presence of uveitis, dermatitis, or fever, other organ involvement, laboratory findings, results of metabolic tests for mucopolysaccharidosis and mucolipidosis, results of genetic, pathologic, and immunologic tests, radiologic findings to evaluate skeletal dysplasia, and treatment options were collected. RESULTS: The median age at diagnosis of all patients was 6 years (range, 1 to 10 years). Five patients had camptodactyly and bilateral boggy synovitis in the wrists and ankles, one had granulomatous inflammatory changes in the liver and kidney biopsy, and one had attacks of fever and granulomatous dermatitis. None had uveitis. The detected mutations in nucleotide-binding oligomerization domain containing 2 (NOD2) were P268S (rs2066842), M513T (rs104895473), R702W (rs2066844), V955I (rs5743291), H343Y (rs199858111), and M491L (16:50745293). The treatments of patients included corticosteroids, non-steroid anti-inflammatory drugs, methotrexate, infliximab, adalimumab, anakinra, and canacinumab. CONCLUSION: Camptodactyly and boggy synovitis are important signs of BS/EOS. Methotrexate and tumor necrosis factor blockers are more effective in patients with predominantly articular symptoms. In patients 5 and 6 and their mother, we determined a novel M491L mutation in the NOD2 gene. Currently, this work is in progress towards identifying the pathogenesis and treatment options for this disease.

Evaluation of factors affecting the duration of disease-modifying anti-rheumatic drugs application in patients with enthesitis-related arthritis.

OBJECTIVE: Treatments for enthesitis-related arthritis (ERA) consist of a mono- or combination therapy with non-steroidal anti-inflammatory drugs, disease-modifying anti-rheumatic drugs (DMARDs), and biological agents, and they are primarily based on adult studies and studies on other forms of juvenile idiopathic arthritis, depending on whether there is axial or peripheral involvement. We use DMARDs frequently in our daily practice, even in patients with axial involvement. The main reason for this is that the health insurance system in Turkey does not allow the use of Tumor Negrosis Factor (TNF) blockers as the first line of treatment. The aim of this study is to evaluate the factors affecting the duration of DMARDs application in patients with ERA. METHODS: Fifty-two patients with ERA were accepted in this retrospective cohort study. These patients did not have an inflammatory bowel disease, reactive arthritis or undifferentiated arthritis, psoriasis, and familial Mediterranean fever. Demographic characteristics, medical history, the initial and follow-up physical examination, initial Juvenile Spondyloarthritis Disease Activity Index (JSpADA), initial laboratory tests, radiographic tests, Juvenile Arthritis Damage Index-articulary (JADI-A) and extra-articulary (JADI-E) on the last admission, and data on medical treatments were recorded from the registered data. The univariate Cox proportional hazards regression analyses was used to determine factors affecting the non-response time of ERA patients to DMARDs before the biological treatment was started. RESULTS: Twenty-seven patients (52%) achieved remission with DMARDs, while 25 (48%) patients did not. The age at diagnosis (HR=1.12; p=0.247); gender (HR=2.53; p=0.210); family history of ankylosing spondylitis (HR=1.17; p=0.730); inflammatory back pain (HR=0.57; p=0.175); the shoulder (HR=0.75 p=0.706), hip (HR=0.45; p=0.129), and small-joint involvement (HR=1.53; p=0.439); sacroiliitis with physical examination (HR=0.90; p=0.814) and magnetic resonance imaging (MRI) (HR=2.84; p=0.110); enthesitis (HR=0.83; p=0.670); presence of uveitis (HR=2.04; p=0.342); presence of HLA-B27 (HR=1.39; p=0.524); initial high acute phase reactants levels(HR=1.89; p=0.183); initial JSpADA score (HR=0.98; p=0.944); and last JADI-A (HR=1.41; p=0.060) score did not affect the duration of DMARDs treatment before switching to biological treatments. CONCLUSION: In our study, the absence of factors affecting the duration of DMARDs application in patients with ERA showed that DMARDs may still be applied as the first line of treatment.

A potential serious complication in infants with congenital obstructive uropathy: Secondary pseudohypoaldosteronism.

Patients who have secondary pseudohypoaldosteronism (PHA) in addition to hyponatraemia, hyperpotassaemia and high serum aldosterone levels for the age were included in this retrospective study.Among eight patients, seven patients were diagnosed with PHA secondary to obstructive uropathy (OUP), whereas one patient had PHA secondary to ileostomy. Six patients with OUP had simultaneous urinary tract infection (UTI) and in all except one patient, secondary PHA recovered with only UTI treatment before applying surgical correction. All the patients were younger than 3 months age. In three patients with PUV diagnosis, salt wasting recurred in an UTI episode under 3 months of age.

Elemental mercury intoxication in 7 patients admitted to a pediatric rheumatology clinic.

Paç Kisaarslan A, Sözeri B, Bastug F, Gündüz Z, Yel S, Nalçacioglu H, Sahin N, Özdemir Çiçek S, Poyrazoglu H, Düsünsel R. Elemental mercury intoxication in 7 patients admitted to a pediatric rheumatology clinic. Turk J Pediatr 2019; 61: 786-790. Mercury (Hg) is a toxic heavy metal that can be classified into three groups; organic (methyl), inorganic (mercuric), and elemental (metallic) mercury(Hg0). Mercury intoxication occurs mostly with the elemental form which can potentially damage the function of any organ, or any subcellular structure. The target organ of mercury is the brain, but peripheral nerve function, renal function, immune function, endocrine and muscle function, and several types of dermatitis have been described. We present 7 patients admitted to a pediatric rheumatology clinic with severe extremity pain. One of the patients had acrodynia, two of them had hypertension, two of them had tubulopathy, and three of them had neuropathy. The treatments were Dimercaptosuccinic acid and metalcaptase. In this report, we emphasize that mercury intoxication should be kept in mind with unexplained extremity pain. Timely diagnosis and treatment may prevent severe morbidity and mortality.

Increased circulating endothelial microparticles in children with FMF.

OBJECTIVE: Endothelial microparticles (EMPs) are considered as markers of endothelial dysfunction. In this study, we aimed to examine whether there is endothelial dysfunction in children with familial Mediterranean fever (FMF), hypothesizing that endothelial dysfunction would be present especially with acute-phase response in the active period of the disease. METHODS: This cross-sectional study included 65 FMF patients (41 attack free, 24 attack period) and 35 healthy controls. Circulating EMPs, serum amyloid A (SAA), and other inflammation markers were measured in all groups. Circulating EMPs were measured using flow cytometry. Study groups were compared for circulating EMP and inflammatory markers. The relationship between EMPs and the activation of the disease was evaluated. RESULTS: The levels of CD144+ and CD146+ EMPs in the FMF attack period group were significantly higher than those of the control group (p < 0.05). The levels of inflammation markers in the attack period group were significantly higher than those of the control and attack-free groups (p < 0.05). In the FMF attack group, the CD144+ and CD146+ EMP were significantly correlated with CRP. CONCLUSIONS: Our results suggest that endothelial damage is present especially in the active period of the disease in children with FMF. The endothelial dysfunction becomes an overt parallel with inflammation.

Voiding cystourethrogram: How much should we be selective?

OBJECTIVE: In this study, we examined the patients' characteristics, who underwent voiding cystourethrography (VCUG), in order to determine any selectivity for indication of this invasive method. MATERIAL AND METHODS: After exclusion of indications of neurogenic bladder or antenatal hydronephrosis and control VCUGs, 159 VCUGs performed in our clinic within one year were evaluated. Patients are divided into three groups accoding to age. Clinical characteristic and findings of renal ultrasonography (US) and renal scintigraphy were examined. RESULTS: Vesicoureteral reflux (VUR) was detected in 61 (38.3%) of 159 patients who underwent cystourethrographic examinations, in 45.8% of the patients with a history of recurrent urinary tract infection (UTI), in 22.0% of the patients with pathological urinary system US without history of recurrent UTI. High-grade reflux rate was significantly more frequent in renal units with pathological US findings. Severe scar was significantly more frequent in renal units with high-grade reflux when compared to renal units without reflux and those with low-grade reflux. Predictive values of recurrent UTI, scarring status and pathological US for VUR were separately analyzed and seen that likelihood of indicating VUR was increased when all 3 risk factors were assessed together. CONCLUSION: Vesicoureteral reflux is a problem in which diagnostic process and management strategy should have to be considered in individualized manner for each patient. Before prescribing invasive VCUG, imaging urinary system by US and scintigraphy and determining whether there is recurrent UTI will improve selectivity and success of VCUG.

Urinary oxalate to creatinine ratios in healthy Turkish schoolchildren.

AIM: we aimed to establish reference values for urinary oxalate to creatinine ratios in healthy children aged 6-15 years and to investigate the relationship between their nutritional habits and oxalate excretion. MATERIALS AND METHODS: Random urine specimens from 953 healthy children aged 6-15 years were obtained and analyzed for oxalate and creatinine. Additionally, a 24-h dietary recall form was prepared and given to them. The ingredient composition of the diet was calculated. The children were divided into three groups according to age: Group I (69 years, n = 353), Group II (10-12 years, n = 335), and Group III (13-15 years, n = 265). RESULTS: The 95th percentile of the oxalate to creatinine ratio for subjects aged 6-9, 10-12, and 13-15 years were 0.048, 0.042, and 0.042 mg/mg, respectively. The oxalate to creatinine ratio was significantly higher in Group 1 than in Group 2 and Group 3. Urinary oxalate excretion was positively correlated with increased protein intake and negatively correlated with age. A significant positive correlation was determined between urinary oxalate excretion and the proline, serine, protein, and glycine content of diet. Dietary proline intake showed a positive correlation with the urine oxalate to creatinine ratio and was found to be an independent predictor for urinary oxalate. CONCLUSIONS: These data lend support to the idea that every country should have its own normal reference values to determine the underlying metabolic risk factor for kidney stone disease since regional variation in the dietary intake of proteins and other nutrients can affect normal urinary excretion of oxalate.

Neurological Manifestations in Familial Mediterranean Fever: Results of 22 Children from a Reference Center in Kayseri, an Urban Area in Central Anatolia, Turkey.

Background Familial Mediterranean fever (FMF) is an inherited inflammatory disorder characterized by attacks of fever with polyserositis. Objective The purpose of this study was to evaluate pediatric patients with FMF who had central nervous system (CNS) findings. Materials and Methods Our medical records database for 2003 to 2014 was screened retrospectively. In total, 104 patients with FMF were identified, 22 of whom had undergone neurological examination for CNS symptoms. Results Neurological findings included headache in 16 patients (72.7%), epilepsy in 6 patients (27.3%), pseudotumor cerebri in 2 patients (9.1%), tremor in 2 patients (9.1%), and multiple sclerosis in 1 patient (4.5%). The most common MEFV gene mutation was homozygous M694V (40.9%). Conclusions Patients with FMF can present with various CNS manifestations. Further studies that include large populations are needed to elucidate the neurological manifestations of FMF.

A novel assessment tool for clinical care of patients with autoinflammatory disease: juvenile autoinflammatory disease multidimensional assessment report.

OBJECTIVES: To develop and test a new multidimensional questionnaire for assessment of children with auto-inflammatory disease (AID) such as FMF, PFAPA, HIDS, TRAPS in standard clinical care. METHODS: The juvenile auto-inflammatory disease multidimensional assessment report (JAIMAR) includes 16 parent or patient-centered measures and four dimensions that assess functional status, pain, therapeutic compliance and health-related quality of life (physical, social, school, emotional status) with disease outcome. It is proposed for use as both a proxy-report and a patient self-report, with the suggested age range of 8-18 years for use as a self-report. RESULTS: 250 children with FMF were included in the study. Total of 179 forms were filled up by parents and patients, and 71 forms were filled up by parents having children less than 8 years. Completing and scoring the JAIMAR can be done in 15 minutes. For the JAIMAR's dimensions, the Cronbach's alpha coefficient for internal consistency was between 0.507-0.998. There was a significant and a positive correlation between the test-retest scale scores (ICC=0.607-0.966). Concerning construct validity, all factors loadings were above 0.30. For the criterion validity, the correlation level between each dimension and the related scale ranged from medium (r=0.329, p<0.0001) to large (r=0.894, p<0.0001). The parents' proxy-reported and children's self-reported data were outstandingly concordant (r=0.770-0.989). CONCLUSIONS: The development of the JAIMAR introduces a new and multi-dimensional approach in paediatric rheumatology practice. It is a new tool for children with auto-inflammatory dis-ease and it may help enhance their quality of care.

Compare the effects of intravenous and intraperitoneal mesenchymal stem cell transplantation on ultrafiltration failure in a rat model of chronic peritoneal dialysis.

AIM: The purpose of this study was to compare the possible healing effects of intraperitoneal (IP) and intravenous (IV) mesenchymal stem cell (MSC) transplantation on ultrafiltration failure (UFF) in a chronic rat model of peritoneal dialysis (PD). METHODS: Rats were initially divided into two groups. The UFF-group received once-daily IP injections of 20 mL of 3.86% glucose PD solution for six weeks to stimulate the development of UFF, and a control group received no injections. The UFF group was sub-divided into four groups: an UFF-C group, a MSC-IP group, a MSC-IV group and a placebo (P) group. Peritoneal equilibration tests (PETs) and peritoneal biopsies were performed in the control and UFF-C groups. MSCs were administered by IP injection in the MSC-IP group and by IV injection in the MSC-IV group. The P group received IP injection of placebo. PETs and peritoneal biopsies were performed in the MSC-IP, MSC-IV and P groups at the three weeks after receiving MSCs or placebo. RESULTS: When compared with the control group, ultrafiltration capacity significantly decreased, and the submesothelial thickness increased in the UFF-C and P group, but there were no differences between the control and MSC-IP and MSC-IV groups. The rate of glucose transport was high in the UFF-C and P group compared with the control group, and D/PCr rates in the UFF-C and P group were lower than in the control group. However, D/D0glucose was higher and D/PCr was lower in the MSC-IP group than in the UFF-C and P groups, but D/D0glucose rate of MSC-IV group similar to UFF-C and P groups and there was no difference between MSC-IV group and the other groups in terms of D/PCr rates. The MSC-IP, MSC-IV and P groups had significantly decreased tumor necrosis factor α concentrations compared with the UFF-C group. MSC-IP group had lower levels of TGF-ß1 compared with the P group; MSC-IP group had also lower levels of interleukin-6 compared with UFF-C group. CONCLUSION: The UFF group had a high permeability UFF. These results showed that IV and IP MSC transplantation exerted positive effects on UFF in a chronic rat model of PD. However, healing effect of small solute transport in MSC-IP group was better than MSC-IV group. IP MSC transplantation may be more effective than IV MSC transplantation for the renewal of the peritoneum in chronic PD patients with UFF.

Could mini-PET be used to instead of 4 h original-PET to assess peritoneal permeability in children on peritoneal dialysis?

BACKGROUND: Original peritoneal equilibration test (PET) is an implementation that requires hard work for peritoneal dialysis (PD) staff. Therefore, several authors have attempted to validate short and fast PET protocols, with controversial results. The aim of this study was to evaluate the concordance between the mini-PET and original PET in children. METHODS: In 26 stable continuous ambulatory PD patients, we performed an original PET with 2.27% (4 h) and a mini-PET with 3.86% glucose PD fluid (1 h) and compared ultrafiltration (UF) and small solute transports obtained with the two methods. RESULTS: Twenty-six children, 14 males, mean age 11.4 ± 5.6 (range 2.5-19 years), were included. Meantime on PD at time of enrollment was 35.2 ± 24.5 months (range 6-84 months). Based on the 4-h creatinine D/P data, the number of the patients within each transport category was as follow: high, 5; average, 18; low, 3. Kappa test showed a significant concordance between original PET and mini-PET (k=0.610). Based on the 4-h glucose D/D0 data, the number of the patients within each transport category was as follow: high, 5; average, 17; low, 4. Kappa test showed a moderate agreement between original PET and mini-PET (0.514, p=0.000). When Pearson correlation analysis between original PET and mini-PET was performed, there were significant positive correlations between original 2.27% PET and mini-PET (r=0.720, p=0.000, r=0.638, p=0.000, respectively). When comparing the numeric results of mini-PET and 4 h of original PET for D/Creatinine, by simple regression analysis, we found statistically significant correlation among PETs. CONCLUSIONS: In this study, we showed concordance between the mini-PET and original PET. The 3.86% mini-PET is simple and fast methods to assess free water transport. This also gives information about total UF and small solute transports and it is in good agreement with the original PET.

Trace elements in children suffering from idiopathic nephrotic syndrome.

OBJECTIVE: Trace elements play a significant role in several metabolic processes and often circulate in the blood binding to protein. The purpose of this study was to determine the status of selenium, zinc, and boron in idiopathic nephrotic syndrome patients in active and remission phases. MATERIALS AND METHODS: Fourteen patients and fourteen healthy age-matched controls were included in the study. The selenium, zinc and boron level in plasma and urine were measured by the inductively coupled plasma mass spectrometry. RESULTS: The plasma levels of zinc and selenium were significantly lower in both active and remission patients (for all p=0.0001). The plasma boron level was significantly lower only in patients in active phase (p=0.0002 vs control). The concentrations of urinary boron and selenium were significantly higher during active phase compared with remission (p=0.0003 and 0.0001, respectively). CONCLUSION: Supplementation with zinc, selenium and boron may be justified in patients suffering with this disease.

Hemolytic uremic syndrome outbreak in Turkey in 2011.

The aim of this retrospective multicenter study was to define the epidemiological and clinical features and prognostic factors of the first diarrhea-related hemolytic uremic syndrome (D+HUS) outbreak in Turkey in 2011. All pediatric nephrology centers in Turkey were asked about D+HUS patients via e-mail. Seventy D+HUS patients (median age: 5.7 years) participated. The seasonal peak was around the 7th, 8th and 9th months with 44 cases, centered in the east Marmara region. No causative agent could be identified. The rate of neurological complications and mortality was 21.4% and 4.2%, respectively. Eculizumab was used in four cases. Two of them had severe neurological complications despite plasma exchange. Elevated polymorphonuclear leukocyte count during hospital admission was the predictor of both severe disease and poor outcome. Duration of prodrome was the predictor of poor outcome (p<0.05). In conclusion, the median age of the affected children was greater than in the previous reports, while clinical features and outcome were similar.