Influence of Reduced Folate Carrier and Aminoimidazole Carboxamide Ribonucleotide Transformylase gene polymorphisms on the efficacy of methotrexate in juvenile idiopathic arthritis

PubMe

COVID-19 in pediatric nephrology centers in Turkey

Background/aim: There is limited data on COVID-19 disease in children with kidney disease. We aimed to investigate the characteristics and prognosis of COVID-19 in pediatric nephrology patients in Turkey. Materials and methods: This was a national, multicenter, retrospective cohort study based on an online survey evaluating the data between 11th March 2020 and 11th March 2021 as an initial step of a detailed pediatric nephrology COVID-19 registry. Results: Two hundred and three patients (89 girls and 114 boys) were diagnosed with COVID-19. One-third of these patients (36.9%) were between 10–15 years old. Half of the patients were on kidney replacement therapy: kidney transplant (KTx) recipients (n = 56, 27.5%), patients receiving chronic hemodialysis (n = 33, 16.3%) and those on peritoneal dialysis (PD) (n = 18, 8.9%). Fifty-four (26.6%) children were asymptomatic. Eighty-two (40.3%) patients were hospitalized and 23 (28%) needed intensive care unit admission. Fifty-five percent of the patients were not treated, while the remaining was given favipiravir (20.7%), steroid (16.3%), and hydroxychloroquine (11.3%). Acute kidney injury developed in 19.5% of hospitalized patients. Five (2.4%) had MIS-C. Eighty-three percent of the patients were discharged without any apparent sequelae, while 7 (3.4%) died. One hundred and eight health care staff were infected during the study period. Conclusion: COVID-19 was most commonly seen in patients who underwent KTx and received HD. The combined immunosuppressive therapy and frequent exposure to the hospital setting may increase these patients’ susceptibility. Staff infections before vaccination era were alarming, various precautions should be taken for infection control, particularly optimal vaccination coverage

Effect of Wrist Involvement on Median Nerve Electrophysiology in Juvenile Idiopathic Arthritis

Objectives: Localized compression of the median nerve due to wrist arthritis is a frequently reported complication in adult patients with rheumatoid arthritis. However, in only two cases median nerve compression has been reported in juvenile idiopathic arthritis (JIA) patients. In this study, we aimed to assess the effect of wrist involvement on median nerve electrophysiology in JIA patients. Patients and methods: Thirty-three patients fulfilling the diagnostic criteria for JIA according to the International League of Associations for Rheumatology and twenty-three healthy controls were enrolled. All subjects underwent a thorough neuromuscular examination and median and ulnar nerve conduction studies. The presence of wrist arthritis was noted. Complete blood count, erythrocyte sedimentation rate, C reactive protein, renal/liver function tests were measured. Results: Sensory examination and provocative tests for CTS were normal both in patient and control groups. Age, height and electrophysiological data of the subjects were compared within three groups: JIA patients with wrist arthritis, those without wrist arthritis and healthy controls. None of the electrophysiological data of median nerve revealed significant differences between groups. Conclusions: In the light of our preliminary results, the median nerve seems not to be affected due to wrist involvement in patients with JIA.Wo

Rituximab for Children With Difficult-to-Treat Nephrotic Syndrome

Background: Since the early 2000s rituximab (RTX) has been thought of as an alternative treatment for steroid-sensitive nephrotic syndrome (SSNS) and steroid-resistant nephrotic syndrome (SRNS)., Objective: This study aimed to determine the effects of RTX treatment on disease outcome and growth in pediatric SSNS and SRNS patients., Materials and Methods: The medical records of pediatric SSNS and SRNS patients that began RTX treatment at the mean age of 10.8 ± 5.1 years between 2009 and 2017 were retrospectively reviewed. Additionally, the effect of RTX on growth was evaluated based on patient height, weight, and BMI z scores., Results: The study included 41 children, of which 21 had SSNS and 20 had SRNS. Mean age at diagnosis of NS was 5.8 ± 4.7 years. Mean duration of post-RTX treatment follow-up was 2.3 ± 1.6 years. Among the SSNS patients, 6 and 11 patients were steroid free and calcineurin inhibitor free at the last follow-up visit, respectively. The 1-year cumulative steroid and calcineurin inhibitor doses both decreased after RTX treatment, as compared to before RTX (P = 0.001 and P = 0.015, respectively). The median height z-score at the time of RTX initiation was −1.2 and the median height z-score at the last follow-up visit was −0.6 (P = 0.044). The median BMI z-score decreased from 1.6 (IQR; 0.9–3.0) at the time RTX was initiated to 1.1 IQR; [(−0.7)−2.5] at the last follow-up visit (P = 0.007). At the last follow-up visit 4 SRNS patients had complete remission and 4 had partial remission. The 1-year cumulative steroid dosage in the SRNS patients decreased significantly after RTX, as compared to before RTX (P = 0.001). The median height z-score at the time of RTX initiation was −0.8 and the median height z-score at the last follow-up visit was −0.7 (P = 0.81). The median BMI z-score decreased from 0.3 at the time RTX was initiated to −0.1 at the last follow-up visit (P = 0.11)., Conclusion: RTX has a more positive effect on disease outcome and growth in SSNS patients than in those with SRNS.PubMedWoSScopu

Chronic kidney disease in children in Turkey

To determine the incidence, etiology and treatment patterns of chronic kidney disease (CKD) in children a questionnaire was sent to pediatric nephrology centers in Turkey, asking them to report patients under the age of 19 years who had estimated glomerular filtration rates (GFRs) of a parts per thousand currency sign75 ml/min per 1.73 m(2) body surface area, diagnosed in 2005. Twenty-nine centers reported 282 patients (123 female/159 male; mean age 8.05 +/- 5.25 years). Urological problems such as vesicoureteral reflux (18.5%), obstructive uropathy (10.7%) and neurogenic bladder (15.1%) were the leading causes of CKD. The majority of the patients were in stage V (32.5%), IV (29.8%) or III (25.8%). Growth retardation (49.6%) and secondary hyperparathyroidism (72.4%) were very frequent. The GFR levels correlated positively with hemoglobin/hematocrit and calcium levels and negatively with phosphorus and parathyroid hormone (PTH) levels (P < 0.05). Renal replacement therapies were initiated in 35.8% of the patients, peritoneal dialysis in 23%, hemodialysis in 10.6% and transplantation in 2.2%. This was the first multi-center, epidemiologic study done in children with CKD in Turkey. The most striking result was the predominance of vesicoureteral reflux (VUR) or related urological problems as the underlying causes of CKD. Thus, early diagnosis and treatment of these conditions are of vital importance to decrease the incidence of CKD in children

A rare cause of nephrotic syndrome-sphingosine-1-phosphate lyase (SGPL1) deficiency: 6 cases and a review of the literature

Background Recently, recessive mutations in SGPL1 (sphingosine-1-phosphate lyase), which encodes the final enzyme of sphingolipid metabolism, have been reported to cause steroid-resistant nephrotic syndrome, adrenal insufficiency, and many other organ/system involvements. We aimed to determine the clinical and genetic characteristics, and outcomes in patients with SGPL1 mutations