Comparison of the efficacy of once- and twice-daily colchicine dosage in pediatric patients with familial Mediterranean fever - a randomized controlled noninferiority trial

Background: In this study, we examined the efficacy and safety of a once-daily dosage schema of colchicine compared with a twice-daily dosage schema in pediatric patients with familial Mediterranean fever (FMF). Methods: In this 24-week, multicenter, randomized controlled noninferiority trial, pediatric patients newly diagnosed with FMF carrying a homozygous or compound heterozygous mutation and not receiving any treatment were included. Patients were randomly assigned using a block randomization method to receive treatment with a once- or twice-daily dosage. Clinical and laboratory characteristics and medication side effects were recorded and compared between groups. The study was carried out in compliance with Good Clinical Practice and the Consolidated Standards for Reporting of Trials (CONSORT) statement. Results: A total of 92 patients were selected, and 79 patients completed the study. There were 42 patients in the once-daily dosage group and 37 in the twice-daily dosage group. The results indicated that the once-daily dosage was not inferior to the twice-daily dosage regarding decrease in attack frequency and duration as well as improvement in clinical findings and Mor severity scores. Alterations in laboratory findings indicating inflammation, such as erythrocyte sedimentation rate, C-reactive protein, and serum amyloid A, were similar in both groups. The rates of drug side effects were similar between the once- and twice-daily dosage groups, implying comparable safety of colchicine, with the exception of diarrhea, which was slightly higher in the once-daily dosage group. Conclusions: Using colchicine with either a once- or twice-daily dosage provides similar clinical and laboratory improvements. Considering both efficacy and safety, colchicine can be prescribed with a once-daily dosage. Trial Registration ID: ClinicalTrials.gov identifier NCT02602028. Registered 5 November 2015

Acute Hemorrhagic Edema following COVID-19 Infection

Background: Acute hemorrhagic edema of infancy (AHEI) is an immune complex-mediated leukocytoclastic vasculitis. Despite its worrisome appearance, it has a benign disease course with rare systemic involvement. Clinical Description: A 25-month-old male patient was brought to the pediatric outpatient clinic with a pink oval-shaped plaque-like rash all over the body and edema on the lower extremities and left auricle. The child was diagnosed as a case of coronavirus disease 2019 (COVID-19) 5 days before the present complaints. History and physical examination were otherwise unremarkable. Because of the patient's age, the purpuric appearance of lesions, distribution pattern of the rashes, localized edema, and no end-organ involvement, a possibility of AHEI, triggered by COVID-19, was considered and the patient was evaluated for the same. Management and Conclusions: The patient recovered in 15 days with no end-organ involvement. He was advised to continue regular follow-ups to look for long-term complications. AHEI is a benign condition which may occur in children following COVID-19 infection. It is essential to recognize the condition to avoid unnecessary investigations and treatment

Comparison of Clinical Outcomes of Unilateral Atrophic/ Hypoplastic/Nephrectomized and Solitary Kidney: Clinical Outcomes of Solitary Kidney

Background and Aim: The congenital anomalies of the kidney and urinary tract (CAKUT)are the most common findings in the pediatric age group with congenital malformations. Theaim of this study was to evaluate clinical characteristics and follow-up results of children withunilateral solitary kidney (SK)/hypoplasia/atrophy and nephrectomized kidney.Methods: We retrospectively reviewed the medical records of patients with unilateral atrophic/hypoplastic/SK and nephrectomized kidney who presented to the pediatric nephrology clinicbetween January 2010 and January 2023. The review included demographic data, laboratoryfindings, imaging results, and the clinical course of these patients.Results: Eighty-nine patients were included in the study (M/F=52/37). 42(47.2%) patientshad SK, 29(32.6%) patients had atrophy, 6(6.7%) patients had hypoplasia, and 12(13.5%)patients had undergone nephrectomy. At the last examination, hypertension was present ina total of 8 patients (9%). Hypertension was observed in 16.7% of nephrectomized patientsand 11.9% of SK patients. Proteinuria was detected in 15 patients (16.9%) at the last followupvisit, with the highest frequency observed in nephrectomized patients (33.3%). The fourgroups exhibited notable variations in terms of serum creatinine and phosphorus levels,with the nephrectomized group showing a higher creatinine level (P=0.004) and a lowerphosphorus level (P=0.027) compared to the SK group.Conclusion: Nephrectomy patients exhibit relatively high rates of proteinuria, hypertension,and increased creatinine levels, indicating the need for close monitoring

Evaluation of the Clinical Findings of Pediatric Patients With Vesicoureteral Reflux to Assess Disease Severity: Vesicoureteral Reflux and Disease Severity

Background and Aim: This study aims to investigate how to benefit from clinical andlaboratory methods for further selection in the decision-making process to perform acystogram and assess the severity of vesicoureteral reflux (VUR).Methods: We retrospectively reviewed the voiding cystourethrography (VCUG), ultrasound(US), and medical records of pediatric patients with VUR. The exclusion criteria includedhaving neurological lesions, a posterior urethral valve, and a lack of documentation of a renalultrasound or voiding cystourethrography (VCUG). At the time of data entry, we retrieveddemographic findings and laboratory test results, including routine biochemical parameters,complete blood count, and calculated blood sodium/potassium ratioResults: Sixty-three pediatric patients with VUR were enrolled in this study. The mean (SD)age of the patients (female/male=37/26) at the time of diagnosis was 62.0±6.5 months (range1-195 months). Seventeen patients (26.9%) had high-grade VUR, and 46 patients (73.1%)had low-to-moderate-grade VUR. The mean potassium level of the mean serum sodium topotassium (Na/K) ratio was significantly lower in the high-grade VUR group (4.7±0.5 vs.4.3±0.4 mEq/L, P=0.022, 29±3 vs. 32±3, P=0.029, respectively). The proportion of patientswith severe anterior-posterior (AP) diameter dilation was significantly higher in the lowgradeVUR group than in the high-grade VUR group (4 [23.5%] vs. 35 [76.5%], P=0.005).Conclusion: We conclude that the low serum sodium to potassium (Na/K) ratio allows us topredict the VUR grade. The effect of VUR on the renin-angiotensin-aldosterone system canbe demonstrated by prospective controlled studies

Posterior Reversible Encephalopathy Syndrome in Henoch-Schonlein Purpura and Hemolytic Uremic Syndrome.

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological syndrome, composed of symptoms such as headache, seizures, visual disturbances, lethargy, confusion, stupor, focal neurologic findings and radiological findings of bilateral gray and white matter abnormalities suggestive of edema in the posterior regions of the cerebral hemispheres. PRES is associated with significant morbidity and mortality if it is not expeditiously recognized. Magnetic resonance image (MRI) represents the most sensitive imaging technique for recognizing PRES. PRES has been seen in various clinical settings including renal disorders such as acute glomerulonephritis, lupus nephritis, nephrotic syndrome, and drug usage such as calcineurin inhibitors. We aimed to present two study cases for such clinical setting. In this report, we present two patients with PRES in whom the primary diagnosis was hemolytic uremic syndrome (HUS) and Henoch-Schonlein purpura (HSP). Both of them were treated with anticonvulsant and proper antihypertensive drugs. A repeated MRI scan of the head, an ophthalmologic assessment, and a follow-up electroencephalogram produced normal results with no sequelae. Early recognition of PRES as a complication during different diseases and therapies in childhood may facilitate the appropriate treatment, so that intensive treatment should be performed as soon as possible to avoid neurological sequelae

Humoral Immunity Status in Pediatric Patients with Familial Mediterranean Fever: Exploring the Role of IgA: Familial Mediterranean Fever: What about IgA

Background and Aim:Research on the role of the adaptive immune system in familial Mediterranean fever (FMF) is limited. The aim of this study was to investigate the levels of immunoglobulins (Ig) IgG, IgM, IgA and absolute lymphocyte count (ALC) in FMF patients compared to normal subjects. Methods: Patients diagnosed with FMF were included in the study. At the time of data entry, we recorded demographic information, mutation type, ALC, eosinophils and levels of IgA, IgG, IgM and IgE. We compared these variables with those of the control group. Results: Thirty-five FMF patients (M/F=9/26) were included in this study. There was no significant difference between the mean quantitative levels of the immunoglobulins (IgA, IgG and IgM) and Ig E between the patient and control groups (P>0.05). Compared with the biallelic and monoallelic mutations, we found higher Ig A levels in the biallelic group (1.5±0.7 vs 0.9±0.3 mg/dL, respectively P=0.008). An interesting finding was that three patients with homozygous M694 mutation had an Ig A level above the upper limit while. Conclusion: No significant difference was found in terms of humoral immunity between FMF patients and healthy individuals. However, we believe that IgA levels should be examined in patients with severe FMF manifestations; the finding of high IgA levels in three patients with a homozygous M694V mutation supports our hypothesis

The effect of antidiuretic hormone on urine and serum electrolyte levels in children with primary monosymptomatic nocturnal enuresis

Background/aim: The data concerning the effects of desmopressin on water/electrolyte disturbances of children with primary monosymptomatic nocturnal enuresis (PMNE) are limited. In the present study we aimed to evaluate the effect and tolerability of desmopressin on blood and urine electrolytes and osmolality in PMNE. Materials and methods: Thirty-five children with PMNE between the ages of 5 and 15 participated in the study. Patients collected urine during the daytime and acknowledged the night time fluid restriction before starting to use the desmopressin tablets. The medication was taken orally at least 1 h before bedtime. Blood and urine samples were collected before the introduction of the treatment (day 0) and on the third and seventh days of the administration of desmopressin to determine osmolality and electrolyte levels. Results: Thirty-five patients participated in the study. Twenty-one patients (60\%) were male and 14 (40\%) were female. The mean age was 9.6 +/- 2.7 years. There were no significant changes in serum osmolality, urine osmolality, and serum sodium concentration. Mean urine calcium/creatinine ratio was 0.03 +/- 0.01 mg/mg at the beginning, 0.06 +/- 0.02 mg/mg on the third day, and 0.04 +/- 0.01 mg/mg on the seventh day of the study. No significant changes were seen in urine calcium/creatinine ratio before and after treatment. Conclusion: Desmopressin appeared to be a well-tolerated drug and provided a safe and effective treatment for children who were following fluid intake restriction for PMNE

The comparison of the resistivity index values in the ultrasonographic evaluation of a unilateral atrophic/hypoplastic kidney

WOS: 000521274900001PubMed: 32208786Background: In the study, we aimed to determine the sensitivity of the renal resistivity index (RI) in differentiating hypoplastic and atrophic kidneys in patients with small-sized kidneys, and to evaluate its capacity to predict the renal involvement confirmed by the DMSA scintigraphy. Material and methods: We retrospectively reviewed the ultrasonography (US) and DMSA findings, and medical records of pediatric patients with unilateral diminutive kidneys followed between January 2017 and June 2018. The RI measurements were performed twice, and the mean RI was calculated for each kidney of all patients. Results: Sixty-three (male/female, m/f = 28/35) pediatric patients aged 107.2 +/- 49.4 months (range 14-206 months) were included in this study. The DMSA scintigraphy revealed abnormal changes to atrophic kidneys in 38 patients and hypoplastic kidneys in 25. There were no differences between the groups with atrophy and hypoplasia by age, gender, urine density, and creatinine. The patient group with atrophic kidneys had a mean RI of 0.55 +/- 0.21, and patients with hypoplastic kidneys had a mean RI of 0.67 +/- 0.03. The mean RI and systolic/diastolic rates of the patients with atrophy were significantly lower than of the patients with hypoplastic kidneys (p = 0.042 and p = 0.048, respectively). There was a positive correlation between RI and DFR in the group with atrophy (r = 0.461, p = 0.016), but this was not the case for the group with hypoplastic kidneys (r= -0.066, p = 0.889). Conclusions: The resistivity index might be very useful for differentiating atrophy and hypoplasia in patients with unilateral small kidneys and can be used instead of scintigraphic evaluation

Early outcome findings of treatment for transperitoneal laparoscopy-assisted pyeloplasty

Background We retrospectively analyzed the initial results of laparoscopic pyeloplasty, among pediatric patients undergoing the procedure by transperitoneal access. Methods We retrospectively reviewed the medical records of patients who were operated with transperitoneal laparoscopy-assisted pyeloplasty at our institution between 2015 and 2020. Results The mean age of the 51 patients (M/F = 36/15) was 59 +/- 49.8 months. The mean preoperative renal pelvis anterior-posterior diameter was 32.9 +/- 13.4 mm, and the relative renal function was 42 +/- 12.1% on the operation side. Forty-three (84.3%) patients had no excretion on MAG3 examination preop. The number of patients with no excretion decreased to 10 (19.7%) after surgery. One of them underwent a successful endopyelotomy; eight of them underwent a successful laparoscopic re-pyeloplasty. There was nothing to do in only one patient whose DRF decreased to 11%. The mean anterior posterior diameter decreased significantly to 16.7 +/- 11.2 mm after surgery (p < 0.001). Conclusion Preoperative increased renal pelvis anterior-posterior diameter detected in our study was found to be a risk factor in the failure of transperitoneal laparoscopy-assisted pyeloplasty procedure

Evaluation of Oxidative Stress Biomarkers in Patients with Henoch-Schönlein Purpura

Introduction: Henoch-Schönlein Purpura (HSP) is a systemic vasculitic syndrome characterized by non-thrombocytopenic purpura, arthritis/arthralgia, abdominal pain, and glomerulonephritis. The pathogenesis of HSP has not been clearly identified. Oxidative damage has a role in the pathogenesis of most cases. Aim: This study aimed to evaluate changes of oxidative stress by studying parameters like superoxide dismutase (SOD), catalase (CAT), and malondialdehyde (MDA) in an attempt to identify the role of oxidative stress in HSP from another perspective. Materials and methods: This study enrolled 23 pediatric patients (ten girls and thirteen boys) diagnosed with HSP who were under follow-up at Sutcu Imam University School of Medicine Department of Pediatrics between 2014 and 2016 and twenty healthy children as the control group. The parents of all subjects gave informed consent to participate in the study. In the HSP group, the beginning season of the illness and the systemic involvement during follow-up were determined. Blood specimens were obtained at presentation before any treatment was started. SOD, CAT activities, and MDA values in erythrocyte and plasma samples were compared between the patient group and the healthy children. Results: Twenty-three patients with HSP (13 males, 10 females) and 20 healthy children participated in this study. The mean age of the HSP cases was 8.21±3.78 years (range 2-16 years) and of the controls was 8.6±4.2 (range 3-14 years). The mean MDA value was 2.95±0.71 nmol/ml in the patient group and 2.67±0.66 nmol/ml in the control group (p=0.787). The mean level of the CAT enzyme was 1.32±0.35 U/g Hb in the patient group and 7.8±1.74 U/g Hb in the control group (p=0.001). The mean levels of the SOD enzyme were 3.06±0.85 U/g Hb in the patient group and 0.97±0.36 U/g Hb in the control group (p=0.001). Conclusions: Although high MDA levels support the role of lipid peroxidation in the pathogenesis of HSP, statistical significance was not reached owing to a limited number of our patients. The reduced CAT enzyme activity is consistent with the findings of previous reports. This finding supports the notion that oxidative stress can play a role in the pathogenesis of HSP