IgA vasculitis with nephritis (IgAVN) is the most common vasculitis in children. Treatment recommendations are, due to a lack of evidence, based on expert opinion resulting in variation. The aim of ...this study was to describe clinical presentation, treatment and outcome of an extremely large cohort of children with biopsy proven IgAVN to identify prognostic risk factors and signals of treatment efficacy.
Retrospective data were collected on 1148 children with biopsy proven IgAVN between 2005 and 2019 from 41 international paediatric nephrology centres across 25 countries and analyzed using multivariate analysis. The primary outcome was estimated glomerular filtration rate (eGFR) and persistent proteinuria at last follow up.
The median follow up was 3.7 years (IQR 2-6.2). At last follow up, 29% of patients had an eGFR < 90 ml/min/1.73m2, 36% had proteinuria and 3% had chronic kidney disease stage 4-5. Older age, lower eGFR at onset, hypertension and histological features of tubular atrophy and segmental sclerosis were predictors of poor outcome. There was no evidence to support any specific second line immunosuppressive regimen to be superior to others, even when further analysing subgroups of children with reduced kidney function, nephrotic syndrome or hypoalbuminemia at onset. Delayed start of immunosuppressive treatment was associated with a lower eGFR at last follow up.
In this large retrospective cohort, key features associated with disease outcome are highlighted. Importantly there was no evidence to support that any specific immunosuppressive treatments were superior to others. Further discovery science and well-conducted clinical trials are needed to define accurate treatment and improve outcomes of IgAVN.
Objective
Subclinical inflammation, an insidious feature of familial Mediterranean fever (FMF), can lead to life-threatening amyloidosis. We aimed to investigate acute phase reactants and complete ...blood count parameters to identify a useful marker for subclinical inflammation in children with FMF. A secondary aim was to identify an association between subclinical inflammation and specific Mediterranean fever (MEFV) gene mutations.
Methods
This study included 420 pediatric patients with FMF. Laboratory parameters of patients during the attack-free period and MEFV gene mutation analyses were recorded.
Results
Of the 420 patients, 88 (21%) had subclinical inflammation. Of those with subclinical inflammation, 48 (55%) had mutations in exon 10, 36 (41%) had M694V mutation, and 10 (11%) had M694V homozygous mutation. Red cell distribution width (RDW) value was higher in exon 10, M694V, and M694V homozygous mutations compared to other mutations. RDW was positively correlated with serum amyloid A (SAA) (
r
= 0.390,
p
= 0.0001). Analysis of a receiver-operating characteristic curve of RDW revealed that its optimal cut-off value for subclinical inflammation was 12.69%, its sensitivity was 64.10%, and its specificity was 50.90%. The area under the curve was 0.616 (
p
= 0.004, 95% confidence interval = 0.538–0.695).
Conclusion
We suggest that RDW can be used as a screening test as a marker of subclinical inflammation. A high RDW value should alert the clinician about subclinical inflammation in FMF children’s patients with M694V (heterozygous, homozygous, compound heterozygous) mutation.
Key Points
• Subclinical inflammation in FMF patients can lead to amyloidosis.
• RDW can be a predictor of subclinical inflammation.
• RDW can be used as a screening test for subclinical inflammation in FMF patients with M694V mutation.
ABSTRACT Background Two earthquakes on 6 February 2023 destroyed 10 cities in Türkiye. We report our experience with pediatric victims during these catastrophes, with a focus on crush syndrome ...related–acute kidney injury (Crush-AKI) and death. Method Web-based software was prepared. Patient demographics, time under rubble (TUR), admission laboratory data, dialysis, and kidney and overall outcomes were recorded. Results A total of 903 injured children (median age 11.62 years) were evaluated. Mean TUR was 13 h (interquartile range 32.5, max 240 h). Thirty-one of 32 patients with a TUR of >120 h survived. The patient who was rescued after 10 days survived. Two-thirds of the patients were given 50 mEq/L sodium bicarbonate in 0.45% sodium chloride solution on admission day. Fifty-eight percent of patients were given intravenous fluid (IVF) at a volume of 2000–3000 mL/m2 body surface area (BSA), 40% at 3000–4000 mL/m2 BSA and only 2% at >4000 mL/m2 BSA. A total of 425 patients had surgeries, and 48 suffered from major bleeding. Amputations were recorded in 96 patients. Eighty-two and 66 patients required ventilator and inotropic support, respectively. Crush-AKI developed in 314 patients (36% of all patients). In all, 189 patients were dialyzed. Age >15 years, creatine phosphokinase (CK) ≥20 950 U/L, TUR ≥10 h and the first-day IVF volume <3000–4000 mL/m2 BSA were associated with Crush-AKI development. Twenty-two deaths were recorded, 20 of 22 occurring in patients with Crush-AKI and within the first 4 days of admission. All patients admitted after 7 days survived. Conclusions These are the most extensive pediatric kidney disaster data obtained after an earthquake. Serum CK level was significantly associated with Crush-AKI at the levels of >20 950 U/L, but not with death. Adolescent age and initial IVF of less than 3000–4000 mL/m2 BSA were also associated with Crush-AKI. Given that mildly injured victims can survive longer periods in the disaster field, we suggest uninterrupted rescue activity for at least 10 days.
Background
Obesity in the pediatric population is a severe public health problem and is associated with various comorbidities. Renal length is an important clinical parameter for the diagnosis and ...follow-up of renal diseases. The aim of this study was to determine the relationship between renal length (measured ultrasonographically) and body mass index (BMI) in obese children, and to develop nomograms for renal length according to BMI.
Methods
Renal ultrasound was performed in 368 children without renal disease. Each child’s age, gender, weight, height, and BMI (kg/m
2
) were recorded. The children were divided into three groups according to BMI percentiles: obese group: BMI ≥ 95th percentile; overweight group: BMI 85th–94th percentile; normal weight group: BMI 5th–84th percentile.
Results
Weight, height, BMI, and right and left renal length differed significantly between the three groups (
p
= 0.001). There were significant correlations between renal length with age, weight, height, and BMI. Measurement of renal length was independently associated with BMI, age, and height. BMI was used to create renal length nomograms for obese children, based on multiple regression analysis (
R
2
= 0.32 and
p
= 0.0001). Mean renal length was highest in the obese group (96.9 ± 13.4 mm) and lowest in the normal weight group (88.3 ± 12.9 mm).
Conclusions
Ultrasonographic measurement of the renal length according to BMI in children can be a useful method in evaluating these children. Smaller-than-normal kidneys can easily remain undiagnosed in obese and overweight children and this nomogram offers an additional method to evaluate the renal size in obese children.
Background
Reflux nephropathy is the most serious complication of vesicoureteral reflux (VUR). The aim of this study was to assess the role of urinary levels of neutrophil-gelatinase-associated ...lipocalin (NGAL),kidney injury molecule-1 (KIM-1), and liver-type fatty-acid-binding protein (L-FABP) in the early diagnosis of reflux nephropathy in patients with VUR.
Methods
This study assessed 123 patients with primary VUR and 30 healthy children as a control group. The children were divided into five groups: Group A, patients with VUR and renal parenchymal scarring (RPS); Group B, patients with VUR and without RPS; Group C, patients with RPS and resolved VUR; Group D, patients with resolved VUR and without RPS; Group E, healthy reference group.
Results
Median urinary NGAL (uNGAL)/Creatinine (Cr) was significantly higher in patients with than those without RPS and the control group (
p
= 0.0001). Median uKIM-1/Cr was similar in all groups (
p
= 0.417). Median uL-FABP/Cr was significantly higher in patients with RPS than in the reference group (
p
< 0.05).
Conclusions
Urinary NGAL levels may be used as a noninvasive diagnostic marker for predicting renal scarring in reflux nephropathy.
Objective: It is unclear whether exon 2 mutations are variations or a mutations that causes the disease. This study aimed to evaluate the clinical features and prognosis exon 2 mutations in Familial ...Mediterranean Fever.
Methods: The clinical features, disease severity and prognosis of all patients with at least one exon 2 mutations were evaluated retrospectively. These data were compared seperately for homozygous (Group 1), heterozygous (Group 2), compound heterozygous (Group 3), and complex alleles (Group 4), and the data were compared by grouping patients into those with and without exon 10 mutations.
Results: There were a total of 119 patients with exon 2 mutations, including 11.7% in Group 1, 36.1% in Group 2, 21.8% in Group 3, and 30.2% in Group 4 were similar in terms of demographic data, clinical characteristics, and disease course. When compared patients with exon 10 mutations (+) to those with exon 10 mutations (-), the exon 10 mutations (+) group had a higher presence of chest pain (100%, p=0.02) and a significantly higher mean Pras severity score (6.66±1.87, 6.01±1.40; p=0.02). Additionally, a higher number of patients with exon 10 mutation (-) achieved remission with treatment (76 (67.9%), 36 (32.1%); p=0.03).
Conclusion: Exon 2 mutations have a milder course and higher remission rates but they should be considered as Familial Mediterranean Fever disease because of their similar clinical presentation and response to colchicine treatment with exon 10 mutations. Early treatment and close follow-up should be performed.
BACKGROUND: Crush Syndrome is a major cause of morbidity and mortality following large-scale catastrophic earthquakes. Since there are no randomized controlled studies on Crush Syndrome, knowledge on ...this subject is limited to expert experience. The primary objective is to analyze the epidemiological and demographic characteristics, clinical outcomes, and mortality factors of earthquake victims after the Pazarcik and Elbistan earthquakes on February 6, 2023. METHODS: This cross-sectional and observational retrospective study evaluated 610 earthquake victims who presented to our center between February 6 and April 30, 2023. Among these patients, 128 with Crush Syndrome were included in the study. Patient information was gathered from hospital records during their stay and from national registries upon referral. The primary outcome was to identify risk factors for mortality. Demographic and laboratory data were analyzed by acute kidney injury (AKI) stages; mortality-affecting factors were identified through regression analysis. RESULTS: Of the 128 Crush Syndrome patients (100 adults, 28 children), 64 were female. The AKI rate was 32.8%. Among patients with AKI, the frequency of hemodialysis requirement was 69%, and the mortality rate was 14.2%. The overall mortality rate for patients with Crush Syndrome was 4.6%, compared to 3.9% (19/482) in earthquake victims without Crush Syndrome (p=0.705). Notably, low systolic blood pressure at admission was the only factor significantly affecting mortality in Crush Syndrome patients (Hazard Ratio HR: 1.088, p=0.02l, 95% Confidence Interval CI). CONCLUSION: Our study highlights low systolic blood pressure upon admission as a significant risk factor for increased mortality in Crush Syndrome patients. This finding may contribute to the literature by emphasizing the importance of monitoring blood pressure under rubble and administering more aggressive fluid therapy to patients with low systolic blood pressure. Keywords: Crush Syndrome; Pazarcik; Elbistan earthquake; renal disaster. AMAC: Crush Sendromu, buyuk olcekli katastrofik depremlerden sonra morbidite ve mortalitenin en onemli nedenlerinden biridir. Ezilme Sendromu ile ilgili randomize kontrollu bir calisma bulunmadigindan, bu konudaki bilgiler uzmanlarin deneyimleriyle sinirlidir. Bu calismanin temel amaci, 06 Subat 2023 tarihinde meydana gelen Pazarcik ve Elbistan depremleri sonrasinda depremzedelerin epidemiyolojik, demografik ozelliklerini, klinik sonuclarini ve mortalite faktorlerini analiz etmektir GEREC VE YONTEM: Bu kesitsel ve gozlemsel retrospektif calismada, 6 Subat--30 Nisan 2023 tarihleri arasinda merkezimize basvuran 610 depremzede degerlendirildi. Bu hastalar arasinda Crush Sendromu olan 128 hasta calismaya dahil edildi. Hastalara ait bilgiler, hastaneye yatislari sirasinda hastane kayitlarindan ve sevk edildiklerinde ulusal kayitlardan elde edildi. Birincil sonucumuz mortalite icin risk faktorlerini belirlemekti. Demografik ve laboratuvar verileri akut bobrek hasari evrelerine gore karsilastirildi; mortaliteyi etkileyen faktorler regresyon analizi ile belirlendi. BULGULAR: 128 Crush Sendromu hastasinin (100 yetiskin, 28 cocuk) 64'u kadindi. AKI orani %32.8 idi. AKI hastalari arasinda hemodiyaliz gereksinimi sikligi %69 ve mortalite orani %14.2 idi. Olum orani Crush Sendromu olanlarda %4.6 iken, Crush Sendromu olmayan depremzedelerde %3.9 (19/482) idi. (p: 0.705) Carpici bir sekilde, Crush sendromlu hastalarda mortaliteyi onemli olcude etkileyen sadece basvuru sirasindaki dusuk sistolik kan basincidir. (HR: 1.088, p: 0.021 %95 C.I.) SONUC: Calismamiz, basvuru sirasindaki dusuk sistolik kan basincinin Crush Sendromlu hastalarda artmis mortalite icin onemli bir risk faktoru oldugunu vurgulamistir. Bu sonuc, enkaz altinda kan basincinin olculmesi ve dusuk sistolik kan basinci olan hastalara daha agresif sivi tedavisi verilmesi konusunda literature katki saglayabilir. Anahtar sozcukler: Crush Sendromu, Pazarcik, Elbistan Depremi, Renal Afet
Cardiac Function in Children After Kidney Transplant Arslan, Alev; Parmaksız, Gönül; Noyan, Zekiye Aytül ...
Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation,
01/2023, Letnik:
21, Številka:
1
Journal Article
Recenzirano
OBJECTIVESRenal transplant improves echocardiographic markers of systolic and diastolic heart functions. The aim of this study was to evaluate the gradual changes in left and right ventricle ...functions in children and young adults before and after renal transplant. MATERIALS AND METHODSThirty kidney recipients of median age 13 years (range, 5-19 years) were included the study. Tissue Dopplerimaging from the septal and lateral mitral annulus ofthe left ventricle and free wall of the right ventricle was performed. Right ventricle systolic excursion velocity and tricuspid annular plane systolic excursion were calculated. Systolic and diastolic heart functions-which gained just before transplant, were compared with posttransplant early- term (6 months to 1 year) and long-term (longer than 1 year) functions. RESULTSTwelve patients received deceased-donor and 18 patients received living donor renal transplant. Follow-up after transplant was 44 ± 23 months. Left ventricle ejection fractions were normal. The left ventricle, right ventricle, and interventricular septalTei indices were significantly higher before transplant.The posttransplantation early- and late-term results of left ventricle,right ventricle, and interventricular septal Tei indices were similar. Tricuspid annular plane systolic excursion levels were abnormal in 11 patients (36%), and right ventricle systolic excursion velocities were abnormal in 7 patients (23%) before transplant. All tricuspid annular plane systolic excursion levels and 94% ofright ventricle systolic excursion velocities were normal, but left ventricle Tei indices were higher in 8 (26%) and right ventricle Tei indices were higher in 14 patients (46%) at late-term follow-up. CONCLUSIONSThe systolic and diastolic dysfunctions of both ventricles appear to be highly prevalent in pediatric renal transplant recipients, especially soon after transplant, and were shown to usually decrease with time. Improvements in right ventricle dysfunction are slower, even in optimally treated posttransplant patients.
