This study aimed to evaluate the efficacy of rituximab in children with difficult-to-treat nephrotic syndrome,
considering the type of disease (steroid-sensitive or –resistant) and the dosing ...regimen.
This multicenter retrospective study enrolled children with difficult-to-treat nephrotic syndrome on rituximab treatment from 13 centers. The patients were classified based on low (single dose of 375 mg/m2) or high (2-4 doses of 375 mg/m2) initial dose of rituximab and the steroid response. Clinical outcomes were compared.
Data from 42 children 20 steroid-sensitive (frequent relapsing / steroid-dependent) and 22 steroid-resistant nephrotic syndrome, aged 1.9–17.3 years were analyzed. Eleven patients with steroid-sensitive nephrotic syndrome (55%) had a relapse following initial rituximab therapy, with the mean time to first relapse of 8.4 ± 5.2 months. Complete remission was achieved in 41% and 36% of steroid-resistant patients, with the median remission time of 3.65 months. At Year 2, eight patients in steroid-sensitive group (40%) and four in steroid-resistant group (18%) were drug-free. Total cumulative doses of rituximab were higher in steroid-resistant group (p = 001). Relapse rates and time to first relapse in steroid-sensitive group or remission rates in steroid-resistant group did not differ between the low and high initial dose groups.
The current study reveals that rituximab therapy may provide a lower relapse rate and prolonged relapse-free survival in the steroid-sensitive group, increased remission rates in the steroid-resistant group, and a significant number of drug-free patients in both groups. The optimal regimen for initial treatment and maintenance needs to be determined.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
In the absence of specific symptomatology in children, the early diagnosis of acute pyelonephritis is a challenge, particularly during infancy. In an attempt to differentiate acute pyelonephritis ...from lower urinary tract infection (UTI), we measured intrarenal resistive index (RI). We evaluated its ability to predict renal involvement as assessed by dimercaptosuccinic acid (DMSA) scintigraphy. In total 157 patients admitted to the pediatric department of the Sişli Etfal Hospital with clinical signs of febrile UTI were included in the study. The children were divided into groups according to their age at the time of ultrasonography (US). RI was measured from the renal arteries with Doppler US in the first 72 h in all 157 children. Renal involvement was assessed by (99m)Tc-DMSA scintigraphy in the first 7 days after admission. The examination was repeated at least 6 months later if the first result was abnormal. All available patients with an abnormal scintigraphy underwent voiding cystourethrography 4-6 weeks after the acute infection. All patients with vesicoureteral reflux and scarred kidneys were excluded from the study. DMSA scintigraphy demonstrated abnormal changes in 114 of 157 children and was normal in the remaining 43 children. Of these 114 children, 104 underwent repeat scintigraphy, of whom 77 showed partially or totally reversible lesion(s). Of these 77 children, 17 children (22%) with vesicoureteral reflux were excluded. Thus, we compared the 43 children with lower UTI with the 60 children with definite acute pyelonephritis at admission. Kidneys with changes of acute pyelonephritis had a mean RI of 0.744+/-0.06 in infants, 0.745+/-0.03 in preschool children, and 0.733+/-0.09 in patients of school age with upper UTI. However, the mean RI was 0.703+/-0.06 in infants, 0.696+/-0.1 in preschool children, and 0.671+/-0.09 in school-aged patients with lower UTI. The mean RI values were significantly higher in patients with upper UTI ( P<0.001). There was a highly significant correlation between RI values and the severity of the renal lesion as ranked by DMSA scintigraphy ( P<0.001). When the cut-off RI value was 0.715, there was an 80% sensitivity and a 89% specificity for diagnosing upper UTI. Refluxing kidneys and scarred kidneys also had higher RI values. In conclusion, RI values were increased significantly in children with febrile UTI when renal parenchymal involvement (assessed by DMSA scintigraphy) was present. Our results also support the view that the children with high RI values are at a high risk of reflux, scarring, or both, which was frequently observed in febrile UTI. This might allow identification of patients at risk for severe renal lesions that require more aggressive therapy, investigation, and follow-up than those with lower UTI.
In this study, it was aimed to investigate the
quality characteristics of fried zucchini slices pre-dried using osmotic dehydration
(OD) method at varying conditions (salt concentration, vacuum or ...ultrasound
application, time). Textural properties (hardness, elasticity, cohesiveness,
chewiness, firmness), surface color values (L*, a*, b*), moisture and oil
content of the final product were determined. Textural properties did not
change with OD applications (p>0.05). Vacuum and solution concentration
applied during OD method had significant effects on surface color values
(p≤0.05). OD did not cause any significant change in final moisture and oil
content of the fried product. In case of ultrasound assisted osmotic
dehydration (US-OD), it was seen that the effect of time on moisture content
was important the solution concentration
of 5% (p≤0.05). On the other hand, changes at salt concentration have created significant
differences in both the application process (p≤0.001). When solution
concentration was 0%, effect of time was negligible to oil content (p>0.05).
Notwithstanding, the solution concentration for both processing time was found
to be significant (p≤0.05).
Byline: Gul Ozcelik (1,3), Tugcin Bora Polat (1), Seniha AktaA (2), Feyzullah Cetinkaya (1) Author Affiliation: (1) Department of Pediatrics, SiAli Etfal Hospital, Istanbul, Turkey (2) Department of ...Radiology, SiAli Etfal Hospital, Istanbul, Turkey (3) PK.198, 80622, 1 Levent, Istanbul, Turkey Article History: Registration Date: 01/01/2004 Online Date: 10/03/2004 Article note: The online version of the original article can be found at
Glucose-6-phosphatase catalytic subunit 3 (G6PC3) deficiency is a recently identified form of congenital neutropenia associated with developmental anomalies. The severity of neutropenia and the ...clinical spectrum are highly variable. Aside from infectious complications and extrahematologic features, inflammatory bowel disease and autoinflammatory complications are less frequently observed manifestations. However, amyloidosis has never been reported in G6PC3 deficiency. Here, we present a 12-year-old patient with incidentally discovered neutropenia because of the p.E65A (c.194A>C) variant of the G6PC3 gene. He had recurrent aphthae and abdominal pain episodes, and developed nephrotic-range proteinuria, amyloidosis, and end-stage renal failure during follow-up.
Objective: Health-related quality of life and its influencing factors in pediatric patients with voiding dysfunction and their families were assessed. Method: The patients who were admitted with ...urinary incontinence for at least 6 months to the clinics of pediatrics and pediatric nephrology were enrolled in the study. The patients were divided into three groups as daytime urinary incontinence (DUI), enuresis and both DUI and enuresis. The Pediatric Quality of Life InventoryTM (PedsQLTM) was administered to patients and mothers. Results: A total of 100 patients with voiding dysfunction admitted with urinary incontinence aged from 5 years to 18 years (mean age: 8.8+ or -2.6) were included in the study. The mean dysfunctional voiding symptom score was 15.5+ or -6.6 in all groups and significantly higher in the group who experienced both DUI and NE (p<0.001). No significant difference was observed in terms of mean PedsQL scores of the children and their mothers between groups with urinary incontinence (65.5+ or -16 and 58.4+ or -13.5, respectively), but a strong positive correlation was detected between them (p<0.001). The mean PedsQL score for the children aged from 8 years to 12 years was significantly lower than the scores for the children aged from 5 to 7 years (p<0.05). The mean PedsQL score for the children aged between 13, and 18 years was not different from the other age groups. Conclusion: The early diagnosis and treatment of patients aged from 8 to 12 years who had the lowest PedsQL score is important. Additionally, the success in the improvement of the quality of life of children with voiding dysfunction requires cooperation with parents. Keywords: Enuresis; incontinence, quality of life, voiding dysfunction Amac: iseme bozuklugu olan cocuklarda ve ailelerinde yasam kalitesinin ve etkileyen faktorlerin incelenmesidir. Yontem: Pediatri ve pediatrik nefroloji polikliniklerine idrar kacirma nedeniyle basvuran ve sikayetleri en az 6 aydir devam eden cocuk hastalar calismaya alindi. Hastalar gunduz idrar kaciranlar, gece idrar kaciranlar ve hem gunduz hem gece idrar kaciranlar olmak uzere uc gruba ayrildi. Cocuklar icin Yasam Kalitesi Olcegi (CIYKO) calismaya katilan tum cocuklara ve annelerine uygulandi. Bulgular: 5-18 yas arasi (ortalama yas: 8,8+ or - 2,6, E:K=0,31) idrar kacirma yakinmasi olan 100 cocuk hasta calismaya alindi. Ortalama disfonksiyonel iseme semptom skorlamasi (DISS) 15.5+ or - 6.6 saptandi. DISS hem gece hem gunduz kaciran grupta istatistiksel olarak anlamli daha yuksek saptandi (p<0.001). Anne ve cocuklarin yasam kalitesi skorlarinda iseme bozuklugu gruplari arasinda anlamli farklilik saptanmadi (58,4+ or -13,5; 65,5+ or -16) fakat kuvvetli pozitif yonde baginti saptandi (p<0.001). 8-12 yas grubunda ortalama CIYKO skoru 5-7 yas grubuna oranla istatistiksel olarak anlamli dusuk bulundu (p<0.05). 13-18 yas grubunun CIYKO skoru diger gruplardan farkli degildi. Sonuc: En dusuk CIYKO skoru saptanan 8-12 yas grubu cocuk hastalarin erken tani ve tedavisi onem tasimaktadir. Ayrica iseme bozuklugu olan cocuklarin yasam kalitelerinin iyilestirilmesindeki basarida ailelerin katkisi onemlidir. Anahtar kelimeler: Enurezis, idrar kacirma, yasam kalitesi, iseme disfonksiyonu
Renal involvement is a fairly frequent development in children with acute lymphoblastic leukemia, but palpable renal enlargement at time of diagnosis is very unusual. We report the case of a young ...girl who presented with enlarged kidneys and was diagnosed with this form of leukemia. This case is of interest because of the rarity of this presentation. The importance of renal biopsy in identifying the etiology of this patient’s nephromegaly is emphasized.
Children requiring kidney replacement therapy experience high burden of cardiovascular (CV) disease leading to increased mortality. Intima-media thickness (IMT) indicating atherosclerosis is a ...validated surrogate marker for future CV events.
We investigated the effect of different treatment modalities (dialysis, preemptive kidney transplantation (KTx), late KTx after dialysis) on IMT by multivariable linear mixed-effect modeling. Patients were enrolled in a prospective cohort study.
A total of 261 analyzed children had a mean follow-up of 3 y. Children after preemptive and late KTx had lower levels of IMT when compared with dialysis. Using an interaction term, a significant progression of IMT over time was seen during dialysis (β = 0.0053 mm/y, P = 0.004). IMT before the start of therapy was the most influential determinant in all models. Low IMT was associated with maintenance steroid treatment after preemptive KTx. High IMT on dialysis was associated with higher systolic blood pressure, lower body mass index, lower serum albumin, and lower bicarbonate.
IMT remained rather stable in children several years after KTx. In contrast, children on dialysis had higher IMT values, which increased over time. In these children, blood pressure control, calorie and protein intake, and acid-base homeostasis seem important. Taken together, children might profit from early transplantation to limit accumulation of CV risk.
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