To determine the incidence of renal scarring among patients with primary vesicoureteral reflux (VUR) and the possible risk factor(s), we studied 90 children (60 girls and 30 boys) with VUR followed ...in the Pediatric Nephrology Unit at the Ege University Hospital from 1998 to 2003. All the patients were assessed for VUR grade by voiding cystoureterography and for presence of renal scarring by (99 m) technetium dimercapto-succinic acid scintigraphy. All infants with VUR were given low-dose prophylactic antibiotics and followed-up until resolution of the reflux. Grade of reflux and number of urinary tract infection (UTI) episodes (≥3) were found to be statistically significant risk factors for renal scarring (P <0.05). However, gender, familial history and laterality of the disease were not found to be statistically significant risk factors (P >0.05). Similarly, there was no statistically significant difference of frequency of renal scarring among the different age groups (P >0.05). We conclude that recurrences of UTI and VUR severity are significant risk factors for renal scarring in children with VUR. Therefore, identification of VUR at an early age may offer the opportunity to prevent episodes of UTI and possible formation of renal scars that may result in end-stage renal failure.
Nephrotic syndrome (NS) in the first year of life is uncommon and makes up a heterogeneous group of disorders. Subsequent studies have further defined the phenotype associated with mutations in the ...NPHS2 gene, revealing that patients usually develop NS from birth to 6 years of age. We report a child aged 4 months with steroid-resistant NS who had polymorphism of NPHS1 (E117K) and mutation of NPHS2 (P118L). Our patient was carrying a polymorphic NPHS1 mutation, while phenotypically she had a poor prognostic NPHS2 mutation. However, it must be questioned whether this polymorphic change (E117K) alters the signaling pathways of the podocytes and leads to P118L mutation, thus making it behave differently. Perhaps, this would be called a genetic modifier in future.
Focal and segmental glomerulosclerosis (FSGS) is a major cause of idiopathic steroid-resistant nephrotic syndrome (SRNS) and end-stage renal disease (ESRD). In this retrospective study, we report on ...34 pediatric patients with FSGS who were diagnosed and treated from 1992 to 2006. The mean age at onset was 6.3 + or - 4.3 years. All patients had nephrotic-range proteinuria. Microscopic hematuria was seen in three patients and hypertension was seen in 15 patients at presentation. All patients were treated with steroids (oral and/or methylprednisolone), while 23 patients received cytotoxic therapy in addition. The mean follow-up period was 8.6 + or - 3.3 years at the end of which, 59% of patients achieved complete or partial remission, 20.5% continued to have active renal disease while 20.5% of the patients developed CKD. Our study suggests that most of the patients with FSGS progress to renal insufficiency. Steroid therapy increases the chances of remission and preserves renal function in patients with sporadic primary FSGS.
Renal abscess is uncommon in childhood. The common manifestations include fever, lumbar pain, abdominal pain and occasional flank mass. Renal ultrasonography enables us to achieve an early diagnosis, ...however; it may still be insufficient to distinguish it from pyelonephritis. Renal abscesses are usually associated with different predisposing conditions. In the present report, we aim to describe a case of a previously healthy child who developed a renal abscess.
Proteinuria is an important factor for renal injury and prognosis in many diseases. The most valuable method for evaluation of proteinuria is quantitative protein analysis in appropriately 24-h ...collected urine. But urine collection is difficult and cumbersome especially in children and working adults. The aim of the present study is to define the usefulness of urinary protein/urine osmolality (Uprot/Uosm) ratio in quantification of proteinuria. One hundred and seventy-one patients whose age ranged between 3 and 14 years were included in the study. Uprot/Uosm (
r
= 0.85,
P
< 0.001) and urinary protein/creatinine Uprot/Ucrea (
r
= 0.81 and
P
< 0.001) ratios were significantly correlated with 24-h protein excretion. Twenty-four-hour protein excretion was correlated with Uprot/Ucrea (
r
= 0.76,
P
< 0.001) and Uprot/Uosm (
r
= 0.79,
P
< 0.001) ratios in proteinuric group. But, there was no correlation between Uprot/Uosm and Uprot/Ucrea ratios with 24-h protein excretion in non-proteinuric group. The positive and negative predictive value of Uprot/Uosm ratio of 0.28 mg/l/mOsm/kg was 89.9 and 90.8% and Uprot/Ucrea ratio of 0.24 mg/mg was 85.7 and 90% for estimating proteinuria (above 4 mg/m
2
h
−1
). The 95th percentile of Uprot/Uosm ratio was 0.25 mg/l/mOsm/kg and Uprot/Ucrea ratio was 0.27 mg/mg in normal group. The best estimate was Uprot/Uosm ratio of 1.42 (sensitivity 100%, specificity 94.9%) and Uprot/Ucrea ratio of 0.75 (sensitivity 100%, specificity 92.9%) for nephrotic proteinuria (40 mg/m
2
h
−1
). Uprot/Uosm which is a reliable and simple method can be used for quantification of proteinuria in pediatric patients with normal renal function. Using Uprot/Uosm ratio for quantification of proteinuria can remove the necessity of 24-h urine collection, urine creatinine measurement and spending additional cost and time if the center measures the urine osmolality routinely in urine analysis.
An infant with Imersland-Gräsbeck syndrome Bulut, Ipek Kaplan; Mutlubas, Fatma; Mir, Sevgi ...
Saudi journal of kidney diseases and transplantation,
05/2012, Letnik:
23, Številka:
3
Journal Article
Recenzirano
Odprti dostop
The Imersland-Gräsbeck Syndrome (IGS) is a rare inherited disorder characterized by megaloblastic anemia due to a selective Vitamin B₁₂ malabsorption in association with mild proteinuria. This ...syndrome can be diagnosed and treated easily. Herein, we describe an infant with IGS as a rare etiology of growth retardation with diarrhea, vomiting and therapy-resistant proteinuria.
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