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Nephrotic syndrome in the first year of life (NSFL) is a heterogeneous group of disorders, the management of which is supportive, as most patients do not respond to immunosuppression. Prognosis is guarded, as the syndrome tends to lead to end-stage renal failure. We describe four cases, all of which went into spontaneous remission. These patients had severe nephrosis that began postnatally at ages 15 days to 7 months and had preceding symptoms of viral infections. One infant had proven pertussis and required ventilation for respiratory failure. Renal biopsies showed varying degrees of mesangial expansion and increased cellularity. Two biopsies showed mild mesangial sclerosis and the other two only scattered globally sclerosed glomeruli. Supportive treatment was started with 20% albumin infusions, diuretics, penicillin, and thyroxine. Angiotensin-converting enzyme (ACE) inhibitors were used to reduce proteinuria in all infants, and one was also treated with indomethacin. The nephrosis gradually resolved, and protein-lowering medications were successfully weaned completely 5-30 months after presentation. The patients were protein free with normal renal function at last follow-up. Investigations including viral studies and autoimmune profiles were negative. Genetic studies for NPHS1, NPHS2, WT1, and LAM-|3 were negative. We therefore describe a subgroup of NSFL with good prognosis associated with infectious prodromes. This is also the first-described case of pertussis causing nephrotic syndrome. Keywords Nephrotic syndrome * Congenital * Diffuse mesangial sclerosis * Pertussis * ACE inhibitor, Introduction The prognosis of nephrotic syndrome presenting in the first year of life (NSFL) depends on the diagnosis, but the age of presentation is a strong indicator of likelihood of [...]

Byline: H.-K. Yap (1), E. J. S. Han (2), C.-K. Heng (2), W.-K. Gong (2) Keywords: Keywords Idiopathic nephrotic syndrome; Steroid dependency; Risk factors Abstract: Minimal change disease, the most common cause of idiopathic nephrotic syndrome (INS) in children, has a high relapse rate, with approximately half of patients developing steroid dependency. This study was aimed at determining the predictive risk factors for the development of steroid dependency in children diagnosed with INS. A retrospective study of 123 children with steroid-responsive INS, followed for at least 6 months between December 1974 and December 1999, was conducted. The following parameters were studied as predictors of steroid dependency: age at onset, gender, race, microscopic hematuria at onset, atopy, concomitant upper respiratory tract infections (URTI) during relapses, and days to remission with initial steroid therapy. Of the 91 children who fulfilled the inclusion criteria, 61.5% became steroid dependent. Both univariate and logistic regression analyses revealed that initial remission time of 9 or more days (P=0.02, OR=3.0, 95% CI=1.2--7.9) and concomitant URTI during relapses (P=0.01, OR=3.4, 95% CI=1.3--8.8) were significant predictors of steroid dependency. By identifying those children with predictive factors of steroid dependency, the clinician will be better able to plan the long-term management of these patients and reduce the morbidity seen with the frequent relapses and steroid treatment, in a disease that is otherwise associated with a favorable prognosis. Author Affiliation: (1) Department of Pediatrics, National University Hospital, Lower Kent Ridge Road, Singapore 119074, Singapore. Paeyaphk@nus.edu.sg, SG (2) Department of Pediatrics, National University of Singapore, Singapore, SG Article note: Received: 4 October 2000 / Revised: 27 August 2001 / Accepted: 28 August 2001

Byline: Kiyoshi Hamahira (1), K. Iijima (1), Ryojiro Tanaka (1), Hajime Nakamura (1), Norishige Yoshikawa (2) Keywords: KeywordsCyclosporine; Arteriolopathy; Tubulointerstitial lesions; Focal glomerular lesions; Drug discontinuation Abstract: Cyclosporine (CS) is well recognized to be effective in the treatment of children with steroid-dependent and steroid-resistant nephrotic syndrome (NS), but its use can result in chronic nephrotoxicity. The histological changes that occur after CS discontinuation are unknown. Therefore, we examined the histological changes [CS-associated arteriolopathy (CAA), tubulointerstitial lesions, and focal glomerular lesions] in renal biopsy specimens after the drug had been discontinued in eight children with idiopathic nephrotic syndrome (NS). These children had been treated with long-term moderate-dose CS and had shown mild-to-moderate chronic CS nephrotoxicity. The degree of CAA improved significantly after CS discontinuation (mean CAA grade from 1.30+-0.46 to 0.25+-0.46, P=0.028). CAA disappeared in six of these children after CS discontinuation. However, the tubulointerstitial lesions and the focal glomerular lesions did not change after CS discontinuation. In conclusion, in children with idiopathic NS, CAA is improved by discontinuation of CS, but tubulointerstitial changes and focal glomerular lesions do not regress with drug discontinuation. Author Affiliation: (1) Department of Pediatrics, Kobe University School of Medicine, 5--2 Kusunoki-cho, 7 chome, Chuo-ku, Kobe, 650--0017 Japan. kidney@kobe-u.ac.jp, JP (2) Department of Pediatrics, Wakayama Medical University, Wakayama, Japan, JP Article note: Received: 16 January 2001 / Revised: 26 April 2001 / Accepted: 27 April 2001

Children with nephrotic syndrome who respond quickly to corticosteroid therapy and do not have blood in their urine have a low risk of relapse. Nephrotic syndrome is a type of kidney disease. In a study of 56 children with nephrotic syndrome, those who responded to therapy within one week and did not have blood in their urine had fewer than three relapses in the next 12 months. No other factors were so closely related to relapse., Objective. More than half of the children diagnosed with nephrotic syndrome will have relapses. These can be infrequent relapses (IRs: [is less than] 2 in 6 months or [is less than] 3 in a year) or frequent relaspses (FRs: [is greater than] 2 in 6 months or [is greater than] 3 in a year). Patients who relapse while on alternate day steroids or within 1 month of discontinuation of steroid therapy are considered steroid-dependent (SD; J Pediatr. 1982; 101:514-518). Patients with an IR course have a better long-term prognosis, and many of them have minimal-change disease without mesangial hypercellularity or sclerosis. The purpose of our study was to identify factors at initial presentation that could predict the relapse pattern in the first year after diagnosis, without taking into consideration the histopathology found on renal biopsy. Design. We analyzed the medical records of children who were seen by us before March 1997 and followed for at least 1 year. Variables selected in the study were age, sex, race, presence or absence of hematuria, and days to remission (defined as protein-free) at the initial presentation, because they could relate to the pattern of relapses (ie, IR, FR, and SD). Results. Of 70 patients, 14 were excluded because of insufficient data. There were 38 males (67.9%) and 18 females (32.1%), giving a male:female ratio of 1.8:1. Median age at presentation was 3.25 years (range: 1.5-13), and 76.9% were white, 8.9% black, 7.1% Hispanic, and 7.1% other. Of all the patients, 23 were IR (41.1%), 9 were FR (16.1%), and 24 were SD (42.9%). Median days to remission were 10 (range: 2-60), on Prednisone 60 mg/[M.sup.2] daily. Hematuria was present initially in 26 patients (46.4%), and absent in 30 (53.6%). Age, sex, race, and hematuria, as independent variables, were not predictors of relapses in the first year. However, using a stratified analysis based on the presence or absence of hematuria, we found that if the remission occurred within the first week of therapy, the patients without hematuria were more likely to be IR. The sensitivity and specificity of this finding were 67% and 89%, respectively, with a positive predictive value of 94%. Conclusion. We conclude that of all the presenting features, the rapidity of initial response to steroid therapy combined with the presence of hematuria, could predict future relapses and should be well documented. Pediatrics 2000;105:492-495; nephrotic syndrome, children, steroid therapy, hematuria., ABBREVIATIONS. NS, nephrotic syndrome; MCD, minimal-change disease; FR, frequent relapser; SD, steroid-dependent; IR, infrequent relapser; FSGS, focal segmental glomerulosclerosis. Idiopathic, noncongenital nephrotic syndrome (NS) of childhood is characterized by massive [...]

Dear Editor, We read with great interest the articles by Azib et al. [1] and Zagury et al. [2] that recently appeared in Pediatric Nephrology on the long-term prognosis of [...]