Background: Idiopathic nephrotic syndrome is not uncommon in children. The disease affects children's physical development. Idiopathic nephrotic syndrome cause many complication in which effecting to physical development has been influenced much to their life, study and living activities of children. Objectives:This study aims to learn about the physical development and to find some factors impacting on physical development ofchildren with idiopathic nephrotic syndrome. Subjects and method: A prospective and descriptive study was conducted on 100 patients diagnosed with idiopathic nephrotic syndrome disease who have been followed up for from 1 year to 6 years in that duration since September 2006 to March 2007. Results:Height standard deviation score (SDS) was less than -2 SO in 15.0% patients. Six per cent of patients had weight SDS ofless than 250. Delayed puberty was seen in 21.6% patients. Some factors impacting on physical developmentwere duration of the disease, relapses, nephrtitis-nephrotic syndrome, prolonged corticoid treatment. Conclusion:Idiopathic nephrotic syndrome retarded physical development and puberty in children. It should be considered careful before indicate to use corticoid because using corticoid for a long period will effect to the height development of children.
Nephrotic Syndrome/ pathology ; therapy ; Idiopathic minimal change nephrotic syndrome ; Child

Background: Acute renal failure (ARF) is an uncommon but alarming complication of idiopathic nephrotic syndrome. The renal failure could be secondary to causes evident from the history and evaluation, such as severe intravascular volume depletion, acute tubular necrosis but there is no report on this population in Vietnamese adult patients. Objective: To investigate on acute renal failure complicating of nephrotic syndrome. Subjects and method: Patients with idiopathic nephrotic syndrome who were admitted with acute renal failure have been enrolled to investigate the clinical findings, laboratory check up and histology examination. Results:We present 33 patients with idiopathic nephrotic syndrome who were admitted with acute renal failure between June 1997 and June 1998. We describe the clinical and renal pathology features of these patients in whom reversible idiopathic acute renal failure developed during the course of primary nephrotic syndrome (PNS). Improvement in renal function occurred in 80% of patients over a variable period of 10 days to 14 days. The histology findings are interstitial edema, tubular obstruction. Conclusions: Acute renal failure complicating of nephritic syndrome is reversible, the exact pathophysiology of ARF is not understood. Possible causes include edema, tubular obstruction, altered glomerular permeability, and unrecognized hypovolemia.
Acute Kidney Injury/ pathology Nephrotic Syndrome ; Adult

Female ; Humans ; Infant ; Male ; Nephrotic Syndrome ; congenital ; pathology ; therapy

Child ; Humans ; Hypereosinophilic Syndrome ; drug therapy ; pathology ; Male ; Nephrotic Syndrome ; pathology

Humans ; Kidney Neoplasms ; diagnosis ; pathology ; Lymphoma ; diagnosis ; pathology ; Nephrotic Syndrome ; diagnosis ; pathology

Diffuse mesangial sclerosis (DMS) is one of the underlying pathology of congenital and infantile nephrotic syndrome. Infants with DMS develop nephrotic syndrome before 2 years of age and progress to end stage renal disease within 3 years of age. The authors experienced a case of isolated DMS in a 4-month-old male infant who had nephrotic syndrome for 1 month. The diagnosis was confirmed on the basis of clinical, laboratory, pathological and molecular genetic findings. This is the 3rd case report of DMS in our country and the 1st case report of isolated DMS confirmed by molecular genetic study.
Denys-Drash Syndrome ; Diagnosis ; Humans ; Infant ; Kidney Failure, Chronic ; Male ; Molecular Biology ; Nephrotic Syndrome* ; Pathology ; Sclerosis*

OBJECTIVETo study the clinical significance of urinary podocyte marker protein podocalyxin (PCX) in the diagnosis of primary nephrotic syndrome (PNS) in children and the evaluation of disease severity.

METHODSPCX levels in the first morning urine were measured by turbidimetric immunoassay (TIA) in 175 children, including 53 children with acute PNS [36 cases of simple nephrotic syndrome (NS) and 17 cases of nephritic NS], 56 children with PNS in the remission stage (relapsed: 42 cases) and 66 healthy children (control group). Twenty-four hour urinary protein was measured in the 53 children with acute PNS. The optimal operating points for the diagnosis of acute PNS and nephritic NS were determined using the receiver-operating characteristic curve (ROC curve).

RESULTSSignificant increasd levels of urinary PCX were found in children with acute PNS compared with those in the remission stage and the control group (P<0.01). A positive correlation was found between urinary PCX and 24 hour urinary protein in children with acute PNS (r=0.39, P<0.01). In children with acute PNS, urinary PCX levels were significantly higher in the nephritic NS group than in the simple NS group (P<0.05). In children in the remission stage, a significant increase in levels of urinary PCX was found in children who had relapsed compared with those who had not (P<0.05). The area under the ROC curve for the diagnosis of acute PNS and nephritic NS was 0.915 and 0.784 respectively. The optimal operating point for the diagnosis of acute PNS and nephritic NS was 7.97 and 10.28 ng/mL respectively, with a sensitivity and specificity of 81.1% and 93.4% respectively for acute PNS and of 94.1% and 52.8% respectively for nephritic NS.

CONCLUSIONSThe quantitative detection of urinary PCX is useful in the evaluation of podocyte dynamic changes. It is helpful in the diagnosis of acute PNS and in the differentiation of nephritic NS and simple NS.

Child ; Child, Preschool ; Female ; Humans ; Male ; Nephrotic Syndrome ; pathology ; urine ; Podocytes ; pathology ; ROC Curve ; Sialoglycoproteins ; urine

Lessons learned over 25 years from treating focal segmental glomerulosclerosis (FSGS) in a regional pediatric nephrology program will be reviewed. In a cohort of children with biopsy-proven FSGS, consisting of a large percentage of African-American patients, the clinical characteristics and long-term outcomes are different from previously published data on mostly white children with the same condition. Because Asian and African children with nephrotic syndrome have the same higher incidence of FSGS, it is possible that Chinese children with FSGS have clinical characteristics, therapeutic responses, and long-term outcomes similar to African-American children as reviewed in this article.
African Americans ; Child ; Glomerulosclerosis, Focal Segmental ; diagnosis ; pathology ; Humans ; Nephrotic Syndrome ; diagnosis ; pathology ; Prognosis

PURPOSE: Percutaneous renal biopsy in children with primary nephrotic syndrome(NS) contributed to establish the renal pathology and clinicopathological correlation. The most common minimal change lesion(MCL) was steroid sensitive and could be predicted by clinical and laboratory findings. It was uniformly agreed that most nephrotic children who were predicted as MCL, should receive an 8 week course of prednisolone before considering renal biopsy. Early indications of renal biopsy has been those nephrotic children with the age below 1 year and above 8 years, hypertension, hematuria, low serum C3, renal insufficiency which are not compatible with MCL. Late indications has been frequent relapser(FR), steroid dependent(SDNS) and steroid resistant nephrotic syndrome(SRNS). The indications have been challenging recently and we tried to evaluate the commonly recommended indications. METHODS: Clinical, laboratory, pathologic findings and therapeutic responses were compared in 81 children with primary nephrotic syndrome who had renal biopses beteen 1984 and 1996. RESULTS: 1) Among 11 children with the age indication, MCLs were diagosed in 9(81.8%), mesangial proliferative glomerulonephritis(MsPGN) in 1(9.1%) and membranous nephropathy(MGN) in 1(9.1%). 2) Among 4 children with microscopic hematuria, MCLs were diagosed in 3(75.0%), MsPGN in 1(25.01%). In 13 children with hypertension, macrohematuria, azotemia and low serum C3, focal segmental glomerulosclerosis(FSGS), membrano-proliferative glomerulonephritis(MPGN) and IgA nephropathy(IgA) were frequently diagnosed instead of MCL. 3) All 5(100%) frequent relapsers were diagnosed as MCLS Among 30 children with the indication of SDNS, MCLs were diagosed in 28(93.3%), MsPGN in 2(6.7%) 4) Among 18 children with the indication of SRNS, MCLs were diagosed in 6(33.3%), MsPGN in 6(33.3%), FSGS in 6(33.3%) 5) The probability to diagnose MCL was 81.8%(9/11) in age indication, 75.0%(3/4) in microscopic hematuria, 100%(5/5) in FR and 93.3%(28/30) in SDNS. 6) The response rate to standard steroid treatment were 81.8%(9/11) in age indication 75.0%(3/4) in microscopic hematuria, 100%(5/5) in FR and 96.7%(29/30) in SDNS. CONCLUSIONS: Among the commmon indications of renal biopsy in children with primary nephrotic syndrome, age, microscopic hematuria, frequent relapser and steroid dependant should be reevauated to reduce the unnecessary renal biopsy.
Azotemia ; Biopsy* ; Child* ; Hematuria ; Humans ; Hypertension ; Immunoglobulin A ; Nephrotic Syndrome* ; Pathology ; Prednisolone ; Renal Insufficiency

Spontaneous bacterial peritonitis is one of the important complication of childhood nephrotic syndrome which occurs not infrequently but this complication in adults with nephrotic syndrome is ,however, very rare. The fact that ascites formation is more frequently seen in childhood nephrotic syndrome and that minimal change disease, the commonest pathology found in childhood is associated with impairment in both cellular and humoral immunities may be an explanation for the discrepancy. We have experienced two cases of spontaneous bacterial peritonitis complicated in adults with nephrotic syndrome. The age of patients is 23 and 68 years respectively. The serum IgG level of younger patient is markedly decreased (375 mg/dL) and that of elderly patient is lower normal limit (765 mg/dL). Peritonitis was complicated during relapse in the younger patient. Both of the patients were receiving steroid therapy before complicated by peritonitis. Both patients recovered from peritonitis by antibiotic therapy but, elderly patient died from nosocomial pneumonia.
Adult* ; Aged ; Ascites ; Humans ; Immunoglobulin G ; Nephrosis, Lipoid ; Nephrotic Syndrome* ; Pathology ; Peritonitis* ; Pneumonia ; Recurrence