Transition from endocapillary proliferative glomerulonephritis to membrane proliferative glomerulonephritis：a case report / 临床儿科杂志

ObjectiveTo investigate the mechanism, diagnosis and treatment of membrane proliferative glome-rulo-nephritis (MPGN) transitioned from endocapillary proliferative glomerulonephritis (EnPGN).Methods The clinical data and the results of pathological examination of one case of MPGN transitioned from EnPGN were retrospectively analyzed.Results The child was presented with proteinuria, microscopic hematuria, and persistent low level of complement C3. The type of renal pathology was transitioned from EnPGN to MPGN. Complete remission was achieved in this child with the treatment of oral prednisolone and tacrolimus, but the level of plasma complement C3 remained low.Conclusions The type of renal pathology in children with persistent low level of complement C3 could make a transition, and the early diagnosis, timely and effective treat-ment are important.

Clinical Patterns of Acute Poststreptococcal Glomerulonephritis: A Single Center's Experience

PURPOSE: The aim of this study is to investigate the change in incidence and clinical characteristics of acute poststreptococcal glomerulonephritis (APSGN) through a single center's experience. METHODS: We retrospectively analyzed the medical records of 53 children who were diagnosed with glomerular nephritis at National Health Insurance Corporation Ilsan Hospital between March 2000 and December 2009. Twenty-six of 53 patients falled to meet the dragnostic criteria of APSGN and were excluded. Chief complaints, physical examinations, urinalysis, urine culture, laboratory results, chest x-ray, and clinical manifestations were all retrieved from the remaining 27 patients. RESULTS: The incidence of APSGN by age was highest in children between six to seven years of age, with similar distributions in both genders, and seasonal incidence was higher from November to February when compared to other months, which was similar to the previously reported studies. However, the annual number of APSGN patients did not show a significant decrease from 2000 to 2009. The incidences of edema and hypertension were 59.3% and 48.1% respectively, suggesting lower incidences than those from previous studies. Six patients (22.2%) experienced hematuria for more than 6 months. Hypertensive encephalopathy, one of APSGN critical complications occurred in one patient but resolved with conservative treatment. CONCLUSION: Our study shows that APSGN still occur as a common renal disease but the disease severity seems to decrease with milder clinical manifestations and less complications compared to past studies.

Change of lipxin A4, leukotriene B4 and 15-1ipoxygenase in chiMren with acute poststreptococcal glomerulonephritis / 中华肾脏病杂志

ObjectiveTo investigate the change of lipoxin A4 (LXA4), leuotriene B4(LTB4) in blood and urine and leukocyte 15-lipoxygenase (15-LO) of the children with acute poststreptococcal glomendonephritis (APSGN) and to evaluate its significance. MethodsBlood and urinary levels of LXA4 and LTB4 were measured with ELISA within 3 days (acute phase), 10 to 14 days (early resolution phase) and 6 to 8 weeks (late resolution phase) respectively after onset of APSGN in 22 patients. In 8 children with APSGN, expression level of leukocyte 15-LO mRNA was examined with RT-PCR. Leukocyte LTB4 synthesis was assessed with ELISA. Chemotactic effect of LTB4, LXA4 and 15-S-hydroxyeicosatetraenoic acid (15-S-HETE) on neutrophils was determined by in vitro chemotaxis assay. Twenty-two healthy children were served as control. ResultsBlood and urinary levels of LXA4 and leukocyte 15-LO mRNA were up-regnlated in acute phase, further increased in early resolution phase, and decreased in late resolution phase of APSGN, which were stir higher than those in the controls (P＜0.01). Blood and urinary levels of LTB4 were increased in acute phase (P＜0.01) and then were decreased in early resolution phase and hte resolution phase of APSGN, which were still higher than those in the controls (P＜0.01). Administration of 15-S-HETE or LXA4 in vitro inhibited LTB4-induced chemotactic effect on neutrophils of the patients,and inhibited the production of leukocyte LTB4. ConclusionsChanges of blood and urinary levels of LXA4 and LTB4 in early resolution phase of APSGN are contrary. 15-S-HETE and LXA4may play a role in anti-inflammation and resolution of APSGN via inhibiting LTB4.

Prognosis of Proteinuria in Children with Aacute Poststreptococcal Glomerulonephritis(APSGN)

PURPOSE: The prognosis of acute poststreptococcal glomerulonephritis(APSGN) has been reported to be favorable. However, several studies have reported that patients with nephrotic range proteinuria in the acute phase or persistent proteinuria may progress to chronic renal failure. To elucidate this further, we analyzed the features of proteinuria and its prognosis in pediatric patients with APSGN. METHODS: A total of 48 children with APSGN admitted to our hospital between Jan. 2000 and Dec. 2004 were included. After discharge from the hospital, patients were regularly followed up every month by clinical evaluations and laboratory tests including routine urinalysis and quantification of proteinuria. RESULTS: Age of the patients ranged from 3 to 15 years(median 5.8 years) at the time of disease onset. Proteinuria was present in 34(70.8%) patients and 5 of them showed heavy proteinuria. Proteinuria normalized within one month in most patients(82.3%) and there was no one with proteinuria after 6 months. Cyclosporine A(5 mg/kg/day in two divided doses) was given to 3 patients with massive proteinuria that lasted longer than 2 months and the result was complete remission within 4 months. CONCLUSIONS: Our data indicated that the prognosis of APSGN during childhood is excellent. Children with severe proteinuria or subnormal renal function in poststreptococcal glomerulonephritis had favorable prognosis without chronic renal failure, and children with crescentic formation also had favorable prognosis. Three patients who continued to have heavy proteinuria for more than 2 months received cyclosporine A and remission of proteinuria was achieved within a couple of months.

A Clinical Study of Acute Poststreptococcal Glomerulonephritis in Children, from 1994 to 2003 / 소아과

PURPOSE: Acute poststreptococcal glomerulonephritis (APSGN) is a common form of glomerulonephritis in children. Most patients recover completely after the acute phase but a few patients have acute complications or progress to chronic renal disease. In recent years, the frequency of APSGN has been was decreasing but is still common in children. So we studied the clinical characteristics of APSGN from 1994 to 2003 and compared it with past studies. METHODS: We studied 105 patients who were diagnosed with APSGN in the Department of Pediatrics, Asan Medical Center between January 1994 and December 2003, with a retrospective chart review. RESULTS: The mean age was 8.5+/-2.6 years. The male to female ratio was 2: 1. Average annual incidence was 10.5+/-4.9 most patients (60.0 percent) occurred from October to January. Edema was seen in 82 cases (78.1 percent), gross hematuria in 70 cases (66.7 percent), hypertension in 50 cases (47.6 percent) and oliguria in 22 cases (20.9 percent). Microscopic hematuria was seen in 105 cases (100 percent), positive ASO in 99 cases (94.2 percent), proteinuria in 67 cases (63.8 percent) and azotemia in 38 cases (36.2 percent). Serum complement 3 (C3) level decreased in 96 cases and returned to normal within eight weeks in 70 patients (75.3 percent). Kidney biopsy was carried out in 22 cases. Most acute symptoms subsided within 2 weeks of onset. CONCLUSION: We concluded that there was no significant difference between clinical features of recent and past APSGN in children, and short term prognoses were excellent.

Prognosis of Acute Poststreptococcal Glomerulonephritis (APSGN) in Children

PURPOSE: Acute poststreptococcal glomerulonephritis(APSGN) follows infection of group A beta-hemolytic streptococci. The prognosis of APSGN has been reported as favorable. However, several studies have reported that some patients progress to chronic renal failure. In an attempt to clarify this, we analyzed the clinical course of patients with APSGN. METHODS: Between January 2000 and December 2004, a total of 48 children who were diagnosed with APSGN according to the presence of hematuria, transient hypocomplementemia and evidence of group A beta-hemolytic streptococcal infection were evaluated. RESULTS: Six(12.5%) patients showed elevation of serum creatinine level but there was no patient with persistent renal dysfunction. Blood pressure was controlled with ease in all patients and there was no case of persistent hypertension. Renal biopsy was done in 5 patients who showed heavy proteinuria or renal insufficiency and the outcomes showed findings consistent with ordinary APSGN except one with findings of rapidly progressive glomerulonephritis(RPGN). Serum complement levels normalized within 8 weeks(92.9%). Hematuria disappeared within 6 months(79%) and proteinuria within 6 months(100%) from the disease onset. CONCLUSION: Prolonged renal dysfunction or heavy proteinuria found in five patients(10.4%) led to renal biopsy. All these problems resolved within 6 months. Our data support that the prognosis of childhood APSGN is favorable without any serious sequela.

The Study of Clinical Characteristic Changes in Acute Poststreptococcal Glomerulonephtritis: Comparision of Clinical Manifestations between Recent 5 Years and Past 5 Years / 소아과

PURPOSE: The purpose of this study is to evaluate the clinical characteristic changes of acute poststeptococcal glomerulonephritis (APSGN) in recent. The cases of APSGN occurred for the last ten years were divided into group I and II by the unit of 5 years and clinical manifestations and laboratory findings of each groups were analyzed. METHODS: Total of 74 cases of APSGN, diagnosed at the department of Pediatrics, Chungnam National University Hospital from January 1995 to August 2004 were divided into group I (31 cases from January 1995 to December 1999) & II (43 cases from January 2000 to August 2004). In addition, the clinical records of each group were reviewed retrospectively and compared. RESULTS: In both groups, the prevalence rate was much higher in males than females, the seasonal prevalence rate was higher in winter, the upper respiratory infection was most frequent predisposing illness, and one to two weeks of latent period was the highest. However, the average age for group II were younger than group I. The prevalence rate of gross hematuria was higher in group II, but there was no significant difference between two groups. In the course of clinical symptoms, edema and hypertension were relieved within two weeks in both groups. In group II, the prevalence rate of proteinuria was higher, and the duration of proteinuria and gross hematuria were longer than group I. CONCLUSION: In this comparative study, the average age became younger. Clinically the duration of gross hematuria and proteinuria became longer, and the prevalence rate of proteinuria increased in recent 5 years. However, there was no difference between two groups in clinical courses and prognosis.

A Case of Acute Poststreptococcal Glomerulonephritis Accompanied with Acute Pyelonephritis

Acute poststreptococcal glomerulonephritis(APSGN) is the most common form of postinfectious glomerulonephritis, and acute pyelonephritis(APN) is the most severe form of urinary tract infection in childhood. However, the concurrence of two diseases is uncommon in the literature. We describe a case of APSGN accompanied with APN in a 5-year-old female who presented with fever, left flank pain, headache and facial edema. Urinalysis showed pyuria, microscopic hematuria, and mild proteinuira. Serial urine cultures grew Escherichia coli. (99m)Tc-DMSA renal scan revealed a cortical defect in the upper pole of left kidney. She had a history of preceding pharyngitis, in addition, showed high blood pressure, high anti-streptolysin O titer, and low serum complement levels. The patient improved completely with supportive treatment, including antibiotic and antihypertensive therapy. These findings suggested that APSGN and APN could be manifested simultaneously or be superimposed on each other.

A Case of Acute Poststreptococcal Glomerulonephritis Associated with Chronic Granulomatous Disease

Chronic granulomatous disease(CGD) is a rare inherited disorder of phagocytic cells which results in a susceptibility to infections by catalase-positive bacteria and fungi, as well as granuloma formation. And acute poststreptococcal glomerulonephritis(APSGN) is one of the most common glomerular lesions of gross hematuria in children. We experienced a case of APSGN accompanied with CGD presenting with a liver granuloma.

A Case of Acute Poststreptococcal Glomerulonephritis Associated with Chronic Granulomatous Disease

Chronic granulomatous disease(CGD) is a rare inherited disorder of phagocytic cells which results in a susceptibility to infections by catalase-positive bacteria and fungi, as well as granuloma formation. And acute poststreptococcal glomerulonephritis(APSGN) is one of the most common glomerular lesions of gross hematuria in children. We experienced a case of APSGN accompanied with CGD presenting with a liver granuloma.

Alport Syndrome Associated with Poststreptococcal Glomerulonephritis in Brothers

Alport syndrome is the most common type of hereditary nephritis, and acute poststreptococcal glomerulonephritis(APSGN) is a common disease in children. We experienced the clinical and pathologic findings of Alport syndrome and APSGN in brothers of one family. Both patients presented with heavy gross hematuria and proteinuria. ASO titer was elevated in both cases, and the C3 level was reduced in one of the cases. In renal pathology, both showed characteristics of Alport syndrome as well as the glomerular changes of APSGN with hump-like subepithelial deposits by electron microscopy. These clinical observation indicated that the patients had APSGN superimposed on Alport syndrome, and that the episode of APSGN might exacerbate the clinical course of Alport syndrome.

A Case of Membranoproliferative Glomerulonephritis Type II: Dense Deposit Disease, DDD

Type II membranoproliferative glomerulonephritis (Dense deposit disease ) is an acquired primary glomerular disease characterized by electron microscopic evidence of a continuous dense membrane deposition replacing the lamina densa. It is a subtype of idiopathic membra- noproliferative glomrulonephritis, and was described as a separate entity by Berger and Galle in 1963. It frequently occurs in older chilren and young adults and the clinical course is variable, but is generally progressive. The presenting feature is nephrotic syndrome in many patients, and proteinuria and hematuria are also seen frequently. The purpose of this paper is to present a case of DDD (Dense deposit disease) from a 10 year old boy who was diagnosed as a acute poststreptococcal glomurulonephritis with protenuria, hematuria, and facial edema by renal biopsy 4 years ago.

Two Cases of Acute Poststreptococcal Glomerulonephritis Superimposing to IgA Nephropathy

The pathogenesis of IgA nephropathy and acute poststreptococcal glomerulonephritis is not fully understood. In the past, acute poststreptococcal glomerulonephritis was the most common cause of gross hematuria in children, but now IgA nephropathy is the most common one. We experienced two cases of acute poststreptococcal glomerulonephritis superimposing to IgA nephropathy in boys. Case 1 had upper respiratory infection before elevation of anti-streptolysin O, generalized edema, gross hematuria and proteinuria. The complement levels were normal. Electron microscopic findings of renal biopsy at ten days after onset showed a few big subepithelial 'humps' and localized heavy subendothelial and mesangial deposits. Immunofluoroscopic findings revealed predominant IgA deposition in the mesangium. The electron microscopic findings were diagnostic of acute poststreptococcal glomerulonephritis. On the other hand, immunoflorescence microscopic findings were compatible to IgA nephropathy. In case 2, the renal biopsy which was done 2 years after onset showed only finding of IgA nephropathy. To our knowledges, there has been few reports of acute poststreptococcal glomerulonephritis superimposing to IgA nephropathy which was confirmed by renal biopsy. We report two cases of acute poststreptococcal glomerulonephritis superimposing to IgA nephropathy with a brief review of the literatures.

Diagnostic Value of Serum Immunoglobulins in Glomerulonephritis

Serum immunoglobulin concenatrations were measured in 156 patients with various types of glomerulonephritis such as acute poststreptococcal glomerulonephritis, minimal lesion nephrotic syndrome and Henoch-Sch?lein purpura nephritis. 1) Serum IgG level was above normal mean in acute poststreptococcal glomerulonephritis, and normal range in Henoch-Scholein purpura nephritis. Serum IgG concentrations were significantly reduced in minimal lesion nephrotic syndrome. 2) Serum IgA concentrations were normal limit in minimal lesion nephrotic syndrome. In one-forth of acute poststreptococcal glomerulonephritis and one-half of Henoch-Sch?lein purpura nephritis, serum IgA concentrations were elevated. 3) Serum IgM concentrations were normal in acute poststreptococcal glomerulonephritis, minimal lesion nephrotic syndrome and Henoch-Sch?lein purpura nephritis. We concluded that serum immunoglobulin concentrations were not useful as an aid in diagnosing glomerulonephritis.