Nephrotic syndrome associated with varicella infection

A 4-year-old boy developed nephrotic syndrome following varicella infection. Serologic studies during the early phase of the disease demonstrated a decrease in serum C3, C4, and properdin factor B. Renal biopsy revealed an acute proliferative glomerulonephritis with deposition of immunoglobulins A (IgA) and M, C3, C1q, and varicella virus antigen in the glomerulus, suggesting an immune complex deposition. Ultrastructurally, this suggested a postinfectious immune complex glomerulonephritis. These phenomena suggested that varicella virus antigen antibody complexes were deposited in the glomerulus and activated the classic and alternative pathway of complements, leading to an immune complex glomerulonephritis. During the nephrotic phase, an increase in OKT8 cells and decrease of the OKT4 cells were demonstrated. Two months later, this alteration returned to normal as the renal disease was in remission. This change of lymphocyte subsets during varicella infection may play a role in the pathogenesis of nephrotic syndrome.     

Hepatitis B surface antigen associated nephrotic syndrome

Hepatitis B virus (HBV) has been reported in association with the nephrotic syndrome from different parts of the world, but its role as a cause of the pathological findings of nephrotic syndrome is still controversial. We report seven nephrotic children with positive hepatitis B markers in which members of their families were also positive for the markers but without clinical, renal or hepatic involvement. Four showed haematuria at onset and three developed hypertension later in the course of the disease. Only two were responsive to steroid therapy. Renal biopsy was performed in four, of whom three showed membranous nephropathy and the other showed mesangioproliferative glomerulonephritis. Four patients developed end-stage renal disease. We conclude that in our environment HBV, when detected in children with nephrotic syndrome, should not be considered as a chance finding, but may have a definite role in its pathogenesis. Moreover, the prognosis of HBV-associated nephrotic syndrome appears poor.     

Glomerulonephritis after recombinant hepatitis B vaccine

Renal complications after vaccination with hepatitis B recombinant vaccine are rare; very few cases are reported without a histopathologic definition. We describe a case of glomerulonephritis in a 12-year-old girl, beginning after the inoculation of hepatitis B vaccine. The immunohistochemical examination of her renal biopsy, with the use of monoclonal antibodies, shows the presence of hepatitis B surface antigen in renal tissue. After 2 years chronic glomerulonephritis persisted and was treated with angiotensin-converting enzyme inhibitors. After 3 years of follow-up, the renal disease was in remission, and treatment was stopped.     

Treatment of hepatitis B virus-associated membranous glomerulonephritis with interferon alfa in a 7 year old boy

BACKGROUND: The association between Hepatitis B virus infection and membranous nephropathy has been confirmed by sources in several countries. Most commonly, the illness is seen as a nephrotic syndrome. Optimal treatment remains undefined. Antiviral therapies observed with recombinant human interferon alpha may be the best treatment option. CASE REPORT: We present a 7-year old boy with membranous glomerulonephritis and nephrotic syndrome. Twelve months after the initial hospitalization therapy was started with recombinant alpha-interferon s.c. three times weekly for six months. After the therapy the patient is stable, without proteinuria, edema or renal failure. He was seronegative for HBsAg, HBV-DNA and antibody to HBeAg. CONCLUSIONS: This case report suggests that alpha interferon is effective in the complete resolution of proteinuria in HBV membranous nephropathy.     

No evidence of hepatitis G virus in fulminant hepatic failure in children

BACKGROUND: The cause of fulminant hepatic failure in children remains unknown, but a viral origin has been suspected in most cases. The recently discovered blood-borne virus, hepatitis G, has been suggested as a possible causative agent. METHOD: Six consecutive children who underwent liver transplantation for fulminant hepatic failure were studied. The children were tested for hepatitis G virus antibodies and hepatitis G virus RNA by polymerase chain reaction after excluding other causes of fulminant hepatic failure. RESULTS: No evidence of hepatitis G virus infection was found in these patients. CONCLUSION: Hepatitis G virus is unlikely to be a common cause of fulminant hepatic failure in pediatric patients from the upper midwestern United States.     

Irreversible renal failure following minimal change nephrosis

A boy developed a nephrotic syndrome at the age of two years, and remained steroid sensitive for twelve years. In this time more than twenty relapses occurred. Two renal biopsies showed minimal changes. Renal function was normal all along. At an age of 14 years he developed an irreversible renal failure. Since this time he remained dependent to dialysis. A third renal biopsy now shows a perimembranous glomerulonephritis with a diffuse interstitial fibrosis.     

Terminal renal failure caused by interstitial nephritis following mushroom poisoning by Cortinarius speciocissimus

A 14 year old boy was admitted for vomiting, anorexia, flank pain and leukocyturia/hematuria. Shortly after admission, he developed anuria and acute renal failure so that hemodialysis had to be started. Pre- and post-renal causes were excluded. There were no signs of acute glomerulonephritis; liver enzymes were normal. The 123Iodine-Hippuran scan showed a shock kidney pattern lacking tubular clearance. Renal biopsy revealed an interstitial nephritis with edema and a mixed cellular infiltration. History was empty for nephrotoxic agents except for mushroom ingestion: Five days before admission the boy ate Cortinarius speciocissimus mushrooms, the toxine of which is known to be nephrotoxic, causing irreversible renal failure in severe cases (Orellanus Syndrome). Renal function did not improve much and renal transplantation was performed after 14 months on hemodialysis. In interstitial nephritis of unknown etiology the possibility of mushroom poisoning should be considered.     

Aetiology of icteric hepatitis and fulminant hepatic failure in children and the possible predisposition to hepatic failure by sickle cell disease

The aetiological agents of acute icteric hepatitis and fulminant hepatic failure were investigated in 47 children less than 12 years of age presenting at our hospital during the period January to December 1987. Hepatitis A virus was the aetiological agent in 72%, hepatitis B virus in 11%, cytomegalovirus in 2% and non A non B in 15%. These results confirm the endemicity of these viruses in the Saudi population and that they can be contracted in early life and could lead to severe hepatitis. Three of the patients who had hepatitis A virus infection developed fulminant hepatic failure and two of them died. Two of the patients who developed fulminant hepatic failure were also suffering from sickle cell disease. This raises the question of a possible predisposition to hepatic failure in sickle cell disease upon infection with hepatitis A virus.     

乙型肝炎病毒相关性肾炎患儿的肝脏病变

目的：乙型肝炎病毒相关性肾炎(HBVGN)是我国常见的继发性肾小球疾病之一,以往人们只关心HBVGN时肾脏病变的问题,对无明显肝脏临床症状的“病毒携带者”或轻微的临床患者的肝脏的情况没有引起注意。该文探讨HBVGN时肝脏的病理变化。方法：选择13例HBVGN患儿同时进行肝肾病理活检。结果：13例患儿均有肝组织不同程度的受累,主要以轻度肝脏病理改变为主(9/13,占69.2%),HBV抗原在肝组织中主要以HBsAg检出率为高(10/13例,占76.9%);肾病理改变以膜性肾病为主(9/13,占69.2%),其次为系膜增生性肾炎(4/13例,占30.8%),HBV抗原在肾组织HBsAg和HBcAg的检出率均达100%,几乎所有的肾小管间质中有HBV抗原成分的分布。肝组织与肾组织中同时出现HBcAg的有4例。结论：HBVGN患儿均有不同程度的肝损害,而肾及肝组织病理改变不一定呈平行关系,肝组织与肾组织中同时出现HBcAg时肝肾同时损害均较重。提示对于HBVGN患者要重视肝肾同治。     

Lupus nephritis. Collaborative study by the French Society of Paediatric Nephrology.

Sixty two children were included in a collaborative study to determine the prognosis for lupus nephritis. Renal involvement was confirmed by histologic study of renal biopsy specimens which were classified into five categories: minimal lesions (11 cases, 18%); focal segmental glomerulonephritis (15, 24%); diffuse proliferative glomerulonephritis (30, 48%); membranous nephropathy (5, 8%); and glomerular sclerosis (1,2%). That the predictive value of the early biopsy is limited was indicated by the most recent status of 37 patients five years after onset--total remission (13, 35%); urinary abnormalities or nephrotic syndrome (7, 19%); moderate renal failure (4, 11%); chronic renal failure (7, 19%); and hypertension (6, 16%). Treatment did not always prevent the development of severe renal failure; in particular, plasmapheresis failed to avert the death of one patient and the development of chronic renal failure in two others.     

Autoimmune complications associated with hepatitis A virus infection in children.

Hepatitis A virus infection is usually a self-limited disease during childhood. Autoimmune manifestations are rarely reported among patients. We describe two children with acute hepatitis A infection who developed immune thrombocytopenia and hepatic venous thrombosis during the course of acute infection. Antiphospholipid antibodies were increased in both of them during the thrombocytopenic and thrombotic complications and decreased during the resolution of these events.     

Measles and acute glomerulonephritis

A 5-year-old boy had transient glomerulonephritis following measles infection. Serologic studies at the early onset of the disease showed decrease of serum C3, C4, and Clq levels with normal serum properdin factor B. Renal glomerular histology revealed a mild acute proliferative glomerulonephritis. Measles virus antigen, C3, C4, and immunoglobulin G (IgG) were demonstrated in the glomerulus, suggesting an immune complex deposition. Ultrastructurally, subepithelial, intramembranous, and subendothelial electron-dense deposits were noted, suggesting a postinfectious immune complex glomerulonephritis. It is suggested that the measles virus antibody complexes are deposited in the glomerulus and activate the classic pathway of complement, leading to a self-limited glomerulonephritis.     

Anti-glomerular basement membrane antibody-mediated glomerulonephritis due to glue sniffing

A 16-year-old girl developed rapidly progressive glomerulonephritis and renal failure. The disease was associated with high titres of antiglomerular basement membrane antibodies in serum, linear deposits of immunoglobulin G and diffuse epithelial crescents on renal biopsy. Past history revealed heavy smoking and deliberate sniffing of Pattex glue, a mixture of hydrocarbons which possibly may affect the structure of glomerular basement membrane. After treatment by repeated plasmapheresis and drug immunosuppression autoantibodies disappeared from serum but renal function was not influenced. Renal damage is a potential hazard for glue sniffing adolescents.Abbreviation GBM glomerular basement membrane     

检测尿HBV抗原诊断乙型肝炎相关性肾炎的价值

探讨检测尿乙型肝炎病毒（ＨＢＶ）抗原诊断乙型肝炎相关性肾炎的价值。肾小球病伴尿ＨＢＶ抗原阳性者可临床诊断乙肝肾炎。     

Hepatitis B virus-associated nephropathy: 17 year progression from onset to end-stage renal failure

Hepatitis B virus-associated nephropathy (HB nephropathy) was first described in 1971. There have been few reports on the long-term prognosis in children with HB nephropathy. A case is reported here of a child who presented with symptoms of acute glomerulonephritis at 12 years of age and progressed to end-stage renal failure 17 years after the clinical onset, in spite of the seroconversion of HB virus by formation of HBe antibody.     

Aetiology of lcteric Hepatitis and Fulminant Hepatic Failure in Children and the Possible Predisposition to Hepatic Failure by Sickle Cell Disease

ABSTRACT. The aetiological agents of acute icteric hepatitis and fulminant hepatic failure were investigated in 47 children less than 12 years of age presenting at our hospital during the period January to December 1987. Hepatitis A virus was the aetiological agent in 72 %, hepatitis B virus in 11 %, cytomegalovirus in 2 % and non A non B in 15 %. These results confirm the endemicity of these viruses in the Saudi population and that they can be contracted in early life and could lead to severe hepatitis. Three of the patients who had hepatitis A virus infection developed fulminant hepatic failure and two of them died. Two of the patients who developed fulminant hepatic failure were also suffering from sickle cell disease. This raises the question of a possible predisposition to hepatic failure in sickle cell disease upon infection with hepatitis A virus.     

Acute and crescentic glomerulonephritis

Acute nephritic syndrome is clinically characterized by hematuria, proteinuria, oliguria, and volume overload with or without azotemia and histologically be acute proliferative glomerulonephritis. Acute post streptococcal glomerulonephritis is the commonest cause in children. There is a preceding infection prior to this condition in majority. This is one of the comonest causes of renal edema in children. Early recognition, prompt and aggressive therapy and adequate follow-up are mandatory. Prognosis is usually good unless associated with severe renal failure and crescentic glomerulonephritis where the outcome is relatively poor unless treatment is early and adequate. Pathologically acute proliferative nephritis is with diffuse proliferative glomerulonephritis with or without crescents. Immunosuppressive therapy is not needed in simple acute proliferative glomerulonephritis but is essential in modifying the outcome of crescentic glomerulonephritis. Delayed resolution, severe renal failure at onset, progressive renal failure and associated systemic features like skin rashes, joint pains, hepatosplenomegaly and persistent fever are the indications for biopsy. Overall the prognosis in classical post streptococcal acute proliferative glomerulonephritis is good.     

Acute and crescentic glomerulonephritis

Acute nephritic syndrome is clinically characterized by hematuria, proteinuria, oliguria, and volume overload with or without azotemia and histologically be acute proliferative glomerulonephritis. Acute post streptococcal glomerulonephritis is the commonest cause in children. There is a preceding infection prior to this condition in majority. This is one of the comonest causes of renal edema in children. Early recognition, prompt and aggressive therapy and adequate follow-up are mandatory. Prognosis is usually good unless associated with severe renal failure and crescentic glomerulonephritis where the outcome is relatively poor unless treatment is early and adequate. Pathologically acute proliferative nephritis is with diffuse proliferative glomerulonephritis with or without crescents. Immunosuppressive therapy is not needed in simple acute proliferative glomerulonephritis but is essential in modifying the outcome of crescentic glomerulonephritis. Delayed resolution, severe renal failure at onset, progressive renal failure and associated systemic features like skin rashes, joint pains, hepatosplenomegaly and persistent fever are the indications for biopsy. Overall the prognosis in classical post streptococcal acute proliferative glomerulonephritis is good.     

Horseshoe kidney with mesangioproliferative glomerulonephritis and goiter

Horseshoe kidney is a relatively common renal anomaly with which many structural and developmental anomalies have been shown to be associated. However, there are only a few case reports regarding the association of membranous glomerulonephritis and focal sclerosing glomerulonephritis in patients with horseshoe kidneys. We report a girl who was evaluated for hematuria and proteinuria, and found to have horseshoe kidney. Renal biopsy demonstrated mesangioproliferative glomerulonephritis. She also had simple diffuse goiter.     

Acute interstitial nephritis in T‐cell leukemia in a pediatric patient

Acute lymphoid leukemia is the most frequently occurring malignancy in childhood, but acute tubulointerstitial nephritis with associated acute renal failure as the leading manifestation of leukemia is extremely rare. Only a few pediatric cases have been described in the literature. We present a surprising case in which physical examination and initial investigation were not typical for leukemia. Ultrasound showed only modest kidney enlargement while laboratory results indicated acute renal failure. Renal biopsy indicated tubulointerstitial nephritis, and subsequent steroid treatment led to sudden clinical improvement. One month later, however, the patient returned with typical clinical features of leukemia. Re‐evaluation of the original kidney biopsy block indicated T‐cell acute lymphoid leukemia. The present case highlights the importance of renal biopsy.