Effect of vitamin E treatment on the oxidative damage occurring in Henoch-Schönlein purpura.

AIM: To evaluate the role of reactive oxygen molecules (ROMs) in the pathogenesis of Henoch-Schönlein purpura and the effect of vitamin E on oxidative damage. ROMs have been suggested to contribute in many pathological conditions including renal diseases and vasculitis. METHODS: The activities of superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px) as antioxidant enzymes were measured, and the level of malondialdehyde (MDA) as an indicator of lipid peroxidation in 27 children with Henoch-Schönlein purpura at the onset of the disease and during the remission period. The results of this study were compared with those of 11 healthy children studied as a control group. RESULTS: With regard to all the oxidative damage parameters such as SOD, GSH-Px and MDA, significant differences were detected between the patients and the control group in both the acute and remission periods. But no such differences were detected between patients with and those without renal involvement. In 15 patients receiving vitamin E treatment, oxidative damage parameters and clinical course showed no improvement despite significant increases in plasma vitamin E levels. CONCLUSION: Oxidative damage and lipid peroxidation may play an important part in the pathogenesis of Henoch-Schönlein purpura but vitamin E given after initiation of lipid peroxidation, which is the last phase of cellular damage, is of no use in breaking down the oxidative chain reactions that have already been triggered.

Acute necrotizing tubulointerstitial nephritis due to systemic adenoviral infection.

To date, all the reported cases of acute necrotizing tubulointerstitial nephritis (TIN) secondary to systemic adenovirus infection have occurred in individuals with primary or secondary immunodeficiency, and have resulted in renal failure and death. We present the case of a 12-year-old, immunologically competent girl who developed acute necrotizing TIN with acute renal failure (ARF), hepatitis and meningoencephalitis secondary to a systemic adenoviral infection who completely recovered with supportive care.

Microfocal radiography in the diagnosis of childhood renal osteodystrophy.

BACKGROUND: Renal osteodystrophy is common in children with chronic renal failure (CRF) and X-ray is an intensive method in the diagnosis of the disease. In this study we compared microfocal radiography with conventional method for the diagnosis of renal osteodystrophy. METHODS: The X-rays of left wrists of 21 children with CRF and chronic renal insufficiency were taken by conventional and microfocal methods. RESULTS: Both methods revealed osteopenia in all patients (100%), widening, fraying and cupping of ulnar and radial metaphysis in 10 (47.6%), osteosclerosis in three (14.2%) and pseudofracture in one (4.7%) patient. Microfocal radiography demonstrated osteosclerosis in one patient, pseudofracture in four and subperiosteal resorption in five patients that were not detected by conventional method. CONCLUSION: Two methods were found to be significantly different in demonstrating the changes due to rickets and hyperparathyroidism and it is concluded that microfocal radiography may be preferred in the diagnosis of childhood renal osteodystrophy.

Henoch Schönlein purpura and amebiasis.

The pathogenesis of Henoch Schönlein purpura (HSP) is unknown but is believed to result from an immune complex reaction to various antigenic stimuli, such as infectious agents. However, its association with Entamoeba histolytica has not been reported before. We present an 11-year-old boy with HSP, confirmed by the demonstration of leukocytoclastic vasculitis from skin and diffuse endocapillary proliferative glomerulonephritis, together with immunoglobulin A and complement component C3 deposition from renal biopsies. Cysts and trophozoites of Entamoeba histolytica were detected from the stool of the patient at the same time and disappeared after the treatment with metranidasole. The temporal association of these two disorders is either coincidental or due to a causal relationship between them.

Erythrocyte superoxide dismutase activity and plasma malondialdehyde levels in children with Henoch Schönlein purpura.

Reactive oxygen molecules (ROM) have been suggested to contribute to many pathological conditions including vasculitides and renal diseases. In the present study we measured the activity of superoxide dismutase (SOD) as an antioxidant enzyme in red blood cells and the level of malondialdehyde (MDA), which is a product and an indicator of lipid peroxidation, in the plasma of 16 children (7M, 9F) with Henoch Schönlein purpura (HSP) at the onset of the disease (SOD 1 and MDA 1) and at the remission period (SOD 2 and MDA 2). The results were compared with the results of 17 healthy children studied as a control group. There was no significant difference for SOD activities between the patients in each period and the control group (p > 0.05). There was a statistically significant difference between MDA 1 and MDA 2 levels (p < 0.01), each of which were also significantly different from the MDA levels of control group (p < 0.001 and p < 0.01, respectively). The effect of ROMs on different clinical conditions of HSP was also examined and lipid peroxidation was found to be increased more in patients with renal involvement. It is concluded that oxidant stress especially lipid peroxidation plays an important role in the pathogenesis of HSP and in development of renal injury.

Fatal outcome of infantile oxalosis: case reports from three families and a review of literature.

Infantile oxalosis is a rare, autosomal recessive disorder. We present three unrelated cases of infantile oxalosis and their families, emphasizing its place as a cause of acute renal failure in infancy, and showing the clinical heterogeneity of the disease within the same family. The affected infants (two males, one female) were 2.5, 3.5, and five months old. Two families had first degree parental consanguinity; two revealed a history of nephrolithiasis; and one of these two had a member who received liver and kidney transplants because of primary hyperoxaluria type I. All the patients presented with the symptoms and findings of acute renal failure. Their hemoglobin levels were between 6.8-9.6 g/dl, urinalysis revealed (+) to ( +) proteinuria and microscopic hematuria. All had metabolic acidosis with BUN levels 67-113 mg/dl and creatinine 3.5-7.7 mg/dl. The abdominal ultrasonographies revealed normal sized hyperechogenic kidneys with the loss of corticomedullary junctions. Calcium oxalate crystals were demonstrated in retina and bone marrow of two patients, and in renal parenchyma of all the patients. The patients were treated with peritoneal dialysis. Renal functions continued to be abnormal (BUN: 47-168 mg/dl, creatinine: 2.8-11 mg/dl) after dialysis, and the outcome was fatal in all. In the presented families, because of the variation of the clinical presentation and the fatal outcome, presence of the multiple genetic loci appeared to be most likely. Further molecular studies will clarify the heterogeneity of this disorder.

Retransplantation after post-transplant lymphoproliferative disease.

We present a boy who developed post-transplant lymphoproliferative disease (PTLD) 3.5 months after a first kidney transplant. The diagnosis was made after histopathological examination of the renal graft which was removed because of Pseudomonas aeruginosa septicaemia. After 2 years on dialysis, the patient received a second renal transplant. This graft continues to function after 5 years and there has been no evidence of recurrence of PTLD. This suggests that retransplantation can be undertaken in patients who have recovered from PTLD in a previous graft.

Hepatitis-B-associated glomerulonephritis in children.

Three cases with hepatitis B virus (HBV)-related, biopsy-diagnosed glomerulopathies, one of which was membranous glomerulonephritis and the others membranoproliferative glomerulonephritis, are reported, emphasizing the clinical course. Two patients had spontaneous remission after seroconversion to anti-HBe-positivity, while the third patient was lost to follow-up. We reviewed the management of HBV-associated glomerulonephritis and concluded that immunosuppressive drugs should be avoided since spontaneous remission can be expected in these types of glomerulopathies.

Etiological and clinical patterns of urolithiasis in Turkish children.

OBJECTIVE: To evaluate the clinical and etiological characteristics of childhood urolithiasis in Turkey. METHODS: Ninety-two children with urolithiasis were studied retrospectively according to clinical patterns and etiological factors between January 1990 and January 1995. RESULTS: The age range of the patients was from 2 months to 14 years (mean age 6.9 years), and there was a male/female ratio of 1.6. The onset of the disease was earlier in boys than in girls. The most striking features were the initial admission of 14 (15.2%) children after the development of chronic renal failure and that most of them (64.3%) had infection stones. The stones were localized in the upper urinary system in 68.5% of the patients; bladder stones were rare (10.9%). The recurrence rate at presentation was 15.2% in all patients. As etiological factors, an anatomical defect was found in 30.4% of the patients, infections in 31.5%, and metabolic disorders in 26.1%; 11 (12.0%) of them were classified as idiopathic. The earliest presentation was seen with metabolic and infection stones and the highest recurrence rate (37.5%) in patients with metabolic stones. CONCLUSION: Childhood urolithiasis is a serious problem in Turkey. In order to prevent the development of end-stage renal failure and to improve the patients' quality of life, more efforts should be made with respect to early diagnosis and management of renal stones and urinary tract infections.

Henoch-Schonlein nephritis associated with subacute thyroiditis.

We present a 12-year-old boy who developed subacute thyroiditis during the course of rapidly progressive glomerulonephritis due to Henoch-Schonlein purpura (HSP) proven by clinical findings and percutaneous renal needle biopsy. The thyroid gland of the patient suddenly enlarged with mild tenderness while he was on steroid and dipyridamole therapy. Thyroid hormone levels revealed T3 0.31 ng/ml (nl: 0.52-1.75 ng ml), T4 2.53 ug/dl (nl: 4.8-12.8 ug/dl), free T3 0.80 pg/ml (nl: 2.14-5.34 pg/ml), free T4 0.2 ng/dl (nl: 0.73-1.95 ng/dl) and TSH 1.02 U/ml (nl: 0.36-3.25 U/ml). Antimicrosomal antibody was negative while antithyroglobulin antibody was slightly positive (1/80+). Hypoactivity with a spotty pattern was demonstrated by thyroid scanning. Serologically proven mumps infection was detected and may have been a triggering factor in the development of both HSP and subacute thyroiditis.

Acute rheumatic fever in children in the Ankara area in 1990-1992 and comparison with a previous study in 1980-1989.

Two hundred and twenty-eight patients with acute rheumatic fever (ARF), who were admitted to Dr Sami Ulus Children's Hospital between January 1990 and November 1992, were evaluated. Compared with the 1980s, an increase in the frequency of the disease was observed. The majority of patients (56.5%) were between 9 and 12 years old and 36.8% were admitted in winter. One hundred patients had arthritis only, 59 carditis and 40 chorea; 5 had carditis and chorea and 24 had arthritis and carditis. Nineteen percent of patients had a history of a previous attack. Seven of 84 patients with carditis had congestive heart failure and 2 had pericarditis. Cardiomegaly was present in 36 patients. The mitral valve was affected in 77 patients, tricuspid valve in 1 patient and both miral and aortic valves in 6 patients. One patient died as a result of severe congestive cardiac failure. Twenty-one patients had a recurrent attack. We observed that ARF is still a significant cause of morbidity in our country. As the disease is preventable by the eradication of streptococcus, we conclude that more effort should be made in the early detection and treatment of streptococcal infections.