Author: Leo
Keywords: HSPN | Childhood case | Purpura nephritis | Crescent | Tripterygium
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Hench-Schonleinpurpuranephritis (HSPN) is a rare chronic kidney disease. Since the prognosis of the kidney is due to the progress of the crescent of sclerosis, the treatment should be taken as soon as possible to prevent the pathological type from changing to the fibrosis process.
Hench-Schonleinpurpuranephritis (HSPN) is a rare chronic kidney disease. HSPN-induced chronic renal failure in children the incidence of cases up to 1.8%, cumulative adult chronic renal failure rate even as high as 10.41, so to understand the risk of HSPN in children and effective treatment means of great significance. Most retrospective studies have shown that some treatments are very effective for the disease, but still lack the clinical control study to confirm the effectiveness of the treatment. This article describes some of the new knowledge of children with HSPN in prevention and treatment.
First, children with HSPN risk factors and prevention
1. Risk factors for HSPN in children
Kidney involvement is the most serious long-term complication of Henoch-Schonleinpurpura (HSP), with 85% 90% occurring within 1 month of onset of HSP, and almost all children appear within 6 months of onset of HSP.
HSPN clinical signs of different performance, can be temporary hematuria and / or proteinuria, can also be manifested as rare nephritis or nephrotic syndrome and other obvious kidney damage. The average incidence of HSPN fluctuated between 20% and 60%. 1% to 2% of HSPN patients developed a chronic kidney disease (chronickidneydisease, CKD), the expected risk is: 5% to 18%, 10 years 10% to 20%, 20 years 20% to 32% In nonselective HSP cases, the expected risk of developing CKD was less than 5% after 20 years. Persistent or repeated purpura, the incidence of more than 1 month or more, severe abdominal symptoms, older children with the future development of kidney disease are related. There have also been studies confirming a decrease in serum clotting factor-factor activity as a predictor of risk factors for developing nephritis and significant proteinuria.
Only cases with isolated hematuria or proteinuria have a low incidence of long-term kidney damage (.6%), but the incidence of long-term renal damage in cases of nephritis or nephrotic syndrome is 19.5%. Disease progression to CKD Risk incidence: nephrotic syndrome is higher than 40%, acute nephritis syndrome is higher than 15%, gross hematuria and / or non-nephropathy range 5% to 15% proteinuria, microscopic hematuria ± mild proteinuria below 5%.
Compared with mild or no renal symptoms, the onset of severe renal symptoms in patients with severe long-term prognosis is poor. The risk of disease progression is associated with increased mean levels of proteinuria. Glomerular epithelial cell crescent formation of more than 50%, glomerular sclerosis or interstitial fibrosis in patients with long-term prognosis is poor.
However, the severity of the first biopsy does not correlate with the prognosis of the disease [long-term follow-up renal biopsy helps assess the final outcome of the disease. According to the presence and number of neonatal biopsy in renal tissue, the International Society of Pediatric Nephrology (ISKDC) divides HSPN histological damage into five grades (ie, I, II, Ⅲ, Ⅳ, Ⅴ).
The risk of disease progression to ckd is associated with the severity of clinical manifestations of histological damage. ISKDC grading results showed that the clinical manifestations of renal tissue grade II or higher can be presented as severe clinical complications such as acute kidney disease and / or CKD, endstagerenaldis ease (ESRD).
Class I (ie, including minor glomerular abnormalities) is the only type of type that does not have long-term complications. However, studies have shown that patients with low levels of tissue damage may also develop CKD, and high levels of histopathological changes may also be completely healed.
These manifestations of inconsistency may be related to pathologic biopsy transitions that exacerbate or underestimate the kidney damage of the patient itself, causing disparity in pathological and clinical facts, or disruptive factors such as anthropogenic deviations in clinical selection of specimens. Recent studies have suggested that the percentage of renal interstitial fibrosis and sclerosis (not crescent) is associated with poor prognosis.
In conclusion, there is no gold standard for predicting the risk factors for long-term prognosis of disease, so finding new markers for detection is of great importance. These markers require reproducibility in blood or urine specimens. High reliability, such as the cycle of different cytokines and complement system after activation of the product, urinary glomerular podocyte excreta and so on.
2. Prevention of HSPN in children
Because nephritis is the most serious complication of HSP children, it is important to use HSP to prevent the occurrence of HSPN when diagnosing HSP. HSP acute stage of corticosteroid therapy can effectively reduce the symptoms and duration of abdominal and joint pain, the current multi-recommended use of prednisone 1 2.5mg.kg-1.d-1, once every 14 weeks.
However, there is controversy in the use of corticosteroids to prevent HSPN in the early stages of disease. It has been reported that corticosteroids can significantly reduce the risk of nephritis, but studies have suggested that corticosteroids are ineffective in preventing nephritis.
Char-tapisak et al reported that the number of patients with persistent kidney disease following 3, 6, and 2 months follow-up of short-term prednisone treatment after onset of HSP was significantly higher than that of the control group and those without special treatment Significance, clinical use of glucocorticoids is not recommended to prevent the occurrence of HSPN, the doctor can occur in the presence of renal symptoms of prednisolone.
HSP pathological changes in the acute phase of a wide range of capillaries to the main small vasculitis. Vascular endothelial damage at the same time after the initiation of internal and external coagulation system, so that the body is in a state of hypercoagulable. Heparin by blocking the internal and external source of coagulation, reduce and prevent platelet aggregation, reduce blood viscosity, to achieve anticoagulant effect.
Clinical use of heparin can reduce the number of kidney cases in children with HSP, but there are bleeding, thrombocytopenia and osteoporosis risk, so the clinical use of such potentially dangerous therapy is still controversial.
In addition, aspirin is a more effective antiplatelet preparation for the treatment of vasculitis, but current prospective results for children with HSP show that antiplatelet preparations, including aspirin, are not effective in preventing HSPN.
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