Children with IgA nephropathy renal damage which several types?

IgA nephropathy (1gAGN) is a group associated with system disease, renal biopsy immune pathology in glomerular mesangial granular deposition of IgA-based disease.IgAGN for the pathological diagnosis of an immune name, clinical glomerulonephritis with hematuria as the main manifestation, but also led to one of the main end-stage renal failure. That the Department of an independent clinical a pathological syndrome. Renal damage following types:
First, episodes of gross hematuria
Often in a variety of respiratory tract infections in 1-3d after gross hematuria, it is also known as pharyngitis synchronization hematuria, this common sense of acute chain glomerulonephritis. Deformability of urinary red cell morphology, suggestive of glomerular hematuria. I have seen homes in addition to gross hematuria, and in some cases accompanied by the waist and (or) severe abdominal pain, often misdiagnosed as urinary calculi, acute abdomen (appendicitis). Such abdominal pain mechanism may be associated with inflammatory lesions of small arteries in the ureter or gastrointestinal mucosa manifestations of systemic small vessel vasculitis lesions. In addition, the electron microscope shows submucosal small artery injury was fibrinoid necrosis, IgA, and C3, and fibrin deposition to support IgAGN gastrointestinal tract has also been compromised. Low back pain associated with urinary retention or temporary oliguria may be of small blood clots occur in the urinary tract caused by a temporary obstruction.
Second, the nephrotic syndrome
Individually or in some cases associated with hypertension, hematuria. If the histopathological glomerular sclerosis and renal vascular sclerosis poor prognosis.Shanghai Children's Hospital have reported 20 cases IgAGN, which manifested as nephrotic-type, 9 cases (47%), showing that this type of a certain proportion of children IgAGN.
Third, the nephritic syndrome
This type of performance with / without mild edema of asymptomatic microscopic hematuria, often misdiagnosed as the chain sense glomerulonephritis more common in urine screening examination and confirmed by biopsy.
Fourth, the simple proteinuria
Proteinuria as the first symptom in the pediatric majority showed mild or moderate proteinuria without edema and other symptoms of kidney damage.
Fifth, rapidly progressive glomerulonephritis
Rare. Continuous gross hematuria, proteinuria associated with heart, brain involvement, the abrupt deterioration of short-term renal function, renal biopsy widely (50% -100%) glomerular crescent form. Severe hematuria hemoglobin on renal tubular toxicity and tubular obstruction with acute tubular necrosis.

IgA nephritis should be treated?

IgA nephritis should be treated?
The traditional method of treatment of IgA there is no satisfactory treatment options. Of the disease associated with renal insufficiency adrenal corticosteroids with or without immunosuppressants results are not consistent. Recent data suggest that the proteinuria of more than 1g / d, and subjected to every other day medication adrenal cortex hormone beneficial to the improvement of proteinuria. IgA deposition of minimal change nephropathy may alleviate proteinuria. Used in combination with cyclophosphamide, dipyridamole and warfarin to reduce proteinuria and glomerular filtration rate; combined use of cyclosporin A may also reduce proteinuria, and then also reduced creatinine clearance. Efficacy of phenytoin, anti-platelet drugs, the anthocyanin acid disodium diphenyl Sealand due to such drugs is uncertain. Notwithstanding the reports of urokinase may have a role to protect the glomerular filtration rate, but far from conclusive.Recurrent tonsillitis, tonsillectomy may be useful; antibiotics to prevent and treat infections that may be helpful to some acute nephritic syndrome and acute renal failure for the performers. A small series of observations found that the use of fish oil preparations reducing the role of proteinuria and increased glomerular filtration rate.Severe IgA nephropathy (glomerular filtration rate monthly decline 2 ~ 4ml/min) the use of large doses of immune globulin intravenous infusion period, to stop the glomerular filtration rate, improvement of hematuria and proteinuria, but after stopping often relapse.Cases of hypertension and severe proteinuria, glomerular filtration rate of converting enzyme inhibitors may slow down the rate of descent and reduce proteinuria and severe IgA nephropathy, converting enzyme inhibitors are the preferred antihypertensive drugs.Conversion of normal blood pressure, whether effective inhibitors is unclear.
End-stage IgA nephropathy in a kidney transplant, the transplanted kidney happen soon mesangial IgA deposition; subclinical IgA nephropathy if the donor kidney for renal mesangial IgA deposits after implantation of non-IgA nephropathy uremia often rapidly disappearing. Renal transplantation with recurrence does not necessarily progressive renal failure in IgA nephropathy, however Shi after renal transplantation immunosuppressive therapy including cyclosporine A also did not prevent its development. Cadaveric renal transplantation, 1 year and 3-year graft survival up to 87% and 77%, however, individual IgA antibody IgA anti-HLA antigens of renal transplant recipients, 2-year graft survival of up to 100%. reason to believe that these antibodies against HLA antigens played a useful role in increasing graft survival.

Governance experience in the one case of focal proliferative sclerosing IgA nephropathy

Patients with Zhou, male, 42 years old, newly diagnosed on January 22, 2010. The main reason: proteinuria, hematuria 1 month to diagnosis. Patients 1 month before the examination found that urinary protein, occult blood, and many times at the Peking University First Hospital, Xiyuan Hospital of review were seen hematuria, proteinuria, were not system diagnosis and treatment. Wear said: Dongzhimen Hospital, kidney renal biopsies showed 40 glomerular five global sclerosis, 4 ischemic sclerosis, two segmental glomerular sclerosis, the rest of glomerular mesangial cells and matrix light todegree of diffuse hyperplasia, focal segmental severe increase mesangial addicted complex red protein deposition, tubular epithelial vacuoles and granular degeneration, spotty atrophy, multiple foci of the renal mass-like lymph nodes and mononuclear cell infiltration with fibrosis, wall thickening of small arteries, in line with: Home stove proliferative sclerosing IgA nephropathy. Check: 24-hour urine protein excretion 1.76g; protein, occult blood; of creatinine 165.3umol / L, urea nitrogen 8.38mmol / L Blood pressure: 120/80mmHg, oral Aprovel 150mg 1, the pressure's up to 5mg day control of blood pressure. Zheng Jian: fatigue, mouth bitter stick, irritability, foamy urine, lumbar pain, stool viscosity, when the stool, no fever, cough, sputum, lower extremity edema, is satisfied that the poor sleep can red tongue, yellowish greasy, slippery pulse. Diagnosis: focal proliferative sclerosing IgA nephropathy, renal insufficiency, decompensated; hypertension in three high-risk groups. TCM diagnosis: kidney slow wind syndrome is a qi deficiency and liver stagnation and heat block the kidney by blood stasis paralysis.Governing Law: Qi stagnation, heat and dampness, Huoxuetongluo. Recipe: raw Astragalus 50g, the bupleurum 15g, turmeric 15g skullcap 15g, gardenia Health 15g, France Pinellia 10g raw barley 30g, lotus leaf 15g, earthworm 20g, silkworm 20g, raw citrus aurantium 15g, fried betel nut 30g Sophora japonica 15g, Health Burnet 30g, contact Ishido 15g, Atractylodes 20g, Treats 15g, 6 a casual 30g. 7 water, a daily, morning and evening hours service. Antihypertensive drugs to control blood pressure following the service.
29: two clinics in January, fatigue, backache relief, urinary Mo reduced, still upset, irritability, mouth bitter stick, the big can. Red tongue, yellow greasy moss, pulse string.Urine: protein, erythrocyte :10-15 / HP. Stool disappeared, still proteinuria, suggesting that reduce the blood heat; renal wear visible part of the glomerular ball, segmental sclerosis and ischemic sclerosis, renal interstitial multiple spotty fibrosis, Chinese interpreted as network interest into the plot, I strengthen Poxue Xiaozheng products.Recipe: the cases of newly diagnosed party to Huaihua, Health Burnet, plus triangular 15g, Curcuma 15g, Chuan Xiong 18, Smilax glabra 50g in order to strengthen the power of of detoxification Poxue Xiaozheng.
3 attending February 12th: The patient complaint when hypochondriac pain, lumbar pain disappeared, I no obvious discomfort, red tongue, yellow greasy moss, pulse string. 24-hour urinary protein excretion: 0.47g; creatinine: 140umol / L, urea nitrogen 7.9mmol / L,.Prompts the liver qi stagnation, not General pain. Go to the top of a lotus leaf, network Ishido, Yuan Hu 15g, Toosendan 12g Liver Qi to relieve pain.
4 attending March 1: The patient complained of sore throat, irritability, mouth bitter sticky, sticky stool, dark tongue, yellow greasy moss, pulse string. Urine: protein -, 3 - to 5 red blood cells. Show the wind poison off the pharynx into the network, the liver heat.Recipe: the bupleurum 15g, skullcap 15g, Health and gardenia 15g, gentian 10g raw barley 20g, Agastache 15g, Folium 30g, forsythia 15g of Fritillaria 15g, Shegan 15g Mint 15g, Shichangpu 15g earthworm 20g, silkworm 20g, bellflower 15g, the Chuanxiong 18g, black snake 15g, contact Ishido 15g, raw licorice 6g to detoxification LiYan, found the wind Tongluo Soothing heat and dampness.
5 clinic March 15: patients with sore throat disappeared, irritability, mouth bitter sticky stool viscosity. Dark tongue, yellow greasy moss, pulse string. 24-hour urinary protein excretion: 0.41g; the creatinine 115umol / L, urea nitrogen 6.4mmol / L,. . Recipe: top to Agastache, Fritillaria the Shegan, Folium, Fritillaria, Platycodon, the addition pinellia 10g, the Coptis 9g, the Magnolia 9g, lotus leaf 15g, Perrin 15g strengthening heat dampness.
6 attending March 29: patients with stable disease, mouth bitter sticky, no other obvious discomfort, dark tongue yellowish greasy moss, pulse strings. Urine: (-); Biochemical: (-).The cases above the white cardamom 10g continue to take medication.
Thereafter follow-up year, with no discomfort. Urinalysis, blood biochemistry were normal.
IgA nephropathy is one of the world's most common primary glomerular diseases, glomerular mesangial IgA deposition of immunoglobulin, IgA with C3 granular deposition in the mesangial area diseases. IgA nephropathy can be expressed for a variety of different forms of pathology, focal proliferative sclerosing IgAN in the IgAN in a certain proportion, and pathological changes mainly as a glomerular mesangial cell proliferation, extracellular matrix hyperplasia, severe pathological damage can varying degrees of glomerulosclerosis, tubular atrophy, interstitial fibrosis, easy hematuria, proteinuria, edema, hypertension, and renal function decline, if left untreated, can eventually lead to end-stage renal failure ( of ESRD).