Children's familial recurrent hematuria syndrome

　　One, Etiology

　　The familial recurrent hematuria syndrome is generally autosomal dominant inheritance, with some being autosomal recessive inheritance.

　　Two, Pathogenesis

　　The etiology and pathogenesis of benign familial hematuria have not been elucidated. Most scholars believe that the disease is an autosomal dominant inheritance, with the genetic gene located in the COL4A3/COL4A4 region on chromosome 2. This region encodes the genes for the α3 and α4 chains of type IV collagen. Due to the obstruction of type IV collagen synthesis, the glomerular basement membrane matures incompletely and becomes thin, which may be the direct cause of the disease:

　　1、免疫荧光通常为阴性，偶见有IgM和（或）C3在系膜区沉积，但强度一般较弱，

　　1. Immunofluorescence is usually negative, and occasionally IgM and (or) C3 may be deposited in the mesangial area, but the intensity is generally weak.

　　2. Light microscopy does not show clear pathological changes with diagnostic significance. Most cases show normal glomeruli and renal tubular interstitium, with some cases showing mild proliferation of glomerular mesangial cells and matrix, and a few cases showing globular sclerosis.

　　A few children with familial recurrent hematuria syndrome may develop renal insufficiency, and there are reports that 10% of the children have hearing loss, which is high-frequency hearing impairment. Therefore, timely treatment is essential.

　　1. Hematuria:Persistent microscopic hematuria, some patients may have paroxysmal gross hematuria. The clinical characteristics of this syndrome are persistent microscopic hematuria accompanied by recurrent gross hematuria, the latter often induced by upper respiratory tract infection and may be related to strenuous exercise. Long-term persistent hematuria while renal function is almost normal, or slightly reduced, this non-progressive recurrent hematuria is not only seen in familial cases but also in sporadic cases. Some children, in addition to hematuria, may have proteinuria, hypertension, and a few children may develop renal insufficiency.

　　2. Extrarenal manifestations:Generally, TBMN patients have no extrarenal manifestations, and there are reports that about 10% of TBMN patients have hearing loss, and the hearing examination shows high-frequency hearing impairment, but it is different from Alport syndrome, the hearing loss of TBMN is usually mild and does not progress.

　　Children with familial recurrent hematuria syndrome should avoid infection and overexertion, strengthen the control of a few children with hypertension, and avoid unnecessary treatment and the use of nephrotoxic drugs.

　　Urine examination:The main symptom is hematuria, a few may have a small amount of proteinuria, blood biochemistry, renal function, and immunological examination are all normal, and it is often necessary to perform imaging examinations such as ultrasound and routine examinations to exclude other causes of hematuria.

　　Therapeutic diet for hematuria:
　　1) Celery 1500 grams. Wash the celery clean, crush it to extract the juice, heat it to boiling, take 60 milliliters each time, and take it 3 times a day.
　　Effect: Cool blood and stop bleeding.
　　2) Radish 1500 grams, honey, and salt to taste. Wash the radish clean, peel and slice it, soak it in honey for 10 minutes, then dry it on a pot lid, and then soak and dry it again (BF) (do not burn) (BFQ) for 3 times in a row. Chew a few slices continuously each day, and take it with salt water, 4 to 5 times a day.
　　Effect: Clear heat and dissipate blood stasis.
　　3) A moderate amount of peanuts. Roast the peanuts, take half a cup of the red skin outside, grind into fine powder, and take with boiling water. Effect: Clear heat and stop bleeding.
　　4) 2 grams of black locust seed powder, 2 grams of rhubarb powder, 1 egg. Make a hole in the egg, put the black locust seed powder and rhubarb powder inside, mix well, seal the hole with white flour, steam and eat. Take 2 eggs each time, once a day, for 4 days, stop for 2 days, and drink plenty of water after taking it.
　　Effect: Cool blood and stop bleeding.
　　5) 3 grams of rhubarb, 1 egg. Make a hole in the egg, put the ground rhubarb powder inside, seal the hole with wet paper, steam and eat. Take once a day.
　　Effect: Clear heat and stop bleeding.
　　6) 60 grams of June Snow root, 15 grams of reed heart grass, 2 eggs. Boil together until the eggs are cooked, remove the dregs and eggshell, and eat the eggs and drink the soup on an empty stomach in the morning and evening.
　　Effect: Clear heat and cool blood, suitable for children's hematuria.
　　7). Take 100-200 grams of Chinese toadflax flower, cook and drink, take twice a day, which is effective for general hematuria and chyle hematuria.
　　8). Use 50 grams of lotus seeds and half a cup of lotus juice. After the lotus seeds are cooked, take them with lotus juice. Take twice a day, which is effective for hematuria.
　　9). Take a moderate amount of fresh celery, mash and squeeze juice, take 1 small bowl each time, and take it on an empty stomach, for 5-7 days, which can treat hematuria.
　　10). Use 3-5 grams of the outer skin of roasted peanuts, grind into fine powder, and take with warm water or use cowpea roasted and ground into powder, take 3 grams each time, twice a day, with boiling water or yellow wine, and take for several days, which is effective for treating hematuria.
　　11) Take 200 grams of green bean sprouts, mix with sugar and eat, which can treat hematuria.
　　12). Use 200 grams of bitter melon head, decocted in water; 1 pinch of clean soybean curd, decocted in water; 100 grams of grape root and sugar, boiled in boiling water; 15 grams of black plum, decocted in water. All of these remedies have the effect of treating hematuria, and can be selected according to circumstances.

　　1. Treatment:There is no effective treatment for hematuria in this symptom, and long-term follow-up observation is required.

　　2. Prognosis:The renal function of this symptom is not significantly impaired, so the prognosis is good.