Children with nephrotic syndrome

Children with nephrotic syndrome

Clinical manifestations

First, simple primary nephrotic syndrome.

Mainly in children and adolescents. The ratio of male to female is about 2: 1.

1, the body can be concave edema, to face, lower limbs, scrotum as obvious, often ascites, the general condition is still good, no hypertension.

2, oliguria, urinary protein mostly +++ ~ ++++, quantitative> 0.1g / (kg · d). Urine microscopic examination with a small amount of red blood cells.

3, plasma total protein below normal, albumin decreased more significantly (<30g / L), serum protein electrophoresis showed a decrease in the proportion of albumin, globulin ratio increased, r globulin decreased. Blood cholesterol was significantly increased (> 5.7mmol / L), serum complement normal.

4, normal renal function, edema period was oliguria, may have transient mild azotemia.

Second, nephritis nephrotic syndrome.

The age of onset in school-age, clinical features are as follows:

1, age of onset more common in children over 7 years of age, edema generally not serious.

2, blood pressure may have increased to varying degrees, often paroxysmal or persistent high blood pressure and hematuria.

3, serum complement can be reduced, may have varying degrees of azotemia.

Third, congenital nephrotic syndrome is rare, mostly recessive genetic disease.

More common in the neonatal period and within three months after birth, morbidity, performance and simple nephropathy similar.

treatment

Primary renal disease in children with spleen and kidney, Western medicine to control the principle of side effects, the use of Chinese and Western medicine to adrenal cortex hormone-based comprehensive treatment. Including maintaining water and electrolyte balance supply, control of edema, proper nutrition prevention, control of concomitant infection, hormone resistance with the application of immunosuppressive drugs, the proper use of adrenal cortex hormones recurrent.

Symptomatic treatment

General application of hormones after seven to four days, most children began diuretic swelling can not diuretics; but a high degree of edema associated with skin infections are not sensitive to hypertensive hormone test diuretics.

General treatment

High blood pressure and low salt edema severe avoid high salt water edema and / or oliguria in children should be appropriate to limit the amount of water, but a lot of diuretic or diarrhea vomiting loss of salt to be appropriate when water and salt. In addition to a high degree of edema complicated by infection in general do not need absolute bed rest after remission of the gradual increase in activity after three to six months to gradually resume work but not tired.

treatment

Indications: refractory nephropathy and / or severe side effects of hormones can be added or change with immunosuppressive agents;

① cyclosporine toxin A: The drug can specifically inhibit helper T cells and cytotoxic T cell activation and proliferation does not affect the B-cell and granulocyte dose daily 6 ~ 8mg / kg often need to monitor the blood concentration to adjust the dose course of eight weeks For the treatment of nephrotic syndrome can be summarized as hormone effects of the agent is also effective for this type of patients when the hormone toxic side effects can be replaced with the drug but withdrawal may still be re-use is still effective; hormone-resistant, such as Early application to alleviate its toxic side effects, the most obvious nephrotoxicity Acute renal toxicity showed prerenal azotemia, usually reversible changes associated with dose-related chronic nephrotoxicity, the role of renal tissue structure changes in performance For interstitial and tubular lesions, hypertension, hyperuricemia, sodium retention, high serum creatinine clearance decreased renal toxicity can also be caused by hairy gingival hyperplasia and hypomagnesemia.

② chlorambucil: to reduce the recurrence of hormone-sensitive people commonly used daily 0.2mg / kg course of 6 to 8 weeks total dose of not more than 10mg / kg side effects and cyclophosphamide similar to the gonads have some damage also reported Leukemia and solid tumors were.

③ chloramphenicol hydrochloride: every other day fast intravenous or slow intravenous injection: ten to twenty times as a course of small doses began after the first 1 ~ 2mg can be increased until 0.1mg / kg side effects of gastrointestinal symptoms can be Before the first sedative to prevent the addition of local can cause phlebitis should therefore use a larger thick vein.

④ cyclophosphamide: can reduce the recurrence of prolonged remission; hormones are sensitive to add after the induction can be completely relieved; hormone resistance after application can sometimes improve the effect of hormones in children with a dose of 2 ~ 2.5mg / (kg D) The course of treatment to three months The total dose of 200 ~ 250mg / kg 1 year of the following: the gastrointestinal tract may have liver damage, alopecia, bone marrow suppression of hemorrhagic cystitis and bacterial susceptibility to long-term gonadal Affect the attention of puberty or pre-adolescent boys after the application of this drug can affect testicular spermatogenesis caused by infertility gonadal injury and drug dose-related application should have indications and dosage.

⑤ Tripterygium glycosides: for Wei Mao plants Tripterygium wilfordii root extract of immunosuppressive effects Dose daily 1mg / kg Maximum daily 30mg 3 times a course of treatment for 3 months side effects of leukopenia, gastrointestinal reactions, skin pigmentation May affect gonadal function (menstrual disorders menstrual disorders menstrual sperm motility or reduced number)

Adrenocorticotropic hormone therapy

Celebrex hormone Although there are some side effects, and has not yet resolved the recurrence problem, but clinical practice has proved that hormones can still induce the disappearance of the effective protein drugs, and as the treatment of choice for nephropathy, the mechanism of action has not yet clear:

① diuretic effect.

② improve the glomerular filtration membrane of the overall reduction in urinary protein filtration.

③ immunosuppressive effect.

Medication principles:

① drug selection to biological half-life of 12 to 36 hours of the effect of agents is appropriate, such as the pineapple can be induced faster remission is also applicable to the next day when the therapy.

② the beginning of treatment should be enough time to take sub-fast heart-induced urinary protein negative conversion;

③ urinary protein after the maintenance of the negative phase to the next day Dayton Dayton clothing is appropriate due to adrenal secretion of cortisol was morning high and low day and night fluctuations in the law every morning Dayton method of hypothalamic - pituitary - adrenal axis (HIP) inhibition Min.

④ maintenance treatment should not be too short to be stable condition and then discontinued to reduce recurrence and urinary protein is also easy to make repeated when relieved.

Chinese treatment

Chinese medicine treatment in addition to dialectical treatment of edema, and treatment of hormone immunosuppressants cause side effects, edema, oliguria available Plantain, money grass, flat storage, corn, with blood stasis symptoms plus Chuanxiong, Angelica, Motherwort , Radix Salviae Miltiorrhiza, Zelan Ye, on the lack of kidney deficiency is not solid, give Jianpi Gushen such as Poria, Atractylodes, Astragalus, Codonopsis, Psoralea, yam, Lingpi, silk, medlar, hormone induction process In the red veins of the red veins of the tongue if excited, give Ziyinjiangyan basic prescription for the non-Senate, Anemarrhena, habitat, Cork, gentian, Dan, Alisma, licorice, hormone reduction process If there is deficiency of the kidney is the basic prescription for licorice, Qi Si, Astragalus Sunburn, Schisandra, Yang plus Psoralen Sinple Spleen Yin plus Ligustrum lucidum, Eclipta prostrata, immunosuppressive agent process In the white blood cell count decline can give qi and blood drugs such as Angelica, Millettia, Polygonatum, Motherwort, Agrimony and so on.

Other treatments

① captopril acid: angiotensin Ⅱ conversion enzyme inhibitors Some people think that can improve the glomerular hemodynamic state and reduce urinary protein excretion can be used for hormone-assisted therapy, especially with hypertension.

② levamisole: immune regulator is generally used for adjuvant therapy of hormones, especially often accompanied by infection of hormone-dependent cases or frequent recurrence dose of 2.5mg / kg every other day after medication can reduce the concurrent respiratory infection and hormone-dependent hormone The amount of reduction.

③ anticoagulant application: nephrotic syndrome is often hypercoagulable state appears, it was argued should be added with anti-platelet aggregation agent or anticoagulant such as Chinese medicine, heparin, blood circulation, dipyridamole, Salvia and so on.