What Is Corticobasal Degeneration?

Pulmonary hemorrhage-nephritis syndrome, also known as Goodpasture syndrome, is a group of symptoms characterized by rapid nephritis syndrome accompanied by hemoptysis and rapidly developing acute renal failure. It can occur at any age, children are rare, and men are significantly more than women, ranging from 3: 1 to 10: 1. Most patients progress rapidly and the prognosis is dangerous.

Basic Information

nickname: Goodpasture syndrome
Visiting department: Nephrology

Multiple groups: male
Common symptoms: Hemoptysis, hematuria, fatigue, weakness, weight loss, anemia, etc.

Causes of pulmonary hemorrhage-nephritis syndrome in children

It is not completely clear yet, it may be caused by the glomerular basement membrane-specific autoimmunity, and environmental and genetic factors are involved. Respiratory tract infections, especially viruses, vapors, and smoking may be incentives.

Clinical manifestations of pulmonary hemorrhage-nephritis syndrome in children

The clinical features are triads: lung damage, kidney damage, and anemia.

General performance

Frequent symptoms of fatigue, weakness, and weight loss. The vast majority of patients have anemia, and a few may have hypertension.

2. Symptoms of lung damage

Clinically, respiratory symptoms are the first symptoms, hemoptysis is not obvious in the early stage, or only a small amount of hemoptysis is intermittent. May be accompanied by cough and shortness of breath. May be accompanied by cough, chest pain, and fever. 10% to 30% of patients with symptoms of respiratory infection as the initial symptoms. Pulmonary symptoms can precede kidney disease for days to years.

3. Symptoms of Kidney Damage

Hematuria was the main manifestation, accounting for 80% to 99%. Gross hematuria, red blood cell casts, often accompanied by moderate proteinuria, can progress rapidly. Most manifested as oliguria, anuria, hypertension, and edema, with symptoms exacerbating progressively. It usually develops into renal failure within a few days to 2 to 3 months.

4. Other symptoms

Anemia may occur with repeated bleeding or heavy bleeding, and chronic cases include hepatomegaly, splenomegaly, and jaundice.

Pulmonary hemorrhage-nephritis syndrome in children

Laboratory inspection

(1) Urine test Hematuria, proteinuria and leukocyte urine often have red blood cell casts.

(2) Sputum examination Microscopic examination showed macrophages containing heme.

(3) Blood test The anti-human globulin test (Coomb test) is negative. If the pulmonary hemorrhage is severe or lasts for a long time, there may be small cells and low pigment anemia, and the white blood cells of more than half of the patients exceed 10 × 10 ⁹ / L. Serum complement generally does not decrease.

(4) Blood biochemistry In the early stage, urea nitrogen (BUN) and blood creatinine (Scr) can be normal, and BUN and Scr can progress progressively.

(5) Specific examination Serum anti-glomerular basement membrane antibodies (anti-GBM antibodies) are mostly positive, which is of great value in the diagnosis of this disease.

2. Other auxiliary inspections

(1) Imaging examination The early changes of lung X-rays are similar to pulmonary edema. During the period of pulmonary hemorrhage, both lungs are diffuse or scattered in miliary to nodular changes.

(2) Pathological examination Typical lesions in the lungs Alveolar hemorrhage, hemosiderin deposition and fibrosis. Electron microscopy showed degeneration, rupture, and focal hyperplasia of the alveolar wall capillaries, and electron dense deposits were visible. Immunofluorescence showed linear deposition of IgG and C. Typical renal lesions Light microscopy showed diffuse glomerular damage, a large number of crescent formations, and severe glomerular atrophy with diffuse glomerular fibrosis and interstitial fibrosis. Immunofluorescence examination showed linear deposits (mainly IgG, IgA, IgM, C3, and fibrinogen) along the glomerular basement membrane endothelium. Electron microscopy showed degeneration, rupture, or diffuse thickening of the glomerular basement membrane.

Diagnosis and differential diagnosis of pulmonary hemorrhage-nephritis syndrome in children

According to the patient's repeated hemoptysis with hematuria, sputum containing hematin-containing macrophages, decreased alveolar and arterial partial oxygen pressure, and typical changes in lung X-rays can be initially diagnosed.

Three major characteristics of pulmonary hemorrhage, glomerulonephritis, and GBM antibodies in the blood can be diagnosed.

Need to be distinguished from:

Lupus nephritis

More common in women, usually with skin, joint lesions, and multiple systemic damage to the body. Serum immunological tests and renal biopsy can help identify.

2.Wegener's granuloma

Typical cases are rhinitis or sinusitis, pulmonary infiltration and necrotizing glomerulonephritis, c-ANCA positive and renal biopsy can be identified.

3. Idiopathic pulmonary hemosiderin

No hematuria, proteinuria, normal renal function, positive serum lectin test, negative serum anti-GBM antibody.

4. Nodular polyarteritis

Fever, but mild proteinuria and hematuria, anuria and renal failure may occur, but no coughing blood and changes in lung X-rays, and serum anti-GBM antibodies are negative.

5. Other primary and secondary systemic vasculitis

At the same time, kidney and lung invasion need to be identified, mainly through serum immunological detection and renal biopsy.

Treatment of children with pulmonary hemorrhage-nephritis syndrome

Adrenal hormone

High-dose methylprednisolone impact therapy is the main treatment method, which can slow the progression of the disease.

2. Plasma exchange

The curative effect is definite, especially in the early stage of the disease, which has not yet developed into oliguria-type renal failure. The combined application of immunosuppressive agents and glucocorticoid therapy can effectively prevent pulmonary hemorrhage and improve renal function.

3. Immunoadsorption

It can also remove the anti-basement membrane antibodies in the circulation, reduce the antigen-antibody response, and control the disease's activity.

4. Kidney replacement therapy

In patients with renal failure, early hemodialysis or peritoneal dialysis is performed. In addition to dialysis, patients with end-stage renal disease may be treated with kidney transplantation.

5. Symptomatic treatment

Correct anemia and transfusion if necessary. Antibiotic therapy is not effective for lung lesions, and it is used when combined with secondary infections. The efficacy of anticoagulant drugs and drugs to prevent platelet aggregation is not certain.

Prognosis of childhood pulmonary hemorrhage-nephritis syndrome

The disease progresses rapidly, and most often die of respiratory failure or renal failure within a few months of onset. The prognosis is related to the number of glomeruli forming the crescent. The more glomeruli forming the crescent, the worse the prognosis. Early diagnosis and treatment can help improve the prognosis.