1.Infection
The main cause of death, which can also lead to recurrence and exacerbation of the disease.
Susceptibility factors: loss of immunoglobulin from urine leads to decreased IgG levels
Peritonitis, cellulitis and pneumonia can occur
Peritonitis.
Presenting with abdominal pain, diarrhea, vomiting
Common pathogens: Streptococcus pneumoniae, Escherichia coli, Haemophilus influenzae
Duration of treatment 10~14 days
Pneumonia.
Presenting with fever, tachycardia, cough
Common pathogens: Streptococcus pneumoniae, Haemophilus influenzae
Duration of treatment 7~10 days
Cellulitis.
Redness of skin, tenderness, hard nodules
Common pathogens: beta-hemolytic streptococcus, Haemophilus influenzae, Streptococcus pneumoniae, Staphylococcus
Duration of treatment: 10 days or more
Fungal infection.
Presentation of pulmonary infiltrates, persistent fever, ineffective antibiotic therapy, fungal spores in sputum or urine
Common pathogens: yeast, aspergillosis
Skin mucosal infections, fluconazole for 10-14 days
Systemic fungal infections: amphotericin B 14~21 days
Tuberculosis infections.
High rate of infection in children with nephrotic syndrome in developing countries
Prophylaxis with isoniazid 5 mg/kg/d orally alone or with rifampicin 10 mg/kg for 6 months if tuberculin test is positive without evidence of other disease
Patients with active TB given standard anti-tuberculosis drugs, at least 2 weeks prior to corticosteroid therapy
Nail fungus.
Red, swollen and painful skin at the nail groove
Topical care treatment
Remaining.
High doses of hormones and immunosuppressants may induce herpesvirus infection and Pneumocystis carinii infection, which are very serious
Children with chickenpox should be treated with intravenous or oral application of acyclovir 40-60mg/kg/d in 4 doses for 5-7 days
Infection prophylaxis.
Avoid severe edema, edema pay attention to skin cleaning
Recommend completion of major vaccinations, pneumococcal and varicella vaccination
Consider 13-valent pneumococcal vaccine and 23-valent polysaccharide conjugate vaccine depending on the patient’s age and previous vaccination history Studies have shown that most patients can have an adequate serologic response after vaccination despite high-dose hormone therapy
Prophylactic oral penicillin has been recommended for severe ascites
Children treated with prednisone at 2 mg/kg/d or a total of 20 mg or more for more than 2 weeks may be immunosuppressed and cannot receive live attenuated vaccine, but can receive dead vaccine
Live vaccine can only be given after the child has been off hormones for 6 weeks
If the child is exposed to chickenpox, immunoglobulin should be applied within 96 hours of exposure
For children who have not received varicella vaccine, 2 doses of varicella vaccine are recommended 4 weeks apart after discontinuation of hormones
Prophylactic application of gammaglobulin after contact with measles patients in unvaccinated children
Pneumococcal vaccine is recommended during remission and non-daily hormone administration for children over 2 years of age with nephrotic syndrome
Hepatitis B vaccine in remission
2. Hypercoagulable state and blood clots
Severe and life-threatening
Susceptibility factors: age of onset of nephrotic syndrome >12 years, severe proteinuria, history of thrombosis before diagnosis of nephrotic syndrome, congenital nephrotic syndrome, membranous nephropathy, blood volume deficiency, high dose diuretic application
Mechanism: increased procoagulant substances, decreased anticoagulant substances, blood concentration, etc.
One study reported that 9% of 326 children with nephrotic syndrome experienced at least one thromboembolic event, and the median time to thrombosis was 70 days after the diagnosis of nephrotic syndrome
Site of thrombosis: lung, brain, renal vein, lower extremity arteries and veins, mesenteric vessels, etc.
Pulmonary embolism: It is not uncommon, and may manifest weakness, chest tightness, shortness of breath, cough, hemoptysis, and in severe cases, sudden death may be confirmed by CT examination of pulmonary artery.
Cerebral embolism: it may show headache, vomiting, or even convulsion, and can be confirmed by cranial vascular examination.
Renal vein thrombosis: it may show lumbar pain, hematuria, and bilateral kidneys of unequal size, which can be diagnosed by renal vascular ultrasonography or CT examination.
Lower extremity artery thrombosis: manifesting pain in the extremity, numbness, coldness of the lesioned extremity, and obstruction of movement
Lower extremity venous thrombosis: manifesting swelling and prominence of the affected limb
Mesenteric vascular embolism: manifesting abdominal pain, intractable ascites, etc. Severe cases of intestinal wall necrosis and peritonitis
Preventive measures.
Avoid hypovolemia, pay attention to rehydration especially when vomiting and diarrhea occur
Anticoagulation in case of unremitting nephrotic syndrome, e.g. disulfiram (1-2mg/kg, max 100mg, once every 8 hours), heparin (note: stop anticoagulation at least 1 week before renal biopsy)
If thrombosis occurs, anticoagulation therapy, intervention or surgery if necessary
3.Disorders of calcium and vitamin D metabolism
Loss of 25 hydroxylated osteopontin-binding protein
Resulting in decreased 25 hydroxy-vitamin D, decreased blood calcium, increased PTH, osteochondrosis and fibrous osteitis
Supplementation is needed when blood vitamin D decreases
4.Low blood volume
Severe comorbidities, life-threatening
Susceptibility factors: unremitting nephrotic syndrome (especially microscopic lesions), poor feeding, low water intake, vomiting, diarrhea, combined sepsis
Clinical manifestations: poor mental health, weakness, cold hands and feet, decreased or undetectable blood pressure, postural hypotension, thirst, tachycardia, poor capillary reperfusion, often complaining of moderate to severe abdominal pain
Ancillary tests: increased erythrocyte pressure, increased blood urea nitrogen and uric acid
Prevention: ensure basic food and water intake for the child, and timely rehydration if vomiting and diarrhea occur.
Treatment.
Rehydration, rapid intravenous application of saline 20ml/kg 1~2 hours input to correct hypovolemia
For those with a history of long-term glucocorticoid application, if rehydration cannot maintain blood pressure, hydrocortisone 5~10mg/kg/d can be given at the same time.
When available, 20% albumin 1g/kg can be administered over 2-4 hours, but albumin should be used with caution as there is a risk of pulmonary edema.
Plasma also helps to maintain effective blood volume
5.Acute renal insufficiency
Cause.
Insufficient blood volume
Severe glomerular lesions, especially proliferative lesions, resulting in decreased GFR
Significant interstitial edema, tubular obstruction by proteinuric tubular pattern to increased hydrostatic pressure in the proximal tubule and renal capsule, and decreased effective glomerular filtration rate
Extensive fusion of glomerular epithelial cells in the dirty layer of the glomerulus with reduced effective filtration area
Interstitial nephritis
Deterioration of the original glomerular lesion, or new crescentic lesion
May show decreased urine output, increased blood pressure, and increased blood creatinine
6. Abnormal thyroid function
Loss of large amounts of albumin and thyroid-binding globulin causes a decrease in T3 and T4 and an increase in TSH.
With increased TSH, hypothyroidism is very rare
Subclinical and symptomatic hypothyroidism is more common in children with treatment-resistant nephrotic syndrome
Regular thyroid function monitoring is recommended
7. Microcytic hypochromic anemia
Transferrin and erythropoietin can be lost in the urine of patients with nephrotic syndrome
Since transferrin is responsible for transporting iron to red blood cells, loss of transferrin leads to microcytic anemia and poor response to iron supplementation
Erythropoietin combined with iron therapy can increase hemoglobin levels in patients with nephrotic syndrome with anemia who have normal renal function
8. Abnormal lipid metabolism and predisposition to cardiovascular disease
The risk of heart attack is significantly higher in adults with nephrotic syndrome than in healthy individuals
The abnormalities of lipid metabolism in nephrotic syndrome include:
Increased low-density lipoprotein (LDL)
Hypertriglyceridemia
Prevention and treatment.
Control of proteinuria and hypoalbuminemia with ACEI or ARB therapy may improve lipid metabolism abnormalities and control blood pressure
Consider statins in patients with chronic nephrotic syndrome with persistent hyperlipidemia
It has been suggested to start statin therapy for LDL >3.36 mmol/L
Statins.
Lovastatin 0.4-0.8 mg/kg nightly, with monthly dose increases up to 40 mg every 12 hours
Atorvastatin 0.2 to 1.6 mg/kg nightly, with the possibility of monthly dose increases up to a maximum of 80 mg nightly
Avoid simvastatin, which increases the risk of rhabdomyolysis, especially with concomitant application of calcium-modulated neurophosphatase inhibitors
Monthly liver function and kinase tests for the first 3 months of treatment and every 3 months thereafter
Lipid testing every 6 months after starting statin therapy
9. Hypertension
Can occur
Families with children should have a blood pressure monitor and monitor blood pressure regularly
Control of hypertension can delay the progression of chronic kidney disease
Blood pressure target value.
Generally controlled below the 90th percentile of reference values for the same age and sex and height
If urine protein is >1g/d/1.73m2, blood pressure should be controlled below the 50th percentile
Choice of antihypertensive drugs.
ACEI/ARB preferred in the absence of contraindications
Avoid dihydropyridines (e.g., nifedipine, amlodipine, etc.) unless blood pressure control requires it as much as possible
Beta-blockers may be considered
Collated by: Guan Na, Department of Pediatrics, Peking University First Hospital (All rights reserved!)
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Reference.
Hui-Kim Yap, Isaac Desheng Liu, Woo-Chiao Tay. Pediatric Nephrology On the go.
Man Chun Chiu, Hui Kim Yap. Practical Paediatric Nephrology. An update of Current Practices
Yang Jiyun, Bai Kemin. Basic and Clinical Pediatric Nephrology. People’s Health Press.