LESSON 13
KIDNEY AND URINARY DISEASES
PRIMARY NEPHROTIC SYNDROME
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Target
1. Present the definition and diagnostic criteria according to international convention and state the epidemiological characteristics of primary nephrotic syndrome in children.
2. Describe the clinical and paraclinical manifestations and main complications of primary nephrotic syndrome in children.
3. Presentation is classified according to cause, clinical form, progression, and treatment of primary nephrotic syndrome in children.
4. Present the treatment regimen for primary nephrotic syndrome in children and outline preventive measures.
1. General
Primary nephrotic syndrome in children (PNS) is a set of symptoms showing glomerular disease, the cause of which is mostly idiopathic (90%).
2. Definition of HCTHTPTE
The definition of HCTHTP is mainly clinical-biological and includes massive proteinuria and edema. The cause of HCTHTP is often unclear, so it is also called idiopathic HCTH.
3. Epidemiology
HCTHTPTE is a syndrome that represents chronic glomerular disease commonly found in children. The incidence of the disease varies by age, gender, race, geography, constitution...
3.1. Incidence of disease
In some pediatric departments or pediatric hospitals, the number of children with HCTH accounts for about 0.5-1% of the total number of inpatients and 10-30% of the total number of children with kidney disease. In the Pediatric Department of Hue Central Hospital, the number of children with HCTH accounts for 0.73% of the total number of inpatients and 30% of the total number of children with kidney disease.
3.2. Age
The average age of onset of the disease in Vietnamese children is 8.7 years old, while in foreign countries the age of onset is lower and is often seen in preschool children.
3.3. Gender
Boys are more affected than girls (ratio 2:1)
3.4. Race
Asian children are more affected than Europeans (ratio 6:1). African children are less affected by IBD, but if black children do develop IBD, they are often resistant to steroids.
3.5. Geography
In Vietnam, the rate of HCTHTPTE at the Institute of Pediatrics was 1.78% (1974-1988); Children's Hospital I was 0.67% (1990-1993); Department of Pediatrics - Hue Central Hospital was 0.73% (1987-1996).
In the US, the incidence is 16/100,000 children under 16 years old, or 0.016%.
3.6.Some favorable factors
The disease often appears after acute respiratory infections and on allergic conditions (eczema, asthma...)
4. Clinical manifestations
4.1. Edema: Whole body edema with characteristics of white, soft, pitting edema (positive Godet sign), painless. Edema usually starts suddenly from the face spreading to the whole body, in addition, there is the phenomenon of multi-membranous edema, meaning there is edema in the peritoneum, testicular membrane in boys, possibly in the pleura, pericardium, meninges, so when examining clinically, attention should be paid to the above organs.
4.2. Oliguria : Clear yellow urine
4.3. Abdominal pain: Abdominal pain is an infrequent and nonspecific symptom, which can be caused by abdominal distension when there is too much ascites causing pain or by mesenteric vascular occlusion, digestive disorders, primary peritonitis...
5. Paraclinical manifestations
5.1. Urine test
- Biochemistry: Proteinuria is mostly > 100mg/kg/24 hours, selective proteinuria
- Cells: Red blood cells are almost absent or only in a mild and transient microscopic form. Radiolucent casts
5.2. Blood test
- Total protein decreased significantly, mostly < 40g/l
- Blood protein electrophoresis shows: Blood albumin decreased significantly (<25g/l), alpha2Globulin and beta Globulin increased, gamma Globulin decreased significantly in the late stage.
- Increased blood lipids and cholesterol.
- Blood formula: Red blood cells decrease, white blood cells and platelets may increase.
- Blood flow rate usually increases in the first hour > 50mm
- Electrolyte chart: Sodium, Potassium, Calcium often decrease
- Urea, Creatinine within normal limits
6. Classification of nephrotic syndrome in children
6.1.According to the cause 6.1.1. Congenital HCTH (rare). 6.1.2. Primary HCTH (idiopathic).
6.1.3. Secondary HCTH (after systemic diseases, metabolic diseases, infections-poisoning.)
6.2. Clinically
6.2.1.Primary simple HCTH.
6.2.2. Primary combined or non-simple HCTH (nephritis-nephrotic syndrome).
6.3. According to progress
6.3.1.First primary HCTH.
6.3.2. Primary HCTH relapse (edema and increased protein when switching from attack dose to maintenance dose or after stopping maintenance dose).
6.4. According to treatment
6.4.1. “Corticoid-sensitive” HCTH (urine is clear of protein within 2 weeks). 6.4.2. “Corticoid-dependent” HCTH (recurrence when changing dose or stopping the drug). 6.4.3. “Corticoid-resistant” HCTH (proteinuria still increases significantly after attack treatment).
6.5. According to pathological anatomy 6.5.1. Minimally invasive HCTH (85%) 6.5.2. Membrane-proliferative HCTH (5%) 6.5.3. Focal fibrosis HCTH (10%)
6.6. Healing criteria
Primary nephrotic syndrome in children is called "cured" when treatment is stopped for more than 2 years without any recurrence.
7. Diagnosis
Early diagnosis requires relying on
7.1.Circumstances of discovery
Epidemiology, predisposing factors and early clinical signs are rapid generalized edema with white, soft, pitting, painless characteristics.
7.2. Biological testing
Biological tests help determine HCTH with the following criteria:
- Proteinuria over 3g/24h is mostly Albumin or over 50mg/kg/24h for children. Selective proteinuria (meaning > 80% Albumin is lost in the urine due to its smaller molecular weight than globulins)
- Blood protein below 60g/l and blood albumin below 25g/l
- Edema and hyperlipidemia are common but are not essential for diagnosis.
7.3. Kidney biopsy
In children, kidney biopsy is not necessary because the majority (80-90%) have minimal damage and respond well to corticosteroids, especially for children under 8 years old. Kidney biopsy is only performed in a few cases such as congenital HCTH (children under 1 year old); combined HCTH; corticosteroid-resistant HCTH
8. Progression and complications
8.1. Progress
There are 4 ways as follows:
8.1.1. One batch only
Only one episode in a few weeks (edema and proteinuria disappear): this is a corticoid-sensitive form (25%)
8.1.2.Recurrent episodes
Recurred many times over many years but eventually healed completely (25%)
8.1.3.Continuous recurrence
Continuous relapse when reducing dose or stopping medication: this is corticoid-dependent form (30-35%)
8.1.4.Failure
(previously used prednisone then Methyl-prednisone): this is corticoid resistance (15-20%)
8.2. Complications
8.2.1.Complications of the disease
- Infection is the most common complication (primary peritonitis, pneumonia, cellulitis, urinary tract infection, sepsis)
- Deficiency syndrome: Slow growth, malnutrition, osteoporosis, convulsions due to hypocalcemia (tetany), anemia, simple goiter...
- Mesenteric embolism, pulmonary embolism, and limb embolism.
- Abdominal pain: May be caused by mesenteric edema, pancreatic edema, primary peritonitis, gastric ulcer
8.2.2. Complications of treatment
- Corticoids usually only cause complications when used in high doses and for a long time, causing water and electrolyte disorders; endocrine and metabolic disorders; affecting most organs in the body...
- Immunosuppressants and cancer cell inhibitors: can cause bone marrow failure, infertility, leukemia, infections, hemorrhagic cystitis, baldness...
- Diuretics: when used suddenly in large amounts, it can cause electrolyte disturbances (hyponatremia, hypokalemia) and volume loss (cardiovascular collapse, kidney failure).
9. Treatment and prevention
9.1. Symptomatic treatment (edema)
9.1.1. Rest 9.1.2. Diet
Limit salt and water and eat lots of protein and enough vitamins (salt 2-3g/day; water
<15ml/kg/day; protein 2-4g/kg/day)
9.1.3 Keep your body clean; keep warm
9.1.4. Rarely give diuretics and protein infusions.
9.2. Pathogenesis treatment (specific)
Corticosteroid therapy (Prednisone 5mg)
Corticoids have anti-inflammatory and immunosuppressive effects.
- Attack dose: Prednisone 2mg/kg/day x 4-8 weeks. Take during or after meals once in the morning or divide into 2-3 times a day. Then, depending on whether proteinuria is negative or not, choose a maintenance dose.
- Maintenance dose
+ If proteinuria is negative: 1mg/kg/day x 6 consecutive weeks then stop the drug or reinforce with a dose of 0.5mg-0.15mg/kg used 4/week for 4-6 months
+ If proteinuria is still positive: 2mg/kg/every other day x 4 consecutive weeks
+ If the attack dose fails, you can try Methyl-prednisone (Solu-Medron) 30mg/kg (intravenous) x 2-3 days a week. Then re-evaluate the response to steroid treatment.
- Treat again as before if relapse or dependence. If drug resistance occurs, change to another drug.
9.3. Treatment of complications
7.3.1. Antibiotics in infections
- Penicillin 100,000 units per kg body weight per day, orally or intramuscularly
7.3.2.Heparin in embolism
- Heparin dose 200-300 units per kg body weight per day by deep intramuscular injection 7.3.3. Compensate for deficiencies such as Calcium, Potassium...
9.4. Reserves
Because the disease often recurs, regular monitoring is required for many years (at least 5 years), so it is necessary to convince patients and parents to strictly comply with inpatient and especially outpatient treatment regimens through outpatient medical records to monitor...
Preventing favorable factors leading to HCTHTP recurrence
Monitor clinical symptoms (height, weight, blood pressure), paraclinical symptoms (erythrocyte sedimentation rate, proteinuria), and drug side effects.
EVALUATION QUESTION:
SITUATIONAL QUESTION:
Patient Long, 5 years old, 3rd day of hospital treatment. The child urinates 300-400ml of yellow urine per day; very large edema, chubby limbs, accompanied by peritoneal and testicular effusion. Urinary protein test shows 5g/l in 400ml of urine per day; blood protein 48g/l; 70ml of sedimentation per hour. The child is from a poor family, the family feeds the child mainly rice with vegetables and fish sauce every day, and the hygiene conditions are poor.
Based on clinical and paraclinical symptoms, please state the definitive diagnosis of the above disease.
1. List the most common complications:
A. Risk of infection
B. Oliguria due to water retention in the interstitial space
C. Large edema and effusion of the peritoneum and testicles
D. The family does not fully understand the role of diet for the patient.
2. To eliminate edema, ascites and hydrocele, the patient needs:
A. Bed rest and strong diuretics
B. Eat lots of meat, fish, eggs, milk and use strong diuretics
C. Eat lots of meat, fish, eggs, milk and use high doses of prednisolone
D. Eat lots of meat, fish, eggs, milk, use prednisolone and strong diuretics
3. Instructions for Long to use prednisolone:
A. Take 6 pills once a day, after breakfast, continuously for 4-8 weeks.
B. Take 6 pills per day, divided into 3 times on full meals, continuously for 4-8 weeks.
C. Take 3 capsules daily, once after breakfast, for 8 weeks.
D. Take 1 capsule daily, once after breakfast, for 12 weeks.
4. What should Long do to prevent the risk of infection?
A. Maintain food hygiene, personal hygiene, and environmental hygiene
B. Keep warm, maintain food hygiene, personal hygiene, and environmental hygiene
C. Eat lots of meat and fish; keep children warm; maintain food hygiene, personal hygiene, and environmental hygiene.
D. Use antibiotics; eat lots of meat and fish; keep warm; maintain food hygiene, personal hygiene, and environmental hygiene.
FEVER
Target
LESSON: 14
FEVER- SEIZURE- MENINGITIS
1. Describe the definition and causes of fever.
2. Presentation of fever classification according to body temperature
3. Describe how to treat a patient with fever.
1. GENERAL
Definition : Fever is when the body temperature exceeds the normal limit. A child is considered to have a fever when the rectal temperature is 38 0 C or higher (axillary temperature is 37.5 0 C or higher).
Fever is the main symptom when: the mother reports having a fever for several days, or has an armpit temperature of 37.05C or feels hot to the touch.
Fever is an elevation of body temperature due to a specific biological response, mediated and controlled by the central nervous system. Fever is a symptom of many infectious and non-infectious diseases.
2. CAUSES OF FEVER
2.1 Causes of infection
2.1.1 Viral infection
Viral infections are the leading cause of fever in children. The illness is usually self-limited within 7 days.
2.1.2 Bacterial infection
Common infections include respiratory infections (pneumonia, pharyngitis, tonsillitis), digestive and urinary tract infections, ear infections (otitis media, mastoiditis).
2.1.3 Parasitic infections
Malaria is a common cause of fever in children living in malaria-endemic areas.
2.1.4 Labor
Tuberculosis is an important cause of fever, especially prolonged fever, in developing countries.
3. Classification of fever according to body temperature
- Mild fever: below 38 0 C
- Moderate fever: 38 0 - 39 0 C
- High fever: 39 0 - 41 0 C
- Very high fever: over 41 0 C
4. Treatment of fever symptoms and consequences of fever
Fever is an adaptive response of the body when infected, a sign that helps monitor the progression of the disease to determine a further step of clinical diagnosis, and at the same time evaluate the results of specific treatment. In infectious diseases, antipyretics should not be given mechanically to deal with fever symptoms without trying to find the cause for specific treatment, except in cases where the child has a very high fever and harmful symptoms.
4.1 Using antipyretics for children, dosage, how to use
The recommended antipyretic drug for children is Paracetamol, in addition to oral suppositories used rectally, antipyretic drugs should not be used intramuscularly or intravenously for children.
Contraindicated in children with hypersensitivity to Paracetamol, impaired liver function.
Children's Paracetamol dosage is: 10-15 mg/kg/dose, or 60mg/kg/24 hours, divided into 4 doses/day. Temperature should be checked before taking the next dose.
For children with a history of high fever causing convulsions: Gardenal is given orally at a dose of 7-10 mg/kg. If the child cannot take it orally, it can be given by intramuscular or intravenous injection and the fever should be lowered early. If the child is having a febrile convulsion, at all costs, the seizure must be stopped as a priority before other treatment indications are given.
4.2 Physical cooling measures
Children should not be kept too warm, and should be dressed in cool clothes. You can use a fan, air conditioner, cool wipes, wet towels on the forehead, and warm wipes with warm water lower than the fever temperature. Do not apply ice directly to the forehead or abdomen, and do not use ice water to wash the stomach, to avoid making the child feel cold and too cold causing shivering and peripheral vasoconstriction.
4.3 Advice for mothers on diet and drinking when children have fever
- Monitor for severe signs: a child with fever who does not require inpatient treatment should be told to bring the child back for a check-up after two days and the mother should be advised of symptoms that require immediate re-examination.
- Give children enough water to eat and drink: Advise mothers about diet, eat normally, eat many meals, do not let children eat less, especially do not let children eat less nutrients, drink more water than usual.
- Do not wear too many clothes: especially warm clothes. Note that there is a difference when a child has a fever in the season that is too hot or too cold.
In summary, fever must be properly assessed for both its benefits and harms. Treating the underlying cause is the best and most correct method of reducing fever. However, it is still necessary to prevent the harmful effects of fever by combining heat dissipation through the skin with antipyretics to reduce the high temperature threshold in the hypothalamus in parallel with specific treatment.
SEIZURE SYNDROME
Target :
1. Describe the causes of seizures in children.
2. Describe the initial treatment of seizures.
1. Introduction
Seizures are a common neurological disorder in children, with a frequency of 3-5%. Seizures are not a disease but a symptom of a neurological disease that needs to be carefully examined to have an appropriate treatment plan.
Seizures are defined as involuntary, paroxysmal brain dysfunction that may be characterized by decreased or loss of consciousness, abnormal motor activity, behavioral disturbances, sensory disturbances, and autonomic dysfunction.