Pediatric Periodic Hyperkalemic Paralysis
Introduction
Brief introduction of children with periodic hyperkalemia Periodic paralysis is a group of metabolic diseases related to potassium metabolism. It is a continual, self-limiting episode of skeletal muscle flaccid paralysis or weakness. It lasts for several hours to several weeks. Characteristics of the disease. According to the change of blood potassium at the time of onset, it can be divided into three types: hypokalemia type, hyperkalemia type and normal blood potassium type. This disease is autosomal dominant. Hyperkalemic peripheral disorder (hyperkalemic peripheral disorder) is also known as hereditary paroxysmal weakness (adynamia episodeicahereditaria). basic knowledge The proportion of sickness: 0.00001% Susceptible people: children Mode of infection: non-infectious Complications: periodic paralysis, periodic paralysis
Cause
The cause of periodic hyperkalemia in children
Causes:
Foreign reports have periodic family history, autosomal dominant inheritance, high penetrance rate, and extremely rare family history in China.
Pathogenesis:
The pathogenesis is still unclear. It is generally believed that the concentration of potassium ions is related to fluctuations inside and outside the cell. Except for thyroid and adrenal gland, which may be the pathogenesis of the disease, many of the effects on glucose metabolism, water and electrolyte balance are also affected. It can cause similar episodes of periodic paralysis. In short, potassium and glucose metabolism disorders are the main aspects of the pathogenesis of this disease; there is also a complex relationship between endocrine dysfunction and myasthenia gravis.
Prevention
Children with periodic hyperkalemia paralysis prevention
Avoid predisposing factors, avoid strenuous exercise, cold, hunger, nervousness, etc., and avoid the application of potassium preparations and high potassium diets. Early detection, early diagnosis, and early treatment are important for preventing this disease. Regular examination should be done during pregnancy. If the child has a tendency to develop abnormalities, chromosome screening should be done in time, and abortion should be performed in time to avoid the birth of the diseased child.
Complication
Children with periodic hyperkalemia paralysis complications Complications periodic paralysis periodic paralysis
Generally no complications. The pathogenesis is still unclear. It is generally believed that the concentration of potassium ions is related to fluctuations inside and outside the cell. Except for thyroid and adrenal gland, which may be the pathogenesis of the disease, many of the effects on glucose metabolism, water and electrolyte balance are also affected. It can cause similar episodes of periodic paralysis. In short, potassium and glucose metabolism disorders are the main aspects of the pathogenesis of this disease; there is also a complex relationship between endocrine dysfunction and myasthenia gravis.
Symptom
Symptoms of periodic hyperkalemia paralysis in children Common symptoms Painful hyperkalemia, paralysis, limb weakness, muscle tension, pain, muscle cramps
Hyperkalemia type periodic has a genetic history, childhood onset, often caused by cold or potassium salt, daytime onset, often after the daytime exercise, duration does not exceed 1h, clinical manifestations of limb weakness and hypokalemia cycle Paralysis is similar, but to a lesser extent; often accompanied by muscle pain and muscle rigidity, more common in the muscles of the facial muscles, the tongue muscles and the hands, the children often start from the lower limbs, can affect the upper limbs, muscles in the trunk, brain nerves The muscles and respiratory muscles that are in control are not often involved. Children often have seizures during the day, rest after strenuous activities, cold, hunger, emotional stress and potassium can induce paralysis. The attack time can last from several minutes to several hours. Short-term hair as a feature, post-puberty onset time, severity and frequency increased, sputum reflexes decreased or disappeared during the attack, in addition, some children may show mild muscle rigidity, which can occur in the tongue, eyelids, face and palm Small inter-muscle muscles, increased in cold, such as patients only have muscle tonic discharge during electromyography, but when the limbs are immersed in cold water, it is easy to cause muscle stiffness, so it is also known as Ankylosing periodic paralysis.
Examine
Examination of periodic hyperkalemia paralysis in children
At the time of onset, the blood potassium level is increased to 5-7 mgEq/L, and the potassium excretion in the urine is also increased.
1. Electromyography: A strong tonic discharge occurs.
2. The electrocardiogram can be changed by hyperkalemia: When the hyperkalemia type periodic paralysis occurs, the electrocardiogram changes, the T wave is increased at the beginning, the QT interval is prolonged, and the R wave is gradually decreased, the S wave is deepened, and the ST segment is decreased. The PR interval and QRS time are extended.
Diagnosis
Diagnosis and differential diagnosis of children with periodic hyperkalemia
diagnosis
According to the clinical characteristics and laboratory results can be diagnosed, the diagnosis is based on:
1. There has been a history of similar attacks in the past.
2. There are predisposing factors: there may be predators, cold, excessive fatigue, alcohol abuse, application of potassium preparations and high potassium diet and other predisposing factors.
3. Limb symmetry flaccid paralysis: acute or subacute onset of limb symmetry flaccid paralysis, characterized by lower limb weight, upper limbs light, proximal end, and distal distal.
4. Other symptoms: Some patients may have thirst, palpitation and muscle pain.
5. Laboratory examination: serum potassium is elevated.
6. ECG examination: there is a change in hyperkalemia.
7. Electromyography examination: It indicates that the potential amplitude is reduced and the number is decreased. When the total sputum is removed, the motor unit potential disappears and the electrical stimulation has no response.
8. Exclude hyperkalemia caused by other diseases.
Differential diagnosis
It is differentiated from hypokalemia type periodic spasm and normal blood potassium type periodic paralysis.
According to the laboratory and auxiliary examination results, it can be differentiated from hypokalemia type periodic paralysis and normal blood potassium type periodic paralysis.
