Pediatric Cushing's Syndrome
Introduction
Introduction to Pediatric Cushing's syndrome Typical clinical symptoms and signs, such as bloody full moon face, high blood pressure, osteoporosis, high insulin blood, etc., are called Cushing's syndrome. basic knowledge The proportion of illness: 0.003%--0.007% Susceptible people: children Mode of infection: non-infectious Complications: electrolyte imbalance heart failure
Cause
The cause of pediatric Cushing syndrome
Excessive secretion of adrenal cortisol hormones (30%):
Causes a variety of metabolic disorders caused by clinical syndromes, cortisol mobilizes fat to decompose triacylglycerol into glycerol and free fatty acids, while blocking glucose into fat cells to inhibit fat synthesis, cortisol allows insulin secretion, can promote fat synthesis, various parts of the body Protein, adipose tissue has different sensitivities to corticosteroids, and there is centripetal obesity.
Cortisol promotes protein breakdown (30%):
Inhibition of tissue uptake of amino acids and synthesis of proteins, the body is in a state of negative nitrogen balance, affecting muscle, bone, skin over-consumption of protein, bone decalcification and osteoporosis, affecting bone growth and development of children, excessive cortisol promotes liver Glycogen is also eclipsed. In addition, cortisol also antagonizes the effect of insulin on glucose metabolism, reduces the utilization of glucose by tissues, and increases blood sugar, which can lead to steroid diabetes.
Adrenal cortex hormones (30%):
The mineralocorticoids in the adrenal cortex have deoxycorticosterone (DOCA) and corticosterone, which can also increase secretion, causing sodium retention in the body, increased potassium and chlorine excretion, and elevated blood pressure may be due to cortisol strengthening the noradrenal gland. The effect of the pigment on the small arteries, and the retention of sodium in the body, the patient's plasma renin activity is increased, angiotensin II causes an increase in blood pressure, the resistance of the patient to infection is significantly reduced, skin and systemic infections are common, due to the cortex The alcohol reduces the immune function of the antibody and hinders the formation of antibodies.
Prevention
Pediatric Cushing's Syndrome Prevention
More cortisolism caused by adrenal hyperplasia caused by pituitary adenoma or adrenal gland tumor caused by excessive secretion of cortisol, early detection of the diagnosis of the disease, according to different causes, early treatment, clinical attention should be paid to the iatrogenic Cushing syndrome Prevention, when a variety of diseases are treated with a large number of glucocorticoids, a reasonable treatment plan should be designed, and the dose should be gradually reduced when the condition is relieved.
Complication
Pediatric Cushing's syndrome complications Complications, electrolyte imbalance, heart failure
Often complicated by electrolyte imbalance, sodium retention, hypokalemia, high blood pressure, complicated left ventricular hypertrophy, heart failure; growth disorders, decreased academic performance, memory loss; may have fractures, osteoporosis, plethora, resistance Decreased, often secondary to infections, especially skin fungal infections. The boy showed an enlarged penis, normal testicles, pseudo precocious puberty, and an enlarged clitoris in women. In addition, there are hairy, acne, low voice, pubic hair, mane, and even breast development. Decreased academic performance, memory loss, etc.
Symptom
Symptoms of pediatric Cushing's syndrome Common symptoms Cushing's syndrome full moon face hypokalemia hypertension centripetal obesity slow growth osteoporosis secondary infection muscle atrophy masculinization
1. Lipid metabolism disorder Progressive central obesity, full moon face, mandibular, neck, back abdominal fat accumulation, limbs relatively thin, due to the accumulation of subcutaneous fat in the back and neck, expressed as "buffalo back."
2. Protein metabolism disorders Protein breakdown increases, muscle atrophy, muscle strength is reduced, skin elastic fibers are broken, and typical purple lines are visible in the lower abdomen, buttocks and thighs, and capillaries become brittle.
3. Electrolyte disorder makes sodium and water retention, showing hypokalemia, high blood pressure, severe left ventricular hypertrophy, heart failure and so on.
4. Children with growth disorders grow slowly or stagnate, and their height is shorter, mostly below the 3rd percentile. If accompanied by masculinization, the growth can exceed the height of normal children of the same age.
5. Sexual organ changes such as adrenal androgen increase when the boy shows penis enlargement, testicular normal, pseudo precocious puberty, women may have clitoris enlargement, and other hairy, hemorrhoids, low voice, pubic hair, mane, and even breast development, etc. Decreased grades, memory loss, etc.
6. Other symptoms of limb pain, sometimes fractures, X-ray manifestations of general osteoporosis, can increase red blood cells, plethora, due to increased androgen, visible hairy and acne, children with reduced resistance, often secondary infections Especially skin fungal infections.
Examine
Examination of pediatric Cushing's syndrome
1.24h urinary free cortisol and 17-hydroxycorticosteroids were both increased, and the body surface area was still high after correction. The value of children with simple obesity was also high, but not high after correction.
2. Serum cortisol concentration circadian rhythm disorder, under normal circumstances, plasma cortisol at 8:00 in the morning is higher than 11:00 in the evening; if the rhythm is disordered, the night secretion is higher or close to the morning, which is helpful for diagnosis.
3. Plasma ACTH is significantly elevated when measuring pituitary disease or atopic ACTH syndrome.
6h intravenous injection of ACTH stimulation test, can increase the plasma cortisol in patients with Cushing's disease and adrenal adenoma, but in the adrenal cancer, atopic ACTH syndrome and iatrogenic Cushing's syndrome, cortisol does not increase.
4. Dexamethasone inhibition test
(1) overnight dexamethasone test: 1 mg of dexamethasone at 11:00 pm, if plasma cortisol is still high and not inhibited, and urinary free cortisol is also increased, the diagnosis of Cushing's syndrome can be established.
(2) Low-dose dexamethasone inhibition test: If the dexamethasone inhibition test cortisol secretion is inhibited, this test can be carried out, taking 2mg / d (0.5mg every 6 hours, a total of 2 days) of dexamethasone If it is still not inhibited, it suggests the diagnosis of hypercortisolism.
(3) high-dose dexamethasone inhibition test: oral administration of 8mg / d (2mg every 6 hours, a total of 2 days) of dexamethasone, mainly used to identify the cause of hypercortisolism, such as high-dose dexamethasone If it is still not inhibited, it is likely to be adrenal adenoma or cancer. If it is inhibited, it may be adrenal hyperplasia.
Plasma cortisol or 24h urine 17-OH steroid indicators, after inhibition must be reduced to below 50% of the control value is normal.
5. Other tests can see high blood sodium, low potassium alkalosis, increased white blood cells, and decreased eosinophils.
Adrenal imaging:
X-ray inspection
(1) X-ray bone: Osteoporosis can be seen in the spine, bone mass can be seen in the limbs, occasionally pathological fracture, bone age observed in the wrist bone, showing bone age growth, bone cortex is generally loose.
(2) Skull piece: The saddle can show an increase.
(3) abdominal plain film and intravenous pyelography: visible renal translocation, adrenal calcification, etc., if necessary, abdominal adrenal angiography can determine the tumor site.
2. Abdominal adrenal B-mode ultrasound can help the location and size of the tumor.
3. CT and other examination of abdominal CT, CT scan of the adrenal gland can determine the location and shape of the tumor, X-ray saddle or CT can be used to detect pituitary lesions.
4. MRI images can identify tumors or hyperplasia. Large tumors can be seen in cancer. Adrenal gland scans with cholesterol labeled with cholesterol. The radioactivity on both sides suggests hyperplasia. One side of the radiation is concentrated, and one side has little or no radioactivity. .
Diagnosis
Diagnostic identification of pediatric Cushing syndrome
diagnosis
According to typical clinical symptoms and signs plus laboratory and auxiliary examinations can be diagnosed.
Differential diagnosis
The clinical symptoms of hypercortisolism are related to the length of cortisol secretion and the amount of secretion. Therefore, it is difficult to diagnose at the initial stage of the disease, especially difficult to distinguish from simple obesity. Generally, simple obesity is usually above the mean value. Weight gain, history of super-calorie diet, irrational diet and living system with a small amount of activity, but a few simple obese children can also find high blood pressure, buffalo back, skin purple lines and other signs, even 24h urinary cortisol excretion The amount is also increased, which is difficult to distinguish from early cortisol. The growth rate of simple obesity is normal. Repeated examination and follow-up are needed when the identification is difficult.
Most children with hypercortisolism have growth disorders and short stature, while most obese growth and development are higher than normal children of the same age. Low-dose dexamethasone suppression test can make 17-hydroxyl or cortisol in children with simple obesity. Displacement down to normal levels helps identify.
