Pregnancy with chronic adrenal insufficiency

Introduction to pregnancy with chronic adrenal insufficiency Chronic adrenal insufficiency is divided into primary and secondary categories. The primary is also known as Addison's disease, which is caused by insufficient secretion of bilateral adrenal cortex hormones due to the lesions of the adrenal cortex itself. Secondary is caused by insufficient secretion of adrenocorticotropic hormone (ACTH) due to hypothalamic-pituitary lesions. basic knowledge The proportion of illness: 0.001% - 0.002% (incidence rate is about 0.001% - 0.002%, more common in pregnant women with postpartum hemorrhage) Susceptible population: pregnant women Mode of infection: non-infectious Complications: premature birth, low blood sugar

Pregnancy with chronic adrenal insufficiency

(1) Causes of the disease

Chronic adrenal destruction (35%):

Adrenal tuberculosis is the most important cause of adrenal insufficiency. After liberation, tuberculosis in our country is significantly reduced, so patients with this disease are also reduced accordingly; other such as syphilis, fungal infection can also cause damage to the adrenal cortex; vascular lesions such as thrombosis, embolus Blocking blood supply or bilateral cortical hemorrhage can also be caused.

Other (27%):

Idiopathic adrenal atrophy is another important cause and is associated with autoimmunity. Adrenal tumors are caused by subtotal or total resection of the adrenal gland. Congenital adrenal insufficiency and so on.

(two) pathogenesis

The main hormones produced by the adrenal cortex include cortisol, aldosterone and dehydroepiandrosterone. When adrenal insufficiency, sugar and mineralocorticoid secretion are insufficient, and the patient's urine, sweat, saliva and gastrointestinal sodium excretion increase. Reduced excretion, resulting in low serum concentrations of sodium, chlorine and high serum concentrations of potassium, due to the body can not concentrate the urine well, coupled with electrolyte imbalance, can cause severe dehydration, blood volume decline, hypotension and circulation Deficiency, lack of cortisol can also cause protein, fat and carbohydrate metabolism disorders and severe insulin hypersensitivity, leading to hypoglycemia and liver glycogen reduction, reduction of adrenocortical hormone secretion weakens resistance to infection, trauma, etc. , myocardial contraction weakness and decreased blood volume, so that the reduction of cardiac output can cause collapse, nerves, muscle function decline can cause weakness, cortisol reduction can increase the production of pituitary ACTH and the rise of -lipotropin High, the latter can stimulate the melanocytes, causing pigmentation of the skin and mucous membranes.

Untreated patients with adrenal insufficiency in pregnancy often cause infertility due to adrenal insufficiency. After corticosteroid treatment, combined pregnancy is not uncommon. Due to abnormal glucose metabolism, hypoglycemia is prone to occur in early pregnancy, especially glucocorticoid deficiency. When hypoglycemia is easy to aggravate, the fetus is prone to growth restriction during pregnancy. When there is a certain reserve of adrenal function, some pregnant women can often maintain normal daily life. I do not know that I have this disease, but under certain stress, such as labor. Sudden occurrence of Addison crisis during childbirth, surgery and puerperium, ie extreme weakness, nausea, vomiting, upper abdomen and back, leg pain, hypothermia and hypotension, peripheral vascular collapse, renal failure and other life-threatening conditions, At this time, it is only diagnosed with Addison's disease. It is important to diagnose the disease before pregnancy. Seaward et al (1989) collected 5 cases of pregnancy with Addison disease since 1972, and 1 case failed before pregnancy. Diagnosis, the Addison crisis during pregnancy with early placental ablation and fetal death, and Abett et al (1989) reported that 6 cases of diagnosis of Addison disease before pregnancy, all obtained In a successful pregnancy, the fetal outcome is often parallel to the condition of the pregnant woman.

Pregnancy with chronic adrenal insufficiency prevention

Educate patients to understand the nature of the disease, adhere to lifelong hormone replacement therapy, including long-term physiological dose replacement and short-term stress replacement therapy, usually with the appropriate basic amount of physiological needs, such as complications or surgery In order to prevent a crisis, it is necessary to increase the amount by 3 to 5 times or more. Educate patients to carry disease cards with their contacts and addresses to facilitate timely treatment.

Pregnancy with complications of chronic adrenal insufficiency Complications, premature hypoglycemia

If the appropriate amount of glucocorticoid replacement therapy is not used during pregnancy, the fetus will have premature birth and the probability of stillbirth will increase. The mild pregnant woman can complete the full-term birth. The baby's weight can be 500g lower than that of the normal pregnant woman. This may be Caused by low blood sugar in the mother.

Pregnancy with chronic adrenal insufficiency symptoms Common symptoms Amenorrhea abdominal pain, low blood pressure, loss of appetite, constipation, coma, high fever, bloating, diarrhea

The disease is more common in adults, the elderly and younger are less common, tuberculosis more men than women, autoimmune caused by "esthetic" women more than men, slow onset, early performance fatigue, weakness, weakness, The spirit is wilting, the appetite is weak, the body weight is obviously reduced, and it resembles neurosis. The typical clinical manifestations are as follows:

Pigmentation

Found in primary patients, due to increased secretion of pituitary ACTH, melanocyte stimulating hormone is also increased, skin, mucous membrane pigmentation, especially in the friction, palm print, areola, scar, etc., secondary anterior pituitary dysfunction This symptom.

2. Cardiovascular system

Nearly half of patients with blood pressure can be lower than 90/60mmHg (12/8kPa), can be reduced to zero in crisis, often dizziness, orthostatic hypotension, fainting.

3. The gastrointestinal system

Loss of appetite, salty food, weight loss, nausea, vomiting, bloating, indigestion, diarrhea, abdominal pain, constipation, etc.

4. Nerves,

Mental manifestation of muscle weakness is one of the main symptoms, apathy, fatigue, lethargy, mental disorders, lack of energy, memory loss, irritability, and more insomnia.

5. Other

Hyperkalemia, hyponatremia, chronic water loss, body weight reduction of 5 ~ 10kg or more, gluconeogenesis weakened, can occur fasting hypoglycemia, sexual dysfunction, male impotence, female pubic hair, hair loss, menstrual disorders or amenorrhea Mild patients can be pregnant, such as tuberculosis infection, often have low fever, night sweats and other symptoms, physical weakness and weight loss is more serious, such as accompanied by other autoimmune diseases, may be associated with the corresponding disease performance.

6. crisis

The symptoms of sudden increase in the disease are often caused by infection, trauma, surgery, childbirth and other stress stimuli or sudden interruption of treatment, such as nausea, vomiting, abdominal pain, diarrhea, severe dehydration, lower blood pressure, rapid heart rate, pulse Thin, shock, hypoglycemia, low blood sodium, high fever, mental disorders, if not rescued in time, can quickly develop into shock, coma, death.

Pregnancy with chronic adrenal insufficiency examination

Metabolic disorder

Blood sodium is lowered, blood potassium is elevated, serum chloride is lowered, blood sugar is lowered, and blood sodium/blood potassium ratio is <30.

2. Adrenal function test

(1) 24h urine 17-hydroxycorticosteroid (17-OHCS) and 17-ketosteroid (17KS) were significantly lower than normal, generally below 5mg, even below 3mg or close to zero.

(2) 24h urinary free cortisol is often lower than the normal low limit, generally below 20g / d.

(3) Plasma 17-hydroxycorticosteroid concentrations are often significantly lower than the normal lower limit.

(4) ACTH test can reflect the cortical reserve function.

3. The blood lymphocytes increase, often above 40%, eosinophils, often more than 4%, the absolute count is often above 300 × 10 ⁶ /L.

4. Radiological examination of abdominal plain film, caused by tuberculosis can see calcification in the adrenal gland, chest X-ray examination can sometimes find tuberculosis and heart shrinkage.

5. The electrocardiogram has low voltage and PR, and the QT interval is prolonged. The EEG is a wide-ranging -rhythm slow wave.

6. CT examination of the history of tuberculosis can increase or calcification of the adrenal gland, the adrenal gland may not increase due to autoimmune causes.

Diagnosis and differential diagnosis of pregnancy complicated with chronic adrenal insufficiency

diagnosis

Chronic adrenal insufficiency during pregnancy is more difficult to diagnose because its clinical symptoms (thinning, skin mucosal pigmentation, loss of appetite, fatigue) can occur in normal pregnancy. Certain features may contribute to the diagnosis of chronic adrenal insufficiency during pregnancy, such as severe vomiting in the early and middle stages of pregnancy, weakness in early pregnancy, hypotension or hyperpigmentation. A correct diagnosis must also be made in conjunction with laboratory tests. Mild and atypical patients have a positive finding only after stress or after ACTH stimulation.

Differential diagnosis

Partial or mild adrenal insufficiency, clinical manifestations are atypical, should be differentiated from neurosis, mild early tuberculosis, cancer, cirrhosis, hemochromatosis, chronic metal poisoning.

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