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Disease, congenital adrenal hyperplasia described (CAH) is a group of the adrenal hormone synthesis enzymes in the process of diseases caused by defects, is
Congenital adrenal hyperplasia autosomal recessive disease, caused by masculine adrenogenital syndrome, also known as. CHA typical incidence rate is about 10/10 million, and the incidence of atypical about 10 times the typical, and race specific. Signs and symptoms of this disease more common in girls, male to female ratio is about 1:2, the clinical manifestations of this disease depends on the site of enzyme defect and the severity of defects, common
Symptoms are the following types: 1,21 - hydroxylase deficiency (ZD-OHD) Congenital adrenal hyperplasia is the most common type, accounting for 90% of typical cases -95%, 21 - hydroxy enzyme gene was located on the short arm of chromosome 6 (6p21.3), closely linked with the HLA gene family, by the A gene (CYP21A) and the B gene (CYP21B) constitute the two loci, CYP21B, also known CYP2, is 21 - hydroxylase gene: CIP21A known CYP21p, is non-functional pseudogenes. CYP2 gene mutations, including point mutations, defects and gene conversion, resulting in 21 - hydroxylase partial or complete lack of, due to inadequate secretion of cortisol synthesis, synthetic androgen excess, leading to varying severity of clinical symptoms appear. Can be expressed as simple virilizing typical loss of salt type, atypical type of three types. (1) simple virilizing (SV), Department of 21 - hydroxylase is not a complete lack of due enzyme deficiency was moderate, 11 - deoxy-cortisol and cortisol, 11 - deoxy corticosterone, which are not
Pathogenesis to the normal synthesis, the precursor of 17 - hydroxyprogesterone, progesterone, DHEA dehydroepiandrosterone more different, but still a small amount of cortisol and aldosterone synthesis, so the main clinical symptoms without loss of performance of salt increased the symptoms of androgen signs. Two girls showed off deformity: As the synthesis of steroid hormones in the fetal defects that exist, so the girl at birth showed varying degrees of masculine signs, such as clitoral hypertrophy, similar to the male hypospadias, boys like the labia majora scrotum, but no testicles, labia, or have different degrees of integration. Although the external genital hermaphroditism, but still within the female genital type, have ovaries, fallopian tubes, uterus, can occur in children age 2-3 pubic hair, armpit hair, in adolescence, the lack of female sexuality, no breast development and menstrual cramps. Boys showed pseudo precocious puberty, birth asymptomatic 4 months after birth, after the signs of precocious puberty occurs, usually after 1-2 significantly increased the external genitalia, scrotum increased, but testicular size and age appropriate. May be the early stage pubic hair, armpit hair, beard, acne, Adam's apple, deep voice and muscular. Win boys and girls over there physical development block, bone age exceeded the age, due to early epiphyseal fusion, the ultimate short stature, due to increased ACTH may have mucocutaneous pigmentation. (2) loss of salt type (SW) is a 21 - hydroxylase due to a complete lack of cortisol precursors such as progesterone, 17 - hydroxyprogesterone and other secretion increased, but decreased aldosterone synthesis, the distal row too much sodium, potassium row is too small, because the masculine addition to the performance of children, but shortly after birth can have a poor feeding, vomiting, diarrhea, body weight increased or decreased, dehydration, hyponatremia, hyperkalemia, metabolic acidosis. If not treated early, can be due to circulatory failure and death. Children have been born female hermaphroditism, easy really tons, men are more difficult to diagnose in children, often misdiagnosed as pyloric stenosis surgery or misdiagnosis delayed treatment for the infant diarrhea. (3) atypical type (NC) also known as delayed, occult or light, is due to 21 - hydroxylase due to a slight lack of. Different clinical manifestations of the disease, age of onset varies. Only in childhood or adolescence appear masculine performance. Boys pubic hair, precocious puberty, growth acceleration, advanced bone age; women can occur in children with delayed menarche, primary amenorrhea, hirsutism and infertility and so on. 2,11 -hydroxylase deficiency (11 -OHD) representing approximately 5% -8% disease, this enzyme deficiency, androgen and 11 - deoxy-corticosterone were increased, and the clinical manifestations of the 21 - hydroxylase lack of
Congenital adrenal hyperplasia symptoms similar to masculine, but to a lesser extent, may have high blood pressure and sodium retention, the majority of children with moderately elevated blood pressure, which is characterized by giving corticosteroids can decrease blood pressure, and blood pressure after withdrawal rose again. 3,3 -hydroxysteroid dehydrogenase deficiency (3 -HSD) of this type are rare, the enzyme deficiency, aldosterone, cortisol, testosterone synthesis are blocked, the boy appeared pseudohermaphroditism, such as poor penile development, hypospadias crack. Light when the girl was born masculine phenomenon. Because aldosterone secretion is low, which occurred in the neonatal loss of salt, dehydration, serious condition. 4,17 - hydroxylase deficiency (17-OHD) of this type are rare, due to blocked synthesis of cortisol and sex hormones, and 11 - deoxy corticosterone secretion, hypokalemic alkalosis and hypertension clinic there, due to the lack of sex hormones, girls can have infantile sexuality, primary amenorrhea, boys showed male pseudohermaphroditism, feminine external genitalia, with breast development, but the children had testicles. Cause disease by the adrenal cortex zona, ZF, mesh generation of the composition. Spherical zone is located in the outermost cortex about 5% to 10%, which is stimulated mineralocorticoid
Factors related images - the only of aldosterone, fascicular zone in the middle layer, is the largest leather belt, about 75%, which is a small amount of cortisol and the mineralocorticoid (deoxy corticosterone, deoxy cortisol, corticosterone) and synthetic spaces , mesh belt is located in the innermost, the main adrenal androgen and a small amount of synthetic estrogen. Normal adrenal cholesterol synthesis by glucocorticoid, mineralocorticoid hormones, sex hormones (male, estrogen and progesterone) three main types of hormones are cholesterol derivatives. The process is extremely complicated, each step of the comparison after a series of enzyme catalysis, synthesis of some enzymes is one of the hormones or hormone in the course of two types of common needs. Adrenal synthesis of cortisol secretion in the pituitary under the control of ACTH, congenital adrenal hyperplasia, due to the synthesis of these hormones in different parts of the enzyme defect resulting in glucocorticoid, mineralocorticoid cortisol levels in the blood, elimination of negative feedback, resulting in an increase in anterior pituitary ACTH secretion and stimulate the adrenal hyperplasia quality, and to the middle of androgen metabolites, and some increase in aldosterone synthesis and secretion of the long type of CAH has also see the flaws are: 21 - hydroxylase (CYP21), 11 -hydroxylase (CYP17), 3 -hydroxysteroid alcohol dehydrogenase (3 -HSD-free), 18 - hydroxylase (CYP11B2) and so on. Diagnostic tests (a) Biochemical detection of 1, urine 17 - hydroxysteroid (17-OHCS), 17 - ketone steroids (17-KS) and estriol determination of pregnancy, including 17-KS is a reflection of the secretion of adrenal cortex an important indicator of androgen , the diagnosis of this disease better than the 17-OHCS. Adrenal hyperplasia was significantly higher in patients with 17-KS.
Automatic biochemical detector 2, blood 17 - hydroxyprogesterone (17-OHP), renin angiotensinogen (PRA), aldosterone (Aldo), dehydroepiandrosterone different androsterone (DHEA), deoxy corticosterone (DOC) and testosterone (T) ,17-OHP determination of such value increase is based on 21 - hydroxylase deficiency of the specific indicators, it can also be used to detect drug dose and efficacy. 3, determination of serum electrolytes: sodium loss of salt type may have high A blood. (B) other tests 1, chromosome severely deformed external genitalia, the karyotype analysis can do to identify gender. 2, X-ray film sheets supporting bone left wrist anteroposterior films, to determine bone age, bone age in patients over age. 3, B ultrasound or CT examination showed bilateral adrenal gland can be increased. 4, gene diagnosis by direct polymerase chain reaction, oligonucleotide hybridization, restriction fragment length polymorphism and sequence analysis of genetic information can be found in gene mutation or deletion. Treatment 1, the early application of hydrocortisone or prednisone. 2, should insist on lifelong medication. 3, surgical treatment. The purpose of the treatment of this disease: to correct the lack of adrenal cortical hormone to maintain normal physiological metabolism, inhibited masculine, promote normal growth and development. 1, in time to correct water and electrolyte imbalance (loss of salt type for children) are available normal saline intravenous infusion, metabolic acidosis is a 0.45% sodium chloride and sodium bicarbonate solution. Avoid using potassium solution. Severe loss of salt type required intravenous hydrocortisone 25-100mg, if not easy to correct hyponatremia and dehydration, can be injected deoxy corticosterone acetate (DOCA) 1-3mg / d or oral cortisone 0.05-0.1 hydrogen fluoride mg / d, dehydration corrected, changed to oral corticosteroids and long-term maintenance, while oral administration of sodium 2-4d / d. The volume can be adjusted according to the disease. 2, the long-term treatment (1), glucocorticoid corticosteroid treatment on the one hand can compensate for the lack of adrenal secretion of cortisol, one can inhibit the excessive release of ACTH, thereby reducing the overproduction of androgen, it can improve the masculine, sexual early symptoms to ensure that children with normal growth and development process. General daily oral dose of hydrocortisone 10-20mg/m2, 2 / 3 volume before bedtime, 1 / 3 of the morning service. (2) mineralocorticoid mineralocorticoid may be synergistic effects of glucocorticoids, to further reduce the secretion of ACTH. Hydrogen fluoride can be oral cortisone 0.05-0.1mg / d, symptoms improved gradually reduction and withdrawal. Due to long-term use can cause high blood pressure. Hydrogen fluoride is equivalent to 1.5mg 0.1mg hydrocortisone cortisone, it should be the amount calculated on the amount of cortisol in order to avoid excessive cortisol. In the course of corticosteroid therapy, should be monitored blood 17 - OH progesterone or urinary 17 - ketone steroids, type of salt should also be monitoring the loss of potassium, sodium and chlorine. Regulating hormone dosage, children in stress situations (such as: infection, excessive fatigue, surgery, etc.) or adolescent, the dose of corticosteroids should be increased 1.5-2 times than usual. 3, surgical treatment of male children Needless to surgery. Female hermaphroditism in children 6 months -1 year-old should be part of the clitoris surgery or orthopedic surgery. Disease preventi, newborn screening using dried blood drops paper method, the ELSA, fluorescence immunoassay 17-OHP can be screened 21-OHD. 2, prenatal diagnosis (1) 21-OHD: 9-11 weeks of gestation fetal cells obtained biopsy chorionic DNA analysis of amniotic fluid testing pregnant 16-20 weeks pregnant to take calcitriol ,17-OHP, etc., as most SARS children with type 21-OHD level of 17-OHP after birth, no significant increase, and thus genetic testing is the only children suffering from this type of early diagnosis. (2) 11 -OHD: DOC principal measurement of amniotic fluid and chorionic taken for gene analysis for diagnosis. Health Tips of the disease is more common in girls, mainly for women masculine, serious addition to the above symptoms can also occur pseudohermaphroditism. Baby out
When molecular biology is the virus penis is slightly larger than normal, but after six months of the gradual emergence of symptoms of precocious puberty, between 4 and 5 years of age is more apparent. Can occur early in the pubic hair, acne skin raw, with Adam's apple, deep voice changed, muscular, physical development too fast, taller than children the same age. But if not treated in time, but has become adult short stature, but normal mental development. The disease is completely curable, mainly medication. Increase girls clitoris, surgical treatment, surgery is the most suitable age of 6 months to 1 year old. After treatment the patients should be based on age and the response after starting treatment, you need 3 to 12 months for each retime, to adjust the dosage of the drug. Disease preventi, newborn screening using dried blood drops paper method, the ELSA, fluorescence immunoassay 17-OHP can be screened 21-OHD. 2, prenatal diagnosis (1) 21-OHD: 9-11 weeks of gestation fetal cells obtained biopsy chorionic DNA analysis of amniotic fluid testing pregnant 16-20 weeks pregnant to take calcitriol ,17-OHP, etc., as most SARS children with type 21-OHD level of 17-OHP after birth, no significant increase, and thus genetic testing is the only children suffering from this type of early diagnosis. (2) 11 -OHD: DOC principal measurement of amniotic fluid and chorionic taken for gene analysis for diagnosis.


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