Endocrinology / / April 27, 2016
The disease was first described in 1894 Voelcker Later there were reports of similar disease caused by adrenal hemorrhage.A more complete description of the clinical picture of acute adrenal insufficiency gave Waterhouse in 1911.on the basis of the collected them from the literature 15 cases, as well as his own observations.In 1918 Friderichsen published a review of the literature on the subject.
Acute adrenocortical insufficiency occurs in both sexes with the same frequency and can occur at any age, but mainly in infants, children and young people.
Etiology The most frequent cause of the syndrome Waterhouse Friderichsen find hemorrhages in both adrenal glands.Neonates massive bleeding into the adrenal glands can occur as a result of injuries in severe prolonged labor with fetal asphyxia or forceps.The cause of bilateral adrenal hemorrhage in newborns is sometimes hereditary syphilis, and toxemia of pregnancy, especially eclampsia.
In children and adults, bleeding in the adrenal glands are infect
Pathogenesis The pathogenesis syndrome Waterhouse - Friderichsen are two processes: loss of function of the adrenal cortex and intoxication due to acute infection.
When syndrome Waterhouse - Friderichsen adrenal observed numerous small hemorrhagic necrosis of the adrenal gland or the whole is a solid mass with extensive bloody bloody clusters located retroperitoneal.Sometimes there is a gap of the capsule of the adrenal gland, reaching the peritoneum.The place of greatest congestion of hemorrhage is usually netted area.Other layers of the adrenal cortex are also involved in the process, but to a lesser degree.Often glomerular zone remains unaffected.In some cases, there are large thrombosis or vascular sinusoids adrenals.Also described are degenerative changes with focal necrosis of the adrenal cortex without hemorrhage.In acute, severe meningococcal or streptococcal infections involving vascular collapse, there are extensive hemorrhagic necrosis and degenerative changes in the adrenal glands: transformation of dense cellular bands in the beam and to a lesser extent in the zona glomerulosa into hollow tubular structure «tubular degeneration», normally filled with liquidexudate or polynuclear leukocytes, fibrin and cellular debris or remaining unfilled.Occasionally the process involves the adrenal medulla.
In the skin there are widespread destructive changes of the capillaries and arterioles due to massive bacterial emboli, or in connection with the action of toxins on the walls of blood vessels.
Approximately half of the cases in meningococcal sepsis, meningitis or meningoencephalitis note.
Quite often, there is an enlarged thymus gland and hyperplasia of lymphatic tissue.
often joins acute myocarditis.And in the rest of the organs are changes inherent in severe sepsis.
Acute adrenal insufficiency may develop suddenly in a healthy until that time, the child or adult.The disease has a fulminant course;Death can occur within 24 hours Early symptoms -. sudden nervous agitation, malaise, headache, diffuse abdominal pain, vomiting and diarrhea.If you have intense abdominal pain peritoneal irritation symptoms are often absent.The initial temperature rise can be moderated, but the disease progresses rapidly, within 1-2 hours, it reaches very high numbers.Sometimes raising chills precede temperature.Soon the symptoms of central nervous system are growing, increasing headache, neck stiffness appears mild muscle cramps are often common, the patient falls into a coma or soporous that remains to death.
At the beginning of the disease there is a characteristic cyanosis, which very quickly joins the heavy collapse and hypotension, and hypoglycemia often.On the skin there are petechiae, hemorrhages in the conjunctiva and oral mucosa.There may be a diffuse spotty rash - purple.In the terminal period developed tachycardia, pulmonary edema, decreased kidney function.The temperature can drop to subnormal levels, blood pressure is not determined.Symptoms of meningitis often occur - sometimes acute abdomen.
Some clinicians distinguish between two basic forms of acute adrenal insufficiency - with symptoms of abdominal organs and nervous phenomena.In the first form, suddenly come spilled sharp pain around the abdomen, vomiting, choleriform diarrhea, cramps, cold sweat, weak and rapid pulse, the temperature drops below the normal level.Patients die very quickly.
When the nervous form of the disease develops suddenly coma, simulates brain apoplexy, convulsions seizures, delirium, sudden death.Currently, most clinicians do not subdivides acute insufficiency of the adrenal cortex in the individual forms, as usually is mixed clinical picture of the disease - the symptoms of nervous system lesions combined with symptoms of dysfunction of the gastrointestinal tract and begin to show signs of acute infection.
in blood marked leukocytosis (up to 80,000) with a shift to the left, rarely - thrombocytopenia.Attention is drawn to the absence of Eosinopenia.In shock eosinophils different origin is usually less than 50 per 1 mm 3, whereas the number of acute adrenal insufficiency eosinophils often much higher.Blood sugar mostly sharply reduced (up to 44 mg%), residual nitrogen of blood significantly increased (up to 96.9 mg%) due to vascular collapse and disorders of protein and salt metabolism.If oliguria or anuria may occur hyperkalemia.Serum sodium and chloride levels decreased or normal, depending on the state of the kidneys, fever, vomiting, etc.
- Physiology of the adrenal cortex
- Chronic adrenal insufficiency.Addison's disease
- combination of chronic insufficiency of the adrenal cortex with other endocrine diseases sistemy.Beremennost in patients with chronic insufficiency of the adrenal cortex
- blood supply and innervation of the adrenal
- Clinical evaluation of laboratory studies of the functional state of the adrenal cortex
- Determination of corticosteroids and their metabolites in urine
- diagnosis and differential diagnosis of chronic adrenal insufficiency
- Treatment of chronic adrenal insufficiency
- Additional adrenal
- biosynthesis and metabolism of hormones of the adrenal cortex
- Acute adrenocortical insufficiency
diagnosis and differential diagnosis
diagnosis isbased on the characteristic symptoms: sudden onset, mostly in infants and children, lightning flowing sepsis caused by meningococcus, abdominal pain, high temperature, cyanosis, extensive skin lesions, kollpasa often hypoglycemia.A certain importance is the absolute number of eosinophils in the blood, which is always increased (more than 50 in 1 mm3).
In the old days this disease is always fatal.The outlook remains very serious, and at the moment, mainly because of the difficulties of timely diagnostics.
the differential diagnosis Waterhouse syndrome - Friderichsen should take into account the prevalence of symptoms of the abdominal organs and the nervous system.In the first case it is necessary to exclude acute gastrointestinal infections and severe intoxication occurring with sharp diffuse pain throughout the abdomen, vomiting, profuse diarrhea, collapse, fever.Diagnosis helps some carefully collected history, the existence of abrupt cyanosis in the initial period, skin rashes and hemorrhages on the mucous membranes of the mouth and conjunctiva of the eye.With the prevalence of symptoms of the nervous system to exclude cerebral hemorrhage and coma.In such cases it is necessary to consider first of all the acute onset of the disease, age of the patient, no history of hypertension, renal disease, diabetes mellitus.Urine and determining the amount of sugar in the blood eosinophils and provide an opportunity to make a correct diagnosis.
To determine the plasma and urine of 17 glucocorticoids have no time - so at lightning speed picking up all the symptoms.
extensive hemorrhages in the adrenal glands may simulate acute peritonitis.The character and location of pain in the absence of express abdominal wall voltage (the adrenal glands are located retroperitoneal) quickly orient the doctor to the correct diagnosis.
Treatment syndrome Waterhouse - Friderichsen
Treatment consists of measures aimed at addressing the symptoms associated with insufficiency of the adrenal cortex in the first place with the collapse and the fight against infection.
Regarding adrenal insufficiency treatment is carried out in the same manner as in the crises in patients with chronic insufficiency of the adrenal cortex.
to fight infection assigned vigorous antibiotic therapy.Forsham indicates Sulfazin high efficiency in the treatment of meningococcal sepsis.Sulfazin administered to children at a dose of 0.1 g per 1 kg of body weight for the first reception, followed by 0.03 g per 1 kg of body weight every 4 hours, adults - at the first reception - 4 g, followed by 1 g every 4 hours.