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Endocrine pancreatic insufficiency



Cystic fibrosis of the pancreas (cystic fibrosis) is a hereditary disease with a recessive mode of inheritance. The frequency in the population 2-8: 100 000 population. In patients with cystic fibrosis, the chlorine channel on the apical part of the cell membrane “does not work”, resulting in impaired chlorine release from the cell, which contributes to an increased withdrawal of sodium ions from the lumen into the cell, followed by the water component of the intercellular space. The result is a thickening of secretions of external secretion glands (bronchopulmonary system, pancreas, salivary glands, sex glands). The respiratory system and pancreas are most commonly affected. The disease is manifested by respiratory and intestinal syndromes already in the first year of life. The degree of interest of systems and organs is different. Paraclinical markers are: coprogram - the presence of a large amount of neutral fat and almost always its predominance over muscle fibers and polysaccharides.

Ultrasound of the pancreas - diffuse induration of the parenchyma, and with age decrease in the size of the pancreas.

High sweat chlorides (60.0 mmol / l or more), repeatedly positive (at least 3 times).

Molecular genetic examination. But it should be noted that the negative results of this study do not exclude the diagnosis of cystic fibrosis.

Shwachman-Diamond syndrome (congenital hypoplasia of the pancreas, combined with neutropenia, short stature and bone abnormality).

Diagnosis based on pancreatic insufficiency in the first year of life, neutropenia, normal sweat chlorides.

Chronic pancreatitis with exocrine insufficiency - the causes can be postponed diseases, including epidemic parotitis, trauma, drugs, toxins, diseases and anomalies of the bile and pancreatic ducts, systemic diseases.

Specific enzyme defects (lipase, trypsinogen).

one.
An isolated deficiency of pancreatic lipase (Shedon-Rey syndrome) is manifested in fatty diarrhea due to neutral fats. Children have a good appetite, develop adequately in the physical and neuro-mental attitude.

Diagnosis: based on fatty stools, neutral fat in feces, normal sugar curve, d-xylose test, sharp decrease or absence of lipase in pancreatic juice, absence of morphological changes in the pancreas, normal sweat chlorides.

Treatment: replacement therapy.

2. Isolated trypsin deficiency occurs with a frequency of 1: 10 000; autosomal recessive mode of inheritance.

It is clinically manifested shortly after birth by a mushy or watery fetid stool on the background of natural feeding, poor development and increasing hypotrophy. A large amount of protein and fat is found in feces.

Treatment: replacement therapy.

3. Amylase deficiency - diagnosis based on clinical manifestations, watery, sour-smelling stools. Coprogram (starch). Decrease or absence of amylase activity. The effect on the background of the elimination (starchless) diet.

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Endocrine pancreatic insufficiency

  1. Exocrine pancreatic insufficiency
    The most common cause of exocrine pancreatic insufficiency is chronic pancreatitis, which is usually the result of alcoholism. The development of chronic pancreatitis is usually gradual, so early diagnosis is often difficult. Over time, the disease progresses, there is serious damage to the pancreas. When the loss of exocrine pancreatic function is impaired
  2. Pancreatic secretion
    The volume of pancreatic secretion is 1500 ml per day. It is secreted into the small intestine and contains enzymes that hydrolyze proteins, fats and starch. The main regulation of secretion is hormones. Cholecystokinin stimulates the secretion of enzymes, and secretin, above all, the secretion of bicarbonates. There is also a regulation of secretion through the vagus nerve. General secretion of the pancreas
  3. Pancreatic secretion stimulants
    VIP and secretin stimulate pancreatic secretion by activating adenylate cyclase. As in other cell types, adenylate cyclase promotes the formation of cAMP, as a result of which protein kinase A is activated, which enhances the secretion of pancreatic juice rich in bicarbonates. Other agonists (cholecystokinin, acetylcholine, gastrinrilizing peptide, substance P) act through specific
  4. Characteristics of the endocrine glands in children, the effects of their hormones on target organs and cells, semiotics of insufficiency or redundancy of the activity of the endocrine glands.
    Characteristics of endocrine glands in children, the effects of their hormones on target organs and cells, semiotics of insufficiency or redundancy of endocrine activity
  5. Diseases of the endocrine glands. Diseases of the endocrine pancreas. Diabetes. Diseases of the thyroid gland. Thyroid tumors
    1. Etiological factors of diabetes mellitus 1. intoxication 2. tobacco smoking 3. helminth infections 4. viral infections 5. genetic predisposition 2. Pathogenetic mechanisms of acute pancreatitis development 1. duct dyskinesia 4. fungal poisoning 2. biliary reflux 5. alcohol poisoning 3. gastroduodenal 3. reflux 6. overeating 3. Establish compliance by functional
  6. Endocrine function
    Four types of endocrine cells are distinguished. B-cells (P) are most numerous in the islets of Langerhans, they secrete insulin and are localized in the center of the islets. Other endocrine cells are located on the periphery of the islets around the B-cells: A-cells (a) secreting glucagon; Somatostatin-secreting D cells; F-cells (PP) producing pancreatic polypeptide. The ratio of A-, D- and
  7. Endocrine sterility
    For endocrine sterility is characterized by the absence of ovulation caused by hormonal disorders. The cause of endocrine infertility can be pathological processes in the female genital glands - the ovaries, as well as diseases of the endocrine organs: central, in the brain - pituitary, hypothalamus, and peripheral - thyroid, adrenal glands, etc. Endocrine disorders
  8. ENDOCRINE OF THE PANCIRAL GLAND
    The pancreas consists of exocrine and endocrine parts. The endocrine portion is represented by groups of epithelial cells (islets of Langerhans), separated from the exocrine portion of the gland by thin connective tissue layers. Most of the islets are concentrated in the area of ​​the tail of the pancreas. The dimensions of the pancreatic islets range from 0.1–0.3 mm, and their total mass
  9. DISEASES OF THE ENDOCRINE SYSTEM
    The endocrine system is a collection of organs, cell populations and individual cells that produce hormones. Hormones (in a broad sense) are any biologically active substances of non-fermented origin released into the extracellular environment. The structure of the endocrine system includes three main components: central regulatory structures (hypothalamus, epiphysis, pituitary gland),
  10. ENDOCRINE RAW MATERIALS
    Veterinary and sanitary requirements for the collection, primary processing and preservation of endocrine raw materials. Preparations from organs, tissues and glands derived from slaughter animals are called organ preparations. Raw materials for their production are divided into three groups: endocrine, enzyme and special. Endocrine raw materials are the pituitary, thyroid and parathyroid glands, adrenal glands, pancreas,
  11. Endocrine system
    The nervous system controls the rapidly changing processes in the body through the direct activation of muscles and glands. The endocrine system acts more slowly and indirectly affects the work of groups of cells of the whole organism through substances called hormones. Hormones are released into the bloodstream by various endocrine glands and are transferred to other parts of the body, where they have specific effects on
  12. Multiple endocrine neoplasia.
    This is a group of hereditary diseases characterized by tumors and / or hyperplasia of the endocrine cells of several organs. The development of multiple endocrine neoplasias is probably associated with mutations inherited in an autosomal dominant manner. In addition to damage to the endocrine glands, combined changes in other organs and tissues are possible (Table 35.4). Multiple type I endocrine neoplasia
  13. Endocrine system
    impaired growth (gigantism and nanism) and body weight (obesity, exhaustion), the distribution of the subcutaneous fat layer. Palpation of the thyroid gland. Genital organs: secondary sexual characteristics (severity, formula). On the state of the endocrine systems can be judged on the basis of already conducted studies of the skin, subcutaneous tissue and somatometric measurements. When hormonal disorders can
  14. ENDOCRINE INFERTILITY IN WOMEN
    One of the most difficult and pressing problems of gynecology so far continues to be the problem of barren marriage. According to epidemiological studies, the frequency of infertility in general in various countries of the world and in the territory of the Russian Federation ranges QJ 8 to 18%. Currently, not only the structure of a barren marriage has been studied, but the frequency and clinical significance of various
  15. Pathology of the endocrine system
    Pathology of the endocrine system can have a significant impact on the choice of anesthesia and special patient safety measures during surgery and in the postoperative period. Pathology of the adrenal glands. For an anesthesiologist, the safety of patients operated on the background of acute and chronic insufficiency of the adrenal cortex, primary
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