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Endocrine pancreatic insufficiency



Pancreatic cystofibrosis (cystic fibrosis) is a hereditary disease with a recessive type of inheritance. Frequency in a population of 2-8: 100,000 of the population. In patients with cystic fibrosis, the chlorine channel "on the apical part of the cell membrane" does not work, leading to disruption of the release of chlorine from the cell, which contributes to increased sodium ions leaving the lumen inside the cell, and after it the aqueous component of the intercellular space. The consequence is a thickening of the secrets of the endocrine glands (bronchopulmonary system, pancreas, salivary glands, sex glands). Most often, the respiratory system and pancreas are affected. The disease manifests itself in respiratory and intestinal syndromes in the first year of life. The degree of interest of systems and organs is different. Paraclinic 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 is a diffuse densification of the parenchyma, and with age, a decrease in the size of the pancreas.

High sweat chlorides (60.0 and more mmol / l), 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.

Schwachman-Diamond syndrome (congenital pancreatic hypoplasia, combined with neutropenia, low growth and bone anomaly).

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

Chronic pancreatitis with exocrine insufficiency - the causes can be past diseases, including mumps, injuries, medications, toxins, diseases and anomalies of the bile and pancreatic ducts, systemic diseases.

Specific enzyme defects (lipases, trypsinogen).

1.
Isolated pancreatic lipase deficiency (Shedon-Rey syndrome) is manifested by fatty diarrhea due to neutral fats. Children have a good appetite, develop adequately in physical and neuropsychic terms.

Diagnosis: based on stool, neutral stool fat, normal sugar curve, d-xylose test, a sharp decrease or absence of lipase in the pancreatic juice, the 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; the type of inheritance is autosomal recessive.

Clinically manifests itself shortly after birth by a mushy or watery fetid stool against the background of natural feeding, poor development and increasing hypotrophy. In feces, a large amount of protein and fat is found.

Treatment: replacement therapy.

3. Amylase deficiency - diagnosis based on clinical manifestations, watery, acidic stool. Coprogram (starch). Decreased or lack of amylase activity. Effect on the background of elimination (starch-free) 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, serious damage to the pancreas occurs. With a loss of exocrine pancreatic function, a violation
  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 carried out by hormones. Cholecystokinin stimulates the secretion of enzymes, and secretin, above all, the secretion of bicarbonates. There is also regulation of secretion through the vagus nerve. Total pancreatic secretion
  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, resulting in the activation of protein kinase A, which enhances the secretion of pancreatic juice rich in bicarbonates. Other agonists (cholecystokinin, acetylcholine, gastrinrilizing peptide, substance P) act through specific
  4. Characterization of endocrine glands in children, effects of their hormones on target organs and cells, semiotics of endocrine gland insufficiency or redundancy.
    Characterization of endocrine glands in children, effects of their hormones on target organs and cells, semiotics of endocrine deficiency or redundancy
  5. Diseases of the endocrine glands. Diseases of the endocrine pancreas. Diabetes. Thyroid disease. Thyroid tumors
    1. Etiological factors of diabetes 1. intoxication 2. tobacco smoking 3. helminth infections 4. viral infections 5. genetic predisposition 2. Pathogenetic mechanisms of the development of acute pancreatitis 1. duct dyskinesia 4. mushroom poisoning 2. biliary reflux 5. alcohol poisoning 3. gastroduodenal reflux 6. overeating 3. Match 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 infertility
    Endocrine infertility 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 - ovaries, as well as diseases of the endocrine organs: the central ones located in the brain - the pituitary, hypothalamus, and peripheral - the thyroid gland, adrenal glands, etc. Endocrine disorders
  8. ENDOCRINE PART OF THE Pancreas
    The pancreas consists of exocrine and endocrine parts. The endocrine part is represented by groups of epithelial cells (islets of Langerhans), separated from the exocrine part of the gland by thin connective tissue layers. Most of the islets are concentrated in the tail region of the pancreas. The sizes of pancreatic islets vary between 0.1–0.3 mm, and their total mass
  9. DISEASES OF THE ENDOCRINE SYSTEM
    The endocrine system is a combination of organs, cell populations and individual cells that produce hormones. Hormones (in the broad sense) are called any biologically active substances of unfermented origin, secreted into the extracellular environment. The structure of the endocrine system includes three main components: central regulatory formations (hypothalamus, pineal gland, pituitary gland),
  10. ENDOCRINE RAW MATERIAL
    Veterinary and sanitary requirements for the collection, primary processing and preservation of endocrine raw materials. Preparations from organs, tissues and glands obtained from slaughtered animals are called organ preparations. Raw materials for their development are divided into three groups: endocrine, enzymatic 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 cell groups throughout the body through substances called hormones. Hormones are secreted 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 neoplasias.
    This is a group of hereditary diseases characterized by tumors and / or endocrine cell hyperplasia 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
    violation of growth (gigantism and nanism) and body weight (obesity, exhaustion), distribution of the subcutaneous fat layer. Palpation of the thyroid gland. Genital organs: secondary sexual characteristics (severity, formula). The state of the endocrine systems can be judged on the basis of already conducted studies of the skin, subcutaneous tissue and somatometric measurements. With hormonal disorders, they can
  14. ENDOCRINE INFERTILITY IN WOMEN
    One of the most complex and pressing problems of gynecology to this day continues to be the problem of barren marriage. According to epidemiological studies, the frequency of infertility as a whole in different countries of the world and on the territory of the Russian Federation ranges from QJ 8 to 18%. Currently, not only the structure of barren marriage has been studied, but also the frequency and clinical significance of various
  15. Pathology of the endocrine system
    The pathology of the endocrine system can have a significant impact on the choice of anesthetic and special safety measures for the patient during surgery and in the postoperative period. Pathology of the adrenal glands. Ensuring the safety of patients operated on against the background of acute and chronic adrenal cortex insufficiency, the primary value, is of greatest importance to the anesthesiologist
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