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DISORDER OF CARBOHYDRATE METABOLISM Ph.D., MD, Assistant Professor Hanna Saturska

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Title: DISORDER OF CARBOHYDRATE METABOLISM Ph.D., MD, Assistant Professor Hanna Saturska


1
DISORDER OFCARBOHYDRATE METABOLISM Ph.D., MD,
Assistant Professor Hanna Saturska
2
  • Diabetes mellitus
  • Introduction
  • Major index which describes metabolism of
    carbohydrates, is a sugar level in blood.
  • In healthy people it is
  • 4,4-6,6 mmol/l.

3
  • This value is summary result of complicated
    interaction of many exogenous and endogenous
    influences.
  • 1. The first it reflects a balance between

amount of glucose which is utilized by cells
amount of glucose which entrance in blood
4
  • 2. The second, glucose level in blood reflects
    an effect of simultaneous regulatory influence on
    carbohydrates metabolism of the nervous system
    and endocrine glands

adrenal cortex (adrenalin, noradrenalin) layer
pituitary gland (somatotropic thyreotropic
adrenocorticotropic hormones)
pancreas (insulin, glucagone, somatostatin)
thyroid (thyroxin, triiodthyronine)
5
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6
  • Among enumerated hormones only insulin lowers
    glucose concentration in blood the rest of
    hormones increase it .
  • The glucose concentration in blood describes
    carbohydrates metabolism both of healthy man and
    sick.
  • Illnesses base of which is disorder of
    carbohydrates metabolism can flow with rise of
    glucose concentration in blood and with lowering
    of it.

7
  • Rise of glucose concentration is named
    hyperglicemia lowering hypoglicemia.
  • For example, hyperglicemia is very typical for
    diabetes mellitus, hypoglycemia for
    glycogenosis.

hypoglicemia
hyperglicemia
diabetes mellitus
glycogenosis
8
Diabetes mellitus
  • Diabetes mellitus, often simply referred to as
    diabetesis a group of metabolic diseases in
    which a person has high blood sugar,
  • either because the body does not produce enough
    insulin,
  • or because cells do not respond to the insulin
    that is produced.

9
  • This high blood sugar produces the classical
    symptoms

polyuria (frequent urination),
  • polydipsia (increased thirst)
  • polyphagia (increased hunger).

10
There are three main types of diabetes
  • Type 1 diabetes results from the body's failure
    to produce insulin, and presently requires the
    person to inject insulin.
  • (Also referred to as insulin-dependent diabetes
    mellitus,
  • IDDM for short, and juvenile diabetes.)

11
  • Type 2 diabetes results from insulin resistance,
    a condition in which cells fail to use insulin
    properly, sometimes combined with an absolute
    insulin deficiency. (Formerly referred to as
    non-insulin-dependent diabetes mellitus, NIDDM
    for short, and adult-onset diabetes.)

12
  • Gestational diabetes is when pregnant women, who
    have never had diabetes before, have a high blood
    glucose level during pregnancy. It may precede
    development
  • of type 2 DM.

13
  • Other forms of diabetes mellitus include
  • congenital diabetes, which is due to genetic
    defects of insulin secretion,
  • cystic fibrosis-related diabetes, steroid
    diabetes induced by high doses of
    glucocorticoids,
  • several forms of monogenic diabetes.

14
  • As of 2000 at least 171 million people worldwide
    suffer from diabetes, or 2.8 of the population.
  • Type 2 diabetes is by far the most common,
    affecting 90 to 95 of the diabetes population

15
Definition
  • The word diabetes is from the Greek diabanein
    which means to pass through, in reference to the
    excessive urine produced as a symptom of these
    diseases. The term diabetes, without
    qualification, usually refers to diabetes
    mellitus, which roughly translates to excessive
    sweet urine (known as "glycosuria"). Several rare
    conditions are also named diabetes. The most
    common of these is diabetes insipidus in which
    large amounts of urine are produced (polyuria),
    which is not sweet (insipidus meaning "without
    taste" in Latin).

16
Feature Type 1 diabetes Type 2 diabetes
Onset Sudden Gradual
Age at onset Any age(mostly young) Mostly in adults
Body habitus Thin or normal Often obese
Ketoacidosis Common Rare
Autoantibodies Usually present Absent
Endogenous insulin Low or absent Normal, decreasedor increased
Concordancein identical twins 50 90
Prevalence Less prevalent More prevalent- 90 to 95 ofU.S. diabetics
17
Type 1
  • Type 1 diabetes mellitus is characterized by loss
    of the insulin-producing beta cells of the islets
    of Langerhans in the pancreas leading to insulin
    deficiency.

18
  • In 1869, while looking down the microscope, the
    Berlin physician Paul Langerhans discovered small
    islets of cells scattered throughout the
    pancreas. These cells are responsible for the
    production of insulin.

19

Somato- statin
glukagon
insulin
20
  • Digestive enzymes include trypsin, chymotrypsin,
    pancreatic lipase, and pancreatic amylase, and
    are produced and secreted by acinar cells of the
    exocrine pancreas.

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22
  • This type of diabetes can be further classified
    as immune-mediated or idiopathic. The majority of
    type 1 diabetes is of the immune-mediated nature,
    where beta cell loss is a T-cell mediated
    autoimmune attack. There is no known preventive
    measure against type 1 diabetes, which causes
    approximately 10 of diabetes mellitus cases in
    North America and Europe. Most affected people
    are otherwise healthy and of a healthy weight
    when onset occurs. Sensitivity and responsiveness
    to insulin are usually normal, especially in the
    early stages. Type 1 diabetes can affect children
    or adults but was traditionally termed "juvenile
    diabetes" because it represents a majority of the
    diabetes cases in children

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24
Type 2
  • Type 2 diabetes mellitus is characterized by
    insulin resistance which may be combined with
    relatively reduced insulin secretion. The
    defective responsiveness of body tissues to
    insulin is believed to involve the insulin
    receptor. However, the specific defects are not
    known. Diabetes mellitus due to a known defect
    are classified separately. Type 2 diabetes is the
    most common type.
  • In the early stage of type 2 diabetes, the
    predominant abnormality is reduced insulin
    sensitivity. At this stage hyperglycemia can be
    reversed by a variety of measures and medications
    that improve insulin sensitivity or reduce
    glucose production by the liver.

25
Diagnosis
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27
Gestational diabetes
  • Gestational diabetes mellitus (GDM) resembles
    type 2 diabetes in several respects, involving a
    combination of relatively inadequate insulin
    secretion and responsiveness. It occurs in about
    25 of all pregnancies and may improve or
    disappear after delivery. Gestational diabetes is
    fully treatable but requires careful medical
    supervision throughout the pregnancy. About
    2050 of affected women develop type 2 diabetes
    later in life.

28
  • When a woman has high blood sugar only while she
    is pregnant, it is a special type called
    Gestational Diabetes. Usually the blood sugar is
    kept in the normal range by insulin made by the
    body. Most of the time, pregnant women make more
    insulin to lower the blood sugar.
  • However, some women cannot do this, and these are
    the women who
  • develop gestational diabetes. This usually
    occurs in the second half of pregnancy.

29
The woman most likely to develop gestational
diabetes has had
  • Gestational diabetes before
  • a baby that weighed 10 pounds or more
  • a history of diabetes in her family
  • high blood sugar while using birth control pills
  • a stillborn baby
  • is very overweight

30
  • Even though it may be transient, untreated
    gestational diabetes can damage the health of the
    fetus or mother. Risks to the baby include
    macrosomia (high birth weight), congenital
    cardiac and central nervous system anomalies, and
    skeletal muscle malformations. Increased fetal
    insulin may inhibit fetal surfactant production
    and cause respiratory distress syndrome.
    Hyperbilirubinemia may result from red blood cell
    destruction. In severe cases, perinatal death may
    occur, most commonly as a result of poor
    placental perfusion due to vascular impairment.
    Labor induction may be indicated with decreased
    placental function. A cesarean section may be
    performed if there is marked fetal distress or an
    increased risk of injury associated with
    macrosomia, such as shoulder dystocia.

31
Complications
  • Low blood glucose or illness in the newborn
  • Increased incidence of newborn deaths
  • Development of diabetes later in life

32
Symptoms
  • Increased thirst
  • Increased urination
  • Weight loss in spite of increased appetite
  • Fatigue
  • Nausea and vomiting
  • Frequent infections including those of the
    bladder, vagina, and skin
  • Blurred vision
  • Note Usually there are no symptoms.

33
Signs and tests
  • An oral glucose tolerance test between the 24th
    and 28th weeks of pregnancy is the main test for
    gestational diabetes.

34
Principles of a meal plan
  • a very small breakfast with little carbohydrate,
    such as 8 oz milk with one slice of toast and an
    egg or peanut butter for protein
  • 3 small meals and 2-3 small snacks (smaller meals
    cause lower blood sugars)
  • avoidance of concentrated sweets and sugars,
    including fruit juice
  • plenty of non-starchy vegetables
  • low-fat protein foods such as chicken, tuna, or
    lean meat
  • lower-fat food choices in general, to keep weight
    gain at a healthy level
  • low-fat or non-fat milk, yogurt or cheese for
    adequate calcium

35
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36
  • Avoid Sugar and Concentrated Sweets
  • No cookies, cakes, pies, soft drinks, chocolate,
    table sugar, fruits juices, fruit drinks,
    Kool-Aid, Hi-C, nectars, jams, jellies, syrup,
    honey
  • Read labels avoid foods containing sucrose,
    fructose, corn syrup, dextrose, honey, molasses,
    natural sweeteners, cornstarch, concentrated
    fruit juices

37
  • Avoid Convenience Foods
  • No instant noodles, canned soups, instant
    potatoes, frozen meals or packaged stuffing

38
  • Eat Small Frequent Meals
  • Eat about every 3 hours
  • Include a good source of protein at every meal
    and snack. High-protein foods are low-fat meat,
    chicken, fish, low-fat cheese, nuts, peanut
    butter, cottage cheese, eggs, turkey

39
  • Eat a very small breakfast
  • No more than 1 starch/bread exchange
  • No fruit or fruit juice
  • Eat a protein

40
  • Choose High-Fiber Foods
  • Whole-grain breads and cereals
  • Fresh and frozen vegetables
  • Beans and legumes
  • Fresh fruits (except at breakfast)

41
  • Lower Fat Intake
  • Buy lean protein foods chicken, beef, turkey,
    ham, and fish. Limit lunch meat, bacon, sausage,
    scrapple, and hot dogs
  • Remove all visible fat
  • remove skin from poultry
  • trim fat from meat
  • Bake, broil, steam, boil or barbecue foods (no
    frying)
  • Use nonstick pan, vegetable oil spray or small
    amounts (1 to 2 teaspoons) of oil for cooking.
  • Use skim or low-fat (1) milk and dairy products.
  • Eat boiled or canned legumes (not refried)
  • Reduce added fat in the diet, such as butter,
    margarine, sour cream, mayonnaise, nuts,
    avocados, cream, cream cheese or salad dressings.

42
Other types
  • Following is a comprehensive list of other causes
    of diabetes
  • Genetic defects of ß-cell Function
  • Maturity onset diabetes of the young (MODY)
  • Mitochondrial DNA mutations

43
  • Genetic defects in insulin processing or insulin
    action
  • Defects in proinsulin conversion
  • Insulin gene mutations
  • Insulin receptor mutations

44
  • Exocrine Pancreatic Defects
  • Chronic pancreatitis
  • Pancreatectomy
  • Pancreatic neoplasia
  • Cystic fibrosis
  • Hemochromatosis

45
Endocrinopathies

  • Growth hormone excess (acromegaly)
  • Cushing syndrome
  • Hyperthyroidism
  • Pheochromocytoma
  • Glucagonoma

46
Infections
  • Cytomegalovirus infection
  • Coxsackievirus B

47
Drugs
  • Glucocorticoids
  • Thyroid hormone
  • ß-adrenergic agonists

48
Signs and symptoms
  • The classical symptoms of diabetes are polyuria
    (frequent urination), polydipsia (increased
    thirst) and polyphagia (increased hunger).14
    Symptoms may develop rapidly (weeks or months) in
    type 1 diabetes while in type 2 diabetes they
    usually develop much more slowly and may be
    subtle or absent.

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  • Prolonged high blood glucose causes glucose
    absorption, which leads to changes in the shape
    of the lenses of the eyes, resulting in vision
    changes sustained sensible glucose control
    usually returns the lens to its original shape.
    Blurred vision is a common complaint leading to a
    diabetes diagnosis type 1 should always be
    suspected in cases of rapid vision change,
    whereas with type 2 change is generally more
    gradual, but should still be suspected.

51
  • People (usually with type 1 diabetes) may also
    present with diabetic ketoacidosis, a state of
    metabolic dysregulation characterized by the
    smell of acetone a rapid, deep breathing known
    as Kussmaul breathing nausea vomiting and
    abdominal pain and an altered states of
    consciousness.

52
  • A rarer but equally severe possibility is
    hyperosmolar nonketotic state, which is more
    common in type 2 diabetes and is mainly the
    result of dehydration. Often, the patient has
    been drinking extreme amounts of sugar-containing
    drinks, leading to a vicious circle in regard to
    the water loss.
  • A number of skin rashes can occur in diabetes
    that are collectively known as diabetic
    dermadromes.

53
Causes
  • The cause of diabetes depends on the type. Type 2
    diabetes is due primarily to lifestyle factors
    and genetics.

54
Causes
  • Type 1 diabetes is also partly inherited and then
    triggered by certain infections, with some
    evidence pointing at Coxsackie B4 virus. There is
    a genetic element in individual susceptibility to
    some of these triggers which has been traced to
    particular HLA genotypes (i.e., the genetic
    "self" identifiers relied upon by the immune
    system). However, even in those who have
    inherited the susceptibility, type 1 diabetes
    mellitus seems to require an environmental
    trigger.

55
Pathophysiology
  • Insulin is the principal hormone that regulates
    uptake of glucose from the blood into most cells
    (primarily muscle and fat cells, but not central
    nervous system cells). Therefore deficiency of
    insulin or the insensitivity of its receptors
    plays a central role in all forms of diabetes
    mellitus.

56
  • Humans are capable of digesting some
    carbohydrates, in particular those most common in
    food starch, and some disaccharides such as
    sucrose, are converted within a few hours to
    simpler forms most notably the monosaccharide
    glucose, the principal carbohydrate energy source
    used by the body. The rest are passed on for
    processing by gut flora largely in the colon.
    Insulin is released into the blood by beta cells
    (ß-cells), found in the Islets of Langerhans in
    the pancreas, in response to rising levels of
    blood glucose, typically after eating. Insulin is
    used by about two-thirds of the body's cells to
    absorb glucose from the blood for use as fuel,
    for conversion to other needed molecules, or for
    storage.

57
  • Insulin is also the principal control signal for
    conversion of glucose to glycogen for internal
    storage in liver and muscle cells. Lowered
    glucose levels result both in the reduced release
    of insulin from the beta cells and in the reverse
    conversion of glycogen to glucose when glucose
    levels fall. This is mainly controlled by the
    hormone glucagon which acts in the opposite
    manner to insulin. Glucose thus forcibly produced
    from internal liver cell stores (as glycogen)
    re-enters the bloodstream muscle cells lack the
    necessary export mechanism. Normally liver cells
    do this when the level of insulin is low (which
    normally correlates with low levels of blood
    glucose).

58
  • Higher insulin levels increase some anabolic
    ("building up") processes such as cell growth and
    duplication, protein synthesis, and fat storage.
    Insulin (or its lack) is the principal signal in
    converting many of the bidirectional processes of
    metabolism from a catabolic to an anabolic
    direction, and vice versa. In particular, a low
    insulin level is the trigger for entering or
    leaving ketosis (the fat burning metabolic
    phase).

59
  • If the amount of insulin available is
    insufficient, if cells respond poorly to the
    effects of insulin (insulin insensitivity or
    resistance), or if the insulin itself is
    defective, then glucose will not have its usual
    effect so that glucose will not be absorbed
    properly by those body cells that require it nor
    will it be stored appropriately in the liver and
    muscles. The net effect is persistent high levels
    of blood glucose, poor protein synthesis, and
    other metabolic derangements, such as acidosis.

60
  • When the glucose concentration in the blood is
    raised beyond its renal threshold (about
    10 mmol/L, although this may be altered in
    certain conditions, such as pregnancy),
    reabsorption of glucose in the proximal renal
    tubuli is incomplete, and part of the glucose
    remains in the urine (glycosuria). This increases
    the osmotic pressure of the urine and inhibits
    reabsorption of water by the kidney, resulting in
    increased urine production (polyuria) and
    increased fluid loss. Lost blood volume will be
    replaced osmotically from water held in body
    cells and other body compartments, causing
    dehydration and increased thirst.

61
Diagnosis
  • Diabetes mellitus is characterized by recurrent
    or persistent hyperglycemia, and is diagnosed by
    demonstrating any one of the following
  • Fasting plasma glucose level  7.0 mmol/L
    (126 mg/dL).
  • Plasma glucose  11.1 mmol/L (200 mg/dL) two
    hours after a 75 g oral glucose load as in a
    glucose tolerance test.
  • Symptoms of hyperglycemia and casual plasma
    glucose  11.1 mmol/L (200 mg/dL).
  • Glycated hemoglobin (Hb A1C)  6.5
  • Glycosylated hemoglobin and Glucose tolerance
    test

62
Management
  • Diabetes mellitus is a chronic disease which is
    difficult to cure. Management concentrates on
    keeping blood sugar levels as close to normal
    ("euglycemia") as possible without presenting
    undue patient danger. This can usually be with
    close dietary management, exercise, and use of
    appropriate medications (insulin only in the case
    of type 1 diabetes mellitus. Oral medications may
    be used in the case of type 2 diabetes, as well
    as insulin).

63
  • Patient education, understanding, and
    participation is vital since the complications of
    diabetes are far less common and less severe in
    people who have well-managed blood sugar levels
  • Wider health problems may accelerate the
    deleterious effects of diabetes. These include
    smoking, elevated cholesterol levels, obesity,
    high blood pressure, and lack of regular exercise.

64
Complications
  • Diabetes doubles the risk of vascular problems,
    including cardiovascular disease.
  • Glycated hemoglobin is better than fasting
    glucose for determining risks of cardiovascular
    disease and death from any cause
  • The complications of diabetes mellitus are far
    less common and less severe in people who have
    well-controlled blood sugar levels.
  • Wider health problems accelerate the deleterious
    effects of diabetes. These include smoking,
    elevated cholesterol levels, obesity, high blood
    pressure, and lack of regular exercise.

65
Complications of diabetes mellitus
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67
AcuteComplications of diabetes mellitus
  • Diabetic ketoacidosis
  • Hyperglycemia hyperosmolar state
  • Hypoglycemia
  • Diabetic coma
  • Respiratory infections

68
Chronic Complications of diabetes mellitus
  • Angiopathy Chronic elevation of blood glucose
    level leads to damage of blood vessels
  • The endothelial cells lining the blood vessels
    take in more glucose than normal, since they do
    not depend on insulin.

69
Fundus photo showing scatter laser surgery for
diabetic retinopathy
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  • Symptoms of diabetes mellitus
  • Hyperglycemia is connected, foremost with
    lowering of glucose utilization by muscular and
    fatty tissues. Lowering of glucose utilization
    has membranogenic nature. In case of
    insulinopenia and in case of insulin-resistance
    nteraction of insulin and receptor is damaged.
    Therefore protein-transporters of glucose are not
    included in membranes of cells-targets. This
    limits glucose penetration in cells. It is use on
    power needs (in myocytes) diminishes. Lypogenesis
    is slowed-glucose deposit in fats form (in
    lypocytes). Glycogenesis slows- synthesis of
    glycogene (in hepatocytes and myocytes). On other
    hand, attached to diabetes a supplementary amount
    of glucose is secreted in blood. In liver and
    muscles of diabetics glycogenolysis is a very
    active.

72
  • Glucosuria. In healthy man practically has not
    glucose in urine. It is excreated in amount not
    more 1 g. Attached to sugar diabetes amount of
    exreted glucose increases repeatedly. It is
    explainet by next way. If glucose concentration
    in blood and primary urine does not exceed 9
    mmol/l, epithelium of canaliculi reabsorbed it.
    This maximum concentration is called nephritic
    threshold. If a glucose concentration exceeds a
    nephritic threshold (9 mmol/l), part of glucose
    goes in secondary urine (glucosuria).
  • Polyuria. Glucose is osmotic active substance.
    Increasing of its concentration in primary urine
    raises osmotic pressure. Water is exuded from
    organism together with glucose (osmotic
    diuresis). Patient excretes 3-4 l of urine per
    day, sometimes till 10 l.

73
  • Macroangiopathy. Macroangiopathy is vessels
    atherosclerosis of cerebrum, heart, kidneys,
    legs. Diabetes lead to atherosclerosis
    development. There are three acceleration way of
    atherogenesis in patients with diabetes. In
    conditions of insulin insufficiency growth
    hormone synthesis increases. Here upon
    proliferation of smooth myocites accelerates key
    stage of atherogenesis. Attached to diabetes
    vessels endothelium damages. Synthesis of
    thromboxane increase, and this helps to adhesion
    of thrombocytes. Thrombocytes excret mitogene
    (thrombocytic growth factor). It also stimulates
    proliferation of smooth myocytes. Attached to
    diabetes concentration of lipoproteids low
    density, increase concentration of lipoproteids
    of high density.

74
  • Microangipathy develop in shallow vessels
    arterials, venues, capillaries. Two process form
    their pathogenic base thicking of basal
    membrane and
    reproduction endothelium. Direct cause of
    microangiopathy is hyperglycemia and synthesis of
    glycoproteids in basal membrane. There are two
    main clinical forms microangipathy diabetic
    retinopathy and diabetic nephropathy.
  • Neuropathy manifest by disorder of nerves
    function sensible, motor, vegetative. Essence of
    these decreases is demyelinisation of nervous
    fibres, decrease of axoplasmatic flow.

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  • Galactosemia
  • This is hereditary illness. In its base lies an
    blockade of galactose metabolism. In organism
    intermediate metabolits accumulate. There are two
    the main forms of galactosemia on base of
    transferase insufficiency and on base of
    galactokinase insufficiency.
  • Deficit of glucose-1-phosphaturidyltransferase.
    This enzyme converts galactose-1-phosphate in
    glucose-1-phosphate. Attached to its
    insufficiency galactose-1-phosphate and sugar
    alcohol of galactose (galactit) accumulates in
    tissues lens of the eye, liver, brain, buds.
    Mammal and cow milk contains lactose. Therefore
    the illness symptoms appear with first days of
    child life. Diarrhea, vomiting, dehydrotation
    occur. Liver increases (splenomegalia).
    Hepatocytes lose ability to conjugate bilirubine.
    Children become yellowish.

77
  • Affection of kidneys displays in proteinuria,
    aminoaciduria and acidosis. For galactosemia
    cataract is very typical. Their beginnings
    related to accumulation of osmotic active
    galactite in vitreous bodies of eyes. Galactite
    absorb in water, and water breaks tissues.
    Dangerous consequences arise in the brain. This
    foremost is delay of mental development. Mortal
    end is possible. Cure method is diet without
    galactose.
  • Deficit of galactokinase. Attached to this
    illness variant a process of phosphorilation of
    galactose is blocked, that is transformation of
    galactose in galactose-1-phosphat. Illness
    displays in cataracts. Other symptoms are absent
    or minor. Cure is diet without galactose.

78
  • Glycogenoses
  • Simple carbohydrates deposit in organism as
    polysaccharides. In muscles and liver accumulates
    glycogen. It consist of 4 of liver weight and 2
    of muscles weight. Muscles glycogen is used
    as of ready fuel source for immediate
    guaranteeing by energy. Liver without
    interruption provides cerebrum and erythrocytes
    with glucose .
  • Synthesis and splitting of glycogen are exactly
    adjusted and coordinated processes. Attached to
    immediate need in glucose ?cells of pancreas
    secret glucagone. It activates adenylatcyclase of
    hepatic cells. Adenilatcyclase stimulates
    derivation of cAMP. Under action of cAMP takes
    place activation of proteinkinase and this enzyme
    raises activity glycogenphosphorilase and
    oppresses activity of glucogensynthase.

79
  • In other situation after consuming of
    carbohydrates in blood accumulates surplus of
    glucose. ß-cells of pancreas multiply insulin
    synthesis. Insulin raises activity of
    glycogensyntase. Active glucogenesis starts too.
    Surplus of glucose reserves in appearance of
    glucogen in liver and muscles.
  • There are illnesses in base of which is
    accumulation of glycogen in organs. They are
    called glycogenoses. All of them are hereditary
    enzymopathy. There are seven main types of
    glycogenoses.

80
  • Glycogenosis type I Girkes disease. Girkes
    disease cause deficit of glucose-6-phosphatase.
    This enzyme provides 90 of glucose which
    disengages in liver from glycogen. It play
    central role in normal glucose homeostasis.
    Glucose which disengages attached to
    disintegration of glycogen or is derivated in
    process of gluconeogenesis obligatory goes over
    stage of glucose-6-phosphate. Enzyme
    glucose-6-phosphatase tears away a phosphate
    group from glucose. There free glucose is formed
    it goes out in blood. Attached to Girkes
    disease stage of tearing phosphate group is
    blocked. There are no free glucose hypoglycemia
    occur. Hypoglycemia arises. Attached to Girkes
    disease glycogen is deponed in liver and kidneys.

81
  • Type ?? glycogenosis Pompes disease. Illness
    is related to deficit of lysosomal enzyme sour
    maltase, or ?-1,4-glucosidase. This enzyme slits
    glycogene to glucose in digestive vacuoles.
    Attached to its deficit glycogen accumulates at
    first in lysosomes and then in cytosole of
    hepatocytes and myocytes.
  • Type ??? glycogenosis Coris disease, Forbs
    disease. This illness is named limitdecstrinosis.
    In its base lies a deficit of amylo-1,6-glucosida
    se. Degradation of glycogen pauses in sites of
    branching. Glycogen accumulates in liver and
    muscles. Cure is diet with big proteins
    maintenance.
  • Type ?V glycogenosis Andersons disease. It is
    called by deficit of amilo-1,4,1,6-transglucosidas
    e (branching enzyme). As result of this There is
    derivated anomalous glycogen with very long
    branches and rare points of branching. It is not
    exposed to degradation and accumulates in liver,
    heart, kidneys, spleen, lymphatic nods, skeletal
    muscles.
  •  

82
  • Type V glycogenosis McArdels disease. Its
    cause is deficit of phosphorilase of myocytes.
    Typical pain displays in muscles after physical
    loading. Glycogene does not slit only in muscles.
    Here it accumulates. In liver mobilization of
    glycogen comes normal.
  • Type V? glycogenosis Hers disease. Illness
    arises as result of insufficiency of hepatic
    phosphorilase complex. Glycogen accumulates in
    liver. Typical sign is hepatomegalia.
  • Type V?? glycogenosis. Illness essence is in
    oppression of muscle phosphofrutkinase. Symptoms
    are similar to McArdles disease.

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