Disorders of metabolism of proteins,

lipids, carbohydrates, nucleid acid.

Impaired metabolism of water.
Impaired metabolism of calcium, iron and
copper

Crystals, concrements, pigments.

Disorders of metabolism of proteins

Dystrophy derrangement of cell metabolism
Accumulation of metabolite (low-molecular vacuolisation;
high-molecular hyaline droplets)
In some cases no accumulation of metabolite and only
discrete changes of cell
Reversible in majority of cases
Structural changes: (a) enlargement of mitochondria, (b)
vacuolar dystrophy, (c) hyalinne droplets,(d) mucous
dystrophy, (e) fibrinoid dystrophy, (f) hyalinosis, (g)
amyloidosis

 Enlargement of mitochondria: liver, kidney, myocardium,

skeletal muscle; microscopicaly enlargement of cells,
granular cytoplasm (due to enlargement of mitochondria - see
ELMI)
Vacuolar dystrophy (hydropic degeneration): accumulation of
electrolytes and water, vacuolisation of cytoplasm, oedema
Hyalinne droplets (hyaline astructural pink material in
hematoxylin-eosin staining): hepatocytes in viral hepatitis
(Councilmann bodies), renal tubuli in albuminuria,
plasmocytes in chronic inflammation (Russell bodies)
Mucous dystrophy: accumulation of mucopolysacharides
(mucoviscidosis, alopecia mucinosa, mucopolysacharidoses
Hurler, Hunter, Sanfilippo, Morqui, Maroteaux-Lamy, betaglukuronidase deficit, ganglion, myxedema, atherosclerosis,
cystic medionecrosis
Fibrinoid dystrophy/hyalinosis: colagen fibril changes (vessel
wall), reticuline fibril changes (endocardium, joints, vessels,
colagenoses, hyalinosis (polyserositis)

Mallory hyalin

Mallory hyalin

Amyloidosis
Clinical classification

Amyloid protein

Localisation

Primary
(myeloma associated)

AL (Ig light chains)

Kidney, spleen, heart, liver,

tongue

Secondary
(reactive RA, chronic
infections, IBD, tumors)

AA (amyloid associated)

Tongue, heart, liver, kidney,

spleen

Senile

AS (prealbumin)

Heart

Hemodialysis associated

AH (b 2 mikroglobulin)

Kidney

Alzheimer associated

A4 (b amyloid)

Brain

Endocrine
(medulary thyroid carcinoma)

AE

Thyroid

Disorders of metabolism of lipids

Sphingolipidosis - ELMI

Lysozomal storage diseases

Disorders of metabolism of carbohydrates

Glycogenoses
Hepatic type (von.-Gierke)
Myopathic type (McArdle)
others (Pompe myocardial)

Impaired metabolism of water.

Related to distribution of electrolytes (K+phospates
intracellulary; Na +Cl+bicarbonates +Mg+sulphates
extracellulary)
Intracellular accumulation of water - vacuolar dystrophy
(sponge-like cytoplasm).
Causes: hypoxia, starving, osmotic effects (osmotic
nephrosis), hyperaldosteronism, viroses (herpes).
Extracellular changes:
a) dehydratation
b) hyperhydratation

Dehydratation
Loss of water (hypertonic dehydratation) diabetes
insipidus
Loss of water and Na (isotonic dehydratation)
vomiting, diarrhoea, burns
Loss of Na (hypotonic dehydratation) impaired
resorbtion of Na in kidney, hypoaldosteronism

Hyperhydratation
Hypotonic
Isotonic
Accumulation of fluid:

Oedema
Hydrops
Anasarka
Transsudation (hydrothorax, hydropericardium, ascites).

Oedema

Venostatic (venous thrombosis, gravitation)

Cardial (left/right ventricle failure)
Hypoalbuminotic (low oncotic pressure)
Lymphostatic (elephantiasis)
Inflammatory (increased capillary permeability exsudate)
Angioneurotic (Quincke)
Hormonal
Others (intoxication, hypoxia, etc)

Impaired metabolism of calcium.

Level of Ca (9-11mg%) is in balance with phosphate ionts
and is regulated by parathormone, calcitonin and vitamin
D.
Impaired metabolism:
Dystrophic calcification
Metastatic calcification (hyperparathyroidism,
hypovitaminosis D).
Calcinosis (skin, muscles = myositis ossificans
progressiva, nerves; normal level of Ca).
Calcifylaxis

Impaired metabolism of iron

Fe presented in haemoglobin (73%), myoglobin (11%),
feritin,enzymes and transferin.
Absorption in small intestine regulated by apoferitin,
efflux of Fe very limited
Improper acculumation of Fe leads to:
Haemochromatosis geneticaly related impaired
absorption of Fe (50-times increase). Deposition of
haemosiderin in skin and pancreas (bronze diabetes),
liver (pigmented cirrhosis), heart (failure), salivary
glands, kidney, smooth muscle
Haemosiderosis hemolysis, increased intake of Fe

Haemochromatosis

Haemochromatosis

Impaired metabolism of copper

Accumulation of copper due to insufficient production of
ceruloplasmin : hepatolenticular degeneration - liver
cirrhosis; destruction of cells in basal ganglia, damage of
proximal tubuli in kidney, Kaiser-Fleischer ring in cornea.
2 clinical variants:
Wilson disease damage of liver, extrapyramid
symptoms, dementia, start since childhood.
Westfal-Strmpell pseudosclerosis small neurological
symptoms after puberty

Crystals
Uric acid arthritis uratica (gout) impaired metabolism
of purines
Oxalates colourless crystals in renal tubuli or
myocardium in oxalosis
Cholesterol atherosclerosis, postinflammatory
Paraprotein renal tubuli in plasmocytoma
Cystine cystinosis (Lignac-Fanconi disease) spleen,
lymph nodes, kidnely, cornea
Charcot-Leyden crystals destruction of eosinophils

Gout

Concrements
Various tissues :
Gallbladder, bile ducts, uropoietic system, salivary glands,
pancreas, prostate

Three main factors modulating concrement development:

Increases concentration of substance
Changes in colloid millieu (inflammation)
Changes of pH (urine)
Clinical consequences: obstruction of duct

Pigments
Autogeneous
melanin (Addison disease, freckle, naevus, malignant melanoma /
albinisms, vitiligo, leukoderma)
lipofuscin
haemosiderin
bilirubin
Exogeneous
pigmentation by respiratory tract, trauma, gastrointestinal tract,
blood
pneumokoniosis, koniofibrosis
argyrosis Ag
chrysocyanosis Au