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Hemorrhagic diatheses in
children. Gastrointestinal
bleedings.
Sakharova I. Ye., MD, PhD
The stopping of a bleeding is
carried out due to three parts of a
hemostasis:
1. Vascular integrity.
2. Qualitative and quantitative
characteristics of platelets.
3. Presence of coagulation factors in
blood.
2
According to this all hemorrhagic
diatheses are divided into 3
groups:
1. Vasopathies
2. Thrombopathias
3. Coagulopathies
3
Schönlein-Henoch purpura (synonims anaphylactoid purpura, allergic angiitis,
small-vessel vasculitis, hemorrhagic
vasculitis, Henoch-Schönlein disease) is
one of the collagen vascular diseases in
which basis lays immune complex
mechanism of small vessels wall damaging
with skin, joints, intestine and kidneys
affection.
4
Clinical features.
пѓј Papular- macular hemorrhagic purpuric
lesions.
Typical
places
of
rush
localization: extensor surfaces of legs,
on the feet, over the joints, on the
buttocks; occasionally, they may occur
on the hands, extensor surfaces of arms,
elbows, and face, but very rarely on the
trunk.
5
6
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Clinical features of the SchönleinHenoch purpura.
пѓ� joints involvement
пѓ� acute abdominal pain, vomiting,
melena
пѓ� renal involvements (microscopic
hematuria, with or without
proteinuria)
пѓ� scrotal involvement (epydidimitis,
orchitis, and scrotal bleeding)
10
Platelet defects
Throm bocytopenies
C ongenital
Throm bocytopathias
A cquired
(Fanconi pancytopenia,
W iskott-Aldrich syndrom e)
Throm bocytes
synthesis
disturbances
Increase
of pletelets
destruction
Im m une
C ongenital
A cquired
(Glanzm ann throm boasthenia
B ernard-Soulier syndrom ,
Von W illebrand disease)
D isturbances
of platelets
distribution
N onim m une
(sepsis, D IC syndrom , H U S)
H eteroim m une
A utoim m une
Izoim m une
11
Idiopathic thrombocytopenic
purpura (ITP, primary immune
thrombocytopenic purpura,
autoimmune thrombocytopenic
purpura)
describes an autoimmune disorder in
which the number of circulating
platelets is less than 150 G/l.
12
Clinical features.
• The onset of the disease is usually sudden. The
symptoms of intoxication and fever usually are
absent.
• Skin purpura, which arises either spontaneously or
secondary to trauma. The type of rush is petechialbruise.
• The second frequent clinical sign are bleedings. In
the beginning of the disease can be nose bleeding
(epistaxis),
bleeding
from
gums,
mucous
membranes, gastrointestinal tract, kidneys and
metrorrhagias (uterinal bleedings).
• Hemorrhage into the central nervous system, the
most serious complication of thrombocytopenia.
13
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15
Hemophilia A and B are inherited
bleeding disorders caused by
deficiencies of clotting factor VIII (F
VIII - antigemophilic globulin (AHG))
and factor IX (F IX - plasma
thromboplastin component (PTC) or
Christmas factor) correspondingly.
16
Mechanism of hemophilia inheritance
Xy
xx
xy
Xx
Xx
xy
xy
Xy
xy
xx
Xx
Xy
XX
Xx
xy
Xy
17
Prophylactic treatment
The aim of this treatment is to maintain
5 % factor activity in patient’s blood.
Start from the age of 1-2 years.
Use monoclonal-antibody purified F VІІІ ,
F ІХ and recombinant F VІІІ , F ІХ 3 times
in week in hemophilia A and 2 times in
week in hemophilia B 25-40 IU/kg.
18
Treatment of acute bleeding episodes
Hemophilia A
– Fresh frozen plasma (100 ml=80IU АHG)
Dose is 10-15 ml/kg IV during 30-60 min,
repeat after 8-12 hours.
– Cryoprecipitate
– Monoclonal-antibody purified F VІІІ and
recombinant F VІІІ
Hemophilia B
– Fresh frozen plasma
– Monoclonal-antibody purified F IX and
recombinant F IX
19
During severe or dangerous (e.g. CNS,
retroperitoneal) bleeds need to obtain
50-100% factor activity for 7-10 days.
For less critical situations (e.g. dental
extractions, haematuria, soft tissue
bleeds), 20-50% factor activity for 2-7
days are generally sufficient. For
uncomplicated haemarthroses or
superficial muscle or soft tissue bleeds,
20-30% for 1-2 days.
20
Laboratory differential diagnostics of
hemorrhagic diatheses
Count of
platelets
Time of
bleeding
Coagula
tion
time
Hem. vaskul.
n
n
n
n
nп‚­
ITP
п‚Ї
nп‚­
n
п‚Ї
n
Trombopathia
n
nп‚­
n
nп‚Ї
n
Hemophilia
n
n
п‚­
n
п‚Ї
Clot
retraction
Coagulo
gram
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Success is not final,
failure is not fatal.
It is the courage to
continue that counts.
Winston Churchill
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