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Diagnosis of diseases of blood at children

BLOOD DISEASES

Disease

Symptom complex

195 Joseph's anemia - Dayemonda-Blekfena

Emergence of clinical signs of a disease at early age
The progressing weakness, slackness, anorexia
Dispepsichesky frustration
Vosyuovidny pallor
Wide nose bridge
Fair hair
Reinforced upper lip with a bright red border
Normokhromny anemia, reduced quantity of reticulocytes
Miyelogramma — an erythroidal hypoplasia

196 Anemia of premature

 

Pallor of integuments in mucous
The systolic noise which is especially expressed on the basis of heart
Decrease in concentration of hemoglobin is lower than 90 g/l
Erythrocytopenia

Reticulocytosis
Decrease in content of serumal iron
Refrakternost to the carried-out antianemic therapy

197 Anemia is drepanocytic

Jaundice
Hepatolienal syndrome
Normal osmotic resistance of erythrocytes
Existence of crescent erythrocytes in smears after preliminary creation of conditions of a hypoxia
Synthesis of pathological HbS instead of normal NVA

198 Fankoni's anemia

Nanism
Hypogenitalism
Xanthopathy
Anomalies of a skeletogeny
Inborn malformations of internals
The progressing pancytopenia arising at the age of 6 — 8

199 Anemia of Estrena-Damesheka

Ch|asty developing of infectious diseases
Lag in physical development
The expressed pallor of integuments with a grayish shade
Recurrent hemorrhagic syndrome in the form of bleedings or hemorrhages
Systolic noise with a maximum on the basis of heart
8 the gepatosplenomegaliya Is possible
Normokhromny anemia, reticulocytopenia, granulocytopenia, thrombocytopenia
Miyelogramma — oppression of all sprouts of a hemopoiesis

200 Brill's illness - Simmersa (a macrofollicular lymphoma)

Local hyperadenosis of a dense consistence, not soldered among themselves and surrounding fabrics, painless at a palpation
Splenomegaly
Neutrophylic leukocytosis, the increased SOE
Existence of huge follicles in a bioptata of lymph nodes

201 Verlgof's illness (idiopathic Werlhof's disease)

More often girls get sick
The skin polymorphic, polikhromny, asymmetrical hemorrhages arising spontaneously
Hemorrhages on mucous, are more often than an oral cavity
Bleedings (gastrointestinal, meno-and metrorrhagias, from a hole of the extracted tooth, from eyes symptoms of "bloody tears")
Positive tests of Konchalovsky, can, etc.
Thrombocytopenia
Considerable lengthening of a bleeding time
Insufficient retraction of a blood clot
Miyelogramma - a hyperplasia of a megakariotsitarny sprout

202 Villebranda-Yurgens's illness

Plentiful long bleedings of various localization at insignificant wounds and injuries
Formation of hematomas
Reduction of the hemorrhagic phenomena with age
Increase in duration of bleeding
Normal blood clotting time
Decrease of the activity of koagulyantny part of a factor of VIII
(anti-hemophilic globulin)

203 Gippelya-Lindau's illness (angioreticulomatosis, cerebroretinal angiomatosis)

Development of a clinical picture at early children's age
Multiple flat angiomas in the form of red specks on face skin, extremities, a stomach, a thorax
The unilateral angiomas of a retina causing its amotio in the course of growth
Hydrocephaly
Focal neurologic symptomatology with a lack of coordination of movements and statics

204 Lucky's illness - Loranda

The plentiful and long bleedings from an umbilical wound developing from the moment of the birth
Slow mending of an umbilical wound
Developing of hematomas and long bleedings later
injuries at advanced age
Compliance to norm of all coagulative tests
Deficit of a fibrinstabiliziruyushchy factor — a factor of XIII

205 Illness to Letterera-Ziva

Emergence of clinical symptomatology at early age
Gepatosplenomegaliya
Hyperadenosis
Bone infiltrates
Hemorrhagic diathesis
Ulcer and necrotic defeats of mucous
Elastic swelling and morbidity at a palpation in flat bones of a calvaria with the subsequent multiple destruction of the specified bones
8 Detection of a triad of histiocytic cells (atypical histiocytes, multinucleate and ksantomny cells) at research of proliferat of the struck bodies

806 Minkowski's illness - Shoffara

Signs of a dizembriogenez (tower skull, wide ridge of the nose, high standing of gums, narrow tooth arches, iris heterochromia)
Periodic developing of jaundice of various intensity without skin itch, discoloration of urine, decolorization a calla
Splenomegaly
Anemia, reticulocytosis
Increase of level of an indirect bilirubin in blood
Decrease in osmotic firmness of erythrocytes
Price-Jones curve shift to the left

207 Riley's illness -
Smith

The progressing increase in the head from the moment of the birth
Lack of symptoms of hydrocephaly or increase of intracranial pressure
Emergence on skin within the first year of life of the hemangiomas tending to growth
Lack of neurologic symptomatology

208 Randyu-Osler's illness (inborn hemorrhagic atsgiomatoz, hereditary hemorrhagic
teleangiektayazya)

The teleangiectasias in the form of points, spiders or small knots which are not disappearing when pressing on lips, skin of cheeks, wings of a nose, a mucous digestive tract, respiratory, urinary tract
Bleedings (nasal, pulmonary, gastrointestinal, seldom hamaturia)
Gepatomegalmya
Posthemorrhagic anemia of various degree

209 Stewart-Prauera's illness

Plentiful bleedings not of an umbilical wound in the neonatality period
Bleedings after injuries, extractions of tooth, a tonsilectomy at children of more advanced age
Hemorrhages in skin, hypodermic cellulose, muscles
Lengthening of a prothrombin time on Kvika at normal thrombin time
Lengthening of a blood coagulation at test with snake poison
Deficit of a factor of X

210 Taratynov's illness (eosinophilic granuloma)

The isolated damage of a separate bone (dense tumorous education in the place of defeat with the subsequent flattening in process of development of destruction and formation of defect of a bone)
Lack of symptoms of intoxication
Moderate eosinophilia, increase in SOE
In biopsy material — a triad of gistiotsntarny cells (atypical histiocytes, multinucleate and ksantomny cells)

211 Illness of Henda-Shyulera-Krischena

Development of clinical symptomatology in younger school
age
Exophthalmos
Granulomas in flat bones of a skull, a basin, in jaws with destruction of a bone tissue
Not diabetes mellitus
Pituitary nanism
Gepatosplenomegaliya
Triad of gistiotsigarny cells (atypical histiocytes, much nuclear and ksantomny cells at histologic research of the struck fabrics)

212 Inborn amegakariotsitarny Werlhof's disease

Emergence of extensive hemorrhagic rash on skin and mucous in the first days after the child's birth
Plentiful nasal, gastrointestinal bleedings, hamaturia
Existence of congenital anomalies of bone system. The most typical of them — a bilateral aplasia of beam bones
Anemia, thrombocytopenia
In a miyelogramma — hypo - or an aplasia of a megakariotsitarny sprout

213 Hemolitic illness of newborns (anemic form)

The pallor of integuments appearing within the first week of life
Gepatosplenomegaliya
Slackness
Some decrease in reflexes of newborns
Anemia, reticulocytosis
Moderate increase of level of bilirubin at the expense of indirect
The child's mother — Rh-negative

214 Hemolitic illness of newborns (icteric form)

The yellowness of integuments appearing at the end of the 1st, the beginning of 2 days zhnann
Gepatosplenomegaliya
Decrease in reflexes of newborns
Anemia, reticulocytosis
Substantial increase of an indirect bilirubin of blood
Urobilin in urine
The child's mother — Rh-negative

215 Hemolitic illness of newborns, edematous form (Shridde's illness)

The large weight of the child at the birth
Areflexia
Hypotonia
Sharp pallor
Anasarca
Tachycardia
Hepatolienal syndrome
The expressed anemia, eritreblastoz
The child's mother — Rh-negative

216 Hemorrhagic illness of newborns

Development of a disease for 2 — 4 day of life
Petechias, ecchymomas on skin
Hematemesis
Melena
Bleeding of an umbilical wound
Negative reaction of Apt

217 Hemorrhagic vasculitis (Shenleyna-Genokh's illness), abdominal form

Pristupoobrazny, a little calming down, but completely not a disappearing abdominal pain without accurate localization
Vomiting
Liquid chair with blood impurity
Development along with above-mentioned symptomatology or a little later than joint and skin hemorrhagic syndromes
Intoxication symptoms
Increase of coagulability of blood

218 Hemorrhagic vasculitis (Shenleyna-Genokh's illness), fulminant form

The symmetric plentiful, quickly extending on all body surface hemorrhages with tendency to merge and a nekrotizirovaniye
Arthritis
The expressed abdominal syndrome which is followed by vomiting, a melena
Symptoms of acute adrenal insufficiency
Spasms
Increase of coagulability of a venous blood
Neutrophylic leukocytosis, toxic granularity of neutrophils, aneosinophilia
Development of the above-stated symptomatology of a disease 1 — 2 week later after influence of a provocative factor (a SARS, quinsy, vaccination, introduction scale blueberry etc.) at children of younger age

219 Hemorrhagic vasculitis (Shenleyna-Genokh's illness), simple form

Intoksikatsionny syndrome
The symmetric not disappearing when pressing, hemorrhagic rash on skin of shins, an internal surface of hips and buttocks, in knee, ankle, elbow, radiocarpal joints
Lack of rashes on mucous
Increase of coagulability of a venous blood

220 Hemorrhagic vasculitis (Shenleyna-Genokh's illness), rhematoid form

Inflammatory damage of large joints
Symmetric hemorrhagic rash on skin of shins, an internal surface of hips and buttocks, in knee, ankle, elbow, radiocarpal joints
Intoksikatsionny syndrome
Lack of a hemarthrosis
The increased coagulability of a venous blood
Development of the above-stated clinical symptomatology in 1 — 2 weeks after influence of a provocative factor (a SARS, quinsy, vaccination, administration of gamma-globulin etc.)

221 Hemophilia And

1 Emergence of symptomatology at the end of the 1st, the beginning of the 2nd year of life
Bleeding of an umbilical wound
Stomatorrhagia
Long bleedings at disturbance of integrity of skin, inadequate to an injury
Deep, massive hemorrhages in the soft tissues which are followed by a pain syndrome
Forming of a hemarthrosis
Hamaturia
Lengthening of coagulability of a venous blood
Deficit of the VIII factor (anti-hemophilic globulin)

222 Cristmas disease (Kristmas's illness)

Emergence at the end of the 1st, the beginning of the 2nd year of life of bleedings after insignificant injuries of skin and mucous
Emergence of hematomas and formation of a hemarthrosis at advanced age
Lengthening of coagulability of a venous blood
Deficit of a factor of IX (plasma component of thromboplastin)

223 Hemophilia With (Rosenthal's illness)

Absence of spontaneous bleedings, hemarthrosis
Developing of bleedings at injuries and operations (extraction of tooth, a tonsilectomy, appendectomy)
The decrease in coagulative activity of blood revealed by autokoagulyatsionny test
Decrease in level of the plasma predecessor of thromboplastin — a factor of XI

224 Hypoproconvertinemia

Bleedings from an umbilical wound in the neonatality period
Skin, hypodermic, intramuscular hematomas, bleedings from mucous, including a digestive tract at more advanced age
Lengthening of a prothrombin time on Kvika at normal indicators of other koagulyantny tests
Deficit of proconvertin (factor of VII)
Normalization of coagulative disturbances at test with snake poison

225 Prothrombinopenia

 

1 Bleedings from an umbilical wound after the birth
Bleedings from a mucous mouth and a nose, a digestive tract
Long bleedings at surgical interventions, injuries, cuts, after extraction of teeth
Heavy menorrhagias at girls during puberty

Lengthening of protrombiiovy time on Kvika at normal thrombin time
Deficit of a prothrombin — (a factor of II)

226 Hypofibrinogenemia

Bleedings from an umbilical stump
Emergence of ecchymomas, petechias, is more rare — hypodermic hematomas
Bleedings at the minimum injuries of skin and mucous
Meno-and metrorrhagias
The lengthening of coagulation of a venous blood which is not improving after thrombin addition
Decrease in level of fibrinogen in plasma

227 Wegener's granulomatosis

Persistent cold with bloody purulent separated
Cough
Gemoptoe
Pains in adnexal bosoms of a nose, in a throat, in a thorax
Dysphagy
Damage of eyes (corneoscleral ulcers, necrotic keratitis, granulematozny sclerouveitis)
Pan-vasculitis and polymorphic cellular granulomas in bioptata of the struck bodies

228 "Jaundice from maternal milk" (Arias's jaundice - Lyutsey)

Increase in intensity of physiological jaundice by the end of the first — the beginning of the second week of life at clinically healthy child
Increase of level of bilirubin at the expense of indirect
Decrease in level of bilirubin by 85 — 171 µmol/l within 24 hours after cancellation of breast milk

229 The leukosis is lymphoblastoid

Fever
Arthralgias, ossalgiya, brevispondiliya
Wax-like pallor of integuments
System increase in peripheral lymph nodes, painless at a palpation
Hepatolienal syndrome
In peripheral blood blast cells, increase in SOE
Miyelogramma — more than 5% of imperious cells
Positive reaction of imperious cells with a glycogen
Positive reaction of imperious cells with acid phosphatase

230 The leukosis is miyeloblastny

Fever
Arthralgias, ossalgiya, brevispondiliya
Hemorrhagic syndrome
Ulcer and necrotic defeats of mucous
Peripheral blood — the anemia, imperious cells raised by SOE
Miyelogramma — more than 5% of blast cells
Positive reaction of blast cells from miyeloperoksidazy
Positive reaction of imperious cells with Sudan black

231 Lymphogranulomatosis, I stage

Subfebrile condition
The progressing hyperadenosis of one or two adjacent groups of a plotnoelastichesky consistence which are not soldered to skin painless
The moderate anemia, neutrocytosis raised by SOE
In a bioptata of lymph nodes — Berezovsky's cells - Shternberga-Read

233 Lymphogranulomatosis, II stage

All-toxic syndrome
Defeat of two or more non-adjacent groups of the lymph nodes located on one side of a diaphragm
Anemia, neutrocytosis, the thrombocytopenia raised by SOE
Fibrinosis
Berezovsky-Shternberga-Read's cells in a bioptata of the struck lymph nodes

233 Lymphogranulomatosis, III stage

Defeat of various groups of the lymph nodes located on both sides of a diaphragm
Splenomegaly
Toxic syndrome
The progressing anemia, neutrocytosis, the thrombocytopenia raised by SOE
Fibrinosis
Berezovsky-Shternberga-Read's cells in a bioptata of the struck lymph nodes

234 Lymphogranulomatosis, IV stage

Defeat of various groups of lymph nodes on both sides of a diaphragm
Damage of a liver, lungs, pleura, bone tissue, kidneys, skin
The expressed toxic syndrome
Anemia, neutrocytosis, the thrombocytopenia raised by SOE
Fibrinosis
Berezovsky-Shternberga-Read's cells in a bioptata of the struck lymph nodes

235 Hereditary
Kostman's neutropenia

Emergence of clinical symptomatology at early children's age
Chronic recurrent damages of skin and mucous, not followed by a gnoyeobrazovaniye, having ulcer and cicatricial character
Generalized hyperadenosis
Frequent pneumonia with formation of cavities
Neytropennya remaining even at accession of inflammatory process
Unripe granulocytes in peripheral blood

236 Parahemophilia

Spontaneous bleedings at babies
Epistaksis
Long bleedings at injuries, small surgical interventions
Easily arising ecchymomas
Menorrhagias during puberty
Absence of hematomas and hemarthrosis
Lengthening of a prothrombin time at normal thrombin
Deficit of pro-accelerin — a factor of V

237 Periodic (cyclic) neutropenia (crisis)

Pustulous damages of skin
Ulcer and necrotic defeat of mucous
Development of the abscessing pneumonia
Leukopenia with a neutropenia
Spontaneous recovery of a gemogramma in 3 — 5 days after the beginning of hematologic crisis

238 Mayoki's purpura

Develops against long allergenic action of infectious factors
The symmetric spots of dark red color which are located on skin of the lower extremities, buttocks, hips
Teleangiectasias
Skin atrophy
Satisfactory condition of patients

239 Bernard-Sulye's syndrome

Emergence of clinical signs of a disease in the neonatality period
Skin hemorrhagic syndrome
Hemorrhages in mucous
Nasal, gastrointestinal bleedings
In a gemogramma — huge forms of thrombocytes

240 Valleder's syndrome (infectious eozinofilez)

Catarral phenomena
Arthralgias
Fever
Lymphadenopathy
Gepatosplenomegaliya
Hyperleukocytosis, hypereosinophilia
Mature forms of eosinophilic granulocytes in punctates of marrow and lymph nodes
Lack of a helminthic invasion

241 Valkvista-Gasser's syndrome (chronic granulocytopenia of children's age)

Emergence of clinical displays of a disease at early age
The recurrent infections having an easy current
Lag of children in physical development
Gemogramma — absence segmentoyaderny neutrophylic
granulocytes at normal quantity of leukocytes
5 Neutrophylic reaction of white blood due to increase in metamyelocytes and band neutrophils

242 A syndrome Wang - Bogart-Divri (a kortiko-meningeal diffusion angiomatosis)

Emergence of clinical symptomatology of a disease at early children's age
Metamericly located star-shaped teleangiectasias on skin
Poikiloderma signs
The strapiramidny disturbances expressed pyramidal and ek leading to disorder of motor and static functions
Convulsive attacks like epilepsy
Narrowing of fields of vision with a hemianopsia

213 Viskota-Aldrich's syndrome

Only boys get sick
Emergence of clinical symptomatology in the first weeks or months of life
Hemorrhages of petekhialny character on skin and mucous
Plentiful bleedings of various localization
Melena
Moderate hyperadenosis and spleens
Hamaturia
Anemia, leukocytosis with shift to the left, a lymphocytopenia, thrombocytopenia with existence of a large number of microforms of thrombocytes, lengthening of a bleeding time, increase in SOE
Reduction or total absence of Ig M at simultaneous increase in IgA, IgC, Ig E

241 Kazabakha-Merrit's syndrome

Huge hemangioma on a trunk or extremities
Bleedings of various localization
Hemorrhagic manifestations on skin and mucous, characteristic of a Werlhof's disease
Thrombocytopenia, fibrinogenopenia
Increase in a bleeding time

245 Kriglera-Nayar's syndrome

Emergence of clinical symptomatology soon after the child's birth
Yellowness of integuments
Oculomotor frustration
Tonic and clonic spasms
Opisthotonos
Akholichny of kcal
Increase of level of an indirect bilirubin in blood
Absence of anemia

246 Meya-Hegglin's syndrome

Small amount of hemorrhagic rashes
Thrombocytopenia
Existence of defective thrombocytes of the large and huge sizes in peripheral blood
Anomaly of leukocytes with existence in them of specific little bodies Business

247 Syndrome Moshkovich (trombogemolitichesky Werlhof's disease)

Skin hemorrhagic syndrome,
Tonic spasms
Acute renal failure
Acute adrenal insufficiency
Acute heart failure

6 Anemia with a reticulocytosis, on-likhromatofiliyey, poykilotsito-

zy and anisocytosis of erythrocytes, thrombocytopenia

7 Insufficient retraction of a blood clot

8 Increase in duration of bleeding

9 Increase of level of an indirect bilirubin

10 Irritation of an erythroidal sprout and a hyperplasia of a megakariotsitarny row in a miyelogramma

248 Nezelov's syndrome

1 Growth inhibition of the child

2 Existence of long septic process

3 Sharp decrease in maintenance of lymphocytes

4 Sharp oppression of reaction of blast transformation

5 The normal maintenance of all classes of immunoglobulins in blood

249 Syndrome of Chediaka-Higassi

1 The reduced slezootdeleniye, a photophobia

2 Pigmental damages of skin

3 Excessive sweating

4 Gepatosplenomegaliya

5 Recurrent purulent infections with involvement of regional lymph nodes

6 Anemia, neutropenia, thrombocytopenia

7 Morphological anomalies of all forms of leukocytes

250 Evans-Fischer's syndrome

1 The reduced slezootdeleniye, a photophobia

2 Pigmental damages of skin

3 Excessive sweating

4 Gepatosplenomegaliya

5 Recurrent purulent infections with involvement of regional lymph nodes

6 Anemia, neutropenia, thrombocytopenia

7 Morphological anomalies of all forms of leukocytes

251 Thalassemia (targetoid and cellular anemia Sacks)

kulotsitozy, anisocytosis and poikilocytosis of erythrocytes
5 Thrombocytopenia
Lurid color of integuments
Significant increase in a spleen, less — a liver
The accruing symptoms of dystrophy
Radiological — thinning of outside and internal tables of the scull, expansion of a spongy layer
The progressing normokhromny anemia, reticulocytosis
Existence of targetoid erythrocytes in smears of peripheral blood
Decrease minimum and increase of the maximum osmotic firmness of erythrocytes

252 Glantsman's thrombasthenia

Bleedings from mucous
Hemorrhages on skin and mucous as a Werlhof's disease
Normal quantity of thrombocytes
Lengthening of a bleeding time
Coloring of thrombocytes in blue color

253 Chronic granulomatosis

Developing of a disease in the first two years of life
Recurrent purulent infections of various localization, resistant to an antibioticotherapia
Increase and morbidity of regional lymph nodes, their suppuration
Hepatolienal syndrome
Neutrocytosis, existence of unusually large granules and vacuoles at neutrophils
Disturbance of the digesting ability of neutrophils at the kept chemotaxis and opsonization
Reduced NBT-test (low formation of peroxide of hydrogen neutrocytes)

According to Ostropolets S. S., V. N. Beet, Diagnostic syndromes of diseases of children's age.

 
"Diagnosis of diseases of a digestive tract at children   Diagnosis of diseases of an urinary system at children"