At first the disease was
described by Virhow in 1864 under the name "brown lung induration".
The basis of the disease is bleeding in lung alveoli, which has usually a
diffuse character. Free iron as a result of the collapse of erythrocyte is
absorbed by macrophages, which may be found in the sputum of patients (siderophages).
It is considered
that the anomaly of arteriolar-venular anastomoses and pathological structure
of the connective tissue of the lungs is hereditary.
Pathogenesis.
As a result of an inherited
defect forced hemocirculation of lung tissue occurs with a significant exit in it
red blood cells (per diapedesis) and the progressive sensitization of the
organism to them. Iron from haemolysed red cells becomes stable connected with
pathologic sulfated mucopolysaccharides and dose not return to the blood, dose
not include in the metabolism and synthesis of hemoglobin. Therefore, anemia
accompanying IPH is related with iron deficiency. Iron deposition, hemorrhage,
inflammation leads to pneumosclerosis, pulmonary hypertension and chronic
pulmonary failure. Allergic lesions of the joints, skin, heart, kidneys may
develop.
Morphologically (based on open lung biopsy)
there are revealed red blood cells in the cavity of the alveoli,
hemosiderin-containing macrophages (siderophages), diffuse interstitial
fibrosis, sclerosis of small vessels of the lungs.
Clinic
The clinical
picture of IPH is composed of the respiratory and hematological
symptoms. At exacerbation cough appears, in older children accompanied by
hemoptysis - the appearance of blood in the sputum. Small children do not
expectorate sputum, they swallow it. With abundant pulmonary hemorrhage, they
may have vomiting with ingested blood.
Severity of the clinical picture in
the lungs depends on the amount of haemorrhage into the alveoli. Together with
cough and fever dyspnea and cyanosis occur. Wheezing in the lungs is listened.
Radiographic changes appear in the form of large focus shadows in both lungs.
The disease is often diagnosed as pneumonia, which leads to the appointment of
inadequate treatment and the worsening of patient's condition.
Laboratory data
1. Complete blood count: signs of anemia, aniso- and poicylocytosis,
reticulocytosis, during exacerbation - leukocytosis, leukocyte shift to the
left, increasing ESR.
2. Biochemical blood analysis: an increase of the level
of indirect bilirubin and decrease of serum iron.
3. General sputum analysis: siderophages are found.
4. Investigation of lung biopsy: at a period of
remission hemorrhage is weakly expressed, mainly in the cortical areas of the
lung, pleura in these areas is thickened, in the alveoli, intralobulary, around
vessels, peribronhially and in connective tissue there are many hemosiderophages,
reactive fibrosis. At exacerbation alveolars are filled with fresh red cells,
alveolar macrophages containing hemosiderin.
5. Peculiarity of radiological changes in the IPH is the
rapid regression of foci shadows.
Review chest X-ray of patients with idiopathic
pulmonary hemosiderosis: small meshy diffuse lung pattern deformation caused by
compaction of interstitial lung tissue, multiple foci of diffused small
monomorphic shadows.
Idiopathic pulmonary
hemosiderosis.
Hemosiderophages in
hemoptysis of a patient with IPH.
Materials obtained through
bronchoalveolar lavage.
In some cases, the X-ray of
the chest indicates diffuse small shadows in both lungs, which are the reason
of mistake diagnosis of miliary tuberculosis of the lungs.
Changes in the lungs that maybe
detected with X-rays can vary widely: from small to massive infiltration shadows
accompanied by atelectasis, emphysema, and reaction from the lymph nodes of the
roots of the lungs.
Immediately after the exacerbation,
which lasts 3-5 days, there is marked anemia - microcytic and hypochromic. The
level of serum iron falls. In the biochemical analysis of blood there may be
elevated levels of bilirubin. Since the regenerative bone marrow function does
not suffer, in the peripheral blood reticulocytes appear. In young children in
the fecal analysis blood test may be positive (swallowed by coughing phlegm
with blood). Often there is hepatosplenomegaly.
The course
of idiopathic pulmonary hemosiderosis is undulating.
Periods of crises alternate with periods of remission varying duration. Acute
and subacute forms are relatively distinguishing.
At acute form of disease there are a significant worsening, weakness, dyspnea. Older
children complain of chest pain, cough with small amount of sputum, relaxed
breathing and wet wheezing are listened in lungs. Perhaps may be raising the
temperature to febrile scores. There is rapidly increasing cell anemia.
In the subacute form of IPH pallor of
the skin, the symptoms of intoxication gradually develop. Exacerbations of the
disease are more severe.
Diagnose.
Diagnostic significance has
the discovery in sputum or endotracheal aspirate, and in some cases, in gastric
lavage sidergophages. Puncture biopsy of the lung is accompanied with serious
complications.
Investigation of respiratory function
detects or normal rates of ventilation, if the duration of the disease is little,
or restrictive severe violations, reduced lung diffusion capacity, if the
disease is prolonged with severe exacerbations.
If repeated respiratory diseases
occur every time with anemia, an unusual radiological pattern in the lungs and ineffectiveness
of antiinflammatory therapy are present such a patient should be examined for
the presence of IPH.
Spirometer SPIROVIT
SP-1
Lung Volumes and capacities
Lung
Volumes and Capacities
Differential diagnosis should be conducted with
tuberculosis and fungal infections of the lungs.
Treatment.
Treatment of
patients with IPH involves the appointment of corticosteroid drugs and
symptomatic treatment. During periods of exacerbation oxygen therapy,
glucocorticoids, antibiotics, disintoxication, vitamin and treatment of anemia are
indicated. If you find high levels of precipients to cow's milk this product is
excluded from the diet. Prednisolone is appointed by the dose of 1 -1.5 mg / kg
to achieve clinical and laboratory remission. There are reports that after
splenectomy resistant prolonged remission may occur.
Prognosis is serious. Half of the children die in
the first five years after onset. Subsequent exacerbations are more difficulty
to treat, the average life span of a sick is 2-3 years, rarely – more. Immediate
causes of death often are acute massive pulmonary hemorrhage or progressive
pulmonary heart failure. Perhaps there is a combination of both reasons. And to
predict the probability of acute pulmonary hemorrhage is not possible.
Primary pulmonary hypertension (PPH)
Primary pulmonary hypertension is a rise in pressure in the
pulmonary artery and right ventricular hypertrophy which are not associated
with congenital or acquired pathology of the heart and lungs.
In 1901 Auerza described patient with
marked cyanosis, right ventricular hypertrophy and chronic bronchitis. Family
form of primary PH was first described in the report Clarke et al. in 1927. The
authors have noted a similar clinic and morphological changes in autopsy
material from two sisters, 5 - and 8-years of age and confirmed the presence of
primary PH. There are more than 20 titles of the disease. Among them:
Idiopathic right ventricular hypertrophy, primary pulmonary artery sclerosis,
isolated pulmonary hypertension, a disease Aersa etc.
Currently there is established the
gene BMPR2, localized in the locus of the second chromosome 2q33, which
regulates growth and proliferation of endothelial cells. This gene is
responsible for the development of a family primary pulmonary hypertension. The
disease is inherited in an autosomal-dominant type with incomplete penetrance,
which manifests itself in a population with a frequency of 1-2 cases per 1
million people. Family nature of PPH is approximately in 6% of patients, the
remaining cases are sporadic. Family form of PPH is not clinically different
from sporadic, but after the first symptoms is usually diagnosed earlier. In
sporadic cases diagnose of PPH is usually established in the advanced stages of
disease.
Pathomorphology.
Pathomorphologic changes in primary
pulmonary hypertension are clear. There is right ventricular hypertrophy,
expansion of large branches of pulmonary artery with layer fibroelastosis of
the intima, the presence of atriovenose anastomoses, thrombosis and fibrinous
necrotizing arteritis of small branches of the pulmonary artery.
Clinic.
In the initial stages the
objective symptoms are mild, cardialgia in children is atypical. There is a
poor tolerance to physical activity through the development of shortness of
breath, sometimes accompanied by attacks of breathlessness. The appearance of syncope
point at severe phase of the disease, they often appear at an exercise, RV
heart failure increases.
There
are three stages of development of pulmonary hypertension in children. At I stage
increased pulmonary pressure is the only hemodynamic abnormality, patients do
not have distinct symptoms, there may be shortness of breath during physical
activity, which often does not wary the doctors and patients for the presence
of the disease and is usually associated with poor training of the body.
When
a decrease in cardiac output (II stage) occurs, there are detailed clinical
symptoms in the form of hypoxemia, dyspnea and syncope. Pressure in the
pulmonary artery remains stable at a high level.
With
the advent of right heart failure III stage of disease occurs: in this case,
despite the consistently high values of pulmonary pressure, cardiac output
falls sharply, there are venous congestion and peripheral edema.
There
is variability in the duration of different stages of the disease, an average from
6 months to 6 years or more from the stage of minimal hemodynamic changes to
the lethal disease. The reason of death is the developing prolonged functional
overload the right heart, destructive and sclerotic changes in lung tissue and
myocardium.
PPH
affects mainly young patients, and the disease is usually fatal, although in
the literature there are described isolated cases of spontaneous remission.
It is
assumed that the therapeutic activities of the patients of childhood will be
more effective, as the remodeling of the pulmonary vessels in children can be
prevented and even reversed. The development of new directions in treatment can
increase survival and improve quality of life of patients.
The child 13 yrs
old with PPH.
Weight - 33,5 kg (norm -
44,1 ± 8,68 kg), height - 144 cm (average 155,7 ± 6,57 cm). There are mixed
apnea, tachypnea to 38 per minute. Skin is pale, cyanosis of the lips,
acrocyanosis.
Deformation of the terminal phalanges as
"drumsticks" and "watch glasses", enlargement of the
abdomen (ascites) in patient with PPH.
ECG of patient with PPH. Sinus tachycardia
(cardiac rate 140 per minute).
Rightgram. Atrioventricular blockade Іst.
(PQ–0,20"). Expressed hypertrophy of right
ventricular. Dilatation of the both auricles.
A
B
C
D
A. Hypertrophy of media and adventitia of small
pulmonary arteries.
B. Concentric intimal fibrosis of pulmonary
arteries with a diameter of 180 microns, there is also hypertrophy of the
middle and the outer shell of the vessel.
C. Obliteration of the lumen of the pulmonary
artery.
D. Plexiform defect. Note the absence of
elastic membrane in the wall plexiform structure.
Increasing x 100. Coloured by Verhoeff's - Van
Gieson.
Muscularization of arterioles: a - hematoxylin
and eosin. ґ400 b - immunohistochemical coloration on actin of smooth muscle
(SMA). ґ200.
Necrotizing arteritis. Coloured Rechanaled thrombus of branch
of
by hematoxylin and pulmonary
artery. Coloured
eosin ґ100.. by hematoxylin and
eosin ґ40.
EchoCG. A-wave is escaped.
Treatment is symptomatic:
antihypertensives, anticoagulants, antiplatelet agents. Effects are minor.
Prognosis is poor. Life expectancy is
from several months to 10 years from onset of the first symptoms.
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