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It is only for medical professionals to read for reference.
Is this disease actually related to the lungs? Antecedent summary Dandan, a 2-year-old girl, was diagnosed with "iron deficiency anemia" at a local hospital 3 months ago because of her pale complexion
.
Due to severe anemia (58 g/L of hemoglobin), the local doctor gave red blood cell infusion and iron treatment, and his condition gradually improved, his hemoglobin rose to 90 g/L, and he was discharged
.
He continued to take the protein iron succinate oral solution (10 ml/time, 2 times/day) outside the hospital.
The hemoglobin rose to 108 g/L one month ago, and the parents stopped the medication by themselves
.
Recently, Dandan once again experienced symptoms such as pale complexion, loss of appetite, cough and sputum.
The local hospital found that hemoglobin was lowered again (55 g/L) and the condition was serious.
He immediately rushed to the higher-level hospital for treatment.
.
.
According to Dandan’s mother’s description Anemia during pregnancy, taking iron therapy, breastfeeding after birth, adding complementary foods (eggs, rice noodles, porridge) at 6 months, Dandanping is a picky eater, rarely eating meat, eggs, milk and vegetables, and there are exceptions.
Food addiction, like to lick iron
.
Because Dandan's anemia was severe, the doctor immediately gave him an infusion of red blood cells to correct the anemia, and at the same time perfect other related examinations
.
Figure 1 blood routine shows that Dandan is a small cell hypochromic anemia, with 9.
80% reticulocytes (normal range 0.
5%-1.
5%), serum iron is slightly lower, iron binding capacity is normal, bone marrow aspiration results show obvious red blood cell line proliferation, Granulocyte cell line and megakaryocyte cell line are normal
.
Dandanma has a history of anemia during pregnancy, and there is a lack of congenital hematopoietic materials.
In addition, Dandan is picky eaters, intake of meat, eggs, milk is low, and acquired hematopoietic materials are also insufficient.
Previous iron treatments are effective, so doctors consider the possibility of iron deficiency anemia
.
Dandan's cough and sputum symptoms were severe.
Chest X-ray film showed increased and thickened lung texture.
Enhanced lung CT showed that Dandan's lungs had ground-glass-like changes
.
Figure 2 combined with his severe anemia, at this time, he has been highly suspected of "pulmonary hemosiderinosis"
.
The doctor continued to search for evidence and found hemosiderin cells in sputum and gastric juice
.
Figure 3 Therefore, the revised diagnosis is "Pulmonary hemosiderinosis"
.
At this time, Dandan’s disease is in an advanced stage, and hemoptysis, fatal pulmonary hemorrhage, respiratory failure, multiple organ dysfunction, etc.
are likely to occur.
The doctor stopped iron supplementation and gave methylprednisolone sodium succinate intravenously.
Supported by oxygen inhalation and red blood cell infusion, Dandan's condition gradually stabilized after 1 week
.
Pulmonary hemosiderosis is mainly seen in children.
Idiopathic pulmonary hemosiderosis (IPH) is a relatively rare chronic lung disease caused by pulmonary capillary oozing, hemoglobin dissolution, and hemosiderin It is caused by the deposition of pulmonary elements, and the imaging manifestations of repeated hemoptysis, iron-deficiency anemia, and diffuse pulmonary infiltration make it a typical triad [1]
.
It can occur from newborn to adulthood, and it is mainly seen in children (especially 1 to 7 years old)
.
Five hypotheses about the etiology of IPH The etiology of IPH is still unclear.
It is currently believed to be related to autoimmunity, genes, allergies, environment, and infection factors, but none of the claims has been confirmed [2]
.
■ Environment—The hemolysin produced by Botrytis botryoides has a certain effect on the pathogenesis of IPH; ■ Heredity—Two siblings were diagnosed with IPH successively, and their serum Ig E was found to be significantly elevated; ■ Allergies—alveoli caused by milk allergy Anti-bovine milk autoantibodies were detected in the sera of children with hemorrhage; ■ Immune-antigen-antibody complex-mediated alveolar autoimmune damage, resulting in increased alveolar capillary permeability and hemorrhage of small pulmonary blood vessels; ■ Infection — suffering Children with pulmonary hemorrhage often have respiratory infections
.
What are the clinical manifestations of IPH? The clinical manifestations of IPH are diverse.
Common clinical symptoms are repeated coughing, hemoptysis, shortness of breath, anemia, etc.
The signs may include pale complexion, low breath sounds, tachycardia, hepatosplenomegaly, clubbing, and jaundice
.
Long-term repeated bleeding leads to thickening of the alveolar basement membrane, which can eventually lead to pulmonary interstitial fibrosis
.
According to the severity of the disease, it can be divided into three stages [3]
.
Table 1.
Clinical staging of IPH.
IPH that is often misdiagnosed.
IPH is often misdiagnosed due to its diverse clinical manifestations, insidious onset, lack of typical triad in some cases, and low incidence, lack of understanding of the disease by clinicians, often resulting in IPH being often misdiagnosed For the following diseases [4]: iron deficiency anemia thalassemia hemolytic anemia pneumonia with anemia pulmonary tuberculosis IPH diagnosis is exclusive, first of all to establish the existence of diffuse alveolar hemorrhage, has the following three characteristics: 1.
Hemoptysis, Hematemesis or old blood in infant gastric juice; 2.
Small cell hypochromic anemia; 3.
Chest radiograph or lung CT showing pulmonary hemorrhage-like changes, diffuse flocculent or ground-glass shadows in both lungs
.
The final diagnosis of IPH requires the following auxiliary examinations: Extracted from literature [2] The treatment of IPH is the current basic treatment for IPH, mainly including adrenal cortex hormones, immunosuppressants, antimalarial drugs, etc.
, with the purpose of quickly alleviating emergency and critical situations And maintenance treatment to improve the symptoms of patients [5]
.
1 Glucocorticoid acute intravenous hormone therapy, methylprednisolone 2 mg/(kg·d), intravenous drip or hydrocortisone 5-10 mg/(kg·d), intravenous drip; in acute alveolar hemorrhage, High-dose methylprednisolone 10~30 mg/(kg·d) shock therapy; after bleeding control, change to oral hormone and take prednisone 2 mg/(kg·d); after the symptoms are completely relieved (about 2~3 weeks) The dose is gradually reduced to the minimum maintenance dose (3-6 months); long-term low-dose glucocorticoid oral or inhalation treatment can reduce the risk of IPH recurrence and improve survival
.
2Immunosuppressive agents can be selected as second-line treatment drugs for glucocorticoid treatment ineffective and hormone dependence
.
Methotrexate: 2 mg/(kg·d), can be used in combination with hormones; mercaptopyrine: 1～2 mg/(kg·d) to 3～5 mg/(kg·d), the course of treatment is usually 1 Over years, it can effectively control symptoms and prevent acute attacks; 6-mercaptopurine: used for maintenance treatment of IPH, 60 mg/(m2·d) combined with prednisone orally, after the prednisone is gradually reduced until the drug is stopped, orally 6-MP maintenance treatment can reduce local adverse reactions: hydroxychloroquine: 6-10 mg/kg, twice a day
.
3 Intravenous human immunoglobulin is used for the treatment of the acute phase of IPH, which has the functions of neutralizing antigens, clearing immune complexes, and blocking antigen-antibody reactions
.
4 Deferramine, deferoxamine, has obvious toxic effects: adverse reactions to local medication, gastrointestinal tract, bone and central nervous system, etc.
It is not recommended for children to use it when it is not necessary
.
In addition to drug treatment, attention should also be paid to symptomatic support for IPH, timely oxygen inhalation, and extracorporeal membrane oxygenation for patients with circulatory and respiratory failure
.
Can IPH be transfused or iron supplemented? In children with acute or chronic recurrent anemia, iron supplementation and blood transfusion for severe anemia are the methods to correct anemia.
However, it is necessary to be aware of the risk of increased iron deposition in the lungs and should be used after fully weighing the pros and cons
.
The prognosis of IPH is related to timely diagnosis and treatment.
The prognosis of children is worse than that of adults.
Early active treatment can delay the process of pulmonary fibrosis and improve the prognosis
.
Only a small number of children with IPH can smoothly stop glucocorticoids.
Most children still have repeated episodes of pulmonary hemorrhage during or after the reduction of glucocorticoids.
Therefore, children with IPH need to be treated for a long time during treatment and after treatment is stopped.
Follow up
.
References: [1]IOACHIMESCU OC,SIEBER S,KOTCH A.
Idiopathic pulmonary haemosiderosis revisited[J].
European respiratory journal,2004,24(1):162-169.
[2],,.
Research progress of idiopathic pulmonary hemosiderin in children[J].
Journal of Pediatric Pharmacy,2019,25(09):52-57.
[3],.
Zhufutang Practical Pediatrics[M].
8 Edition.
Beijing: People's Medical Publishing House, 2015:1316-1320.
[4] Zeng Maojun, Shen Jie, Zeng Zhihui, Zhao Mingyi, Kuang Shoujin.
Three cases of childhood idiopathic pulmonary hemosiderinosis misdiagnosis and diagnosis ideas Analysis[J].
Chinese General Practice,2019,22(14):1751-1754.
[5]Sun Jialiang,Li Yanchun,Cheng Huanji.
Treatment progress of idiopathic pulmonary hemosiderin in children[J].
Chinese Journal of Practical Pediatrics 2019,34(05),429-433
Is this disease actually related to the lungs? Antecedent summary Dandan, a 2-year-old girl, was diagnosed with "iron deficiency anemia" at a local hospital 3 months ago because of her pale complexion
.
Due to severe anemia (58 g/L of hemoglobin), the local doctor gave red blood cell infusion and iron treatment, and his condition gradually improved, his hemoglobin rose to 90 g/L, and he was discharged
.
He continued to take the protein iron succinate oral solution (10 ml/time, 2 times/day) outside the hospital.
The hemoglobin rose to 108 g/L one month ago, and the parents stopped the medication by themselves
.
Recently, Dandan once again experienced symptoms such as pale complexion, loss of appetite, cough and sputum.
The local hospital found that hemoglobin was lowered again (55 g/L) and the condition was serious.
He immediately rushed to the higher-level hospital for treatment.
.
.
According to Dandan’s mother’s description Anemia during pregnancy, taking iron therapy, breastfeeding after birth, adding complementary foods (eggs, rice noodles, porridge) at 6 months, Dandanping is a picky eater, rarely eating meat, eggs, milk and vegetables, and there are exceptions.
Food addiction, like to lick iron
.
Because Dandan's anemia was severe, the doctor immediately gave him an infusion of red blood cells to correct the anemia, and at the same time perfect other related examinations
.
Figure 1 blood routine shows that Dandan is a small cell hypochromic anemia, with 9.
80% reticulocytes (normal range 0.
5%-1.
5%), serum iron is slightly lower, iron binding capacity is normal, bone marrow aspiration results show obvious red blood cell line proliferation, Granulocyte cell line and megakaryocyte cell line are normal
.
Dandanma has a history of anemia during pregnancy, and there is a lack of congenital hematopoietic materials.
In addition, Dandan is picky eaters, intake of meat, eggs, milk is low, and acquired hematopoietic materials are also insufficient.
Previous iron treatments are effective, so doctors consider the possibility of iron deficiency anemia
.
Dandan's cough and sputum symptoms were severe.
Chest X-ray film showed increased and thickened lung texture.
Enhanced lung CT showed that Dandan's lungs had ground-glass-like changes
.
Figure 2 combined with his severe anemia, at this time, he has been highly suspected of "pulmonary hemosiderinosis"
.
The doctor continued to search for evidence and found hemosiderin cells in sputum and gastric juice
.
Figure 3 Therefore, the revised diagnosis is "Pulmonary hemosiderinosis"
.
At this time, Dandan’s disease is in an advanced stage, and hemoptysis, fatal pulmonary hemorrhage, respiratory failure, multiple organ dysfunction, etc.
are likely to occur.
The doctor stopped iron supplementation and gave methylprednisolone sodium succinate intravenously.
Supported by oxygen inhalation and red blood cell infusion, Dandan's condition gradually stabilized after 1 week
.
Pulmonary hemosiderosis is mainly seen in children.
Idiopathic pulmonary hemosiderosis (IPH) is a relatively rare chronic lung disease caused by pulmonary capillary oozing, hemoglobin dissolution, and hemosiderin It is caused by the deposition of pulmonary elements, and the imaging manifestations of repeated hemoptysis, iron-deficiency anemia, and diffuse pulmonary infiltration make it a typical triad [1]
.
It can occur from newborn to adulthood, and it is mainly seen in children (especially 1 to 7 years old)
.
Five hypotheses about the etiology of IPH The etiology of IPH is still unclear.
It is currently believed to be related to autoimmunity, genes, allergies, environment, and infection factors, but none of the claims has been confirmed [2]
.
■ Environment—The hemolysin produced by Botrytis botryoides has a certain effect on the pathogenesis of IPH; ■ Heredity—Two siblings were diagnosed with IPH successively, and their serum Ig E was found to be significantly elevated; ■ Allergies—alveoli caused by milk allergy Anti-bovine milk autoantibodies were detected in the sera of children with hemorrhage; ■ Immune-antigen-antibody complex-mediated alveolar autoimmune damage, resulting in increased alveolar capillary permeability and hemorrhage of small pulmonary blood vessels; ■ Infection — suffering Children with pulmonary hemorrhage often have respiratory infections
.
What are the clinical manifestations of IPH? The clinical manifestations of IPH are diverse.
Common clinical symptoms are repeated coughing, hemoptysis, shortness of breath, anemia, etc.
The signs may include pale complexion, low breath sounds, tachycardia, hepatosplenomegaly, clubbing, and jaundice
.
Long-term repeated bleeding leads to thickening of the alveolar basement membrane, which can eventually lead to pulmonary interstitial fibrosis
.
According to the severity of the disease, it can be divided into three stages [3]
.
Table 1.
Clinical staging of IPH.
IPH that is often misdiagnosed.
IPH is often misdiagnosed due to its diverse clinical manifestations, insidious onset, lack of typical triad in some cases, and low incidence, lack of understanding of the disease by clinicians, often resulting in IPH being often misdiagnosed For the following diseases [4]: iron deficiency anemia thalassemia hemolytic anemia pneumonia with anemia pulmonary tuberculosis IPH diagnosis is exclusive, first of all to establish the existence of diffuse alveolar hemorrhage, has the following three characteristics: 1.
Hemoptysis, Hematemesis or old blood in infant gastric juice; 2.
Small cell hypochromic anemia; 3.
Chest radiograph or lung CT showing pulmonary hemorrhage-like changes, diffuse flocculent or ground-glass shadows in both lungs
.
The final diagnosis of IPH requires the following auxiliary examinations: Extracted from literature [2] The treatment of IPH is the current basic treatment for IPH, mainly including adrenal cortex hormones, immunosuppressants, antimalarial drugs, etc.
, with the purpose of quickly alleviating emergency and critical situations And maintenance treatment to improve the symptoms of patients [5]
.
1 Glucocorticoid acute intravenous hormone therapy, methylprednisolone 2 mg/(kg·d), intravenous drip or hydrocortisone 5-10 mg/(kg·d), intravenous drip; in acute alveolar hemorrhage, High-dose methylprednisolone 10~30 mg/(kg·d) shock therapy; after bleeding control, change to oral hormone and take prednisone 2 mg/(kg·d); after the symptoms are completely relieved (about 2~3 weeks) The dose is gradually reduced to the minimum maintenance dose (3-6 months); long-term low-dose glucocorticoid oral or inhalation treatment can reduce the risk of IPH recurrence and improve survival
.
2Immunosuppressive agents can be selected as second-line treatment drugs for glucocorticoid treatment ineffective and hormone dependence
.
Methotrexate: 2 mg/(kg·d), can be used in combination with hormones; mercaptopyrine: 1～2 mg/(kg·d) to 3～5 mg/(kg·d), the course of treatment is usually 1 Over years, it can effectively control symptoms and prevent acute attacks; 6-mercaptopurine: used for maintenance treatment of IPH, 60 mg/(m2·d) combined with prednisone orally, after the prednisone is gradually reduced until the drug is stopped, orally 6-MP maintenance treatment can reduce local adverse reactions: hydroxychloroquine: 6-10 mg/kg, twice a day
.
3 Intravenous human immunoglobulin is used for the treatment of the acute phase of IPH, which has the functions of neutralizing antigens, clearing immune complexes, and blocking antigen-antibody reactions
.
4 Deferramine, deferoxamine, has obvious toxic effects: adverse reactions to local medication, gastrointestinal tract, bone and central nervous system, etc.
It is not recommended for children to use it when it is not necessary
.
In addition to drug treatment, attention should also be paid to symptomatic support for IPH, timely oxygen inhalation, and extracorporeal membrane oxygenation for patients with circulatory and respiratory failure
.
Can IPH be transfused or iron supplemented? In children with acute or chronic recurrent anemia, iron supplementation and blood transfusion for severe anemia are the methods to correct anemia.
However, it is necessary to be aware of the risk of increased iron deposition in the lungs and should be used after fully weighing the pros and cons
.
The prognosis of IPH is related to timely diagnosis and treatment.
The prognosis of children is worse than that of adults.
Early active treatment can delay the process of pulmonary fibrosis and improve the prognosis
.
Only a small number of children with IPH can smoothly stop glucocorticoids.
Most children still have repeated episodes of pulmonary hemorrhage during or after the reduction of glucocorticoids.
Therefore, children with IPH need to be treated for a long time during treatment and after treatment is stopped.
Follow up
.
References: [1]IOACHIMESCU OC,SIEBER S,KOTCH A.
Idiopathic pulmonary haemosiderosis revisited[J].
European respiratory journal,2004,24(1):162-169.
[2],,.
Research progress of idiopathic pulmonary hemosiderin in children[J].
Journal of Pediatric Pharmacy,2019,25(09):52-57.
[3],.
Zhufutang Practical Pediatrics[M].
8 Edition.
Beijing: People's Medical Publishing House, 2015:1316-1320.
[4] Zeng Maojun, Shen Jie, Zeng Zhihui, Zhao Mingyi, Kuang Shoujin.
Three cases of childhood idiopathic pulmonary hemosiderinosis misdiagnosis and diagnosis ideas Analysis[J].
Chinese General Practice,2019,22(14):1751-1754.
[5]Sun Jialiang,Li Yanchun,Cheng Huanji.
Treatment progress of idiopathic pulmonary hemosiderin in children[J].
Chinese Journal of Practical Pediatrics 2019,34(05),429-433
