Modern Pediatrics. Ukraine. 7(111): 72-80. doi 10.15574/SP.2020.111.72
Tsymbalista O. L.
Ivano-Frankivsk National Medical University, Ukraine

For citation: Tsymbalista OL. (2020). Intra-alveolar hemorrhage in certain orphan lung diseases in children (lecture). Modern Pediatrics. Ukraine. 7(111): 72-80. doi 10.15574/SP.2020.111.72
Article received: Aug 22, 2020. Accepted for publication: Nov 07, 2020.

The theme is relevant due to the diagnostic difficulties, severe clinical course and prognosis of idiopathic pulmonary hemosiderosis and Goodpasture syndrome. Idiopathic pulmonary hemosiderosis and Goodpasture syndrome are severe, life-threatening immunopathologic diseases due to alveolar hemorrhage and a hundred percent mortality within a short period of time after the onset of clinical manifestations. Idiopathic pulmonary hemosiderosis generally occurs in children at the age of 3–8 years as a separate condition, or as a stage of Goodpasture syndrome. It manifests itself as shortness of breath, pneumonia, prune juice sputum, hemoptysis, hemorrhage. During exacerbation, the patients' condition is determined by the degree of pulmonary hemorrhage, pulmonary heart disease, acute posthemorrhagic anemia. The exacerbation lasts from a few hours to 1–2 weeks. The duration of each episode and remission varies among patients being unpredictable. Each new exacerbation is more severe.
In Goodpasture syndrome, predominant pulmonary and renal vascular lesions of autoimmune nature are observed. It affects young males more frequently; is rare in children. Hemorrhagic alveolitis as a form of lung damage develops first; then, the kidneys are involved, and anemia occurs. Glomerulonephritis (GN) manifests itself as nephrotic syndrome with rapid progression of kidney failure. In case of the predominant pulmonary pathology, recurrent hemoptysis and pulmonary hemorrhage are observed; in end-stage disease with cardiopulmonary failure manifestations, rapidly progressive GN and kidney failure develop. The second variant of Goodpasture syndrome is characterized by relatively slow progression of pulmonary changes and renal lesions. Goodpasture syndrome is rarely accompanied by GN from the onset to the end of the disease and pulmonary pathology manifests itself at the terminal phase of the disease. The treatment of both diseases includes lifetime therapy with glucocorticoids, cytostatics. Pulse therapy using these preparations, discrete plasma exchange and intravenous immunoglobulin administration, syndromic treatment are carried out.
No conflict of interest was declared by the authors.
Keywords: children, idiopathic pulmonary hemosiderosis, Goodpasture syndrome, clinical course, therapy.

REFERENCES

1. Borodina LV. (2018). A case of late diagnosis of Goodpasture's syndrome with the leading syndrome of hematuria. Journal of Young Scientist. 1: 43-47.

2. Bush A, Cunningham S, de Blic J et al. (2015). European protocol for the diagnosis and initial treatment of interstitial lung disease in children. Thorax. 70(11): 1078-1084. https://doi.org/10.1136/thoraxjnl-2015-207349; PMid:26135832

3. Chernyaev AL, Samsonova MV. (2005). Diffuse alveolar lesion: the etiology, pathogenesis, and pathological anatomy. Pulmonology. 4: 65-69. https://doi.org/10.15360/1813-9779-2005-5-13-16

4. Chernyakhovskaya NE, Korzheva IYu, Povalyaso AV et al. (2010). Etiopathogenesis of pulmonary hemorrhage. Pulmonology. 4: 106–109.

5. Chuchalin AG. (2010). Diffuse alveolar hemorrhage. Pulmonology. 3: 5–8.

6. Chuchalin AG. (2017). Respiratory medicine: a guideline. Moscow: Littera. 3: 464.

7. Chuchalin AG. (2018). Pulmonology: a national guideline. Moscow: GEOTAR-Media. 800.

8. Collard HR, Schwarz MI. (2004). Diffuse alveolar hemorrhage. Clin Chest Med. 25(3): 583-592. https://doi.org/10.1016/j.ccm.2004.04.007; PMid:15331194

9. Dudov TR, Borodina LV, Yagoda AV. (2017). Goodpasture syndrome. Journal of Young Scientist. 4: 17-24.

10. Fulmer JJ, Langston C, Dishop MK et al. (2005). Pulmonary capillaritis in children: a review of eight cases with companion to other alveolar hemorrhage syndromes. J Pediatr. 146(3): 376-381. https://doi.org/10.1016/j.jpeds.2004.10.025; PMid:15756223

11. Gavrisyuk VK. (2016). An essay on clinical pulmonology. Kyiv. 336.

12. Hagag AA, Haman MA, Taha OA et al. (2015). Therapeutic efficacy of different iron chelators in Egyptian children with Beta Thalassemia with iron overload. Infect Disord Drug Targets. 15(2): 98-105. https://doi.org/10.2174/1871526515666150724111721; PMid:26205801

13. Ilkovich MM. (2016). Interstitial and orphan lung diseases. Moscow: GEOTAR-Media. 560.

14. Jensen PD, Jensen FT, Christensen T et al. (2003). Evaluation of myocardial iron by magnetic resonance imaging during iron chelation therapy with deferrioxamine: indication of close relation between myocardial iron content and chelatable iron pool. Blood. 101(11): 4632-4639. https://doi.org/10.1182/blood-2002-09-2754; PMid:12576333

15. Jindal SK, Agarwal R. (2005). Autoimmunity and interstitial lung disease. Curr Opin Pulm Med. 11(5): 438-446. https://doi.org/10.1097/01.mcp.0000170522.71497.61; PMid:16093819

16. Martinez DPM, Sivasothy P. (2009). Vasculitis of the upper and lower airway. Best Pract Res Clin Rheumatol. 23(3): 403-417. https://doi.org/10.1016/j.berh.2009.02.003; PMid:19508947

17. Mayaud C, Fartoukh M, Prigent H et al. (2005). Acute forms of diffuse interstitial hypoxemic pneumonia in immunocompetent patients. Rev Pneumol Clin. 61(2): 70-77. https://doi.org/10.1016/S0761-8417(05)84792-3

18. Ovsyannikov DYu, Ilyinskaya AS, Akhvlediani SD et al. (2015). Pulmonary-renal syndrome in children: clinical observation of granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) and Goodpasture syndrome. Journal «Pediatria» Named after G.N. Speransky. 94(4): 43-57.

19. Phillip R, Lugmani R. (2008). Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol. 26(5 Suppl 51): 94-104.

20. Porter JB, Evangeli M, El-Beshlawy A. (2011). Challenges of adherence and persistence with iron chelation therapy. Int J Hematol. 94(5): 453-460. https://doi.org/10.1007/s12185-011-0927-3; PMid:21993873

21. Rozinova NN, Mizernitskiy YuL. (2015). Orphan lung diseases in children. Moscow: Publishing House «Medpraktika-M»: 240.

22. Severgin VE, Shipulin PP, Agrakhari A. (2015). Possibilities of endovascular hemostasis in treatment of pulmonary hemorrhage. Clinical Surgery. 6: 46-48.

23. Shah NR. (2017). Advances in iron chelation therapy: transitioning to a new oral formulation. Drugs Context. 6: 212-502. https://doi.org/10.7573/dic.212502; PMid:28706555 PMCid:PMC5499896

24. Smetanina NS. (2019). Patients' satisfaction with dosage formulation and chelation therapy effectiveness. Journal «Pediatria» Named after G.N. Speransky. 98(4): 217-220. https://doi.org/10.24110/0031-403X-2019-98-4-217-220

25. Taher AT, Origa R, Perrotta S et al. (2017). New film-coated tablet formulation of deferasirox is well tolerated in patients with thalassemia or lower-risk MDS: Results of the randomized, phase II ECLIPSE study. Am J Hematol. 92(5): 420-428. https://doi.org/10.1002/ajh.24668; PMid:28142202 PMCid:PMC6585741

26. Tsymbalista OL. (2019). Pediatric pulmonology. Kyiv: All-Ukrainian Specialized Publishing House «Medicine». 600.

27. Tsymbalista OL, Melnyk TM, Velychkovych RS. (2008). Goodpasture syndrome: current views on the pathogenesis, clinical course, diagnosis and treatment (a case report). Sovremennaya Pediatriya. 1: 173-176.

28. Vladimirova EB, Shmelev EI, Stepanyan IE et al. (2018). Interstitial lung disease: search for the cause (a clinical case). Pulmonology. 28(4): 490-495. https://doi.org/10.18093/0869-0189-2017-28-4-490-495

29. Voronzhev IO. (2014). Fluoroscopic image of pulmonary hemorrhage in young children with perinatal injuries. Problems of Interrupted Medical Training and Science. 4: 27-31.

30. Wanko SO, Broadwater G, Folz RJ et al. (2006). Diffuse alveolar hemorrhage: retrospective review of clinical outcome in allogeneic transplant recipients treated with aminocaproic acid. Biol Blood Marrow Transplant. 12(9): 949-953. https://doi.org/10.1016/j.bbmt.2006.05.012; PMid:16920561

31. Yakovlev VN, Khayrutdinov ER, Arablinskiy AV et al. (2015). Current opportunities of endovascular diagnosis and treatment of pulmonary hemorrhage. Pulmonology. 25(2): 217-223. https://doi.org/10.18093/0869-0189-2015-25-2-217-223

32. Yassin MA, Soliman AT, de Sanctis V. (2018). Jadenu® Substituting Exjade® in Iron Overloaded β-Thalassemia Major (BTM) Patients: A Preliminary Report of the Effects on the Tolerability, Serum Ferritin Level, Liver Iron Concentration and Biochemical Profiles. Mediterr J Hematol Infect Dis. 10(1): e2018064. https://doi.org/10.4084/mjhid.2018.064; PMid:30416696 PMCid:PMC6223544

33. Yoon W, Kim JK, Kim H et al. (2002). Bronchial and nonbronchial systemic artery embolization for life-threatening hemoptysis: a comprehensive review. Radiographics. 22(6): 1395-1409. https://doi.org/10.1148/rg.226015180; PMid:12432111