КЛИНИЧЕСКИЕ СЛУЧАИО стрый лимфобластный лейкоз -наиболее частое онкологическое заболевание детского возраста. Данная патология характеризуется неконтролируемым ростом незрелых клеток-предшественников в костном мозге с последующей лейкемизацией органов и тканей. Врожденный лимфобластный лейкоз чрезвычайно редко регистрируется у новорожденных (4,3-8,6 случая на 1 млн новорожденных), составляя менее 1% всех случаев лейкемии у детей [1,2]. Пик заболеваемости острым лейкозом приходится на возраст 2-4 года. Такая высокая частота в данной возрастной группе -следствие физиологической пролиферации лимфоидной ткани в процессе становления иммунитета [3,4]. К характерным особенностям врожденного лейкоза относятся быстрое течение, клинически агрессивный характер и отсутствие спонтанной ремиссии, заставляющие предположить развитие заболевания еще в фетальном периоде.В развитии острого лимфобластного лейкоза придается значение химерному гену ТЕL/AML, формирование которого происходит внутриутробно в результате спонтанных ошибок в процессе репликации ДНК. Транслокация реаранжированного гена MLL, локализующегося на хромосоме 11q 23, выявляется у 68-81% больных детей первого года жизни и ассоциируется с плохим исходом [5][6][7]. То, что патогенез острых лейкозов связан с генетическими поломками, подтверждается обнаружением различных хромосомных аберраций (транслокаций, делеций, инверсий и т.д.) практически у всех больных острым лей-
Background: Interstitial lung disease (ILD) is one of the most difficult conditions in pulmonology due to difficulties in diagnosing, classifying, and treating this condition. They require invasive approaches to diagnose (e.g., lung biopsy), non-applicable methods (e.g., lung function tests in newborns), or potentially non-accessible methods (e.g., genetic testing in not-well-equipped facilities, and several weeks are required for results to be announced). They represent a heterogeneous group of diseases in which the alveolar epithelium, parenchyma, and capillaries of the lungs are damaged, which leads to changes in the pulmonary interstitium, proliferation of connective tissue, and thickening of the alveolar-capillary membranes and alveolar septa. These changes are accompanied by impaired oxygen diffusion, progressive respiratory failure, and radiographic signs of bilateral dissemination. Although adult and child classifications for ILD have evolved over the years, classification for ILD in neonates remains a challenge. Case presentation: Here we discuss ILD in neonates briefly, and report two rare cases of ILD (a male white neonate, two-day-old with fibrosing alveolitis, and another male white neonate, one-day old with desquamative interstitial pneumonitis), with these diagnoses initially thought to be presented only in adulthood. Lung biopsy and histopathological findings of the two neonates have shown mononuclear cells in the alveolar spaces, and thickening of the alveolar walls confirmed the diagnosis of fibrosing alveolitis in one neonate, and desquamation of the large mononuclear cells in the intra-alveolar space in the other neonate, with the diagnosis of desquamative interstitial pneumonitis being confirmed. Interstitial lung disease lacks a consensus guideline on classification and diagnosis in neonates, rendering it one of the greatest challenges to pediatricians and neonatologists with remarkable morbidity and mortality rates. Conclusions: Fibrosing alveolitis and desquamative interstitial pneumonitis (DIP) are not adult-only conditions, although rare in neonates, histopathological examination and clinical practice can confirm the diagnosis. Based on our clinical practice, prenatal and maternal conditions may serve as potential risk factors for developing IDL in neonates, and further studies are needed to prove this hypothesis.
The article describes a clinical case of idiopathic fibrosing alveolitis – a rare antenatal disease of a newborn. The disease began with the clinical manifestations of transient tachypnea in a full-term child born in the operative way. Chest X-ray was used for dynamic control of lung tissue damage. Due to the absence of specific clinical symptoms of interstitial lung damage, the disease was considered as a course of congenital pneumonia. The researchers used antibacterial and symptomatic therapy. Despite treatment, the respiratory failure progressed, resulting in fatal outcome.
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