Pulmonary Alveolar Proteinosis: Case Report of Rare Diffuse Lung Disorder in Pediatric Age Group

被引:2
作者
Gandhi, Vipulkumar [1 ]
Kadam, Shashank [1 ]
机构
[1] KEM Hosp, Dept Pediat, Pune 411011, Maharashtra, India
关键词
Bronchoalveolar lavage; diffuse alveolar infiltrate; pulmonary alveolar proteinosis; CHILDREN;
D O I
10.4103/ijrc.ijrc_130_21
中图分类号
R56 [呼吸系及胸部疾病];
学科分类号
摘要
Nonspecific symptoms and variable clinical course are a few of the hurdles in diagnosing pulmonary alveolar proteinosis (PAP). Lack of accessible health care and efficient health infrastructure, including diagnostic and treatment facilities, are the major challenges for early detection and prompt management of PAP in developing countries such as India. A 6-month-old child was brought to the tertiary health care center for cough, dyspnea, and intermittent fever. The history of third-degree consanguineous parents was evident. The patient had a history of evolving respiratory complaints at the age of 4 months, for which she was hospitalized for 12 days. There was a relapse of similar symptoms within 2 weeks of discharge and required readmission. The patient was cyanosed with signs of severe respiratory distress. Chest X-ray revealed bilateral diffuse alveolar infiltration. High-resolution computed tomography imaging showed diffuse interstitial thickening with adjacent ground-glass opacities along with crazy-pavement appearance involving both lungs. With flexible bronchoscopy, bronchoalveolar lavage (BAL) was performed. Congenital PAP was confirmed with periodic acid-Schiff -positive proteinaceous extracellular globules on smear. The child was intubated and mechanically ventilated during a hospital stay to treat uncontrollable respiratory failure. The child succumbed on the 28th day of admission despite repeated therapeutic BAL procedures and systemic corticosteroids. The possibility of missed/delayed diagnosis of PAP is widespread in resource-limited health-care settings. Postnatal onset of PAP should be suspected in every child with chronic respiratory distress and failure to thrive with diffuse alveolar infiltrates.
引用
收藏
页码:169 / 172
页数:4
相关论文
共 11 条
  • [1] A newly identified novel variant in the CSF2RA gene in a child with pulmonary alveolar proteinosis: a case report
    Al-Haidary A.S.
    Alotaibi W.
    Alhaider S.A.
    Al-Saleh S.
    [J]. Journal of Medical Case Reports, 11 (1)
  • [2] Whole lung lavage therapy for pulmonary alveolar proteinosis: a global survey of current practices and procedures
    Campo, Ilaria
    Luisetti, Maurizio
    Griese, Matthias
    Trapnell, Bruce C.
    Bonella, Francesco
    Grutters, Jan
    Nakata, Koh
    Van Moorsel, Coline H. M.
    Costabel, Ulrich
    Cottin, Vincent
    Ichiwata, Toshio
    Inoue, Yoshikazu
    Braschi, Antonio
    Bonizzoni, Giacomo
    Iotti, Giorgio A.
    Tinelli, Carmine
    Rodi, Giuseppe
    [J]. ORPHANET JOURNAL OF RARE DISEASES, 2016, 11
  • [3] de Blic Jacques, 2004, Paediatr Respir Rev, V5, P316
  • [4] Garg G, 2009, INDIAN PEDIATR, V46, P521
  • [5] Congenital Pulmonary Alveolar Proteinosis
    Hammami, Saber
    Harrathi, Khaled
    Lajmi, Khaled
    Hadded, Samir
    BenMeriem, Chebil
    Guediche, Andmohamed Neji
    [J]. CASE REPORTS IN PEDIATRICS, 2013, 2013
  • [6] Pulmonary alveolar proteinosis in children: An unusual presentation with significant clinical impact
    Iyengar, Jayaram N.
    Reddy, Bharath Kumar K. R.
    [J]. INDIAN JOURNAL OF PATHOLOGY AND MICROBIOLOGY, 2018, 61 (03) : 418 - 420
  • [7] Anti-GM-CSF antibodies in paediatric pulmonary alveolar proteinosis
    Latzin, P
    Tredano, M
    Wüst, Y
    de Blic, J
    Nicolai, T
    Bewig, B
    Stanzel, F
    Köhler, D
    Bahuau, M
    Griese, M
    [J]. THORAX, 2005, 60 (01) : 39 - 44
  • [8] Pulmonary alveolar proteinosis: from classification to therapy
    Salvaterra, Elena
    Campo, Ilaria
    [J]. BREATHE, 2020, 16 (02) : 1 - 12
  • [9] Tabatabaei SA, 2010, J RES MED SCI, V15, P120
  • [10] Congenital pulmonary alveolar proteinosis related to a surfactant protein B deficiency: Report of two cases
    Verhasselt-Crinquette, Marie
    Franquet-Ansart, Helene
    Rakza, Thameur
    Storme, Laurent
    Copin, Marie-Christine
    Devisme, Louise
    [J]. ANNALES DE PATHOLOGIE, 2009, 29 (06) : 481 - 484