{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T15:14:45Z","timestamp":1770822885122,"version":"3.50.1"},"reference-count":21,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2005,6,17]],"date-time":"2005-06-17T00:00:00Z","timestamp":1118966400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2005,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Niemann\u2010Pick disease (NPD) is a rare, autosomal\u2010recessively inherited lipid storage disease which is characterized by intracellular deposition of sphingomyelin in various body tissues. The disease is heterogeneous and classified into six groups. Pulmonary parenchymal involvement may be a feature of several subtypes of NPD, including type B. Progressive pulmonary involvement in NPD type B is a major cause of morbidity and mortality. It is usually diagnosed at older ages. Only a few cases with early pulmonary involvement have been reported. In this report, a patient with NPD type B, hospitalized with the diagnosis of pneumonia at age 3 months, is presented. Following treatment for pneumonia, she continued to have persistent respiratory symptoms and became oxygen\u2010dependent. High\u2010resolution computed tomography of the chest revealed diffuse interstitial changes. During follow\u2010up, the patient developed hepatosplenomegaly. Lung, liver, and bone marrow biopsies showed characteristic findings for NPD. Biochemical studies also confirmed the diagnosis, and the sphingomyelinase enzyme level of the patient was low. Unilateral lung lavage was performed in order to decrease lipid storage as a treatment modality. However, there was no clinical or radiological improvement. The patient died at age 15 months due to progressive respiratory failure. Pulmonary involvement is a rare entity in early childhood in patients with NPD type B, but should be considered in the differential diagnosis of persistent interstitial lung disease. It may cause progressive respiratory failure, but the treatment options remain limited. Pediatr Pulmonol. 2005; 40:169\u2013172. \u00a9 2005 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ppul.20248","type":"journal-article","created":{"date-parts":[[2005,6,17]],"date-time":"2005-06-17T20:20:22Z","timestamp":1119039622000},"page":"169-172","source":"Crossref","is-referenced-by-count":24,"title":["Early pulmonary involvement in Niemann\u2010Pick type B disease: Lung lavage is not useful"],"prefix":"10.1002","volume":"40","author":[{"given":"Z.S.","family":"Uyan","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B.","family":"Karada\u011f","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Ersu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"K\u0131yan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"Kotilo\u011flu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Sirvanc\u0131","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Feriha","family":"Ercan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"Da\u011fl\u0131","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F.","family":"Karako\u00e7","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"Da\u011fl\u0131","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,6,17]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1053\/rmed.2000.0942"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.295.6610.1375"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1023\/B:BOLI.0000009950.81514.c8"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.165.1.2103113"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1159\/000196305"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0344-0338(89)80006-8"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1097\/00062752-200001000-00009"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1097\/00004728-199611000-00023"},{"key":"e_1_2_1_10_2","first-page":"837","article-title":"An autopsy case of Niemann\u2010Pick disease","volume":"23","author":"Kawai K","year":"1973","journal-title":"Acta Pathol Jpn"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00447373"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01813906"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(86)80841-1"},{"key":"e_1_2_1_14_2","first-page":"139","article-title":"Lipidosis with sea blue histiocytes: report of two siblings with lung involvement","volume":"36","author":"G\u00f6\u011f\u00fcs S","year":"1994","journal-title":"Turk J Pediatr"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2001.113103"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1002\/path.1711060208"},{"key":"e_1_2_1_17_2","first-page":"1017","article-title":"Niemann\u2010Pick disease: ultrastructural features","volume":"37","author":"Santhl P","year":"2000","journal-title":"Indian Pediatr"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.94.07020409"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0248-8663(99)80109-X"},{"key":"e_1_2_1_20_2","first-page":"267","article-title":"Thoracic computed tomography data and cytology of bronchoalveolar lavage fluid in an adult case of Niemann\u2010Pick disease. The physiognomy of the disease in Tunisia","volume":"7","author":"Bouziani A","year":"1990","journal-title":"Rev Mal Respir"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1259\/bjr.74.887.741059"},{"key":"e_1_2_1_22_2","doi-asserted-by":"publisher","DOI":"10.1038\/labinvest.3780311"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fppul.20248","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.20248","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,16]],"date-time":"2023-11-16T20:17:12Z","timestamp":1700165832000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.20248"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,6,17]]},"references-count":21,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2005,8]]}},"alternative-id":["10.1002\/ppul.20248"],"URL":"https:\/\/doi.org\/10.1002\/ppul.20248","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2005,6,17]]}}}