PDF(Pubmed)
Abstract：
UNASSIGNED: Pulmonary alveolar microlithiasis (PAM) is a rare disease whose clinical and imaging manifestations are non-specific, characterized by the deposition of microliths, which primarily consist of calcium and phosphorus, within the alveoli. In the cases of PAM, patients combined with calcification of other organs such as gastric mucosal calcification are less common.
UNASSIGNED: A 59-year-old woman was admitted to our hospital due to cough producing white, foamy sputum, accompanied by dyspnea and fever for 20 days. The CT scan showed diffuse ground-glass opacities and calcification of the gastric mucosa. Lung tissue biopsy revealed the presence of calcification and granulomatous foreign bodies in the interstitium and alveolar cavity. In the later stages, she developed painful skin petechiae. For this patient, the diagnosis of PAM, gastric mucosal calcification, and purpura fulminans was made. However, the genetic test results hinted that the patient and her son had a heterozygous mutation in the FBN1 gene, but her daughter\'s genetic test results were normal. Although the patient received anti-infection treatment, steroids, and oxygen therapy, her condition did not improve.
UNASSIGNED: We reported a rare case of PAM combined with calcification of other organs and purpura fulminans. Treatment of steroids did not show any benefit. The causative mechanism and effective treatment of this disease remain unclear. More treatments need to be explored.
UNASSIGNED: A 59-year-old woman was admitted to our hospital due to cough producing white, foamy sputum, accompanied by dyspnea and fever for 20 days. The CT scan showed diffuse ground-glass opacities and calcification of the gastric mucosa. Lung tissue biopsy revealed the presence of calcification and granulomatous foreign bodies in the interstitium and alveolar cavity. In the later stages, she developed painful skin petechiae. For this patient, the diagnosis of PAM, gastric mucosal calcification, and purpura fulminans was made. However, the genetic test results hinted that the patient and her son had a heterozygous mutation in the FBN1 gene, but her daughter\'s genetic test results were normal. Although the patient received anti-infection treatment, steroids, and oxygen therapy, her condition did not improve.
UNASSIGNED: We reported a rare case of PAM combined with calcification of other organs and purpura fulminans. Treatment of steroids did not show any benefit. The causative mechanism and effective treatment of this disease remain unclear. More treatments need to be explored.
摘要：
■肺泡微石症（PAM）是一种罕见的疾病，其临床和影像学表现是非特异性的，以微石沉积为特征，主要由钙和磷组成，在肺泡内。在PAM的情况下，合并其他器官钙化如胃粘膜钙化的患者较少见。
一名59岁女子因咳嗽产生白色而入院,泡沫痰，伴有呼吸困难和发热20天。CT扫描显示弥漫性磨玻璃混浊和胃粘膜钙化。肺组织活检显示间质和肺泡腔中存在钙化和肉芽肿异物。在后期阶段,她出现了疼痛的皮肤瘀斑。对这个病人来说,PAM的诊断，胃粘膜钙化，制成了紫癜。然而,基因检测结果表明，患者和她的儿子在FBN1基因中存在杂合突变，但她女儿的基因检测结果正常。虽然患者接受了抗感染治疗,类固醇,和氧疗,她的情况没有改善。
■我们报道了一例罕见的PAM合并其他器官钙化和暴发性紫癜的病例。类固醇治疗没有任何益处。该病的致病机制和有效治疗方法尚不清楚。需要探索更多的治疗方法。
一名59岁女子因咳嗽产生白色而入院,泡沫痰，伴有呼吸困难和发热20天。CT扫描显示弥漫性磨玻璃混浊和胃粘膜钙化。肺组织活检显示间质和肺泡腔中存在钙化和肉芽肿异物。在后期阶段,她出现了疼痛的皮肤瘀斑。对这个病人来说,PAM的诊断，胃粘膜钙化，制成了紫癜。然而,基因检测结果表明，患者和她的儿子在FBN1基因中存在杂合突变，但她女儿的基因检测结果正常。虽然患者接受了抗感染治疗,类固醇,和氧疗,她的情况没有改善。
■我们报道了一例罕见的PAM合并其他器官钙化和暴发性紫癜的病例。类固醇治疗没有任何益处。该病的致病机制和有效治疗方法尚不清楚。需要探索更多的治疗方法。
