Pulmonary Hypertension and Polycythemia Secondary to Pulmonary Alveolar Microlithiasis Treated with Sequential Bilateral Lung Transplant: A Case Study and Literature Review.

Autor: Alrossais NM; College of Medicine, Alfaisal University, Riyadh, Saudi Arabia., Alshammari AM; College of Medicine, Alfaisal University, Riyadh, Saudi Arabia.; Department of Thoracic Surgery, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia., Alrayes AM; College of Medicine, Alfaisal University, Riyadh, Saudi Arabia., Mohammad N; College of Medicine, Alfaisal University, Riyadh, Saudi Arabia., Al-Amoodi MJH; The School of Medicine and Health Sciences, George Washington University, Washington, DC, USA., Almutairi AM; Department of Medicine, Security Forces Hospital, Riyadh, Saudi Arabia., Alsuhaymi AO; Department of Emergency, King Faisal General Hospital, Makkah, Saudi Arabia., Alhadid DA; College of Medicine, Alfaisal University, Riyadh, Saudi Arabia., Alhammad FA; College of Medicine, Alfaisal University, Riyadh, Saudi Arabia., Ouf NH; College of Medicine, Alfaisal University, Riyadh, Saudi Arabia., Ahmed MH; Department of Thoracic Surgery, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.; Department of Cardiothoracic Surgery, Ain Shams University, Cairo, Egypt., Saleh W; Department of Thoracic Surgery, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia., AlAmodi AA; College of Medicine, Alfaisal University, Riyadh, Saudi Arabia.
Jazyk: angličtina
Zdroj: The American journal of case reports [Am J Case Rep] 2019 Jul 28; Vol. 20, pp. 1114-1119. Date of Electronic Publication: 2019 Jul 28.
DOI: 10.12659/AJCR.911045
Abstrakt: BACKGROUND Pulmonary alveolar microlithiasis is an autosomal recessive disease in which a mutation in the SLC34A2 gene that codes for a sodium phosphate type IIb transporter protein (expressed in human epithelial tissues and functions in the clearance of phosphate ions) leads to the formation of extensive pulmonary intra-alveolar microliths. The subsequent characteristic clinical features of dyspnea and hypoxia are a manifestation of these microliths. There have been fewer than 1000 cases of pulmonary alveolar microlithiasis reported worldwide, and there have been 19 reported lung-transplanted patients. CASE REPORT A 49-year-old Saudi male patient presented with longstanding history of easy fatigability and tiredness on exertion since he was 16 years old. Throughout his follow-up in different hospitals (1986-1989), tuberculosis and pulmonary fibrosis were suspected. The patient was lost to follow-up between 1989 and 2001. In 2002, he presented to the emergency room with coughing, shortness of breath on exertion, abdominal swelling, and pedal edema. An investigation with chest x-rays, CT scan, electrocardiogram, and an echocardiogram was conducted. After referral to a tertiary care center, the patient was diagnosed with pulmonary alveolar microlithiasis. He subsequently developed pulmonary hypertension and polycythemia and therefore received a bilateral lung transplant in 2016. Following the lung transplant, he developed a mild reperfusion injury and tonic-clonic seizures, requiring ICU admission. After a successful extubatation with stable vitals and good recovery, he was discharged home in stable condition with planned follow-up. CONCLUSIONS We report a case of pulmonary alveolar microlithiasis successfully treated with a bilateral lung transplant. Although pulmonary alveolar microlithiasis is a rare entity, healthcare providers should consider it in the differential diagnoses of parenchymal lung diseases and differentiate it from tuberculosis and pulmonary fibrosis.
Databáze: MEDLINE