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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

It is well known that there is a close correlation between blood viscosity and blood flow. To clarify any relationship between blood viscosity and regional cerebral blood flow (rCBF) in the elderly, we simultaneously studied both CBF with PET (positron emission tomography) and blood viscosity with viscosimeter before and after phlebotomy in the elderly with various kinds of polycythemia. These subjects consisted of five male cases of secondary polycythemia due to pulmonary fibrosis, one male case of essential erythrocytosis (average age 66.6 +/- 4.6 years old) and one female case of stress polycythemia (47 years old). Before phlebotomy an increase in blood viscosity, decrease in rCBF and regional cerebral metabolic rate of oxygen (rCMRO2) were observed in all cases. After phlebotomy (total amount of 800 to 1,000 ml) blood viscosity rapidly decreased, and both rCBF and rCMRO2 tended to increase. There was a significant negative or positive correlation between CBF and blood viscosity or rCMRO2, respectively. However, no increase in cerebral oxygen transport was observed in any subject after phlebotomy. It was noted that cerebral infarction is not infrequent among elderly visitors to Kusatsu spa, which is characterized by high temperature hot spring water. From the authors' observation of 23 cases of cerebral infarction encountered during the last five years, it is noteworthy that the disease tended to occur more frequently during midnight to morning, specially 3:00 to 6:00. Thus, to clarify the pathogenetic mechanism of the cerebral infarction occurring after bathing in hot spring water, we studied the changes in blood viscosity, blood pressure and coagulation-fibrinolytic system after bathing in hot spring water.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Blood viscosity and cerebral blood flow in aged]. 833 18

At least 31 cases of familial fibrocystic pulmonary dysplasia, within 10 families, have been described in the world literature. The mode of genetic transmission of this disease, however, has been uncertain until now. The author observed three unequivocal and five probable cases of familial fibrocystic pulmonary dysplasia among 56 members of one family. Diagnostic criteria included progressive dyspnea and cyanosis, digital clubbing, pulmonary hypertension, negative sweat tests, polycythemia, arterial hypoxia and hypocapnia, chest radiographs showing diffuse bilateral pulmonary fibrosis, and diffuse fibrocystic pulmonary dysplasia at postmortem examination (two cases). Among the three unequivocal cases one father-to-son transmission was observed. Non-sex-linked dominant transmission of familial fibrocystic pulmonary dysplasia is thereby proved for the first time. One patient also developed a bronchial carcinoma in addition to fibrocystic pulmonary dysplasia; this is considered to be a cause-and-effect relationship and not a coincidental complication.
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PMID:FAMILIAL FIBROCYSTIC PULMONARY DYSPLASIA: OBSERVATIONS IN ONE FAMILY. 1427 97

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.
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PMID:Pulmonary Hypertension and Polycythemia Secondary to Pulmonary Alveolar Microlithiasis Treated with Sequential Bilateral Lung Transplant: A Case Study and Literature Review. 3135 64