Respiratory profile in patients after liver transplantation

Patients in the immediate post-operative liver transplantation (LxT) period can develop respiratory and functional complications. In the postoperative months, these functions tend to improve. Nevertheless, there are few studies that evaluate precisely and specifically respiratory function in post-LxT long-term after surgery. The objective of the study was to describe the respiratory profile of patients 1 to 6 months after LxT, accompanied by LxT outpatients. Methods. We included patients between 25 and 60 years old. We excluded patients with chronic renal or cerebrovascular impairment, severe heart disease, and history of lung surgery or liver re-transplantation. Evaluations were carried out on 3 occasions: 1 month, 3 months, and 6 months after LxT. The following evaluations were submitted: respiratory muscle strength (manuvacuometer), value flows and lung volumes (spirometer), and surface electromyography analyzing root mean square in the right (RMS-R) and left (RMS-L) diaphragm. We analyzed MELD (Model for End-Stage Liver Disease). After normality tests, we used the Friedman test (non-parametric values) and ANOVA (parametric values), P >= .5 with the use of SPSS 21.0. Results. Patients (n = 15) had a mean age of 53.0 +/- 7.5 years and 25.9 +/- 4.6 MELD score. The statistically significant value obtained at the 3 occasions of evaluation was RMS-R, with a decline during periods of evaluation. This can be caused by removal of the liver, resulting in a denervation and reduction in compliance of this portion of the muscle. Conclusions. Patients between 1 and 6 months after transplantation have a specific respiratory profile, close to normal values. However, there are few studies on this subject, and we suggest that more research be done.Patients in the immediate post-operative liver transplantation (LxT) period can develop respiratory and functional complications. In the postoperative months, these functions tend to improve. Nevertheless, there are few studies that evaluate precisely and4872352235514th Brazilian Transplantation Association (ABTO) and 14th Luso-Brazilian Transplantation Congress together with the 13th Transplantation Nursing Meeting and Histocompatibility Forumde 24 a 27 de outubroGramado, Brasi

Lysosomal acid lipase deficiency leading to liver cirrhosis: a case report of a rare variant mutation

Lysosomal acid lipase deficiency is a poorly diagnosed genetic disorder, leading to accumulation of cholesterol esters and triglycerides in the liver, with progression to chronic liver disease, dyslipidemia, and cardiovascular complications. Lack of awareness on diagnosis of this condition may hamper specific treatment, which consists on enzymatic replacement. It may prevent the progression of liver disease and its complications. We describe the case of a 53-year-old Brazilian man who was referred to our center due to the diagnosis of liver cirrhosis of unknown etiology. He was asymptomatic and had normal body mass index. He had dyslipidemia, and family history of myocardial infarction and stroke. Abdominal imaging tests showed liver cirrhosis features and the presence of intrahepatic calcifications. Initial investigation of the etiology of the liver disease was not elucidated, but liver biopsy showed microgoticu-lar steatosis and cholesterol esters deposits in Kuppfer cells. The dosage of serum lysosomal acid lipase was undetectable and we found the presence of a rare homozygous mutation in the gene associated with the lysosomal acid lipase deficiency, (allele c.386A > G homozygous p.H129R)18123023