Liver Transplantation in a Patient With Clinical Manifestations of Cryptogenic Cirrhosis: A Case Report of Hepar Lobatum as a Primary Liver Condition

Background

This article reports a case of hepar lobatum, a peculiar and rare type of liver deformity, originally described in association with infectious or parasitic diseases and with malignancies.

Case Report

We have described a 42-year-old woman with this disorder, which was unrelated to the known conditions and referred for liver transplantation for having clinical manifestations of cirrhosis, portal hypertension, and impaired hepatic function.

Conclusions

The observed histologic pattern suggests that hepar lobatum could be, in some patients, the effect of a primary process of hamartomatous origin involving the organ vascular supply.     

Effects of a Respiratory Physiotherapeutic Program in Liver Transplantation Candidates

Background

Candidates for liver transplantation may have malnutrition, fatigue, loss of muscle mass and function. The combination of these factors leads to overall physical disability and physical inactivity.

Objective

The aim of the study was to evaluate the effects of a respiratory physiotherapeutic program on liver transplantation candidates.

Method

Forty-two patients were evaluated by respiratory muscle strength, surface electromyography of the rectus abdominis and diaphragm, and spirometry. We also applied the SF-36. The patients were divided into two groups: 12 randomly assigned to the control group and 5 in the intervention group. The intervention consisted of an explanatory and illustrative manual to be followed at home with diaphragmatic breathing exercises, diaphragmatic isometric exercise, Threshold IMT, lifting the upper limbs with a bat, and strengthening the abdominals.

Results

Significant difference was found between initial forced expiratory flow (FEF)_25–75% (P = .042) and final FEF_25–75 in the intervention group. The control group had significant difference (P = .036) in the diaphragm RMS between initial time and end time. In conclusion, the control group showed greater electrical activity of the diaphragm after 3 months.

Conclusion

The intervention group benefited from the exercise, thus improving the FEF_25–75%.     

Efficacy of Rho kinase inhibitor fasudil in secondary Raynaud's phenomenon

Objective

The RhoA/Rho kinase pathway plays a pivotal role in cold‐induced vasoconstriction, vascular smooth muscle cells function, and vascular homeostasis. This study evaluates the efficacy of fasudil, a RhoA/Rho kinase inhibitor, to reverse cold‐induced vasospasm in patients with Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc; scleroderma).

Methods

This is a single‐center, double‐blind, placebo‐controlled, randomized, 3‐period crossover study of oral fasudil (40 mg or 80 mg) or placebo administered 2 hours before a standardized cold challenge. The fall in skin temperature after the cold challenge and time to recover 50% and 70% of prechallenge digital skin temperature were used as primary outcomes. Digital blood flow assessed by laser Doppler, time to minimum skin temperature, and rate of skin cooling were also measured.

Results

A total of 17 patients with SSc and RP completed the study. After the cold challenge, skin temperatures and the average time (minutes) to recover 50% (7.9 minutes for placebo, 7.5 minutes for fasudil 40 mg, and 8.2 minutes for fasudil 80 mg; P = 0.791) and 70% (18.2 minutes for placebo, 15.0 minutes for fasudil 40 mg, and 17.1 minutes for fasudil 80 mg; P = 0.654) of prechallenge skin temperature were not significantly different across the 3 groups. The digital blood flow measurements were higher in fasudil‐treated groups than placebo, but differences were not significant (P = 0.693).

Conclusion

Fasudil administered at a single oral dose of 40 mg or 80 mg was not associated with significant benefit in terms of the skin temperature recovery time and the digital blood flow after the cold challenge.     

FURTHER STUDIES ON THE PRESENCE OF TREPONEMA TALLIDUM IN SUBCUTANEOUS NODULES OF THF JUXTA ARTICULAR TYPE WITH A REPORT OF THIRTY-ONE CASES OBSERVED IN NORTH CHINA

This report deals with further observations on subcutaneou nodules of the juxta.articular type in syphilitic individuals. In 1933 we reported on five such cases (1). Since then 26 patients have been studied. It was felt that a statistical analysis of these 31 cases would be of someinterest. With this larger experience, points brought out in the earlier report are confirmed while new findings are added.     

Spinal abnormalities in pediatric patients: MR imaging findings compared with clinical, myelographic, and surgical findings

Eighty-one pediatric patients with a variety of spinal disorders, including suspected dysrhaphism, scoliosis, neoplasia, and neurofibromatosis, underwent magnetic resonance (MR) imaging. The results were retrospectively compared with those of myelography followed by computed tomography (CT) and surgery. In patients with dysrhaphism, most abnormalities, including hydromyelia, inclusion tumors, and sites of cord tether, were demonstrated with MR imaging. Diastematomyelia and small hydromyelic cavities were indistinguishable on routine coronal and sagittal T1-weighted images; axial images with T2 weighting were optimal for this differentiation. MR imaging did not enable direct visualization of a thickened filum or evaluation of tethering with a thin, dorsally positioned neural placode. Congenital or severe scoliosis required lengthy studies with multiple planes of imaging or myelography and CT. Milder curvatures were readily evaluated with MR imaging, and neoplastic lesions, with the exception of intrathecal tumor seeding, were adequately defined.     

Lack of detection of agonist activity by antibodies to platelet‐derived growth factor receptor α in a subset of normal and systemic sclerosis patient sera

Objective

To investigate whether agonist anti–platelet‐derived growth factor receptor α (anti‐PDGFRα) antibodies are present in the serum of patients with systemic sclerosis (SSc; scleroderma).

Methods

Sera were obtained from healthy subjects and scleroderma patients. An electrochemiluminescence binding assay was performed for detection of serum autoantibodies to PDGFRα, PDGFRβ, epidermal growth factor receptor (EGFR), and colony‐stimulating factor receptor 1 (CSFR1). Serum immunoglobulin was purified by protein A/G chromatography. To assess Ig agonist activity, PDGFRα‐expressing cells were incubated with pure Ig and the level of receptor phosphorylation determined in an enzyme‐linked immunoassay, as well as by Western blotting. Ig agonist activity was also assessed in a mitogenic assay and by MAP kinase activation in a PDGFRα‐expressing cell line.

Results

Sera from 34.3% of the healthy subjects and 32.7% of the SSc patients contained detectable autoantibodies to PDGFRα and PDGFRβ, but not EGFR or CSFR1. Purified Ig from these sera was shown to retain PDGFR binding activity and, at 200‐1,000 μg/ml, exhibited no agonist activity in a cell‐based PDGFRα phosphorylation assay and did not stimulate a mitogenic response or MAP kinase activation in a PDGFRα‐expressing cell line. Two purified Ig samples that were unable to bind PDGFRα did exhibit binding activity to a nonglycosylated form of PDGFRα.

Conclusion

Although approximately one‐third of sera from scleroderma patients contained detectable autoantibodies to PDGFR, these antibodies were not specific to scleroderma, since they were also detected in a similar percentage of samples from normal subjects. PDGFRα agonist activity was not demonstrated when purified Ig from these sera was tested in cell‐based assays.