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Background Calcineurin inhibitor (CNI)-related nephrotoxicity significantly contributes to chronic renal failure after liver transplantation. Methods In this prospective study, liver transplantation patients with renal dysfunction were randomized either to receive mycophenolate mofetil (MMF) followed by stepwise reduction of CNI with defined minimal CNI-trough levels (MMF group), or to continue their maintenance CNI dose (control group). Immune monitoring was performed in a subgroup of the patients. Results In the MMF group ( n = 50), renal function assessed by serum creatinine improved >10% in 62% of patients, was stable in 36% and deteriorated >10% in 2% after 12 months compared with baseline values. Mean serum creatinine levels (± s.d.) significantly decreased from 1.90 ± 0.44 mg/dL to 1.61 ± 0.39 mg/dL and the corresponding calculated glomerular filtration rate significantly increased from 38.8 ± 9.6 mL/min/1.73 m2 to 47.0 ± 11.8 mL/min/1.73 m2 over a 12-month follow-up period. Blood pressure and levels of liver enzymes significantly decreased. In the control group ( n = 25), there were no significant changes with respect to the investigated parameters. The MMF group had significantly lower numbers of circulating cytotoxic T cells compared with the controls; whereas regulatory T cells significantly increased. Conclusion Combined MMF and minimal dose CNI therapy after liver transplantation is nephroprotective and may promote allograft tolerance. [ABSTRACT FROM AUTHOR]

Purpose: Assisted peritoneal dialysis (PD) involving caregivers allows more patients to get started on home-based dialysis with good clinical outcomes, but evidence on patient-reported and caregiver-reported outcomes is lacking. This study aimed to compare assisted PD versus self-care PD on quality of life (QoL) and psychosocial outcomes for patients and caregivers. The effect of PD modality [automated PD (APD); continuous ambulatory PD (CAPD)] in relation to self-care or assisted care was also examined.Methods: A cross-sectional sample of 231 PD patients [142 self-care (57 APD/85 CAPD) and 89 assisted care PD (45 APD/44 CAPD)], 72 caregivers of assisted PD patients and 39 family members of self-care PD patients completed the Kidney Disease Quality of Life Short Form (KDQOL-SF), World Health Organisation Quality of Life Instrument-brief and the Hospital Anxiety and Depression Scale. Caregivers and family members completed the Lay Care-Giving for Adults Receiving Dialysis questionnaire and Zarit Burden Interview.Results: Case-mix-adjusted comparisons indicated comparable QoL in all dimensions with the exception of physical SF-12 (p = .001) and the KDQOL effects of kidney disease in favour of self-care PD. Levels of anxiety (9.72 ± 4.90; 8.25 ± 5.22) and depression (8.63 ± 3.80; 6.35 ± 4.76) were equivalent in assisted PD and self-care PD, respectively. Assisted PD caregivers reported more task-orientated duties (p = .007), yet levels of perceived burden were equal to those reported by family members of self-care PD.Conclusion: Our findings of mostly comparable patient and caregiver outcomes in assisted PD and self-care PD suggest that caregiver burden and QoL should not be a barrier to using assisted PD. [ABSTRACT FROM AUTHOR]

Glanzmann's thrombasthenia (GT) is a rare bleeding disorder characterized by spontaneous mucocutaneous bleeding. The disorder is caused by quantitative or qualitative defects in integrin αIIbβ3 (encoded by ITGA2B and ITGB3) on the platelet and is more common in consanguineous populations. However, the prevalence rate and clinical characteristics of GT in non‐consanguineous populations have been unclear. We analyzed 97 patients from 93 families with GT in the Han population in China. This analysis showed lower consanguinity (18.3%) in Han patients than other ethnic populations in GT‐prone countries. Compared with other ethnic populations, there was no significant difference in the distribution of GT types. Han females suffered more severe bleeding and had a poorer prognosis. We identified a total of 43 different ITGA2B and ITGB3 variants, including 25 previously unidentified, in 45 patients. These variants included 14 missense, 4 nonsense, 4 frameshift, and 3 splicing site variants. Patients with the same genotype generally manifested the same GT type but presented with different bleeding severities. This suggests that GT clinical phenotype does not solely depend on genotype. Our study provides an initial, yet important, clinical and molecular characterization of GT heterogeneity in China. [ABSTRACT FROM AUTHOR]

The article discusses clinical characteristics of patients having alpha-1-antitrypsin (A1AT), protease inhibitor (serpins). Topics discussed include controlling of coagulation, usage of an optical aggregometer, and separation of globulin by agarose gel electrophoresis. Particular focus is also given to approval of the study by the board of research ethic at Jiangsu Institute of Haematology in China.