Ivica Mokos

Anderson-Fabry Disease in Kidneys from Deceased Donor

Anderson‐Fabry disease (AFD) is a rare, X‐linked lysosomal storage disease that leads to progressive intracellular accumulation of globotriaosylceramide in visceral organs and the vascular endothelium. We report two patients with end‐stage renal disease who received renal allograft from deceased female donor who died from heart failure. A 62‐year‐old women received a renal allograft in July 2006. Except for low‐range proteinuria, renal function was normal until 6 months after transplantation when serum creatinine increased from 120 to 150 μmol/L. A renal biopsy was performed. Based on the specific pathological finding, AFD in donor was suspected. In order to prove the diagnosis, the other recipient also underwent renal biopsy 3 months later. This was 45‐year‐old female with stable graft function and nonnephrotic proteinuria. Light microscopic findings included a ‘foamy’ appearance of affected cells with swelling and vacuolization of podocytes. Electron microscopic finding show mesangial cells and podocytes filled with dense lysosomal granules appearing as myelin figures and ‘zebra bodies’. Changes were less intensive than in the biopsy of the first recipient. The donor was 54‐year‐old Italian women who died on the Adriatic coast after heart attack. This is the first case of AFD found in a kidney allograft from deceased donor.

Influence of Long-Term Dialysis Treatment on Operative Complications After Renal Transplantation

BACKGROUND We sought to assess the influence of long-term dialysis treatment on operative complications after kidney transplantation. METHODS We identified and prospectively followed 2 groups of patients who underwent renal transplantation: group I were on dialysis <5 years and group II, >15 years. We compared the groups regarding mismatches, residual diuresis, operative and medical complications, as well as duration of hospitalization after transplantation. RESULTS Groups I and II included 36 and 24 patients with 33.3% and 45.8% female of mean ages at transplantation of 49 (range, 9-73) and 45 (range, 27-56) years, respectively. Group I contained 9 seniors and 4 subjects <18 years. There were no significant differences in mean donor age, number of marginal donors, mismatches, postoperative bleeding, wound infections, urine leakage, or duration of hospitalization. However, the number of postoperative lymphoceles was significantly higher in group II: 5 (20.8%) versus 1 (2.7%) (P = .01). Graftectomy was performed in 2 group II patients including 1 primary graft nonfunction, and another for recurrent focal segmental glomerulosclerosis. CONCLUSION Our results suggested that the duration of dialysis treatment was not a significant factor for postoperative complications and prolonged hospital stay, except for an increased risk of lymphocele formation.

Do we need more intensive enzyme replacement therapy for Anderson-Fabry disease?

tive state and only certain stimuli (eg. the mechanical stretch during skin expansion) are capable of activating these genes. During skin expansion, such genes could be activated, initiating sequential nuclear regulation. This regulation promotes cell proliferation, increased collagen synthesis, and enhanced vascularization, thus yielding the excess skin formed in the skin expansion process. In conclusion, skin expansion is an interesting model system for skin regeneration study and microarray analysis of conventional expanded skin could allow for determination of the genes regulating skin regeneration. If our hypothesis is correct and if the identified genes can be modulated (up-regulated or down-regulated) via gene therapy, our research would be of significant value to the wound healing and regeneration medicine as it has the potential to completely regenerate skin. Thus, the scarless wound healing would no longer be a myth but a reality.

Therapeutic challenges in the mature patient

With the tremendous increase in the proportion of seniors in the global population, geriatric health care has become of greater interest and concern. Increased emphasis on geriatric medicine, along with the growth in the development of age-related skin disorders, has led to particular attention for geriatric, dermatology and dermatopharmacology. An aging population has brought many therapeutic challenges that we need to recognize and overcome by applying geropharmacologic principles. The purpose of this paper is to inform dermatologists of the age-related changes in the pharmacokinetics of common dermatologic drugs, their various interactions potentially occurring in the elderly, and the principles and evidence-based strategies for detection, management, and prevention to improve medication adherence. By implementing these principles and strategies, we can ensure the best and the safest treatment to promote the desired therapeutic outcome and improved quality of life for this fragile subpopulation.