Adherence self report assessment in solid-organ pre and post transplant recipients.

OBJECTIVE: There is strong evidence that many patients with chronic diseases have difficulties to adhere to the medical regimens. As to transplantation, nonadherence to the prescribed medical regimen has repeatedly been found to predict morbidity and mortality, both in adults and in children. Many instruments are available in order to assess nonadherence to immunosuppressive medication, whereas only few are available on behavioural components. Aim of the study was to present the Adherence Schedule in Transplantation (ASiT, in its three versions: ASiT-A, Adults; ASiT-PA, Proxy Adult and ASiT-PC, Proxy Child) that evaluates the Cognitive Relational Antecedents of adherence to treatment and the Self-efficacy in disease management in patients before and after transplantation. METHOD: (1) Review and construct analysis of the already existing adherence Schedules, on the basis of literature review and clinical experience; (2) Construction of the ASiT in its three versions from an adaptation of the already existing adherence Schedules; (3) Administration of the ASiT to pre and post transplant recipients followed by a semi-structured interview in order to discuss with the patients pros and cons; (4) ASiT correction on the basis of patients' comments; (5) Translation in English by a bilingual English mother tongue. RESULTS: On the whole 56 Schedules were administered to pre and post transplant (liver, heart, lung and kidney) recipients and their proxy and resulted to have good construct validity, were easily administered and were well accepted by patients. CONCLUSIONS: As to research the ASiT could cover a wide range of nonadherence sources. Within a clinical context our Schedule could be a not time consuming tool useful in favouring the communication about adherence and able to enhance patients' personal limits and resources.

Combined liver-kidney transplantation in glycogen storage disease Ia: a case beyond the guidelines.

Glycogen storage disease type Ia (GSD Ia) is a rare metabolic disorder due to hepatic glucose-6-phosphatase deficiency. Although great progress has been made in managing affected patients, severe hypoglycemia, lactic acidosis, hyperlipidemia, hepatic cytolysis, and impaired kidney function are frequent. Liver transplantation is the only radical treatment, for which the main indications are hepatic adenomatosis, hepatocellular carcinoma, or severe hepatic dysfunction. We present the case of a patient with end-stage renal disease without focal hepatic lesions and with moderate hepatic metabolic control, and we explain how combined liver-kidney transplantation (LKT) made it possible to correct the metabolic defects responsible for the impaired glucose homeostasis, liberalize the diet, and give birth to a healthy child after an uneventful pregnancy. Patients with end-stage renal disease that resulted from GSD Ia should be considered for LKT even in the absence of hepatic lesions with the aim of improving their quality of life.