Effect of Repeated Structured Diabetes Education on Lifestyle Knowledge and Self-Care Diabetes Management in Kidney Transplant Patients With Posttransplant Diabetes.

OBJECTIVES: Diabetes knowledge among kidney transplant recipients with posttransplant diabetes has not been clearly assessed. We evaluated whether diabetes education in kidney transplant recipients with posttransplant diabetes affected self-care, metabolic control variables, and reversibility of early diabetic microangiopathies. MATERIALS AND METHODS: In this prospective randomized controlled study, we enrolled 210 renal transplant recipients with posttransplant diabetes. Group 1 patients (n = 140) received structured diabetes education, and group 2 patients (n = 70) received conventional education. Patient data were collected through patient identification and metabolic control parameter forms and a diabetes self-care scale questionnaire (scores between 0 and 7). RESULTS: Diet knowledge improved and waist circumference was reduced with mild to moderate exercise in group 1 (P < .001), despite no differences between the 2 groups in mean body weight or body mass index. Patients in group 1 (structured diabetes education with repeated reinforcement) showed significant improvement in healthy lifestyle parameter scores versus group 2 (P < .05) and versus values before education (P < .05). At end of study, these achievements were translated into proper blood sugar monitoring, management of both hypoand hyperglycemia, improvements in logbook use and healthy sharp disposal, Ramadan fasting, sick day management, and knowledge on the importance of HbA1c (P < .05), which translated to decrease of HbA1c in group 1 by 1.35%. In group 1, proteinuria decreased significantly compared with before education and compared with group 2 values (P = .016). Diabetic retinopathy and neuropathy remained comparable between groups (P > .05). CONCLUSIONS: Structured diabetes education improved lifestyle knowledge, self-care diabetes management, and metabolic control variables among kidney transplant recipients with posttransplant diabetes. Structured diabetes education also resulted in partial reversibility of the present early diabetic nephropathy. We recommended such education to be delivered to all kidney transplant recipients with diabetes.

Use of recombinant activated factor VII to arrest uncontrolled bleeding: a case series.

A retrospective analysis is described to assess the effects of using recombinant activated factor VII to control bleeding in a series of patients who had failed to respond to conventional hemostatic measures. In all, 18 patients (aged 16-65 years) with a range of conditions resulting in bleeding refractory to conventional methods of control were treated with recombinant activated factor VII (60-120 Amicrog/kg; 1-4 doses). The effects of recombinant activated factor VII on bleeding were noted together with the patients' transfusion requirements and hematological parameters. Administration of recombinant activated factor VII successfully stopped bleeding in 17 of the 18 patients. Therapy with recombinant activated factor VII significantly decreased transfusion requirements for packed red blood cells, fresh frozen plasma, platelets, and cryoprecipitate compared with pretreatment values along with significant improvement in hemostasis. In various serious bleeding situations, treatment with recombinant activated factor VII may effectively arrest bleeding, which has remained refractory to conventional methods of control.

Assessment of Diabetes Knowledge Among Renal Transplant Recipients With Posttransplant Diabetes Mellitus: Kuwait Experience.

OBJECTIVES: Diabetes knowledge among kidney transplant recipients with posttransplant diabetes has not been exhaustively assessed. We evaluated levels of diabetes knowledge among our kidney transplant patients using a 35-item diabetes self-care management questionnaire. MATERIALS AND METHODS: The study comprised renal transplant patients with posttransplant diabetes mellitus who were referred from Hamed Al-Essa Organ Transplant Center of Kuwait to the Dasman Diabetes Institute. Patient data were collected through patient identification forms, metabolic control parameters forms, and diabetes self-care scale questionnaires (with score from 0-7). RESULTS: Of 356 (25.6%) kidney transplant recipients with posttransplant diabetes, 210 patients were enrolled in this study. Most were Kuwaiti (60%), men (48.8%), and with high school education level (43.8%). Some were smokers (11.9%), and the original kidney disease was glomerulonephritis in 37.6% of patients. Most patients (71.9%) received hemodialysis pretransplant. Most patients (> 88%) reported low mean score of healthy diet (0-3), with > 93% reporting low mean score of practicing exercise (0-3), > 62% not checking blood sugar at home, 85% not following the recommended frequency, and > 72% not caring for their feet (except washing in 86.7%). Moreover, most patients lacked information about sharp disposal, diet regimen, using logbooks, hypoglycemia and hyperglycemia, sick day management, and the importance of hemoglobin A1c and regular fundus examination. Mean score of practicing exercise was significantly higher in men (especially non-Kuwaiti; P < .05); otherwise, other mean scores were comparable between sexes and different nationalities (P > .05). CONCLUSIONS: Diabetes knowledge is deficient in patients with posttransplant diabetes. Seminars, counseling sessions, and workshops should be arranged periodically for renal transplant recipients to improve their low level of diabetes knowledge. This is a preliminary report of our randomized controlled study evaluating the impact of structured diabetes education on self-care activities and metabolic control variables.

Exome sequencing for the differential diagnosis of ciliary chondrodysplasias: Example of a WDR35 mutation case and review of the literature.

Exome sequencing is becoming widely popular and affordable, making it one of the most desirable methods for the identification of rare genetic variants for clinical diagnosis. Here, we report the clinical application of whole exome sequencing for the ultimate diagnosis of a ciliary chondrodysplasia case presented with an initial clinical diagnosis of Asphyxiating Thoracic Dystrophy (ATD, Jeune Syndrome). We have identified a novel homozygous missense mutation in WDR35 (c.206G > A), a gene previously associated with Sensenbrenner Syndrome, Ellis-van Creveld syndrome and Short-rib polydactyly syndrome type V. The genetic findings in this family led to the re-evaluation of the initial diagnosis and a differential diagnosis of Sensenbrenner Syndrome was made after cautious re-examination of the patient. Cell culture studies revealed normal subcellular localization of the mutant WDR35 protein in comparison to wildtype protein, pointing towards impaired protein-protein interaction and/or altered cell signaling pathways as a consequence of the mutated allele. This research study highlights the importance of including pathogenic variant identification in the diagnosis pipeline of ciliary chondrodysplasias, especially for clinically not fully defined phenotypes.

Annual Data for Kidney Transplant Services in Kuwait (2015).

OBJECTIVES: Renal transplant services have been provided by the Hamed Al-Essa Organ Transplant Center in Ibn Sina Hospital since 1979 for Kuwaitis and non-Kuwaiti citizens residing in Kuwait. We aimed to monitor the activity and outcome of annual renal transplants in 2015. MATERIALS AND METHODS: Data on transplant patients were collected from hospital records from January 1, 2015, through December 31, 2015, at Ibn Sina Hospital. RESULTS: Eighty-one patients underwent a renal transplant in Kuwait in 2015; 46 patients (56.8%) were male and 35 (43.2%) were female. Of these 81 patients, 24 (29.6%) received a kidney from a deceased donor, 19 (23.5%) received a kidney from a living-unrelated donor, and 38 (46.9%) received a kidney from a living-related donor. Thirty-four patients (41.98%) who were highly sensitized immunologically underwent successful desensitization before transplant according the local protocol; 13 (38.2%) of these patients were male and 21 (61.8%) were female. Two patients (2.47%) experienced acute rejection within the first week after transplant. One diabetic female patient underwent a successful simultaneous deceased-donor kidney and pancreas transplant. Seventy-nine patients who underwent a transplant outside of Kuwait in 2015 were added to the follow-up list; 62 (78%) of these patients were male and 17 (22%) were female. Of these 79 patients, 31 patients (39%) received a kidney from a living-related donor, 45 (57%) received a kidney from a living-unrelated donor, and 3 (4%) received a kidney from a deceased donor. In addition, 303 patients were assessed for fitness for kidney transplant; 204 (67.3%) of these patients were male and 99 (32.7%) were female. Eight (2.6%) patients were not placed on the waiting list for a kidney transplant for medical reasons. CONCLUSIONS: A total of 81 patients underwent a renal transplant in Kuwait in 2015, only 2 (2.47%) of whom experienced acute rejection in the first week after transplant.

Diabetic kidney disease: world wide difference of prevalence and risk factors.

Diabetic kidney disease - which is defined by elevated urine albumin excretion or reduced glomerular filtration rate (GFR) or both - is a serious complication that occurs in 20% to 40% of all diabetics. In this review we try to highlight the prevalence of diabetic nephropathy which is not uncommon complication of diabetes all over the world. The prevalence of diabetes worldwide has extended epidemic magnitudes and is expected to affect more than 350 million people by the year 2035. There is marked racial/ethnic besides international difference in the epidemiology of diabetic kidney disease which could be explained by the differences in economic viability and governmental infrastructures. Approximately one-third of diabetic patients showed microalbuminuria after 15 years of disease duration and less than half develop real nephropathy. Diabetic kidney disease (DKD) is more frequent in African-Americans, Asian-Americans, and Native Americans. Progressive kidney disease is more frequent in Caucasians patients with type 1 than type 2 diabetes mellitus (DM), although its overall prevalence in the diabetic population is higher in patients with type 2 DM while this type of DM is more prevalent. Hyperglycemia is well known risk factor for in addition to other risk factors like male sex, obesity, hypertension, chronic inflammation, resistance to insulin, hypovitaminosis D, and dyslipidemia and some genetic loci and polymorphisms in specific genes. Management of its modifiable risk factors might help in reducing its incidence in the nearby future.

Recurrent Focal Segmental Glomerulosclerosis and Abatacept: Case Report.

Focal segmental glomerulosclerosis is a common cause of end-stage renal disease in children. Focal segmental glomerulosclerosis recurrence in renal transplants is a challenging disease, and can cause graft dysfunction and loss. Different therapies exist with varying responses, from complete remission to resistance to all modes of treatment. Abatacept was recently introduced as a treatment for primary focal segmental glomerulosclerosis in native kidneys and in recurrent disease after transplant. We present a pediatric case with immunosuppression-resistant primary NPHS2-negative focal segmental glomerulosclerosis recur-rence after renal transplant. The standard therapy for recurrent focal segmental glomerulosclerosis (rituximab, plasmapheresis, high-dose cyclosporine, and corticosteroids) was tried but failed to induce remission. Abatacept (10 mg/kg) was given at 0, 2, and 4 weeks (total, 3 doses) with no good response. We conclude that abatacept may work in patients with B7-1-positive focal segmental glomerulosclerosis recurrence and its efficacy is uncertain in disease with B7-1-negative or unknown staining status.

Long-Term Follow-Up of Active Treatment Versus Minimization of Immunosuppressive Agents in Patients With BK Virus-Associated Nephropathy After Kidney Transplant.

OBJECTIVES: There is no active treatment for postrenal transplant BK virus-associated nephropathy proven to be effective so far. We assessed the effectiveness of actively treating this condition with combined leflunomide, intravenous immunoglobulin, and ciprofloxacin on long-term graft outcome compared with minimization of immunosuppressive drugs. MATERIALS AND METHODS: Kidney transplant recipients were screened for BK virus-associated nephropathy. Group 1 comprised 22 kidney trans plant recipients with twice-positive BK virus polymerase chain reaction results in urine and blood. After diagnosis was confirmed with graft biopsy, antimetabolite (mycophenolate mofetil or azathioprine) was changed to leflunomide and intravenous immunoglobulin and oral ciprofloxacin were given. Group 2 comprised 33 BK virus-associated nephropathy patients treated conventionally with reduced immunosuppressive medications. RESULTS: Fifty-five patients were treated (38 males [69%], 28 patients [50.9%] with type 2 diabetes mellitus). Mean HLA antigen mismatches were 3.65, and 28 patients (50.9%) were HLA-Cw7 negative. All patients received induction therapy, 30 patients (55.6%) received thymoglobulin, and 29 patients (52.7%) received antirejection therapy before BK virus-associated nephropathy diagnosis. Maintenance immunosuppression was prednisolone in 53 patients (96.3%), mycophenolate mofetil (2 g daily) in 52 patients (94.5%), and tacrolimus in 28 patients (50.9%). Subsequent rejection episodes occurred in 38% of patients after diagnosis. Basal mean estimated glomerular filtration rate was 52.5 ± 25.5, which was reduced significantly to 38.1 ± 27.8 mL/min/1.73 m(2) (P < .0001) at end of study but without significant differences between the groups (P = .08 and P = .17). Follow-up was 7.3 ± 4.99 years. Although no significant differences were shown in patient outcome, graft survival was significantly better in group 2 (P = .032). CONCLUSIONS: Administration of 3 different anti-BK virus agents (leflunomide, intravenous immunoglobulin, ciprofloxacin) added no benefit to longterm outcome in patients with BK virus-associated nephropathy. Reduction of immunosuppressive medications appears to be a more effective treatment.

Protracted febrile myalgia syndrome in a kidney transplant recipient with familial Mediterranean fever.

Drug-induced toxic myopathy is a complication of familial Mediterranean fever in patients who receive colchicine, especially when combined with cyclosporine. Protracted febrile myalgia syndrome is a severe form of familial Mediterranean fever. A 34-year-old man who had familial Mediterranean fever for > 15 years developed kidney failure because of secondary amyloidosis. He received living-unrelated-donor kidney transplant that functioned normally. He was on colchicine prophylaxis that was continued after transplant, and he received immuno-suppression induction with antithymocyte globulin and maintenance with prednisolone, mycophenolate mofetil, and cyclosporine. After 2 months, he presented with severe myopathy and elevated creatine kinase. Muscle biopsy showed evidence of drug-induced toxic myopathy, most likely caused by cyclosporine in combination with colchicine. Cyclosporine was replaced with sirolimus and colchicine was stopped. Symptoms partially improved and creatine kinase decreased to normal. The prednisolone dosage was reduced gradually to 5 mg daily. At 8 months after transplant, he was readmitted because of severe arthralgia, prolonged fever, pleuritic chest pain, diffuse abdominal pain, purpuric rash, macroscopic hematuria, proteinuria, and diarrhea. The C-reactive protein and erythrocyte sedimentation rate were elevated. The clinical diagnosis was recurrent familial Mediterranean fever presenting as protracted febrile myalgia syndrome. Despite the history of toxic myopathy, he was restarted on colchicine (0.5 mg, twice daily), and colchicine was well tolerated. There was marked improvement of most symptoms within several days. Follow-up 5 years later showed normal kidney graft function and no familial Mediterranean fever activity on colchicine prophylaxis. In summary, familial Mediterranean fever reactivation and protracted febrile myalgia syndrome after kidney transplant may be treated with colchicine and modulation of immunosuppressive therapy.

Active management versus minimization of immunosuppressives of BK virus-associated nephropathy after a kidney transplant.

OBJECTIVES: Thus far, there is no active treatment for BK virus-associated nephropathy after a kidney transplant that has proven to be effective. We sought to assess the effectiveness of treatment with leflunomide, intravenous immunoglobulin, and ciprofloxacin on graft outcome after 1 year compared with a historical group treated with reduced immunosuppressive medications strategy. MATERIALS AND METHODS: Group 1 (n = 19) was composed of kidney transplant recipients with twice positive BK virus-polymerase chain reaction in urine and blood who underwent graft biopsy to confirm BK virus-associated nephropathy. Once BK virus-associated nephropathy was diagnosed, antimetabolite (mycophenolate mofetil or azathioprine) was changed to leflunomide, and intravenous immunoglobulin and oral ciprofloxacin were given. Group 2 (n = 14) was composed of BK virus-associated nephropathy patients treated conventionally with reduced immunosuppressive medications. RESULTS: Thirty-three patients were treated, 23 were males (70%), there were 15 were deceased donors (45.5%), 15 were diabetics (45.5%), mean human leukocyte antigen mismatches were 3.76, seven had a zero DR mismatch (21.2%), and 8 were CW7 negative (24.2%). All patients received induction therapy (thymoglobulin in 22 [66.6%]), 7 had delayed graft function (21.2%) and 18 received antirejection therapy before receiving BK virusassociated nephropathy diagnosis (52.9%). Maintenance immunosuppression was prednisolone and mycophenolate mofetil (2 g/d) in 31 patients (94%), and tacrolimus in 13 (39.4%). Tacrolimus was given to 12 patients in group 1 (63.1%), while sirolimus was given to 7 patients in group 2 (50%). One graft was lost in each group by the end of the study, and 1 patient died with functioning graft in group 2. CONCLUSIONS: No significant difference existed in 1-year graft outcomes between treatment of BK virus-associated nephropathy by reduction of immunosuppressive medications or actively by leflunomide, intravenous immunoglobulin, and ciprofloxacin.

Combined liver and kidney transplantation in primary hyperoxaluria: a report of three cases and review of the literature.

Primary hyperoxaluria type-1 (PH-1) is a rare autosomal recessive metabolic disorder leading to excessive oxalate production, deposition of calcium oxalate crystals in the kidney, nephrocalcinosis, progressive renal failure and systemic deposition of oxalate (oxalosis). Combined liver and kidney transplantation (LKT), which has been accepted as the treatment of choice for PH-1, has considerably improved patient and graft survival. Herein, we report our experience of three children with PH-1 who underwent combined LKT, with a review of the literature.

Effective therapy for acute antibody-mediated rejection with mild chronic changes: case report and review of the literature.

To reduce the long-term toxicities of immunosuppressant drugs, corticosteroid-sparing and calcineurin-inhibitor-sparing immunosuppression protocols have become increasingly popular in managing kidney transplant recipients. The most vexing clinical condition caused by antibodies in organ transplants is antibody-mediated rejection. Limitations of the current antibody-mediated rejection therapies include (1) antibody-mediated rejection reversal tends to be gradual rather than prompt, (2) expense, (3) rejection reversal rates below 80%, (4) common appearance of chronic rejection after antibody-mediated rejection treatment, and (5) long-term persistence of donor specific antibodies after therapy. Because these limitations may be due to a lack of effects on mature plasma cells, the effects of bortezomib on mature plasma cells may represent a quantum advance in antihumoral therapy. Our experiences represent the first clinical use of bortezomib as an antihumoral agent in renal allograft recipients in Kuwait. We present 2 cases with resistant-acute antibody-mediated rejection to the standard therapies that were managed successfully with bortezomib.