Therapeutic Problems and Pregnancy in a Patient With Infantile Nephropathic Cystinosis: A Case Report.

BACKGROUND: Cystinosis is a rare genetic disorder characterized by the abnormal accumulation of cystine in the lysosomes of various tissues and organs leading to their dysfunction. The most common type is the infantile nephropathic cystinosis which without treatment leads to renal failure and before the introduction of cysteamine was the cause of death before puberty. CASE PRESENTATION: A 27-year-old female patient with infantile cystinosis developed end-stage renal disease at the age of 10. The first kidney transplantation from patient's father was carried out at the age of 12. The recurrent urinary tract infections led to the graft failure after 6 years. Following the removal of right appendages due to the ovarian tumor, the patient underwent the second kidney transplantation from her mother at the age of 19. After the transplantation, the cysteamine treatment was irregular due to limited availability of the medicine. When it became regular in 2017 the patient did not tolerate full doses. Despite elevated blood levels of cystine and the removal of right appendages, the patient naturally became pregnant in August 2017. Except for recurrent urinary tract infections, the renal parameters remained normal throughout the entire pregnancy. However, in the 32nd week of gestation, due to preeclampsia a caesarean section was performed. A healthy daughter was born, 1400/41 and with a 9 point Apgar score. CONCLUSIONS: Due to the possibility of treatment with cysteamine and kidney transplantations, patients with cystinosis live longer and their quality of life improves. These female patients can even naturally become pregnant and give birth to healthy children.

Successful Pediatric Kidney Transplantation Into an Adult Recipient: A Case Report.

BACKGROUND: The constant shortage of kidney donors prompts exploration into new strategies of transplantation. One of these strategies is the use of pediatric donors aged up to 5 years whose kidneys can be transplanted into adult recipients, mainly en bloc. This involves retrieving kidneys en bloc with aorta and inferior vena cava and anastomosing them to the recipient's external iliac vessels. CASE PRESENTATION: In our hospital, kidneys from a 3-year-old child were transplanted to a 30-year-old man. The recipient with end-stage renal failure, due to glomerular nephritis, was dialyzed for 12 years and had 1 failed transplantation with consequent graftectomy. In 2009, kidneys were transplanted to the external iliac artery and vein with reconstruction of the renal vessels. Shortly after transplantation the patient had normal renal measures. Three months later a critical stenosis of 1 renal artery was detected. Angioplasty was performed but technical reasons did not allow for effective dilatation of the vessel. Further, 6 months after kidney transplantation (KTx) nephrotic proteinuria appeared and features of membranous nephropathy were detected in a renal biopsy. The proteinuria subsided after administration of ramipril and losartan. Doppler ultrasound revealed that 1 artery remained 90% stenotic with a peak systolic velocity of 377 cm/sec. Despite reported complications, renal function appeared normal over 7 years of observation. CONCLUSIONS: A transplantation of 2 pediatric kidneys into an adult recipient has very high efficacy. The survival of both graft and recipient is similar to the results obtained after living donor kidney transplantation.

Long-term Outcomes in Simultaneous Pancreas-Kidney Transplant Recipients: Single-center Experience From Poland.

BACKGROUND: Simultaneous pancreas-kidney transplantation (SPKT) is the treatment of choice for patients with end-stage renal disease (ESRD) due to type 1 diabetes mellitus (DM1). Since the 1980s, pancreas transplantation has become the most effective strategy to restore normoglycemia in patients with DM1. The aim of this study was to present long-term outcomes data for SPKT. METHODS: We performed a retrospective analysis of 73 SPKT recipients followed in our outpatient center who underwent transplantation between 1988 and 2015. RESULTS: A total of 50.7% of the patients were male. At the time of surgery, patients' mean age was 37.38 ± 7.44 years. Patients were diagnosed with DM1 at an average of 25 ± 6.08 years before SPKT. For 21.9% of patients, the transplant was done preemptively. Most (91.8%) had enteric drainage. All patients received induction of immunosuppression (either polyclonal immunoglobulins anti-thymocyte globulin or thymoglobulin [64.4%] or monoclonal globulins daclizumab or basiliximab [35.6%]). Patient survival at 1, 5, 10, 15 years was 99%, 97%, 89%, and 75%; kidney survival was 99%, 96%, 84%, and 67%; and pancreas survival was 95%, 92%, 84%, and 64%, respectively. There was a notable tendency toward increased creatinine level (from 1.18 at 1 year to 1.78 at 15 years) and decreased hemoglobin level (from 13.84 at 1 year to 12.65 at 15 years). CONCLUSION: Diabetic patients with ESRD have a poor prognosis without transplantation. SPKT provides marked prolongation of the patient's life and freedom from insulin injections. Enteric drainage is currently the surgical technique of choice. SPKT should remain as the treatment of choice in this patient population.

Analysis of Hospitalizations in Simultaneous Pancreas-Kidney Transplant Recipients: A Single-center Experience in Poland.

BACKGROUND: End-stage renal disease due to type 1 diabetes mellitus appears to be a regular indication for simultaneous pancreas and kidney transplantation (SPKT). Although transplantation improves a patient's health condition, it does not mean that all complications will be eliminated. METHODS: We performed a retrospective analysis of 73 patients who underwent SPKT and follow-up between 1988 and 2015 at our institute. The number, duration, and reasons for hospitalization at 1, 5, 10, and 15 years after SPKT were analyzed. RESULTS: The average number of hospitalizations at 1, 5, 10, 15 years after SPKT were 1.66, 0.39, 0.36, and 0.33, respectively. The main reason for hospitalization over the 15-year period was infections, at 32.4% (SD, 6.8%). Within the first year after SPKT, 6.8% of hospital admissions were caused by cytomegalovirus (CMV) infection. Over time, the percentage of hospitalizations for cardiovascular complications increased from 0.6% at 1 year to 29% at 12-15 years. Incidence of hospitalization due to cardiovascular complications correlated with a longer period of dialysis and a diagnosis of ischemic heart disease before transplant (r = 0.56, P = .004; r = 0.54, P < .0001, respectively). At 12-15 years after transplantation, 18.2% of hospitalizations were caused by secondary complications of diabetes. CONCLUSION: The most common reason for hospitalization after SPKT is infectious complications. In the first year posttransplant, there is a high percentage of CMV infections. Hospitalization associated with cardiovascular complications was found to be most common in the latter follow-up period and showed a correlation with longer dialysis period.

Posttransplant Lymphoproliferative Disorder in Kidney and Liver Transplant Recipients: A Single-Center Experience.

INTRODUCTION: Posttransplant lymphoproliferative disorder (PTLD) is a heterogeneous group of lymphoid malignant neoplasms arising after solid organ transplantation or hematopoietic stem cell transplantation. The current World Health Organization classification identified 4 basic histologic types of PTLD: early, polymorphic variant, monomorphic variant, and classical Hodgkin lymphoma-type lesions. METHODS: Data of 12 PTLD cases of was retrospectively analyzed in terms of the transplanted organs, time to diagnosis of PTLD, type of immunosuppressive treatment in regard to the induction treatment and acute transplant rejection, and long-term survival. RESULTS: Most of the analyzed cases of PTLD occurred in men (n = 8, 67%); 83% of patients were renal transplant recipients and 17% were liver transplant recipients. Of the kidney recipients, 8% received induction of antithymocyte globulin and 17% received daclizumab. An episode of acute rejection occurred in 6 (50%) patients. All patients were treated with pulses of methylprednisolone and received triple immunosuppressive regimen. Histopathologic examination revealed polymorphic form of PTLD in 5 (42%) patients and classical Hodgkin lymphoma in 3 (25%) cases. Diffuse large B-cell lymphoma was diagnosed in 3 (25%) patients, and diffuse large B-cell lymphoma rich in T lymphocytes and histiocytes was diagnosed in 1 (8%) patient. ALK4- anaplastic lymphoma was diagnosed in 1 (8%) recipient. Four (25%) patients died as a result of PTLD progression (including all 3 patients with central nervous system involvement), and 8 survived with stable graft function. CONCLUSIONS: PTLD is a heterogeneous group of lymphoproliferative disorders occurring in organ recipients. The unusual location changes (especially central nervous system or intestine) can impede the proper diagnosis.

Efficacy of Follow-up Care System of Living Kidney Donors in Monitoring of Residual Kidney Function.

BACKGROUND: The possibility of an increased risk of end-stage renal disease is a major concern associated with living kidney donation. Therefore, monitoring of residual kidney function becomes most essential. METHODS: A data analysis of 156 living kidney donors (LKDs) was conducted. The efficacy of the long-term care system with regard to monitoring residual kidney function was evaluated. RESULTS: The analyzed group consisted of 102 (65.4%) women. The mean follow-up period was 5.44 years. The rise in value of mean serum creatinine concentration after donation was observed, but it was within the range of normal during the observation period. Despite its initial decline after nephrectomy, mean glomerular filtration rate (GFR) remained >60 mL/min/1.73 m2. A MDRD (Modification of Diet in Renal Disease) GFR in the range of 45-60 mL/min/1.73 m2 was observed in 53 donors (33.97%). It was found to be <45.0 mL/min/1.73 m2 in 15 cases (9.6%). No patient developed end-stage renal disease. Only 25.0% of those analyzed had their CKD-EPI (Chronic Kidney Disease Epidemiology Collaboration) GFR estimated on 45-60 mL/min/1.73 m2 and 4.49% were found to have levels of <45 mL/min/1.73 m2 (down to 33.7 mL/min/1.73 m2). Mean postdonation CKD-EPI GFR was estimated at 69.99% of its predonation value. CONCLUSION: A reliable qualification process could minimize the probability of kidney donation by someone with an increased risk of chronic kidney failure. The CKD-EPI formula seems to be more precise than the MDRD for estimatation of LKDs' GFR, as their loss of GFR is a result of nephrectomy and not kidney or systemic disease. Using the MDRD formula may lead to inappropriate diagnosis of CKD in some cases.

Analysis of Distribution of Expanded- and Standard-Criteria Donors and Complications Among Polish Recipients by Kidney Donor Risk Index Value.

INTRODUCTION: The approach toward transplanting kidneys from expanded-criteria donors (ECDs) in Poland is largely site-dependent. The Kidney Donor Risk Index (KDRI) allows for obtaining a more precise characteristic of ECDs and further stratification into "better" and "worse" quality grafts. METHODS: Comparison of the incidence of delayed graft function (DGF) and biopsy-proven acute rejection (BPAR), median of hospitalization time and median of estimated glomerular filtration rate (eGFR) at 1 year after transplantation among kidney graft recipients (n = 468), divided by donor status (ECD/standard-criteria donor [SCD]) and KDRI value (I: 0.67-1.2, II: 1.21-1.6, III: 1.61-2.0, IV: 2.01-3.48). RESULTS: ECD kidneys have been transplanted to 32.47% of recipients. There were no ECD recipients in KDRI compartment I, 16.55% in compartment II, 79.22% in compartment III, and 100% in IV. In KDRI compartment II, DGF was diagnosed in 34.9% of SCDs and 56% of ECDs (P = .003), BPAR occurred in 7.8% of SCDs and 16% of ECDs (P = .073), median hospital stay was 12 days for SCDs and ECDs (P = 1), and eGFR was 50.7 mL/min for SCDs and 49.4 mL/min for ECDs (P = .734). In KDRI compartment III, DGF was diagnosed in 43.8% of SCDs and 49.2% of ECDs (P = .139), BPAR occurred in 6.3% of SCDs and 31.7% of ECDs (P = .001), median hospital stay was 10 days for SCDs and 12 days for ECDs (P = .634), and eGFR was 49.5 mL/min for SCDs and 45.2 mL/min for ECDs (P = .382). Among ECD recipients, DGF was diagnosed in 56.0%, 49.2%, and 47.7% of patients for KDRI compartments II, III, and IV respectively (P = .776); BPAR occurred in 16% (compartment II), 31.7% (compartment III), and 23.1% (compartment IV) (P = .273); the median hospital stay was 12 days (compartment II), 12 days (compartment III), and 12.5 days (compartment IV) (P = 1); and eGFR was 49.5 mL/min (compartment II), 45.4 mL/min (compartment III), and 36.1 mL/min (compartment IV) (P = .002). CONCLUSION: Assessment using both the ECD and KDRI systems allows for a more precise evaluation of prognosis and predicting complications among recipients.

Islets Allotransplantation Into Gastric Submucosa in a Patient with Portal Hypertension: 4-year Follow-up.

BACKGROUND: Islets transplantation is an established treatment method for patients suffering from brittle diabetes with hypoglycemia unawareness. The standard implantation technique is through the portal vein into the liver. In case of liver diseases or portal hypertension, finding an extra-hepatic site is recommended. There have been attempts to perform islets transplantations into muscles and into the gastric submucosa. OBJECTIVE: The aim of this study is to show a 4-year follow-up of allotransplantation into gastric submucosa in a case of portal hypertension observed during the procedure of islets infusion. PATIENTS AND METHODS: A 36-year-old woman with complicated diabetes for over 30 years was selected to receive simultaneous islets and kidney transplantation. The patient underwent an unsuccessful simultaneous pancreas and kidney transplantation 2 years earlier in another transplantation center. The patient's daily insulin requirement was 60 IU, which corresponded to 1.15 IU/kg of body weight. The HbA1c level was 7.4%. C-peptide levels, both fasting and stimulated, were 0.01 ng/mL. On December 7, 2013, the patient received transplanted kidney and islets procured from the same donor. Only 124,000 islets equivalents (IEQ) were isolated (2400 IEQ/kg body weight). Islets were suspended in 300 mL of Ringer's solution along with albumin, antibiotics, and heparin. After infusing 100 mL of the islets suspension into the portal vein, pressure in portal vein increased from 5 mm Hg to 23 mm Hg. Despite stopping the infusion, pressure did not drop after 30 minutes. The decision was made to transplant the reminder of the islets (200 mL) into the gastric wall. RESULTS: No complications were observed after the procedure. Serum creatinine level was 1.6 mg/dL on day 10 and 1.5 mg/dL 4 years after the transplantation. Fasting C-peptide levels were 1.7, 0.65, 0.55, 0.69, 0.68, and 0.2 ng/mL at 1, 3, 6, 12, 18, and 36 months after the transplantation, respectively. HbA1c levels were 5.2, 6.4, 4.7, 5.2, and 5.9% at 3, 6, 12, 18, and 36 months, respectively. The patient's insulin requirement dropped to 15 U/day immediately after transplantation and equaled 20 and 27 U/day at 18 and 48 months after the simultaneous islet and kidney transplantation, respectively. CONCLUSION: Allotransplantation of islets into the gastric wall may be a safe alternative in cases of contraindications for transplantation into the portal vein.

Pathologies in Living Kidney Donors Diagnosed in the Long-Term Care System.

Kidney donation should not lead to deterioration of the donor's health condition, both during the perisurgical period and in the long term. Safety of a living kidney donor becomes a prerequisite for his/her qualification. Detailed diagnostic procedures are performed to exclude any abnormalities of his/her health condition. Additionally, a long-term post-donation follow-up system for kidney donors has been set up in Poland besides the restrictive qualification system. Transplantation centers are obligated to provide a diagnostic procedures for living organ donors as a part of the monitoring of their health condition and to ensure them a medical follow-up for 10 years after the donation. A total of 141 cases of unilateral nephroureterectomy performed in 2003-2014 to obtain a kidney for transplantation were considered. Medical files of post-donation diagnostic or therapeutic methods and their outcomes were retrospectively analyzed. The aim of the study was to assess the efficacy of monitoring of donors' health condition within the framework of the long-term follow-up system for kidney donors in the aspect of detection of the donation-independent abnormalities.