Long-term results of kidney transplantation in patients with familial mediterranean fever.

INTRODUCTION: Long-term kidney transplantation (KT) results in patients with familial Mediterranean fever (FMF)-related amyloidosis are not well studied. This study reviewed the long-term survival outcomes of FMF patients who underwent KT. METHODS: We compared the outcomes of 31 patients who underwent (KT) for biopsy-proven amyloidosis secondary to FMF with 31 control patients (five with diabetes mellitus and 26 with nondiabetic kidney disease) undergoing KT between 1994 and 2021 at Baskent University Hospital. All data were recorded retrospectively from patients' files. RESULTS: THE MEDIAN AGE (QUARTILE DEVIATION: QD) at the time of KT in the FMF and control group were 31 (6.7) and 33 (11), respectively. The median follow-up period (QD) after KT was 108 (57) months in the FMF and 132 (72) months in the control group. In the FMF group, graft and patient survivals were 71% and 84% at 5 years and 45% and 48% at 10 years, respectively. In the control group, graft and patient survivals were 79% and 100% at 5 years and 63% and 71% at 10 years, respectively. Patient survival in the FMF group at 5 years was significantly lower than in the control group (p = .045). There was no statistically significant difference between the FMF and control groups in terms of graft and patient survival, and serum creatinine levels at 10 years. All patients were given triple immunosuppressive treatment with cyclosporine, mycophenolate mofetil, and prednisolone. Three patients received anakinra and one received canakinumab in addition to colchicine treatment. One FMF patient also underwent heart transplantation due to AA amyloidosis. Of the FMF patients, 11 died during follow-up. CONCLUSION: We have found that the long-term outcome of KT in patients with FMF amyloidosis is numerically worse but not statistically different from the control group. However, short- and long-term complications still need to be resolved.

Administration of oral medication by parents at home.

AIMS AND OBJECTIVE: The objective of this study was to determine parents' experiences and problems with administering medication to their children at home. BACKGROUND: Parents' experiences and access to information for the treatment of their children's illnesses at home is necessary for their children's safety. DESIGN: A descriptive cross-sectional study. METHODS: Four hundred parents from children's hospital outpatient clinics were included. A questionnaire was used to determine parents' experiences and problems with administering medication to their children at home. Descriptive statistical analyses were performed using the spss software package (version 22.00). RESULTS: Antipyretics (59%) and antibiotics (25%) were the most commonly used medicines by parents without prescription. Nearly half of the parents stated that they gave liquid medicine with a household spoon. It was found that 54% of the parents whose children refused to take tablets or liquid medicine mixed these medications into foods. Treatment was delayed in 20·7% of the children who refused to take tablets and in 29·1% of the children who refused to take liquid medicine. As a result of the project, a form and device were developed as a solution to the problems experienced by parents while administering oral medication in the home environment. CONCLUSION: The results of the study showed that a significant percentage of the parents did not use the correct equipment to administer medications, used non-prescription medicines, did not administer medications at correct intervals and mixed medication into foods. RELEVANCE TO CLINICAL PRACTICE: Healthcare professionals, especially nurses, should continually evaluate medication administration by parents at home and the readmission rate in the emergency department to further improve children's health.

Clinical importance of mean platelet volume in children with nephrotic syndrome.

AIM: The importance of changes about platelet emphasized in most chronically diseases in recent years. Mean platelet volume (MPV) and platelet count can be used as a prognostic biomarker. In this study, clinical importance of the changes of MPV during active and remission phases in children with nephrotic syndrome was investigated. PATIENTS AND METHODS: Fifty-five children with nephrotic syndrome (30 females, 25 males) and 29 healthy children (18 females, 11 males) were included to the study. Patients were divided in two groups (steroid sensitive nephrotic syndrome and focal segmental glomerulosclerosis). Demographic characteristics of the patients, type of nephrotic syndrome were recorded and laboratory parameters in active and remission phases were evaluated. RESULTS: Mean platelet count in the patient group was significantly higher than control group. Mean platelet count of FSGS group was significantly higher than SSNS group. Mean MPV value was significantly lower in active period of nephrotic syndrome when compared with control group. A significant negative relation between mean MPV value and mean platelet count was found. Significant positive correlations between mean platelet count and mean total cholesterol and mean triglyceride levels were demonstrated. CONCLUSION: MPV in nephrotic syndrome patients can be an easy, cheap and simple method for determine the prognosis of the disease and steroid resistance.

The effects of stem cells and platelet-rich fibrin on colonic anastomosis: An experimental study.

AIM - Anastomotic leakage is among the most common complications following gastrointestinal surgery. MATERIAL AND METHODS - This study aimed to determine the effects of stem cells and platelet-rich fibrin (PRF) on anastomotic healing. The study included 60 rats that were randomly divided into 3 groups, each with 2 subgroups. The study included the control group (no treatment post anastomosis), the PRF group (PRF administered following anastomosis), and the PRF + stem cell group (PRF + stem cells administered following anastomosis). Anastomosis was performed at the descending colon in all groups. Anastomosis bursting pressure was determined, and histopathological and angiographic examination were performed on postoperative D 7. RESULTS - Intraabdominal adhesion was significantly more common in the control group. Anastomosis bursting pressure was significantly higher and angiogenesis was significantly more common in the PRF + stem cell group (P < 0.005). Based on histopathologic examination, vascular proliferation and inflammation were significantly more common in the PRF + stem cell group than in the control group (P < 0.005). CONCLUSION : In cases of risky gastrointestinal system anastomosis, PRF + stem cells might reduce the incidence of anastomotic healing. KEY WORDS: Anastomosis, Platelet-rich fibrin, Stem cell, Wound healing.

Approach to optimal assessment of right ventricular remodelling in heart transplant recipients: insights from myocardial work index, T1 mapping, and endomyocardial biopsy.

AIMS: Right ventricular (RV) dysfunction is an important cause of graft failure after heart transplantation (HTx). We sought to investigate relative merits of echocardiographic tools and cardiac magnetic resonance (CMR) with T1 mapping for the assessment of functional adaptation and remodelling of the RV in HTx recipients. METHODS AND RESULTS: Sixty-one complete data set of echocardiography, CMR, right heart catheterization, and biopsy were obtained. Myocardial work index (MWI) was quantified by integrating longitudinal strain (LS) with invasively measured pulmonary artery pressure. CMR derived RV volumes, T1 time, and extracellular volume (ECV) were quantified. Endomyocardial biopsy findings were used as the reference standard for myocardial microstructural changes. In HTx recipients who never had a previous allograft rejection, longitudinal function parameters were lower than healthy organ donors, while ejection fraction (EF) (52.0 ± 8.7%) and MWI (403.2 ± 77.2 mmHg%) were preserved. Rejection was characterized by significantly reduced LS, MWI, longer T1 time, and increased ECV that improved after recovery, whereas RV volumes and EF did not change MWI was the strongest determinant of rejection related myocardial damage (area under curve: 0.812, P < 0.0001, 95% CI: 0.69-0.94) with good specificity (77%), albeit modest sensitivity. In contrast, T1 time and ECV were sensitive (84%, both) but not specific to detect subclinical RV damage. CONCLUSION: Subclinical adaptive RV remodelling is characterized by preserved RV EF despite longitudinal function abnormalities, except for MWI. While ultrastructural damage is reflected by MWI, ECV, and T1 time, only MWI has the capability to discriminate functional adaptation from transition to subclinical structural damage.

Nutcracker syndrome manifesting with severe proteinuria: a challenging scenario in a single-kidney patient.

Nutcracker syndrome (NS) refers to compression of the left renal vein between the aorta and the superior mesenteric artery which results in left renal venous hypertension. The typical clinical presenting feature is hematuria. In this report we describe the case of patient with a single kidney who developed severe proteinuria due to NS. She was successfully treated with left renal vein transposition. This case clearly shows the relation between NS and severe proteinuria based on normal biopsy findings and the complete disappearance of proteinuria following surgery.

Respiratory-chain deficiency presenting as diffuse mesangial sclerosis with NPHS3 mutation.

Renal manifestations of mitochondrial cytopathies have been described, but nephrotic syndrome with respiratory-chain disorders have been described extremely rarely. We report a 9-month-old boy with a mitochondrial cytopathy preceded by a 2-month history of steroid-resistant nephrotic syndrome. Percutaneous renal biopsy revealed diffuse mesangial sclerosis, and mutational analysis was compatible with PLCE1 mutation. However, electron microscopic findings of renal tissue, sensorineural hearing loss, and other ocular and neurologic findings led us to suspect mitochondrial cytopathy. Muscle tissue analysis showed a deficiency of the respiratory chain complex IV. The clinical presentation of our patient is not typical for primary cytochrome oxidase (COX) deficiency but showed similarities with patients carrying AR mutations in COX10. This was the first case in the literature with both PLCE1 mutation and COX deficiency. We could not identify pathogenic mutations in the COX10 gene, suggesting that PLCE1 deficiency could be the cause of the secondary deficiency of COX. Another, more likely, possibility is that the mitochondriopathy phenotype is caused by another mutation homozygous by descent in a yet unidentified recessive gene.

Clinical impact of complement deposition findings on biopsies in acute rejection episodes of pediatric renal transplant patients.

INTRODUCTION: Rejection is the most important problem for renal graft function and survival. Complement system plays a key role in immune responses from host to graft. It was demonstrated that complement system activation is related with renal fibrosis. We evaluate clinical impact of complement deposition findings on biopsies in acute rejection episodes of pediatric renal transplant patients. METHOD: Demographics of the patients, graft functions, acute rejection episodes and graft loss were recorded from data files of 165 pediatric renal transplant patients. Findings of 98 renal biopsies were retrospectively evaluated. RESULTS: Thirty three patients with kidney transplant had 44 acute rejection episodes (32 pure cellular acute rejection episodes / 1 pure humoral acute rejection episode / 11 combined acute cellular and acute humoral rejection episodes) proven by biopsy. C1q staining was positive in 7 biopsies, C3 staining in 15 biopsies and, C4d staining in 15 biopsies. 26 patients had graft fibrosis. All patients with a rejection history had a significant decrease in GFR value during follow-up. Patients who did not have fibrotic changes in first biopsy had same level of deterioration of GFR when compared with patients who had fibrotic changes in first biopsy. CONCLUSION: We could not demonstrate a significant relation between complement deposition and renal fibrosis, and between complement deposition and GFR values. Our data demonstrated that graft outcomes and graft loss after acute rejection episodes cannot be predicted only with complement deposition on graft or only with graft fibrosis.

Recurrence and Outcomes of Complement-Related Renal Disease After Pediatric Renal Transplantation.

Complement dysregulation is related to different glomerular pathologies. Patients with complement dysregulation have high recurrence risk after transplant; however, with trough-effective therapeutics, renal transplant can be an option for these patients. Here, we present 2 boys with renal disease related to complement dysregulation and their outcomes after renal transplant. Patient 1 had atypical hemolytic uremic syndrome, which was treated with eculizumab before renal transplant; eculizumab therapy was also continued after transplant as preventive therapy. Eculizumab therapy was stopped at year 2 post-transplant. At year 4 post-transplant, his serum creatinine level was 0.87 mg/dL. Patient 2, who had chronic renal disease related to C3 glomerulopathy, was not responsive to eculizumab before renal transplant. At month 4 posttransplant, C3 glomerulopathy recurrence was demonstrated with biopsy, and serum creatinine level was 1.96 mg/dL at this time. Eculizumab was started as a rescue therapy. At year 4 posttransplant, his serum creatinine level was 2.07 mg/dL. In our 2 patients with complement dysregulation, eculizumab was an effective and preventive therapy after renal transplant. However, more studies are needed to understand the long-term efficacy and safety of eculizumab after renal transplant.

The efficiency of propranolol on occurrence and development of 4-nitroquinoline 1-oxide-induced squamous cell carcinoma of the tongue in rats.

AIMS: The aim of this study to investigate the efficiency of propranolol on occurrence and development of 4-nitroquinoline 1-oxide (4NQO)-induced squamous cell carcinogenesis of the tongue in rats. SUBJECTS AND METHODS: The sample was composed of 27 male Sprague Dawley rats that received 50 ppm 4NQO for 20 weeks in drinking water. Group 1 (n = 9) was treated with 50 mg/kg/day propranolol for 20 weeks, Group 2 (n = 9), after carcinogenesis inducement for 20 weeks, received propranolol (50 mg/kg/day) for 2 weeks and Group 3 (n = 9) received no treatment. At the end of the experimental stage, the tongue specimens were evaluated under a light microscope and categorized as low- or high-risk lesions according to a binary system. STATISTICAL ANALYSIS USED: The statistical comparison was performed with a likelihood ratio test. RESULTS: Histopathological analysis revealed the risk of malignant transformation rates as 33.3% in Group 1, 55.5% in Group 2 and 77.8% in Group 3; however, the difference between the groups was not statistically significant (P > 0.05). CONCLUSION: The results of the study suggest that propranolol has a tendency to preventive effect against carcinogenesis.