ABSTRACT
The COVID-19 infection is a worldwide disease that causes numerous immune-inflammatory disorders, tissue damage, and lung dysfunction. COVID-19 vaccines, including those from Pfizer, AstraZeneca, and Sinopharm, are available globally as effective interventions for combating the disease. The severity of COVID-19 can be most effectively reduced by mesenchymal stromal cells (MSCs) because they possess anti-inflammatory activity and can reverse lung dysfunction. MSCs can be harvested from various sources, such as adipose tissue, bone marrow, peripheral blood, inner organs, and neonatal tissues. The regulation of inflammatory cytokines is crucial in inhibiting inflammatory diseases and promoting the presence of anti-inflammatory cytokines for infectious diseases. MSCs have been employed as therapeutic agents for tissue damage, diabetes, autoimmune diseases, and COVID-19 patients. Our research aimed to determine whether live or dead MSCs are more suitable for the treatment of COVID-19 patients. Our findings concluded that dead MSCs, when directly administered to the patient, offer advantages over viable MSCs due to their extended presence and higher levels of immune regulation, such as T-reg, B-reg, and IL-10, compared to live MSCs. Additionally, dead and apoptotic MSCs are likely to be more readily captured by monocytes and macrophages, prolonging their presence compared to live MSCs.
Subject(s)
COVID-19 , Communicable Diseases , Mesenchymal Stem Cell Transplantation , Mesenchymal Stem Cells , Infant, Newborn , Humans , SARS-CoV-2 , COVID-19 Vaccines , Cytokines , Anti-Inflammatory Agents/pharmacology , Anti-Inflammatory Agents/therapeutic useABSTRACT
BACKGROUND: Some publications have recently been released on the safety of non-papillary access (NPA) in percutaneous nephrolithotomy (PCNL) by a Greek group. The purpose of this study was to prospectively examine the outcome of NPA during two years in a referral center. METHODS: This prospective cohort study was conducted on PCNL operations performed from January 2020 to April 2022 in Labbafinejad Hospital. In cases in which obtaining papillary access (PA) was not obtainable after several attempts and NPA was obtained, or in cases in whom after entry to the pyelocalyceal system, a NPA was observed, the cases were categorized in the NPA group (n = 67). The control group (PA) was composed of patients who had undergone PCNL with papillary access with similar stone bulk (n = 67). The primary endpoints of interest were hemoglobin drop and transfusion frequency. The secondary endpoint included: stone free rate (SFR), operation duration, and complications. RESULTS: A total of 134 patients were enrolled during the study period which included 33 female patients (25%). The mean ± SD age of patients was 49.6 ± 13.2 years. The frequency of transfusion and residual stones was not different between NPA and PA groups: 6 (9%) versus 8 (11%), P = 0.29; and 16 (24%) versus 12 (18%), P = 0.26. Likewise, the operation time (87.6 ± 25.8 versus 90.2 ± 22.6 min, P = 0.45), and the frequency of intraoperative and postoperative complications were not different between the NPA and PA groups. CONCLUSION: The results of this prospective study did not reveal a higher frequency of residual stones, transfusion, or complications in the NPA group. Nevertheless, our study is not powered enough to reveal complications of low frequency including delayed bleeding.
Subject(s)
Kidney Calculi , Nephrolithotomy, Percutaneous , Nephrostomy, Percutaneous , Humans , Female , Adult , Middle Aged , Nephrolithotomy, Percutaneous/methods , Prospective Studies , Nephrostomy, Percutaneous/methods , Kidney Calculi/surgery , Treatment OutcomeABSTRACT
CRISPR/Cas9 (clustered regularly interspaced short palindromic repeats-associated protein 9) shows the opportunity to treat a diverse array of untreated various genetic and complicated disorders. Therapeutic genome editing processes that target disease-causing genes or mutant genes have been greatly accelerated in recent years as a consequence of improvements in sequence-specific nuclease technology. However, the therapeutic promise of genome editing has yet to be explored entirely, many challenges persist that increase the risk of further mutations. Here, we highlighted the main challenges facing CRISPR/Cas9-based treatments and proposed strategies to overcome these limitations, for further enhancing this revolutionary novel therapeutics to improve long-term treatment outcome human health.
Subject(s)
CRISPR-Cas Systems , Neoplasms , Gene Editing , Genetic Therapy , Humans , Mutation , Neoplasms/genetics , Neoplasms/therapyABSTRACT
Metal-Organic Frameworks (MOFs) are a new class of crystalline porous structures which can be used as a novel structure in diverse fields of medical science. Several studies have shown that chromium supplementation can be effective in amelioration of biochemical parameters of diabetes and its renal complications. Therefore, a chromium-containing MOF (DIFc) was synthetized by nanochelating technology in the present study and then its effect on biochemical indices in diabetic rats was evaluated. Diabetes was induced by high-fat diet consumption and streptozotocin (35 mg/kg) injection and then the treatment started 8 weeks after disease induction and continued for 8 weeks. The results showed that DIFc treatment decreased HOMA-IR index, blood urea nitrogen, uric acid and malondialdehyde in plasma samples. This nano MOF also reduced albumin, malondialdehyde and 8-isoprostane in urine specimen, while it increased creatinine clearance. In conclusion, DIFc MOF demonstrated promising results in the present study, indicating that it can be developed and evaluated in future investigations with the aim of designing a novel agent for management of diabetes and its renal complications.
Subject(s)
Chromium/pharmacology , Diabetes Mellitus, Experimental/drug therapy , Diabetic Nephropathies/drug therapy , Metal-Organic Frameworks/pharmacology , Animals , Biomarkers/blood , Metal-Organic Frameworks/chemical synthesis , Nanotechnology , RatsABSTRACT
BACKGROUND: The dietary habits and lifestyle changes during the COVID-19 pandemic could affect the urinary risk factors in kidney stone formers. In this study, we investigated the effects of the COVID-19 pandemic on 24-h urine metabolites, as a surrogate for dietary intake, in patients with kidney stones, in Tehran, Iran. METHODS: We evaluated the medical records of all patients with urolithiasis who visited in our stone prevention clinic from the beginning of COVID-19 in Iran to 1 year later (Feb 2020-Feb 2021) and compared it with the patients' medical records in the same period a year before COVID-19 (Feb 2019-Feb 2020). RESULTS: The results of our stone prevention clinic showed a decrease in the number of visits during COVID-19. Twenty-four-hour urine urea, sodium, and potassium were significantly lower, and 24-h urine magnesium was significantly higher during COVID-19. Higher 24-h urine oxalate was only shown in patients with the first-time visit, whereas lower 24-h urine uric acid and citrate were only shown in patients with the follow-up visits. CONCLUSIONS: COVID-19 pandemics may change some of the dietary habits of the patients, including lower salt, protein, and fruit and vegetable intake. Although economic issues, restricted access, or sanitation issues may be the reason for the undesirable dietary changes, the importance of a quality diet should be discussed with all patients, as possible. Since the number of patients visited in the stone clinic was lower during COVID-19, virtual visits could be an excellent alternative to motivate patients with kidney stones.
Subject(s)
COVID-19 , Kidney Calculi , Humans , Iran/epidemiology , Kidney , Kidney Calculi/epidemiology , Kidney Calculi/prevention & control , Pandemics , Risk Factors , SARS-CoV-2ABSTRACT
OBJECTIVES: To investigate potential oxalate-degrading bacteria, including Oxalobacter formigenes, Lactobacillus (Lac) and Bifidobacterium (Bif) genera, and Oxalyl-CoA decarboxylase (oxc) encoding Lac (LX) and Bif (BX) species in participants with recurrent calcium kidney stones, and their correlation with 24-h urine oxalate. PARTICIPANTS AND METHODS: Stool and 24-h urine samples were collected from 58 patients with urolithiasis (29 cases with and 29 without hyperoxaluria) and 29 healthy controls. Absolute quantitation and relative abundance of the bacteria were measured by real-time PCR. The relationship between the investigated bacteria and 24-h urine oxalate were assessed statistically. RESULTS: The count per gram of stool and relative abundance of O. formigenes, Lac, Bif, LX and BX and the number of participants carrying O. formigenes, LX and BX bacteria were not significantly different between the groups; however, the relative abundance of O. formigenes in the kidney stone group was lower than in healthy controls (P = 0.035). More healthy controls were O. formigenes-positive compared with participants in the kidney stone group (P = 0.052). The results of the linear regression model, including all study participants, showed that the presence of O. formigenes could decrease 24-h urine oxalate (ß = -8.4, P = 0.047). Neither Lac and Bif genera nor LX and BX species were correlated with calcium stones or urine oxalate. CONCLUSION: These results emphasize the role of O. formigenes in kidney stone formation and its role in hyperoxaluria, which may be independent of kidney stone disease. Moreover, our results suggest that, although some Lac and Bif strains have oxalate-degrading potential, they may not be among the major oxalate-degrading bacteria of the gut microbiome.
Subject(s)
Bifidobacterium/metabolism , Calcium , Hyperoxaluria/microbiology , Kidney Calculi/microbiology , Lactobacillus/metabolism , Oxalates/metabolism , Oxalobacter formigenes/metabolism , Adult , Calcium/analysis , Carboxy-Lyases/metabolism , Case-Control Studies , Cross-Sectional Studies , Female , Humans , Kidney Calculi/chemistry , Male , Middle Aged , RecurrenceABSTRACT
BACKGROUND: Kidney transplant improves patients' survival and quality of life. Worldwide, concern about the equality of access to the renal transplant wait-list is increasing. In Iran, patients have the choice to be placed on either the living or deceased-donor transplant wait-list. METHODS: This was a prospective study performed on 416 kidney transplant recipients (n = 217 (52.2%) from living donors and n = 199 (47.8%) from deceased donors). Subjects were recruited from four referral kidney transplant centers across Tehran, Iran, during 2016-2017. The primary outcome was to identify the psycho-socioeconomic factors influencing the selection of type of donor (living versus deceased). Secondary objective was to compare the outcomes associated with each type of transplant. The impact of psycho-socioeconomic variables on selecting type of donor was evaluated by using multiple logistic regression and the effect of surgical and non-surgical variables on the early post-transplant creatinine trend was assessed by univariate repeated measure ANOVA. RESULTS: Based on standardized coefficients, the main predictors for selecting living donor were academic educational level (adjusted OR = 3.25, 95% CI: 1.176-9.005, p = 0.023), psychological status based on general health questionnaire (GHQ) (adjusted OR = 2.46, 95% CI: 1.105-5.489, p = 0.028), and lower monthly income (adjusted OR = 2.20, 95% CI: 1.242-3.916, p = 0.007). The waiting time was substantially shorter in patients who received kidneys from living donors (p < 0.001). The early post-transplant creatinine trend was more desirable in recipients of living donors (ß = 0.80, 95% CI: 0.16-1.44, p-value = 0.014), patients with an ICU stay of fewer than five days (ß = - 0.583, 95% CI: - 0.643- -0.522, p-value = < 0.001), and those with less dialysis duration time (ß = 0.016, 95% CI: 0.004-0.028, p-value = 0.012). Post-operative surgical outcomes were not different across the two groups of recipients (p = 0.08), however, medical complications occurred considerably less in the living-donor group (p = 0.04). CONCLUSION: Kidney transplant from living donors was associated with shorter transplant wait-list period and better early outcome, however, inequality of access to living donors was observed. Patients with higher socioeconomic status and higher level of education and those suffering from anxiety and sleep disorders were significantly more likely to select living donors.
Subject(s)
Donor Selection/statistics & numerical data , Kidney Transplantation/psychology , Socioeconomic Factors , Tissue Donors/statistics & numerical data , Transplant Recipients/psychology , Adult , Female , Humans , Iran/epidemiology , Logistic Models , Male , Middle Aged , Multivariate Analysis , Prospective Studies , Treatment Outcome , Waiting ListsABSTRACT
The use of bioactive scaffolds in tissue engineering has a significant effect on the damaged tissue healing by an increase in speed and quality of the process. Herein, electrospinning was applied to fabricate composite nanofibrous scaffolds by Poly lactic-co-glycolic acid (PLGA) and Polyurethane (PU) with and without poly-phosphate (poly-P). Scaffolds were characterized morphologically by scanning electron microscope (SEM), and their biocompatibility was also investigated by SEM, protein adsorption, cell attachment and survival assays. The applicability of the scaffolds for bladder tissue engineering was also evaluated by culturing mesenchymal stem cells (MSCs) on the scaffolds and their differentiation into smooth muscle cell (SMC) was studied at the gene and protein levels. The results demonstrated that scaffold biocompatibility was increased significantly by loading poly-P. SMC related gene and protein expression level in MSCs cultured on poly-P-loaded scaffold was also increased significantly compared to those cells cultured on empty scaffold. It can be concluded that poly-P hasn't also increased scaffold biocompatibility, but also SMC differentiation potential of MSCs was also increased while cultured on the poly-P containing scaffold compared to the empty scaffold. Taken together, our study showed that PLGA-PU-poly-P alone and in combination with MSCs has a promising potential for support urinary bladder smooth muscle tissue engineering.
Subject(s)
Cell Differentiation/drug effects , Mesenchymal Stem Cells/cytology , Myocytes, Smooth Muscle/cytology , Nanofibers/chemistry , Polylactic Acid-Polyglycolic Acid Copolymer/chemistry , Polyphosphates/pharmacology , Polyurethanes/chemistry , Tissue Scaffolds/chemistry , Adsorption , Cell Separation , Gene Expression Regulation/drug effects , Humans , Mesenchymal Stem Cells/drug effects , Myocytes, Smooth Muscle/drug effects , Nanofibers/ultrastructureABSTRACT
The Urological Association of Asia, consisting of 25 member associations and one affiliated member since its foundation in 1990, has planned to develop Asian guidelines for all urological fields. The field of stone diseases is the third of its guideline projects. Because of the different climates, and social, economic and ethnic environments, the clinical practice for urinary stone diseases widely varies among the Asian countries. The committee members of the Urological Association of Asia on the clinical guidelines for urinary stone disease carried out a surveillance study to better understand the diversity of the treatment strategy among different regions and subsequent systematic literature review through PubMed and MEDLINE database between 1966 and 2017. Levels of evidence and grades of recommendation for each management were decided according to the relevant strategy. Each clinical question and answer were thoroughly reviewed and discussed by all committee members and their colleagues, with suggestions from expert representatives of the American Urological Association and European Association of Urology. However, we focused on the pragmatic care of patients and our own evidence throughout Asia, which included recent surgical trends, such as miniaturized percutaneous nephrolithotomy and endoscopic combined intrarenal surgery. This guideline covers all fields of stone diseases, from etiology to recurrence prevention. Here, we present a short summary of the first version of the guideline - consisting 43 clinical questions - and overview its key practical issues.
Subject(s)
Urinary Calculi/diagnosis , Urinary Calculi/surgery , Urology/standards , Asia , Endoscopy , Humans , Nephrolithotomy, Percutaneous , Recurrence , Secondary Prevention , Societies, Medical , Systematic Reviews as Topic , Urinary Calculi/drug therapy , Urinary Calculi/prevention & controlABSTRACT
A healthy 37 - year - old woman referred to our clinic with one - year history of recurrent urinary tract infection, dysuria and frequency. Her past medical history informed us that an IUD (Copper TCu380A) had been inserted 11 years ago. Eleven months after the IUD insertion she had become pregnant, unexpectedly. At that time, she had undergone gynecological examination and abdominal ultrasound study. However, the IUD had not been found, and the gynecologist had made the diagnosis of spontaneous fall out of the IUD. She had experienced normal pregnancy and caesarian section with no complications. On physical examination, pelvic examination was normal and no other abnormalities were noted. Urinalysis revealed microhematuria and pyuria. Urine culture was positive for Escherichia coli. Ultrasound study revealed a calculus of about 10 mm in the bladder with a hyperdense lesion. A plain abdominal radiograph was requested which showed a metallic foreign body in the pelvis. We failed to remove the IUD by cystoscopic forceps because it had strongly invaded into the uterine and bladder wall. Despite previous papers suggesting open or laparoscopic surgeries in this situation (1, 2), we performed a modified cystoscopic extraction technique. We made a superficial cut in the bladder mucosa and muscle with J - hook monopolar electrocautery and extracted it completely with gentle traction. This technique can decrease the indication of open or laparoscopic surgery for extraction of intravesical IUDs. In the other side of the coin, this technique may increase the risk of uterovesical fistula. Therefore, the depth of incision is important and the surgeon should cut the bladder wall superficially with caution. Although present study is a case report which is normally classified as with low level of evidence, it seems that our modified cystoscopic extraction technique is a safe and useful method for extraction of partially intravesical IUDs.
Subject(s)
Cystoscopy/methods , Device Removal/instrumentation , Intrauterine Devices , Urinary Bladder/surgery , Adult , Female , Foreign-Body Migration/surgery , Gynecologic Surgical Procedures/methods , Humans , Intrauterine Devices/adverse effects , Laparoscopy/methods , PregnancyABSTRACT
PURPOSE: Hypercalciuria is one of the risk factors for calcium kidney stone formation (the most common type of urinary stones). Although vitamin D deficiency is prevalent among urolithiasis patients, the effect of vitamin D supplementation on urine calcium in these patients is still unclear. MATERIALS AND METHODS: In this retrospective study, medical and laboratory tests records of 26 patients with recurrent calcium kidney stones and vitamin D deficiency treated with 50000IU vitamin D per week for 8-12 weeks were analyzed. The changes in 24-hour urine calcium (24-h Ca), serum 25-hydroxyvitamin D (25 (OH) D), serum parathormone (PTH), other 24-hour urine metabolites and calculated relative supersaturations of calcium oxalate (CaOxSS), calcium phosphate (CaPSS) and uric acid (UASS) were assessed. Moreover, correlations between changes in 24-h Ca and other aforementioned variables were assessed. RESULTS: Serum 25 (OH) D and 24-h Ca increased after vitamin D supplementation, while serum PTH decreased (p < 0.001, for all analyses). The levels of 24-hour urine sodium and urea increased significantly (p = 0.005 and p = 0.031, respectively). The levels of CaOxSS and CaPSS increased, but the changes were not significant (p = 0.177, and p = 0.218, respectively). There were no correlations between the changes in 24-h Ca and serum 25 (OH) D or PTH. CONCLUSIONS: The result of current study suggests that although urine Ca increased in vitamin D supplemented patients, this increase was not associated with the increase in serum vitamin D and may be due to other factors such as dietary factors. Further randomized clinical trials considering other factors associated with urine Ca are warranted.
Subject(s)
Calcium/urine , Urolithiasis/urine , Vitamin D Deficiency , Vitamin D/therapeutic use , Vitamins/therapeutic use , Aged , Dietary Supplements , Female , Humans , Hypercalciuria/complications , Male , Middle Aged , Parathyroid Hormone/blood , Retrospective Studies , Vitamin D/administration & dosage , Vitamin D/blood , Vitamin D Deficiency/drug therapy , Vitamin D Deficiency/etiologyABSTRACT
PURPOSE: Adherence to preventive recommendations improves clinical outcomes and is compulsory for long-term prevention in urolithiasis patients. Service quality can affect patients' adherence to treatment and care. The purpose of this paper is to compare perceived service quality, using the SERVQUAL model, between urolithiasis patients who were nonadherent to their follow-up visits and those who were adherent, in a stone prevention clinic, Tehran, Iran. DESIGN/METHODOLOGY/APPROACH: This was a cross-sectional study on patients with urolithiasis referred to the Shahid-Labbafinejad Hospital stone prevention clinic between 2010 and 2014. All patients withdrawing from follow-up visits were selected as the nonadherent group. Patients with follow-up visits were randomly selected and appointed as the adherent group. Data included demographic, service quality (assessed using the SERVQUAL) and a questionnaire about the reason for withdrawal from follow-up visits. Man-Whitney U test, χ2 and binary logistic regression were used for data analyses. FINDINGS: In total, 531 nonadherent and 51 adherent patients entered the study. SERVQUAL results revealed that patients' expectations were significantly higher than their perceptions in all five service quality dimensions in both groups. The adherent group had better-quality scores. Responsibility, assurance and empathy scores significantly increased patient adherence odds. RESEARCH LIMITATIONS/IMPLICATIONS: Uni-center design; missing data, such as socioeconomic status and disease severity, which may influence treatment adherence; and missing data regarding adherence to medication and dietary advice were limitations. PRACTICAL IMPLICATIONS: Service quality needs to be improved in all dimensions. ORIGINALITY/VALUE: Since responsiveness, assurance and empathy dimensions determined patients' adherence, giving special attention to these dimensions could improve patient adherence.
Subject(s)
Ambulatory Care/organization & administration , Attitude to Health , Patient Compliance/statistics & numerical data , Primary Prevention/organization & administration , Quality of Health Care , Urolithiasis/prevention & control , Academic Medical Centers , Adult , Aged , Cross-Sectional Studies , Female , Humans , Iran , Logistic Models , Male , Middle Aged , Patient Satisfaction/statistics & numerical data , Retrospective Studies , Risk Assessment , Statistics, NonparametricABSTRACT
The small G protein signaling modulator 3 (SGSM3) has been shown to be associated with small G-protein-coupled receptor signaling. There is little data regarding the impact of SGSM3 polymorphisms on cancer risk. In the present study, we aimed to evaluate the impact of 4-bp insertion/deletion (rs56228771) polymorphism in the 3'UTR of SGSM3 and susceptibility to bladder cancer in a sample of the Iranian population. This case-control study included 143 pathologically confirmed bladder cancer patients and 144 healthy subjects. The SGSM3 4-bp ins/del (rs56228771) variant was determined by mismatch PCR-RFLP. The findings showed that ins/del genotype and ins allele of SGSM3 4-bp ins/del polymorphism significantly increased the risk of bladder cancer (OR = 3.11, 95%CI = 1.70-5.71, P < 0.0001 and OR = 2.11, 95%CI = 1.27-3.52, P = 0.004, respectively). Our findings support an association between 4-bp ins/del polymorphism in the 3'UTR of SGSM3 and the risk of bladder cancer in an Iranian population. Additional studies with larger sample sizes and diverse ethnicities are warranted to establish if such an association exists in general.
Subject(s)
3' Untranslated Regions , INDEL Mutation , Intracellular Signaling Peptides and Proteins/genetics , Neoplasm Proteins/genetics , Polymorphism, Restriction Fragment Length , Urinary Bladder Neoplasms/genetics , Aged , Case-Control Studies , Female , Humans , Iran , Male , Middle AgedABSTRACT
PURPOSE: This study is a systematic analysis of the evidence regarding oncological, perioperative and postoperative outcomes and the cost of open retropubic radical prostatectomy (ORP), laparoscopic radical prostatectomy (LRP) and robotic-assisted laparoscopic radical prostatectomy (RALP). METHODS: Summary data was abstracted from 104 original research articles representing 227,400 patients. PubMed/Medline, Scopus, Google Scholar, EMBASE and the Cochrane Library were reviewed in December 2016. A total of 104 publications were selected for inclusion. The primary outcomes were positive surgical margin (PSM) and major complication rate according to Clavien classifications. Secondary outcomes were operative time, length of hospital stay, estimated blood loss, transfusions, conversions, rate of post-operative erectile dysfunction and incontinence and total cost of procedure. RESULTS: ORP had a significantly higher rate than RALP for PSM (OR: 1.18; 95% CI 1.05-1.32; p = 0.004), but the rate of PSM was not significantly different between ORP versus LRP (OR: 1.37; 95% CI 0.88-2.14; p = 0.17) and RALP versus LRP (OR: 0.83; 95% CI 0.40-1.72; p = 0.62). The major Clavien complication rate was significantly different between ORP and RALP (OR: 2.14; 95% CI 1.24-3.68; p = 0.006). Estimated blood loss, transfusions and length of hospital stay were low for RALP, moderate for LRP and high for ORP. The rate of erectile dysfunction (OR: 2.58; 95% CI 1.77-3.75; p < 0.001) and incontinence (OR: 3.57; 95% CI 2.28-5.58; p < 0.001) were significantly lower after RALP than LRP and equivalent for other comparisons. Total cost was highest for RALP, followed by LRP and ORP. CONCLUSIONS: For PSM and peri- and post-operative complications, RALP showed better results than ORP and LRP. In the context of the biases between the studies, one should interpret the results with caution.
Subject(s)
Laparoscopy , Prostatectomy , Prostatic Neoplasms/surgery , Robotic Surgical Procedures , Comparative Effectiveness Research , Humans , Laparoscopy/adverse effects , Laparoscopy/economics , Laparoscopy/methods , Male , Outcome and Process Assessment, Health Care/methods , Prostatectomy/adverse effects , Prostatectomy/economics , Prostatectomy/methods , Robotic Surgical Procedures/adverse effects , Robotic Surgical Procedures/economics , Robotic Surgical Procedures/methodsABSTRACT
OBJECTIVES: To present the safety and efficacy of fluoroscopy-free ultrasound-guided PCNL for the treatment of renal calculi in pediatric patients of all ages. METHODS: 30 children with mean age of 5 years (6 months-12 years) underwent totally ultrasound-guided PCNL from March 2013 to August 2016. The pyelocalyceal system was punctured in prone position using only ultrasonography guidance, and the tract was dilated using a single shot dilation technique. No fluoroscopy was used during any of the stages of renal access. The procedure was performed using adult-sized instruments. RESULTS: The mean stone size was 27.1 ± 8.7 mm. Mean access time was 4.3 ± 2 min. Mean nephroscopic time was 34.6 ± 15.2 min. Mean hospital stay of patients was 3 days (range 2-5). 21 patients were stone-free after the procedure (70% success rate). Only four patients out of 30 experienced postoperative complications. CONCLUSIONS: The results of this study showed that fluoroscopic-free ultrasound-guided PCNL in pediatric patients of all ages is safe, highly efficient, and minimizes potential radiation exposure risks associated with the procedure.
Subject(s)
Kidney Calculi/surgery , Kidney , Nephrolithotomy, Percutaneous , Radiation Exposure/prevention & control , Surgery, Computer-Assisted/methods , Ultrasonography/methods , Child , Child, Preschool , Female , Humans , Infant , Iran , Kidney/diagnostic imaging , Kidney/surgery , Male , Nephrolithotomy, Percutaneous/adverse effects , Nephrolithotomy, Percutaneous/methods , Postoperative Complications , Retrospective Studies , Treatment OutcomeABSTRACT
BACKGROUND/AIMS: Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common inherited cystic kidney diseases caused by mutations in two large multi-exon genes, PKD1 and PKD2. High allelic heterogeneity and duplication of PKD1 exons 1-32 as six pseudo genes on chromosome 16 complicate molecular analysis of this disease. METHODS: We applied targeted next-generation sequencing (NGS) in 9 non-consanguineous unrelated Iranian families with ADPKD to identify the genes hosting disease-causing mutations. This approach was confirmed by Sanger sequencing. RESULTS: Here, we determined three different novel frameshift mutations and four previously reported nonsense mutations in the PKD1 gene encoding polycystin1 in heterozygotes. CONCLUSION: This study demonstrates the effectiveness of NGS in significantly reducing the cost and time for simultaneous sequence analysis of PKD1 and PKD2, simplifying the genetic diagnostics of ADPKD. Although a probable correlation between the mutation types and phenotypic outcome is possible, however for more extensive studies in future, the consideration of renal hypouricemia (RHUC) and PKD1 coexistence may be helpful. The novel frameshift mutations reported by this study are p. Q1997X, P. D73X and p. V336X.
Subject(s)
Frameshift Mutation , High-Throughput Nucleotide Sequencing/methods , Polycystic Kidney, Autosomal Dominant/genetics , TRPP Cation Channels/genetics , Exons , Family , Female , Frameshift Mutation/genetics , High-Throughput Nucleotide Sequencing/economics , Humans , Iran , Male , Pedigree , Renal Tubular Transport, Inborn Errors/genetics , Time Factors , Urinary Calculi/geneticsABSTRACT
BACKGROUND: Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the fourth most frequent cause of endstage renal disease (ESDR), occurring at a varying frequency of 1/400 to 1/800 persons. The disease affects all ethnic groups worldwide, and there is a need for population based studies to be carried out for better diagnostic, genetic counselling, and treatment purpose. METHODS: Eighteen unrelated probands (10 males and 8 females) with a familial history of ADPKD were selected for the study. Their clinical evaluation was performed to diagnose and assess disease progression. PKD1 and PKD2 genes were genotyped in each proband by next generation sequencing (NGS). RESULTS: Mutational analysis of PKD1 and PKD2 genes using NGS in eighteen unrelated Iranian ADPKD families revealed a total of eighteen heterozygous variations. PKD1 genotype revealed eight frameshift deletion mutations, two frameshift insertion mutations, five nonsense mutations and one splice mutation and PKD2 showed one frameshift deletion mutation and one frameshift insertion mutation. Four of the variants reported were novel and were present in the PKD1 gene. Further, PKD1 truncating mutations reached ESRD earlier than patients with non-truncating PKD1 mutations (52 ± 3.2 years vs. 58 ± 10.8 years, p = 0.01). CONCLUSIONS: The PKD1 and PKD2 genotyping of ADPKD Iranian patients with familial history showed no mutational hotspot. The screening has given four novel variants that will contribute to diagnosis, genetic counselling, and treatment of ADPKD patients in general.
Subject(s)
Polycystic Kidney, Autosomal Dominant/genetics , TRPP Cation Channels/genetics , Adult , Aged , DNA Mutational Analysis , Female , Humans , Iran , Male , Middle Aged , MutationABSTRACT
OBJECTIVES: To explore intelligence quotient in boys with primary nocturnal enuresis compared with normal boys considering their socioeconomic status. METHODS: A total of 152 school-aged boys (including 55 boys with primary nocturnal enuresis and 97 matched normal controls) were assessed. Boys with a history of any neurological or urological disease were excluded. Two different districts of Tehran: Khani-Abad (a poor district) and Pirouzi (a middle class district) districts were enrolled according to socioeconomic status data reported by the World Health Organization. Intelligence tests were carried out using a validated Iranian translation of the Wechsler Intelligence Scale for Children Revised. Total, as well as performance intelligence quotient and verbal intelligence quotient scores and verbal-performance discrepancy (the difference between verbal and performance intelligence quotient scores for each individual) were compared using a t-test between boys with primary nocturnal enuresis in each district and their matched controls. RESULTS: Considering each district separately, the total intelligence quotient score was lower in primary nocturnal enuresis cases than controls only in the lower income district (90.7 ± 23.3 vs 104.8 ± 14.7, P = 0.002). Similarly, boys with primary nocturnal enuresis ranked lower in verbal intelligence quotient (P = 0.002) and performance intelligence quotient (P = 0.004) compared with their matched normal controls only in lower income district, whereas in the higher income district, boys with primary nocturnal enuresis ranked similar in total intelligence quotient to their matched controls. CONCLUSIONS: Boys with primary nocturnal enuresis had a lower intelligence quotient compared with the control participants only in low-income district. It seems important to adjust the results of the intelligence quotient assessment in these children according to their socioeconomic status.
Subject(s)
Intelligence , Nocturnal Enuresis/psychology , Social Class , Case-Control Studies , Child , Humans , Iran , Male , Wechsler ScalesABSTRACT
OBJECTIVES: To evaluate the success of ultrasonography directed renal access in entering the target calyx from proper entry site and in the direction of renal pelvis during percutaneous nephrolithotomy (PCNL). MATERIALS AND METHODS: PCNL cases who were operated on by one fellow from May-June 2014 were included in this study. A vertically placed ultrasound probe on the patient flank in prone position was used to identify the preselected target calyx. Needle was advanced through needle holder and fluoroscopy was used to document the entered calyx, site and angle of entry. RESULTS: Successful entering to the target calyx was achieved in 43 cases (91%). Successful entry with appropriate entry site and angle was observed in 34 cases (72%). Reasons for failure were minimal hydronephrosis, upper pole access and high lying kidneys. CONCLUSIONS: Although it is feasible to access a preselected calyx by ultrasonography guidance during PCNL, but entry to the calyx from the appropriate site and direction is another problem and needs more experience. In cases of minimal hydronephrosis, superior pole access or high lying kidneys, ultrasonography is less successful and should be used with care.
Subject(s)
Fluoroscopy/methods , Kidney Calculi/diagnostic imaging , Nephrostomy, Percutaneous/methods , Ultrasonography, Interventional/methods , Adult , Female , Humans , Kidney Calculi/surgery , Male , Middle Aged , Needles , Prone PositionABSTRACT
BACKGROUND: Patients with nephrolithiasis and hyperoxaluria generally are advised to follow a low-oxalate diet. However, most people do not eat isolated nutrients, but meals consisting of a variety of foods with complex combinations of nutrients. A more rational approach to nephrolithiasis prevention would be to base dietary advice on the cumulative effects of foods and different dietary patterns rather than single nutrients. STUDY DESIGN: Randomized controlled trial. SETTING & PARTICIPANTS: Recurrent stone formers with hyperoxaluria (urine oxalate > 40 mg/d). INTERVENTION: The intervention group was asked to follow a calorie-controlled Dietary Approaches to Stop Hypertension (DASH)-style diet (a diet high in fruit, vegetables, whole grains, and low-fat dairy products and low in saturated fat, total fat, cholesterol, refined grains, sweets, and meat), whereas the control group was prescribed a low-oxalate diet. Study length was 8 weeks. OUTCOMES: Primary: change in urinary calcium oxalate supersaturation. SECONDARY: Changes in 24-hour urinary composition. RESULTS: 57 participants were randomly assigned (DASH group, 29; low-oxalate group, 28). 41 participants completed the trial (DASH group, 21; low-oxalate group, 20). As-treated analysis showed a trend for urinary oxalate excretion to increase in the DASH versus the low-oxalate group (point estimate of difference, 9.0mg/d; 95% CI, -1.1 to 19.1mg/d; P=0.08). However, there was a trend for calcium oxalate supersaturation to decrease in the DASH versus the low-oxalate group (point estimate of difference, -1.24; 95% CI, -2.80 to 0.32; P=0.08) in association with an increase in magnesium and citrate excretion and urine pH in the DASH versus low-oxalate group. LIMITATIONS: Limited sample size, as-treated analysis, nonsignificant results. CONCLUSIONS: The DASH diet might be an effective alternative to the low-oxalate diet in reducing calcium oxalate supersaturation and should be studied more.