Promising Results of Associating Liver Partition and Portal Vein Ligation for Staged Hepatectomy for Perihilar Cholangiocarcinoma in a Systematic Review and Single-Arm Meta-Analysis.

BACKGROUND: ALPPS popularity is increasing among surgeons worldwide and its indications are expanding to cure patients with primarily unresectable liver tumors. Few reports recommended limitations or even contraindications of ALPPS in perihilar cholangiocarcinoma (phCC). Here, we discuss the results of ALPPS in patients with phCC in a systematic review as well as a pooled data analysis. METHODS: MEDLINE and Web of Science databases were systematically searched for relevant literature up to December 2023. All studies reporting ALPPS in the management of phCC were included. A single-arm meta-analysis of proportions was carried out to estimate the overall rate of outcomes. RESULTS: After obtaining 207 articles from the primary search, data of 18 studies containing 112 phCC patients were included in our systematic review. Rates of major morbidity and mortality were calculated to be 43% and 22%, respectively. The meta-analysis revealed a PHLF rate of 23%. One-year disease-free survival was 65% and one-year overall survival was 69%. CONCLUSIONS: ALPPS provides a good chance of cure for patients with phCC in comparison to alternative treatment options, but at the expense of debatable morbidity and mortality. With refinement of the surgical technique and better perioperative patient management, the results of ALPPS in patients with phCC were improved.

Assessing textbook outcomes following major liver surgery in association with obesity at a referral academic center.

INTRODUCTION: Outcomes of major surgeries in tertiary educational hospitals have been complicated by the referral of high-risk patients and the participation of trainees in surgical procedures. We analyzed outcomes of major hepatectomies in a tertiary academic setting emphasizing the role of training and obesity on textbook outcomes (TO). MATERIALS AND METHODS: 971 adult patients who underwent open major hepatectomy (Mesohepatectomy [n = 111], hemihepatectomy [n = 610], and extended hepatectomy [n = 250]) were evaluated. A TO was defined as: a negative resection margin, no grade B/C bile leak, no major complications, no in-hospital mortality, and no 30-day readmission. TOs were compared following operations performed by senior surgeons and those performed by junior surgeons under the supervision of senior surgeons and between patients with and without obesity. RESULTS: TO was achieved in 70.1% of patients overall (78.4% in mesohepatectomy, 73.1% in hemihepatectomy, and 59.2% in extended hepatectomy). The rate of TO was similar following operations performed by and supervised by a senior surgeon (69.3% vs 71.0%, p = 0.570). The rate of TO was significantly lower in patients with obesity (41.5% vs 74.6%, p < 0.001). Factors including increased age (odds ratio [OR] for 10-year increase = 0.83, 95% confidence interval [CI]: 0.73-0.96, p = 0.009), obesity (OR = 0.25, 95%CI: 0.16-0.37, p < 0.001), biliodigestive anastomosis (OR = 0.27, 95%CI: 0.19-0.40, p < 0.001), and portal vein resection (OR = 0.49, 95%CI: 0.28-0.87, p = 0.014) lower the rate of TO. CONCLUSION: Promising outcomes are possible after major hepatectomy in an academic setting. Obese patients and those undergoing more complex surgeries had a higher risk of poor postoperative outcomes.

Clinical and immunological characteristics of 69 leukocyte adhesion deficiency-I patients.

BACKGROUND: In order to support the comprehensive classification of Leukocyte Adhesion Deficiency-I (LAD-I) severity by simultaneous screening of CD11a/CD18, this study assessed clinical, laboratory, and genetic findings along with outcomes of 69 LAD-I patients during the last 15 years. METHODS: Sixty-nine patients (40 females and 29 males) with a clinical phenotype suspected of LAD-I were referred to Immunology, Asthma, and Allergy research institute, Tehran, Iran between 2007 and 2022 for further advanced immunological screening and genetic evaluations as well as treatment, were enrolled in this study. RESULTS: The diagnosis median age of the patients was 6 months. Delayed umbilical cord separation was found in 25 patients (36.2%). The median diagnostic delay time was 4 months (min-max: 0-82 months). Forty-six patients (66.7%) were categorized as severe (CD18 and/or CD11a: below 2%); while 23 children (33.3%) were in moderate category (CD18 and/or CD11a: 2%-30%). During the follow-ups, 55.1% of children were alive with a mortality rate of 44.9%. Skin ulcers (75.4%), omphalitis (65.2%), and gingivitis (37.7%) were the most frequent complaints. Genetic analysis of the patients revealed 14 previously reported and three novel pathogenic mutations in the ITGB2 gene. The overall survival of patients with and without hematopoietic stem cell transplantation was 79.3% and 55.6%, respectively. CONCLUSION: Physicians' awareness of LAD-I considering delayed separation of umbilical cord marked neutrophilic leukocytosis, and variability in CD11 and CD18 expression levels, and genetic analysis leads to early diagnosis and defining disease severity. Moreover, the prenatal diagnosis would benefit families with a history of LAD-I.

Comparison of the modified piggyback with standard piggyback and conventional orthotopic liver transplantation techniques: a network meta-analysis.

BACKGROUND: In conventional orthotopic liver transplantation (OLT), the recipient's retrohepatic inferior vena cava (IVC) is completely clamped and replaced with the donor IVC. The piggyback technique has been used to preserve venous return, either via an end-to-side or standard piggyback (SPB), or via a side-to-side or modified piggyback (MPB) anastomosis, using a venous cuff from the recipient hepatic veins with partially clamping and preserves the recipient's inferior vena cava. However, whether these piggyback techniques improve the efficacy of OLT is unclear. To address the low quality of the available evidence, we performed a meta-analysis to compare the efficacy of conventional, MPB, and SPB techniques. METHODS: Literature was searched in Medline and Web of Science databases for relevant articles published until 2021 without any time restriction. A Bayesian network meta-analysis was performed to compare the intra- and postoperative outcomes of conventional OLT, MPB, and SPB techniques. RESULTS: Forty studies were included, comprising 10,238 patients. MPB and SPB had significantly shorter operation times and fewer transfusions of red blood cell and fresh frozen plasma than conventional techniques. However, there were no differences between MPB and SPB in operation time and blood product transfusion. There were also no differences in primary non-function, retransplantation, portal vein thrombosis, acute kidney injury, renal dysfunction, venous outflow complications, length of hospital and intensive care unit stay, 90-day mortality rate, and graft survival between the three techniques. CONCLUSION: MBP and SBP techniques reduce the operation time and need for blood transfusion compared with conventional OLT, but postoperative outcomes are similar. This indicates that all techniques can be implemented based on the experience and policy of the transplant center.

Bariatric surgery in patients with obesity and end-stage renal disease.

BACKGROUND: Bariatric surgery has been suggested as a treatment for obesity and end-stage renal disease (ESRD). Although the number of bariatric surgeries in patients with ESRD is increasing, its safety and effectiveness in these patients are still controversial and the surgical method of choice in these patients is under debate. OBJECTIVES: To compare the outcomes of bariatric surgery between patients with and without ESRD and to assess different methods of bariatric surgery in patients with ESRD. SETTING: Meta-analysis. METHODS: A comprehensive search was conducted in Web of Science and Medline (via Pubmed) until May 2022. Tow meta-analyses were performed: A) to compare bariatric surgery outcomes among patients with and without ESRD, and B) to compare outcomes of Roux-en-Y gastric bypass (RYGB) and sleeve gastrectomy (SG) in patients with ESRD. Using a random-effect model, odds ratios (ORs) and mean differences (MDs) with 95% confidence intervals (CIs) were computed for surgical and weight loss outcomes. RESULTS: Of 5895 articles, 6 studies were included in meta-analysis A and 8 studies in meta-analysis B. The risk of bias was moderate to serious among studies. Major postoperative complications (OR = 2.82; 95% CI = 1.66-4.77; P = .0001), reoperation (OR = 2.66; 95% CI = 1.99-3.56; P < .00001), readmission (OR = 2.37; 95% CI = 1.55-3.64; P < .0001), and in-hospital/90-d mortality (OR = 4.03; 95% CI = 1.80-9.03; P = .0007) were higher in patients with ESRD. Patients with ESRD also had a longer hospital stay (MD = 1.23; 95% CI = .32-2.14; P = .008). Bleeding, leakage, and total weight loss were comparable among groups. SG showed a 10% lower rate of overall complications and significantly shorter hospital stay than RYGB did. The quality of evidence was very low for the outcomes CONCLUSIONS: Bariatric surgery in patients with ESRD seems to have higher rates of major complications and perioperative mortality than in patients without ESRD, but a comparable rate of overall complications. SG has fewer postoperative complications and could be the method of choice in these patients. These findings should be interpreted cautiously in light of the moderate to high risk of bias in most included studies.

Insights and outcomes of single-staged repair of female bladder exstrophy-epispadias complex without osteotomy: 15 Years experience of a single institution.

INTRODUCTION: Female exstrophy-epispadias complex (BEEC) has been considered as a rare malformation of the genito-urinary tract affecting. Combining procedures during the reconstruction of bladder exstrophy-epispadias complex to reduce the number of procedures and improve the outcomes has evoked great interest. OBJECTIVE: we tried to describe the application and results of a single-stage approach for reconstruction of female BEEC during initial reconstruction or following prior failed bladder closure (FBC). STUDY DESIGN: The records of 37 female patients referred for the repair of BEEC without the application of pelvic osteotomies were extracted from an institutionally approved database from September 2002 to August 2018. The mean patient age was 7.24 and 26 patients had a prior FBC. All patients underwent pelvic floor electrical stimulation and toilet training for 1 year after the closure. Complete continence was defined as having the ability to stay dry for more than 3 h without leakage during the day and night. Partial continence has traditionally been defined as retaining urine for 1-3 h or having some stress incontinence. Incontinence was defined as a continence interval of less than 1 h. RESULTS: None of the patients presented bladder prolapse or dehiscence on follow-up; while stricture developed in 2 patients (5.4%). A total of 25 (67.6%) children were dry during the day and night. However, 9 (24.3%) were dry during the day but wet at night; while 3 (8.1%) were totally incontinent. The patients were followed up for a mean of 112.56 months. DISCUSSION: Although earlier reports of this technique seem encouraging, it should be mentioned that postoperative complications are possible and difficult to manage. However, none of our patients were presented with severe postoperative complications in the follow-ups. CONCLUSION: The single-stage technique provides satisfactory outcomes in selected patients with classic bladder exstrophy. The majority of patients attained social dryness without bladder augmentation and intermittent catheterization accompanied with minimum complication rate and best cosmetic results.

Esophagus tissue engineering: from decellularization to in vivo recellularization in two sites.

To produce an esophageal scaffold with suitable features and evaluate the result of in vivo cell seeding after its implantation in the omentum and near its original anatomical position in the rat model. The esophagus of twelve rats were resected, cannulated, and decellularized via a peristaltic pump. After confirmation of decellularization and preservation of extracellular matrix, decellularized scaffolds were implanted either in the abdominal cavity (group I, n = 6) or cervical area (group II, n = 6). Histological evaluations were performed after 3 and 6 months of implantation. The results of histological evaluations, scanning electron microscopy, and the tensile test confirmed the maintenance of extracellular matrix and removal of all cellular constituents. At the time of biopsy, no evidence of inflammation was detected and the implanted scaffolds appeared normal. Histopathological evaluations of implanted tissues revealed that undifferentiated cells were seen in scaffolds of all follow-ups in both groups. Epithelial cell seeding was more advanced in biopsies of group II obtained after 6 months of operation and was accompanied by angiogenesis in surrounding adventitia. It seems that the implantation of scaffold near its original place may have an important role in further cell seeding. This method may be surpassing in comparison with traditional implantation techniques for perfecting esophageal transplantation.

Regeneration of muscular wall of the bladder using a ureter matrix graft as a scaffold.

We investigated a method for bladder augmentation in rats using a decellularized ureter graft. We used 16 rats divided into two groups of eight. After partial cystectomy, the bladders in group 1 were grafted with a 1 cm2 patch of human decellularized ureter. Rats in group 2 were untreated controls. Biopsies of the graft were taken at 1, 3 and 9 months postoperatively for histological investigation. Total removal of cells and preservation of extracellular matrix (ECM) was confirmed in the decellularized ureter. Histological examination after 1 month revealed few cells at the border of the graft. Three months after the operation, the graft was infiltrated by vessels and smooth muscle and the mucosal lining was complete. All bladder wall components resembled native bladder wall by 9 months after implantation. CD34, CD31, α-smooth muscle actin, S100, cytokeratin AE1/AE3 and vimentin were detected 9 months after the operation. We demonstrated the potential of decellularized biocompatible ureteric grafts for use as a natural collagen scaffold for bladder repair in rats.

Exposure to road noise and asthma prevalence in adults.

Noise has been reported as one of the most important risk factors for asthma, but there are some disagreements. This study aimed to investigate the effect of road noise on asthma prevalence in adults. In the current study, 3172 adults were interviewed through the ECRHS standardized questionnaire in Tehran, the capital of Iran. Exposure to road noise was assessed considering distance of individual participants from the noise monitoring stations via the spatial analysis in GIS software. Logistic regression was used to assess the effect of noise on the symptoms of asthma. Findings showed a significant positive association between wheezing with dyspnea as the best marker for asthma and noise levels at daytime (OR 1.03; 0.98-1.05) and nighttime (OR 1.05; 0.84-1.09). Also, a significant positive association was obtained between daytime and nighttime noise levels and other asthma symptoms including wheezing, nocturnal chest tightness, nocturnal dyspnea, wheezing without cold, nocturnal cough, and asthma medication. Association between current asthma and noise level was not significant. There was a significant association between population age and current asthma prevalence (P = 0.001). Therefore, chronic exposure to road noise especially in the nighttime could increase asthma prevalence. So, control of noise sources can be suggested to diminish asthma in adults.

The overall impact of COVID-19 on healthcare during the pandemic: A multidisciplinary point of view.

BACKGROUND AND AIMS: The Coronavirus disease 2019 (COVID-19) pandemic globally changed the priorities of medical and surgical procedures. It has caused many healthcare systems to stop performing their routine screenings. Altering medical clinics to COVID-19 referral centers, lockdowns, and the public fear to refer to medical centers caused a significant reduction in the referral rate; especially in the elderly. This short review article highlights the transform in clinical practice during the post-COVID era and the need for future medical revolutions. METHODS: A comprehensive literature search was separately performed by both authors. The articles published between 2019 and August 2021 were included in this study and selected according to a quality appraisal method. RESULTS: We have summarized the possible corresponding changes in the diagnosis and treatment of all fields of medical care including internal medicine, surgical, and minor subcategories after the COVID-19 pandemic. We have also discussed the potential impacts of the pandemic on all these different categories and subcategories of medicine, including the outpatient setting and clinical work. We do believe that the lack of routine check-ups has led to an increase in the stage of disease in patients with a previously diagnosed problem. On the other hand, the dramatic change in the lifestyle of the population including restricted mobility and increased consumption of unhealthy food has caused metabolic syndrome and other new diseases that have not been diagnosed and properly managed. CONCLUSION: Our findings revealed the urgent need for public health awareness. It indicated the need to carry out both psychological and screening approaches in the post-COVID era to not miss patients with a chronic disease and new cases who were undiagnosed during the COVID pandemic.

Meatal Stenosis and Atopic Condition: A Pilot Study Demonstrating a Possible Unidentified Etiology.

Meatal stenosis (MS) is known as one of the most frequent complications of circumcision. In the present study, we aimed to find any possible relationship between MS and allergic disorders. A total of 36 children with a mean±SD age of 5.84±2.03 years were referred with MS and an atopic background even in themselves or in one of their family members (Group A). There were also age-matched controls with a mean±SD age of 5.70±2.17 years who were referred to our center with allergic symptoms and no urinary complaints (Group B, n=17). The RIDA qLine allergy and allergy explorer (ALEX) tests were performed for all patients to find possible allergen sensitization. Laboratory findings revealed that IgE-sensitization to the main food and aeroallergens in Group A (with the chief complaint of MS in whom a mild atopic condition was found during concise medical history taking) were very similar to the control group with no significant difference (except for ryegrass which was higher in the control group). Although total IgE level was considerably higher in group B compared to group A, food sensitization to cow's milk and ß-lactoglobulin was higher in asthmatic patients of group A compared to the controls. It seems that not all patients with MS should be considered as a complication of circumcision and undergo a surgical procedure for correction of the stenosis. Further investigations are required to determine the role of concise medical history taking and proper treatment of the allergic disorder to reduce failed surgical attempts in atopic boys with MS.

Local tissue reaction and histopathological characteristics of three different bulking agents: a rabbit model

ABSTRACT Purpose: We assessed the efficacy and safety of a single injection of three bulking agents over the short- and long-term follow-ups in rabbits. Dermal and preputial matrices were compared with Deflux (DxHA) injection. Material and methods: Twenty-four rabbits were divided into three groups. Group I (n=8) underwent the injection of a lyophilized dermal matrix (LDM) beneath the seromuscular layer of the bladder wall. Rabbits in group II (n=8) were injected with lyophilized preputial matrix (LPM). Rabbits of group III (n=8) were injected with DxHA as the control group. They were followed up for 1 and 6 months after the injection. Subcutaneous injection of all bulking agents was also performed in nude mice. Biopsies were stained with LCA (leukocyte common antibody), CD68, CD31, and CD34. Scanning electron microscopy (SEM) and MTT assay were also performed. Results: Immunohistochemistry staining with CD68 and LCA revealed higher inflammation grade in LDM as compared with LPM and DxHA. Fibrosis grade was also higher in LDM both in short- and long-term follow-ups. However, no significant difference was detected in CD31 and CD34 staining between control and experimental groups. SEM analysis showed that the particle size of LPM was more similar to DxHA. MTT assay revealed that cell proliferation was similar in DxHA, LDM, and LPM. In-vivo assay in nude mice model showed more promising results in LPM as compared with LDM. Conclusion: The long-term results demonstrated that LPM was more similar to Deflux with the least local tissue reaction, inflammation, and fibrosis grade.

Single-staged male bladder exstrophy-epispadias complex reconstruction with pubic bone adaptation without osteotomy: 15-year single-center experience.

PURPOSE: To represent the 15 years' experience of an academic referral center for the reconstruction of bladder exstrophy-epispadias complex with a modified single-stage approach. Single-staged reconstruction techniques are commonly used for classic bladder exstrophy. However, combined bladder closure and epispadias repair have been taken into great consideration in patients with initially failed reconstruction or delayed primary closure. METHODS: A total of 49 boys underwent 1-stage bladder and epispadias repair with pubic bone adaptation and without the application of pelvic osteotomy. The mean ± SD age at surgery was 5.23 ± 2.04 months. Continence and social dryness were assessed in the follow-ups with 3 months intervals for the first year and biannually thereafter. RESULTS: The mean ± SD of follow-up was 127.25 ± 71.32 months. Urethrocutaneous fistula, stricture, wound infection, and hemiglans were developed in six distinct patients. However, no other major complications were noted. Three patients (6.1%) remained incontinent; while 32 (65.3%) children were socially continent and 14 (28.6%) children were waiting for toilet training. All the patients without previous failed closure were socially continent, while all incontinent patients had two failed closures in their history. No patient was rendered hypospadiac. CONCLUSION: Based on the experience of this institution, the application of single-stage reconstructive techniques can lead to continence, cosmetically pleasing appearance with promising outcomes, and reduction of overall operations, hospital stay and costs in the majority of cases as compared to multiple surgical procedures.

Recellularization of testicular feminization testis in C57bl6 as a natural bioreactor for creation of cellularized seminiferous tubules: an experimental study.

We determined histological aspects of implanted human decellularized testicular matrix (DTM) in C57BL6 as a primitive step for further testis tissue engineering. A total of 4 immature human testicles were obtained after bilateral orchiectomy from patients with testicular feminization syndrome. The optimal decellularization protocol was determined and the efficacy of decellularization was evaluated in two of the testicles. The remaining scaffolds were cut in 3 × 3 mm3 pieces and implanted between the tight muscles in 32 C57BL6. Biopsies were taken at 2, 4, 8, and 24 weeks postoperatively and stained with PLZF, protamine, and tekt1 markers. Histological examination of DTMs confirmed complete absence of nuclear remnants and preservation of the extracellular matrix. Successful cell seeding was observed in all follow-ups confirmed by H&E and IHC staining that increased continuously during the whole study. Interestingly, spermatogonial stem-like cells were observed on decellularized implants that were well differentiated during the follow-ups. Natural bioreactors may provide a good cell source for testes tissue regeneration. This technique may provide testis bioscaffold as a three-dimensional platform and further successful cell seeding to produce a functional testis. This novel technique may be beneficial for patients who require testicular supplementation.

Local tissue reaction and histopathological characteristics of three different bulking agents: a rabbit model.

PURPOSE: We assessed the efficacy and safety of a single injection of three bulking agents over the short- and long-term follow-ups in rabbits. Dermal and preputial matrices were compared with Deflux (DxHA) injection. MATERIAL AND METHODS: Twenty-four rabbits were divided into three groups. Group I (n=8) underwent the injection of a lyophilized dermal matrix (LDM) beneath the seromuscular layer of the bladder wall. Rabbits in group II (n=8) were injected with lyophilized preputial matrix (LPM). Rabbits of group III (n=8) were injected with DxHA as the control group. They were followed up for 1 and 6 months after the injection. Subcutaneous injection of all bulking agents was also performed in nude mice. Biopsies were stained with LCA (leukocyte common antibody), CD68, CD31, and CD34. Scanning electron microscopy (SEM) and MTT assay were also performed. RESULTS: Immunohistochemistry staining with CD68 and LCA revealed higher inflammation grade in LDM as compared with LPM and DxHA. Fibrosis grade was also higher in LDM both in short- and long-term follow-ups. However, no significant difference was detected in CD31 and CD34 staining between control and experimental groups. SEM analysis showed that the particle size of LPM was more similar to DxHA. MTT assay revealed that cell proliferation was similar in DxHA, LDM, and LPM. In-vivo assay in nude mice model showed more promising results in LPM as compared with LDM. CONCLUSION: The long-term results demonstrated that LPM was more similar to Deflux with the least local tissue reaction, inflammation, and fibrosis grade.

The most common allergens according to skin prick test: The role of wheal diameter in clinical relevancy.

The skin prick test (SPT) could be applied as a useful in vivo method for the detection of sensitization in epidemiological and diagnostic studies if the wheal size is ideally evaluated. We focused on SPT wheal size to identify sensitization pattern to common inhalant and food allergens. In this cross-sectional study, SPT results were obtained from a total of 972 allergic patients. Common allergen extracts for SPT were selected according to the type of allergic diseases, and the geographical pattern. SPT with food allergens was performed for patients with atopic dermatitis (AD) and chronic urticaria (CU). A total of 461 male (47.4%) and 511 female (52.6%) participated in this study (median age: 31 years). The majority of individuals were affected with allergic rhinitis (AR) (n = 624) and asthma (n = 224); while 129 and 67 patients suffered from AD and CU, respectively. The most common aeroallergens were Russian thistle (52.1%) and lamb's quarter (50.7%) with the largest wheal diameter. The wheal size of lamb's quarter was significantly different between patients with asthma and AR (P<.001). In addition, a significant difference was detected in wheal diameter in response to the Russian thistle between patients with AR and AD (P = .001). Shrimp (23.6%) and Peanut (22.5%) caused the most common food sensitization in patients with AD and CU. Having in mind the most common weed pollens including the Russian thistle and lamb's quarter, preventive strategies, such as, removing unwanted weeds or preventing them from growing, avoidance, and specific immunotherapy may be crucial for better disease control.

In-vivo regeneration of bladder muscular wall with whole decellularized bladder matrix: A novel hourglass technique for duplication of bladder volume in rabbit model.

OBJECTIVE: To determine histological aspects of decellularized bladder graft to achieve a double-sized bladder by novel hourglass technique; using rabbit models. METHODS: Sixteen rabbit bladders were decellularized and underwent laboratory investigations. After making a laparotomy incision and exposure of bladders in another 16 rabbits (partial detrusor myomectomy), they were separated into two groups. The fundus of the decellularized scaffold was anastomosed to the fundus of the native bladder via the serosal layer, and the omentum and a double-J stent were placed in the decellularized bladder by no direct contact with the urine (Group A, n=8). In group B (n=8), the bladder was augmented applying the decellularized bladder that was in contact with the urine. After 6 months, the omentum was brought out of the neck of the engineered bladder and the anastomosis was opened. Biopsies were taken at 1, 3, and 9 months postoperatively. RESULTS: Cell removal with preservation of extracellular matrix structure was confirmed in decellularized bladders. Histological examination after 1 month demonstrated few cells at the border of the grafts. After 3 months, the region of the graft was indistinguishable from the natural bladder with continuity of transitional epithelium of natural bladder on the decellularized grafted scaffolds. The organization of muscle layers was similar to native bladder muscle layers after 9 months. IHC staining markers were highly expressed after 9 months. Interestingly, bladders had a high fibrosis grade in group B compared with hourglass technique. CONCLUSION: We confirmed that decellularized bladder may be a reliable scaffold and viable material for bladder augmentation.

The Critical Role of Prenatal Genetic Study in Prevention of Primary Immunodeficiency in High-risk Families: The Largest Report of 107 Cases.

This study aims to investigate the role of prenatal diagnosis (PND) in Iranian couples with a previous history of primary immunodeficiency disorders (PIDD) in their family. All referred couples with a family history of PIDD and a tendency for PND were included in this project. Based on gestational age, chorionic villus sampling (CVS) was performed to analyze the molecular defect of the fetus according to the previous gene defect of the affected case in the family. Postnatal confirmation was performed by immunological screening tests. In a total of 100 cases, CVS was not evaluated in 19 patients due to unwillingness (n=5), late prenatal referral (n=7), miscarriage before CVS (n=3), and female fetus with x-linked diseases in previous children (n=4). In the remaining 81 patients, heterozygous and homozygous mutations were found in 33 and 23 cases, respectively. The hemizygous mutation was obtained in 6 and no pathogenic mutations were found in 19 individuals. Postnatal evaluations revealed that a total of 65 babies were healthy, 32 fetuses were aborted (3 cases before CVS, 2 spontaneous abortions of a healthy and as affected fetus in the CVS subgroup, and 27 cases were aborted due to therapeutic causes). One fetus from the heterozygous subgroup was spontaneously aborted with severe combined immunodeficiency (SCID) and one fetus from the homozygous subgroup that was supposed to be healthy was affected by the autosomal dominant-chronic granulomatous disease (AR-CGD). The diagnostic error was 1.2%. PND is highly recommended in families with a history of PID in their previous child to prevent an affected baby being born and to reduce the government, family, and personal burden of these diseases.

Prognostic significance of maternal urinary carbohydrate antigen 19-9 for antenatal diagnosis of posterior urethral valve associated with fetal hydronephrosis.

PURPOSE: To evaluate the predictive role of maternal urinary CA 19-9 as a non-invasive marker for diagnosing antenatal posterior urethral valve (PUV). METHODS: A total of 40 women in the third pregnancy trimester were enrolled. Case group (group A) consisted of 20 women with a diagnosis of antenatal PUV. Twenty women with similar gestational age, fetal sex, normal US, and no history of congenital anomalies were chosen as a control group (group B). Maternal urine samples were collected and urinary CA 19-9 was measured in both groups. The correlations between maternal urinary CA 19-9 and APD (measured during pregnancy and the initial evaluation of the newborn) were assessed. CA 19-9 level in first urine of neonates was also evaluated. RESULTS: The mean ± SD of maternal urine CA 19-9 was higher in PUV group compared to the control group (131.6 ± 23.8 vs. 13 ± 2.7 U/mL). In addition, there was a significant correlation between maternal urinary CA 19-9 and the APD measured at the third trimester (p < 0.001) and the initial evaluation of fetus after birth according to SFU grading system (p < 0.001). However, no significant difference was found between gestational age and urinary CA 19-9 level (p = 0.34). There was also a significant correlation between the CA 19-9 level in first urine of neonates and CA 19-9 level of maternal urine (p < 0.001). CONCLUSIONS: This is the first time that maternal urinary CA 19-9 has been applied as a noninvasive and practical diagnostic marker in antenatal PUV.