Results of Onlay Flap Versus Durham Smith in Proximal Hypospadias

Introduction: Urethroplasty was originally used for the repair of hypospadias of the mid and distal portion of the penis but since complications of the two-stage Durham Smith technique such as mega urethra and proximal anastomotic strictures became apparent; the Onlay island flap technique has been increasingly used in more severe cases of hypospadias. The aim of our study was to compare the outcome of these two techniques in the surgical treatment of hypospadias.Material and Methods: In this retrospective study, thirty -three patients underwent surgery for the treatment of hypospadias. The two- stage Durham smith repair was used for 17 cases and the onlay island flap technique was carried out on 16 patients. Data was analyzed using SPSS software version 21 and complication rates of the two procedures were compared.Results: Altogether, frequency of complications was higher in the smith technique than the onlay flap procedure without any significant difference between the two techniques (47% for Smith technique and 19% for onlay repair; P=0.141). Fistula was the most frequent complication in the two techniques (41% for Smith technique and 13% for onlay repair; P=0.117). Meatoplasty was done for meatal stricture in the smith group. Moreover, the smith technique failed completely in one case who then underwent Tabularized Inside-Plate (TIP). There was a case of chordee recurrence in the smith technique; for which a dorsal Nesbit plication was carried out. The mean days of hospitalization was more with the smith technique (7.1 days vs 5.0, P=0.016). In addition, the mean cases which required reoperation was higher in the smith group than the onlay flap (1.0 vs 0.2, P=0.025).Conclusions: The results of this study showed that the onlay flap technique had lower complications and reoperation rates than the two-stage smith technique. In addition, the onlay flap technique has fewer days of hospitalization than the smith technique, resulting in lower costs to the health system and patients

High Volume Center Experience for Recurrent Primary Disease in Kidney Transplanted Pediatric

Introduction: Over the past 3 decades, kidney transplantation has been recognized as the treatment of choice for children with End Stage Renal Disease (ESRD) and stage 5 of chronic kidney disease (CKD). One of the most important drawbacks to this treatment is the recurrence of the primary disease in the transplanted kidney, which is considered the third most common leading cause of graft failure. Materials & Methods: In this study, the data of 550 patients below 18 years who underwent kidney transplantation during a 33-year period from 1985 to 2017 due to kidney failure or ESRD were included to fill out a standard questionnaire. Those who suffered from primary disease relapse were included in the study to investigate the association of relapse with factors such as gender, age, and donor type, time to relapse with type of disease, and post-transplant immunosuppressive drugs with severity of pre-transplant kidney injury. Results: Of 31 pediatric patients with primary disease recurrence (out of 550 transplanted kidney), 62.5% were male with a mean age of 10.55 (± 0.665) years. Totally, 10 cases showed recurrence of the primary disease 18 (± 22.95) months after transplantation on average. The final status of these 10 patients was significantly undesirable compared with that of the other 21 patients without recurrence (p= 0.002). Of these 10 patients, 8 had graft failure. Conclusion: The results of this report confirm the necessity of follow-up considering the importance of the recurrence of the primary disease, especially FSGS, in children after kidney transplantation

Management and outcomes of hidden penis in children

Introduction: Hidden penis may have different categories: Concealed, trapped, and buried types. The aim of this study was to evaluate the results and outcomes of repaired cases.Material and Methods: A total of 40patients (5months -12years old) underwent surgical repair for different types of hidden penis from April 2010 to December 2015 in our hospital. Multiple surgical procedures were performed for each type. Data were collected and evaluated for number of patient’s, type of anomalies, surgical techniques, outcome and follow-up.Results: All forty cases underwent surgical repair. In all cases of concealed and buried penis penile degloving and penile fixation was used. In all of our cases improved function and good outcome was reported.Conclusions: Patients with hidden penis are in great psychological risks. The aim of hidden penis repair is to restore an aesthetic and functional penis which we are happy that we provided for our patients

Effects of Heparin on Early Patency of Arteriovenous Fistula in Angioaccess Surgery of Patients with End-Stage Renal Disease

Background: Arteriovenous fistula (AVF) is now the optimal method of obtaining vascular access for dialysis. Measures such as systemic anticoagulation have been proposed as means of increasing patency rates but enough evidence does not exist to support their application. We aimed to evaluate the efficacy of preoperative heparin injection on patency of AVF during the first 24 hours after surgery and to determine whether such measure can be used to prevent early thrombosis of the vascular access.Methods: The study was carried out on 150 patients admitted to Shohada-e-Tajrish hospital for permanent vascular access placement during 2011-2012. 75 patients were randomly assigned to receive 100 units/kg of heparin intraoperatively and at 24 hours post-surgery AVF patency rate was assessed and compared to the control group. Results: All the 75 patients who had received heparin intraoperatively had a patent arteriovenous fistula 24 hours post-surgery which showed a statistically significant difference compared to the control group among which only 69 (92%) patients had a functioning AV fistula (p-value= 0.028).Conclusions: Our results show that systemic anticoagulation with heparin can be considered as an effective option in preventing vascular access failure. However, considering the contradictory data on the usefulness of heparin injection, larger trials are needed to evaluate efficacy and adverse effects of systemic intraoperative anticoagulation in End-Stage Renal Disease (ESRD) patients before qualifying it as a method of increasing AVF patency in these patient

Prevalence of central venous stenosis among Black and White ESKD patients with dysfunctional dialysis access

In the United States, significant racial and ethnic disparities exist in chronic kidney disease (CKD) and its management. Hemodialysis constitutes the main stay of renal replacement therapy for end-stage kidney disease (ESKD), which is initiated using central venous catheters (CVC) in most CKD patients in the United States. Black ESKD patients have higher usage and greater time on CVC for hemodialysis compared to White patients. This trend places Black patients at a potentially higher risk for CVC-related complications such as central venous stenosis (CVS). We posited that Black patients would have a higher prevalence and a greater risk of CVS. A retrospective review was performed of ESKD patients who underwent a fistulogram for dialysis access malfunction. CVS was defined as \u3e 50% stenosis in the central veins. Fistulograms of 428 ESKD patients were adjudicated, and CVS was noted in 167 of these patients. Of the entire cohort, 370 fistulograms belonged to self-reported unique Black and White ESKD patients, of whom 137 patients were noted to have CVS. There was no difference in the of CVS between Black (40%) and White (41%) ESKD patients. However, a higher severity of stenosis (\u3e70%) (P = 0.03) was noted in White ESKD patients. An unadjusted model showed a significant association between CVS and cardiovascular disease and the use of CVCs. The risk-adjusted model showed a significant association between diabetes and CVS. Unlike arterial stenotic lesions, this work for the first time demonstrated higher prevalence of severe venous stenotic lesions in White ESKD patients and linked diabetes to stenotic venous disease. This work paves the way for future studies investigating the risk and influence of race and ethnicity on CVS using a larger and diverse data set

Epidemiology of Cancer-Associated Venous Thromboembolism in Patients With Solid and Hematologic Neoplasms in the Veterans Affairs Health Care System

IMPORTANCE: Identifying changes in epidemiologic patterns of the incidence and risk of cancer-associated thrombosis (CAT), particularly with evolving cancer-directed therapy, is essential for risk stratification. OBJECTIVE: To assess the incidence of CAT over time and to determine pertinent patient-specific, cancer-specific, and treatment-specific factors associated with its risk. DESIGN, SETTING, AND PARTICIPANTS: This longitudinal, retrospective cohort study was conducted from 2006 to 2021. Duration of follow-up was from the date of diagnosis until first venous thromboembolism (VTE) event, death, loss of follow-up (defined as a 90-day gap without clinical encounters), or administrative censoring on April 1, 2022. The study took place within the US Department of Veterans Affairs national health care system. Patients with newly diagnosed invasive solid tumors and hematologic neoplasms were included in the study. Data were analyzed from December 2022 to February 2023. EXPOSURE: Newly diagnosed invasive solid tumors and hematologic neoplasms. MAIN OUTCOMES: Incidence of VTE was assessed using a combination of International Classification of Diseases, Ninth Revision, Clinical Modification and International Statistical Classification of Diseases, Tenth Revision, Clinical Modification and natural language processing confirmed outcomes. Cumulative incidence competing risk functions were used to estimate incidence of CAT. Multivariable Cox regression models were built to assess the association of baseline variables with CAT. Pertinent patient variables included demographics, region, rurality, area deprivation index, National Cancer Institute comorbidity index, cancer type, staging, first-line systemic treatment within 3 months (time-varying covariate), and other factors that could be associated with the risk of VTE. RESULTS: A total of 434 203 patients (420 244 men [96.8%]; median [IQR] age, 67 [62-74] years; 7414 Asian or Pacific Islander patients [1.7%]; 20 193 Hispanic patients [4.7%]; 89 371 non-Hispanic Black patients [20.6%]; 313 157 non-Hispanic White patients [72.1%]) met the inclusion criteria. Overall incidence of CAT at 12 months was 4.5%, with yearly trends ranging stably from 4.2% to 4.7%. The risk of VTE was associated with cancer type and stage. In addition to confirming well-known risk distribution among patients with solid tumors, a higher risk of VTE was observed among patients with aggressive lymphoid neoplasms compared with patients with indolent lymphoid or myeloid hematologic neoplasms. Compared with no treatment, patients receiving first-line chemotherapy (hazard ratio [HR], 1.44; 95% CI, 1.40-1.49) and immune checkpoint inhibitors (HR, 1.49; 95% CI, 1.22-1.82) had a higher adjusted relative risk than patients receiving targeted therapy (HR, 1.21; 95% CI, 1.13-1.30) or endocrine therapy (HR, 1.20; 95% CI, 1.12-1.28). Finally, adjusted VTE risk was significantly higher among Non-Hispanic Black patients (HR, 1.23; 95% CI, 1.19-1.27) and significantly lower in Asian or Pacific Islander patients (HR, 0.84; 95% CI, 0.76-0.93) compared with Non-Hispanic White patients. CONCLUSIONS AND RELEVANCE: In this cohort study of patients with cancer, a high incidence of VTE was observed, with yearly trends that remained stable over the 16-year study period. Both novel and known factors associated with the risk of CAT were identified, providing valuable and applicable insights in this current treatment landscape

Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

Necrolytic Migratory Erythema as the First Manifestation of Glucagonoma

Necrolytic migratory erythma (NME) as a rare skin disorder that can affected Perineum, distal extremities, lower abdomen and face are the most commonly affected sites.It can be as a part of Glucagonoma syndrome that is defined as an association of glucagonoma with NME, hyperglucagonemia, glucose intolerance, anemia and weight loss. Here, Authors describe a woman admitted to the dermatology ward with NME which was later found to be associated with glucagonoma and multiple hepatic lesions

End Organ Affection in Sickle Cell Disease

Sickle cell disease is an orphan disease affecting ethnic minorities and characterized by profound systemic manifestations. Although around 100,000 individuals with SCD are living in the US, the exact number of individuals is unknown, and it is considered an orphan disease. This single-gene disorder leads to red blood cell sickling and the deoxygenation of hemoglobin, resulting in hemolysis. SCD is associated with acute complications such as vaso-occlusive crisis, infections, and chronic target organ complications such as pulmonary disease and renal failure. While genetic therapy holds promise to alter the fundamental disease process, the major challenge in the field remains the target end organ damage and ways to mitigate or reverse it. Here, we provide an overview of the clinical manifestations and pathogenesis with a focus on end-organ damage and current therapeutic options, including recent FDA-approved stem cell and gene editing therapies

Objective Evaluation of the Therapeutic Effects of Oral Steroids in the Management of Biliary Atresia: Therapeutic Effects of Oral Steroids in Biliary Atresia

Introduction: Biliary atresia is a progressive fibro-obliterativecholangiopathy that affects both intrahepatic and extrahepatic biliary ducts causing cholestasis and neonatal jaundice. The use of anti-inflammatory agents such as corticosteroids may decrease inflammation and recurrent stricture. Materials and Methods: This clinical trial had been performed on 24 patients admitted to Children’s Hospital with biliary atresia. Prednisolone was not administered in the control group after the operation, but in the intervention group, 2 mg/kg/day of prednisolone was given for 6 weeks and then tapered. Then, its therapeutic effect in the management of biliary atresia was compared. Results: The mean age of the patients in the case and control groups was 2.19 and 2 months, respectively. Cholangitis (P= 0.3), direct hyperbilirubinemia (P= 0.6), ascites (P= 0.5), pigmented stools (P= 0.7), and esophageal varices (P= 0.1) between the two groups was not significant. But the age of the patients, outcome of treatment (P= 0.05), total hyperbilirubinemia (P= 0.05), growth failure (P=0.03), worsening grade of splenomegaly (P= 0.04), hepatomegaly (P= 0.03), fecal pigmentation (P= 0.003), death (P= 0.01), and portal hypertension (P= 0.02) in two groups were significant. Conclusion: Regarding the results, corticosteroids have a significant effect on reducing the bilirubin levels, improving the survival rate, and decreasing the mortality rate, although these results are closely related to the patient’s age at the time of surgery