Epidemiology and outcomes of pediatric autosomal recessive polycystic kidney disease in the Middle East and North Africa.

The incidence of rare diseases is expected to be comparatively higher in the Middle East and North Africa (MENA) region than in other parts of the world, attributed to the high prevalence of consanguinity. Most MENA countries share social and economic statuses, cultural relativism, religious beliefs, and healthcare policies. Polycystic kidney diseases (PKDs) are the most common genetic causes of kidney failure, accounting for nearly 8.0% of dialysis cases. The development of PKDs is linked to variants in several genes, including PKD1, PKD2, PKHD1, DZIP1L, and CYS1. Autosomal recessive PKD (ARPKD) is the less common yet aggressive form of PKD. ARPKD has an estimated incidence between 1:10,000 and 1:40,000. Most patients with ARPKD require kidney replacement therapy earlier than patients with autosomal dominant polycystic kidney disease (ADPKD), often in their early years of life. This review gathered data from published research studies and reviews of ARPKD, highlighting the epidemiology, phenotypic presentation, investigations, genetic analysis, outcomes, and management. Although limited data are available, the published literature suggests that the incidence of ARPKD may be higher in the MENA region due to consanguineous marriages. Patients with ARPKD from the MENA region usually present at a later disease stage and have a relatively short time to progress to kidney failure. Limited data are available regarding the management practice in the region, which warrants further investigations.

Efficacy and Safety of Infliximab Versus Adalimumab in Adult Subjects With Moderate to Severe Ulcerative Colitis: A Systematic Review and Meta-Analysis.

Ulcerative colitis (UC) is an inflammatory disorder affecting the colon, and typically, during the disease course, the condition may exacerbate, relapse, and remit. One of the most successful lines for inducing and maintaining clinical remission in subjects with UC is biological therapy with anti-tumor necrosis factor α (anti-TNF) agents, including adalimumab (ADA) and infliximab (IFX). This meta-analysis is an attempt to obtain complementary information driven by real-world experience (RWE) concerning the efficacy and safety of two of the most popular anti-TNFs in treating UC. This is a systematic review and meta-analysis of RWE studies comparing ADA and IFX as naïve anti-TNF agents for the treatment of subjects with UC. Studies were obtained by searching Scopus, Google Scholar, the Cochrane Central Register of Controlled Trials, Embase, and the PubMed Central databases. Patients treated with IFX showed significantly higher induction responses. No statistically significant difference was found in the comparison of response in the maintenance treatment period. Higher overall adverse events were related to IFX treatment, with serious adverse events that were nonsignificantly higher in the ADA-treated group. In conclusion, IFX demonstrated significantly higher induction responses compared to ADA in patients with moderate-to-severe UC. IFX was associated with higher overall adverse events, whereas serious adverse events were non-significantly higher in the ADA-treated group. IFX may be favored as a first-line agent for its induction efficacy, and the choice between IFX and ADA should be individualized based on comprehensive clinical evaluation.

Internal Herniation of Small Bowel Underneath Iliac Vascular Axis After Pelvic Surgery: A Systematic Review.

Internal abdominal hernias are rare entities that most commonly present with acute small bowel obstruction. These hernias can be congenital or acquired. While congenital hernias are considered the most common type, acquired hernias are becoming more common. Recently, a rare type of internal herniation has been reported underneath iliac vasculature in patients who have undergone pelvic lymph node dissection in the past. This study was carried out to assess the prevalence of this rare type of internal hernia. Two reviewers searched the literature in three online databases using the Cochrane methodology for systematic reviews. The search of databases yielded 70 articles. The studies which reported internal herniation underneath iliac vasculature were included. Studies that reported herniation underneath other pelvic organs or vasculature were excluded. After screening, 17 articles were deemed suitable and selected. All 17 cases reviewed underwent pelvic lymph node dissection in the past. The median latency period between index surgery and clinical presentation with the incarcerated hernia was 20 months. All 17 cases were managed surgically with small bowel resection carried out in 13 cases. Eleven authors reported closing the hernia defect with various techniques, while five decided not the close it. All 17 cases were alive at the time of discharge from the hospital, with a mean hospital stay of 12.7 days. Given our findings, there should be a high index of suspicion of internal hernia in patients presenting with small bowel obstruction with a history of pelvic lymph node dissection. In our review, internal herniation was always preceded by pelvic lymph node dissection, so the closure of the peritoneum should be considered while pelvic lymph node dissection is carried out.

Strangulated Small Bowel Internal Hernia Under the External Iliac Artery After Robotic Cystoprostatectomy.

Small bowel obstruction is a common surgical pathology encountered in the emergency department. The most common cause of small bowel obstruction is adhesions secondary to previous abdominal surgery. While strangulated external hernias are also a common cause of obstructions encountered, internal hernias leading to obstruction are rare. We present a 76-year-old male who presented with an acute abdomen and was later diagnosed with an internal hernia underneath his right external iliac artery.  Internal herniation underneath the iliac vasculature is a recent phenomenon encountered very rarely after the natural anatomy has been disturbed in patients who have undergone pelvic lymph node dissection. Patients with a previous history of pelvic lymph node dissection should be suspected of having an internal hernia if they present with an acute abdomen. Closure of the peritoneum should also be considered in these patients as it may help prevent herniation.

Nutritional Complications After Laparoscopic Roux-en-Y Gastric Bypass and One-Anastomosis Gastric Bypass: A Comparative Systematic Review and Meta-Analysis.

A systematic review and meta-analysis were carried out involving studies that compared the nutritional complications of Roux-en-Y gastric bypass (RYGB) and one-anastomosis gastric bypass (OAGB); these included the incidence of malnutrition as well as deficiencies of other nutritional elements, such as total protein, albumin, calcium and iron. A comprehensive search strategy was implemented in PubMed, Embase, and the Cochrane Library. Effect sizes included the pooled odds ratios (ORs) and 95% confidence intervals (95% CIs), as well as mean differences (MDs) and 95% CIs of the percentage total weight loss (%TWL) and excess weight loss percentage (%EWL). Thirteen studies were included (12,964 patients, 66.27% females, 53.82% underwent OAGB). At the longest follow-up period (≥3 years), OAGB was associated with significantly higher %TWL (MD=5.41%, 95%CI, 1.52 to 9.29) and %EWL (MD=13.81%, 95%CI, 9.60 to 18.02) compared to RYGB. However, OAGB procedures were associated with malnutrition (OR=3.00, 95%CI, 1.68 to 5.36, p<0.0001), hypoalbuminemia (OR=2.38, 95%CI, 1.65 to 3.43, p<0.0001), hypoproteinemia (OR=1.85, 95%CI, 1.09 to 3.14, p=0.022), anemia (OR=1.38, 95%CI, 1.08 to 1.77, p=0.011), and hypocalcemia (OR=1.78, 95%CI, 1.01 to 3.12, p=0.046). On subgroup analyses, the proportions of anemia and hypoalbuminemia remained significantly higher at longer follow-up periods and in studies published in Asia. Despite the favorable weight loss profile, the unfavorable nutritional consequences of OAGB merits further investigations to explore the malabsorptive element, ethnic variation, and the role of biliopancreatic limb length.

The Impact of Drain and Reinforcement on the Outcomes of Bariatric Surgery: A Prospective Study.

Purpose We aimed to investigate the impact of reinforcement and abdominal drains on the outcome of laparoscopic sleeve gastrectomy (LSG). Methods The present study was a prospective study that included obese patients scheduled to undergo LSG. Patients were assigned to receive drain, reinforcement, or both according to the surgeon's preference and followed up for one month after surgery. The present study's primary outcome was the identification of the association between intraoperative drain/reinforcement and the incidence of postoperative complications. Results A total of 125 (20.3%) patients received intraoperative drains. The proportion of postoperative morbidity was comparable between the drain and non-drain groups (3.2% versus 1.6%; p = 0.25). Patients in the drain group had similar incidence of blood transfusion (2.4% versus 1.7% in non-drain group; p = 0.43) and postoperative leakage (0.8% versus 0.2% in non-drain group; p = 0.36). The incidences of blood transfusion (p = 0.56) and reoperation (p = 0.98) were comparable between the drain and non-drain groups. There were no statistically significant differences between the drain and non-drain groups regarding postoperative mortality and wound infection (p > 0.05). On the other hand, 440 (71.3%) patients received reinforcement. The proportion of postoperative morbidity was comparable between the reinforcement and non-reinforcement groups (1.6% versus 2.8%, p = 0.07). Patients in the reinforcement group were less likely to develop postoperative bleeding (0.7% versus 4% in the non-reinforcement group; p = 0.004), while no significant difference was detected in terms of postoperative leakage (p = 0.33) and in-hospital mortality. Conclusion In conclusion, abdominal drainage did not reduce the complications of LSG patients. Reinforcement has some role in controlling the bleeding but not leaks. Both techniques did not significantly impact the mortality rate. In the future, additional, large randomized trials are needed to examine the gastrointestinal-related quality of life.

Sarcopenia increases 1-year mortality after surgical resection of hepatocellular carcinoma.

BACKGROUND: Liver resection is a well-recognized modality for hepatocellular carcinoma. Cirrhotic patients are more prone to adverse consequences after liver resection. This work assesses the prognostic significance of sarcopenic hepatocellular carcinoma cases for whom surgical resection was performed. METHODS: The present prospective work included 52 cirrhotic cases. Computed tomography scans were used to determine the skeletal muscle index (SMI) at the plane of the third lumbar vertebra (L3). L3 SMI was used for the definition of sarcopenia. The primary outcome measure was the predictive value of sarcopenia for 1-year post-hepatectomy mortality. RESULTS: Sarcopenia was diagnosed in 27 patients (51.9%). All patients had a Child-Turcotte-Pugh score A. At a 1-year follow-up, 20 cases died; that is the 1-year mortality rate was 38.5%. Sarcopenia was more commonly associated with older age and non-viral causes of cirrhosis. The risk of 1-year mortality is 7.6 times higher in sarcopenic patients with a risk ratio of 3.7 (95% confidence interval 1.4-9.6). CONCLUSION: Sarcopenia diagnosed using L3 SMI is an independent prognostic factor for 1-year deaths in cases with hepatic malignancy with Child-Turcotte-Pugh score A undergoing surgical resection.