Congenital aflatoxicosis, mal-detoxification genomics & ontogeny trigger immune-mediated Kotb disease biliary atresia variant: SANRA compliant review.

Biliary atresia (BA) is the most common indication for pediatric liver transplantation. We describe The BA variant: Kotb disease. Liver tissue in the Kotb disease BA is massively damaged by congenital aflatoxicosis resulting in inflammation, adhesions, fibrosis, bile duct proliferation, scarring, cholestasis, focal syncytial giant cell transformation, and typical immune response involving infiltration by CD4+, CD8+, CD68+, CD14+, neutrophil infiltration, neutrophil elastase spill, heavy loads of aflatoxin B1, accelerated cirrhosis, disruption of p53 and GSTPi, and have null glutathione S transferase M1 (GSTM1). All their mothers are heterozygous for GSTM1. This inability to detoxify aflatoxicosis results in progressive inflammatory adhesions and obliterative cholangiopathy early in life. The typical disruption of both p53 and GSTPi causes loss of fidelity of hepatic regeneration. Hence, regeneration in Kotb disease BA typically promotes accelerated cirrhosis. The immune response in Kotb disease BA is for damage control and initiation of regeneration, yet, this friendly fire incurs massive structural collateral damage. The Kotb disease BA is about actual ongoing hepatic entrapment of aflatoxins with lack of ability of safe disposal due to child detoxification-genomics disarray. The Kotb disease BA is a product of the interaction of persistent congenital aflatoxicosis, genetic lack of GSTM1 detoxification, ontogenically impaired activity of other hepatic detoxification, massive neutrophil-elastase, immune-induced damage, and disturbed regeneration. Ante-natal and neonatal screening for aflatoxicosis, avoiding cord milking, and stringent control of aflatoxicosis content of human, poultry and live-stock feeds might prove effective for prevention, prompt diagnosis and management based on our recent understanding of its patho-genomics.

Homemade Glove Retrieval Bag in Laparoscopic Complete Excision of Benign Ovarian Teratoma with Ovarian Tissue Preservation in Children: A Case Series.

STUDY OBJECTIVE: To present our experience of laparoscopic resection of pediatric benign ovarian teratomas with gonadal preservation, using a homemade glove retrieval bag. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: Review of all girls with benign ovarian teratomas who were managed with laparoscopic ovarian-sparing surgery (OSS) at our hospital between January 2013 and December 2018. RESULTS: Eleven patients were included for analysis with a mean age of 6.1 years. Ten patients received elective surgery, whereas 1 patient received emergency surgery because of ovarian torsion. Main indication for OSS was the existence of a dissection plane between tumor margins and healthy ovarian tissue. Postoperative outcome and follow-up were uneventful with a median follow-up of 30.1 months (range; 12-60 months). CONCLUSION: Laparoscopic OSS can be safely performed for these tumors. Laparoscopic magnification with energy devices are excellent tools in such procedures. The homemade glove bag can be used to retrieve the tumor effectively in countries with limited resources.

Laparoscopic Posterolateral Suture Rectopexy for Recurrent Rectal Prolapse in Children.

Introduction: Laparoscopic rectopexy for recurrent rectal prolapse (RP) is more widely used nowadays. Strict indications are needed to get proper outcomes. The advantages rely on the careful dissection of peritoneal sac and fixation of rectum. One of the theories of recurrent RP is hernia-like physiology in front of the rectum. Purpose: The aim of this study is to present our experience of posterolateral laparoscopic suture rectopexy (LSR) in indicated children of recurrent RP. Patients and Methods: Sixteen patients were included with recurrent RP wherein all were subjected to LSR procedure after exclusion of other probable causes. Dissection of peritoneal sac anterior to the rectum was carried out followed by closure of the deep pouch by nonabsorbable sutures then fixation of the right side of rectum and sigmoid to the lateral wall of areolar tissue. Fixation to sacral promontory is done by Ethibond or Prolene sutures when redundancy is obvious. Results: The study included 11 girls and 5 boys with age ranging between 3 and 12 years in the past 5 years. Ten cases were treated earlier with injection therapy and 6 following Thiersh procedure after failure of conservative treatment for 6 months. Operative time ranged between 40 and 100 minutes. Follow-up period ranged between 6 and 36 months with mean of 19.5 months. Postoperative mucosal prolapse reported in 1 case 6 months postsurgery with no full thickness recurrence. Conclusions: LSR is an efficient technique in well-selected children of recurrent RP and could reverse this underlying pathology. Longer follow-up and evidence are needed to standardize the technique.