Management of cesarean delivery in a parturient with osteogenesis imperfecta：A case report

Osteogenesis imperfecta (OI) is characterized by bone fragility, defects in the teeth, blue sclera, and deficits in hearing and vision [1,2]. Because of an anticipated difficult airway and back anatomy, there is a high risk of choosing either general or spinal anesthesia, especially in critically ill obstetric patients. It is still controversy about the anesthesia method in patients with OI. Ultrasound-guided transversus abdominis plane block (TAPB) has been used for analgesia after cesarean section, but rarely for anesthesia in this operation [3-5]. We describe a critically parturient with OI who underwent cesarean section under ultrasound guided TAPB with spontaneous breathing general anesthesia. The patient's vital signs have remained stable during the operation, and a live female infant was delivered successfully by cesarean section. The mother and daughter were safe at last. Written informed consent was provided by the patient for publication of this report with photos.created.

The Magistral Preparation of Advanced Therapy Medicinal Products (ATMPs)

Advanced Therapy Medicinal Products (ATMPs) embody innovative therapies that have created great hope for patients suffering from previously untreatable diseases. Unfortunately, the pharaonic cost to produce and authorise ATMPs is a challenge for both patients and public health care systems, ultimately reducing patients’ access to treatment. Over the last 11 years, only 15 ATMP marketing authorisation applications received a positive draft opinion from the European Medicines Agency’s (EMA’s) Committee for Advanced Therapies (CAT). Moreover, due to poor return on investment, several ATMPs have already been removed from the market. In addition to the centralised procedure to obtain a marketing authorisation, the legislator foresees an alternative route for authorising ATMPs, the so-called “ATMP Hospital Exemption”. However, such ATMPs must be produced on a limited scale, on a non-routine basis. As a result, valuable ATMP therapies that have been used for years in hospitals may disappear. To avoid this, we propose, in this paper, an additional possibility to regularise ATMPs: the “Magistral Preparation of ATMPs”. It is a feasible pathway, which was already proposed for bacteriophage therapy, and which is particularly suitable for personalised therapies and considerably decreases the cost of the final products. We also discuss the practical impact of the ATMP regulation for (for-profit) industries and for (non-profit) hospitals. Two practical examples, the cultured human chondrocytes and the cultured human keratinocytes, are discussed.

Enterostomies for acute abdominal surgical condition in children: An experience in a developing country

Background: Pediatric abdominal surgical condition that is complicated by gross peritoneal contamination may require enterostomy as a damage control or salvage procedure. Late presentations mostly seen in developing countries make creation of enterostomy a relatively common surgical procedure. The aim of this study was to evaluate the creation of enterostomy in children who presented with acute abdominal surgical conditions. Methods: This was a retrospective study of children that had enterostomy in the pediatric surgery unit of Enugu State University Teaching Hospital, Enugu, Nigeria. Medical records of pediatric patients that had enterostomy over a 10-year period were assessed. Results: There were 32 cases of enterostomies performed during the study period. There were 25 males (78.1%) and 7 females (21.9%) with a male to female ratio of 3.6:1. The age range of the patients was 3 weeks to 180 months, with a median age of 96 months. There were 1 neonate (3.1%), 10 infants (31.3%) and 21 children (65.6%) older than 1 year. The primary diagnoses were typhoid intestinal perforation in 21 patients (65.6%), intussusception 10 (31.3%) and intestinal atresia 1 (3.1%). Ileostomy was performed in 31 patients (96.9%) and jejunostomy in 1 patient (3.1%). Enterostomy was created at the time of initial laparotomy (damage control) in 21 patients (65.6%) while 11 patients (34.4%) had their enterostomy as a salvage procedure at the time of re-exploration. Peristomal skin complication was the most common complication recorded in our patients. Mortality was 12.5%. Conclusion: Enterostomy is lifesaving in the management of acute abdominal surgical condition when there is gross peritoneal contamination in severely ill children. Proper surgical technique and electrolyte derangements are important considerations when enterostomies are created.