Acute but transient neurological deterioration revealing adult polyglucosan body disease

Adult polyglucosan body disease (APBD) is a metabolic disorder usually caused by glycogen branching enzyme (GBE) deficiency. APBD associates progressive walking difficulties, bladder dysfunction and, in about 50% of the cases, cognitive decline. APBD is characterized by a recognizable leukodystrophy on brain MRI. We report here a novel presentation of this disease in a 35-year old woman who presented with an acute deterioration followed by an unexpected recovery. Enzymatic analysis displayed decreased GBE activity in leukocytes. Molecular analyses revealed that only one mutated allele was expressed, bearing a p.Arg515His mutation. This is the first observation reporting acute and reversible neurological symptoms in APBD. These findings emphasize the importance of searching GBE deficiency in patients presenting with a leukodystrophy and acute neurological symptoms mimicking a stroke, in the absence of cardiovascular risk factors.     

Advagraf®, a once‐daily prolonged release tacrolimus formulation,in kidney transplantation: literature review and guidelines from a panel of experts

The efficacy and safety of tacrolimus twice‐a‐day (BID) and once‐a‐day (QD) formulations are similar. However, the available information regarding the initiation and management of tacrolimus QD is sparse and practical information is lacking. A panel of French experts extensively reviewed the available literature on tacrolimus pharmacokinetics, clinical efficacy, and safety in kidney transplantation and, based on their own day‐to‐day experience, provided the practitioners with practical guidelines for the daily use and management of tacrolimus QD in de novo initiation or early conversion.     

An OxPLORE Initiative Evaluating Children’s Surgery Resources Worldwide: A Cross-sectional Implementation of the OReCS Document

World Journal of Surgery - The Global Initiative for Children's Surgery (GICS) group produced the Optimal Resources for Children’s Surgery (OReCS) document in 2019, listing standards of...     

Shielding islets with human amniotic epithelial cells enhances islet engraftment and revascularization in a murine diabetes model

Hypoxia is a major cause of considerable islet loss during the early posttransplant period. Here, we investigate whether shielding islets with human amniotic epithelial cells (hAECs), which possess anti‐inflammatory and regenerative properties, improves islet engraftment and survival. Shielded islets were generated on agarose microwells by mixing rat islets (RIs) or human islets (HI) and hAECs (100 hAECs/IEQ). Islet secretory function and viability were assessed after culture in hypoxia (1% O₂) or normoxia (21% O₂) in vitro. In vivo function was evaluated after transplant under the kidney capsule of diabetic immunodeficient mice. Graft morphology and vascularization were evaluated by immunohistochemistry. Both shielded RIs and HIs show higher viability and increased glucose‐stimulated insulin secretion after exposure to hypoxia in vitro compared with control islets. Transplant of shielded islets results in considerably earlier normoglycemia and vascularization, an enhanced glucose tolerance, and a higher β cell mass. Our results show that hAECs have a clear cytoprotective effect against hypoxic damages in vitro. This strategy improves β cell mass engraftment and islet revascularization, leading to an improved capacity of islets to reverse hyperglycemia, and could be rapidly applicable in the clinical situation seeing that the modification to HIs are minor.     

Soil surface colonization by phototrophic indigenous organisms,in two contrasted soils treated by formulated maize herbicide mixtures

Soil phototrophic microorganisms, contributors to soil health and food webs, share their particular metabolism with plants. Current agricultural practices employ mixtures of pesticides to ensure the crops yields and can potentially impair these non-target organisms. However despite this environmental reality, studies dealing the susceptibility of phototrophic microorganisms to pesticide mixtures are scarce. We designed a 3 months microcosm study to assess the ecotoxicity of realistic herbicide mixtures of formulated S-metolachlor (Dual Gold Safeneur^®), mesotrione (Callisto^®) and nicosulfuron (Milagro^®) on phototrophic communities of two soils (Limagne vertisol and Versailles luvisol). The soils presented different colonizing communities, with diatoms and chlorophyceae dominating communities in Limagne soil and cyanobacteria and bryophyta communities in Versailles soil. The results highlighted the strong impairment of Dual Gold Safeneur^® treated microcosms on the biomass and the composition of both soil phototrophic communities, with no resilience after a delay of 3 months. This study also excluded any significant mixture effect on these organisms for Callisto^® and Milagro^® herbicides. We strongly recommend carrying on extensive soil studies on S-metolachlor and its commercial formulations, in order to reconsider its use from an ecotoxicological point of view.     

Nonalcoholic Fatty Liver Disease after Liver Transplant

Nonalcoholic fatty liver disease (NAFLD) is one of the most common causes of chronic liver disease in the world. The rising prevalence of nonalcoholic steatohepatitis (NASH) has led to a 170% increase in NASH cirrhosis as the listing indication for liver transplantation from 2004 to 2013. As of 2018, NASH has overtaken hepatitis C as an indication for liver transplantation in the USA. After liver transplantation, the allograft often develops recurrent NAFLD among patients with known NASH cirrhosis. In addition to recurrent disease, de novo NAFLD has been reported in patients with other indications for liver transplantation. In this review, we will discuss the risk factors associated with recurrent and de novo NAFLD, natural course of the disease, and management strategies after liver transplantation.     

Participating in an exercise group after anterior cruciate ligament reconstruction (ACLR) is perceived to influence psychosocial factors and successful recovery: a focus group qualitative study

ObjectivesTo explore the patients’ experiences of participating in an exercise group following anterior cruciate ligament reconstruction (ACLR).DesignHermaneutic phenomenological qualitative study of two focus groups.SettingOutpatient care, private rehabilitation centre.ParticipantsNine adults who had participated in an exercise group led by a physiotherapist following ACLR.ResultsThree major themes emerged from the data: psychosocial factors, physical outcomes and identity of the exercise group. The most significant perception of engaging in an exercise group following ACLR was its influence on psychosocial factors, especially motivation, self-confidence and social support. The group influenced the participants’ motivation, enjoyment and commitment to exercise during their rehabilitation. Social support, self-confidence and reassurance were mostly gained. The participants taking part in sport experienced the ACLR group as a substitute for sport trainings. The group was perceived to help enhance speed of recovery and facilitate the return to normal life, especially for participants with lower reported motivation and adherence to home-exercises. The authors interpreted that the subjective physical outcomes’ improvements described by all the participants was potentially an increased level of self-efficacy.The challenging role of the physiotherapist was highlighted as well as the promotion of shared accountability between patients and the group’s leader. The exercise group’s identity was questioned within the rehabilitation process, and the need for more knowledge of its existence in order to promote exercise group therapy was suggested.ConclusionParticipating in an exercise group therapy influences psychosocial factors such as motivation, self-confidence, social support, potentially self-efficacy and helps enhance a faster successful recovery following ACLR. Our findings indicate that participants with a lower reported adherence to home-exercises may especially benefit from it.     

Protective effect of immunization with nonviral antigens against Friend leukemia virus in mice

DBA/2 mice immunized with poly(A).poly(U) complexed with methylated bovine serum albumin and emulsified in Freund's complete adjuvant were protected against challenge with Friend leukemia virus. There was no correlation between the level of antibody to the immunogen in the prechallenge serum and induced resistance to the virus. Although prechallenge sera of mice given the same amount of the duplex in a single inoculum bound 9.7% of poly(A).poly([(3)H]U) input, as compared to 45.3% bound by the prechallenge sera of mice given the immunogen in divided doses, both groups of mice were equally resistant to infection. Immunization with two other nonviral agents, bovine serum albumin fraction V or dinitrophenylated keyhole limpet hemocyanin, induced the same level of protection. A sparing effect of approximately 10(1.5) in infectivity was afforded the immunized mice. Immunization with either poly(A).poly(U) alone or with the carrier methylated bovine serum albumin was ineffective.In addition to antibodies to the respective immunogens, the prechallenge sera of the immunized mice also contained antibody to Friend leukemia virus gp71. The presence of such viral antibodies was not always related to resistance to infection by Friend virus. Some immunized mice that survived infection did not have gp71 antibody in their serum before challenge, and mice immunized with poly(A).poly(U) alone were susceptible to infection, although their prechallenge sera contained antibody to gp71. The mechanism involved in the induction of resistance to infection is not known. The effect may be mediated through a modification of the expression of both endogenous and exogenous type C viruses and affect immunological mechanisms controlling cellular responses.