Acute liver failure in low-income and middle-income countries.

Acute liver failure is a rare condition involving the rapid development, progression, and worsening of liver dysfunction, characterised by coagulopathy and encephalopathy, and has a high mortality unless liver transplantation is performed. Population-based studies are scarce, and most published data are from high-income countries, where the main cause of acute liver failure is paracetamol overdose. This Review provides an overview of the scanty literature on acute liver failure in low-income and middle-income countries, where patients are often admitted to primary care hospitals and viral hepatitis (especially hepatitis E), tropical infections (eg, dengue), traditional medicines, and drugs (especially anti-tuberculosis drugs) have an important role. We discuss incidence, cause, occurrence in children and pregnant women, prognostic factors and scores, treatment, and mortality. To conclude, we advocate for international collaboration, the establishment of central registries for the condition, and better diagnostics.

Case Report: First Two Identified Cases of Fabry Disease in Central Asia.

Background: Fabry disease (FD, OMIM #301500) is a rare, progressive, X-linked inherited, genetic disease due to the functional deficiency of lysosomal α-galactosidase (α-GAL) that leads to the accumulation of glycosphingolipids (mainly globotriaosylceramide or Gb3) and its derivative globotriaosylsphingosine or lyso-Gb3. Classic FD is a multisystem disorder which initially presents in childhood with neuropathic pain and dermatological, gastrointestinal, ocular, and cochleo-vestibular manifestations. Over time, end-organ damage such as renal failure, cardiac arrhythmia and early stroke may develop leading to reduced life expectancy in the absence of specific treatment. Case presentation: We describe two Kazakh patients who presented in adulthood with a delayed diagnosis. We conducted also a family screening through cascade genotyping. Conclusion: This is the first description of cases of Fabry disease in Central Asia. An extensive family pedigree enabled the identification of ten additional family members. Patients with rare genetic diseases often experience substantial delays in diagnosis due to their rarity and non-specific symptoms, which can negatively impact their management and delay treatment. FD may be difficult to diagnose because of the non-specificity of its early and later-onset symptoms and its X-linked inheritance. Raising awareness of clinicians is important for earlier diagnosis and optimal outcome of specific therapies.

Everyday ethical challenges of nurse-physician collaboration.

BACKGROUND: Collaboration between physicians and nurses is key to improving patient care. We know very little about collaboration and interdisciplinary practice in African healthcare settings. RESEARCH QUESTION/AIM: The purpose of this study was to explore the ethical challenges of interdisciplinary collaboration in clinical practice and education in Botswana Participants and research context: This qualitative descriptive study was conducted with 39 participants (20 physicians and 19 nurses) who participated in semi-structured interviews at public hospitals purposely selected to represent the three levels of hospitals in Botswana (referral, district, and primary). ETHICAL CONSIDERATIONS: Following Institutional Review Board Approval at the University of Pennsylvania and the Ministry of Health in Botswana, participants' written informed consent was obtained. FINDINGS: Respondents' ages ranged from 23 to 60 years, and their duration of work experience ranged from 0.5 to 32 years. Major qualitative themes that emerged from the data centered on the nature of the work environment, values regarding nurse-doctor collaboration, the nature of such collaboration, resources available for supporting collaboration and the smooth flow of work, and participants' views about how their work experiences could be improved. DISCUSSION: Participants expressed concerns that their work environment compromised their ability to provide high-quality and safe care to their patients. The physician staffing structure was described as consisting of a few specialists at the top, a vacuum in the middle that should be occupied by senior doctors, and junior doctors at the bottom-and not a sufficient number of nursing staff. CONCLUSION: Collaboration between physicians and nurses is critical to optimizing patients' health outcomes. This is true not only in the United States but also in developing countries, such as Botswana, where health care professionals reported that their ethical challenges arose from resource shortages, differing professional attitudes, and a stressful work environment.

Portal Vein Thrombosis in a 21-Year-Old Man with Membranoproliferative Glomerulonephritis and Nephrotic Syndrome.

Membranoproliferative glomerulonephritis, one of the main causes of nephrotic syndrome, is associated with a state of hypercoagulability that leads to increased risk of thrombotic events. Portosystemic collaterals may reopen due to reversal of the flow within the existing veins and be a presenting feature of thrombosis. We describe a patient who presented with large portosystemic collaterals and signs of portal hypertension and was subsequently found to be affected by membranous proliferative glomerulonephritis. Proteinuria and microscopic haematuria in a patient with signs of portal hypertension and no pre-existing liver disease should raise the suspicion of an underlying kidney disease.

Defensive medicine: It is time to finally slow down an epidemic.

Defensive medicine is widespread and practiced the world over, with serious consequences for patients, doctors, and healthcare costs. Even students and residents are exposed to defensive medicine practices and taught to take malpractice liability into consideration when making clinical decisions. Defensive medicine is generally thought to stem from physicians' perception that they can easily be sued by patients or their relatives who seek compensation for presumed medical errors. However, in our view the growth of defensive medicine should be seen in the context of larger changes in the conception of medicine that have taken place in the last few decades, undermining the patient-physician trust, which has traditionally been the main source of professional satisfaction for physicians. These changes include the following: time directly spent with patients has been overtaken by time devoted to electronic health records and desk work; family doctors have played a progressively less central role; clinical reasoning is being replaced by guidelines and algorithms; the public at large and a number of young physicians tend to believe that medicine is a perfect science rather than an imperfect art, as it continues to be; and modern societies do not tolerate the inevitable morbidity and mortality. To finally reduce the increasing defensive behavior of doctors around the world, the decriminalization of medical errors and the assurance that they can be dealt with in civil courts or by medical organizations in all countries could help but it would not suffice. Physicians and surgeons should be allowed to spend the time they need with their patients and should give clinical reasoning the importance it deserves. The institutions should support the doctors who have experienced adverse patient events, and the media should stop reporting with excessive evidence presumed medical errors and subject physicians to "public trials" before they are eventually judged in court.

Detection of Hepatitis E Antibodies in Kazakhstan: A Pilot Study.

INTRODUCTION: Hepatitis E virus exposure is associated with sporadic cases of acute hepatitis and outbreaks in many countries worldwide. It is particularly dangerous for pregnant women, in whom the mortality rate is high. There are no previously published data reporting circulation of this virus in Kazakhstan. METHODS: We tested blood samples for IgG anti-hepatitis E virus antibodies in 199 Kazakh participants; of these 119 were workers at the EXPO 2017 building site in Astana, 35 were volunteers who got tested at the Astana City Hall on the World Hepatitis Day 2017, and 45 were volunteers who presented for screening at the Hepatogastroenterology Outpatient Clinic of the Republican Diagnostic Center, University Medical Center. RESULTS: 11 (5.5%) individuals were positive for IgG anti-HEV antibodies, with a higher seroprevalence in males (7; 6.8%) vs females (4; 4.5%). The highest number of positive samples was in the 32-46 years age group. CONCLUSIONS: This pilot study suggests that Hepatitis E virus has been circulating in Kazakhstan. Studies are needed to determine whether it continues to be present, which viral genotypes are involved and what are the best methodologies for preventing its spread.