Achalasia is a chronic idiopathic disease characterized by the absence of esophageal body peristalsis and by defective lower esophageal sphincter (LES) relaxation. The incidence rate ranges from 1.07 to up to 2.8 new cases per year per 100,000 population. Presenting symptoms include dysphagia, regurgitation, vomiting, and weight loss. The diagnosis of achalasia has undergone a revolution in the last decade due to the advent of high-resolution manometry (HRM) and the consequent development of the Chicago Classification. Recent progress has allowed achalasia to be more precisely diagnosed and to be categorized into three subtypes, based on the prevalent manometric features of the esophageal peristalsis. Treatment options are pharmacotherapy, endoscopic management (Botox injection or pneumatic dilation), and surgery, e.g. laparoscopic Heller myotomy (LHM). More recently, a new endoscopic technique, per oral endoscopic myotomy (POEM), has developed as a less invasive approach alternative to the traditional LHM. Since the first POEM procedure was performed in 2008, increasing evidence is accumulating regarding its efficacy and safety profiles. Currently, POEM is being introduced as a reasonable therapeutic option, though randomized controlled trails are still lacking. The current review sheds light onto the diagnosis and manage¬ment of achalasia, with special focus on the recent advances of HRM and POEM.
To date, the only known effective treatment for celiac disease is a strict gluten-free diet for life. We reviewed the literature to evaluate the upper limit for gluten content in food, which would be safe for patients with celiac disease. Patients with celiac disease should limit their daily gluten intake to no more than 10–50 mg. Most health authorities define gluten-free products as containing less than 20 parts per million gluten.
Stents are widely use in endoscopic urological procedures. One of the most important indications is the treatment of urinary tract strictures. Allium™ Medical has introduced several types of stents for the treatment of different types of urinary tract strictures, based on anatomic location. All the stents are made of nitinol and coated with a co-polymer that reduces encrustations. These stents are self-expandable and have a large caliber and a high radial force. They have different shapes, designed especially for the treatment of each type of stricture. One of the most important features of Allium-manufactured stents is the ease of removal, due to their special unraveling feature. The company has introduced the Bulbar Urethral Stent (BUS) for treatment of bulbar urethral strictures; a rounded stent available in different lengths. Initial data on 64 patients with bulbar urethral stricture treated with the BUS showed a significant improvement in symptoms, with minimal complications and few adverse events. For treatment of prostate obstruction in patients unfit for surgery or unwilling to undergo a classical prostatic surgery, the Triangular Prostatic Stent (TPS) was introduced, which has a triangular shape that fits in the prostatic urethra. Its body has a high radial force attached to an anchor (which prevents migration) through a trans-sphincteric wire (which reduces incontinence rate). Initial data on 51 patients showed significant improvement in symptoms and in urinary peak flow rate, with a relatively small number of complications. The Round Posterior Stent (RPS) was designed for treatment of post radical prostatectomy bladder neck contracture. This short, round stent has an anchor, which is placed in the bladder neck. This stent being relatively new, the clinical data are still limited. Ureteral strictures can be treated with the Ureteral Stent (URS), which is round-shaped, available in different lengths, and has an anchor option (for very distal or very proximal strictures). We have previously published data on 107 URSs inserted in patients with ureteral stricture due to several etiologies, including patients who failed previous treatment. All patients were asymptomatic for a long period of follow-up after stent removal, with only one case of re-stenosis. In this paper, we review the urological “covered” stents produced by Allium Medical with the relevant clinical data available at the present time.
Gaucher disease (GD) is an autosomal recessive disease characterized by the buildup of glucocerebrosides in macrophages, resulting in the formation of “Gaucher cells.” These cells predominantly infiltrate the liver, spleen, and bone marrow leading to hepatosplenomegaly, cytopenia, and bone pain. Anemia in GD is typically considered to result from non-hemolytic processes. Although rare, a higher rate of hemolytic anemia of the autoimmune type has been reported in GD than in the general population. The literature on non-immune hemolytic anemia in GD is scarce. We review the literature on hemolytic anemia in GD and report on a case of non-immune hemolytic anemia secondary to GD. We believe this is the first description of a patient with confirmed GD and symptomatic non-immune hemolytic anemia that responded to GD-specific treatment.
Background: Transthyretin (TTR), also known as prealbumin, has been suggested as an indicator of protein and nutritional status.
Objective: The aim of this study was to examine the maternal and umbilical cord (UC) TTR in relation to intrauterine growth, and the serum TTR of preterm infants in relation to nutritional status and growth.
Methods: After application of exclusion criteria, 49 preterm infants (mean gestational age and birth-weight 32.9±2.9 weeks and 1822±556 g) were included in the study. Transthyretin was sampled at birth and on days 14, 28 or at discharge with growth parameters and nutritional laboratories.
Results: Mean UC and maternal TTR were positively correlated (8.5±2.4 mg/dL and 20.4±7.0 mg/dL, r=0.31, P=0.07). Umbilical cord TTR was neither an index of maturity nor of intrauterine growth. Umbilical cord TTR was higher in females (9.4±2.6 versus 7.6±1.8 mg/dL, P=0.015). Maternal TTR was lower in twin pregnancies (16.8±4.9 versus 22.5±7.3 mg/dL, P=0.007). Although TTR levels gradually increased over time in correlation with post-menstrual and chronological ages (r=0.24, P=0.011 and r=0.40, P<0.001, respectively), there was no correlation to weight gain (r=0.10, P=0.41), nutritional status, protein intake, or laboratories. The only significant correlations were between TTR and glucose and triglycerides levels (r=0.51, P<0.001 for both).
Conclusions: Although TTR levels increased over time, we could not demonstrate significant correlations between TTR and indices of the nutritional status in preterm infants at birth or during the neonatal course.
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare systemic small-vessel disease, with heterogeneous clinical manifestations. While arthralgia and myalgia are common in the disease course, frank myositis is exceedingly rare. Immune-mediated necrotizing myopathy (IMNM) is a subtype of idiopathic inflammatory myopathies (IIMs), characterized by severe myositis. We report herein a case of prominent diffuse myositis with shared features of AAV and IMNM.
Purpose: This case series analyzed the appropriateness of computed tomography (CT) and magnetic resonance imaging (MRI) for visualization of rhinoorbitocerebral mucormycosis (ROCM) patterns associated with type 2 diabetes (T2D) post-recovery from coronavirus disease 2019 (COVID-19).
Methods: The study included 24 patients with invasive ROCM after having recovered from COVID-19. All patients underwent CT examinations and microbiological and histological verification; 5 patients underwent MRI.
Results: The CT and MRI patterns noted in our patients revealed involvement of skull orbits, paranasal sinuses, large arteries, and optic nerves, with intracranial spread and involvement of the cranial base bones. Using brain scan protocol for CT provided better soft-tissue resolution. We found that extending the MRI protocol by T2-sequence with fat suppression or STIR was better for periantral fat and muscle evaluations.
Conclusion: Computed tomography of the paranasal sinuses is the method of choice for suspected fungal infections, particularly mucormycosis. However, MRI is recommended if there is suspicion of orbital, vascular, or intracranial complications, including cavernous sinus extension. The combination of both CT and MRI enables determination of soft tissue invasion and bony destruction, thereby facilitating the choice of an optimal ROCM treatment strategy. Invasive fungal infections are extremely rare in Europe; most of the related data are provided from India and Middle Eastern or African nations. Hence, this study is notable in its use of only diagnosed ROCM cases in Russia.
Introduction: When authorship disputes arise in academic publishing, research institutions may be asked to investigate the circumstances. We evaluated the association between the prevalence of misattributed authorship and trust in the institution involved.
Methods: We measured trust using a newly validated Opinion on the Institution’s Research and Publication Values (OIRPV) scale (range 1–4). Mayer and Davies’ Organizational Trust for Management Instrument served as control. Association between publication misconduct, gender, institution type, policies, and OIRPV-derived Trust Scores were evaluated.
Results: A total of 197 responses were analyzed. Increased reporting of authorship misconduct, such as gift authorship, author displacement within the authors’ order on the byline, and ghost authorship, were associated with low Trust Scores (P<0.001). Respondents from institutions whose administration had made known (declared or published) their policy on authorship in academic publications awarded the highest Trust Scores (median 3.06, interquartile range 2.25 to 3.56). Only 17.8% favored their administration as the best authority to investigate authorship dispute honestly. Of those who did not list the administration as their preferred option for resolving disputes, 58.6% (95/162) provided a Trust Score <2.5, which conveys mistrust in the institution.
Conclusions: Increased reporting of publication misconducts such as gift authorship, author displacement within the order of the authors’ byline, and ghost authorship was associated with lower Trust Scores in the research institutions. Institutions that made their policies known were awarded the highest Trust Scores. Our results question whether the research institutions’ administrations are the appropriate authority for clarifying author disputes in all cases.
It has been the policy of Rambam Maimonides Medical Journal to limit the number of editorials published. However, silence and standing on the sidelines is not an option in light of the atrocities and inhumanity we witnessed on October 7. The savagery of the Hamas massacre was executed indiscriminately upon children, women, older people (some of whom are Holocaust survivors), infants, and even medical professionals caring for the casualties. Currently, there are about 230 women, men, children, and babies being held hostage by Hamas; among them are cancer patients and others with serious disorders, doctors, and other medical professionals. We cannot rest and must address the plight of our hostages who are being held by terrorists motivated by hatred and showing no respect for life, whether that of their enemies, their own people, or even themselves. ...
This case study describes the successful short-term outcome of staged minimally invasive pectus excavatum correction and endoscopic mitral valve repair in a patient with severe mitral valve regurgitation and pectus excavatum.
