Effects of the COVID-19 Pandemic on Patients With NMO Spectrum Disorders and MOG-Antibody-Associated Diseases: COPANMO(G)-Study.

Background And Objectives: To evaluate the effects of the coronavirus disease 2019 (COVID-19) pandemic on the life of patients with neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated diseases (MOGAD).

Methods: This multicenter, cross-sectional study included data of 187 patients recruited from 19 different German and Austrian Neuromyelitis Optica Study Group (NEMOS) centers between July 2021 and March 2022. The effects of the pandemic on immunotherapeutic treatment and access to care, the possible severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, and the potential effect of vaccination against SARS-CoV-2 on disease incidence and relapse risk were assessed using a patient questionnaire.

Allogeneic hematopoietic cell transplantation in patients with myelodysplastic syndrome using treosulfan based compared to other reduced-intensity or myeloablative conditioning regimens. A report of the chronic malignancies working party of the EBMT.

Allogeneic haematopoietic-cell transplantation (allo-HCT) is a potentially curative therapy for high-risk myelodysplastic syndrome (MDS). Reduced-intensity conditioning (RIC) is usually associated with lower non-relapse mortality (NRM), higher relapse rate and similar overall-survival (OS) as myeloablative-conditioning (MAC). Fludarabine/treosulfan (FT) is a reduced-toxicity regimen with intense anti-leukaemia activity and a favourable toxicity profile.

Outcomes of pneumatic dilation in achalasia: Extended follow-up of more than 25 years with a focus on manometric subtypes.

Background And Aim: Pneumatic dilation (PD) is the most popular nonsurgical treatment for achalasia. This study investigated predicting factors, including manometric subtypes for symptom recurrence in the long term, in patients with achalasia treated with a single PD.

Methods: Between 1983 and 2013, a total of 107 patients were treated initially with a single PD and included in this longitudinal cohort study.

Frequency and epitope specificity of anti-factor VIII C1 domain antibodies in acquired and congenital hemophilia A.

Several studies showed that neutralizing anti-factor VIII (anti-fVIII) antibodies (inhibitors) in patients with acquired hemophilia A (AHA) and congenital hemophilia A (HA) are primarily directed to the A2 and C2 domains. In this study, the frequency and epitope specificity of anti-C1 antibodies were analyzed in acquired and congenital hemophilia inhibitor patients (n = 178). The domain specificity of antibodies was studied by homolog-scanning mutagenesis (HSM) with single human domain human/porcine fVIII proteins and antibody binding to human A2, C1, and C2 domains presented as human serum albumin (HSA) fusion proteins.

Achalasia-an unnecessary long way to diagnosis.

Although achalasia presents with typical symptoms such as dysphagia, regurgitation, weight loss, and atypical chest pain, the time until first diagnosis often takes years and is frustrating for patients and nevertheless associated with high costs for the healthcare system. A total of 563 patients were interviewed with confirmed diagnosis of achalasia regarding their symptoms leading to diagnosis along with past clinical examinations and treatments. Included were patients who had undergone their medical investigations in Germany.

Sequential Intensified Conditioning Regimen Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Intermediate- or High-Risk Acute Myeloid Leukemia in Complete Remission: A Study from the Acute Leukemia Working Party of the European Group for Blood and Marrow Transplantation.

Post-transplant relapse is the leading cause of treatment failure in acute myeloid leukemia (AML) patients after reduced-intensity conditioning allogeneic hematopoietic stem cell transplantation (allo-HSCT). To improve their outcome, we evaluated the outcome of a sequential intermediate-intensity conditioning regimen combining fludarabine, cytosine arabinoside, amsacrine, cyclophosphamide, and either total body irradiation or busulfan (FLAMSA) in patients with intermediate or high-risk AML in first or second complete remission (CR). A total of 265 patients (median age, 55 years; range, 19 to 76) with AML who underwent allo-HSCT using a FLAMSA regimen were included.

Comprehensive epidemiological and genotype-phenotype analyses in a large European sample with idiopathic achalasia.

Background And Aim: Although an eight-residue insertion in HLA-DQβ1 has been recently identified as a genetic risk factor for idiopathic achalasia, other risk factors are still unknown. In the present study, we carried out an epidemiological survey and a genotype-phenotype (G×P) analysis to gain further insights into the etiology of achalasia.

Methods: We obtained medical data from 696 achalasia patients and 410 controls, as well as their first-degree relatives (2543 of patients and 1497 of controls).

Impact of high-resolution manometry on achalasia diagnosis and treatment.

Achalasia is a primary neurodegenerative disorder of the esophagus characterized by loss of function of the lower esophageal sphincter (LES) and of esophageal peristalsis, which causes symptoms such as dysphagia, regurgitation, weight loss, and chest pain. Esophageal manometry is the gold standard for the diagnosis of achalasia. The typical manometric features are incomplete relaxation of a frequently hypertensive LES and lack of peristalsis in the tubular esophagus.

Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from the GTH-AH 01/2010 study.

Acquired hemophilia A (AHA) is caused by autoantibodies against factor VIII (FVIII). Immunosuppressive treatment (IST) results in remission of disease in 60% to 80% of patients over a period of days to months. IST is associated with frequent adverse events, including infections as a leading cause of death.

Common variants in the HLA-DQ region confer susceptibility to idiopathic achalasia.

Idiopathic achalasia is characterized by a failure of the lower esophageal sphincter to relax due to a loss of neurons in the myenteric plexus. This ultimately leads to massive dilatation and an irreversibly impaired megaesophagus. We performed a genetic association study in 1,068 achalasia cases and 4,242 controls and fine-mapped a strong MHC association signal by imputing classical HLA haplotypes and amino acid polymorphisms.

Prospective evaluation of the routine use of a nasopharyngeal airway (Wendl Tube) during endoscopic propofol-based sedation.

Background/aims: Insertion of a nasopharyngeal airway (NPA) during endoscopic sedation is only recommended in the event of respiratory problems. We evaluated the safety and efficacy of routine insertion of an NPA during sedation in gastrointestinal (GI) endoscopy.

Methods: Between July 2009 and April 2012, patients with colonoscopy or expected longer-lasting or therapeutic upper GI endoscopy were pseudo-randomized in a weekly alternating fashion to perform sedation (midazolam in combination with propofol) with or without NPA insertion.

Murine genetic deficiency of neuronal nitric oxide synthase (nNOS(-/-) ) and interstitial cells of Cajal (W/W(v) ): Implications for achalasia?

Background And Aim: Nitric oxide (NO) is an important inhibitory mediator of esophageal function, and its lack leads to typical features of achalasia. In contrast, the role of intramuscular interstitial cells of Cajal (ICC-IM) and vasoactive intestinal peptide (VIP) in lower esophageal sphincter (LES) function is still controversial. Therefore, we examined the function and morphology of the LES in vivo in NO-deficient (nNOS(-/-) ), ICC-IM-deficient (W/W(v) )-, and wild-type (WT) mice.

Treating neuromyelitis optica with the interleukin-6 receptor antagonist tocilizumab.

We present a 32-year-old female patient with fulminant neuromyelitis optica. After the initial treatment with the monoclonal antibody rituximab failed, therapy with the anti-IL-6 receptor antagonist tocilizumab was initiated. The patient experienced a clinically relevant improvement from severe tetraparesis to low-grade paresis, which is still maintained.

Endoscopic approach to achalasia.

Achalasia is a primary esophageal motor disorder. The etiology is still unknown and therefore all treatment options are strictly palliative with the intention to weaken the lower esophageal sphincter (LES). Current established endoscopic therapeutic options include pneumatic dilation (PD) or botulinum toxin injection.

Endoscopic findings in patients with Schatzki rings: evidence for an association with eosinophilic esophagitis.

Aim: To investigate endoscopic findings in patients with Schatzki rings (SRs) with a focus on evidence for eosinophilic esophagitis (EoE).

Methods: We consecutively approached all adult patients scheduled for elective outpatient upper endoscopy for a variety of indications at the German Diagnostic Clinic, Wiesbaden, Germany between July 2007 and July 2010. All patients with endoscopically diagnosed SRs, defined as thin, symmetrical, mucosal structures located at the esophagogastric junction, were prospectively registered.

A prospective, randomised study on the use of well-fitting masks for prevention of invasive aspergillosis in high-risk patients.

The problem of inhalation of Aspergillus spores outside rooms with high-efficiency particulate air (HEPA) filtration has not been resolved as yet. Well-fitting masks are used in industrial and health care settings to protect from inhaling particles of 0.3-0.

Long-term results of conventional myotomy in patients with achalasia: a prospective 20-year analysis.

Myotomy has proved to be an efficient primary therapy in patients with achalasia, especially in younger patients (<40 years of age). The results of laparoscopic myotomy cannot be finally assessed, on account of the shorter postoperative follow-up. Thus, there are considerable data regarding intermediate-term outcomes after laparoscopic myotomy.

Effects of pneumatic dilation and myotomy on esophageal function and morphology in patients with achalasia.

Only two treatment modalities-pneumatic dilation and Heller myotomy-promise long-term relief from dysphagia and regurgitation in patients with achalasia. The objective of this study was to determine whether both options differ in their effects on esophageal function, morphology, and improvement in symptoms. Eighty-nine patients diagnosed with achalasia between January 1980 and December 2002 at a single center were enrolled in this study.

Involuntary contractions of the striated anal sphincters as a cause of constipation: report of a case.

Patient History: We present a case of anismus in a 36-year-old patient. He complained of therapy refractory constipation that had been present for 15 years, with delayed micturition and voiding by stages.

Methods And Results: During digital examination of the anal canal, we found spontaneous contractions of the sphincters at rest.