The inability to belch: a neglected disease in the era of high-resolution esophageal manometry.

Retrograde upper esophageal sphincter dysfunction (R-UESD) is characterized by the inability to belch. Evidence of using high-resolution manometry (HRM) in diagnosing R-UESD has emerged in recent years. We describe the clinical picture and HRM patterns of two patients with R-UESD. Case 1: A 23-year-old female presented with a two-year complaint of inability to belch. We performed HRM with a belch provocation test for which the patient drank 500 ml of carbonated water. The study revealed increased esophageal pressure, an absence of UES relaxation and secondary peristalsis once the patient mentioned the need to belch. Case 2: A 21-year-old male presented to our medical office with a history of an incapacity to belch during the last three years. We performed HRM with a belch provocation test. During the study, he reported an incapacity to belch and his symptoms coincided with increased esophageal pressure, an absence of UES relaxation, and secondary peristalsis. Retrograde upper esophageal sphincter dysfunction is a rare condition characterized by a lack of UES relaxation during esophageal distension. The incapacity to belch is the hallmark of the disease. We encourage the use of HRM, looking for an increase in esophageal pressure to the level of gastric pressure, failure of UES relaxation with consequently no venting of air across the UES, and secondary peristalsis. In conclusion, diverse R-UESD clinical presentations represent a diagnostic challenge for physicians. This case series highlights the need to actively search for typical HRM findings when encountering patients referring an incapacity to belch.

[Prevalence and genotypic characteristics of anal papillomavirus infection in a cohort of HIV-positive men who have sex with men]. / Prevalencia y características genotípicas de la infección anal por papilomavirus en una cohorte de hombres que tienen sexo con hombres VIH-positivos.

OBJECTIVE: To determine the prevalence and genotypic characteristics of anal papillomaviruses in HIV-positive men who have sex with men (MSM). MATERIALS AND METHODS: This is a prospective cross-sectional observational study of HIV-positive MSM at Almenara General Hospital between September 2017 and December 2018. HPV detection and typing was performed using a polymerase chain reaction technique that evaluated 21 genotypes stratified according to oncogenic risk into six low-risk and fifteen high-risk. RESULTS: we evaluated 214 HIV-positive MSM. The overall prevalence of anal infection by papillomavirus infection was 70% (150/214). 86% (129/150) were caused by high-risk genotypes, 79% (102/129) of them were affected by a two or more-papillomavirus genotype. The most frequent high-risk genotypes were HPV-16, 31% (46/150); HPV-52, 22% (33/150); HPV-33, 21% (31/150); HPV-58, 21% (31/150) and HPV-31, 20% (30/150). In addition, HPV-18 reached 7% (10/150). The most frequent low-risk genotypes were HPV-6, 30% (45/150) and HPV-11, 29% (44/150). CONCLUSIONS: Prevalence of anal papillomavirus infection in HIV-positive MSM is very high in the hospital investigated. Most of these infections occurs with high-risk oncogenic genotypes. Papillomavirus 16 was the most frequent high-risk genotype.

Comparing fine needle biopsy techniques in solid pancreatic lesions: A prospective randomized study.

INTRODUCTION: Endoscopic ultrasound-guided fine-needle biopsies (EUS-FNB) are the best technique for sampling solid pancreatic lesions. However, the most appropriate biopsy technique has not been standardized using Fine Needle Biopsy (FNB) needles. The aim of this work was to identify the best biopsy technique to achieve the best tissue integrity and cause the least blood contamination. MATERIAL AND METHODS: Patients ≥18 years of age with solid pancreatic lesions who underwent EUS-FNB at our institution from January 2020 to May 2021 were consecutively selected. Three passes were performed with each of the threee techniques to obtain tissue: suction with 10 ml of vacuum, capillary, and wet. An independent pathologist evaluated the received tissue integrity and the degree of blood contamination of each sample according to scales. RESULTS: Seventy-five patients were recruited for our study. A superior tissue integrity was observed using the wet-suction technique in lesions located in the body and/or tail of the pancreas, and an average score of 4.40 (p = 0.027) was assigned for this technique. Regarding the contamination of the sample in the whole cohort, the simple-suction technique shown a higher contamination, 1.55 (p < 0.001). There was no statistically significant difference among the techniques when evaluating tissue integrity or contamination in lesions larger or smaller than 3 cm. CONCLUSION: When performing EUS-FNB for solid pancreatic lesions located in the head/uncinated process, the three methods provided similar diagnostic yields. The wet-suction technique had a higher score in tissue integrity when lesions were located in the body and/or tail of the pancreas.

First report of bovine viral diarrhea virus subgenotypes 1d and 1e in southern Chile.

Bovine viral diarrhea virus (BVDV) affects cattle worldwide causing severe productive and economic loss. In this study, we investigated the subgenotypes of BVDV circulating in cattle samples from the Aysén region, an active cattle breeding area located in southern Chile. Partial amplification of the 5' untranslated region (UTR) was performed by polymerase chain reaction (PCR), and twelve samples were analyzed by Sanger sequencing and phylogenetic analysis. Eight samples were identified as belonging to Pestivirus bovis subgenotype 1e, three to 1-b, and one to 1-d. The phylogenetic analyses performed revealed a marked distance between these now-identified strains and those previously reported in the country. These findings support the need to continually expand the analysis of the variability of the viral phylogeny for the currently circulating BVDV strains and to update the vaccines recommended for this livestock area and surrounding areas.

Acute respiratory failure secondary to megaesophagus: think beyond COVID-19.

A 61-year-old man with a history of progressive dysphagia in the last three years and current COVID-19 diagnosis, presented to our emergency department with sudden dyspnea and loss of consciousness. Initial examination revealed a respiratory rate of 30 bpm with an oxygen saturation of 94% with room air. A venous blood gas exam showed acute hypercapnic respiratory failure; therefore, he was intubated and admitted to the medical intensive care unit.

Glanzmann thrombasthenia: an uncommon cause of acute upper gastrointestinal bleeding.

The major function of platelets is to contribute to hemostasis. If an impairment in their production and/or function occurs, abnormal bleeding can develop. An 18-year-old male presented to our hospital after four episodes of hematemesis. His medical history was relevant for Glanzmann thrombasthenia diagnosed during early childhood. On initial examination, he appeared pale and with normal blood pressure. His complete blood count included a hemoglobin concentration of 11.0 g/dL, additional laboratory tests were within the normal ranges. The initial approach consisted of a high dose of proton pump inhibitors. Hours later, esophagogastroduodenoscopy revealed diffuse oozing bleeding from gastric mucosa with no other visible lesions such as peptic ulcers or varices.

Risk factors for mortality in patients with acute bacterial cholangitis - Type-2 diabetes is a significant clinical predictor.

Acute cholangitis is a life-threatening infection, and several features are associated with a worse prognosis if prompt treatment is not started. Eighty-six subjects with acute cholangitis were included in a retrospective analysis. Patients had a median age of 53 years (range: 34.5 to 70 years) and 55 (64.0 %) were female. Of the entire cohort, 16 patients had a history of diabetes mellitus. Regarding acute cholangitis severity, 17 (19.8 %) cases were classified as severe, 41 (47.7 %) as moderate, and 28 (32.6 %) as mild.

Angiogenesis, hereditary hemorrhagic telangiectasia and COVID-19.

Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal-dominant disease characterized by pathologic angiogenesis that provokes vascular overgrowth. The evidence about the influence of Acute Respiratory Syndrome Coronavirus-2 (SARS-CoV-2) in patients with rare diseases is scarce. We aimed to know the prevalence of coronavirus disease 2019 (COVID-19) in HHT patients. The HHT pathogenic angiogenesis and endothelial injury in COVID-19 are discussed using data from RiHHTa (Computerized Registry of Hereditary Hemorrhagic Telangiectasia) registry. RiHHTa is an open, multicenter, prospective, observational registry including adult patients with HHT. A 27-item survey that captured clinical data of admitted HHT patients for COVID-19 was distributed to all RiHHTa investigators from June 8th to June 24th 2020. Only one out of 1177 HHT patients was admitted for COVID-19 pneumonia. She is a 74 years-old woman with a pathogenic variant in ACVRL1 gene. Her clinical course did not involve mechanical ventilation or worsening epistaxis, and she was successfully discharged after two weeks. The endothelial damage and the consequent angiogenic process in COVID-19 patients deserve further investigation.

Performance of serum procalcitonin as a biochemical predictor of death in hematology patients with febrile neutropenia.

INTRODUCTION: Historically, the measurement of serum procalcitonin (PCT) levels in patients with leukopenia has been rejected without sufficient prospective evidence to justify this argument. On the other hand, the accumulated use of broad spectrum antibiotics in these patients and their consequences make the use of PCT attractive in an effort to reduce its use. PATIENTS AND METHODS: We conducted a prospective study between 2016 and 2018, recruiting newly diagnosed FN patients, evaluating them with PCT levels during the first 24 h. After this we evaluate them with overall survival throughout the follow-up. RESULTS: A total of 81 episodes of FN in 72 patients were included. We report a mortality of 27.2% in our cohort. The mean serum PCT in these patients was 4.01 ng/mL compared to 0.42 ng/mL in the survivors group (p < 0.01). Using ROC curves, we determined a cut-off point to predict septic shock/death at 0.46 ng/mL. Patients with a procalcitonin >0.46 ng/mL had an increased risk of death, with a HR of 4.43, (p = 0.048). CONCLUSION: In conclusion, in our trial a single PCT on admission at a cut-off value of 0.46 ng/mL was able to predict the occurrence of septic shock and death in FN patients.

Filgrastim-induced rhabdomyolysis in a healthy stem cells donor.

INTRODUCTION: Mobilization of peripheral blood progenitor cells with the use of granulocyte-colony stimulating factors is a mainstay in every protocol for allogenic stem cell transplants. Despite being considered safe, there are multiple adverse effects for this procedure some of which can be severe and bring serious complications to otherwise healthy donors. CASE REPORT: An otherwise healthy 17-year-old patient who underwent progenitor cell mobilization with filgrastim and developed rhabdomyolysis and acute kidney injury.Management and outcome: The urine analysis and kidney ultrasound failed to reveal abnormalities in the kidneys or collector system. We began reanimation with crystalloid solutions for 5 days with normalization of liver and muscle enzymes as well as a complete resolution of the acute kidney injury. DISCUSSION: We present the case of a potentially serious adverse drug reaction during mobilization of peripheral blood progenitor cells with filgrastim. Physicians need to be aware of every possible complication associated with the use of these agents in order to establish a good prognosis via an accurate diagnosis and a timely treatment.

Treatment outcomes and chronicity predictors for primary immune thrombocytopenia: 10-year data from an academic center.

Primary immune thrombocytopenia (ITP) is an intriguing autoimmune disease characterized by autoantibodies against platelets and megakaryocytes. Clinical outcomes, response to treatment, and chronicity predictors were investigated. Patients with newly diagnosed primary ITP treated at a hematology referral center from 2008 to 2018 with complete medical and recent medication history were stratified by age as children < 16 years and adults > 16 years. Responses to treatment including steroids, splenectomy, rituximab, and eltrombopag were classified as response (R) and complete (CR). Factors for developing chronic ITP were determined by multiple regression with uni- and multivariate analysis. p < 0.05 was considered significant. A total of 175 patients were included, 52 children and 123 adults; women predominated with 57.7%. Response to first-line treatment in the whole cohort was 86.18%, CR 43.42% and R 42.76%. The initial response to steroids alone was 83.9% (n = 52/62), rituximab plus high-dose dexamethasone (HDD) 87.2% (n = 34/39), eltrombopag plus HDD 90.9% (n = 10/11), and children receiving IVIG alone 100% (n = 8/8); 9 children were under clinical observation and achieved spontaneous response; loss of response was documented in 15.21% children and 28.3% adults with a median time of 15.95 and 4.07 months respectively; 37.39% of adults and 30.76% of children progressed to a chronic course. Platelets ≥ 20 × 109/L and age ≥ 6 years were risk factors for chronic ITP in the univariate analysis in the adult and children groups, respectively. Clinical course and treatment outcomes for ITP are considerably heterogeneous. Higher platelet counts at diagnosis in adults and age ≥ 6 years in children were associated with an increased risk of chronicity.

Diagnoses, Outcomes, and Chronicity Predictors of Patients with Secondary Immune Thrombocytopenia: Ten-Year Data from a Hematology Referral Center.

BACKGROUND: Secondary immune thrombocytopenia (ITP) is a heterogeneous and unpredictable disease associated with various underlying conditions. OBJECTIVE: The objective of the study was to investigate clinical evolution and chronicity predictors in secondary ITP. METHODS: Patients treated at an academic medical center during 2008-2019 were stratified by age as children <16 years and adults <16 years. Responses to steroids, intravenous immunoglobulin G (IVIG), rituximab, and eltrombopag were classified as response (R) and complete response (CR). Risk factors for chronic ITP were determined by multiple regression with uni- and multi-variate analysis. RESULTS: Eighty-three patients were included, 37 children and 46 adults. The most frequent associated conditions were infections 53%, systemic lupus erythematosus (SLE) 24%, thyroid disease 9.6%, and Evans syndrome 3.6%. Response to first-line treatment in the whole cohort was 94%; CR 45.7%; and R 50.6%. Initial response to steroids alone was 91.3% (n = 21/23), rituximab plus high-dose dexamethasone (HDD) 93.3% (n = 14/15); children receiving IVIG alone 100% (n=12/12); and eltrombopag in adults 100% (n = 3/3). Relapse was documented in 19.4% of children and 34% of adults, at a median time of 15 and 2 months, respectively; 30.4% of adults (15.2% from the miscellaneous group, 10.9% SLE-associated, and 4.3% infection-associated) and 18.9% of children followed a chronic course; age ≥10 years and platelets ≥20 × 109/L were risk factors for chronic ITP in children. CONCLUSION: Evolution was heterogeneous: a better and more sustained response was documented in the infections group compared to SLE or the miscellaneous group.

Toluene toxicity presenting with hypokalemia, profound weakness and U waves in the electrocardiogram.

We present the case of a 25-year-old man with progressive limb weakness. His electrocardiogram showed prominent U waves which made us consider hypokalemia. The final diagnosis was toluene intoxication with severe hypokalemia and metabolic acidosis. Intravenous potassium administration and hydration effectively corrected the electrolyte and acid-base alterations; weakness resolved and the patient was discharged. The approach to a patient with acute weakness can be challenging. This case reminds us that the electrocardiogram can be a valuable tool in the evaluation and differential diagnosis of patients presenting to the emergency department with these conditions.

Round opacity as a presentation of pneumocystis jirovecii pneumonia in an HIV-infected patient.

We present the case of a human immunodeficiency virus (HIV)-infected patient who arrived at our emergency department with fever, headache and exertional dyspnea. Throughout their stay, a chest x-ray was taken and a rounded opacity in his left lung was observed. CT images showed same abnormality and also ground glass opacities were seen. Symptoms and images strongly suggested a pulmonary infection due to pneumocystis jirovecii, however a presence of a round lesion should always lead to neoplasia being suspected. We empirically started treatment based on trimethoprim and sulfamethoxazole. Once available, flexible bronchoscopy and bronchoalveolar lavage was performed and stained preparations from his respiratory specimens confirmed the diagnosis of pulmonary pneumocystis infection. Finally, after 4 days of antibiotic therapy, an important clinical improvement was documented; a new chest x-ray was performed and the previous rounded opacity was absent. This finding strongly suggested a case of round pneumonia.

A tick beat in the electrocardiogram: Persistent third degree block as only manifestation of Lyme disease.

Lyme disease is the most common tick-borne illness in North America. A 23-year-old female presented to our emergency department with a chief complaint of sudden dyspnea and chest pain. An electrocardiogram revealed a third degree heart block. She was a resident of the Northeast region of Mexico and referred a recent travel to an endemic area for Borrelia burgdorferi in the center of Mexico in the past weeks. Lyme carditis was diagnosed after enzyme linked immunosorbent assay for IgM antibodies against B. burgdorferi was reported positive and corroborated by a confirmatory immunoblot analysis. Persistent AV block was the only manifestation in our patient, a presentation scarcely reported in literature.

A case of aseptic meningitis in a cetuximab-experienced patient with metastatic colon cancer.

Cetuximab is a monoclonal antibody against epidermal growth factor receptor and is used in the treatment of head and neck cancer, non-small cell lung cancer, and colorectal cancer. This case report describes a rare (<1% incidence) side effect of cetuximab administration: aseptic meningitis. We report a case which is, to our knowledge, the only case at the time of submission of this manuscript of aseptic meningitis in a patient being treated for metastatic colon cancer who was not cetuximab-naïve. This case report may help inform clinicians about the identification and outcome of this adverse event.

TNF-α increases in the CSF of children with acute lymphoblastic leukemia before CNS relapse.

There is scarce information regarding the concentration of cytokines in cerebrospinal fluid (CSF) of children with acute lymphoblastic leukemia (ALL) and their clinical association with CNS status. A prospective analysis of 40 patients <18years with newly diagnosed ALL was performed. Human cytokine magnetic bead panel assay values of IL-2, IL-4, IL-6, IL-8, IL-10, MCP-1, TNF-α in CSF at diagnosis, end of induction to remission, and 6months after diagnosis were determined. IL-6 and MCP-1 values showed a significant increment at the end of induction. From the whole group 4 (10.0%), patients relapsed to the CNS at a median of 11.48months. A significantly higher value of TNF-α at third determination in these CNS-relapsed patients was documented, 7.48 vs. 2.86pg/mL in 36 children without relapse (p=0.024). TNF-α concentration increased at a median 5.48months before CNS relapse. By receiver-operating characteristic curve (ROC) analysis, the best cut-off point of TNF-α concentration that better predicted CNS relapse was ≥1.79pg/mL. In conclusion an increase in TNF-α concentration on CSF preceded CNS relapse in children with ALL. An increase in MCP-1 and IL-6 was not associated to CNS relapse and appears to result from an inflammatory response after IT injection of chemotherapy.

Hospitalization rate and costs in acute lymphoblastic leukemia of childhood in a low-income group: Financial impact in Northeast Mexico.

BACKGROUND: Acute lymphoblastic leukemia (ALL) is one of the main and most expensive and prolonged causes of hospitalization for childhood cancer. We describe the hospitalization rate and its costs for an open population with ALL in a low-middle income country. PROCEDURE: We retrospectively analyzed 449 hospital admissions for 101 pediatric patients with ALL over 8 years. Clinical files and electronic databases were scrutinized to document causes, duration, readmission rate, costs, and outcome of each admission. Hospitalizations were divided into two categories: general pediatric ward and pediatric intensive care unit (PICU). Hospitalization rates and its costs per patient were estimated considering person-time at risk. RESULTS: Patients had an admission rate of 2.09 hospitalizations per patient-year and median length of stay per admission was 5 days. Most admissions occurred during the first 2 years from diagnosis. Mean cost per day was 239 US dollars (USD) and mean cost per stay was 2,246 USD versus 1,016 and 19,004 USD (P = 0.001) in the PICU, respectively. Total hospitalization cost per patient per year (PPPY) was 5,991 USD for high-risk patients and 3,038 USD for standard-risk patients. Patients between ages 1 and 9 years had a PPPY cost of $4,057; while for children younger than 1 year or older than 9 years, it was 7,463 USD. The popular medical insurance program covered 70% of hospitalizations and 63% of its total cost; patients contributed 2%, with the hospital absorbing 35%. CONCLUSIONS: Hospitalizations for children with ALL were less expensive than in high-income countries but had a significant cost to low-income families and to the healthcare system.

Real-world outcomes of treatment for acute lymphoblastic leukemia during adolescence in a financially restricted environment: Results at a single center in Latin America.

OBJECTIVE: There is a paucity of the studies of adolescents with acute lymphoblastic leukemia (ALL). This is more noticeable in low- and middle-income countries. The international 5-year event-free survival (EFS) and overall survival (OS) for this age group is around 80%, with pediatric-inspired protocols offering better results. METHODS: A retrospective analysis of adolescents aged 16-20 diagnosed with ALL during the period 2004-2015 treated with a high-risk pediatric protocol at an academic center from a middle-income country was performed. Five-year OS and EFS were estimated by the Kaplan-Meier analysis. Hazard ratios of relapse and death were estimated by the Cox regression model. RESULTS: Five-year EFS and OS for 57 adolescents were 23.3% and 48.9%, respectively. From the 41 patients who achieved complete remission, 24 (58.5%) relapsed. Bone marrow and central nervous system were the most frequent sites of relapse. Hazard ratio of treatment failure and death for patients with organomegaly at diagnosis was 2.026 and 2.970, respectively. Treatment-related toxicity developed in 31 (54.4%) patients and febrile neutropenia was the most frequent in 14 (24.6%) cases. Twelve patients (21.1%) had poor adherence to treatment. CONCLUSIONS: High relapse rate and low 5-year EFS compared with international standards, was documented. Use of intensified pediatric regimens, adherence to proven effective medications, improved supportive care, and prevention of abandonment are necessary to improve survival rates in these patients.

Plateletpheresis efficiency and mathematical correction of software-derived platelet yield prediction: A linear regression and ROC modeling approach.

BACKGROUND: Advances in automated cell separators have improved the efficiency of plateletpheresis and the possibility of obtaining double products (DP). We assessed cell processor accuracy of predicted platelet (PLT) yields with the goal of a better prediction of DP collections. STUDY DESIGN AND METHODS: This retrospective proof-of-concept study included 302 plateletpheresis procedures performed on a Trima Accel v6.0 at the apheresis unit of a hematology department. Donor variables, software predicted yield and actual PLT yield were statistically evaluated. Software prediction was optimized by linear regression analysis and its optimal cut-off to obtain a DP assessed by receiver operating characteristic curve (ROC) modeling. RESULTS: Three hundred and two plateletpheresis procedures were performed; in 271 (89.7%) occasions, donors were men and in 31 (10.3%) women. Pre-donation PLT count had the best direct correlation with actual PLT yield (r = 0.486. P < .001). Means of software machine-derived values differed significantly from actual PLT yield, 4.72 × 1011 vs.6.12 × 1011 , respectively, (P < .001). The following equation was developed to adjust these values: actual PLT yield= 0.221 + (1.254 × theoretical platelet yield). ROC curve model showed an optimal apheresis device software prediction cut-off of 4.65 × 1011 to obtain a DP, with a sensitivity of 82.2%, specificity of 93.3%, and an area under the curve (AUC) of 0.909. CONCLUSION: Trima Accel v6.0 software consistently underestimated PLT yields. Simple correction derived from linear regression analysis accurately corrected this underestimation and ROC analysis identified a precise cut-off to reliably predict a DP.