Ineffective esophageal motility: Characterization and outcomes across pediatric neurogastroenterology and motility centers in the United States.

OBJECTIVES: Ineffective esophageal motility (IEM) on high-resolution manometry (HRM) is not consistently associated with specific clinical syndromes or outcomes. We evaluated the prevalence, clinical features, management, and outcomes of pediatric IEM patients across the United States. METHODS: Clinical and manometric characteristics of children undergoing esophageal HRM during 2021-2022 were collected from 12 pediatric motility centers. Clinical presentation, test results, management strategies, and outcomes were compared between children with IEM and normal HRM. RESULTS: Of 236 children (median age 15 years, 63.6% female, 79.2% Caucasian), 62 (23.6%) patients had IEM, and 174 (73.7%) patients had normal HRM, with similar demographics, medical history, clinical presentation, and median symptom duration. Reflux monitoring was performed more often for IEM patients (25.8% vs. 8.6%, p = 0.002), but other adjunctive testing was similar. Among 101 patients with follow-up, symptomatic cohorts declined in both groups in relation to the initial presentation (p > 0.107 for each comparison) with management targeting symptoms, particularly acid suppression. Though prokinetics were used more often and behavioral therapy less often in IEM (p ≤ 0.015 for each comparison), symptom outcomes were similar between IEM and normal HRM. Despite a higher proportion with residual dysphagia on follow-up in IEM (64.0% vs. 39.1%, p = 0.043), an alternate mechanism for dysphagia was identified more often in IEM (68.8%) compared to normal HRM (27.8%, p = 0.017). CONCLUSIONS: IEM is a descriptive manometric pattern rather than a clinical diagnosis requiring specific intervention in children. Management based on clinical presentation provides consistent symptom outcomes.

Current practice in the care of children with functional constipation: What is the hold up?

OBJECTIVES: For children with intractable functional constipation (FC), there are no evidence-based guidelines for subsequent evaluation and treatment. Our objective was to assess the practice patterns of a large, international cohort of pediatric gastroenterologists. METHODS: We administered a survey to physicians who attended the 2nd World Congress of Pediatric Neurogastroenterology and Motility held in Columbus, Ohio (USA) in September 2023. The survey included 29 questions on diagnostic testing, nonpharmacological and pharmacological treatment, and surgical options for children with intractable FC. RESULTS: Ninety physicians from 18 countries completed the survey. For children with intractable FC, anorectal manometry was the most commonly used diagnostic test. North American responders were more likely than Europeans to use stimulant laxatives (97% vs. 77%, p = 0.032), prosecretory medications (69% vs. 8%, p < 0.001), and antegrade continence enemas (ACE; 83% vs. 46%, p = 0.009) for management. Europeans were more likely than North Americans to require colonic transit testing before surgery (85% vs. 30%, p < 0.001). We found major differences in management practices between Americans and the rest of the world, including use of prosecretory drugs (73% vs. 7%, p < 0.001), anal botulinum toxin injections (81% vs. 58%, p = 0.018), ACE (81% vs. 58% p = 0.018), diverting ileostomies (56% vs. 26%, p = 0.006), and colonic resections (42% vs. 16%, p = 0.012). No differences were found when respondents were compared by years of experience. CONCLUSIONS: Practice patterns in the evaluation and treatment of children with intractable FC differ widely among pediatric gastroenterologists from around the world. A clinical guideline regarding diagnostic testing and surgical decision-making is needed.

Prolonged Impact of COVID-19 on Job Prospects and Training for Pediatric Gastroenterology Fellows in North America.

As the COVID-19 pandemic persisted into the 2020 to 2021 academic year, there was a continued effect on graduate medical education trainees and graduating trainee job attainment. Our survey aims to investigate how the pandemic has continued to affect job search and attainment for pediatric gastroenterology fellows as well as to re-evaluate the pandemic's impact on pediatric gastroenterology fellow educational experiences. Methods: An anonymous survey was distributed to all North American pediatric gastroenterology fellows from May to June 2021. Survey questions included topics related to job search and fellowship training and were tailored to respondent year of training. Results: Of 453 pediatric gastroenterology fellows in the 2020 to 2021 academic year, 158 fellows (35%) responded to the survey. Of graduating fellow respondents with job contracts, 74% reported willingness to make compromises in their job search, 76% reported accepting academic positions that were primary clinical, and 42% estimated staying at their accepted job for less than 5 years. When asked about the impact of COVID-19 on various aspects of fellowship education, a negative impact was reported in the following areas: 76% in research, 94% in clinical experience, 73% in procedural skills, and 84% in didactics. Conclusion: The COVID-19 pandemic continues to make a significant impact on pediatric gastroenterology fellowship education and the job attainment process. Regarding accepted job positions, we found substantial willingness to compromise, a shorter duration to stay at the job than expected, and minimal research focus. This raises concern regarding job preparedness and satisfaction as fellows complete their medical training.

Delayed Diagnosis in a Male With Congenital Chloride Losing Diarrhea.

Congenital chloride diarrhea is a secretory type of diarrhea, inherited in as autosomal recessive. Our case involves a 12-month-old male who initially presented in infancy and was treated with an exclusive elemental formula diet. At 12 months of age, he presented with significant hypokalemia, hypochloremia, and metabolic alkalosis. The diagnosis was established with stool electrolytes demonstrating a stool chloride of 145 mmol/L. He initially was treated with sodium and potassium supplementation and a proton pump inhibitor. Genetic testing revealed a large 4.3-kb deletion encompassing exons 15 to 17 of the SLC26A3 gene and a sequence variant of the SLC26A3 gene, c.610T>G; pTyr204Asp initially reported as a variant of unknown significance. His parents had genetic testing confirming that the deletion and sequence variant were found in opposite alleles in the patient, meaning the sequence variant is a pathogenic variant. He is maintaining stable serum electrolytes and gaining appropriate weight on oral electrolyte supplementation.

Signo de «spiked helmet» (casco prusiano) en electrocardiograma de una paciente con cirugía abdominal

RESUMEN En la evaluación de pacientes con eventos abdominales o torácicos agudos se ha encontrado una elevación del segmento ST similar a la que se presenta en el infarto agudo de miocardio. Este nuevo patrón se caracteriza por presentar elevación de la línea isoeléctrica precedente a los QRS, seguido de una onda R picuda y supradesnivel convexo del segmento ST que se denomina, debido a su semejanza gráfica, signo de «spiked helmet» (casco prusiano), lo cual -traducido al español- significa casco con púas. Se presenta el caso de una paciente de 56 años de edad, con antecedentes de hipertensión arterial, a quien se le realizó una cirugía abdominal urgente y presentó, en el electrocardiograma, un supradesnivel del segmento ST > 1 mm en DII, DIII y aVF, con ondas R picudas, que desaparecieron a las 6 horas, sin que se demostrara enfermedad cardíaca asociada.

ABSTRACT An ST-segment elevation similar to that in acute myocardial infarction has been found during the assessment of patients with acute abdominal or thoracic events. In this relatively new electrocardiographic pattern, the elevation of the isoelectric line precedes the QRS, followed by a sharp R wave and convex ST-segment elevation known as the "spiked helmet" sign because of its resemblance to the Prussian military helmet, the Pickelhaube. We present the case of a 56-year-old female patient with a history of high blood pressure who underwent emergency abdominal surgery. The electrocardiogram showed ST-segment elevation > 1 mm in DII, DIII and aVF, with sharp R waves that disappeared at 6 hours. No associated cardiac disease was identified.

Case Report: Acute Abdominal Pain as Presentation of Pneumonia and Acute Pancreatitis in a Pediatric Patient With COVID-19.

Abdominal pain, nausea, and vomiting are known gastrointestinal symptoms of symptomatic SARS-CoV-2 infection (COVID-19 disease) in pediatric patients.1 There is little literature regarding pancreatitis in COVID-19. We describe a 16-year-old male diagnosed with acute pancreatitis in the setting of a SARS-COV-2 infection and associated fluid balance considerations.

Síndrome de Eisenmenger en una paciente de la tercera edad / Eisenmenger syndrome in an elderly patient

Se describe el caso clínico de una paciente de 62 años de edad con cardiopatía congénita cianótica, quien fue ingresada en el Servicio de Cardiología del Hospital Provincial Docente Clinicoquirúrgico "Saturnino Lora Torres" de Santiago de Cuba por presentar disnea. Al examen físico se constató cianosis, arritmia completa y soplo sistólico en mesocardio, con signos de insuficiencia cardíaca refractaria del lado derecho. Los exámenes complementarios efectuados confirmaron el diagnóstico de síndrome de Eisenmenger atribuible a comunicación interventricular. A pesar de la medicación que recibía evolucionó desfavorablemente hasta que falleció. Es poco frecuente que pacientes con esta complicación sobrevivan hasta la tercera edad.

A case report of a 62 year-old patient with cyanotic congenital heart disease, who was admitted to the Cardiology Department of "Saturnino Lora" Provincial Teaching Clinical Surgical Hospital of Santiago de Cuba to present with shortness of breath was described. On physical examination cyanosis, complete arrhythmia and systolic murmur in mesocardium with signs of refractory heart failure of the right side were found. Complementary tests confirmed the diagnosis of Eisenmenger syndrome attributable to ventricular septal defect. Despite receiving medication she made bad progress and died. It is rare for patients with this complication survive into old age.

Síndrome de Eisenmenger en una paciente de la tercera edad / Eisenmenger syndrome in an elderly patient

Se describe el caso clínico de una paciente de 62 años de edad con cardiopatía congénita cianótica, quien fue ingresada en el Servicio de Cardiología del Hospital Provincial Docente Clinicoquirúrgico Saturnino Lora Torres de Santiago de Cuba por presentar disnea. Al examen físico se constató cianosis, arritmia completa y soplo sistólico en mesocardio, con signos de insuficiencia cardíaca refractaria del lado derecho. Los exámenes complementarios efectuados confirmaron el diagnóstico de síndrome de Eisenmenger atribuible a comunicación interventricular. A pesar de la medicación que recibía evolucionó desfavorablemente hasta que falleció. Es poco frecuente que pacientes con esta complicación sobrevivan hasta la tercera edad(AU)

A case report of a 62 year-old patient with cyanotic congenital heart disease, who was admitted to the Cardiology Department of Saturnino Lora Provincial Teaching Clinical Surgical Hospital of Santiago de Cuba to present with shortness of breath was described. On physical examination cyanosis, complete arrhythmia and systolic murmur in mesocardium with signs of refractory heart failure of the right side were found. Complementary tests confirmed the diagnosis of Eisenmenger syndrome attributable to ventricular septal defect. Despite receiving medication she made bad progress and died. It is rare for patients with this complication survive into old age(AU)

Angioplastia coronaria sobre arteria descendente anterior con origen anómalo y arteria coronaria derecha / Coronary angioplasty on anterior descending artery with anomalous origin and right coronary artery

Se presenta el caso clínico de un paciente exfumador (sin otro antecedente), que después de una situación de estrés comenzó a experimentar, por más de 20 minutos, dolor precordial intenso, opresivo y retroesternal, irradiado hacia el hombro izquierdo, acompañado de diaforesis. Sobre la base de los resultados de la prueba de esfuerzo y ecografía, se realizó cateterismo cardíaco, lo cual permitió diagnosticar una anomalía en la arteria coronaria izquierda, dada por el nacimiento de la descendente anterior de la coronaria derecha. La obstrucción de ambas arterias fue eliminada exitosamente mediante angioplastia y colocación de una endoprótesis vascular.

The case report of a former smoker is presented who (without any other history) after a stressful situation began to experience during more than 20 minutes a severe retrosternal and oppressive precordial pain irradiated to the left shoulder, accompanied by diaphoresis. Taking into account the results of the stress test and ultrasonography, cardiac catheterization was performed that allowed to diagnose an abnormality in the left coronary artery, which consisted in the birth of the anterior descending artery from right coronary artery. The obstruction of both arteries was successfully removed by angioplasty and vascular stent placement.

The development and use of an innovative laboratory method for measuring arsenic in drinking water from western Bangladesh.

All of Bangladesh's approximately 10 million drinking-water tube wells must be periodically tested for arsenic. The magnitude of this task and the limited resources of Bangladesh have led to the use of low-cost, semiquantitative field kits that measure As to a relatively high 50 microg/L national drinking water standard. However, there is an urgent need to supplement and ultimately replace these field kits with an inexpensive laboratory method that can measure As to the more protective 10 microg/L World Health Organization (WHO) health-based drinking water guideline. Unfortunately, Bangladesh has limited access to atomic absorption spectrometers or other expensive instruments that can measure As to the WHO guideline of 10 microg/L. In response to this need, an inexpensive and highly sensitive laboratory method for measuring As has been developed. This new method is the only accurate, precise, and safe way to quantify As < 10 microg/L without expensive or highly specialized laboratory equipment. In this method, As is removed from the sample by reduction to arsine gas, collected in an absorber by oxidation to arsenic acid, colorized by a sequential reaction to arsenomolybdate, and quantified by spectrophotometry. We compared this method with the silver diethyldithiocarbamate [AgSCSN(CH2CH3)2] and graphite furnace atomic absorption spectroscopy (GFAAS) methods for measuring As. Our method is more accurate, precise, and environmentally safe than the AgSCSN(CH2CH3)2 method, and it is more accurate and affordable than GFAAS. Finally, this study suggests that Bangladeshis will readily share drinking water with their neighbors to meet the more protective WHO guideline for As of 10 microg/L.