RESUMEN

Non-obstructive hypertrophic cardiomyopathy, or apical hypertrophic cardiomyopathy (ApHCM), also referred to as Yamaguchi syndrome, is a type of hypertrophic cardiomyopathy (HCM) characterized by significant thickening of the left ventricular apex without blockage in the left ventricular outflow tract. It is a very rare variant of HCM. Patients with non-obstructive HCM often experience symptoms such as chest pain, palpitations, shortness of breath, and syncope, which may resemble those seen in various cardiovascular and non-cardiac conditions. Yamaguchi syndrome presents as a challenging yet manageable condition in the ED. Early recognition, accurate diagnosis, and appropriate management are crucial for better outcomes. We report a case of a young female who presented to the ED with breathlessness and chest pain. The ECG findings suggested acute coronary syndrome (ACS), but echocardiography and cardiac biomarkers indicated otherwise, leading to the diagnosis of Yamaguchi Syndrome.

RESUMEN

Objective Adult-onset Still's disease (AOSD) is a rare orphan disease, the diagnosis of which remains challenging. This study aimed to identify additional clues for establishing early diagnosis beyond the existing criteria. Methods A retrospective longitudinal cohort study was conducted at two community hospitals in Japan between March 2012 and December 2022. The clinical characteristics and medical histories of patients with AOSD were extracted from the clinical records. The primary outcome was to identify the key manifestations of AOSD for an early diagnosis beyond the existing criteria. Results Twenty-one patients (mean age, 58 years) were included in the study. Fever was the first symptom in 13 out of 21 patients (62%). Six out of 21 patients (29%) presented with a pruritic rash only, while two out of 21 (10%) initially presented with a sore throat. All patients visited more than one medical institution. The median time to reach a correct diagnosis was 41 days (IQR 19-138). Nineteen out of 20 patients (95%) exhibited a pruritic rash, identified as persistent pruritic linear streaks, with a median duration of 21 days (IQR 12-64) before the diagnosis of AOSD as a cutaneous manifestation. Conclusions Persistent pruritic linear streaks were a key feature in the context of an early diagnosis of AOSD, offering an option for reconsidering and revising the existing classification criteria.

RESUMEN

Adult-onset Still's disease (AOSD) stands as a perplexing condition with diverse clinical manifestations, posing significant diagnostic challenges for healthcare professionals. This case report delves into the clinical trajectory, diagnostic challenges, treatment strategies, and outcomes experienced by a 67-year-old female with AOSD. This report advocates for considering AOSD as a potential diagnosis in patients presenting with systemic inflammatory symptoms, especially when other conditions have been ruled out. It highlights the complexity of AOSD and the importance of interdisciplinary collaboration, close monitoring, and personalized treatment strategies to optimize patient outcomes.

RESUMEN

An inflammatory condition known as adult-onset Still's disease (AOSD) is typified by quotidian (daily) fevers, arthritis, and a transient rash. This report details the case of a 21-year-old female patient who presented to our hospital with polyarthritis, a red rash, and a high-grade fever for more than three months. History was suggestive of AOSD, which was further proved by investigation and Yamaguchi criteria for AOSD. Steroids and methotrexate were started, to which the patient responded very well and thereafter.

RESUMEN

Feature extraction plays a pivotal role in processing remote sensing datasets, especially in the realm of fully polarimetric data. This review investigates a variety of polarimetric decomposition techniques aimed at extracting comprehensive information from polarimetric imagery. These techniques are categorized as coherent and non-coherent methods, depending on their assumptions about the distribution of information among polarimetric cells. The review explores well-established and innovative approaches in polarimetric decomposition within both categories. It begins with a thorough examination of the foundational Pauli decomposition, a key algorithm in this field. Within the coherent category, the Cameron target decomposition is extensively explored, shedding light on its underlying principles. Transitioning to the non-coherent domain, the review investigates the Freeman-Durden decomposition and its extension, the Yamaguchi's approach. Additionally, the widely recognized eigenvector-eigenvalue decomposition introduced by Cloude and Pottier is scrutinized. Furthermore, each method undergoes experimental testing on the benchmark dataset of the broader Vancouver area, offering a robust analysis of their efficacy. The primary objective of this review is to systematically present well-established polarimetric decomposition algorithms, elucidating the underlying mathematical foundations of each. The aim is to facilitate a profound understanding of these approaches, coupled with insights into potential combinations for diverse applications.

RESUMEN

Yamaguchi syndrome or apical hypertrophic cardiomyopathy is a rare subtype of non-obstructive hypertrophic cardiomyopathy that is defined as the focused hypertrophy of the left ventricular apex. It is typically seen in Asian populations. Herein, we present a rare case of Yamaguchi syndrome seen in a Hispanic female.

RESUMEN

Apical hypertrophic cardiomyopathy, also called Yamaguchi syndrome, is a rare variant of hypertrophic cardiomyopathy. Yamaguchi syndrome is characterized by hypertrophy almost confined to the apical region of the left ventricle rather than the left ventricular septum. A case of 65-year-old Saudi man presented to the ER with angina, and the ECG, echocardiogram, and nuclear study confirmed the diagnosis with Yamaguchi. Reporting this case serves to help physicians broaden their vision in approaching patients with symptoms mimicking acute coronary syndrome.

RESUMEN

Resumen La enfermedad de Still del adulto (ESA) es una enfermedad reumática e inflamatoria, infrecuente, de etiología desconocida. Su presentación clínica es variada y sus síntomas más frecuentes son fiebre, artralgias, exantema asalmonado evanescente, odinofagia, adenopatías, hepatoesplenomegalia, serositis y presencia en laboratorio de leucocitosis (neutrofilia), hiperferritinemia asociado a ausencia de anticuerpos. El diagnóstico es clínico y por exclusión. Existen criterios clasificatorios como son los de Yamaguchi1, Cush y Fautrel2,3 que ayudan a la orientación diagnóstica. El tratamiento se define según la presentación y la evolución clínica1. Se describen las características clínicas, diagnósticas, tratamiento y evolución de siete pacientes con ESA.

Abstract Adult Still's disease (ASD) is an uncommon rheumatic and inflammatory disorder of unknown etiology. The major clinical features include fever, arthralgia, transient salmon-pink rash, odynophagia, lymphadenopathy, hepatosplenomegaly, serositis, and laboratory findings such as leukocytosis with neutrophilia, hyperferritinemia, and negative immunologic laboratory testing. ASD diagnosis is reached by exclusion. Different classification criteria such as Yamaguchi, Cush and Fautrel have been developed for the identification of ASD. Treatment is based on the degree of disease activity and clinical response1. We describe clinical manifestations, diagnosis, treatment, and prognosis of seven patients with adult's Still disease.

Asunto(s)

RESUMEN

Apical hypertrophic cardiomyopathy is a rare variant of hypertrophic cardiomyopathy characterized by abnormal heart muscle thickening, specifically affecting the left ventricle's apex. Classically revealing both giant T-wave inversions in the precordial leads of an electrocardiogram and a spade-like configuration of the left ventricular cavity on ventriculograms, the diagnosis of the apical variant has evolved with cardiac magnetic resonance imaging. Despite being well known among East Asian populations, the diagnosis of apical hypertrophic cardiomyopathy is often underestimated and overlooked among American patients due to the non-specific nature of echocardiography. In this case report, we present the diagnosis of apical hypertrophic cardiomyopathy in a middle-aged African American male with chronic palpitations. The diagnosis was confirmed using cardiac magnetic resonance imaging, which revealed extensive myocardial fibrosis. Ultimately, the patient was treated with an implantable cardioverter-defibrillator. Our case aims to enhance the understanding and facilitate the recognition and management of apical hypertrophic cardiomyopathy, particularly among non-Asian individuals. Current challenges revolve around robust risk stratification strategies for patients at high risk for sudden cardiac death that require device therapy.

RESUMEN

Apical hypertrophic cardiomyopathy (ApHCM) is thought to be an uncommon variant of hypertrophic cardiomyopathy (HCM). This article is a literature review focusing on the characteristic electrocardiogram (EKG) and 2D echocardiogram findings as currently there are no specific ACC/AHA/ESC guidelines set as diagnostic criteria for ApHCM.

Asunto(s)

RESUMEN

The total synthesis of the 16-membered Polyhydroxylated macrolide, Aspergillide D has been accomplished utilizing the Grignard reaction, Sharpless asymmetric epoxidation Regioselective ring opening of epoxy alcohol, Wittig olefination and Yamaguchi macrolactonisation as key steps. 3-butene-1-ol has been utilized as the starting material.

RESUMEN

An efficient stereoselective total synthesis of (3R, 4S)-4-hydroxylasiodiplodin has been accomplished starting from known starting materials. The key steps involved in this synthesis are Stille cross coupling, alkylation of 1,3-dithiane and Yamaguchi macrolactonization.

Asunto(s)

RESUMEN

Recently, non-specific low back pain without clear diagnosis has been changed of the diagnosis and treatment. I showed the data of Yamaguchi Low Back Pain study in the article. It has been shown that we can select appropriate treatment based on the clear diagnosis. I described the current status and issues of low back pain treatment and outlined the classification and differentiation of non-specific low back pain.

RESUMEN

Electrocardiogram (ECG) still remains a very useful diagnostic method in modern cardiology. Its broad availability, noninvasiveness and good sensitivity explain why it plays a capital role in the very beginning of the process of diagnosis for every patient, with or without cardiac-related complaints. For the practitioner, good training in ECG interpretation is mandatory. Sometimes, the ECG trace reveals particular aspects that may cause confusion and complicate decision-making. In this article, we present several less common situations underlying the general context and ECG features. The syndromes studied have a high pathological significance and may range from acute emergencies that call for a rapid therapeutical response to chronic syndromes that require prolonged observation, monitoring and risk stratification.

RESUMEN

Yamaguchi-variant cardiomyopathy is an underreported but significant cause of cardiac arrest among athletes. We studied the hospital course of one patient who arrived at the emergency department after a sudden cardiac arrest while playing a recreational basketball game. We used the electronic medical record (Epic) to follow the notes, labs, imaging, and procedures that were performed. Although a rare disease, Yamaguchi syndrome should not be overlooked when working up a patient who has suffered a sudden cardiac arrest. Proper knowledge of automatic external defibrillators and basic cardiopulmonary resuscitation principles can have a significant positive impact, and the importance of these interventions should not be overlooked in patients with a sudden cardiac arrest.

RESUMEN

Apical hypertrophic cardiomyopathy is a relatively rare subtype of hypertrophic cardiomyopathy with a wide range of clinical manifestation. The most frequent symptom is chest pain and thus it can mimic the acute coronary syndrome and due to unfamiliarity of this condition by some physicians, the diagnosis is frequently missed or delayed. In this case, report our purpose is to emphasize the importance of keeping apical hypertrophic cardiomyopathy as one of the differential diagnoses in a young patient presented with chest pain.

RESUMEN

Our patient was a 56-year-old Caucasian female who had 34 emergency department visits to our center with recurrent chest pain, of which eleven were of cardiac etiology, involving cardiac causes over the period of seven years. Her chest pain was diagnosed as atypical during her previous visits. Chest CT revealed "ace-of-spades" in the cardiac transverse section. A transthoracic echocardiogram (TTE) demonstrated apical hypertrophy with end-systolic cavity obliteration and an ejection fraction (EF) of 65%-70%, seated amidst a normal-sized left ventricle, with normal wall thickness, indicating Yamaguchi syndrome. In the case report, we portray the need to widen the spectrum of differentials in an encounter with a patient presenting with chest pain.

RESUMEN

Adult-onset Still's disease (AOSD) is a disorder that is occasionally seen. Autoinflammation is a generally accepted pathogenic mechanism leading to systemic signs and symptoms. We report the case of a young female presenting with high-grade fever, rash, and arthralgias. After a thorough assessment, the diagnosis of adult-onset Still's disease was made based on presenting symptoms and elevated serum ferritin, c-reactive protein (CRP), and absence of serologic markers and confirmed based on Yamaguchi criteria. She was treated with corticosteroids and achieved complete clinical remission.

RESUMEN

The total synthesis of Benzannulated macrolide, (+)-Xestodecalactone A was accomplished starting from commercially available enantiomerically pure propylene oxide and 3,5-dihydroxyphenylacetic acid using Grignard reaction, alkylation of 1,3-dithiane and Yamaguchi macrolactonisation as key steps.

Asunto(s)

RESUMEN

Apical hypertrophic cardiomyopathy (ApHCM), also known as Yamaguchi syndrome represents an uncommon morphologic variant of hypertrophic cardiomyopathy (HCM) in which the myocardial hypertrophy predominantly involves the apex of the left ventricle (LV). It is exemplified by "giant" negative precordial T-waves on electrocardiography and a peculiar "spade-like" configuration of LV cavity on ventriculography historically, and more recently, on echocardiography with use of image enhancing agents. The disease entity was first described in 1976. Available literature reveals that it is prevalent largely among the East-Asian population but is rare among non-Asians. Here, we report a case of a 66-year-old Hispanic male with multiple cardiac histories including persistent atrial fibrillation, non-ST-elevation myocardial infarction (NSTEMI), and ventricular fibrillation cardiac arrest with multiple inconclusive evaluations, who later in life was found to have ApHCM. This case highlights the rare incidence of the disease among the Hispanic population and underlines the challenging diagnosis that requires a high index of suspicion in patients with cardiac symptoms, as ApHCM can masquerade as ischemic coronary heart disease. Our case also describes an unusual clinical course for ApHCM presenting with extreme clinical features, including ventricular arrhythmias and cardiac arrest, unlike the usual benign natural history of this disease.