The diagnosis, treatment, and outcomes of Filipinos with Marfan Syndrome

@#<p style="text-align: justify;"><strong>Introduction:</strong> Marfan syndrome is an autosomal dominant disorder of connective tissue that results from abnormal fibrillin-1. It is caused by a mutation in the FBN1 gene making it a multisystemic disease. It is the most common but may have overlap with other thoracic aortic syndromes. It is primarily diagnosed using the 2010 revised Ghent nosology criteria that rely on the presence of family history, progressive aortic root dilatation or dissection, ectopia lentis, genetic testing, and systemic features. Unfortunately, a lack of nationwide awareness, family history, and medical access may lead to delayed diagnosis and treatment in our setting.</p><p style="text-align: justify;"><strong>Case Summary:</strong> We report 4 cases of Marfan Syndrome from May to October 2019, which were seen at our institution. They presented with either recently diagnosed aortic aneurysm, dissection, or severe heart failure. We used the Revised Ghent Nosology for diagnosis and treated them with the standard of care. However, outcomes were different across patients.</p><p style="text-align: justify;"><strong>Discussion:</strong> The life expectancy is comparable to the general population If early diagnosis is achieved. A myriad of known cardiovascular complications can manifest from severe valvular heart disease secondary to mitral valve prolapse, aortic root dilatation, and, most commonly, aortic dissection.</p><p style="text-align: justify;"><strong>Conclusion:</strong> A late diagnosis presenting as aortic dissection or severe valvular heart disease may have a poor quality of life and prognosis. A thoracic aortic syndrome, most commonly Marfan, should still be considered in patients with aortic criterion and premature aortic syndrome even if a systemic score of 7 is not fulfilled. The screening of first-degree relatives of affected patients is an effective strategy to diagnose them at an early stage where they are still asymptomatic.</p>

Modern treatment strategies for penile prosthetics in Peyronie's disease: a contemporary clinical review / 亚洲男科学杂志(英文版)

Peyronie's disease is a common condition resulting in penile deformity, psychological bother, and sexual dysfunction. Erectile dysfunction is one common comorbid condition seen in men with Peyronie's disease, and its presence significantly impacts treatment considerations. In a man with Peyronie's disease and significant erectile dysfunction who desires the most reliable treatment, penile prosthesis placement should be strongly considered. In some instances, such as those patients with relatively mild curvature, prosthesis placement alone may result in adequate straightening. However, many patients will require additional straightening maneuvers such as manual modeling, penile plication, and tunica albuginea incision with or without grafting. For patients with severe penile shortening, penile length restoration techniques may also be considered. Herein, we provide a comprehensive clinical review of penile prosthesis placement in men with Peyronie's disease. Specifically, we discuss preoperative indications, intraoperative considerations, adjunctive straightening maneuvers, and postoperative outcomes.

What Can We Do for Chronic Scrotal Content Pain?

Chronic scrotal content pain remains one of the more challenging urological problems to manage. This is a frustrating disorder to diagnose and effectively treat for both the patient and clinician, as no universally accepted treatment guidelines exist. Many patients with this condition end up seeing physicians across many disciplines, further frustrating them. The pathogenesis is not clearly understood, and the treatment ultimately depends on the etiology of the problem. This article reviews the current understanding of chronic scrotal content pain, focusing on the diagnostic work-up and treatment options.

Aptamers as a promising approach for the control of parasitic diseases

ABSTRACT Aptamers are short single-stranded RNA or DNA oligonucleotides that are capable of binding various biological targets with high affinity and specificity. Their identification initially relies on a molecular process named SELEX (Systematic Evolution of Ligands by EXponential enrichment) that has been later modified in order to improve aptamer sensitivity, minimize duration and cost of the assay, as well as increase target types. Several biochemical modifications can help to enhance aptamer stability without affecting significantly target interaction. As a result, aptamers have generated a large interest as promising tools to compete with monoclonal antibodies for detection and inhibition of specific markers of human diseases. One aptamer-based drug is currently authorized and several others are being clinically evaluated. Despite advances in the knowledge of parasite biology and host-parasite interactions from "omics" data, protozoan parasites still affect millions of people around the world and there is an urgent need for drug target discovery and novel therapeutic concepts. In this context, aptamers represent promising tools for pathogen identification and control. Recent studies have reported the identification of "aptasensors" for parasite diagnosis, and "intramers" targeting intracellular proteins. Here we discuss various strategies that have been employed for intracellular expression of aptamers and expansion of their possible application, and propose that they may be suitable for the clinical use of aptamers in parasitic infections.

Holopothrips chaconi sp.n. (Thysanoptera: Phlaeothripinae) from leaf-vein galls on Piper species (Piperaceae) in Costa Rica

AbstractMost species of the Neotropical genus Holopothrips are associated with plant galls but very little is known about their biology. Here, we provide observations on the biology of a new species of Holopothrips associated with leaf-vein galls on four species of Piper at a cloud forest site (Zurquí de Moravia) in Costa Rica. This species colonized the galls soon after the gall-inducing cecidomyiid or one of its parasitoids emerged, and several generations of thrips appeared to occupy the empty galls. A total of 175 empty galls from 34 leaves were collected, brought to the laboratory for dissection under the microscope, and the contents quantified. Holopothrips occupied approximately 75 % of the galled leaves and among the latter they occupied about 40 % of the galls. Every combination of adults, nymphs and eggs was found, which implies that adult thrips move in and out of galls, and possibly do not defend their galls from invasion by other members of their species. However, when disturbed, both nymphs and adults raise the tip of their abdomen and emit a distinctive odor, suggesting a defensive reaction against potential predators. Preliminary evidence suggests that they feed on gall tissue lining the inner cavity and spend very little time outside the galls. The thrips is described as a new species, and is one of only four known species of Holopothrips to have the unusual condition of two pairs of epimeral setae on the pronotum. This new species was compared to these other three Holothrips species, and the remarkable variation of the female spermatheca and the male sternal pore plate is illustrated. Further research is needed to confirm that several generations of thrips occupy empty galls, to determine whether adult thrips do indeed move between galls, and to explore in greater detail their possible chemical defense. Rev. Biol. Trop. 63 (4): 1035-1042. Epub 2015 December 01.

ResumenLa mayoría de las especies del género neotropical Holopothrips se asocian a las agallas de plantas pero se sabe muy poco de su biología. Aquí, se provee observaciones sobre la biología de una nueva especie de Holopothrips que se asocia a las agallas de venas foliares en cuatro especies de Piper en un bosque nuboso (Zurquí de Moravia) de Costa Rica. Esta especie coloniza las agallas luego de que el cecidómido inductor o un parasitoide del mismo emerge, y aparentemente varias generaciones de trips ocupan las agallas vacías. Un total de 175 agallas vacías en 34 hojas fueron recolectadas, llevadas al laboratorio para disección bajo el microscopio y se cuantificó su contenido. Holopothrips ocupaba aproximadamente el 75 % de las hojas con agallas y en ellas el 40 % de las agallas. Se encontró toda combinación de adultos, ninfas y huevos, lo cual implica que los trips adultos entran y salen y posiblemente no defienden sus agallas de la invasión de otros miembros de su especie. Sin embargo, adultos y ninfas, levantan la punta del abdomen al ser perturbados, emitiendo un olor distintivo que sugiere una reacción defensiva ante posibles depredadores. La evidencia preliminar indica que se alimentan del tejido interior de la agalla y pasan muy poco tiempo fuera de la misma. Se describe la especie nueva y es una de sólo cuatro especies conocidas de Holopothrips que poseen dos pares de setas epimorales en el pronoto. Al compararla con estas otras especies surge una marcada variación en la espermateca de la hembra y en las placas del poro esternal en el macho, los cuales se ilustran. Se requiere más investigación para confirmar la sugerencia que varias generaciones de trips ocupan las agallas vacias, para determinar si es cierto que los trips adultos se mueven entre agallas, y para explorar en más detalle la supuesta defensa química.

DEAD/DExH-Box RNA Helicases in Selected Human Parasites

DEAD/DExH-box RNA helicases catalyze the folding and remodeling of RNA molecules in prokaryotic and eukaryotic cells, as well as in many viruses. They are characterized by the presence of the helicase domain with conserved motifs that are essential for ATP binding and hydrolysis, RNA interaction, and unwinding activities. Large families of DEAD/DExH-box proteins have been described in different organisms, and their role in all molecular processes involving RNA, from transcriptional regulation to mRNA decay, have been described. This review aims to summarize the current knowledge about DEAD/DExH-box proteins in selected protozoan and nematode parasites of medical importance worldwide, such as Plasmodium falciparum, Leishmania spp., Trypanosoma spp., Giardia lamblia, Entamoeba histolytica, and Brugia malayi. We discuss the functional characterization of several proteins in an attempt to understand better the molecular mechanisms involving RNA in these pathogens. The current data also highlight that DEAD/DExH-box RNA helicases might represent feasible drug targets due to their vital role in parasite growth and development.

Nomenclatural problems among Thysanoptera (Insecta) of Costa Rica

We present data to argue that several recent papers on the Thysanoptera of Costa Rica are affected by unsatisfactory technical procedures, including failure to recognize intraspecific structural variation. Fourteen new synonyms are recognized for Costa Rica Thysanoptera, nine generic and five specific. Rev. Biol. Trop. 56 (2): 961-968. Epub 2008 June 30.

Presentamos datos para apoyar nuestro argumento de que varios artículos recientes sobre los Thysanoptera de Costa Rica se han visto afectados por procedimientos técnicos insatisfactorios, incluyendo el no reconocer la variación estructural intraespecífica. Presentamos nueve sinonimias en los tisanópteros de Costa Rica: nueve a nivel de género y cinco a nivel de especie.

Liothrips tractabilis sp.n. (Thysanoptera: Phlaeothripinae) from Argentina, a potential biocontrol agent of Weedy Campuloclinium macrocephalum (Asteraceae) in South Africa

A new species, Liothrips tractabilis, is described from northern Argentina. Feeding by this thrips causes severe damage to the leaves of Campuloclinium macrocephalum, a plant that has been introduced to South Africa where it is a serious weed of grasslands. A key is provided to the four species of the genus Liothrips recorded from Argentina.

Uma nova espécie, Liothrips tractabilis, é descrita do norte da Argentina. Essa espécie causa severo dano nas folhas de Campuloclinium macrocephalum, que foi introduzida à África do Sul, onde é planta daninha em pastagens. Uma chave é apresentada para as quatro espécies do gênero Liothrips registradas na Argentina.

Non-surgical therapy of Peyronie's disease / 亚洲男科学杂志(英文版)

The present paper provides a review of the available non-surgical treatments for Peyronie's disease (PD). A review of published literature on oral, intralesional, external energy and iontophoresis therapies for PD was performed, and the published results of available treatment options reviewed. The authors recommendations for appropriate non-surgical management of PD are provided. Although there are many published reports that show the efficacy of non-surgical therapies for PD, there is a lack of large scale, multicenter controlled clinical trials, which makes treatment recommendations difficult. Careful review of the literature does suggest that there are treatment options that make scientific sense and appear to stabilize the disease process, reduce deformity, and improve function. Offering no treatment at all will encourage our patients to pursue alternative treatments, which might do harm, and misses the opportunity to do some good. Clearly further work is necessary to develop safe and effective non-surgical treatments for PD.

Thrips species (Thysanoptera) collected at Parque Estadual de Itapuã, Viamão, RS, Brazil / Espécies de tripes (Thysanoptera) coletadas no Parque Estadual de Itapuã, Viamão, RS

The order Thysanoptera comprises mostly phytophagous or fungivorous species, with a few species that are predators. Recent studies have emphasized the diversity of behavioural patterns amongst these diminutive insects. From the 5,500 species known worldwide, about 10 percent are recorded from Brazil, mostly tropical areas. In this work we surveyed the Thysanoptera fauna of a southern locality, 800 km south of the Tropic of Capricorn. The study site was ''Parque Estadual de Itapuã'' (30°22'S 51°02'W), Viamão, RS, and thrips were sampled from wild flowers, branches, grass and litter, from June 1999 to May 2001. A total of 83 species in 32 genera was found, but only 29 of these species could be identified, emphasizing the poor knowledge of the insect fauna of southern Brazil.

A ordem Thysanoptera reúne espécies fitófagas, fungívoras e algumas predadoras. Estudos recentes têm enfatizado a diversidade de padrões de comportamentos desses pequenos insetos. Das 5.500 espécies conhecidas dispersas pelo mundo, aproximadamente 10 por cento foram registradas no Brasil (a maioria em áreas tropicais). Neste trabalho, efetuou-se o levantamento da tisanopterofauna meridional, 800 km ao sul do Trópico de Capricórnio. A área de estudo foi o Parque Estadual de Itapuã (30°22'S 51°02'W), Viamão, RS, com os tripes coletados em flores, ramos, gramíneas e folhedo, de junho/1999 a maio/2001. O total de 83 espécies foi encontrado em 32 gêneros, porém apenas 29 dessas espécies puderam ser identificadas, demonstrando como a fauna de insetos do sul do Brasil carece de estudos.

South American Haplothrips Species (Thysanoptera: Phlaeothripidae), with a new species of biological control interest to Australia against weedy Heliotropium amplexicaule (Boraginaceae) / Espécies Sul-Americanas de Haplothrips (Thysanoptera: Phlaeothripidae), com uma nova espécie de interesse para o controle biológico da invasora Heliotropium amplexicaule (Boraginaceae), na Austrália

Uma espécie nova, Haplothrips heliotropica, é descrita da Argentina, causando severos danos às folhas de Heliotropium amplexicaule (Boraginaceae), uma planta sul americana que é invasora agressiva em pastagens da Austrália. São apresentadas observações sobre especificidade de hospedeiros, variações sazonais de população e diapausa do tripes. O genero Haplothrips inclui mais de 230 espéciesem todo o mundo, a maioria alimentando-se de flores. Em contraste, adultos e larvas de H. heliotropica alimentam-se exclusivamente de tecidos verdes. Esta é a terceira espécie de Haplothrips registrada na América doSul. São apresentadas as características para distinguir essas três espécies, e também para Haplothrips heliotropii Priesner do Egypt e do Yemen.

A new species, Haplothrips heliotropica, is described from Argentina causing severe damage to the leaves of Heliotropium amplexicaule (Boraginaceae), a South American plant that is now a serious weed of pastures in Australia. Observations are presented on the host specificity, seasonal population changes and overwintering of this thrips. The genus Haplothrips includes more than 230 species worldwide, mostly feeding in flowers. In contrast, adults and larvae of H. heliotropica feed exclusively on green tissues. This is only the third South American species of Haplothrips. Character states are given for distinguishing these three species, and also for Haplothrips heliotropii Priesnerfrom Egypt and Yemen.

Thysanoptera biodiversity in the Neotropics

It is suggested that descriptive taxonomy of thrips must be integrated into biological studies if we are to understand patterns of evolutionary and ecological diversity. Collecting and describing new taxa is easy, but understanding their position in ecosystems and how they have contributed to the origin and maintenance of biological diversity is more important yet more difficult. Many authors fail to appreciate that individual thrips species are commonly highly polymorphic, both within and between sexes, with the result that 20 of species names and 30 of generic names are currently placed into synonymy. The biological significance of such polymorphism has been little studied, but the presence of large and small males in a species is presumed to indicate some form of male/male competition for resources; this is particularly common in fungus feeding species. Amongst phytophagous species, the recognition of the host plants on which thrips actually breed is a prerequisite to understanding patterns of diversity, some thrips lineages being associated with particular groups of plants whereas others exploit a diverse range of plants. Attempts to understand the diversity of thrips, including the application of cladistic methods, are severely limited by the lack of studies on the biology of individual species, although thrips exhibit a wide range of interesting biological phenomena, including various levels of sociality, gall-induction, specific pollination associations, virus transmission, and ectoparasitism.

Espécies de Thrips (Thysanoptera: Thripidae) no Brasil / Species of Thrips (Thysanoptera: Thripidae) in Brazil

Quatro espécies de Thrips: T. australis, T. palmi, T. simplex e T. tabaci. estão presentes no Brasil. Três delas são pragas de culturas e T. australis está normalmente associada a flores de Eucalyptus spp. Além de polífagas, T. palmi e T. tabaci também são vetoras de viroses. Uma chave para diferenciar essas espécies é apresentada, assim como a sua caracterização morfológica.

Four Thrips species: T. australis, T. palmi, T. simplex e T. tabaci are recorded in Brazil. Three of them are crop pests and T. australis is commonly associated to Eucalyptus spp. flowers. Besides being polyphagous, T. palmi and T. tabaci are also virus-vector. A key to distinguish these species is provided, as well as their characterization.

Espécies de Frankliniella (Thysanoptera: Thripidae) de importância agrícola no Brasil / Species of Frankliniella (Thysanoptera: Thripidae) as pests in Brazil

Seis espécies de Frankliniella: F. brevicaulis Hood, F. condei John, F. occidentalis (Pergande), F. schultzei (Trybom), F. williamsi Hood e F. zucchini Nakahara & Monteiro são consideradas pragas no Brasil, sendo três delas vetoras de viroses. Uma chave para diferenciar essas espécies é apresentada, assim como sua caracterização morfológica.

Six Frankliniella species: F. brevicaulis Hood, F. condei John, F. occidentalis (Pergande), F. schultzei (Trybom), F. williamsi Hood and F. zucchini Nakahara & Monteiro are pests in Brazil, three of them are virus-vector. A key to distinguish these species is provided, as well as their morphological characterization.

Urinary Gonadotropin Fragment ( UGF ) Measurements , Its Efficacy in Patients with Gynecologic and Various Malignancies / 대한암학회지

No abstract available.