ABSTRACT
@#A family was studied in which three members in the sibship belonging to the fourth generation were found to have Rotor’s syndrome. More detailed examinations including blood studies, liver profiles, oral cholecystograms, and liver biopsies where performed on the affected siblings. The results were related to what is at present known about the features and mechanisms of Rotor’s syndrome, pari passu the current concept of bilirubin metabolism. It is suggested that the constant finding, and possibly the only characteristic one in Rotor’s syndrome, is the absence of abnormal hepatic cell pigmentation. Pedigree analysis of the present family shows that the transmission of this disorder may be conditioned by an autosomal recessive gene.
Subject(s)
Hyperbilirubinemia, HereditaryABSTRACT
Las hiperbilirrubinemias hereditarias (HBH) son patologías originadas por defectos en las enzimas y proteínas que participan del metabolismo de la bilirrubina. El clearence de bilirrubina incluye captación y almacenamiento en hepatocitos, conjugación, excreción hacia la bilis y recaptura de su forma conjugada por hepatocitos. Las HBH varían de acuerdo a su patogenia, presentación clínica, niveles de bilirrubinemia y tratamientos disponibles. En general son poco frecuentes, a excepción del Síndrome de Gilbert. Están las que son de predominio indirecto, como el Síndrome de Gilbert y el de Crigler-Najjar, y las de predominio directo, como el Síndrome de Dubin-Johnson y el de Rotor. En general no requieren tratamiento específico y tienen curso benigno, a excepción del Síndrome de Crigler-Najjar para el cual existen medidas terapéuticas específicas a considerar, teniendo un pronóstico reservado para algunas de sus formas de presentación. Es importante el conocimiento de estos síndromes dado el alto índice de sospecha requerido para su diagnóstico y para su diferenciación de otras patologías hepatobiliares de mayor riesgo y severidad.
Hereditary hiperbilirrubinemias (HBH) are pathologies originated from the defect of the enzymes and proteins involved in the metabolism of bilirubin. The bilirubin clearance includes uptake and storage in hepatocytes, conjugation, excretion into bile and recapture of its conjugated form by hepatocytes. HBH vary according to their pathogenesis, clinical presentation, levels of bilirubin and available treatments. Generally they are infrequent, except for Gilbert Syndrome. There are those with indirect bilirubin predominance, such as Gilbert and Crigler-Najjar syndromes, and those with direct bilirubin predominance, including Dubin-Johnson and Rotor syndromes. In general, they do not require specific treatment and have a benign course, with the exception of the Crigler-Najjar Syndrome, for which there are specific therapeutic measures to consider, as well as a reserved prognosis for some of their forms of presentation. The knowledge of these syndromes is important 2 given the high index of suspicion required for its diagnosis and for its differentiation from other hepatobiliary pathologies of greater risk and severity.
Subject(s)
Humans , Crigler-Najjar Syndrome/diagnosis , Gilbert Disease/diagnosis , Hyperbilirubinemia, Hereditary/diagnosis , Jaundice, Chronic Idiopathic/diagnosis , Crigler-Najjar Syndrome/etiology , Gilbert Disease/etiology , Hyperbilirubinemia, Hereditary/etiology , Jaundice, Chronic Idiopathic/etiologyABSTRACT
An introduction was first given to the advantages and application status of multi-rotor unmanned aerial vehicle (UAV) in the field of medical rescue. Then, the latest worldwide application and research status of multi-rotor UAV in this field were analyzed according to available research. On this basis, the application prospects of multi-rotor UAV in the scenarios of medical rescue field were assumed. Finally, it was pointed out that multi-rotor UAV would show more functions in medical rescue field owing to various technological development and breakthrough, signifying the bright deepening application prospect of multi-rotor UAV.
ABSTRACT
The mechanism of triggering and maintaining atrial fibrillation (AF) is still not clear. There are different understandings about triggering and maintenance of AF, and therapeutic strategies relevant to them could not reach an agreement. In recent years, basic researches on AF have made greater progress. For example, the atrial rotor and autonomic nerve plexus theories has also been identified clinically, and it changed the strategies of radiofrequency ablation treatment for AF. This article summarizes the mechanism and current status of AF ablation.
ABSTRACT
La fibrilación auricular (FA) es la arritmia más común en la práctica clínica y por la que más se consulta en los servicios médicos. Recientemente, se ha propuesto un mecanismo de mantenimiento de la FA, el cual consiste en la existencia de uno o varios rotores que activan el tejido a alta frecuencia. La ablación es uno de los tratamientos para la FA, en FA crónica son necesarios patrones de ablación complejos, por lo que actualmente se busca el patrón ideal con un mínimo número de líneas de ablación. En este trabajo se simula la actividad de un rotor en un modelo 2D de tejido auricular humano, bajo condiciones de FA crónica y se localiza su centro de giro (tip). Se proponen y evalúan seis diferentes patrones simples de ablación compuestos por un número reducido de líneas. El estudio demostró que aquellos patrones que atraviesan o encierran el tip del rotor y que adicionalmente se prolongan hasta una frontera de conducción son eficaces en la terminación del rotor.
Atrial fibrillation (AF) is the most common arrhythmia in clinical practice and the most consulted in medical services. Recently it has been proposed a mechanism for maintaining the AF, which consists in one or more rotors activating the tissue at high frequency. Ablation is one of the treatments for AF, for chronic AF is needed complex ablation patterns, so currently it has been looking for an ideal pattern with a minimum number of ablation lines. In this work activity of a rotor was simulated in a 2D model of human atrial tissue, under chronic AF conditions, and the center of rotation (tip) was located. Six different simple ablation patterns composed of a limited number of lines were proposed and evaluated. The study showed that those patterns that passed through or encloses the tip of the rotor and additionally were extended to a conduction boundary are effective in the termination of the rotor.
A fibrilação atrial (FA) é a arritmia mais comum na prática clínica e na consulta que a maioria dos serviços médicos. Recentemente, é proposto um mecanismo para a manutenção da FA, que é a existência de um ou mais rotores que activam o tecido com elevada frequência. A ablação é um tratamento para fibrilação atrial em padrões crônicos de ablação de FA necessárias são complexas, por isso eles estão atualmente buscando o padrão ideal, com um número mínimo de linhas de ablação. Neste documento, a actividade de um rotor é simulada em um modelo em 2D do tecido atrial humano, sob condições de AF crónica e o seu centro de rotação (ponta) está localizado. São propostos e avaliados seis diferentes padrões de ablação individuais compostos de um pequeno número de linhas. O estudo mostrou que as que passam através de padrões ou encerram a ponta do rotor e se estendem para além de um limite de condução são eficazes na cessação do rotor.
ABSTRACT
INTRODUÇÃO: o plano de tratamento ortodôntico deve considerar fatores como apinhamento dentário, protrusão dentoalveolar, discrepâncias maxilares e necessidade de alteração do perfil facial. Também se deve ter em mente as características de normalidade conforme a raça do paciente. Assim, buscando uma abordagem individual do paciente, em casos limítrofes, a terapia ortodôntica pode ser realizada com ou sem extrações. OBJETIVO: realizar uma revisão da literatura sobre a terapia de redução de esmalte interproximal como uma alternativa de tratamento ortodôntico sem extrações para pacientes considerados limítrofes e exemplificar a técnica a partir do relato de um caso clínico. MÉTODOS: para a execução da redução, usou-se brocas Carbide em alta rotação para a remoção do esmalte, discos Sof-Lex para o acabamento e polimento das superfícies desgastadas e aplicação tópica de fluoretos, visando aumentar a proteção do esmalte. CONCLUSÃO: concluiu-se que a técnica é indicada na resolução de discrepâncias no comprimento do arco dentário de até 8,5mm, a fim de evitar as extrações dentárias em pacientes com bom perfil facial. Preferencialmente, os dentes submetidos à técnica devem ser largos, com forma triangular e paredes de esmalte espessas.
INTRODUCTION: The orthodontic treatment plan must consider factors like dental crowding, dental alveolar protrusion, maxillary discrepancies and the facial profile change necessity. It must be considered that the normal characteristics changes in conformity with the ethnic origin of the patient. Then, searching for the individualization of treatment for each patient, in borderlines cases the orthodontic therapy can be done with or without extractions. AIM: To review the literature about the interproximal enamel reduction therapy as an orthodontic treatment alternative to extractions in this borderline patients and to exemplify the technic with a case report. METHODS: To execute the enamel reduction it was used the high-speed Carbide bur and Sof-Lex discs to polish the striped surfaces, followed by topic application of fluoride to increase enamel protection. CONCLUSION: Technic is indicated to solve discrepancies in the dental arch smaller than 8,5mm, to avoid extractions in patients with good facial profile. It's mandatory that the teeth to be striped must be large, triangular and with thick proximal enamel layers.
Subject(s)
Humans , Male , Adult , Dental Enamel , Orthodontics , Face , Tooth AttritionABSTRACT
Objective: To develop the numerical method for the steady and unsteady wet steam condensing flow in turbine stage. Methods: An Eulerian/Eulerian numerical model is used to describe the spontaneous condensation flow in the steam turbine. For the steady condensing flow computations, the mixing plane model was used. For the unsteady condensing flow computations, the sliding mesh method was used to simulate the rotor-stator interactions. Results: The numerical results showed the obvious differences between non-condensing and condensing flows. The results also showed the unsteadiness effect due to rotor-stator interactions had a deep influence on the formation and growth process of water droplets. Conclusion: The numerical methods presented in this paper are valid for the condensing flow in the turbine stage.
ABSTRACT
Objective To develop the numerical method for the steady and unsteady wet steam condensing flow in turbine stage. Methods An Eulerian/Eulerian numerical model is used to describe the spontaneous condensation flow in the steam turbine. For the steady condensing flow computations, the mixing plane model was used. For the unsteady condensing flow computations, the sliding mesh method was used to simulate the rotor-stator interactions. Results The numerical results showed the obvious differences between non-condensing and condensing flows. The results also showed the unsteadiness effect due to rotor-stator interactions had a deep influence on the formation and growth process of water droplets. Conclusion The numerical methods presented in this paper are valid for the condensing flow in the turbine stage.
ABSTRACT
Rotor syndrome is a rare, benign familial disorder characterized by chronic fluctuating, nonhemolytic and predominantly conjugated hyperbilirubinemia with normal hepatic histology. In contrast to Dubin-Johnson syndrome, there is no liver pigmentation in Rotor syndrome. A 36-year-old man was admitted due to asymptomatic persistent jaundice. His siblings had jaundice with direct hyperbilirubinemia. Physical examination revealed icteric sclerae without hepatosplenomegaly. Laboratory findings showed increased serum bilirubin with direct bilirubinmia. Hepatic uptake and storage capacity of indocyanine green was markedly reduced, while excretion into bile was slightly suppressed. Markedly decreased hepatic uptake and poor visualization of the gallbladder and biliary tract were shown in 99mTc-DISIDA scan. Histology of the liver showed mild steatosis without pigmentation. We report a case with the review of literature.
Subject(s)
Adult , Humans , Male , Coloring Agents , Hyperbilirubinemia, Hereditary/diagnosis , Indocyanine Green , Jaundice, Chronic Idiopathic/diagnosis , Liver/diagnostic imaging , Liver Function Tests , Radiopharmaceuticals , Technetium Tc 99m DisofeninABSTRACT
Rotor's syndrome is a hereditary disorder characterized by predominantly conjugated hyperbilirubinemia with normal hepatic histology. It resembles Dubin-Johnson syndrome but the main differences are no dark brown pigmentation in the hepatic cells and visualization of the gallbladder in oral cholangiography. We experienced a 14 year-old male patient who had icteric sclerae and predominantly conjugated hyperbilirubinemia when he was hospitalized for varicocelectomy. His liver biopsy specimen showed no dark brown pigmentation and any other pathologic abnormalities in the hepatic cells. Hepatobiliary scan shows no evidence of obstructive lesions. His urinary excretion of total coproporphyrin was markedly increased.
Subject(s)
Adolescent , Humans , Male , Biopsy , Cholangiography , Gallbladder , Hepatocytes , Hyperbilirubinemia , Jaundice, Chronic Idiopathic , Liver , Pigmentation , ScleraABSTRACT
Rotor syndrome is a rare benign familial disorder characterized by chronic, fluctuating, nonhemolytic and predominantly direct bilirubinemia with normal liver tissue. We have recently experienced two cases of Rotor syndrome in a brother and sister. They revealed icteric sclerae with mild hepatomegaly in physical examination. Laboratory findings showed increased serum bilirubin with direct bilirubin predominance. The urinary excretion of total coproporphyrin was slightly elevated. The 99mTc-DISIDA scan showed a markedly decreased hepatic uptake and poor visualization of gallbladder and biliary tree which could be compatible to the Rotor syndrome. We report two cases with a review of the literature.
Subject(s)
Humans , Biliary Tract , Bilirubin , Gallbladder , Hepatomegaly , Hyperbilirubinemia , Hyperbilirubinemia, Hereditary , Liver , Physical Examination , Sclera , Siblings , Technetium Tc 99m DisofeninABSTRACT
This paper introduces what signals are actually transmitted in every procedure of the start-up process of Mx8000 Exp double-slice spiral CT and what relevant parts are acted.Some troubles related to start-up process is briefly presented.
ABSTRACT
We experienced two cases of Rotor syndrome in brothers who were a 13 year-old boy and an 11 year-old boy, respectively. They presented with icteric scleras for a few months. Their common laboratory characteristics were as follows: Direct bilirubin was more increased than indirect bilirubin, but aminotransferases were normal. Plasma indocyanine green (ICG) test revealed hepatic excretory defect: plasma ICG concentrations 15 minutes after intravenous injection were 80.45% and 78.28%, respectively. 99mTc-DISIDA Hepatobiliary scan showed that severely decreased hepatic extraction with mild cardiac blood pool, markedly delayed biliary excretion in both intra- & extra- hepatic bile ducts, delayed visualization of gall bladder, and markedly delayed intestinal biliary passage. Needle liver biopsy showed normal hepatic histology without pigmentation.
Subject(s)
Adolescent , Child , Humans , Male , Bile Ducts , Bilirubin , Biopsy , Hyperbilirubinemia, Hereditary , Indocyanine Green , Injections, Intravenous , Liver , Needles , Pigmentation , Plasma , Sclera , Siblings , Technetium Tc 99m Disofenin , Transaminases , Urinary BladderABSTRACT
BACKGROUND: Rotor off (R/O) fraction obtained by counterflow centrifugal elutriation (CCE) contains small number of T cells and many hematopoietic stem cells. Since megakaryocytes and its progenitors are larger than other cells in bone marrow, it may be easier to be separated by CCE and newly applied to hematopoietic stem cell transplantation (HSCT) for early megakaryocytes reconstitution. METHODS: The marrow cells of BALB/c mice in each group (17, 25, 28mL/min, and R/O fraction) were cultured for quantifying CFU-MK and measured after 10 days. BALB/c mice were lethally irradiated and transplanted with R/O cells. The dosages of transplanted cells were 5x104 in Group A, 5x105 in Group B, and 5x106 in Group C. The platelet counts in peripheral blood were measured up to post-transplant day 14. RESULTS: After CCE, recovery rate of the loaded cells was 82.2% and the R/O fraction was 35.9%. Most CFU-MK were formed in R/O fraction, and Group C showed the fastest recovery. Group A couldn't reach the level of 100x109/L until post-transplant day 14, and Group B showed slower recovery compared to Group C. All 5 mice survived in Group C, but 2 out of 5 mice survived in Group A and B. CONCLUSIONS: R/O fraction contains higher number of megakaryocyte progenitors, and CCE could be an effective method for separating megakaryocyte progenitors essential for reconstituting platelet after HSCT. The cell dose of 5x106 was required for the effective recovery of platelet and the survival of BALB/c mice in syngeneic bone marrow transplantation with R/O cells.