Pancreatitis crónica: análisis de factores de progresión de la enfermedad / Chronic pancreatitis: analysis of disease progression factors

BACKGROUND: Alcohol and tobacco are important risk factors for chronic pancreatitis (CP). AIM: To analyze the effect of etiological factors such as tobacco and alcohol and pancreatic enzyme replacement therapy (PERT) in the progression of CP. MATERIAL AND METHODS: Patients with a diagnosis of CP were recruited and grouped according to variables such as tobacco, alcohol and PERT. They were followed for 18 months. Subsequently, different variables and analytical parameters involved in the progression of the disease were analyzed. RESULTS: A total of 50 patients diagnosed with CP were included. Of these, 28 patients underwent PERT, 39 were smokers and 33 were alcohol users. Compared with patients without PERT, those with PERT had a higher proportion of diabetes (64 and 32%, respectively), had a higher need for endoscopic treatment (25 and 0%, respectively) and a normal body mass index (71 and 27.3%, respectively. The smokers had higher calcium levels and increased lymphocytosis and leukocytosis. The alcohol consumption group had a higher mean age (p = 0.04) CONCLUSIONS: PERT may improve the nutritional status but does not reduce the need for endoscopic or surgical treatment. Smoking and alcohol consumption favored the progression of CP. Also, smoking induced a pro-inflammatory state.

Enfermedad de Fabry clásica en pacientes pediátricos asintomáticos: aspectos bioéticos de la terapia enzimática / Classic Fabry disease in asymptomatic pediatric patients: Bioethical aspects of enzyme therapy / Doença de Fabry clássica em pacientes pediátricos assintomáticos: aspectos bioéticos da terapia enzimática

Resumen: El propósito de este artículo es proponer un marco bioético para la administración de la terapia enzimática en niños con enfermedad de Fabry asintomáticos. Se realizó un estudio de campo transversal, observacional, descriptivo, con análisis cuantitativo y cualitativo, a la luz de la bioética en médicos que han diagnosticado y tratado pacientes con la enfermedad a nivel nacional e internacional, mediante una encuesta con preguntas cerradas validada por juicio de expertos, se evaluó la aplicación de los principios de no maleficencia, justicia y sacralidad de la vida en la administración del tratamiento enzimático para estos pacientes. La información se agrupó y procesó con estadística descriptiva. El 83,33 % de los encuestados consideró que un niño con enfermedad de Fabry asintomático debe recibir tratamiento enzimático específico, independientemente de su costo (justicia), además, que el tratamiento debe ser financiado por el Estado a fin de prevenir las complicaciones de la enfermedad; esto fue reconocido por el 75 % de los médicos encuestados (no maleficencia). El 66,66 % consideró que la sacralidad de la vida está en función de prevenir las complicaciones que condicionan la muerte. Así, con base en la bioética principialista se justifica iniciar la terapia enzimática específica para el tratamiento de niños con enfermedad de Fabry asintomáticos.

Abstract: This paper aims to propose a bioethical framework for the administration of enzyme therapy in children with asymptomatic Fabry disease. A cross-sectional, observational, descriptive field study was conducted with quantitative and qualitative analysis, in the light of bioethics with physicians who have diagnosed and treated patients with classic Fabry disease at the local and international level, using a survey with closed questions validated by expert judgment. The application of the principles of non-maleficence, justice, and sacredness of life in the administration of enzymatic treatment for these patients was evaluated. The information was grouped and processed with descriptive statistics. 83,33 % of respondents considered that a child with asymptomatic Fabry disease should receive specific enzymatic treatment, regardless of its cost (justice), and that treatment should be funded by the State in order to prevent complications of the disease. This was recognized by 75% of the physicians surveyed (not maleficence). 66,66 % considered that the sacredness of life must prevent the complications that condition death. Thus, based on the principles of bioethics, initiating specific enzymatic therapy for the treatment of children with asymptomatic Fabry disease is sustained.

Resumo: o propósito deste artigo é propor um referencial bioético para administrar a terapia enzimática em crianças com doença de Fabry assintomáticos. Foi realizado um estudo de campo transversal, observacional, descritivo, com análise quantitativa e qualitativa, à luz da bioética em médicos que diagnosticaram e trataram pacientes com a doença no contexto nacional e internacional, mediante questionário com perguntas fechadas validada porjulga- mento de especialistas; foi avaliada a aplicação dos princípios de não maleficência, justiça e sacralidade da vida na administração do tratamento enzimático para esses pacientes. A informação foi agrupada e processada com estatística descritiva. 83,33 % dos pesquisados consideraram que uma criança com doença de Fabry assintomática deve receber tratamento enzimático específico, independentemente de seu custo (justiça), além disso que o tratamento deve ser financiado pelo Estado para prevenir as complicações da doença. Isso foi reconhecido por 75 % dos médicos investigados (não maleficência). 66,66 % consideraram que a sacralidade da vida está em função de prevenir as complicações que condicionam a morte. Assim, com base na bioética principialista, justifica-se iniciar a terapia enzimática específica para tratar crianças com doença de Fabry assintomáticas.

A Randomized, Placebo-controlled Trial of Digestive Enzymes in Children with Autism Spectrum Disorders

OBJECTIVE: There is growing evidence for a gut-brain connection associated with autism spectrum disorders (ASDs). This suggests a potential benefit from introduced digestive enzymes for children with ASD. METHODS: We performed a double-blind, randomized clinical trial on 101 children with ASD (82 boys and 19 girls) aged from 3 to 9 years. ASD patients were diagnosed according to the Diagnostic and Statistical Manual of Mental Disorders 4th edition, text revision (DSM-IV-TR) diagnostic criteria. Structured interviews of at least one hour each both with the parents and the child were performed. Later on, another two hours-session was conducted applying the Childhood Autism Rating Scale (CARS). ASD patients were randomized to receive digestive enzymes or placebo. RESULTS: The ASD group receiving digestive enzyme therapy for 3 months had significant improvement in emotional response, general impression autistic score, general behavior and gastrointestinal symptoms. Our study demonstrated the usefulness of digestive enzyme in our population of ASD patients. CONCLUSION: Digestive enzymes are inexpensive, readily available, have an excellent safety profile, and have mildly beneficial effects in ASD patients. Depending on the parameter measured in our study, we propose digestive enzymes for managing symptoms of ASD. Digestive enzyme therapy may be a possible option in treatment protocols for ASD in the future.

Ativação do estresse oxidativo e da resposta antioxidante por ExoU de Pseudomonas aeruginosa / Activation of oxidative stress and antioxidant response by ExoU of Pseudomonas aeruginosa

ExoU, uma citotoxina produzida pelo patógeno oportunista Pseudomonas aeruginosa e translocada para o citosol de células hospedeiras via sistema de secreção do tipo III, é associada à gravidade de infecções agudas. No presente trabalho, o efeito de ExoU na ativação do estresse oxidativo e da resposta antioxidante foi avaliado em culturas de células epiteliais respiratórias humanas infectadas com a cepa PA103 de P. aeruginosa (produtora de ExoU), com a mutante deletada no gene exoU, PA103∆exoU, ou com a mutante complementada com exoU sem atividade tipo fosfolipase A2, PA103∆UT/S142A. Análises das dosagens de hidroperóxidos lipídicos e isoprostanos, considerados marcadores de estresse oxidativo, revelaram que ExoU promoveu um aumento em suas concentrações. Foi observado, também, que ExoU estimulou a produção de espécies reativas de oxigênio, óxido nítrico e peroxinitrito nas células infectadas, assim como a expressão de iNOS e eNOS, mas não de nNOS. Além disso, ExoU foi responsável pelo aumento da atividade de SOD1 e pela redução dos níveis de GSH, mas não afetou a atividade da catalase ou de NQO1. No modelo in vivo, a dosagem de malondialdeído, um subproduto da lipoperoxidação de membranas, evidenciou uma maior produção deste composto no pulmão de camundongos infectados pela cepa produtora de ExoU, em comparação ao pulmão de camundongos infectados pela cepa mutante. Em conjunto, estes resultados mostram que ExoU ativa a produção de espécies reativas de oxigênio e nitrogênio, levando à peroxidação lipídica e modulando o sistema de defesa antioxidante...

ExoU, a cytotoxin produced by the opportunistic pathogen Pseudomonas aeruginosa and translocated into the cytosol of host cells via a type III secretion system, is associated with severity of acute infections. In the present work, the effect of ExoU in the activation of oxidative stress and antioxidant response was evaluated in cultures of human respiratory epithelial cells infected with P. aeruginosa PA103 strain (producer of ExoU), or with its isogenic mutants, the ExoU-deficient PA103∆exoU or the exoU-depleted mutant complemented with an exoU gene with a site-specific mutation in the PLA2 catalytic site, PA103∆UT/S142A. Analysis of dosages of lipid hydroperoxides and isoprostanes, considered markers of oxidative stress, revealed that ExoU promoted an increase in their concentrations. It was also observed that ExoU stimulated the production of reactive oxygen species, nitric oxide and peroxynitrite in infected cells, as well as the expression of iNOS and eNOS, but not nNOS. Furthermore, ExoU was responsible for the increased activity of SOD1 and the reduced levels of GSH, but did not affect the activity of catalase or NQO1. In the in vivo model, the analysis of malondialdehyde, a byproduct of lipid peroxidation of membranes, showed increased production of this compound in the lungs of mice infected with the ExoU-producing strain compared to the lungs of mice infected with the mutant strain. Together, these results show that ExoU activates the production of reactive oxygen and nitrogen species, leading to lipid peroxidation and modulating the antioxidant defense system...

Advances in superenzyme gene therapy in penile rehabilitation / 中华男科学杂志

Erectile dysfunction (ED) is an almost unavoidable complication of radical prostatectomy. At present, though the concept of penile rehabilitation (PR) is accepted by most clinicians, the outcomes of erectile function recovery vary widely. Prostacyclin (PGI2) is a prostanoid and a main vasoprotectant which induces smooth muscle relaxation, but not used for replacement therapy because of its high unstability. SuperEnzyme is capable of continuous, specific and targeted promotion of PGI2 synthesis, and helps PR in ED patients after radical prostatectomy. SuperEnzyme gene therapy has a promising prospect for PR and the management of ED. This review updates SuperEnzyme gene therapy in PR.

Pancreatitis crónica: nuevas terapias / Chronic pancreatitis: new therapies

Chronic pancreatitis occurs by the prolonged inflammation of pancreatic tissue that induces the irreversible destruction of the organ, leading to a global pancreatic insufficiency. The most common manifestations are abdominal pain, diarrhea, malabsorption, and possibly diabetes mellitus. Chronic pancreatitis treatment includes dietary restrictions, enzymatic supplementation, vitamins, and endoscopic or surgical methods depending on the degree of ductal involvement. In addition to the known therapies, new therapies are under development and research.

La pancreatitis crónica se desarrolla por la inflamación prolongada del tejido pancreático que induce la destrucción irreversible del órgano, llevando a una insuficiencia pancreática global. Las manifestaciones más frecuentes son dolor abdominal, diarrea, malabsorción y eventualmente diabetes mellitus. El tratamiento en pancreatitis crónica incluye restricciones dietarias, suplementación enzimática, vitamínica, y métodos endoscópicos o quirúrgicos, dependiendo del grado de compromiso ductal. Además de lo descrito, están en desarrollo y experimentación nuevas terapias.

Laparoscopic Surgery for Removal of the Multiple Large Gastric Bezoars

Bezoars are retained concretions of undigested animal or vegetable material that can produce gastrointestinal obstruction, ulceration, and bleeding. Therapeutic options for gastric bezoars include enzyme therapy (papain, cellulase, or acetylcysteine), endoscopic disruption and removal, and surgical removal. Multiple large gastric bezoars generally require conventional surgical management through an upper abdominal incision. With the recent improvement of laparoscopy, a lot of portions of abdominal operations have been performed laparoscopically. We successfully removed multiple large gastric phytobezoars in a 52-year-old female completely through laparoscopy. This supported the feasibility of laparoscopic surgery for patients with gastric bezoars.

A Case of Thromboembolism Associated with Central Venous Catheter / 대한소아혈액종양학회지

The central venous catheter(Quinton) was replaced in the right atrium for chemotherapy and blood sampling to a eight-year old girl with acute lymphoblastic leukemia. The catheter was flushed with heparin two times daily and the chemotherapeutic drug regimens by CCSG-105 protocol were vincristine, prednisone, L-asparaginase, daunomycin, methotrexate, cyclophosphamide, cytosine arabinoside, 6-mercaptopurine and adriamycin. On day 31 of catheterization, the lumen for blood sampling was blocked. From day 60 of catheterization, anorexia, nausea, vomiting, abdominal distension and tachypnea were developed. Echocardiogram, lung scan, pulmonary function test(PFT), arterial blood gas analysis (ABGA) were done. Two cemtimeters in diameter of thrombosis was found in the right atrium on echocardiogram. The lung scan showed slight decrease in uptake of Tc-99m on the whole lung fields. Restrictive ventratory impairment on PFT and decreased PaO2(48 mmHg) on ABGA were found. To lysis of thromboembolism, urokinase(4,400 IU/kg for initial 10 minutes and the 4,400 IU/kg for 12 hours) was injected intravenously and aspirin(30 mg/kg/d, po) was given. The thrombosis was disappeared from the atrium on echocardiogram and PaO2 was increased up to 97 mmHg temporary. The catheter was removed but total haziness on the right middle and lower lobes were developed after 5 days. The same dose of urokinase was injected just infront of the embolism through Pitfall catheter for 8 days intermittently as results of pulmonary angiography. Symptoms and PaO2 were alleviated and the lung was expanded with mild atelectasis on day 55 of the first fibrinolytic enzyme therapy. Although the frequent complications of central venous catheter are catheter occlusion and infection, we have to check the development of thromboembolism with echocardiogram periodically or in case of unexplained respiratory symptoms.