Neuropathy associated with “benign” anti-myelin-associated glycoprotein IgM gammopathy: Clinical,immunological, neurophysiological pathological findings and response to treatment in 33 cases

We studied 33 patients presenting with a peripheral neuropathy associated with non-malignant anti-myelin-associated glycoprotein (MAG) IgM monoclonal gammopathy (MG) in an attempt to delineate their clinical, immunological, electrophysiological and pathological characteristics; we also reviewed our experience concerning long-term follow-up and therapy. Peripheral neuropathy associated with non-malignant anti-MAG IgM MG was observed mostly in males (sex ratio 7.2), and mean age at onset was 67 years (range 46–81). A predominantly sensory pattern was noted in more than 80% of cases, although some patients were affected by a predominantly motor peripheral neuropathy. Although disease progression was slow in most cases, 45% of patients suffered severe disability, and in 2 cases, the patient's death appeared to stem directly from the neuropathy. The electrophysiological findings were indicative of a demyelinating process in 90% of cases, and electron microscopic examination of nerve biopsy specimens demonstrated widening of the myelin lamellae in more than 95% of cases. Most of our patients showed a disappointing response to steroids and chemotherapy or plasma exchanges. Intravenous immune globulin, evaluated in 17 patients, had a transient, mostly subjective effect in 35% and led to a clear-cut improvement in 24% of cases. We did not observe any correlation between the severity of the clinical picture and the anti-sulphoglucuronyl paragloboside antibody titre; in individual cases, clinical improvement occurred without lowering of IgM levels. Although the severity and the rate of progression may greatly vary from patient to patient, the combination of clinical, electrophysiological and pathological features delineates a characteristic pattern in peripheral neuropathy associated with non-malignant anti-MAG IgM MG.     

Is there a place for gastroenterostomy in patients with advanced cancer of the head of the pancreas?

There remains doubt about the need for gastroenterostomy in patients with advanced cancer of the pancreatic head, performed either prophylactically or when passage of food becomes impossible. The records of 142 patients admitted for advanced pancreatic cancer to the Erasmus University Hospital over a period of 11 years were reviewed. We concentrated especially on the pre- and postoperative intake of food in cases involving gastroenterostomy and the morbidity and mortality associated with abdominal surgery in these patients. Of 129 patients without symptoms of gastric outlet obstruction at the time of diagnosis, 31 underwent prophylactic gastroenterostomy. The procedure did not prevent gastric outlet obstruction in 4 patients. Of the remaining 98 patients, 15 developed gastric outlet obstruction. Cox proportional hazards analysis showed no significant difference in the interval to the occurrence of a symptomatic obstruction between these two groups, taking into account other covariables. Postoperative complications and mortality regarding a gastroenterostomy were high, ranging from 9% to 41% and 11% to 33%, respectively. Our results do not indicate that prophylactic gastroenterostomy may significantly prevent future gastric outlet obstruction; therefore, as it also increases morbidity, it should not be performed. A gastroenterostomy to relieve symptoms should be considered carefully, as the success rate is low and is accompanied by a considerable incidence of morbidity and mortality.

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Resumen Persiste la duda sobre la necesidad de practicar gastroenterostomía en pacientes con cáncer avanzado de la cabeza del páncreas, así sea profiláctica o en presencia de obstrucción al paso de los alimentos. Se revisaron las historias de 142 pacientes con cáncer avanzado de la cabeza del páncreas en el Hospital de la Universidad de Erasmo observados en un periodo de 11 años. El estudio se concentré especialmente sobre la ingesta pre y postoperatoria de alimentos en los pacientos con gastroenterostomía y en la morbilidad y mortalidad asociada con la cirugía abdominal. De 129 pacientes libres de síntomas de obstrucción en el momento del diagnóstico, 31 fueron sometidos a gastroenterostomía profiláctica; el procedimiento no logró prevenir la obstrucción gástrica en 4 casos. De los 98 pacientes restantes, 15 desarrollaron obstrucción gástrico. El análisis proporcional de Cox no demostró diferencia significativa en el intervalo transcurrido hasta la aparición de los sintomas entre los dos grupos, tomando en consideración diversas variables. Las tasas de complicaciones y de mortalidad postoperatoria en relación con la gastroenterostomía fueron elevadas, 9–41% y 11–33%, respectivamente. Nuestros resultados no indican que la gastroenterostomía profiláctica pueda prevenir la obstrucción gástrica y, por cuanto incrementa la morbilidad, no debe ser realizada. La gastroenterostomía por razones de sintomatologia debe ser cuidadosamente considerada, puesto que la tasa de éxito es baja y se acompana de considerable morbilidad y mortalidad.

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Résumé Réaliser une gastroentérostomie de faÇon prophylactique ou seulement lorsque l'alimentation devient impossible chez un patient ayant un cancer de la tÊte du pancréas reste une question sans réponse. Les dossiers de 142 patients ayant un cancer avancé de la tÊte du pancréas, observés à l'HÔpital Universitaire Erasmus en l'espace de 11 ans, ont été revus. Nous avons noté la possibilité d'alimentation en périodes préet postopératoire ainsi que la morbidité et mortalité en rapport avec la chirurgie chez ces patients. Des 129 patients n'ayant pas de symptÔmes d'obstruction postpylorique au moment du diagnostic, 31 ont eu une gastroentérostomie à titre prophylactique. Cette intervention n'a pu prévenir l'obstruction chez 4 de ces patients. Des 98 autres patients, 15 ont développé une obstruction postpylorique. Une analyse multifactorielle selon le modèle de Cox n'a pu démontrer de différence significative entre les deux groupes pour l'intervalle entre le moment du diagnostic et la survenue de l'obstruction. Le taux de complications et de décès postopératoires après gastroentérostomie était élevée, variant respectivement entre 9% et 41% et 11% et 33%. Nos résultats indiquent que la gastroentérostomie à titre prophylactique ne prévient pas la survenue d'une obstruction postpylorique mais qu'elle accroÎt la morbidité. Dans ces conditions, la gastroentérostomie ne devrait Être réalisée qu'en cas d'obstruction symptomatique, mais en sachant qu'elle n'est pas toujours couronnée de succès et que les taux de mortalité et de morbidité ne sont pas nuls.

     

Comparison Between Incremental and Single-Dose Allergen Administration in Bronchial Provocation Tests

To study the effect of different schedules of allergen administration on the early (EAR) and late allergic reaction (LAR), 15 asthmatic patients with dual response after allergen challenge were submitted to two consecutive bronchoprovocation tests (BPT). BPT₁ was carried out with semilogarithmic increase of allergen doses until a FEV₁ drop of 20% was recorded. BPT₂ was performed 2 weeks later using the highest allergen dose that had elicited the dual reaction in BPT₁. Both EAR and LAR were very similar for a given patient. As the allergen dose used in BPT₂ is smaller than the cumulative allergen dose administered in BPT₁, this protocol takes into account a possible influence on BPT₂ of bronchial hyperreactivity induced by BPT₁ performed 2 weeks before.     

Laparoscopic hysterectomy

Summary Thirty-three patients were selected for laparoscopic hysterectomy and operated on in the Department of Obstetrics, Gynecology and Reproductive Medicine of Clermont-Ferrand University Hospital. Surgical techniques included blunt dissection with scissors and bipolar coagulation to achieve hemostasis. A case was considered successful when all the uterine vessels were treated by laparoscopy. Twenty-four cases were completed laparoscopically (72.7%). None of these patients had postoperative bleeding; 22 had an uneventful postoperative recovery. Nine procedures were converted to laparotomy (27.3%), five because of a difficult or unsatisfactory hemostasis. We conclude that in selected cases, a total hysterectomy can be performed safely by experienced laparoscopists. Further technological progress is necessary to make this procedure more acceptable. Its value as compared to the others will have to be demonstrated.     

Surgical treatment of stapes fixation

The aim of surgical treatment of a fixed stapes is to restore an as effective as possible impedance transfer of the ossicular chain and acoustic impedance of the annular ligament of the footplate, when it has lost elasticity, and this in order to achieve the best possible physiological vibration of the inner ear fluids.     

Hepatobiliary scintigraphy in a patient with bilhemia

A 4-year-old child referred for acute jaundice following percutaneous needle biopsy of the liver underwent hepatobiliary scintigraphy. Although all conventional liver tests suggested preservation of hepatocyte function, the tracer uptake in the liver appeared dramatically reduced at scintigraphy and the blood pool activity did not decrease significantly until the end of the study. Visualization of the bile ducts indicated, however, that the tracer was taken up by the hepatocyte and further excreted into the biliary tree. There was no tracer pooling in the biliary tree although no bowel activity was observed, even on delayed images. The association of persistent blood pool activity, bile duct visualization without tracer pooling, and nonvisualization of the bowel was caused by a continuous recirculation of the tracer from the biliary tree into the bloodstream. The presence of a biliovenous fistula was further proven by percutaneous transhepatic cholangiography performed 24 h later. Since 1975, only 16 cases of bilhemia have been reported. To the best of our knowledge the scintigraphic pattern of this rare but lifethreatening complication has not previously been reported.     

双联抗血小板疗法预防缺血再发

本文对应用双联抗血小板聚集药在预防缺血再发方面较应用单药的优势进行了探讨.动脉粥样硬化是一种隐袭性的全身性过程,可累及多处血管床,包括脑动脉、冠状动脉和周围动脉.粥样硬化斑块破裂是血小板活化、聚集和血栓形成进程中的刺激因素之一.因为该病是沿着整个脉管系统发展的,所以对有任何一种动脉粥样硬化临床表现的患者来说,其他血管床也极易受到侵犯.不同的血管病有着共同的易患危险因素.缺血性冠心病和脑血管病是美国三大常见死亡原因中的两种.阿司匹林在心肌梗死和卒中的二级预防方面的有效性已在众多的研究中得到证实.潘生丁在降低心肌梗死、卒中和血管性死亡方面并不比阿司匹林更好,但噻氯匹定和氯吡格雷却明显优于阿司匹林.目前正在进行评估联合应用抗血小板聚集药预防缺血再发的有效性的临床研究.体外试验和临床研究均支持联合应用两种机制不同的抗血小板聚集药的理论.双联应用抗血小板聚集疗法可增强对血小板聚集和血栓形成的抑制作用.联合应用抗血小板聚集药以不同的作用机制抑制血栓形成途径中的多个位点,有可能进一步改善患者远期临床转归.＃在预防缺血再发方面,应用双联抗血小板聚集药较应用单药可能更具优势.