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51.
The sarcolemmal membrane of mammalian ventricular cardiomyocytes are characterised by the presence of invaginations called transverse tubules (T-tubules) which constitute a network, the transverse tubule system. T-tubules occur at the Z line as transverse elements with also longitudinal extensions. While the existence of T-tubules has been known since a long time, recent studies have suggested their structure and function can be more complex than previously believed. Many of the proteins involved in excitation-contraction coupling process are concentrated within the T-tubular network, suggesting T-tubules are a highly specialized membrane system. Thus, T-tubules are a key determinant of cardiac cell function. The fundamental role of T-tubules is emphasized by changes in their structure and protein expression occurring during pathologies such as cardiac hypertrophy and heart failure. This review summarizes recent studies which highlight the key-role of the T-tubules in the regulation of cardiac function. Changes observed in pathological conditions are also discussed.  相似文献   
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Acute generalized exanthematous pustulosis (AGEP) is a severe adverse cutaneous reaction, which is mostly due to drugs, but which has also been described as occurring after infections. We report a case of severe AGEP with extensive blistering mimicking toxic epidermal necrolysis (TEN) in a 47‐year‐old woman. This was associated with a life‐threatening primary mumps infection, complicated by perimyocarditis and encephalitis. The recent increase in the incidence of mumps should lead physicians to be aware of the uncommon clinical features and complications of this disease.  相似文献   
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Levamisole (an increasingly frequent contaminant of cocaine) can cause antineutrophil cytoplasmic antibody‐associated vasculitis. Dermatologists should consider a diagnosis of cocaine/levamisole‐associated cutaneous vasculopathy syndrome in cases of purpura of the ears and/or extensive retiform purpura in drug users. We report a case of particularly severe levamisole‐induced necrotic purpura and immunological abnormalities in a 40‐year‐old woman.  相似文献   
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Background  Patients undergoing bariatric surgery with a gastric bypass lose about 66% of excess weight. Although this procedure induces weight loss, it is unknown whether it leads to protein malnutrition, which is studied here. Methods  One hundred ten obese patients (body mass index, 47.9 ± 8.6 kg/m2) undergoing gastric bypass had a measurement of plasma albumin and transthyretin (formerly prealbumin) and a calculation of nutritional risk index (NRI) before and throughout the 2 years following the surgery. Results  All but five patients lost more than 15% of initial weight; the mean loss of excess weight was 65.2 ± 26.4% at 2 years. Plasma concentrations of albumin and transthyretin decreased after surgery, but while albumin returned to initial values after 12 months, transthyretin remained low. Only one patient had an albumin below 30 g/l; another one had a transthyretin lower than 110 mg/l. All NRI scores were lower than 83.5 (62 ± 5, ranging 44–70), qualifying patients for severe malnutrition. Conclusion  Malnutrition is difficult to diagnose in obese patients undergoing surgery. The large weight loss is most often not associated with protein malnutrition. Whether gastric bypass induces protein malnutrition remains to be established.  相似文献   
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Background:

Oesophageal atresia is a neonatal emergency surgery whose prognosis has improved significantly in industrialised countries in recent decades. In sub-Saharan Africa, this malformation is still responsible for a high morbidity and mortality. The objective of this study was to analyse the diagnostic difficulties and its impact on the prognosis of this malformation in our work environment.

Patients and Methods:

We conducted a retrospective study over 4 years on 49 patients diagnosed with esophageal atresia in the 2 Paediatric Surgery Departments in Dakar.

Results:

The average age was 4 days (0-10 days), 50% of them had a severe pneumonopathy. The average time of surgical management was 27 h (6-96 h). In the series, we noted 10 preoperative deaths. The average age at surgery was 5.7 days with a range of 1-18 days. The surgery mortality rate is 28 patients (72%) including 4 late deaths.

Conclusion:

The causes of death were mainly sepsis, cardiac decompensation and anastomotic leaks.Key words: Esophageal atresia, Diagnosis, Prognosis, Sub-Saharan Africa  相似文献   
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Diverticula of the right colon are a rare clinical entity. Diverticulitis can occur with these lesions, but the diagnosis is frequently missed as the presentation is similar to that of acute appendicitis and can mimic other acute intraabdominal infections such as cholecystitis. The authors report a case of solitary diverticulitis of the right colon in a young woman, an incidental discovery during surgery for suspected appendicitis, with emphasis on the diagnostic and therapeutic modalities.  相似文献   
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