Anaesthetic management of a paediatric patient with congenital fibre type disproportion myopathy. / Tratamiento anestésico en una paciente pediátrica con miopatía congénita por desproporción del tipo de fibras.

Congenital fibre type disproportion (CFTD) is a rare type of myopathy that is characterised by muscle weakness and hypotonia during childhood. Clinical features include motor delay, feeding difficulties, limb weakness, joint contractures, and scoliosis. A report is presented of the anaesthetic management of a 3-year-old girl with CFTD myopathy associated with a mutation of the TPM3 gene, scheduled for adenotonsillectomy because of obstructive sleep apnoea hypopnoea syndrome (OSAHS). The main concerns were the possible susceptibility to malignant hyperthermia, the risk of anaesthesia-induced rhabdomyolysis, a greater sensitivity to non-depolarising muscle relaxants, and the presence of OSAHS. Total intravenous anaesthesia with propofol and the use of rocuronium/sugammadex appear to be safe options. Given the high risk of respiratory compromise and other complications, patients should be closely monitored in the post-operative period.

Descriptive study of ultrasound images of the upper airway obtained after insertion of laryngeal mask. / Imágenes ultrasonográficas de vía aérea superior tras la inserción de mascarilla laríngea: estudio descriptivo y utilidad clínica.

OBJECTIVE: To evaluate clinical usefulness of ultrasound images of the upper airway in order to check correct laryngeal mask placement. MATERIAL AND METHODS: A prospective observational study was conducted on patients scheduled for abdominal surgery under general anaesthesia, in whom the patency of the upper airway was ensured using an Ambu®AuraGainTM laryngeal mask. An ultrasound scan was performed of the upper-airway in the cranio-caudal direction and with longitudinal scans in the anterior midline and parasagittal axis, in three moments: before, after inserting and after removing the mask. All recorded images were evaluated in a second time by a radiologist-expert in upper airway ultrasound. Subsequently, the ultrasound data were related to the clinical difficulty of the insertion and presence of air leaks. RESULTS: Data was collected from 30 patients (20 females and 10 males) being operated on for abdominal hysterectomy (15), eventroplasty (6), uterine myomectomy (3), and umbilical (4) and inguinal herniorrhaphy (2). The blind insertion of the masks did not present difficulties in 24 (80%) patients. Air leakage was detected in 8 (26.7%) patients, which was moderate in 7 cases and severe in one of them. The ultrasound findings confirmed good mask placement in 22 (73.3%) patients. Anatomical airway changes after laryngeal mask extraction were only observed in 3 (12%) patients, all of them minor. There was a statistically significant association (P<.05) between difficulty in inserting the device and the level of air leakage. CONCLUSIONS: Upper airway ultrasound is a useful diagnostic method to evaluate laryngeal mask placement. Laryngeal oedema was not observed after removal of the device.

Treatment of chronic he1patitis C genotype 1 with peginterferon-alpha2a (40 kDa) plus ribavirin under routine clinical practice in Spain: early prediction of sustained virological response rate.

BACKGROUND: Sustained virological response rates of up to 52% have been obtained with peginterferon alpha2a (40 kDa) plus ribavirin in patients suffering from chronic hepatitis C genotype 1 in randomized-controlled trials. AIM: To assess early virological response and its clinical utility in predicting an sustained virological response in patients suffering from chronic hepatitis C genotype 1 in routine clinical practice in Spain. METHODS: Treatment-naïve patients received pegylated interferon alpha2a (40 kDa) 180 microg/week plus ribavirin 1000/1200 mg/day for 48 weeks, and were followed for a further 24 weeks. Overall, 475 patients received at least one dose of medication and were included in the efficacy population. RESULTS: The overall sustained virological response rate was 48%. Of those with week 12 virological data, 83% had an early virological response. The negative predictive value of an early virological response was 93%. CONCLUSION: If sustained virological response is the goal, a treatment-decision based on a 12-week evaluation during routine clinical practice is feasible.

[Angioma-like liver lesions in patients with chronic liver disease]. / Lesiones hepáticas sugestivas de angioma en pacientes con hepatopatía crónica.

OBJECTIVE: The aim of this study was to evaluate in our healthcare area the clinical, ultrasonographic, and evolutionary features of patients with chronic liver disease and angioma-like liver lesions on ultrasonography. MATERIALS AND METHODS: We conducted a retrospective study amongst patients seen at the Ultrasonography Unit, Gastroenterology Department between January 2000 and June 2004. Included in the study were patients that presented with clinical and/or laboratory complaints consistent with chronic liver disease of any etiology, and those in which abdominal ultrasounds revealed the existence of at least one angioma-like liver lesion. All relevant epidemiological, clinical, ultrasonographic, and evolutionary data were carefully collected and recorded. RESULTS: In the course of our study, 58 patients were diagnosed with chronic liver disease and angioma-like liver lesions, of which 13 showed clinical, laboratory, ultrasonographic, and/or histological signs of liver cirrhosis. In 50% of patients these lesions were less than 10 mm in diameter, and in most cases were located in the right hepatic lobe. During an average follow-up period of 35 months (6-168 months) we verified that, in two patients, these lesions, initially interpreted as angiomas were in fact malignancies (one hepatocellular carcinoma and one metastatic adenocarcinoma of the gallbladder). In both cases, the patients were cirrhotic. Thus, our study revealed that 15% of lesions found in cirrhotic patients initially interpreted as angiomas were actually malignant. CONCLUSIONS: Our study revealed that, in patients with chronic liver disease, particularly in cirrhotic patients, a considerable percentage of ultrasonographic lesions originally interpreted as angiomas are in fact malignant tumors.

[Treatment of hepatic and intra-abdominal hydatidosis with mebendazole: prospective comparison of the cyclic and the continuous regimen]. / Tratamiento de la hidatidosis hepática e intraabdominal con mebendazol: comparación prospectiva de las pautas cíclica y contínua.

AIM: To communicate the results of two different schedules (cyclic and continuous) of mebendazole therapy for hepatic and intraabdominal hydatid disease. DESIGN: Prospective and randomized. PATIENTS: 26 cases of abdominal hydatid disease. RESULTS: No significant differences were registered between both groups. CONCLUSION: Cyclic mebendazole therapy does not improve the results of continuous treatment in abdominal hydatid disease.

[Solitary ulcer of the rectum and granulomatous appendicitis, a casual association?]. / Ulcera solitaria de recto y apendicitis granulomatosa, una asociación casual?

We report a case of solitary rectal ulcer (SRU) in a patient who had been previously diagnosed of granulomatous appendicitis. Both entities were histologically confirmed. The patient is asymptomatic after appendectomy done one and a half years ago, demonstrating the low recurrence rate of granulomatous appendicitis and the silent course of solitary rectal ulcer in many patients. Both entities may be related.

[Recombinant alfa-2 interferon treatment of a patient with chronic hepatitis B and ulcerative colitis]. / Tratamiento con interferón alfa-2 recombinante en un paciente con hepatitis crónica por virus B y colitis ulcerosa.

The case of a patient ulcerative colitis involving an autoimmune base who was treated with recombinant alpha-2 interferon for concomitantly presenting chronic hepatitis B in the replicative phase is reported. With this therapy unreplicative seroconversion of the disease was achieved without modification of the course of the chronic inflammatory intestinal disease (CIID) during treatment or over the posttreatment follow up period. A brief review of the literature was performed concerning the role of autoimmunity in ulcerative colitis, treatment with alpha-interferon in chronic hepatitis B and the exacerbation of autoimmune phenomena which may lead to interferon treatment. According to the evolution of this case and the review of the literature, the authors conclude that the existence of CIID does not contra-indicate the use of recombinant alpha-2 interferon in patients with chronic viral hepatitis, although special control of the disease should be carried out during the treatment period.

[Hepatic granulomas in a patient with acquired hypogammaglobulinemia]. / Granulomas hepáticos en una paciente con hipogammaglobulinemia adquirida.

The case of a young female diagnosed with an IgG deficit and hepatic granulomas is presented. After 6 years of follow up the patient remains asymptomatic without treatment and with moderate biochemical cholestasis. The tests performed did not lead to a definitive etiologic diagnosis, therefore, according to a review of the literature, we believe that the granulomatous reactions are related with the hypogammaglobulinemia itself.

[Is endoscopic retrograde cholangiopancreatography useful in the diagnosis of cholangiocarcinoma?]. / Es útil la colangiopancreatografía retrógrada endoscópica en el diagnóstico del colangiocarcinoma?

We have been interested in demonstrating the usefulness of the reverse endoscopic cholangiopancreatography, a technique already accepted in extrahepatic biliary pathology and the cholangiocarcinoma diagnosis. First of all we reviewed those cases diagnosed as cholangiocarcinoma using the REC and we found that only in 14 (60.9%) of a total of 23 cases the diagnosis was confirmed by surgery, resulting in a false positive ratio of 39.1%. In the second analysis of 22 patients who underwent surgery and whose surgical diagnosis was cholangiocarcinoma, we confirmed a correct presurgery diagnosis by REC in 14 (63.6%) with a false negative ratio of 36.3% which could be reduced to 26.3% after excluding the technically incorrect explorations or those in which only the pancreatic ductus was visualized. We conclude that cholangiocarcinoma diagnosis by REC has a high false positive or negative ratio, thus, treatment, specially non surgical, of a possibly malignant stenosis or obstruction of the bile duct can not only be based on choleangiopatic findings.

Solitary gastric hamartomatous polyp and upper gastrointestinal haemorrhage: an exceptional presentation of an unusual diagnosis.

A case of solitary gastric hamartomatous polyp, presenting as upper gastrointestinal haemorrhage, is reported. Both the entity itself, and the clinical presentation, are exceptional. The significance of the solitary gastric hamartomatous polyp and implications of its diagnosis are discussed.

Randomised controlled trial of efficacy of albendazole in intra-abdominal hydatid disease.

The efficacy of albendazole in hydatid disease is still unclear, because there has been no study that assessed the status of the parasite after treatment. The significance of albendazole-induced echographic changes in the cyst therefore cannot be judged. We did a prospective, controlled, randomised, open study of albendazole in patients with liver hydatid disease, and assessed parasite viability after treatment. 18 patients received no albendazole treatment (controls), 18 received albendazole (10 mg/kg daily) for 1 month (group A), and 19 received the drug for about 3 months (group B). Echography was done before and during treatment; all patients underwent surgery on completion. Parasite (protoscolex viability and development of cysts in mice) and ultrastructure studies were done for all cysts removed. 8 (50%) of cysts in the control group, 13 (72%) in group A, and 16 (94%) in group B were non-viable (p = 0.015). Protoscolex and cyst viability were significantly (p = 0.039 and p = 0.018, respectively) lower in treated patients than in controls. Treatment was also significantly associated with total cyst membrane disintegration. 68% of cysts treated for 3 months showed echographic changes, and only 1 of 20 cysts showing echographic changes during treatment was judged viable. The efficacy of albendazole at a dose of 10 mg/kg daily for 3 months suggests that it is a suitable alternative to surgery in uncomplicated hydatid liver disease, as initial treatment.

[Mesenteric venous thrombosis: a new case of favorable course using conservative treatment]. / Trombosis venosa mesentérica: un nuevo caso de evolución favorable con tratamiento conservador.

Mesenteric venous thrombosis (TVM) is an uncommon entity with a mortality without surgical treatment of virtually 100%. However, recently some cases have been reported of a good evolution with conservative treatment. We present a patient with mesenteric venous thrombosis diagnosed by arteriography who, after refusing surgical intervention, underwent parenteral nutrition and anticoagulant treatment. This case constitutes another proof that mesenteric venous thrombosis is not invariably fatal without surgical treatment.

[Intestinal angiodysplasia: a presentation of 7 cases and review of the literature]. / Angiodisplasia intestinal: presentación de siete casos y revisión de la literatura.

Seven cases of intestinal angiodysplasia that required surgery for hemostatic control are presented, with a review of their clinical, endoscopic, arteriographic and histologic features. The average age of presentation was 52.43 years, there was a predominance in women, and the principal clinical manifestation was recurrent rectal hemorrhage. The most common location was the cecum and ascendant colon. Arteriographic diagnosis was achieved in only two patients.

[Solitary ulcer of the rectum]. / Ulcera solitaria del recto.

In our center solitary ulcer of the rectum has a low incidence. There has been only one case in more than 13,000 distal endoscopic studies performed in the last ten years. Its rarity, and its possible confusion with other pathologies of the distal colon motivated our interest in this disease. The clinical and diagnostic features and therapeutic attitude in solitary rectal ulcer are remarked.