Influence of portal hemodynamics on long-term survival of alcoholic cirrhotic patients after small-diameter portacaval H grafts

Estimating postoperative survival rates after portasystemic shunt procedures has concerned surgeons during the last 40 years. The relationship between survival and Child's classification has clearly demonstrated the importance of preoperative hepatic functional reserve. Maintaining hepatic portal perfusion has been proposed as an additional protective factor but has never been proved clinically. Our analysis of survival after partial shunting with small-diameter portacaval H grafts has shown that both hepatic functional reserve and postoperative portal perfusion correlate with postoperative survival in alcoholic patients, but the latter was a stronger correlate of long-term survival. A predictive model based on both factors has been described for estimating the overall survival rate of alcoholics after partial shunting with small-diameter portacaval H grafts.     

Reliability of the Otago photoscreener A study of a thousand cases

The Otago photoscreener is a 35 mm single lens reflex camera in which the flash light comes from a narrow ring around the outer margin of its lens. The margin is also the limiting aperture of the optic system and in the centre of the lens is a flickering fixation light. In a colour photograph taken at a distance of 66 cm from the face of the subject who is accurately focusing on and fixing the camera fixation light with both eyes, the fundus reflex in each pupil is very dark red and the corneal light reflexes are symmetrical. If either or both eyes are not appropriately focused or fixing, the fundus reflex is brighter and yellow or white. This article describes a prospective trial of the performance of the Otago photoscreener in a series of 1000 infants with actual or suspected amblyopia, refractive error or strabismus. In this study photoscreening showed a sensitivity of 94% and a specificity of 79%. Photoscreening also identified some cases of esotropia and of refractive error which were missed on clinical examination. In this series photoscreening passed as normal three children with mild to moderately severe amblyopia. This represents les than 1 % of the clinically abnormal children.     

Complex I deficiency is associated with 3243G:C mitochondrial DNA in osteosarcoma cell cybrids

143B.206 rho degrees cells were repopulated with mitochondria from a MELAS patient harbouring a mixture of 3243G:C and 3243A:T mitochondrial DNA. A number of biochemical assays were performed on selected cybrids with various proportions of the two types of mitochondrial DNA. These assays revealed a marked decrease in oxygen consumption with pyruvate, a complex I substrate, in cybrids containing 60% to 90% 3243G:C mitochondrial DNA. Moreover, these cybrids showed decreased synthesis of a number of polypeptides in a mitochondrial in vitro translation assay. A cybrid line with a very high level of 3243G:C mitochondrial DNA (95%) had additional deficiencies in complexes III and IV and there was a marked generalised decrease in mitochondrial translation in this cybrid. The observation of complex I deficiency is consistent with previously reported enzymatic measurements of muscle homogenates from MELAS patients with the 3243G:C mutation.      

Spontaneous intramural haematoma of the oesophagus: Radiologic recognition

Spontaneous intramural haematoma of the oesophagus (SIHO) is an uncommon disorder. It presents usually with acute chest pain followed by dysphagia. This condition may mimic spontaneous rupture of the oesophagus (Boerhaave Syndrome), dissection of the thoracic aorta or acute myocardial infarction. Hence early accurate confirmation of the diagnosis by radiology is vital for the appropriate acute management. The condition is frequently mistaken for acute myocardial infarction which may prompt inappropriate thrombolytic therapy. The appearances on contrast studies of oesophagus and on CT scanning are characteristic. Despite this, the diagnosis may easily be missed, if appropriate careful radiological technique is not used. This paper reports three consecutive cases of SIHO managed in one institution. These cases illustrate that early gastrografin contrast radiology followed by barium contrast radiology and if necessary by thoracic CT is diagnostic in all cases of this condition.     

'Juvenile' myasthenia gravis in early infancy

A previously well infant developed severe muscle weakness and hypotonia at 6 months of age. This was reversed by anticholinesterase medication. However, she had subsequent further weakness and died at 10 months after an acute respiratory arrest. The clinical pattern was that of the 'juvenile' form of myasthenia gravis rather than the 'congenital' forms which have previously been described in early infancy.     

Abdominal pain of unknown etiology

The influence of age, sex, duration of symptoms, and exploratory laparotomy on prognosis of adult patients with abdominal pain of unknown etiology was assessed in a retrospective review. Patients with incomplete diagnostic evaluation were excluded. Of the sixty-four patients studied, forty-six were female. Lack of improvement of symptoms in the follow-up period was 67 per cent in females and 22 per cent in males (p < 0.05). Younger patients tended to have higher improvement rates. Of the patients whose duration of symptoms was less than fourteen days, 65 per cent were improved and 25 per cent were subsequently diagnosed, as compared with 14 and 9 per cent, respectively, of those whose symptoms had been present for more than ninety days (p < 0.05). Laparotomy did not influence rate of improvement and established a diagnosis in only one of twenty-three patients explored. It is concluded that few of these patients are likely to benefit from laparotomy, especially adult females whose symptoms have been present for more than three months.     

Defective mitogen-activated protein kinase (ERK2) signaling in gastric mucosa of portal hypertensive rats: potential therapeutic implications.

Portal hypertensive (PHT) gastropathy is a frequent, serious complication of liver cirrhosis. PHT gastric mucosa has numerous abnormalities such as reduced mucosal potential differences, reduced surface oxygenation, and increased susceptibility to injury caused by alcohol, aspirin, and other noxious factors. Because such mucosal injury is initially mediated by oxygen free radicals, and because mitogen-activated protein (MAP) kinase (ERK2) protects against cellular stress and induces cell proliferation, we postulated that oxidative stress-induced ERK2 activation is defective in PHT gastric mucosa. Here we show that in PHT gastric mucosa, ERK2 activation by oxidative stress is impaired. This impairment is mediated by overexpression of MAP kinase phosphatase-1 (MKP-1), which results from the underlying and continual oxidative state associated with portal hypertension, and is ameliorated by inhibiting MKP-1. Furthermore, we found that supplementing vitamin E, a free radical scavenger, reduces the oxidative state in PHT gastric mucosa, normalizes MKP-1 expression, and thereby reverses impairment of oxidative stress-induced ERK2 activation. Finally, we show that orally administered vitamin E completely reverses the increased susceptibility of PHT gastric mucosa to alcohol injury. Our findings point to a new molecular and mechanistic basis for PHT gastropathy and provide a new therapeutic modality for protection of PHT gastric mucosa.