Myelinoclastic diffuse sclerosis (Schilder's disease): report of a case and review of the literature.

Schilder's myelinoclastic diffuse sclerosis is a rare demyelinating disease, which often mimics intracranial neoplasm or abscess. This disease is of importance to the neurosurgeon in that the clinical manifestations and course may closely simulate brain tumour, including the development of papilloedema and other signs of increased intracranial pressure. We have treated a 29-year-old right-handed young man presenting with left hemiplegia, aphasia and behavioural changes. Magnetic resonance imaging showed two large lesions, irregular contrast-enhancing, in the subcortical white matter of the right frontal and parietal lobes, and increased intracranial pressure. Definitive diagnosis was made with biopsy. The characteristic pathological features are demyelinization of the white matter, lymphocytic perivascular infiltrates, microglial proliferation and immunohistochemical signs. The patient improved with steroid treatment. Schilder's disease constitutes an important diagnosis for any neurosurgeon to be aware of which has not received adequate coverage in the neurosurgical literature.

Endoscopic goniotomy with anterior chamber maintainer: surgical technique and one-year results.

The surgical technique of endoscopic goniotomy (EG) using the anterior chamber maintainer (ACM) for congenital glaucoma is evaluated, and one-year follow-up data are presented. Endoscopic goniotomy was performed in 12 eyes of 7 patients. A double-port-special goniotomy knife mounted on the endoscope's probe inserted through the first incision and ACM through the second incision-EG technique was used in 6 eyes of 3 patients, while a three-port-knife, endoscope probe, and ACM inserted through separate incisions-technique was preferred in the remaining 6 eyes of 4 patients. EG of approximately 240 degrees could be done without major complications in all eyes. At the end of the average follow-up period of 14.2 +/- 9.7 months, the mean intraocular pressure was reduced from 38.3 +/- 6.9 mm Hg to 17.6 +/-, 2.8 mm Hg (P = 0.002), the average number of glaucoma medications from 2.1 +/- 0.3 to 0.3 +/- 0.5 (P = 0.001), and the mean cup/disk ratios from 0.84 +/- 0.11 to 0.79 +/- 0.14 (P = 0.014), while there was no statistically significant change in the average corneal diameter (P = 0.16). Therefore, endoscopic goniotomy with ACM was found to be an effective treatment modality for congenital glaucoma.

Self-expandable metallic stent therapy of esophagojejunal stricture in a stapled anastomosis: a case report and review of the literature.

The introduction of the stapler apparatus has provided safe and effective gastrointestinal anastomotic surgical operations for most surgeons. However, the major disadvantage of stapler surgery is an increased risk of anastomotic stricture formation. Treatment of this kind of stricture is performed mainly by using endoscopic balloon dilators. However, this therapy may fail or the patient may become reactive or uncooperative during dilatation sessions. Herein, we present a case to show the successful and uncomplicated insertion of a self-expanding metallic stent into an esophagojejunal anastomotic stricture which developed 1 month after total gastrectomy and stapled esophagojejunal anastomosis in a patient with gastric carcinoma. This is the 3rd report in the literature.

Cavernous transformation of the portal vein is associated with pancreatic duct atrophy.

BACKGROUND/AIMS: To determine whether or not portal venous obstruction affects pancreatic duct size. MATERIAL AND METHODS: The ERCP findings of 43 of 51 subjects with cavernous transformation of the portal vein (CTPV) were studied prospectively. In three, the ERCP failed to demonstrate the pancreatic duct (PD) for technical reasons and in 8 of the original 51 subjects, the ERCP was not attempted because of a lack of indications and unwillingness of the subject to undergo the procedure. The ERCP findings of the remaining 40 subjects were compared with those of 23 normal concurrent controls and those of reference controls. RESULTS: In 21 of the 51 patients, an underlying disorder responsible for portal vein thrombosis and cavernous transformation was identified. Despite a full clinical, radiologic, hematologic and biochemical evaluation, no etiology for CTPV was found in the remaining 30 patients. The pancreatic duct diameter was measured at the widest portion of the duct where it traverses the head of the pancreas. In 34 of the 40 patients with CTPV and a successful ERCP, the pancreatic duct was smaller (less than 2.28 mm in diameter) than that of concurrent control subjects and a reference control group (p < 0.05). In only 6 subjects with CTPV was the pancreatic duct size within normal limits. In 3 of these 6, a confounding common bile duct stone was shown to be present and may have accounted for the pancreatic duct enlargement as compared to the other 34 subjects with CTPV. CONCLUSIONS: These results indicate that a smoothly narrowing pancreatic duct hypoplasia or atrophy can be demonstrated in 85% of cases with CTPV. It is speculated that this finding is a consequence of the obstructed pancreatic venous drainage that occurs with CTPV.

The use of deferoxamine infusions to enhance the response rate to interferon-alpha treatment of chronic viral hepatitis B.

An individual's iron status may affect the response rate achieved with the use of interferon (IFN) as therapy for chronic viral hepatitis. A total of 27 patients with chronic hepatitis B viral infection, who had elevated serum ferritin levels, were randomized to receive either IFN 5 MU, three times weekly by subcutaneous injection alone (n = 14) or in combination with cycles of deferoxamine at a dose od 80 mg kg-1 per cycle (n = 13) administered over 3 consecutive days, to reduce their iron and maintain a serum ferritin level less than 250 ng ml-1. All deferoxamine-treated patients were on a low iron-containing diet. An IFN response was defined as a normalization of the serum alanine aminotransferase (ALT) level and seroconversion from hepatitis B e antigen (HBeAg) positivity to hepatitis B e antibody (HBeAb) positivity. The deferoxamine-treated group experienced a reduction in their serum ferritin level to 226 +/- 73 ng ml-1 as a result of the deferoxamine treatment. Six of the 13 (46%) deferoxamine-treated patients and two of the 14 (14%) control patients normalized their ALT levels. Seven of the 13 (54%) deferoxamine but only 14% of the IFN-treated group seroconverted to HBeAb positivity. A greater rate of histological improvement and loss of hepatitis B virus (HBV) DNA was seen in the deferoxamine-treated group. Two of the deferoxamine-treated patients were treated only once, two were treated twice, seven were treated three times and two were treated four times to achieve a ferritin level below 250 ng ml-1. Based on these data, we conclude that deferoxamine infusion enhances the rate of response to IFN in subjects with chronic hepatitis B. The precise mechanism of this phenomenon is not clear.

Relationship between the serum alanine aminotransferase level at the end of interferon treatment and histologic changes in wild-type and precore mutant hepatitis B virus infections.

Unravelling the role of interferon (IFN) in the treatment of chronic hepatitis B compliance by many factors. Several mutant forms of hepatitis B virus (HBV) have recently been discovered; the most common of these is the precore mutant, characterized by hepatitis B e antigen (HBeAg) negativity and hepatitis B e antibody (HBeAb) positivity in an individual with an active HBV infection. The aim of this study was to compare the response rate to IFN therapy in patients with wild-type HBV infection and in individuals infected with the precore mutant. A second aim was to evaluate the role of an increased serum ferritin in terms of the IFN response rate in these two different types of HBV infection. IFN therapy was administered at a dose of 5 MU subcutaneously three times weekly for 6 months to 41 individuals with a chronic wild-type hepatitis B infection and 16 individuals with a precore mutant chronic HBV infection. An IFN response was defined as normalization of the serum alanine aminotransferase (ALT) level and an HBeAb to HBeAb seroconversion (in wild-type hepatitis infection), and a normalization of the serum ALT in individuals infected with a precore mutant infection. At entry, the two groups were matched for age, gender, serum ALT, serum iron, total iron binding capacity (TIBC), serum ferritin and liver histology. Forty-six per cent of the subjects with wild-type disease responded to IFN therapy. By contrast, only four of the 16 cases (25%) of the precore mutant cases responded (p < 0.05). Ferritin levels correlated well with the type of IFN response; as the serum ferritin level increased, the response rate to IFN declined. Hapatic infection caused by a precore HBV mutant is more resistant to IFN therapy than wild-type infection. The serum ferritin level appears to influence the type of IFN response achieved. Individuals with a serum ferritin level greater than 300 ng ml-1 failed to respond to IFN in 93% of the cases studied.

Is portal hypertension due to liver cirrhosis a major factor in the development of portal hypertensive gastropathy?

OBJECTIVES: The gastric mucosa of patients with portal hypertension frequently manifests changes in its appearance that are readily identifiable by endoscopy. Many of these can be sources of bleeding, and some imply the presence of systemic disease. Although portal hypertension is critical in development of portal hypertensive gastropathy (PHG), the role that other factors might play in its pathogenesis is uncertain. METHODS: Four groups of subjects were studied prospectively: 37 with portal hypertension due to cirrhosis, 26 noncirrhotic subjects with portal hypertension due to extrahepatic portal vein obstruction (PVO), nine cirrhotic patients with extrahepatic PVO, and 57 control subjects. The diagnosis in each case was based on a combination of clinical data, needle liver biopsy, ultrasonography, splenoportography, and upper GI endoscopy. RESULTS: Snake skin, scarlatina rash, diffuse hyperemia, and diffuse bleeding were frequent endoscopic gastric findings in cirrhotic patients. These findings were seen less frequently in noncirrhotic patients with portal hypertension due to PVO than in cirrhotic patients (p< 0.0001). The highest incidence was seen in cirrhotic patients with PVO (P< 0.001). Positive correlations existed among the endoscopic findings, the clinical estimate of the cirrhosis severity (Child-Pugh grade), and the size and appearance of esophageal varices (Beppu score). No endoscopic findings of the gastric mucosa enabled one to distinguish between groups. Hypergastrinemia was present in cirrhotics with and without PVO but not in noncirrhotic patients with portal hypertension resulting from isolated PVO. CONCLUSION: These findings suggest that the endoscopic findings of PHG are affected by the severity of the underlying liver disease and the presence or absence of coexisting PVO. There is no association between PHG and the presence of gastric varices. Thus, the development of the gastric lesions characteristic of PHG requires not only portal hypertension but also some other consequence of parenchymal liver disease.

The "pseudo-cholangiocarcinoma sign" in patients with cavernous transformation of the portal vein and its effect on the serum alkaline phosphatase and bilirubin levels.

OBJECTIVES: The goal of this study was to identify the underlying disorder responsible for portal venous thrombosis and cavernous transformation of the portal vein (CTPV). All patients with this finding underwent a thorough medical examination with intent to determine the cause and biochemical consequences of CTPV. METHODS: During an 8-yr period, a total of 1247 patients with clinical evidence of portal hypertension were examined using ultrasonography. Forty four of these 1247 patients were found to have CTPV. In each case, the finding of CTPV was confirmed by portography using either splenoportography or arterial portography, with digital subtraction angiography. These 44 patients were studied in an effort to determine the etiology of the cavernous transformation. In addition, the specific reason for the increased serum bilirubin and alkaline phosphatase levels in 35 of the 44 cases was evaluated by endoscopic retrograde cholangiopancreatography (ERCP) (34 patients), percutaneous transhepatic cholangiography (one patient), and by CT in 19. The surgical findings in 10 of these 44 patients, who ultimately underwent splenectomy and portal venous decompression for bleeding, were reviewed in light of the ultrasonographic, portographic, and ERCP findings in the same 10 patients. RESULTS: The underlying disorder responsible for cavernous transformation was found to be Behcet's disease in seven patients, chronic liver disease in four, congenital hepatic fibrosis in five, congenital protein C deficiency in one, and a prior abdominal operation for cholelithiasis in one patient. Despite a full clinical, radiological, hematological, and chemical evaluation, no etiology for CTPV was found in the remaining 26 patients. All of these later cases had no indication for liver biopsy or evidence for parenchymal liver disease. In these 26 patients, the serum levels of bilirubin and alkaline phosphatase ranged from mild to moderately increased compared with the moderately to markedly increased levels present in the 18 patients having an identifiable underlying liver disease. Irregular, undulating narrowing and nodular extrinsic defects, the so-called "pseudo-cholangiocarcinoma sign" was present in 33 of the 35 patients who underwent either ERCP or percutaneous transhepatic cholangiography. No such findings were observed in 10 control cirrhotic patients with portal hypertension but without CTPV, who also underwent ERCP. CONCLUSION: The results of this study indicate that mildly increased serum alkaline phosphatase and direct reacting bilirubin levels occur in cases with CTPV associated with a pseudo-cholangiocarcinoma sign. Presumably, these enzyme elevations are a result of compression of the biliary tree by the venous collaterals that run along the extrahepatic biliary tree. None of the 33 cases with this sign had cholangiolar carcinoma. Thus, when a patient with splenomegaly but without documentable parenchymal liver disease demonstrates an increase in the serum direct reacting bilirubin and alkaline phosphatase levels, CTPV and the presence of large extrahepatic venous collaterals partially obstructing the biliary tree should be suspected.

[Prevalence of HBV surface antigens, anti-HBD and anti-HCV antibodies in patients with virus-related chronic liver disease]. / Virusa bagli kronik karaciger hastaliklarinda HBV yüzey antijeni, anti-HDV ve anti-HCV antikor sikligi.

In the present study, we investigated Hepatitis B virus (HBV), Hepatitis C virus (HCV), and Hepatitis D virus (HDV) status in patients with chronic liver diseases seen in the period of 1990-1992 using enzyme-linked immunosorbent assay (ELISA) kits. There were 126 male and 64 female patients with a mean age of 42.3 years (range 17-70). Of the 198 patients, 68 (35.7%) had evidence of HCV infection. One hundred and twenty-three patients (64.7%) were positive for hepatitis B surface antigen (HBsAg). Of the 123 HBsA positive patients, 35 (28.4%) were positive for Anti-HDV. Fourteen patients were negative for markers of both HCV and HBV. Liver histology showed persistent hepatitis in 31 patients (16.3%), chronic active hepatitis in 77 (40.6%). Cirrhosis was diagnosed in 82 patients (43.1%). Our results indicate that HCV infection plays a role in chronic liver disease especially where the etiology appears obscure.

Effects of recombinant alpha interferon on chronic active hepatitis B: preliminary results.

This paper presents the preliminary results of a study designed to evaluate the effects of alpha interferon in chronic hepatitis B. After six months' treatment with interferon alfa-2b (5 million units (MU), three times weekly) 15 of 25 (60%) patients achieved seroconversion of hepatitis B e antigen, 17 (68%) normalised alanine aminotransferase (ALT) activity, and 15 (60%) showed a decrease in the inflammatory reaction on liver histology. No seroconversions occurred in the control group (n = 10), and none of the control patients achieved a normal ALT or showed a reduction in the inflammatory reaction. Adverse effects were experienced by most patients who received interferon but none warranted stopping the treatment.