Prevalence of root dilacerations in Central Anatolian Turkish dental patients.

OBJECTIVE: The aim of this study was to determine, retrospectively, the prevalence and distribution of the dilaceration of the root for each tooth-type in a sample of Central Anatolian Turkish population by using panoramic radiographs. METHOD: Panoramic radiograhs of 6912 patients (3860 women and 3052 men, mean age 29.04 years, range, 15 to 50 years) were examined for the presence of root dilacerations. Chi-square test was also used to compare the prevalence of dilacerations between male and female subjects and upper and lower jaws. RESULTS: Data showed that 1108 (16.0%) of these subjects had one or more teeth that were dilacerated and these were detected in 466 (15.2%) males and 642 (16.6%) females. Statistical analysis (chi2 test) showed a significant difference in the prevalence of dilaceration among male and female patients. Mandibular third molars were dilacerated most often (3.76%), followed by mandibular second molars (1.81%). Dilaceration was found in 1.23% of maxillary second premolars and 1.23% of mandibular second molars. CONCLUSION: Root dilacerations are not uncommon among Turkish dental patients, and their early detection could be important in treatment problems associated with it. However further larger scale studies are required to assess its prevalence in the general population in order to compare it with other ethnic groups.

How confocal laser endomicroscopy can help us in diagnosing gastric lymphomas?

Primary gastric diffuse large cell lymphoma is one of the most common extranodal lymphomas of the gastrointestinal system. Diagnosing gastrointestinal lymphomas can be difficult, since there is no pathognomonic sign in endoscopy to distinguish it from other malignancies. In some cases biopsy can be non-diagnostic. Therefore, multiple endoscopic examinations and biopsies can be necessary. With using confocal endomicroscopy, histology of the tissue can be seen in vivo and a range of diseases can be identified by using this technique. We are presenting a case, which is diagnosed as primary gastric diffuse large cell lymphoma during the evaluation of erythema nodosum etiology. We want to emphasize the role of confocal laser endomicroscopy for in vivo diagnosis of gastric lymphoma and directing the endoscopist for sampling the diseased mucosa. Confocal endomicroscopy decreases non-diagnostic rates in endoscopic biopsy and can be performed successfully in cases of gastric lymphoma. Pit patterns of gastric lymphoma, ring cell gastric carcinoma and gastric adenocarcinoma are similar. To best of our knowledge, this case is the fifth case of confocal laser endomicroscopy aided in diagnosing gastric lymphomas (Tab. 1, Fig. 2, Ref. 13).

Infection with Fasciola hepatica.

Fascioliasis, caused by the liver fluke Fasciola hepatica, is an infection that occurs worldwide, although humans are accidental hosts. F. hepatica infection comprises two stages, hepatic and biliary, with different signs and symptoms. Stool examination and ELISA can be used for the initial diagnosis. Radiographic techniques, such as computerised tomography and ultrasonography, as well as magnetic resonance imaging, are used widely for confirmation and follow-up of the disease. Invasive techniques, such as percutaneous cholangiography, endoscopic retrograde cholangiography and liver biopsy, may aid in the diagnosis but are not essential. Triclabendazole is recommended as the first-line agent for the treatment of F. hepatica infection, with bithionol as an alternative.

Superior vena cava thrombosis and obstructive sleep apnea in a patient with familial Mediterranean fever.

Familial Mediterranean fever (FMF), a paroxysmal, self-limited, inflammatory disease of unknown etiology, may result in thrombotic complications after the development of nephrotic syndrome due to amyloidosis. It has been suggested that there is increased thrombogenic activity in the blood of patients with FMF who did not develop nephrotic syndrome. We describe a patient with FMF who presented with thrombosis in the superior vena cava (SVC) in the absence of nephrotic syndrome, and discuss the contributory role of increased procoagulant activity detected in this disorder. Moreover, the patient was proved to have obstructive sleep apnea (OSA) which we believe was secondary to SVC thrombosis that lead to soft tissue edema in the upper airways. To our knowledge, this is the second reported case in the literature in which OSA occurred secondary to the SVC thrombosis.

The importance of serial measurements of cytokine levels for the evaluation of their role in pathogenesis in familial Mediterraean fever.

Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by recurrent fever of unknown origin, renal amyloidosis, peritonitis, pleuritis and/or synovitis. There have been many studies to elucidate the etiopathogenesis of FMF. IL-6 is a cytokine that can induce the formation of serum amyloid A and C-reactive protein, both of which are important in development of amyloidosis. IL-6 was determined to be strongly associated in the etiopathogenesis of periodic fever in Chinese-pei dogs. The dogs with this syndrome experience periodic fever, arthritis, renal amyloidosis, a clinical picture very alike of human FMF. Here, we aimed to study mainly whether IL-6 had a similar etiopathogenetic role in human FMF as in Chinese-pei dogs syndrome. The median IL-6 blood levels were found to be higher in patients with acute (n=8) FMF attack (1.85 U/ml) compared to those (n=33) with asymptomatic ones (1.0 U/ml) (p=0.16). There are mainly two results: first; the study should be designed with a larger sample size of patients with acute attack in order to alleviate underestimation of significance, second; sampling time may give various results because of dynamic changes of cytokine levels during acute attack period.

Hepatitis C and the controversies it creates relative to liver transplantation and autoimmune hepatitis.

The paradox of hepatitis C. An agent that persists in tissue for years by escaping the immune system and not killing but only damaging its host while simultaneously being associated with autoimmune hepatitis as well as other putative autoimmune diseases is presented. Liver transplantation necessitates immune suppression and is associated with progressive HCV disease albeit at a rather slow rate. Diagnosis and treatment concerns are raised and discussed.

A prospective randomized trial from Turkey comparing octreotide versus injection sclerotherapy in acute variceal bleeding.

BACKGROUNDS/AIMS: Bleeding from gastroesophageal varices continues to be a life threatening complication of chronic liver diseases and portal hypertension. The purpose of this randomized prospective study is to compare the efficacy of octreotide administration and emergency injection sclerotherapy for the control of actively bleeding esophageal varices and prevention of early rebleeding in patients with cirrhosis. METHODOLOGY: A total of 66 episodes of endoscopically proven active variceal bleeding in 52 patients were included in this study. Following admission to the hospital, the patients were resuscitated with blood and plasma, and fiberoptic endoscopy was performed within 2 hours. Thirty-six bleeds in 28 patients and 30 bleeds in 24 patients were randomized to endoscopic variceal sclerotherapy (1% polidocanol) and to octreotide infusion (at 50 micrograms/h for 12 hours following the initial 50 micrograms i.v. bolus), respectively. RESULTS: Bleeding was initially controlled within 6 hours in 75% of episodes by endoscopic variceal sclerotherapy and in 73.3 by octreotide infusion (P > 0.05). There were no significant differences between the 2 groups in early rebleeding (within 72 hours of randomization) (22% vs. 22.7%), blood transfusion (4.2 +/- 1.8 units vs. 4.8 +/- 2.9 units), or hospital mortality (3.6% vs. 3.3%). Treatment failed in 9 episodes (25%) in the sclerotherapy group and in 8 episodes (26.7%) in the octreotide group. CONCLUSIONS: We consider that Octreotide would appear to be as effective as sclerotherapy in both the early control of variceal hemorrhage and in the prevention of early recurrent bleeding and should therefore be considered the treatment of choice in those centers where 24-hour endoscopy is not available. Furthermore, even in hospitals that do have a 24-hour endoscopy service there is good evidence that octreotide therapy should be commenced as soon as a patient enters hospital with a suspected variceal bleed to achieve rapid homeostasis. When initial hemostasis is achieved, elective endoscopic therapies can be undertaken with greater success.

What is the association of primary sclerosing cholangitis with sex and inflammatory bowel disease in Turkish patients?

BACKGROUND/AIMS: In the Western world, primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease that is associated with inflammatory bowel disease (IBD), particularly chronic ulcerative colitis and, to a lesser degree, Crohn's disease. The goal of this study was to determine the prevalence of PSC in Turkish patients with IBD and chronic amebic colitis, a disease that is endemic in Turkey. METHODOLOGY: During a 10-year period, between 1986 and 1996, a total of 81 IBD (64 ulcerative colitis and 17 Crohn's disease) patients and 127 patients with chronic amebic colitis were seen and evaluated with radiologic, serologic, immunologic and pathologic tests. Whenever a clinical or biochemical finding suggested the presence of co-existent hepatic and/or biliary disease, the patient was further evaluated by liver biopsy, auto-antibodies and endoscopic retrograde cholangiopancreatography (ERCP) to determine whether they also had PSC or some other form of liver disease. As a disease control group, a total of 752 patients with clinical and/or laboratory evidence of pancreaticobiliary disease were also studied. In 86 of these 752 patients (10%), a primary disorder of the biliary tree was diagnosed by ultrasonography, computed tomography, peritoneoscopy, liver biopsy, ERCP and abdominal laparotomy. In addition, all 86 patients of the control group were evaluated endoscopically in order to determine whether they had any associated gastrointestinal condition of the upper or lower gastrointestinal tracts. After establishing final diagnoses of IBD, amebic colitis and PSC, these patients were evaluated with respect to their socio-economic status. A high protein diet (1.8 gram/kg/day) was administered to those patients with chronic amebic colitis and IBD during the active period of the disease. RESULTS: Of the 208 patients (81 with IBD and 127 with chronic amebic colitis), no cases of PSC were identified. Of the 86 patients in the control group with primary biliary tract disease, 45 had a biliary system malignancy, 14 had primary biliary cirrhosis (PBC), 16 had PSC, 3 had Caroli's disease, 6 had a common bile duct cyst, and 2 had gallbladder adenomatosis. All but 1 of the 16 patients with PSC were female. CONCLUSIONS: These data suggest that, in contrast to findings in Western Europe and the USA, in Turkey: 1) PSC is not regularly associated with idiopathic IBD; 2) most patients with PSC are female; 3) PSC accounts for only 18% of patients with a primary disorder of the biliary tree; 4) the incidence of small-duct primary sclerosing cholangitis is greater than that reported in the literature; and, 5) the incidence of IBD and PSC in Turkey is relatively lower than in other countries.

Determination of hepatic zinc content in chronic liver disease due to hepatitis B virus.

BACKGROUND/AIMS: Zinc is an essential, mostly intracellular, trace element which participates in many physiologic mechanisms. Some liver functions like urea formation require the presence of zinc; thus the determination of hepatic zinc content may contribute to the understanding of probable zinc-related clinical consequences of chronic liver disease. In this study, we aimed to determine the hepatic zinc concentrations in patients with chronic liver disease due to the Hepatitis B virus and to ascertain the relationship between the severity of liver disease and hepatic zinc content, if one in fact exists. METHODOLOGY: A total of 99 HBsAg positive subjects were included in the study. We performed a liver biopsy on all subjects. Hepatic zinc concentrations were determined by atomic absorption spectrophotometry. RESULTS: The liver biopsies were normal in 25 subjects. There were 33 chronic active hepatitis (CAH), 34 cirrhosis and 7 chronic persistent hepatitis (CPH) patients in the study group. In the control group, CAH, cirrhosis and CPH groups, the mean liver zinc concentrations were 3.83 +/- 1.86, 1.86 +/- 0.92, 1.14 +/- 0.68 and 3.74 +/- 1.81 mumol/g dry weight, respectively. Hepatic zinc in the CAH and cirrhosis groups were lower than that of the control group (p < 0.05). We also found that liver zinc in the cirrhosis group was lower than in the CAH group (p < 0.05). CONCLUSION: According to these results, as the severity of liver damage increases, the hepatic zinc concentration decreases. Therefore, it can be suggested that zinc supplementation may improve hepatic encephalopathy by increasing the efficiency of the urea cycle.

A comparison of the prevalence of autoantibodies in individuals with chronic hepatitis C and those with autoimmune hepatitis: the role of interferon in the development of autoimmune diseases.

BACKGROUND AND OBJECTIVES: Viral hepatitis due to hepatitis C virus results in chronic liver disease in more than 70% of individuals infected with the virus. Hepatitis C virus is also thought to be the cause of autoimmune chronic hepatitis, type II. The only treatment for chronic hepatitis C is interferon (IFN). IFN is both an antiviral agent and an up regulator of the cellular immune system. The latter effect is non-specific. Thus, IFN diffusely activates the cellular immune system and can initiate new autoimmune diseases in patients treated with it. To determine the prevalence of autoantibodies in patients with chronic hepatitis C and in patients with autoimmune hepatitis and to determine the incidence of new onset autoimmune disease in IFN-treated subjects with chronic hepatitis C, the records of 323 unselected patients with chronic hepatitis were reviewed. MATERIAL AND METHODS: A total of 203 patients with a mean age of 45.7 +/- 0.8, ranging 18-81 with either HCV disease or autoimmune hepatitis, were identified and studied. One hundred sixty-two patients with chronic hepatitis C defined by elevations of serum alanine aminotransferase (ALT) for at least 6 months, the presence of detectable anti-HCV (HCV; second generation enzyme immunoassay [EIA2], a positive recombinant immunoblot assay [RIBA], the presence of HCV-RNA by PCR in serum and an abnormal biopsy consistent with chronic hepatitis C) were identified. Each was also negative for HbsAg, HbeAg and anti-Delta. Forty-one patients with a putative autoimmune chronic hepatitis (AIH) diagnosed on the basis of serologic positivity for classical autoantibodies (ANA and anti-smooth muscle antibodies), tissue typing (B8, Dr3 positive), characteristic liver biopsy findings and the absence of anti-HCV and HCV-RNA in serum were identified. The records of both of these groups of patients were reviewed for the following antibodies: anti-nuclear antibodies (ANA), antimitochondrial antibodies (AMA), anti-liver-kidney microsomal antibody (LKM), anti-smooth muscle antibodies (SMA), anti-microsomal antibodies (MSA). RESULTS: The rate of ANA positivity was 63% in both groups; the rate of SMA positivity was 65% in patients with HCV infection (group I) and 63% in patients with AIH (group II). AMA was positive in 4% of the subjects in group I and 50% of the subjects in group II; anti-LKM antibodies were absent in all 91 HCV cases and were present in 4% of the cases in group II; MSA positivity was present in 17% of group I and 10% of group II. Eighty-one of the one hundred sixty-two patients (50%) with chronic hepatitis C received IFN treatment at a dose of 5 MU SQ daily for 6 months. Thirty-two of these eighty-one patients (42 females and 39 males with a mean age of 45.0 +/- 1.3, ranging from 18 to 81 yr.) had at least two autoantibodies detectable prior to the IFN therapy (subgroup 1) and 49 had one or no identifiable autoantibodies (subgroup 2) present prior to IFN therapy. No significant differences in the interferon response rate defined by HCV-RNA negativity and normalization of serum ALT levels at the end of therapy was noted between those with autoantibodies and those without autoantibodies. Fifteen of the interferon-treated patients developed a clinical manifestation of a new onset autoimmune disease during the course of their interferon treatment. Six of the fifteen patients belonged to subgroup 1 (n = 32) and the remaining 9 patients to subgroup 2 (n = 49) (p > 0.05). None were managed by discontinuing the interferon. Most required some form of specific treatment.(ABSTRACT TRUNCATED)

Percutaneous drainage of hepatic abscesses: therapy does not differ for those with identifiable biliary fistula.

BACKGROUND/AIMS: Surgical drainage of pyogenic and amoebic hepatic abscesses has been an accepted therapy for decades. Modern imaging modalities have changed both the diagnostic and therapeutic management of many hepatic lesions, particularly abscesses. Specifically, percutaneous aspiration and drainage with either ultrasound or computed tomography guidance has been reported as an alternative treatment for hepatic abscesses in recent years. Little is known about aspiration of hepatic abscesses that communicate with the biliary tree. MATERIAL AND METHODS: Fifteen patients with hepatic abscesses treated by percutaneous aspiration and drainage are herein reported. Six had a demonstrable fistulous communication between the abscess and the biliary tree. RESULTS: During a four year period of follow-up, only two of these 15 patients experienced a recurrence. Neither had a biliary fistula complicating their abscess initially. CONCLUSIONS: Based upon this experience, it is suggested that percutaneous aspiration and drainage should be the initial treatment of choice of both pyogenic and amoebic liver abscesses even when large. Abscesses with demonstratable biliary fistulas have a larger mean volume than do those without fistulous involvement. The finding of a communication between the abscess and the biliary tree should not change this treatment approach.

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 usefulness of intra-operative endoscopy in Peutz-Jeghers syndrome: a case report.

The Peutz-Jeghers syndrome is an autosomal dominant inherited disease manifested by a combination of mucocutaneous pigmentation and gastrointestinal hamartomatous polyps that usually cause intussusception and intestinal hemorrhage. We report a case in which the patient has been followed-up on for 14 years and who underwent surgical and endoscopic polyp removal several times as well as one intestinal resection. This time, with the use of combined surgery and perioperative endoscopy, 27 polyps were removed, performing only 3 enterotomies. This is the highest number in one session to be reported in the literature. The usefulness of this technique is providing a "clean small intestine" that allows the patient a longer time interval between laparotomies and reduces the complications associated with multiple laparotomies and resections.

The effect of interferon and desferrioxamine on serum ferritin and hepatic iron concentrations in chronic hepatitis B.

BACKGROUND/AIMS: Recent reports indicate that an individual's iron status might affect the response rate achieved with Interferon therapy for the treatment of chronic viral hepatitis. METHODOLOGY: Forty individuals, 29 men and 11 women, with chronic viral hepatitis B, who had elevated serum ferritin levels, were randomized to receive either Interferon (IFN) 5 MU TIW SQ for 6 months alone (n=21) or Interferon in combination with repetitive cycles of desferrioxamine infused at a dose of 80 mg/kg per cycle (n=19) over 3 consecutive days in an effort to reduce their metabolically active iron pool during the course of IFN treatment. These cycles were continued until a serum ferritin level of less than 250 ng/ml (normal values <220 ng/ml) was achieved. Additionally, all desferrioxamine treated subjects were placed on a low iron containing diet. An interferon response was defined as normalization of the serum ALT and seroconversion from eAg positive to eAb positive. All other responses were defined as failures. RESULTS: The mean ages of the subjects in the 2 groups were 39+/-6 and 38+/-5 years. The initial serum ALT levels were 150+/-27 and 151+/-13 IU/l. The hepatic iron concentrations were 916+/-29 and 896+/-15 microg/g/dry liver weight. The serum ferritin levels were 386+/-12 and 393+/-18 ng/ml. None of these values differed significantly between the 2 treatment groups. The desferrioxamine treated group consisted of 14 men and 5 women. This group experienced a reduction in their serum ferritin to a level of 237+/-13 ng/ml as a result of the desferrioxamine treatment (p<0.05). Additionally, a reduction in their hepatic iron concentration, to a level 766+/-29 microg/g/dry liver weight, occurred with treatment (p<0.05). Twelve of the 19 (63%) desferrioxamine-treated subjects and 8 of the 21 (38%) control subjects experienced a normalization of their serum ALT levels with treatment (p<0.05). Thirteen of 19 (68%) of the desferrioxamine-treated subjects but only 8 of 21 (38%) of the IFN alone treated group seroconverted to anti-e positive (p<0.05). Moreover, a greater improvement in the hepatic histologic score and rate of HBV-DNA loss occurred in the desferrioxamine-treated group. CONCLUSIONS: Based upon these data, it can be concluded that desferrioxamine infusion to achieve a normal serum ferritin level enhances the likelihood of an individual with chronic hepatitis B responding to IFN therapy. The precise mechanism responsible for this phenomenon is not clear, but would appear to be due to a reduction in the hepatic free iron pool as reflected by sequential changes in the serum ferritin and hepatic iron concentrations.

A comparison of chemoembolization with conventional chemotherapy and symptomatic treatment in cirrhotic patients with hepatocellular carcinoma.

BACKGROUND/AIMS: The results of transcatheter arterial chemoembolization (TACE) in 28 cirrhotic patients with advanced hepatocellular carcinoma (HCC) were compared to those obtained in 15 cirrhotic patients with HCC treated with systemic chemotherapy, and 14 patients with HCC receiving no specific anti-cancer treatment. MATERIALS AND METHODS: From November, 1986 through May 1994, 235 patients with HCC were seen by the investigators. Twenty eight of these patients actually received TACE. The chemotherapeutic agent used was mitomycin C mixed with Lipiodol and arterial embolization was achieved using Gelfoam. In 19 of 28 cases treated with TACE, the embolization was limited to an artery feeding the tumor, thereby avoiding liver cell injury in non-tumor tissue. In 9 others, the main left or right hepatic artery was embolized. The results obtained in these 28 cases were compared to those obtained in 15 patients with HCC larger than 5 cm. who received systemic mitomycin C and doxorubicin chemotherapy and with those obtained in 14 patients who received no specific anti-tumor therapy. RESULTS: One patient died of liver failure related to the TACE and three patients died of bleeding from esophageal varices within weeks of the TACE procedure. Two of the remaining 24 patients are alive with a follow-up of 6 and 22 months. Twenty-six of the 28 patients treated with TACE died within 1 and 28 months of the initiation of the TACE therapy. The mean survival of those receiving TACE was 13.0 months. Chemotherapy without embolization yielded a mean survival of 7.2 months. The mean survival of the patients receiving no specific anti-cancer treatment was only 6.9 months. There was no statistical differences between the survival of those receiving systemic chemotherapy and those receiving no specific treatment. In contrast, the mean survival of the group receiving TACE was significantly greater than that of either of the other two groups (p < 0.005). CONCLUSIONS: These data suggest that TACE provides the best survival for individuals with larger hepatocellular carcinomas (> 5 cm) not eligible for surgery or percutaneous ethanol injection (PEI).

Periampullary carcinoma detected after sphincterotomy.

BACKGROUND/AIMS: Although periampullary carcinoma can often be diagnosed at an early stage because of its strategic location, in a substantial number of cases (23% to 25% of periampullary carcinoma cases), the papilla can be prominent, but without an identifiable mass or ulceration. As a result, duodenoscopy alone can miss the tumor. In this series, the usefulness of sphincterotomy in establishing a diagnosis of periampullary carcinoma is documented. MATERIALS AND METHODS: A total of 664 patients, who had clinical, biochemical and/or sonographic evidence of pancreaticobiliary disorders underwent endoscopic retrograde cholangiopancreaticography (ERCP) at Hacettepe University Hospital between March 1985 and September 1994. All identified lesions were biopsied. RESULTS: In sixty-six of the 664 patients, a specific periampullary or pancreatic diagnosis could be made. These included 30 cases of pancreatic carcinoma (14 in the head, 15 in the corpus, and 1 in the tail), 10 cases of chronic pancreatitis, 15 cases of periampullary carcinoma and 11 miscellaneous. None of the 15 periampullary carcinomas was demonstrable by abdominal computed tomography, sonography or pancreatic angiography. In 12, the diagnosis was established immediately by endoscopic visualization of the tumor and biopsy. In the remaining 3, the tumor was visualized as a protruding mass only after a papillatomy was performed. All cases were treated surgically with a pylorus preserving pancreaticoduodenectomy. No symptoms of dumping were experienced postoperatively. The survival of these 15 patients was good with a mean survival of 24.2 months. CONCLUSIONS: Careful endoscopic examination of the periampullary mucosa before and after sphincterotomy is required to identify periampullary carcinoma. A pylorus preserving pancreaticoduodenectomy revealed excellent post-surgical outcome with no post operative symptoms of the dumping syndrome.

Four cases with chronic intestinal pseudo-obstruction due to hollow visceral myopathy.

BACKGROUND/AIMS: Chronic intestinal pseudo-obstruction is a rare clinical syndrome characterized by symptoms and signs of intestinal obstruction without any organic lesion obstructing the intestine. Visceral myopathy is one of the etiological causes and full thickness intestinal biopsy is essential for reaching a diagnosis. In this article we describe 4 cases of hollow visceral myopathy; our aim is to stress the importance of full thickness biopsy. METHODOLOGY: Four cases of hollow visceral myopathy are studied herein. All the patients had recurrent abdominal pain and constipation. The onset of symptoms was early in life or in the second to third decade. A diagnosis was established in all cases by full thickness intestinal biopsy obtained during laparotomy. Associated disorders were noted in 2 cases. One patient had Axenfelt syndrome, non-descended testicles and primary hypogonadism, and another had a diagnosis of Kleinfelter syndrome. RESULTS: All of the 4 cases were diagnosed to be suffering from hollow visceral myopathy by full thickness intestinal biopsy and 2 had additional disorders as well. CONCLUSIONS: Patients with chronic intestinal pseudo-obstruction should be carefully evaluated as to whether there is an associated disorder and the diagnosis may be delayed unless full thickness intestinal biopsy is obtained.

Congenital hepatic fibrosis associated with cavernous transformation of the portal vein.

BACKGROUND/AIMS: Congenital hepatic fibrosis (CHF), which is one of the fibropolycystic diseases, occurs in various forms. Portal hypertension, a very common clinical feature of this condition, has been attributed to the compression of portal vein radicles in the fibrous bands. We investigated whether there are any other contributing factors in the development of portal hypertension in patients with CHF. METHODOLOGY: A total of 1285 patients with portal hypertension of different etiologies were studied using ultrasonography as the screening test. Forty-seven (including portal vein involvement and/or CHF) of these 1285 patients were prospectively studied to evaluate the etiology of the portal hypertension by portography, abdominal computed tomography, exploratory laparotomy, peritonoscopy, liver biopsy and laboratory tests. The patients with CHF were divided into two groups, according to whether or not they had portal vein involvement. RESULTS: Eleven (0.8%) of the 1285 patients with portal hypertension had CHF, and 41 (3.2%) had cavernous transformation of the portal vein (CTPV), resulting from different or unknown etiologies. Five patients had both pathologies (CTPV and CHF). In the 11 patients with CHF, there was CTPV in 5 patients, Caroli's disease in 2 patients, cholangiocarcinoma in 1 patient, inferior vena caval obstruction in 1 patient, and CHF in only 2 patients. There were statistically significant differences in the age of the CHF patients at clinical onset, the incidence of bleeding from esophageal varices, and laboratory findings between the 2 groups with and without CTPV. Despite a thorough investigation, we could not distinguish any predisposing factor in 25 of the 41 patients with CTPV. The incidence of CTPV was 48% in patients with CHF and 3.2% in patients with portal hypertension. CONCLUSIONS: These results suggest that the association of CTPV with CHF is not coincidental, but that CTPV may be associated with CHF and a new possible factor in portal hypertension, and that it can be a major factor in the manifestation of esophageal bleeding from varices.

Budd-Chiari syndrome: analysis of 30 cases.

The authors report their experience with 30 adult patients with Budd-Chiari syndrome (BCS), which is a rare and serious disorder, characterized by hepatic outflow obstruction caused by many different conditions. The diagnosis was based on the clinical data, ultrasonography (US), vena cavography and hepatic venography, computed tomography (CT), and liver biopsy. Behçet's disease (BD) was found in 10 patients with BCS as an underlying disease. Two patients used oral contraceptive drugs, 2 had liver tumor hepatocellular carcinoma and liver lymphoma, and 1 patient had chronic lymphocytic leukemia. Despite full investigation, the authors could not find any obvious underlying cause in the other 15 patients. The results suggest that (1) BCS must be considered as a possible complication in patients with Behçet's disease when they have hepatomegaly even if there were no cardinal manifestations of the disease at the time of admission, and BD is the most common etiologic factor in BCS (33%) in Turkey, where the incidence of Behçet's disease is relatively high. (2) Anti-aggregant treatment seems to be effective in many instances. (3) There were space-occupying lesion-like appearances in the liver of 7 cases by CT and US examination in the acute stage, and these disappeared on the follow-up CT and US in 5 patients but continued in 2. BCS should thus be differentiated from other liver lesions. (4) There were other great-vessel involvements in 43% of the cases, mostly venous, but only 1 pulmonary arterial occlusion.