Duodenal lymphangitis carcinomatosa: Endoscopic characteristics and clinical significance

Background and Aim: Duodenal lymphangitis carcinomatosa has been sporadically described, but so far little attention has been paid to duodenal lymphangitis carcinomatosa. Methods: Four cases with duodenal lymphangitis carcinomatosa were endoscopically and histologically examined. Results: The four cases exhibited multiple polypoid lesions along the Kerckring's folds and/or were covered by characteristically granular, non‐ulcerated mucosa upon thickening. The granularity seems to been caused by dilated lymph vessels containing the carcinoma cells. The lesions were microscopically characterized by: (i) involvement of lymph vessels located in the upper portion of the lamina propria; (ii) no inflammatory changes; and (iii) no desmoplastic changes. Primary sites were thought to be the stomach in case 1, the pancreas in cases 2 and 4, and unknown in case 3. All patients died within 6 months after admission or endoscopic examination. Conclusions: As duodenal lymphangitis carcinomatosis shows characteristic endoscopic appearance, endoscopic diagnosis is not difficult. We should realize that the lesion represents extremely poor prognosis, and it should be distinguished from ordinary metastatic duodenal carcinoma.     

Ostial PV Isolation:

Pulmonary vein (PV) isolation by elimination of spike potentials has been reported to cure drug refractory atrial fibrillation. Because of the heterogenous morphology of the PVs, sequential electroanatomic reconstruction of the PVs was performed in 39 patients (group A), who underwent subsequent PV isolation by interruption of all conductive myocardial fibers by distinct RF current applications using a "lasso" approach. In group B (157 patients), only biplane two-dimensional fluoroscopy was performed to guide the diagnostic and the ablation catheters. After reprocedures (in 7% of patients in group A and 22% of group B), which depicted a recurrence of a spike potential inside or at the ostium of  >1 previously isolated PV in all restudied patients, stable sinus rhythm was documented in 69% of patients in group A and 60% of patients in group B. Reasons for the relapse of the previously eliminated spike potentials include a temporary ablation effect and a too distal interruption of the conducting myocardial fiber. Detailed knowledge of the individual three-dimensional morphology enhanced the clinical success rate of PV isolation but is time-consuming using CARTO   (8.0 ± 1.7 vs 5.0 ± 1.6, P < 0.001)   . Further technical improvement to fuse the individual three-dimensional anatomy and the electrophysiological markers to a composed "electroanatomic" map may overcome this limitation in the future. (PACE 2003; 26[Pt. II]:1624–1630)     

Colonic pseudolipomatosis, microscopically classified into two groups

Background: Colonic pseudolipomatosis is rare and the pathogenesis is controversial. The purpose of the present paper was to clarify endoscopic and histological characteristics of colonic pseudolipomatosis and to discuss the etiology. Methods: A total of 15 lesions from 14 patients was reviewed. They were able to be histologically classified into two groups on the basis of variety in size of the vacuoles: Group A, the ratio of largest vacuole to smallest vacuole in size is less than three, Group B, the ratio is more than four. Results: Four of 15 lesions were group A, and were endoscopically polypoid or flat lesions covered with normal‐looking mucosa. They were microscopically characterized by (i) predominant location in the upper portion of the lamina propria; (ii) no submucosal involvement; (iii) less variation in vacuolar size; and (iv) no association with lymph follicles. The vacuoles of group A contained proteinaceous materials in two of four lesions. Group B (11 lesions) had small elevated mucosa with normal‐looking surface or non‐elevated reddish mucosa. Microscopically, the lesions were mainly located in the lower portion of the lamina propria, occasionally also in the submucosa, had variable‐sized vacuoles, and were related to lymph follicles. Conclusion: It is suggested that the vacuoles in group A contain fluid, and may indicate an abnormal stagnation of interstitial fluid. Microscopic appearance of group B is essentially similar to that of pneumatosis coli. It is thought that group B probably results from penetration of gas from the crypts into the mucosa during colonoscopy. It is unclear why group B had a preference for ileocecal valve and an association with lymph follicles.     

Colonoscopic classification of internal hemorrhoids: Usefulness in endoscopic band ligation

Background:  Bleeding is one of the main symptoms of internal hemorrhoids. However, the conventional Goligher's classification of internal hemorrhoids does not consider the severity of bleeding. We intended to establish a useful method for evaluating internal hemorrhoids using a colonoscope that reflected the severity of the symptoms.
Methods:  Using a colonoscope in the retroflexed and forward viewing position, 104 patients with symptomatic internal hemorrhoids were evaluated based on the criteria of range, form and red color signs (RCS). Range was determined by the circumferential distribution of internal hemorrhoids and scaled from 0 to 4. Form was determined by size and scaled from 0 to 2. The presence of RCS was also evaluated. Symptoms were determined by interview and scaled from 0 to 3. Patients were treated by endoscopic band ligation (EBL) and were examined endoscopically before and 4 weeks after the treatment.
Results:  Before the treatment, range, form and RCS were significantly correlated to bleeding ( P <  0.01), and form was significantly correlated to prolapse ( P <  0.05). The endoscopic classification scores at 4 weeks after EBL improved significantly (range from 3.25 ± 0.05–0.56 ± 0.08 [ P <  0.01] and form from 2.81 ± 0.04–0.56 ± 0.07 P <  0.01).
Conclusion:  The new endoscopic classification of internal hemorrhoids proved to be closely correlated to symptoms, particularly bleeding, and thus highly useful in evaluating the effectiveness of the treatment.     

Serum markers for fibrosis and plasma transforming growth factor-β1 in patients with hepatocellular carcinoma in comparison with patients with liver cirrhosis

In order to elucidate collagen metabolism in hepatocellular carcinoma (HCC) tissue, we compared levels of different potential markers of collagen metabolism and plasma transforming growth factor-β₁ in patients with HCC and in patients with liver cirrhosis. Serum levels of prolyl hydroxylase and the tissue inhibitor of metalloproteinase-1 in patients with HCC were significantly higher than those in patients with liver cirrhosis and increased with the size of the HCC tumour, whereas the serum levels of procollagen type III propeptide and type IV collagen 7S domain were similar in the two groups. In HCC, the increased plasma transforming growth factor-β₁ levels were closely correlated with serum levels of prolyl hydroxylase and the tissue inhibitor of metalloproteinase-1. These findings suggest that, in HCC tissue, the intracellular biosynthesis of collagen is enhanced, whereas the secretion of procollagen is disturbed and the degradation of collagen is suppressed by the excess production of the tissue inhibitor of metalloproteinase-1. The results also suggest that plasma transforming growth factor-β₁ plays an important role in the altered metabolism of collagen in HCC.     

ALCOHOL-RELATED SUDDEN DEATH WITH HEPATIC FATTY METAMORPHOSIS: A COMPREHENSIVE CLINICOPATHOLOGICAL INQUIRY INTO ITS PATHOGENESIS

To clarify the pathogenesis of the widely known but obscuresyndrome of sudden death with hepatic fatty metamorphosis observedin alcohol abusers, we have scrutinized both the clinical andpathological data of 11 subjects who died under such circumstancesbetween 1987 and 1993. Death followed several days of uninterrupteddrinking often with little dietary intake. The notable clinicalfeatures on arrival at the emergency room were disturbance ofconsciousness (11/11), hypotension (47/6), hypothermia (3/5),hypoglycaemia (8/11), metabolic acidosis (6/6), renal dysfunction(11/11), and hyperammonaemia (5/5). The common hepatic pathologywas the extensive appearance of numerous microvesicular fattydroplets in the hepatocytes together with varying degrees ofmacrovesicular fatty change; four subjects had an underlyingcirrhosis. Death undoubtedly results from a variety of metabolicdisturbances triggered by the combination of massive ethanolintake and starvation. The appearance of extensive microvesicularfatty change superimposed on macrovesicular fatty change wasconsidered to be an associated phenomenon     

Correspondence: Nerve–muscle specificity

A correspondence item entitled 'A warning against revival of the classic tenets of gross anatomy related to nerve–muscle specificity' was recently published in the Journal of Anatomy (Shinohara, 1996 a ). The present correspondent fully agrees with some aspects of his opinion, in particular, the 3 laws about applications which he described, and he also appreciates his approach to the argument based on the results of embryology and experimentation. However, his denial of morphological interpretations based on the theory of nerve–muscle specificity may be due to an incomplete understanding of the current status of the theory.     

Clinical features of Japanese children and adolescents with systemic lupus erythematosus: Results of 1980–1994 survey

Marked advances have been made in the past decade in the management of adults with systemic lupus erythematosus (SLE). Therefore, a nationwide retrospective survey was conducted between 1980 and 1994 to investigate the clinical manifestations of SLE in Japanese children and adolescents. Questionnaires were sent to 340 hospitals. Of 405 patients reported by 176 hospitals, 373 patients, diagnosed by the criteria established by the Pediatric Study Group of the Japanese Ministry of Health and Welfare in 1985, were enrolled in the study. Forty-nine of the 354 patients (13.8%) had relatives with a connective tissue disease within the third degree of consanguinity. The frequent manifestations in 373 patients were the presence of antinuclear antibody (98.9%), immunologic disorders (93.0%), hypocomplementemia (87.1%), malar rash (79.6%) and fever (74.0%). Lupus nephritis was present in 148 of the 309 patients (47.9%) at their first visit to a clinic, and 261 of the 373 patients (70.0%) developed renal involvement during the observation period. Of 370 patients, 92 patients (24.9%) exhibited central nervous system lupus. Of 368 patients, 192 patients (52.2%) were treated by methylprednisolone pulse therapy and 148 patients (40.2%) received immunosuppressants in combination with steroid therapy at some stage during the observation period. Survival rate at 5 years from onset was 95.9%. Management of infection, coagulopathies, and central nervous system involvement is essential to improve the prognosis of SLE in Japanese children and adolescents.     

Coronary risk factors in school children in relation to their family history of coronary heart disease and hyperlipidemia

A school-based study was implemented to assess the family history of coronary heart disease (CHD) and hyperlipidemia (HL) in relation to serum lipoprotein and apolipoprotein levels. One hundred and twenty-five elementary school students (aged9–10 years) and 297 junior high school students (aged12–13 years) participated. Family history was evaluated by the following scoring method: positive family history in a parent. 2 points: in a grandparent. 1 point: and onset of CHD before age 60, 1 additional point. Family history of HL was positive in 8.2% of elementary school students, and 4.2% in junior high school students. Family history of CHD was positive in 11.5% of elementary students, and 11.0% in junior students. Family history score (FHS) for HL was related to serum total cholesterol (TC), low density lipoprotein cholesterol (LDLC), high density lipoprotein cholesterol, apolipoprotein A-T, apolipoprotein B (apoB) and lipoprotein (a) in elementary students, and to TC, LDLC, triglyceride and apoB in junior students. There was no relationship between FHS for CHD and serum lipoprotein or apolipoprotein levels in any student. The children with a positive FH of HL already demonstrated an atherogenic lipid profile while those with FH of CHD did not. which was probably because lipid profiles in children are more genetically mediated by a FH of HL than of CHD.     

Liver transplantation in a case of hypoproteinemia and coagulopathy

A female infant with hypoproteinemia and coagulopathy associated with hypertyrosinemia was successfully treated with living-related liver transplantation (LRLT). On the 12th day of life plasma amino acid analysis revealed a marked elevation of tyrosine, so the patient was fed on a low-tyrosine and low-phenylalanine diet. However, hepatosplenomegaly. hypotonia, alopecia, eczema and psychomotor delay did not improve and recurrent episodes of disseminated intravascular coagulation (DIC) caused her condition to deteriorate. Liver biopsy on the 230th day revealed marked fatty change accompanied by mild to moderate cholestasis. Therefore. LRLT from her father was performed on the 286th day resulting in improvement of all the aforementioned signs and symptoms. Despite a thorough examination, no diagnosis of a known disorder could be established. However, her elder brother had also been born with severe hypoproteinemia and coagulopathy, and died of DIC on the second day of life. Thus, the disorder is designated as a new entity, namely ‘congenital hypoproteinemia and coagulopathy associated with hypertyrosinemia’.