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At least 289 records · Page 16Linked to original sources

Bilateral intrahepatic lithiasis without extrahepatic bile duct stones.

Bilateral intrahepatic lithiasis is a rare condition, and for this reason a nationwide survey was conducted. Reports on 675 patients with bilateral intrahepatic lithiasis over a 10-year-period were collected. Among these, 258 patients with bilateral intrahepatic lithiasis having no extrahepatic bile duct stones were analyzed. The peak incidence was seen in the fourth to sixth decades. Males and females were equally effected. The stones removed were mainly calcium bilirubinate stones (75.6%). The main clinical symptoms were abdominal pain, fever and jaundice. Charcot's triad was seen in 29.7%, while 12.4% of the patients had no symptoms. Visualization of each segmental duct of the liver by direct cholangiography was excellent in this survey and ranged from 88 to 97.3% of the patients. The most frequent site of stones was the left hepatic duct (60.1%). The site of bile duct dilatation coincided with the location of stones. The most common sites of stenosis were the central part of the lateral segmental duct (32.5%) and the left hepatic duct (37.6%). Hepatic resection was employed in 49.2% of the patients, and drainage procedures were added in 95.6%. Follow-up studies of 236 patients treated by surgery revealed good results in 67.4%, fair in 13.6%, and poor in 7.6%. In these patients, however, endoscopic lithotomy was often employed intra- and/or post-operatively. Conducting hepatic resection, with adequate biliary drainage procedure and cholangiofiberscopic lithotomy may help to improve the therapeutic results of bilateral intrahepatic lithiasis.

Adult↗

[The "nutcracker syndrome" of the renal vein (superior mesenteric artery syndrome) as the cause of gastrointestinal complaints].

HISTORY AND CLINICAL FINDINGS: Since the age of 19 a now 22-year-old man had complained of intermittent abdominal pain, irregular stools and paroxysmal tachycardia. The only preceding illness had been a single episode of iron-deficiency anemia. A laparoscopy, done 8 months after the onset of symptoms, had revealed an inflamed Meckel's diverticulum which was surgically removed. After transient improvement the symptoms recurred 5 months postoperatively. On admission to clarify the cause of the symptoms he had discrete abdominal pain on pressure, but physical examination was otherwise unremarkable. INVESTIGATIONS: Routine biochemical tests and endoscopy were normal. Abdominal computed tomography was suspicious of severe narrowing of the left renal artery by a crossing superior mesenteric artery. As a result the left testicular vein and the peripelvic venous network were markedly dilated by retrograde congestion, strongly suggesting the "nutcracker syndrome" of obstruction of the left renal vein. This diagnosis was confirmed by selective renal phlebography and pressure measurement. TREATMENT AND COURSE: The vascular anomaly was corrected surgically by reimplanting the left renal vein into the inferior vena cava 3-4 cm further caudally. The patients has been completely symptom-free since then. CONCLUSIONS: The nutcracker-syndrome is a rare cause of hematuria. The coexistence of this anomaly with gastrointestinal symptoms has not been previously described, but it is likely that congestion of the splanchnic veins by obstruction of the left renal vein was at least partly responsible for them, in view of the postoperative relief.

Abdominal Pain↗

Virtual endoscopic view of salivary gland ducts using MR sialography data from three dimension fast asymmetric spin-echo (3D-FASE) sequences: a preliminary study.

OBJECTIVES: We performed magnetic resonance (MR) sialography of parotid gland and/or submandibular gland ducts using three-dimensional fast asymmetric spin-echo (3D-FASE) sequencing. The objective was to make three-dimensional (3D) reconstruction images and virtual endoscopic views of the parotid gland ducts using MR sialography data sets of 3D-FASE sequences. METHODS: We reviewed the MR sialography data sets with 3D-FASE sequencing of 10 control volunteers and six patients. Three-dimensional reconstruction images and virtual endoscopic views of the parotid gland and/or submandibular gland ducts were generated with maximum intensity projection (MIP), shaded surface display (SSD), and volume rendering techniques (VRT). RESULTS: The main parotid gland and/or submandibular gland ducts, large branches within the glands, and small branches were fairly well defined in a very short acquisition time on MR sialographic images with 3D-FASE sequencing in nine of the 10 healthy volunteers. The 3D-reconstruction images of the parotid gland ducts and/or submandibular gland ducts showed the entire length of the branch paths and complete images from all angles, and the virtual endoscopic views showed the endoluminal tracts of the main ducts and the large branches in nine. In the patient with Sjogren's syndrome, chronic sialoadenitis, and salivary calculi in the Wharton ducts, the MR sialographic images showed diffuse areas of punctate high signal intensity, dilatation of Stensen's duct, or stones of Wharton's duct, respectively. Furthermore, the 3D-reconstruction images of the salivary gland ducts showed the stenoses and stones in the branch paths and complete images from all angles, and the virtual endoscopic views showed the stenoses and stones in the endoluminal tracts of the main and large branches. CONCLUSIONS: Our initial experience showed that virtual MR endoscopy could be performed to observe the endoluminal tracts of parotid and submandibular glands. The clinical use of the virtual MR endoscopy for salivary gland ducts has not been established yet. Future applications of the 3D-reconstruction images and virtual endoscopic views using MR sialography data sets of 3D-FASE sequences are very attractive and further expansion of this field is expected.

Adolescent↗

Cardiomyopathies with particular reference to the diagnostic relevance of endomyocardial biopsies.

A distinction is made between heart muscle diseases with a known cause, or occurring as part of a systemic disease, and those of unknown cause. The first category is termed "specific heart muscle disease". The term "cardiomyopathy" is then limited to the latter category. Cardiomyopathies are categorized as dilated cardiomyopathy, hypertrophic cardiomyopathy and restrictive cardiomyopathy. The pathology of dilated cardiomyopathy depends on the clinical stage of the disease. Basically, the histology shows hypertrophic myocytes, often with degenerative signs, and interstitial fibrosis. Lymphocytes may be observed, particularly in cases studied shortly after the onset of symptoms. Endocardial thickening may occur in time. The role of endomyocardial biopsies in the clinical setting is limited and relates mainly to excluding other diseases, such as myocarditis. The pathology of hypertrophic cardiomyopathy is characterized by myocardial wall thickening, either asymmetric or symmetric, and disorganization of the normal myocardial texture. The latter phenomenon should be distinguished from disarray, since it may occur as a natural phenomenon. The differentiation between these two may be extremely difficult on the basis of only small tissue samples, as with endomyocardial biopsies. This, therefore, poses serious limitations in the use of endomyocardial biopsies. The pathology of restrictive cardiomyopathy also depends on the stage of the disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Biopsy↗

Idiopathic tumefactive chronic pancreatitis: clinical profile, histology, and natural history after resection.

BACKGROUND & AIMS: Little is known about subjects with idiopathic tumefactive chronic pancreatitis (TCP), that is, chronic pancreatitis whose clinical presentation, usually with a mass or obstructive jaundice, suggests cancer. METHODS: We independently reviewed clinical data and histology of 45 TCP (27 idiopathic, 18 alcohol induced) resected at Mayo Clinic (January 1985-March 2001). Follow-up data were obtained from medical records and mailed questionnaires. RESULTS: Compared with alcoholic subjects, idiopathic TCP patients were older (58 +/- 2 vs. 48 +/- 3 yr, P < 0.001), had shorter symptom duration (median 3 vs. 24 wk, P < 0.001), were more likely to have no or mild abdominal pain (70% vs. 17%, P = 0.001), and were more often jaundiced (67% vs. 33%, P = 0.02). Three distinct histologic patterns were identified in TCP. Typical CP (n = 19) showed lobular atrophy, fat necrosis, and ductal changes (dilatation, protein plugs, and stones). Lymphoplasmacytic sclerosing pancreatitis (LPSP) (n = 14) was characterized by periductal lymphoplasmacytic infiltration, obliterative phlebitis, and cholangitis with edema. Idiopathic duct-centric CP (IDCP) (n = 12) had neutrophil-predominant lobular inflammation, without phlebitis. On correlation of clinical and histologic diagnosis, 17 of 18 (94%) patients with alcohol-induced TCP had typical CP, and 25 of 27 (93%) with idiopathic TCP had LPSP or IDCP. LPSP and IDCP were indistinguishable clinically except for higher incidence of jaundice in LPSP (93% vs. 42%, P = 0.005). In idiopathic TCP no recurrence of symptoms was observed after resection (median follow-up 49 mo). CONCLUSIONS: Idiopathic TCP is clinically and histologically distinct from alcohol-induced TCP. It is unclear whether LPSP and IDCP, 2 unique patterns of histologic injury observed in idiopathic TCP, are part of the spectrum of the same disease or represent 2 or more different entities. Resection of mass prevents recurrence of symptoms in idiopathic TCP.

Bile Ducts↗

"Dilatation" of the left renal vein on computed tomography in children: a normal variant.

Compression of the left renal vein (LRV) between the superior mesenteric artery and the aorta is thought to be a cause of hematuria, periureteral and gonadal varices, and varicocele ("Nutcracker phenomenon"). Previous investigators have suggested that this diagnosis can be made on computed tomography when the LRV ratio greater than or equal to 1.5 (the diameter of the LRV proximal to the aorto-mesenteric angle divided by the diameter of the LRV distal to the aorto-mesenteric angle). This study was designed to establish the normal range for the LRV ratio on CT in children. The LRV ratio was measured in thirty-nine consecutive children undergoing intravenously enhanced CT of the abdomen. None of the children had hematuria on urinalysis immediately before or after the CT. Children with any known abnormality involving the kidneys, adrenal glands, IVC, or renal or gonadal veins were excluded. The patients ranged in age from 3.4 to 18.5 years (mean = 10.6 years). LRV ratio ranged from 0.78 to 2.00 (mean = 1.46; S.D. = 0.312). Twenty of the 39 children (51.3%) had a LRV ratio greater than or equal to 1.50. The conclusion is that the normal range for the LRV ration is too wide for it to be useful in diagnosing LRV entrapment and that a LRV ratio greater than or equal to 1.5 on CT is normal in children.

Adolescent↗

Current problems with intrahepatic bile duct stones in Japan--congenital biliary malformations as a cause.

BACKGROUND/AIMS: In patients with primary intrahepatic bile duct stones, strictures of the biliary duct are often present, but the relationship between these strictures and the formation of the stones remains controversial. Intrahepatic bile duct carcinoma in association with intrahepatic bile duct stones has recently been reported. The present study attempted to ascertain whether bile stasis induced by congenital biliary strictures is the basis for the formation of stones and occurrence of carcinoma. MATERIALS AND METHODS: We analyzed the location of strictures in 58 patients with strictures in the upper portion of the biliary tract including 38 patients with intrahepatic bile duct stones and 9 with intrahepatic bile duct carcinoma. The cell cycle of epithelial cells from the intrahepatic bile duct were analyzed with using proliferating cell nuclear antigen, which is a immunohistochemical staining method. RESULTS: Fifty six of 58 patients had congenital cystic dilatation of the common bile duct (two infant type and 54 adult type). Thirty eight patients had intrahepatic bile duct stones proximal to the strictures at the hepatic hilum. The location of the strictures were classified into four types. Nine patients had intrahepatic bile duct carcinoma and eight of the 9 carcinomas coexisted with intrahepatic bile duct stones. In the nine patients with intrahepatic bile duct carcinoma, the expression of proliferating cellular nuclear antigen (PCNA) in the carcinoma and the normal bile duct epithelium adjacent to the carcinoma was higher than that of patients with hepatocellular carcinoma without anomaly of the biliary duct. CONCLUSION: Considering the location of the strictures and clinical features, the strictures may have been formed congenitally. Furthermore, adult type cysts of the common bile duct with strictures in the upper portion of the biliary tract are thought to be the basis for the formation of primary intrahepatic bile duct stones. The most appropriate treatment for intrahepatic bile duct stones is thus suggested to be removal of the affected hepatic segment including the region of strictures, combined eventually with hepaticoenterostomy.

Adult↗

Histopathological study of intrahepatic aberrant vessels in cases of noncirrhotic portal hypertension.

Aberrant vessels, which are defined as dilated blood vessels immediately adjacent to the peripheral portal tract, appear under conditions of extrahepatic portal obstruction and nodular regenerative hyperplasia as well as idiopathic portal hypertension. Our study was undertaken to compare their morphological aspects in these three disease cases. Aberrant vessels were found in 84% of cases of idiopathic portal hypertension, 67% of cases of extrahepatic portal obstruction infantile type, 78% of cases of extrahepatic portal obstruction adult type and 83% of cases of nodular regenerative hyperplasia. They were divided into three types: type I--no communication with the portal vein, the lumen of which is normally open; type II--communication with the portal vein; and type III--no communication with the portal vein, which is occluded. The most common types of aberrant vessel were type III in idiopathic portal hypertension (51%), type I in extrahepatic portal obstruction infantile type (46%), type II in extrahepatic portal obstruction adult type (43%) and type III in nodular regenerative hyperplasia (45%). Serial sections revealed transition between types I, II and III, at frequencies between types II and III, types I and II, and types I and III of 35.7%, 33.7% and 30.6%, respectively. Aberrant vessels demonstrated the same immunoreactivity as portal veins for collagen type IV, laminin, factor VIII and ulex europaeus agglutinin-I. They were concluded to arise from the vasa septalis or inlet venules, which would be used as intrahepatic shunts draining portal blood flow blocked by stenosed portal veins. Increased portal pressure would be expected to enhance development of aberrant vessels.(ABSTRACT TRUNCATED AT 250 WORDS)

Blood Vessels↗

Clinical and pathologic study of familial dilated cardiomyopathy.

To evaluate the occurrence of familial cases of dilated cardiomyopathy (DC), 165 consecutive patients were studied. Diagnosis of myocardial disease was based on clinical, hemodynamic, bioptic, postmortem or a combination of these criteria. Twelve patients (7% of cases) showed evidence of myocardial disease in greater than or equal to 1 relative; 27 patients with myocardial disease were detected in the 12 families, but a suspected history of myocardial involvement was present in a further 16 cases. In 6 families proband and relatives were affected by DC (total 14 cases); in 1 of these families the disease began with an atrioventricular block. In 4 families the relatives showed the presence of myocarditis at the endomyocardial biopsy. In 2 families the relatives presented a right ventricular cardiomyopathy. The mode of inheritance was autosomal dominant in 7 families, recessive in 4; X-linked pattern may be hypothesized in 1. Nine patients died under the age of 45 years: 2 of sudden death, 6 of chronic heart failure and 1 of cerebral embolism. Familial transmission is not rare. Different modes of genetic transmission (autosomal dominant, recessive and X-linked) and different forms of myocardial disease suggest that familial DC may be a multifactorial disease.

Adolescent↗

Mitral insufficiency secondary to mucocutaneous lymph node syndrome. A case report of successful surgical treatment.

A three-year-old girl with mitral regurgitation due to mucocutaneous lymph node syndrome (MCLS) but with normal coronary arteries was treated surgically. Although no definite pathologic lesions causing severe mitral regurgitation except for dilatation of the mitral annulus were found at operation, some minor changes implied focal ischemia of the posterior papillary muscle. A poster-medial annuloplasty was performed without significant residual regurgitation. This is the first patient successfully treated by surgery for mitral valve incompentence due to MCLS. However, additional clinical experiences will be needed to evaluate surgical treatment for this group patients.

Capillaries↗

Sonography of vesical and perivesical abnormalities in children.

Pelvic sonograms of 40 children who had abnormalities related to the bladder and perivesical region were reviewed. The lesions detected were categorized into three groups, intramural, intravesical, and perivesical; in each category, conditions not related to vesical or perivesical anomalies that could mimic such anomalies were encountered. Real-time ultrasonography was most helpful in identifying ectopic ureteroceles. Aperistaltic loops of bowel were found to mimic perivesical cysts.

Adolescent↗

Dieulafoy's lesion of the jejunum.

Dieulafoy's lesion is a rare vascular anomaly but a potentially life-threatening disease. This lesion can also be found in the small intestine, which can be diagnosed only by angiography. However, the angiography may be normal when the bleeding is inactive. We report a case of jejunal Dieulafoy's lesion with a repeated attack of massive gastrointestinal bleeding with a normal initial angiography. The pathological examination found an unusual picture as a dilated submucosal vessel protruded like a submucosal tumor.

Adult↗

[Stenosis of the celiac tripod due to extrinsic compression. A case report].

The Authors, starting from the observation of a case of stenosis of the celiac tripod, caused by compression "ab estrinseco", examine the physiopathological, clinical, aetiopathogenetic and therapeutical aspects of the pathology in question. In accordance with medical literature interpretative nosological doubts are also raised which, not with standing the improved research techniques, make it extremely difficult to suggest an accurate surgical procedure.

Abdominal Pain↗