Perforation of the gallbladder: analysis of 19 cases.

Perforation of the gallbladder occurred in 19 (3.8%) of 496 patients with acute cholecystitis treated at one hospital in an 8-year period. The average age of the 19 patients was 69 years and the female:male ratio was 3:2. Most had a history suggestive of gallbladder disease and most had coexisting cardiac, pulmonary, renal, nutritional or metabolic disease. The duration of the present illness was short, perforation occurring within 72 hours of the onset of symptoms in half the patients; the diagnosis was not suspected preoperatively in any. In the elderly patient with acute cholecystitis who has a long history of gallbladder disease, cholecystectomy should be performed early, before gangrene and perforation of the gallbladder can occur.     

Prevalence of gallstones and gallbladder disease in Canadian Micmac Indian women.

The prevalence of gallstones and gallbladder disease was studied between October 1973 and June 1976 in Canadian Micmac Indian women aged 15 to 50 years in an inland rural community near Shubenacadie, NS. Of 132 women at risk 98 underwent cholecystography, 6 had a history of cholecystectomy (verified from hospital records) and 3 had cholecystectomy because of cholecystitis during the 3 years of the study. Of the 17 abnormal cholecystograms 10 showed radiolucent gallstones, and repeated studies documented gallstones in 6 of the 7 radiographs on which the gallbladder was not visualized. The prevalence of gallstones was found to be 211/1000, and that of gallbladder disease, 240/1000. The peak prevalence was at 30 to 39 years of age. The women with gallbladder disease were significantly more obese and of greater parity than those without gallbladder disease even when age was controlled. The Micmac Indian women of Nova Scotia appear to be at a much higher risk for the development of cholesterol gallstones and gallbladder disease than Caucasian women in Framingham, Massachusetts.     

Diagnosis and Treatment of Xanthogranulomatous Cholecystitis: A Report of 39 Cases

This study focuses on providing diagnosis and treatment for xanthogranulomatous cholecystitis (XGC). Clinical data from 39 patients diagnosed with XGC by pathological examination between 2002 and 2010 were analyzed retrospectively. As a result, in this group of patients, the male to female ratio was 30:9 and the average age of XGC onset was 62.2?years. Clinical manifestation of the disease was similar to general cholecystitis and preoperative CT examination showed that there were only 4 XGC cases, while the others were possibly misdiagnosed. Intraoperative observations showed that all the patients had gallbladder wall thickening. This was associated with gallbladder stones in 37 patients (94.9?%), choledocholith in 11 patients (28.2?%), and Mirizzi syndrome in 5 patients (12.8?%). In this study, intraoperative frozen section pathology was conducted in 14 patients and no gallbladder cancer was found. Laparoscopic cholecystectomy was performed on 7 patients, of which two were transferred to laparotomy. Of the remaining 32 cases, 25 were subjected to open cholecystectomy, 3 to partial cholecystectomy, and 4 to the cholecystectomy and partial liver wedge resection. It was concluded that XGC is a unique type of cholecystitis with atypical clinical manifestations and is often difficult to diagnose preoperatively. Pathological examination is a key to diagnose XGC and cholecystectomy is the primary surgical treatment. In patients with choledochectasia or jaundice, for whom we cannot exclude calculus of common bile duct, common bile duct exploration should be considered. The prognosis of XGC appears to be good with the above approaches.     

Actue Cholecystitis Following Unrelated Surgery

In a series of 26 cases of acute cholecystitis occurring after an operation for an unrelated condition, 88% of the patients were over 50 years of age and males outnumbered females by 2 to 1. In some of the cases diagnosis was difficult and delay was responsible for the death of one patient. Acalculous cholecystitis occurred in 20% of the cases and in these gangrene or perforation supervened early in the course of the disease.Efforts should be directed to ensuring adequate hydration after operation; resumption of a diet low in fat may be important. Even in the absence of a history of biliary disease, there is a place for the radiological study of the biliary tract before major elective operations are performed. If gallstones are discovered on the occasion of the initial surgery, cholecystectomy should be performed whenever it is feasible. In any patient with postoperative cholecystitis early operation is generally indicated.     

Gallbladder carcinoma: a retrospective analysis of twenty-two years experience of a single teaching hospital

BACKGROUND: The purpose of this study was to retrospectively evaluate our experience with gallbladder cancer since the establishment of a tumour registry in our institute. METHODS: Between 1975 and 1998, 23 consecutive patients with gallbladder cancer were identified using the tumour registry database. There were 18 females (78%) and 5 (22%) males. The mean age at diagnosis was 70.6 (range 42-85) years. The diagnosis was achieved either intra-operatively or following the histological analysis of the gallbladder (n = 17), following gallbladder or liver biopsy (n = 4) or at autopsy (n = 2). Presenting symptoms included upper abdominal pain, weight loss, nausea, vomiting, fever, painless jaundice, hepatomegaly, upper abdominal mass, upper abdominal tenderness, and gastrointestinal haemorrhage. RESULTS: Histological examination revealed 20 adenocarcinomas (87%), 2 squamous cell carcinomas (9%) and one spindle cell sarcoma (4%). At presentation, 14 (61%) gallbladder cancers were stage IV, 5 (22%) were stage III and 4 (17%) were stage II. Kaplan Meier analysis revealed a mean survival of 3.2, 7.8 and 8.2 months for stage IV, III, and II disease respectively. Out of 14 patients with stage IV disease, 8 patients received adjuvant chemotherapy and survived for 4.6 months whereas six patients who did not receive adjuvant chemotherapy survived for 1.3 months. This difference was statistically significant (p = 0.04). CONCLUSION: The majority of patients with gallbladder cancer presented with advanced stage disease (stage IV) which carries a dismal prognosis. Patients who received chemotherapy with stage IV disease, however, did better than those who did not, but this is probably a reflection of patient selection.     

Congenital protein C deficiency and thrombotic disease in nine French families.

Investigation of 118 patients for protein C deficiency using an immunological and a functional assay, and subsequent investigation of those (nine) found to be deficient, identified 22 patients (14 women, eight men) with protein C deficiency, of whom six were asymptomatic, 15 had histories of venous thromboembolism, and one had a history of arterial thromboembolism. Protein C deficiency was associated in the nine probands with young age at first episode of thromboembolic disease (mean 24.1 (SD 11.9) years), absence of a precipitating condition (five (56%], and a family history of thromboembolic disease (six (66%]. Investigation of the nine families suggested autosomal dominant transmission of the defect. Thromboembolic episodes were seen in patients with protein C antigen concentrations below 0.6 U/ml. Mean (SD) protein C antigen concentrations were 0.48 (0.12) U/ml in 18 patients not receiving oral anticoagulant treatment and 0.28 (0.05) U/ml in four receiving such treatment. One patient with severe protein C deficiency (0.16 U/ml) developed skin necrosis soon after starting oral anticoagulant treatment.     

超声引导下经皮经肝胆囊置管治疗老年化脓性胆囊炎伴胆囊积液的应用价值

目的：探讨超声引导下经皮经肝胆囊置管引流治疗化脓性胆囊炎伴胆囊积液的应用价值。方法：老年患者不能耐受手术或不愿手术治疗,胆囊颈部结石嵌顿引起的化脓性胆囊炎伴胆囊积液患者86例,在超声引导下,8F猪尾巴导管经皮经肝进行胆囊穿刺置管治疗。治疗后用超声进行随访观察,结合临床症状判断疗效。结果：在超声引导下,86例化脓性胆囊炎伴胆囊积液患者均成功进行胆囊穿刺置管引流冲洗治疗,患者症状明显改善,经治疗临床症状消失后出院,择期拔管。结论：超声引导下经皮经肝胆囊置管微创治疗是不能手术或不愿接受手术的化脓性胆囊炎伴胆囊积液患者的一种有效治疗方法。     

超声引导下经皮经肝胆囊置管治疗老年化脓性胆囊炎伴胆囊积液

目的:探讨超声引导下经皮经肝胆囊置管引流治疗化脓性胆囊炎伴胆囊积液的应用价值。方法:老年患者不能耐受手术或不愿手术治疗,胆囊颈部结石嵌顿引起的化脓性胆囊炎伴胆囊积液患者86例,在超声引导下,8F猪尾巴导管经皮经肝进行胆囊穿刺置管治疗。治疗后用超声进行随访观察,结合临床症状判断疗效。结果:在超声引导下,86例化脓性胆囊炎伴胆囊积液患者均成功进行胆囊穿刺置管引流冲洗治疗,患者症状明显改善,经治疗临床症状消失后出院,择期拔管。结论:超声引导下经皮经肝胆囊置管微创治疗是不能手术或不愿接受手术的化脓性胆囊炎伴胆囊积液患者的一种有效治疗方法。     

Quantitative analysis of troponin I serum values in patients with acute cholecystitis

The diagnosis and staging of acute cholecystitis, upon a lot of diagnostic methods and some scoring systems, is still a great clinical problem. The aim of the study was to investigate if serum Troponin I is elevated in patients with acute cholecystitis. Following informed consent, 65 patients with clinical and laboratory signs of acute cholecystitis were enrolled. All patients had measured serum Troponin I level and an abdominal ultrasound was done before definitive treatment was performed. Increased serum Troponin I level was found in most patients with severe form of acute cholecystitis (p < 0.00001). It reached sensitivity of 94.5% and specificity of 57.1% of this test. In multiple regression analysis Troponin I significantly correlated (p < 0.05) with the serum aspartate aminotransferase (r = 0.27), gamma-glutamyl transferase (r = 0.25) and gallbladder wall (> 6 mm) thickness (r = 0.58). Our study confirms that in most patients with severe and acute cholecystitis, serum Troponin I is increased. Troponin I level is in a lower range than it would be in patients with cardiac muscle damage or necrosis. Measuring serum Troponin I is a fast, reliable and widely performed test that could, with other routinely measured parameters, help in early diagnosis of the severe form of acute cholecystitis.     

Detection of Helicobacter hepaticus in Human Bile Samples of Patients with Biliary Disease

Background:  Since the discovery of Helicobacter pylori , various enterohepatic Helicobacter spices have been detected in the guts of humans and animals. Some enterohepatic Helicobacters have been associated with inflammatory bowel disease or liver disease in mice. However the association of these bacteria with human diseases remains unknown.
Materials and Methods:  We collected 126 bile samples from patients with cholelithiasis, cholecystitis, gallbladder polyp, and other nonbiliary diseases. Samples were screened for the presence of enterohepatic Helicobacter spp. using cultures, nested PCR, or in situ hybridization. We tested for antibodies to H. pylori and H. hepaticus by Western blot analysis.
Results:  Attempts at cultivation were unsuccessful. However, H. hepaticus was detected in bile samples with nested PCR whereas H. bilis was not. Helicobacter hepaticus in the bile was confirmed by in situ hybridization, but H. hepaticus from bile samples was coccoid in appearance. We detected immunoglobulin G antibodies to H. hepaticus in bile samples by Western blotting. Helicobacter hepaticus was detected in 40 (32%) of total 126 samples as H. hepaticus positive if at least one of the three methods with nested PCR, in situ, or Western blotting. Patients with cholelithiasis (41%) and cholecystitis with gastric cancer (36%) had significantly higher ( p  = .029) prevalence of H. hepaticus infection than samples from patients with other diseases.
Conclusion:  Helicobacter hepaticus may closely associate with diseases of the liver and biliary tract in humans.     

Neutrophils injure gallbladder interstitial Cajal-like cells in a guinea pig model of acute cholecystitis

Acute cholecystitis is a common disease with gallbladder dysmotility. Disease pathogenesis involves immune cell infiltration as well as changes in gallbladder interstitial Cajal-like cells (ICLCs). However, it remains unclear if or how the immune cells affect ICLC morphology, density, distribution, and function in gallbladder tissue during acute cholecystitis. In this study, we explored the acute cholecystitis-related alterations in gallbladder ICLCs in a guinea pig model, focusing on the effects of neighboring neutrophils. Adult guinea pigs were randomly divided into four groups (control, 24 hr common bile duct ligation [CBDL], 48-hr CBDL, and antipolymorphonuclear neutrophil [PMN] treated) and analyzed using methylene blue staining and immunofluorescence. Gallbladder contractility was also monitored. To culture gallbladder ICLCs, collagenase digestion was performed on tissue from 10- to 15-day-old guinea pigs. Neutrophils isolated from the peripheral blood of experimental animals 48-hr postsurgery were also cocultured with the gallbladder ICLCs. Intracellular calcium was detected with Fluo-4 AM dye. Our results showed that gallbladder ICLC density significantly declined during acute cholecystitis and was accompanied by shortening of the cellular processes and damage to their network-like structure. However, pretreatment with anti-PMN partially prevented these changes. Gallbladder contraction was also significantly decreased during acute cholecystitis, and this appeared to be mediated by the neutrophils. Moreover, ICLCs cocultured with neutrophils also had shortened and reduced processes and impaired network-like structure formation. Intracellular calcium transient was less sensitive to contraction agonists and inhibitors when cocultured with neutrophils. Taken together, neutrophils greatly affect gallbladder ICLCs and dysmotility during acute cholecystitis.     

The cystic fibrosis transmembrane conductance regulator gene and ion channel function in patients with idiopathic pancreatitis

Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations are associated with cystic fibrosis (CF)-related monosymptomatic conditions, including idiopathic pancreatitis. We evaluated prospectively enrolled patients who had idiopathic recurrent acute pancreatitis or idiopathic chronic pancreatitis, healthy controls, CF heterozygotes, and CF patients (pancreatic insufficient or sufficient) for evidence of CFTR gene mutations and abnormalities of ion transport by sweat chloride and nasal potential difference testing. DNA samples from anonymous blood donors were controls for genotyping. At least one CFTR mutation or variant was carried in 18 of 40 patients (45%) with idiopathic chronic pancreatitis and in 6 of 16 patients (38%) with idiopathic recurrent acute pancreatitis but in only 11 of the 50 controls (22%, P=0.005). Most identified mutations were rare and would not be identified in routine genetic screening. CFTR mutations were identified on both alleles in six patient (11%). Ion transport measurements in patients with pancreatitis showed a wide range of results, from the values in patients with classically diagnosed CF to those in the obligate heterozygotes and healthy controls. In general, ion channel measurements correlated with the number and severity of CFTR mutations. Twelve of 56 patients with pancreatitis (21%) fulfilled current clinical criteria for the diagnosis of CF, but CFTR genotyping alone confirmed the diagnosis in only two of these patients. We concluded that extensive genotyping and ion channel testing are useful to confirm or exclude the diagnosis of CF in the majority of patients with idiopathic pancreatitis.     

DICER1 mutations in twelve Chinese patients with pleuropulmonary blastoma

Our aim is to examine the impact of DICER1 mutations on the pathogenesis of pleuropulmonary blastoma(PPB) by evaluating the mutation frequency and investigating the family history of Chinese patients with PPB. The family histories of 12 children with PPB recruited consecutively were surveyed. Blood samples from patients and their first-degree relatives were tested for DICER1 mutations. Whole-genome sequencing of blood samples and formalin-fixed and paraffin-embedded(FFPE) tumor tissue was performed in one family with twins. Twelve patients with PPB included six type II and six type III cases. Seven of the12 patients harbored DICER1 mutations, six of which were frameshift or nonsense mutations. Another case carried a germline DICER1 mutation affecting the splice site. FFPE sample had a nonsense mutation in TDG and missense mutations in DICER1.In addition, two cases with DICER1 mutations were found to have lung cysts preceding the diagnosis of PPB. Furthermore, one patient had a family history remarkable for thyroid diseases. Our results indicate that the germline mutation frequency in Chinese patients with PPB is similar to the ones reported for patients from USA, UK, and Japan. Moreover, our study strongly suggests that investigating the family history and detecting germline DICER1 mutations might be of benefit to increasing awareness and improving the accuracy of the differential diagnosis of PPB from non-malignant lung cysts.     

Treatment of Nelson's syndrome by pituitary implantation of yttrium-90 or gold-198.

Eight patients with Nelson''s syndrome were treated with a pituitary implant of yttrium-90 or gold-198 four to 16 years after adrenal surgery. All had considerable pigmentation. One already had cranial nerve abnormalities and visual field defects and had had both a craniotomy and deep x-ray treatment. Radiographs showed that the pituitary fossa was abnormal in seven patients. A biopsy performed in six cases showed mucoid (or basophil) adenoma in all. In the four specimens examined ACTH was identified by electron microscopy or immunofluorescence, or both. Patients were followed up after pituitary implantation for three months to 12 years. All showed decreased pigmentation, and six became normal. Four patients regained normal ACTH levels and the other two studied had decreased levels. In no case did new cranial nerve disease or further sellar expansion develop since operation, and two patients showed remodelling of the sella. Complications were temporary leakage of cerebrospinal fluid and diabetes insipidus in one patient and gonadotrophin deficiency in another.     

Wilson's disease: a common liver disorder?

In two sibships 7 of 24 siblings were homozygous for Wilson''s disease. In family A, the largest kindred of this recessively inherited disease thus far reported, the proband presented with chronic active hepatitis, one sibling died of cirrhosis, a second had clinical evidence of chronic liver disease and two others had biochemical and histologic changes in liver biopsy specimens. In family B the proband had cirrhosis and portal hypertension and one sibling had biochemical and histologic evidence of liver disease. All six living patients had low serum concentrations of ceruloplasmin and copper and a high 24-hour urinary excretion of copper, which was greatly increased by administration of D-penicillamine. None showed neurologic abnormalities and only one had Kayser-Fleischer rings (detectable only by slit-lamp examination). Each patient had an erythrocyte sedimentation rate (ESR) of 8 mm/h or less. After 3 and 2 years, respectively, of D-penicillamine therapy the conditions of the two probands had improved. Liver function became normal in three siblings, and no abnormalities developed in the remaining one. Thus, since Wilson''s disease may present with chronic active hepatitis or cirrhosis with a normal ESR and without ocular or neurologic signs, it may be a more common cause of liver disease in young people than has been appreciated.     

Staphylococcus aureus cholecystitis: a report of three cases with review of the literature

Infection of the hepatobiliary system is most commonly due to enteric bacteria. We report three unusual cases of acute cholecystitis in which Staphylococcus aureus was the primary pathogen. Infection of the gallbladder with this organism has been rarely described and may be associated with gallstones and obstructive disease as well as acalculous cholecystitis in the setting of staphylococcal bacteremia and endocarditis. Two of our patients had multiple chronic medical conditions and were infected with oxacillin-resistant S. aureus (ORSA) suggesting nosocomial acquisition. Including our cases with a review of the literature, three of nine reports of S. aureus cholecystitis were associated with infectious endocarditis. Thus, the finding of S. aureus cholecystitis with bacteremia is rare and should prompt an investigation for a possible endovascular focus of infection.     

Primary carcinoma of the gallbladder; review of 173 cases

One hundred seventy-three cases of primary carcinoma of the gallbladder were analyzed. In the group studied they made 2.11 per cent of all malignant tumors found at autopsy and were found in 1.89 per cent of all cases in which operation was done on the biliary tract. There was no appreciable change in the incidence of this tumor at autopsy during the period studied (1918-1948) at the Los Angeles County Hospital. Sixty-eight per cent of the cases were in females. A particularly high incidence was noted in Mexican females. Upper abdominal pain, loss of weight, nausea and vomiting, jaundice, and palpable mass or enlarged liver were the most common clinical features. Approximately one-third of the patients in whom the lesion was found at operation and one-fifth of all the patients whose records were studied had a history of chronic gallbladder disease. All but two of the 38 patients operated on were dead or had clinical recurrence within two years. One was alive and well 12 years after cholecystectomy. The most common gross appearance, particularly at autopsy, was a large tumor mass replacing the gallbladder and radiating to nearby organs, particularly the liver. In about one-third of the cases the tumor was grossly limited to the gallbladder. Polypoid tumors occurred in only about 10 per cent of the cases and most of the tumors were diffusely growing adenocarcinoma. Perforation appeared in nine cases, usually with fistula to the gastrointestinal tract. All of the tumors were histologically adenocarcinoma, usually of simple glandular structure. No purely squamous cell growth occurred. Gallstones were found in 79.8 per cent of the cases.     

PRIMARY CARCINOMA OF THE GALLBLADDER—Review of 173 Cases

One hundred seventy-three cases of primary carcinoma of the gallbladder were analyzed. In the group studied they made 2.11 per cent of all malignant tumors found at autopsy and were found in 1.89 per cent of all cases in which operation was done on the biliary tract. There was no appreciable change in the incidence of this tumor at autopsy during the period studied (1918-1948) at the Los Angeles County Hospital. Sixty-eight per cent of the cases were in females. A particularly high incidence was noted in Mexican females.Upper abdominal pain, loss of weight, nausea and vomiting, jaundice, and palpable mass or enlarged liver were the most common clinical features. Approximately one-third of the patients in whom the lesion was found at operation and one-fifth of all the patients whose records were studied had a history of chronic gallbladder disease.All but two of the 38 patients operated on were dead or had clinical recurrence within two years. One was alive and well 12 years after cholecystectomy.The most common gross appearance, particularly at autopsy, was a large tumor mass replacing the gallbladder and radiating to nearby organs, particularly the liver. In about one-third of the cases the tumor was grossly limited to the gallbladder. Polypoid tumors occurred in only about 10 per cent of the cases and most of the tumors were diffusely growing adenocarcinoma. Perforation appeared in nine cases, usually with fistula to the gastrointestinal tract. All of the tumors were histologically adenocarcinoma, usually of simple glandular structure. No purely squamous cell growth occurred.Gallstones were found in 79.8 per cent of the cases.     

Subacute sclerosing panencephalitis--the continuing threat

Clinical, epidemiological and laboratory findings of four patients with subacute sclerosing panencephalitis (SSPE), diagnosed in Croatia in 2002, were examined. Patient age at disease onset ranged from 5-11 years. All patients were vaccinated regularly with MMR-vaccine. Two patients had a history of measles infection at the age of six and seven months, respectively. In the other two patients, the disease started immediately after the varicella infection. Complement fixing antibody titre to the measles virus (MV) ranged from 1:1024 to 1:65536 in serum, and from 1:16 to 1:128 in cerebrospinal fluid (CSF). In CSF, no antibodies to varicella-zoster virus were found. Brain tissue samples were obtained at autopsy from two patients. In one patient, electron microscopy demonstrated intranuclear viral inclusions (MV nucleocapsids). MV antigen was detected in brain imprints using IFA in both of them. Viral RNA was found in brain tissue samples only, while plasma, serum and CSF were negative. Nucleotide sequence analysis showed that the viruses detected in brain tissue belong to the wild-type MV D6 genotype.