Home parenteral nutrition improves quality of life and nutritional status in patients with cancer: a French observational multicentre study.

PURPOSE: Malnutrition is a predictor of poor outcomes in patients with cancer. Little is known about the benefit of nutritional support in these patients. The purpose of this study was to assess the impact of home parenteral nutrition (HPN) on quality of life (Qol) in cancer patients. METHODS: We performed an observational prospective study to determine the impact of HPN on Qol in a population of patients with heterogeneous cancer. Physicians, patients and family members had to complete a questionnaire before HPN administration and 28 days after the course of HPN. Qol was evaluated using the self-administered questionnaire FACT-G. RESULTS: We included 767 patients with cancer of whom 437 ended the study. Mean patient age was 63±11.4 years and 60.5% were men. Primary gastrointestinal cancer was reported in 50% of patients and 65.3% were presenting metastases. Malnutrition was reported in 98.3%. After 28 days of HPN intake, significant improvement was observed in the Qol (49.95±5.82 vs. 48.35±5.01 at baseline, p<0.0001). The mean weight, serum albumin and the nutrition risk index had also improved significantly. Most patients (78%) had perceived a positive impact of the HPN. A significant improvement in patient's well-being was perceived also by family members and physicians. CONCLUSIONS: Our data suggest that preventing and correcting malnutrition using HPN in patients with cancer might have a significant benefit on their well-being. Randomized controlled studies are required to confirm this finding.

Fibrosis progression occurs in a subgroup of heavy drinkers with typical histological features.

BACKGROUND: Studies using consecutive liver biopsies constitute an attractive approach to gaining insight into the pathogenesis of alcoholic liver disease. AIM: To analyse histological factors at baseline, which are predictive of fibrosis progression and recurrence of alcoholic hepatitis. RESULTS: A total of 193 drinkers underwent consecutive biopsies at an interval of 4 years. At baseline, 20 had normal livers, 135 steatosis, five fibrosis and 33 alcoholic hepatitis. The fibrosis score increased from 1.07 +/- 0.07 to 1.7 +/- 0.94 (P < 0.001). In multivariate analysis, only steatosis (P = 0.04), alcoholic hepatitis (P = 0.0004) and stage of fibrosis (P < 0.0001) were independent predictive factors of the fibrosis score at the second biopsy. Cirrhosis developed more frequently in patients with steatosis (11%) and alcoholic hepatitis (39%) than in others (0%, P < 0.0001). Alcoholic hepatitis recurred more frequently in patients with alcoholic hepatitis at baseline: 58% vs. 15%, P < 0.0001. In multivariate analysis, alcoholic hepatitis at the first biopsy was the only predictive factor of its recurrence (P < 0.0001). CONCLUSIONS: In a large cohort of drinkers with consecutive biopsies, steatosis, fibrosis stage and alcoholic hepatitis at baseline were independent predictive factors of fibrosis progression. In terms of mechanisms, we propose a novel concept of multiple hits of alcoholic hepatitis occurring in the same patient.

Cardioskeletal mitochondrial myopathy associated with chronic magnesium deficiency.

A 3-year-old boy presenting with convulsions and carpopedal spasm had hypomagnesemia and hypermagnesuria due to congenital magnesium-losing nephropathy. Despite chronic oral and intermittent intravenous magnesium supplementation, he remained chronically hypomagnesemic. At age 4, he developed a progressive proximal myopathy and dilated hypertrophic cardiomyopathy that ultimately contributed to his death at age 14 years. Skeletal and cardiac muscle specimens showed a mitochondrial myopathy with increased numbers of enlarged, structurally abnormal mitochondria. Muscle magnesium content was markedly decreased. Chronic oral and intermittent intravenous magnesium supplementation may be inadequate to prevent the progressive cardioskeletal myopathy associated with the chronic magnesium deficiency of congenital magnesium-losing nephropathy.

Pretreatment expression of the perforin gene by circulating CD8(+) T lymphocytes predicts biochemical response to interferon-alpha in patients with chronic hepatitis C.

It would be of great value to be able to predict, before the initiation of treatment, which patients with hepatitis C virus-induced chronic hepatitis will be cured by interferon-alpha (IFN-alpha). Competitive RT-PCR was used to evaluate spontaneous expression of the perforin gene, a marker of cytotoxic cell activation, by circulating mononuclear cells in 17 patients undergoing IFN-alpha treatment. IFN-alpha increased perforin gene expression (p < 0.003), but this was not correlated with outcome. In contrast, pretreatment perforin gene expression levels were higher in the 8 patients with a sustained biochemical response after treatment than in the 9 non-responsive patients (p = 0.01). This factor predicted favorable clinical outcome with a sensitivity of 75% and a specificity of 89%. Thus, pretreatment immunological status has a major influence on the ability of IFN-alpha to cure chronic hepatitis C, and the evaluation of perforin gene expression may help to select patients that will benefit from IFN-alpha treatment.

A particularly aggressive combined glucagonoma and gastrinoma syndrome.

Duodeno-pancreatic biochemically polyfunctional endocrine tumour is a well known entity. Usually, only one hormone is responsible for the clinical features. We report a case of aggressive combined glucagonoma and gastrinoma tumour without metastases, causing respectively diabetic ketoacidosis and fulminant peptic ulcer, and death. Occasional patients can present with clinical features of both glucagonoma and gastrinoma. Diabetic patients exhibiting migratory skin lesions should be suspected of glucagonoma. In addition, a multidisciplinary approach to such patients including dermatologists, surgeons, radiologists and endoscopists is mandatory.

Comparative study of clinical evaluation of heart murmurs by general pediatricians and pediatric cardiologists.

In a study to compare the clinical diagnostic skills of academic general pediatricians and academic pediatric cardiologists in the evaluation of heart murmurs, a total of 128 patients (aged 1 month to 18 years) newly referred to a university pediatric cardiology clinic were evaluated by one of three general pediatricians and one of four pediatric cardiologists. The murmurs were clinically classified as innocent, pathologic, or possibly pathologic. The classification was revised after the review of electrocardiogram (EKG) and chest radiograph (CXR), if indicated. The definitive diagnosis was ascertained by echocardiography (94 normal, 34 abnormal). The general pediatricians identified as many pathologic heart murmurs as the pediatric cardiologists (27/34 vs. 29/34), with no difference in sensitivity, 79% vs. 85% (p = 0.53). The similarity in sensitivity could be because the general pediatricians were more cautious in the classification of heart murmurs and had classified more innocent heart murmurs as pathologic than the pediatric cardiologists (13/39 vs. 3/23), 41% vs. 13% (p = 0.02). The pediatric cardiologists correctly identified more innocent murmurs than general pediatricians (52/94 vs. 72/94), with a better specificity, 55% vs. 76% (p = 0.001); however, the accuracy of prediction of innocence was similar for both groups (52/59 vs. 72/77), 88% vs. 93% (p = 0.36). The revision of diagnosis with review of EKG and CXR was more often misleading than helpful for either group. Academic general pediatricians would identify most of the pathologic murmurs and are no more likely than an academic pediatric cardiologist to misclassify a pathologic heart murmur as innocent.

[Predictive risk factors for progression to cirrhosis in early stage alcoholic liver disease]. / Facteurs prédictifs d'évolution vers la cirrhose à un stade précoce de la maladie alcoolique du foie.

OBJECTIVE: To assess the risk factors of cirrhosis in early stage alcoholic liver disease. PATIENTS: We investigated 83 heavy drinkers (60 males and 23 females) in whom the first of two liver biopsies showed normal or pure alcoholic fatty liver. RESULTS: When the six following variables: sex, age, delay between the first and last biopsy, total duration of alcohol consumption before the first biopsy, daily alcohol consumption for the last 5 years before the first biopsy and the extent of fatty liver in the first biopsy, were considered together in stepwise regression analysis, the delay between the first and last biopsy (p < 0.0001), sex (P < 0.004) and the extent of fatty liver in the first biopsy (P < 0.06) significantly improved the prediction of cirrhosis. The odds ratio of cirrhosis for a women was 19.1 (confidence interval 95% [1.85-197]). The odds ratio for cirrhosis for a percentage of fatty liver > or = 5/10 was 7.4 (confidence interval 95% [1-92]). CONCLUSION: With the same delay between two liver biopsies, the female sex and the extent of fatty liver are two independent risk factors for the development of cirrhosis in heavy drinkers.

[Ulceronecrotic tracheobronchial involvement in Crohn's disease]. / Atteinte trachéo-bronchique ulcéro-nécrotique au cours d'une maladie de Crohn.

We report the case of a young female patient hospitalized for the first episode of a colonic Crohn's disease with specific ulceronecrotic tracheobronchial involvement leading to chronic and invalidant cough. Symptomatic bronchopulmonary manifestations are very rare in the course of inflammatory bowel diseases and usually not mentioned in Gastroenterology textbooks.

[Biological diagnosis of the type of liver disease in alcoholic patients with abnormal liver function tests]. / Diagnostic biologique du type d'hépatopathie chez des malades alcooliques ayant des tests biologiques hépatiques anormaux.

OBJECTIVES: The histological diagnosis of the different stages of alcoholic liver disease is not systematic. The aim of this study was to assess whether common biological features were useful in identifying the different stages. METHODS: One thousand twenty six alcoholic patients with liver histology and without any associated diseases or infections likely to alter serum liver tests were studied. Diagnostic analyses were performed using stepwise discriminant analysis in the entire population and in asymptomatic patients. RESULTS: a) Serum ASAT activity levels were only normal in 39% of the patients with normal histological liver and in 14% of the patients with steatosis; b) liver failure was already present in patients with fibrosis without cirrhosis; c) betagamma block was the only biochemical parameter which confirmed the diagnosis of cirrhosis without biopsy; d) the diagnostic accuracy of common tests was weak for the diagnosis of alcoholic liver disease without cirrhosis but prothrombin time could be useful in excluding the diagnosis of cirrhosis with and without acute alcoholic hepatitis when liver biopsy is not available. CONCLUSION: Only a prothrombin time of 80% with a negative predictive value of 94% and the presence of beta-gamma [corrected] block with a positive predictive value of 98% were useful for assessing the diagnosis of cirrhosis in all patients with alcoholic liver disease.

[Abdominal tuberculosis: deceptive and still encountered]. / La tuberculose abdominale: trompeuse et toujours d'actualité.

BACKGROUND: Extrapulmonary manifestations of tuberculosis are increasing in incidence. Abdominal tuberculosis may mimic a variety of gastrointestinal disorders. The diagnosis of abdominal tuberculosis is still difficult to establish before surgery. CASE REPORTS: We report 3 cases of abdominal tuberculosis in immunocompetent individuals. One patient presented with an ileocecal mass mimicking cancer. The second one presented with fever, ileocecal mass and ascites leading to the diagnosis of appendiceal peritonitis. The last patient was admitted for ascites, ovarian mass and high CA 125 serum level simulating ovarian cancer with peritoneal carcinomatosis. COMMENTS: In cases of abdominal tuberculosis when standard investigations are unhelpful, a PCR should be performed. Estimation of adenosine deaminase in ascitic fluid is an easy and reliable method for diagnosing tuberculous ascites. With these non invasive diagnostic procedures, surgery should be reserved only to patients with complications.

[Regenerative nodular hyperplasia: variable clinical aspects]. / Hyperplasie nodulaire régénérative: des aspects cliniques très variables.

BACKGROUND: Regenerative nodular hyperplasia can take on very misleading aspects making diagnosis difficult. CASE REPORTS: We report three cases of regenerative nodular hyperplasia (RNH). In the first patient rupture of esophageal varices was associated with myelofibrosis. In the second, extensive portal thrombus formation was associated with consumption coagulopathy and essential thrombocytemia. The third patient had systemic sclerodermia, hepatic macronodules, refractory exsudative ascitis and chronic hepatic encephalopathy following surgery for a porto-cava anastomosis. DISCUSSION: The diagnosis of RNH should be suspected in a variety of clinical situations with search for associated diseases in all cases. The prognosis is related to the consequences of portal hypertension and the severity of the associated diseases.

Fetal echocardiography at West Virginia University: a seven-year experience.

The last decade has seen tremendous growth and utilization of fetal echocardiography. In order to assess the indications and yield of fetal echocardiography at West Virginia University, all fetal echocardiograms performed during a seven-year period were reviewed. There were 931 fetal echocardiograms performed on 803 women during this time period and there was a large increase in referral rate as has been seen in other regions. The highest yield of abnormalities was seen in fetuses referred secondary to the finding of an abnormal fetal cardiac exam on routine screening ultrasound (55.6%), or fetal anomalies of other organs (8.2%). These two indications alone were responsible for 77.8% of identified cases of congenital heart disease by fetal echocardiography during this time period. The resultant findings highlight the need for thorough obstetrical screening ultrasound, including the four chamber view of the heart.

Childhood acute rheumatic fever: a comparison of recent resurgence areas to cases in West Virginia.

A resurgence of acute rheumatic fever (ARF) was noted over the last 10 years in several areas of the United States. West Virginia was no exception with two reports appearing in the literature confirming an increased incidence in the 1980s among children and adults. The Pediatric Cardiology Division of West Virginia University Children's Hospital had 30 cases of ARF referred between 1980 and 1995, and surprisingly 27 of these cases had been diagnosed since 1986. This article describes our chart review of these 30 cases which studied epidemiological aspects, diagnostic criteria and regional differences by chi-square analysis. Other issues we present include "silent" mitral regurgitation and the unreliability of a history of a recent pharyngitis with or without appropriate antibiotic therapy while considering ARF in the differential diagnosis.

Increased susceptibility to gingival colonization by specific HACEK microbes in children with congenital heart disease.

It is well established that infective endocarditis (IE) involving the HACEK (Hemophilus, Actinobacillus, Cardiobacter, Eikenella, Kingella) group of microbes occurs in patients with congenital heart defects (CHD) and in those with prosthetic grafts. Dental caries and gingival disease have been presumed to be the focus of microbial shedding. The purpose of this study was to determine if children with CHD had a more severe gingival inflammatory condition and harbored the HACEK group of microbes to a greater extent than normal children. Two groups of 12 age and sex matched children were selected for this study. The experimental group consisted of twelve children with CHD, 1-1/2 to 8 years of age. The control group consisted of 12 healthy children 2 to 8 years of age. Each child had a gingival index score recorded as described by Massler. Subgingival cultures were obtained. Gingival samples were cultured for HACEK microbes and total Streptococcus (spp) using standard techniques. Fisher's exact test was performed with significance defined at P < 0.05. Children with CHD had more severe gingival inflammatory index than the control group (P < 0.05). 8/12 CHD patient had Actinobacillus actinomycetemcomitans (A.a.) as compared with 2/12 controls (P < 0.05). Furthermore, all cyanotic CHD patients (4/4) had A.a. whereas, only 2/12 controls did (P < 0.05). 4/12 CHD patients harbored Eikenella corrodens (E.c.) compared to 1/12 controls (N.S.). There was no significant difference in colonization with E.c. or A.a. between cyanotic and acyanotic patients. No significant difference in total Streptococcus (spp) was found between the two groups. This study suggests that children with CHD have a more severe gingival inflammatory index and are colonized with specific HACEK microbes more so than normal children.

Blindness in different types of eye disease. A study based on the records of the Department of Eye Diseases in the Medical University, Plovdiv for the period 1982-1991.

Between 1982 and 1991, inclusive, a total of 13718 patients were treated in the Department of Eye Diseases in Plovdiv University of Medicine. Cataract patients formed the most numerous group (19.71%), followed by those with diseases of the retina (9.53%), glaucoma (7.95%), uveitis (4.9%), diseases of the cornea (3.86%), malignant tumors of the eyelids and the eyeball (2.29%) and diseases of the optic nerve (1.54%). Of these 13718 patients, 1727 (12.58%) had monocular and binocular vision below 0.08. The patients with visual acuity from 0 to 0.03 were 1330 (9.69%). Nosologically, they were distributed as follows: glacoma-422 (3.07%), eye traumas-281 (2.04%), diseases of the retina-270 (1.96%), diseases of the cornea-89 (0.64%), cataract-80 (0.58%), uveitis-77 (0.56%), malignant tumors of the eyelids and the eyeball-66 (0.48%), and diseases of the optic nerve-45 (0.32%). Glaucoma was found to be the most common cause of blindness among the patients treated in the Department of Eye Diseases, followed by eye traumas and disease of the retina. The importance of the vascular factor in inducing blindness is undeniably great. It is the underlying cause of the open-angle glaucoma, the diseases of the retina and the optic nerve.