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Crohn's disease is an inflammatory bowel disease characterized by remissions and exacerbations. Immunosuppressants are frequently used to induce and maintain remission in these patients. The use of the immunomodulator azathioprine has been associated to malignancies. Chordomas are rare, locally aggressive tumors arising from remnants of the notochord. A specific trigger for this tumor has not been identified and association to any medication has not been reported. The purpose of this report is to present the first case reported in the literature of Crohn's disease associated to a chordoma. The patient to be presented was on azathioprine therapy, among other medications. A review of literature revealed that Crohn's disease and chordoma have abnormalities in chromosomes 1 and 10. Inflammatory bowel disease and chordoma also have abnormalities in chromosomal regions 1p, 3p, and 7q. Despite these findings, a direct genetic relationship between these diseases is speculative.

Background: Chronic hepatitis C (CHC) is a major health problem in Puerto Rico (PR). More than 50% of the population is insured by a government-sponsored managed care system that does not cover treatment for CHC. Lack of access to treatment will result in an increase in end-stage liver disease with its high socioeconomic impact in the future. In an attempt to identify strategies for the treatment of CHC in the publicly insured population, the PR Health Department and the University of Puerto Rico (UPR) Gastroenterology (GI) Division have developed a pilot clinic for the evaluation and treatment of CHC., Methods: UPR and the PR Health Department negotiated a fee per patient to include all medical care and follow-up laboratories. Viral studies were covered by a grant to the Health Department. Medications were bought at a discount price by the government and dispensed at a government pharmacy. The Health Department allocated funds for 200 patients with government insurance. A dedicated clinic was established at the UPR, staffed by an internist under the supervision of the GI faculty. Patients with a positive HCVab were referred to this clinic. The public insurance covered the CBC, liver tests, metabolic panel, TSH, HBsAg, HIV, ultrasound and liver biopsy, which were required prior to evaluation for possible treatment. In the initial visit, patients underwent a medical evaluation, including assessment of suitability for therapy and counseling. Those deemed to be candidates who still needed a liver biopsy had it performed by the GI staff. Genotype and viral titers were ordered after the decision on treatment had been made. The clinic physician prescribed pegylated interferon and ribavirin, which were dispensed by the government pharmacy. Instruction on proper drug administration was given. Clinic visits were scheduled for 1, 3, 6 and 12 months but also allowed on demand. Laboratory tests were done at the clinic and reviewed by the physician expediently to monitor for toxicity. Any medical problems or treatment for complications of therapy were covered by the primary insurer. Viral load was repeated at 12 weeks to discontinue therapy in those unlikely to respond. The budget per patient for medical visits and laboratory tests was dollars 1,500.00, HCV RNA titers plus genotype costs dollars 200.00, and HCV qualitative RNA costs dollars 123.00, Results: 405 patients have been referred between February 2002 and April 2003 (the number was increased at adjust for no-shows and those not treated). 30% are female, the major risk factor is IVDU, and 80% are unemployed. 101 have started treatment and 48 are awaiting biopsy. A support group has been established at the clinic., Conclusions: The treatment for CHC in practice is not only costly but also resource consuming. Most gastroenterologists in our community refer these patients for treatment. The establishment of a dedicated clinic with a primary physician supervised by the specialists reduces costs and facilitates caring for a larger number of patients. The volume of services allows for negotiation of medical, laboratory and drug costs. In allocating funds for this project, the PR Health Department recognized the importance in reducing the potential spread in the community by treating infected patients as well as reducing the future medical and socioeconomic burden of end-stage liver disease. Although the outcome of this project is still unseen, we believe that this model may serve to establish other clinics for the treatment of CHC at lower costs with the same effectiveness.

Hepatitis C infection is the most common chronic blood-borne pathogen in the United States associated with liver cirrhosis and hepatocellular carcinoma and is the leading reason for liver transplantation. It has been estimated that hepatitis C infection may lead to a substantial health and economic burden over the next 10 to 20 years. The prevalence of hepatitis C virus (HCV) infection varies worldwide, with an estimated overall prevalence of 3%. However, the only available data of hepatitis C in the general population of Puerto Rico suggest an elevated prevalence of hepatitis C infection in the municipality of San Juan (6.3%) in comparison with estimates for the adult population residing in the United States (0.9%-3.9%). Much of the inter-region variability in the prevalence of hepatitis C can be attributable to the frequency and extent to which different risk factors have contributed to the transmission of the virus. Established risk factors for infection include injection drug use, transfusion of blood and solid organ transplantation from infected donors prior to July 1992 and blood clotting products before 1987, occupational injury, vertical transmission, sex with an HCV infected partner, and multiple sexual partners. Other potential exposures for infection that have been investigated in epidemiologic studies include history of intranasal cocaine use, sharing of contaminated equipment and personal care items, tattooing, body piercing, imprisonment, acupuncture, and use of contaminated healthcare instruments. The high incidence of AIDS in Puerto Rico and the large prevalence observed in Puerto Rican inmates and in adults residing in the municipality of San Juan indicate that HCV infection is an emerging public health concern. From a public health perspective, potential targets for intervention to decrease the spread of HCV infection, ongoing surveillance, increased clinician awareness of disease reporting systems and the epidemiology and management of hepatitis C, availability of diagnosis and treatment facilities, and recognition of the need for local resources will be of paramount importance to face this silent infection in Puerto Rico.

Objective: To evaluate the response rate of Hispanics with chronic hepatitis C to combination therapy of interferon alfa-2b plus ribavirin and to assess its adverse events., Background: Hepatitis C virus may lead to chronic infection and multiple complications. Response to combination therapy of interferon plus ribavirin has been studied in many populations. African Americans have been found to have a lower response rate than Caucasians. However, little data exist for Hispanics., Methods: Hispanic patients from Puerto Rico with chronic hepatitis C were eligible for the study between November 1997 and February 2000. The Institutional Review Boards of the participating institutions approved the study. Written informed consents were obtained. Combination therapy was given for 48 weeks and patients were followed for 24 weeks after treatment. Analysis of response to therapy was performed in an intention-to-treat basis., Results: The most frequent adverse event was anemia (89%), associated to ribavirin. Sustained response was 23% for naive patients, 45% for relapsers, and 8% for non-responders to previous interferon monotherapy (p < 0.001). Data to analyze response was not available in 27% of patients. Hispanic patients had a low response rate to combination therapy., Conclusions: Response rates to combination therapy for Hispanic naive and previously non-responder patients are lower than in other reported populations. This may be due to a high prevalence of genotype 1 in Puerto Rico, which is associated to poor response. The higher response rate of relapsers, similar to those reported previously, was expected since these patients showed a previous response to interferon monotherapy. Ethnic factors may play a role in the response to therapy and should be further studied to determine proper treatment strategies for this population.

Objective: To evaluate the comprehension of the scoring system for controlling the serum levels of cholesterol and fat as an instrument of dietary intervention for hypercholesterolemic patients., Methods: The study comprised 153 individuals of both sexes, with ages ranging from 20 to 65 years, who sought medical care in a hospital or in a basic health unit. They were divided into the following 3 groups: hypercholesterolemic individuals, individuals with no diagnosis of hypercholesterolemia, and health care professionals. A 24-hour recall was used with each individual, who applied the cholesterol/saturated-fat index to their diets, consulting the scoring system. The researcher confirmed the calculation of each participant, considering her calculation as the a reference pattern. Understanding of the system was assessed through comparison between the reference pattern and the data obtained by the interviewees, using the intraclass correlation coefficient., Results: The patients without a diagnosis of hypercholesterolemia obtained a high correlation in all meals. The morning and afternoon snacks and supper were the meals with the greatest correlation (r = 1). In hypercholesterolemic individuals, supper was the meal with the greatest concordance with the reference score (r = 1), and the health care professionals obtained a correlation coefficient of 1 in all meals, except lunch (r = 0.99)., Conclusion: The scoring system was fast, simple, and easy to be understood and accepted by the population studied.

Treatment of chronic hepatitis C consists of inteferon plus ribavirin. The major adverse effect of ribavirin is hemolytic anemia, a complication that limits therapy. Folic acid supplementation is used to improve erythropoiesis in chronic hemolytic anemia. The aim of this study was to evaluate the effectiveness of folic acid supplementation in the prevention of ribavirin-induced anemia in patients being treated for hepatitis C. Twenty one patients enrolled in treatment protocols for hepatitis C received folic acid 1 mg daily and 22 did not. Groups were similar in age, gender, ribavirin dose and baseline hemoglobin. Folic acid supplementation had no effect in the decrease in hemoglobin or the measured parameters of hemolysis. No difference between males and females was noted for hemoglobin decrease or lowest hemoglobin levels. In our study, folic acid showed no beneficial effect in the prevention of ribavirin-induced anemia.

Objectives: Limited data exists about Inflammatory Bowel Disease (IBD) in Hispanic populations. The aims of the present study were to estimate overall and specific prevalence of IBD (Crohn's disease and ulcerative colitis) and to describe the characteristics of a group of patients from the University of Puerto Rico's IBD Registry., Methods: To estimate the prevalence of IBD, computerized records of all physician billing and hospital discharges from a major health insurer in Puerto Rico and classified with ICD-9-CM codes 555.0-555.9 (Crohn's disease) and 556.0-556.9 (ulcerative colitis) during 1996 were searched. Prevalence was estimated by age group, sex, and type of insurance. To describe demographic and selected clinical information from patients with IBD, data gathered in the University of Puerto Rico's IBD Registry from 1995 through 2000 was analyzed., Results: Out of 802,726 insured individuals, 332 had a diagnosis of Crohn's disease, 499 of ulcerative colitis and 21 had both diagnoses. The estimated prevalence per 100,000 was 41.4 for Crohn's disease, 62.2 for ulcerative colitis, and 106.1 cases per 100,000 for IBD. Peak prevalence of Crohn's disease occurred among the age groups 50-59 years and > or = 60 years, and the overall female:male prevalence ratio of Crohn's disease was 1.13 (95% CI: 0.91-1.42). Ulcerative colitis was most prevalent among insured individuals aged 50-59 years and 40-49 years. The prevalence of ulcerative colitis was significantly higher among females than among males, with an overall prevalence ratio of 1.42 (95% CI: 1.18-1.71). Of 342 patients participating in the IBD Registry, 155 (45.3%) had Crohn's disease and 187 (54.7%) had ulcerative colitis. Among patients diagnosed with Crohn's disease, 51.6% were females, the mean age was 35.2 +/- 18.3 years, and 18.1% had a family history of IBD. More than half (57.8%) of patients with ulcerative colitis were females, the mean age was 42.6 +/- 17 years, and 17.1% had a family history of IBD., Conclusions: The estimated prevalence of IBD in this insured population in Puerto Rico places it among the middle-range of that reported for other countries. Additional studies must be conducted in Puerto Rico in order to confirm the observed findings. Population-based epidemiologic studies aimed at estimating the burden of IBD in Hispanic populations in the United States and Latin America are essential for health care planning.

The major mechanisms and consequences of free radicals in foods and biological systems that result in the occurrence of chronic diseases of aging (atherosclerosis, cataract, cancers, diabetes, neurological diseases, immune-inflammatory disorders) are described. The main antioxidant defense mechanisms from foods and cells and tissues are also reported. Many nutraceutical substances, with their respective beneficial actions (antioxidant, detoxifying, apoptotic actions) and effects, and most common food sources are also described. There are many options of foods to prevent cancer and chronic diseases, to improve life's quality in maturity.

The study objective was to identify the circumstances where there was risk of contamination of food (hot dogs) prepared and sold on the streets and to establish a preventive action through the creation of key surveillance sites. Data were collected from 20 vending sites using interviews, questionnaires, observations of food handling and storage, and temperature and pH measurements of both meat and sauce, respectively. In 30% of the studied sites, hygienic conditions were rated as regular to extremely poor. Mashed potatoes, chicken and beef preparations were of high risk. These findings showed inappropriate hygienic practices of food preparation and lack of basic knowledge regarding food handling - a public health problem. Given the scarcity of literature and official data on food sold in the streets of Brazil, further studies are recommended.

Background: The etiology of Inflammatory Bowel Diseases, Crohn's disease (CD) and ulcerative colitis (UC), is unknown. These diseases have a higher incidence in industrialized countries and their pathogenesis involves an over-reaction of the immune system. A genetic factor is believed to predispose to the development of chronic inflammation in response to an unidentified stimulus. Exposure to infections in childhood may modulate future immune responses. Parasitosis, particularly Schistosomiasis, stimulate Th2 immune responses. It has been hypothesized that the absence of these parasitic infections, as seen in economically developed countries, favors a Th1 response that may result in the clinical appearance of Crohn's disease later in life., Objective: To determine the prevalence of Schistosoma mansoni antibodies in Puerto Ricans with Inflammatory Bowel Disease and controls., Methods: Serum from 92 Puerto Ricans with IBD and 106 controls was screened for S. mansoni adult microsomal antigens (MAMA) using the FAST:ELISA assay. Those positive were confirmed with an enzyme-linked immunoelectrotransfer blot test., Results: Seven serum samples (3 UC and 4 controls) were positive for S. mansoni antibodies. There was no significant difference between groups in gender, municipality of origin or seroprevalence of Schistosomiasis. The control group was slightly older than the IBD group., Conclusions: Our study did not demonstrate an inverse relation between Schistosomiasis and IBD. However, the decreasing prevalence of Schistosomiasis in the general population of Puerto Rico may account for this result.

Objective: To review the experience with ileal pouch-anal anastomosis surgery for ulcerative colitis at the University Hospital., Background: As many as 40% of patients with ulcerative colitis (UC) and 75% with Crohn's disease (CD) require some surgery for their disease. The number of patients referred to our clinics for evaluation and management of Inflammatory Bowel Disease (IBD) has risen in the past seven years. A multidisciplinary IBD service has been created at the University Hospital for the care of these patients, leading to a dramatic increase in the number of surgeries performed for IBD. Over the past decade the ileal pouch-anal anastomosis (IPAA) has emerged as the procedure of choice in most patients with ulcerative colitis requiring total colectomy for management of their disease. Even though the procedure is associated with a considerable morbidity rate, it has become very popular since it avoids the need for a permanent stoma and presumably rids the patient of disease and subsequent cancer risk., Results: Twenty-five patients were identified as having IPAA for ulcerative colitis between 1993-2000. Indications for surgery were intractability and toxic megacolon. Complications were pouchitis in 11/25 (44%), anastomotic stricture in 6/25 (24%), small bowel obstruction in 4/25 (16%), and pouch failure in 2/25 (8%). Other complications included wound abscess in 1/25 (4%), and sexual dysfunction in 1/25 (4%) patients. There was no mortality; the patients' quality of life was rated as greatly improved in 14 of 17 patients interviewed (82.4%) and 16 of 17 said they would recommend the surgery to others (94.1%)., Conclusions: The results of IPAA surgery, morbidity, mortality, and patient satisfaction in our series were similar to other centers around the world.

Objective: To describe the demographic and clinical characteristics of patients attending the Inflammatory Bowel Disease Clinic of the University of Puerto Rico School of Medicine and determine whether these characteristics are risk factors for the development of a depressive disorder., Method: Sixty-seven patients attending the Inflammatory Bowel Disease Clinic of the University of Puerto Rico School of Medicine were assessed for the presence of depressive symptoms using a simple questionnaire (CES-D) and a detailed diagnostic evaluation (CIDI-DSM IV Module E). Age, gender, education, time of IBD diagnosis, duration of treatment, psychiatric history, treatment with corticosteroids, and activity of IBD were determined and correlated with the presence of depressive symptoms and depressive diagnosis. Study data was tabulated on Epi-Info 6.0 and it was analyzed using SPSS version 10. Univariate (includes means and frequencies), bivariate (t-student, Kruskal Wallis and Chi-square) and multivariate analyses (logistic regression) were performed., Results: Patients older than 34 years old had three times higher probability of developing a depressive disorder (p = 0.043, OR = 3.22). Patients with a psychiatric history had seven times higher probability of developing depressive disorder (p = 0.004, OR = 7)., Conclusion: The risk factors identified with an increased probability of developing a depressive disorder were age older than 34 years and psychiatric history.

Background: Osteopenia has been reported in association to Inflammatory Bowel Disease, and in particular Crohn's disease. The use of corticosteroids, resection of the ileum, malabsorption, poor calcium intake, and the effect of inflammatory cytokines have all been considered as contributing factors. As Crohn's disease is more prevalent in young people, when peak bone mass is achieved, the presence of osteopenia is especially significant., Objectives: The aim of this study was to evaluate the bone density of patients with Crohn's disease in the University of Puerto Rico IBD Clinic; to determine the prevalence of osteopenia in these patients and to correlate bone mineral density with risk factors for osteopenia., Methods: Sixty-six patients, 30 males and 36 females were included. After informed consent, demographic, clinical and metabolic data was obtained. Serum albumin, calcium, inorganic phosphorus and alkaline phosphatase were measured. Body mass index (BMI) was calculated. Bone density was determined by DEXA of the lumbar spine and femur and expressed as the Z score (standard deviations from normal correlated with sex and age). Severe osteopenia was a Z score > or = -2 and osteopenia was Z < or = -1.99 or > or = 1.01. Results were expressed in means. Pearson correlation coefficient was used for quantitative variables and Pearson chi-square for categorical values., Results: Osteopenia was present in the hip in 69% and in the lumbar spine in 68%. Most patients had received steroids; the difference between treated and not treated patients was not significant. Osteopenia did not correlate with ileal resection, gender, BMI, disease characteristics or biochemical parameters., Conclusions: Low bone density was frequent in patients with Crohn's disease, but no specific risk factors could be identified. Bone density should be determined in patients with Crohn's disease in order to institute appropriate therapeutic measures.

The diagnosis of hydatid disease outside endemic areas is usually not suspected. Hydatid cysts in imaging studies can be confused with hepatic tumors, abscesses, cystadenomas, liver cysts or other lesions. Serology is the usual confirmatory test, but cytologic diagnosis has been described. Aspiration of the cysts has not been employed as a routine diagnostic method for fear of spillage and anaphylactic reactions. We report a case of unsuspected hepatic echinococcosis that was confirmed by fine-needle aspiration of the lesion and cytologic confirmation without complications.

Autoimmune hepatitis is a chronic inflammatory liver disorder of unknown etiology associated with serum autoantibodies and hypergammaglobulinemia. This disease has a broad spectrum of presentations ranging from asymptomatic to fulminant hepatic failure. A 36 year old female with past history of hypothyroidism developed jaundice 2 months prior to admission. Outpatient evaluation revealed ANA and anti-SMA antibodies in high titers, negative viral markers for hepatitis, and hypergammaglobulinemia. A presumptive diagnosis of autoimmune hepatitis was made; steroids were recommended but the patient did not take them. She was admitted to the University Hospital due to increased jaundice, general malaise and ascites 5 weeks later. She deteriorated developing coagulopathy, encephalopathy and increasing hyperbilirubinemia. Intravenous corticosteroids were started. The patient improved and was discharged 3 weeks after admission. Fulminant hepatic failure has a high mortality and may require liver transplant. Our patient survived fulminant hepatic failure that resolved after corticosteroid therapy. It is important to identify and distinguish autoimmune hepatitis from other forms of liver disease because of the high percentage of response to immuno-suppressive therapy. Early diagnosis and treatment of this condition could improve survival, quality of life, and defer liver transplantation.

Interferon (IFN) is the only drug that has been approved by the FDA for therapy of chronic hepatitis C. However, optimal dose and duration of therapy are still controversial. This study compares the effectiveness of treatment of chronic hepatitis C patients with 3 vs. 5 million units (MU) of recombinant alpha-interferon 2-b three times per week. We also evaluated the relapse rate with a shorter 12 week-course of therapy in those patients who had normalization of aminotransferases by week 12. Seventy-five patients were randomized to receive either 3 vs. 5 MU of IFN; seventy-two completed the study. A complete response was seen in 11/35 (31%) of those treated with 5 MU vs. 13/37 (35%) in the 3 MU dose (p = 0.74). Patients were followed after IFN was withdrawn and only 2 had persistently normal aminotransferases. Analysis of multiple variables was done to predict response to IFN and only elevations of GGT, ferritin and alkaline phosphatase were found to be predictors of a poor response. Therefore, we recommend initial therapy with 3 MU of IFN for a longer period than 12 weeks in patients who show a response.

An increased incidence of cerebral thromboembolic events has been reported in young patients with inflammatory bowel disease (IBD). It has been suggested that a hypercoagulable state is associated with clinical activity of the disease, with elevation of factors V, VIII, fibrinogen and platelets and a lowering of anti-thrombin III. We present the case of a 35 y/o male with refractory Crohn's disease who complained of headaches, blurred vision and tonic-clonic seizures. The studies demonstrated an ischemic stroke of the left cerebral hemisphere, without vascular abnormalities. Elevation of factor VIII, platelets, and antithrombin III were found. The symptoms were relieved with medical treatment and the patient has continued in good health after resection of the diseased terminal ileum.

Throughout the world there have been several epidemics of food-borne diseases (FBD) about which there is lack of sufficient information for public health institutions to take appropriate measures. This study was conducted for the purpose of contributing to the dissemination of information on these diseases and their etiologic agents, epidemiology, and control. The study was based on data from 61 sources, including review articles, reports of outbreaks, and databases. Results reveal considerable underregistration and lack of data on FBD throughout the various countries, with viruses being the second most important cause of FBD in the United States of America. Two agents, Norwalk virus and hepatitis A virus, were the fifth and sixth most frequent causes, respectively, although the former was the single most frequent cause of FBD in 1982 and the second most frequent cause of water-borne diseases during the period from 1986 to 1988. Despite the scarcity of information on the problem, rotavirus, poliovirus, hepatitis E virus, astrovirus, and small gastroenteric viruses are also important causes of FBD. We also discuss the importance of viral zoonoses, especially hemorrhagic fevers transmitted by contact with rodent feces and tick-borne viral encephalitides (Lassa fever). There is discussion of the controversial mad cow disease and its potential transmission through food products, as well as of dietary aspects of the management of AIDS and other viral infections. Finally, measures for the prevention and control of FBD are described.

Hepatitis C infection progresses faster in patients with hypogammaglobulinemia, often leading to death from liver failure within 10 yr of exposure. Response to interferon treatment has been poor in these patients. We report the case of a patient with common variable immunodeficiency and hepatitis C in whom a sustained biochemical and viral remission was obtained after treatment with interferon.

Objectives: To compare endoscopic sclerotherapy, propranolol and distal splenorenal shunt in the prevention of rebleeding esophageal varices and mortality in alcoholic cirrhotics., Methods: Forty-three alcoholic cirrhotics that had bled from esophageal varices were randomized to sclerotherapy (13), shunt (15) or propranolol(15). Outcomes measured were rebleeding from varices and death., Results: There was no significant difference between the three treatment groups in relation to rebleeding rate or death. Continued alcohol consumption did not influence outcome., Conclusions: All three therapies were equally effective in preventing rebleeding from varices.

This study was undertaken to investigate human leucocyte antigen (HLA) associations with benign migratory glossitis and psoriasis in Brazilian patients and particularly to determine whether benign migratory glossitis is also associated with HLA-Cw6, the classical association observed in psoriasis. The results showed a highly significant association of Cw6 with both psoriasis and benign migratory glossitis, with this antigen being present in 59.1% of the patients with psoriasis, in 43.8% of the patients with benign migratory glossitis, and in only 12.6% of the controls. Other significant positive associations, although at a lower significance level, were with B13, both in psoriasis and in benign migratory glossitis, and with B17, only in psoriasis. To our knowledge, this is the first report on the association of Cw6 with benign migratory glossitis. We believe that this finding reinforces the concept of a pathogenetic relationship between benign migratory glossitis and psoriasis.

One hundred consecutive patients underwent esophageal motility testing at the Gastroenterology Section of the University Hospital for symptoms of esophageal dysfunction. These were dysphagia (55), non cardiac chest pain (11), gastroesophageal reflux (32), and other (2). Fifty five studies were abnormal. The most frequent findings were achalasia in fourteen patients and nonspecific esophageal motility disorder in fourteen. When the clinical presentation was correlated with the results of the study, 35 of the 55 patients with dysphagia had an abnormal study, as compared with 5 of 11 with chest pain and only 12 of 32 with reflux symptoms. These findings compare with those reported elsewhere and suggest that esophageal motility studies are most useful in the diagnosis of patients presenting with dysphagia or non cardiac chest pain.

Charqui is a typical Brazilian meat product obtained by salting and sun-drying beef samples. The chemical, physical and microbiological characteristics of the charqui were evaluated throughout processing and storage. The results confirm charqui is an intermediate moisture meat product (A(w) = 0·70-0·75). A close relationship between moisture, pretein and ash vaiues was found, suggesting the possibility of using the resulting charqui A(w) value as a parameter to define the product instead of the official moisture and mineral residue contents. The TBA determination, which expresses the state of lipid oxidation, rapidly reached the maximum value, corroborating the previous observations on the salt pro-oxidant role, and then decreased gradually. A gradual decrease in microorganism count during processing and storage of charqui was also observed. These results indicate the feasibility of obtaining a final product with a low level of microbial count when raw materials of good quality, and adequate handling conditions, are used for charqui production., (Copyright © 1994. Published by Elsevier Ltd.)

We studied 13 first-degree relatives in a large family with an index case of idiopathic hemochromatosis to detect the relatives with evidence of iron overloading. Serum iron, total iron binding capacity (TIBC), and serum ferritin levels were measured in all family members. We also performed HLA typing to identify the relatives who are homozygous with the proband and genetically predisposed to develop the disease. The family was composed of the parents and 12 siblings including the index case. The mean age of the siblings was 25 years. None presented with evidence of iron overload by the iron biochemical tests. HLA typing demonstrated six homozygous siblings with the proband. In separate analysis these siblings did not present abnormalities in any of the iron biochemical tests. These homozygous relatives were followed for one year after initial evaluation and none presented abnormalities in the iron studies during this period. These results are contradictory to other previous studies done in families with idiopathic hemochromatosis. The most feasible explanations for these findings are the young age of these siblings and the predominance of females among them. We consider that these homozygous relatives must be followed for their life-times with iron studies to detect a possible increase in iron stores as expected in later ages.

A 32-year-old male presented with palpitations and an elevated serum iron. Further evaluation revealed elevated serum ferritin, and a liver biopsy confirmed the diagnosis of hemochromatosis. Cardiovascular workup was normal except for sinus bradycardia alternating with sinus tachycardia in the 24-hour Holter study. Treatment with weekly phlebotomies was started and the family is being studied for early diagnosis of any other case of hemochromatosis. The manifestations, diagnostic workup, and the importance of early diagnosis of hemochromatosis is stressed.

A 21 y/o female presented with fulminant hepatic failure and hemolysis. On the basis of the clinical presentation, levels of ceruloplasmin and serum copper a presumptive diagnosis of Wilson's disease was made. In spite of supportive measures and hemodialysis, the patient died one week after admission. Postmortem examination showed cirrhosis and increased copper stores in the liver, corroborating the clinical diagnosis of Wilson's disease. Study of the four siblings revealed that two are carriers, one is healthy and one may have the disease. Wilson's disease is a rare cause of fulminant hepatic failure that must be suspected specially when hemolysis is associated to the clinical picture. This mode of presentation is virtually fatal and early liver transplantation is the best form of therapy.

A 61 year old female developed fatal hepatic failure after phenytoin administration. A typical multisystem clinical pattern precedes the manifestations of hepatic injury. The hematologic, biochemical and pathologic features indicate a mixed hepatocellular damage due to drug hypersensitivity. In a patient receiving phenytoin who presents a viral-like illness, early recognition and discontinuation of the drug are mandatory.

Cell-mediated and humoral immunity were investigated in 18 patients with West syndrome, 12 with Lennox-Gastaut syndrome and 19 healthy controls. The study included determination of T and B peripheral blood lymphocytes, serum levels of IgG, IgA and IgM, skin sensibilization with DNCB, intracutaneous PHA, leucocyte migration inhibition test and lymphocyte blastic transformation in the presence of PHA. Cell-mediated deficiency was detected in 28 children whereas low levels of immunoglobulins were observed only in 6 children. Immunological disturbances were more prominent in children with West syndrome.

Although ulcerative colitis and Crohn's disease have been known for a long time, the etiology is still unknown, and at times it is difficult to distinguish one from the other. In addition, the characteristics of the disease make their epidemiologic study difficult. We have reviewed 70 cases with the diagnosis of Crohn's disease or ulcerative colitis, and compared our results with those published from other parts of the world. We found a high proportion of ulcerative colitis to Crohn's disease, 5.36:1. There was no difference in affected age groups or most frequent symptoms; but we found Crohn's disease to be more frequent in the colon. We also found a very low incidence of extraintestinal manifestations. We believe an adequate standard protocol should be followed for the evaluation of patients with inflammatory bowel disease, including complementary studies to assess disease patterns and complications.