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J A Bombí

Publications and source records attributed to J A Bombí.

At least 19 recordsLinked to original sources
PubMed2004

[Hospital registry of pancreatic tumors. Experience of the Hospital Clínic in Barcelona (Spain)].

OBJECTIVE: To describe the characteristics of patients included in the pancreatic tumor registry of the Hospital Clínic of Barcelona. PATIENTS AND METHOD: All patients with pancreatic tumors attended between July 1990 and March 2003 were registered. Data collection included: age, gender, date of diagnosis, diagnosis, histology, size, location and tumor stage, and treatment. The correlation between tumor stage and age, date of diagnosis, and tumor location was also evaluated. RESULTS: Six hundred thirty patients with pancreatic tumors were included, representing an incidence of 60 patients/year. The mean age was 66 years and the male-to-female ratio was 1,18:1. The most frequent lesion was malignant tumor of the pancreas (92%), and the most frequent histological type was pancreatic ductal adenocarcinoma (73%). The most frequent location was the head of the pancreas (64%). In 28% of the patients, pancreatic cancer was diagnosed in stage I and II. Resection was performed in 31% of patients, whereas 48% of the patients received no treatment. The ratio between local (stage I)/disseminated (stage IV) disease was 0,34. The ratio between stage I/IV increased with age, diagnosis prior to 1994, and tumor location in the head of the pancreas. CONCLUSION: Hospital tumor registries can be used to define the profile of the attended population, which can help to delineate the best diagnostic-therapeutic strategy and can be useful in clinical research.

Adult↗
PubMed2001

Intestinal damage in gastroschisis is independent of the size of the abdominal defect.

The intestinal damage in gastroschisis (GS) has been attributed to a narrow abdominal wall defect (AWD), among other causes, but this causal effect is difficult to prove in humans. The present experimental study was done to ascertain the damaging effect of clinically extrapolable mild and moderate constriction at the AWD on the intestine of fetuses with GS. AWDs of two different sizes were carried out in the fetal rabbit model: small-ring GS (1.5x bowel diameter, SRG) and large-ring GS (3x bowel diameter, LRG); a group of unoperated littermates served as controls. Fetal body weight, intestinal length and weight, bowel diameter and wall thickness, and histology were checked 7 days later. No statistical difference was found in body weight and bowel diameter among the groups. Intestinal length, weight, and wall thickness were significantly different in the GS groups compared to the controls, but no difference was found between the GS groups. Histology did not show venous stasis, ischemic lesions, or differences in the degree of edema between groups SRG and LRG. Mesothelial hyperplasia was seen in both GS groups. The intestinal changes in length, weight, diameter, wall thickness, and histology in GS should thus not be attributed to the diameter of the AWD.

Abdominal Muscles↗
PubMed1998

Systemic xanthohistiocytoma: a variant of xanthoma disseminatum?

We present a 66-year-old man who had maculopapular pigmented lesions on the skin of the head, neck and trunk suggesting generalized eruptive histiocytoma (GEH). These lesions had a yellowish centre in a target-like pattern that has not been previously described. The patient suffered from diplopia and had a severe sensorimotor polyneuropathy causing progressive paresis of the limbs. The explorations performed disclosed the presence of specific xanthomatous infiltrates in the skin, lungs, respiratory tract, peripheral nerves and meninges, suggesting xanthoma disseminatum (XD) or juvenile xanthogranuloma. Multiple osteolytic lesions of large bones were also found. The infiltrate was CD68, MAC 387 and factor XIIIa positive and S-100 and CD1 negative. Some cells contained worm-like bodies visible by electron microscopy. Our patient presented clinical and immunohistochemical findings suggestive of GEH, juvenile xanthogranuloma or XD, supporting the idea of a wide spectrum of non-Langerhans cell histiocytosis. These specific target-like xanthomatous lesions seem to be unique for this new variant of XD.

Aged↗
PubMed1997

[Chronic enteropathy of unknown etiology in patients with AIDS. An analysis of 40 cases].

BACKGROUND: Data about the etiology of chronic enteropathy in AIDS patients are scarce and are very dependent upon the geographical area. The aim of this study was to detect microorganisms potentially associated with chronic enteropathy in AIDS patients with diarrhoea for more than one month, and initial negative routine stool bacterial cultures and examinations for ova and parasites. The degrees of associated intestinal malabsorption and immunodeficiency were also analysed. PATIENTS AND METHODS: Forty consecutive patients were recruited from January 1993 to December 1994. The following studies were performed: Intestinal absorption tests (d-xylose and 14C-triolein), CD4/CD8 cell counts, microbiological studies (standard stool cultures for detection of bacteria and examinations for ova and parasites including the detection of Enterocitozoon bieneusi spores by the Weber's stain), upper gastrointestinal endoscopy or colonoscopy with intestinal biopsies and blood cultures for CMV and mycobacteria. RESULTS: The median duration of diarrhoea was 4 months and the mean weight loss was 8.4 kg. Ninety percent of patients had less than 0.1 x 10(9) CD4+ cells/l, with a mean CD4+ cell count of 0.035 x 10(9)/l. Malabsorption was found in 84% of patients. An etiological diagnosis of chronic enteropathy was reached in 60% of the patients. The yield of pathological examination was 37% and the microbiological test using samples of faeces and blood were positive in 45% and 20% of cases respectively. The most frequently identified microorganisms were CMV (10 cases), E. bieneusi (9), enterobacteria (8), Cryptosporidium parvum (5), Leishmania donovani (2). Patients with enteropathy caused by E. bieneusi had lower count of CD4 cells (p = 0.005) and with higher serum levels of alkaline phosphatase (p = 0.02) than patients with CMV enteropathy. CONCLUSIONS: Stool Weber's stain and CMV and mycobacterial blood cultures should be added to the standard work-up diagnosis in patients with chronic diarrhoea and a CD4+cells count below 0.1 x 10(9) l. Upper and/or lower gastrointestinal endoscopies with intestinal biopsies should be performed only in patients with persistent diarrhea without microbiological diagnosis or a lack of response to treatment.

Acquired Immunodeficiency Syndrome↗
PubMed1997

Middle-ear adenoma (MEA): a report of two cases, one with predominant "plasmacytoid' features.

Middle-ear adenomas (MEAs) are rare neoplasms which can display several histological patterns and represent a diagnostic challenge. We present two cases of MEA which share some histopathological features such as medium to small cells forming solid infiltrating tumour nests as well as scattered glandular structures with Alcian blue and PAS positive material within. The second case also displayed a distinct and predominant "plasmacytoid' appearance which, in a small biopsy, might have been misleading. Both cases expressed an admixture of epithelial and neuroendocrine immunohistochemical markers, whereas ultrastructural study demonstrated electron dense granules. Taken together, these observations support a mixed epithelial and neuroendocrine nature for these neoplasms, the differential diagnosis of which includes paragangliomas and other tumours or tumour-like lesions involving less frequently the middle ear, such as meningiomas, plasmacytomas and inflammatory polyps. The existence of MEAs with plasmacytoid features should be remembered to avoid confusion with plasmacytomas, plasmacytoid myoepithelioma and plasma cell inflammatory infiltrates.

Adenoma↗
PubMed1996

Myoepithelial tumors of salivary glands: a clinicopathologic, immunohistochemical, ultrastructural, and flow-cytometric study.

Myoepitheliomas of the salivary glands remain a controversial entity. To contribute to the knowledge of this entity, 16 myoepithelial tumors of the salivary glands were studied: 12 benign myoepitheliomas (BME) and 4 malignant myoepitheliomas (MME). The clinical and the histologic findings of each case were studied Immunohistochemistry and flow-cytometry analysis were performed from the paraffin-embedded material in 15 cases. An electron-microscopy study was performed in 8 cases. The myoepithelial tumors affected patients of both sexes equally. The mean age of the patients with BME was 54 years, and the mean age of patients with MME was 62 years. Eight cases of BME originated in the parotid gland and 4 cases originated in the minor salivary glands. All the MME developed from a benign preexistent tumor: two developed from a pleomorphic adenoma in the parotid gland, and the other two MME developed in the minor salivary gland from a BME. The myoepithelial tumors were composed of epithelioid, plasmacytoid, spindle, or clear cell types, and they showed a solid or a myxoid pattern of growth. Immunohistochemical studies revealed marked and diffuse positivity to cytokeratins, vimentin, and S-100 protein in all cases. Glial fibrillary acidic protein was positive in 8 cases (53%), and muscle-specific actin and smooth-muscle actin were positive in only 3 cases (20%); they were all cases of BME. Desmin was negative in all tumors. Ultrastructural studies showed the presence of basal membrane, tight junctions, intermediate filaments, and microvilli as well as actin-like filaments lacking focal densities in all cases. But actin-like filaments with focal densities were not identified. Flow cytometry determined that all BME were diploid with a mean proliferative index of 7.73%. Two of the MME were diploid and the other two MME were aneuploid. The mean proliferative index of MME was 11.93%. In conclusion, BME and MME originated in major and minor salivary glands can display different histologic patterns and cellular features. Some immunohistochemical and ultrastructural characteristics have been found in all these neoplasms, which supports the idea that myoepitheliomas are composed by neoplastic modified myoepithelial cells, not fully differentiated. These techniques can be useful for the diagnosis of these tumors.

Adult↗
PubMed1995

[Intestinal microsporidiosis in patients with AIDS: study of 3 cases].

Enterocytozoon bieneusi is a protozoa belonging to the Microsporidia family which prevalence has increased in AIDS patients. Although diagnosis is performed by the demonstration of the parasite in the epithelium of the small intestine by light and electron microscopy, techniques allowing diagnosis from stools or duodenal or biliary aspirates have recently been described. Three cases of intestinal microsporidiosis diagnosed by the mentioned method are reported. The patients were 3 males with chronic diarrhea of several months of evolution with an important ponderal loss. All were in advanced stages of HIV infection with CD4-lymphocyte counts lower than 0.1 x 10(9)/l. In all the patients in whom intestinal absorption tests were performed these were found to be altered. One of the patients presented concommitant cholestasis with parasitation by E. bieneusi being demonstrated as by the biliary route in this patient. Confirmation of infection by E. bieneusi was performed in the 3 cases by electron microscopy study of stools. A review of intestinal microsporidiosis in AIDS patients is carried out and the therapeutic possibilities available for this infection are discussed.

Acquired Immunodeficiency Syndrome↗
PubMed1995

Congenital generalized follicular hamartoma associated with alopecia and cystic fibrosis in three siblings.

BACKGROUND: Generalized follicular hamartoma is a rare condition that has been described in association with alopecia, myasthenia gravis, and circulating autoantibodies. To date, all reported cases have appeared in female individuals. We report a kindred in which three siblings were affected by this condition in association with alopecia and cystic fibrosis. OBSERVATIONS: Three children of two consanguineous patients were affected by cystic fibrosis. They also had the same phenotype characterized by senilized facies, partial alopecia, and hypohidrosis, severe retardation of physical growth, and hyperelasticity of the skin. In all three children, skin biopsy specimens revealed the presence of basaloid proliferations at the level of the hair follicles that could not be demonstrated in their healthy parents. Myasthenia gravis did not appear during the clinical course of our patients, and circulating autoantibodies were not detected. All three patients died during childhood due to complications of cystic fibrosis. CONCLUSIONS: Generalized follicular hamartoma is a rare condition previously reported in association with alopecia, myasthenia gravis, and/or circulating autoantibodies (antinuclear and antiacetylcholine receptor antibodies). These are the first congenital cases of generalized follicular hamartoma described, and it is also the first time that an association with cystic fibrosis is reported. The striking association of generalized follicular hamartoma with cystic fibrosis in these three siblings suggests that there may be a genetic linkage between the two conditions.

Alopecia↗
PubMed1995

Assessment of histopathologic changes in the colonic biopsy in acute graft-versus-host disease.

The authors reviewed 78 colonic biopsies from 63 patients undergoing bone marrow transplantation (BMT), 56 allogeneic and 7 autologous, to assess different morphologic changes related with intestinal acute graft-versus-host disease (A-GVHD). Only five biopsies were done before the 20th day after BMT. The authors studied the presence of ulcers and atrophy in the mucosa, the nuclear atypia, and inflammatory infiltrate in the surface epithelium, and the architectural abnormalities, as well as dilation of the glands, and the presence of crypt abscesses. The authors explored the glandular epithelium for apoptosis and intraepithelial lymphocytes. They examined the lamina propria for inflammatory infiltrate, especially for so-called "focal periglandular infiltrate (FPGI)," edema fibrosis, increase of capillary vessels and presence of muciphages and clusters of enterochromaffin cells. Cases were grouped according to clinical symptoms and histologic diagnosis of A-GVHD in skin or liver. Group A had 15 asymptomatic cases (control). Group B had 20 cases from asymptomatic patients with a histologically proven A-GVHD. Group C had 43 cases with gastrointestinal symptoms and histologically proven A-GVHD. Then, the relative frequency of every histologic feature previously described were compared in the different groups by means of an univariate analysis. Apoptosis of the glandular epithelium was found in 48 cases, two of them in patients carrying an auto-BMT. Focal periglandular infiltrate was found in 11 cases that was associated with apoptosis (P < .03, Fisher's exact test). In this series, the presence of FPGI was linked with finding apoptosis in a given specimen with a probability of 91%. Glandular architectural changes and dilation were also more frequent in group C than in groups A and B when compared in the univariate analysis.

Acute Disease↗
PubMed1994

Gastric microcirculatory changes of portal-hypertensive rats can be attenuated by long-term estrogen-progestagen treatment.

It has been suggested that estrogen-progestagen therapy may be useful in preventing bleeding from gastric angiodysplasia, a vascular lesion similar to that described in portal-hypertensive gastropathy. In this study we assessed the effects of estrogen-progestagen therapy on gastric microcirculation and systemic and splanchnic hemodynamics in portal-hypertensive and sham-operated rats. One week after the surgical procedure (partial portal vein ligation or sham surgery), animals were given an intramuscular injection of a slow-release preparation of estrogen-progestagen or its vehicle. Two weeks later, gastric mucosal blood flow was measured by means of hydrogen gas clearance, a morphometrical analysis of gastric mucosal blood vessels was performed and systemic and splanchnic hemodynamics were evaluated with a radiolabeled-microspheres technique. In portal-hypertensive rats, estrogen-progestagen therapy induced a significant reduction in gastric mucosal blood flow, number of blood vessels and relative area of vessels. Similar changes, although of lesser magnitude, were achieved with estrogen or progestagen given separately and with the low dose of combined estrogen-progestagen. Estrogen-progestagen treatment also induced significant reductions in portal pressure and porto-collateral resistance without changing systemic or splanchnic hemodynamics. In contrast, estrogen-progestagen treatment did not induce changes in any of the parameters studied in sham-operated rats. We conclude that long-term estrogen-progestagen therapy reduces the gastric hyperemia, increased mucosal vessel density and portal pressure in portal-hypertensive rats.

Animals↗
PubMed1994

Portal hypertensive gastropathy in splenic vein thrombosis.

Portal hypertensive gastropathy has been related to portal hypertension and is a recognized cause of iron deficiency anemia. Although this gastric mucosal lesion has been occasionally described in extrahepatic portal hypertension, its clinical significance and histological features are not well established. We report a case of chronic anemia due to portal hypertensive gastropathy in a patient with splenic vein thrombosis, and suggest that the cure of gastric lesions in this condition may be obtained by splenectomy.

Adult↗
PubMed1993

Delayed appearance of anti-myelin-associated glycoprotein antibodies in a patient with chronic demyelinating polyneuropathy.

A patient who had a polyneuropathy compatible with a chronic inflammatory demyelinating polyneuropathy and was initially negative for anti-myelin-associated glycoprotein (MAG) antibodies developed a double monoclonal gammopathy, IgM kappa and IgM lambda, two years after the diagnosis. The IgM kappa, but not the IgM lambda, exhibited strong anti-MAG antibody activity. The late appearance of the anti-MAG immunoreactivity suggests that in patients with an initial diagnosis of chronic inflammatory demyelinating polyneuropathy, the search for anti-MAG antibodies should be repeated during the course of the neuropathy.

Autoantibodies↗
PubMed1993

Malignant sinonasal epithelioid schwannoma.

Malignant schwannomas are rare neoplasms that are seldom found in the head and neck. Few cases have been reported involving paranasal sinuses and none of them was of the "epithelioid" type. In this report, an unusual case of epithelioid malignant schwannoma involving the maxillary sinus, nasal cavity and orbit is presented. The patient was a 27-year-old male with a history of headache, nasal obstruction and epistaxis. Histologically, the tumour had a biphasic pattern with spindle and epithelioid elements which led to a differential diagnosis with malignant melanoma. It had also to be distinguished from other neoplasms, such as squamous cell carcinoma and olfactory neuroblastoma because of it location. Immunohistochemical positivity for S-100 protein, glial fibrillary acidic protein and vimentin together with negativity for HMB-45 and cytokeratins, as well as mesaxon formation detected with electron microscopy were conclusive in the diagnosis. The patient was treated with surgical excision and radiotherapy but local recurrence and metastases occurred, and he died within 1 year after initial diagnosis.

Adult↗
PubMed1993

Cytomegalovirus colitis in chronic renal failure.

Cytomegalovirus (CMV) is an ubiquitous agent and is recognized as a pathogen in all age groups. Although symptomatic infection by CMV (CMV disease) in normal adults is generally mild, the virus is known to produce severe symptoms, mostly in immunocompromised patients. In this group of patients, the primary involvement of the colon by CMV is reported to be rare. However, chronic renal failure patients are prone to CMV infection. Nevertheless, despite the high incidence of infection in these patients and the recognition of a defective immune response in some of them, clinical manifestations are an exception. Two chronic renal failure patients who developed acute colitis due to CMV infection are presented. Of interest are the rarity of the association, the favorable clinical course and the differential diagnosis with other gastrointestinal disorders which are more common in these patients.

Aged↗
PubMed1992

[Cavernous hemangioma of the spleen with a localized intravascular coagulation syndrome (Kasabach-Merritt syndrome). Ultrastructural study].

Haemangioma is the commonest non-malignant tumour of the spleen. Cytopenia and coagulopathy, secondary to cell trapping and coagulation factors consumption inside the haemangioma, respectively. May occasionally occur as accompanying phenomena. A woman is presented who had splenic haemangioma associated with leucopenia and thrombocytopenia along with decrease fibrinogen and prolonged prothrombin time as well as low complement rates. She had massive splenomegaly with portal hypertension, and the partial oxygen pressure values in mesenteric and portal blood were concurrent with the presence of arteriovenous shunts. The leucocyte and platelet count, the serum complement, the fibrinogen rate and the prothrombin time recovered after splenectomy. The cavernous characteristics of the spleen, containing multiple thrombi, were confirmed upon scanning electron microscope examination. The pathophysiology of the cytopenias and clotting factors consumption is discussed, stress being laid on the ultrastructural findings. A review is presented of the clinical aspects, differential diagnosis and treatment.

Adult↗
PubMed1991

[Superficial gastric cancer in Barcelona. Importance of the detection of severe epithelial dysplasia in biopsies obtained under endoscopic control in the diagnosis of early gastric cancer].

BACKGROUND: Severe mucosal dysplasia (SMD) in endoscopic gastric biopsies is a controversial lesion because some authors consider it as an histologic lesion associated to superficial gastric cancer (SCG). This study is aimed to asses the prevalence of SCG in Barcelona; if the presence of SMD is associated to SCG and, the clinical and endoscopic manifestations which induced the diagnosis. METHODS: We studied a total of 4,800 patients who had been submitted to gastroscopy during 1.5 years. A total of 79 patients suffered gastric cancer, 56 of them were submitted to resection. Ten of these 56 patients (17%) had SCG. The most frequent first clinical manifestation of SCG was gastrointestinal haemorrhage. RESULTS: Endoscopic aspect suggested malignancy in five cases. Biopsies during endoscopy allow diagnosis of gastric cancer in five. In four histologic studies showed SMD. In the tenth patient, the first endoscopy with biopsies showed a gastric peptic ulcer. A later endoscopic control showed the persistence of the macroscopically benign lesion but one among seven biopsies showed adenocarcinoma. The four patients with SMD had gastric cancer in further endoscopic procedures, except in one which was operated after two endoscopies demonstrating SMD. CONCLUSIONS: This study suggests: 1) prevalence of SGC in Barcelona is similar than the other European countries. 2) haemorrhage is a frequent first manifestation of SCG, and 3) the finding of SMD in endoscopic biopsy strongly suggests the presence of SCG in the stomach.

Adenocarcinoma↗