Bladder Fetal Rhabdomyoma Intermediate Type.

INTRODUCTION: Rhabdomyomas are benign tumors of striated muscle, the bladder localization is very rare. CLINICAL CASE: We present an 87-year-old male consulting for gross hematuria. Cystoscopy was done with evidence of bulged bladder mucosa in right side wall and dome. Post-transurethral resection of the bladder (TURB) pathological anatomy was negative for malignancy. As extension study abdominopelvic computed tomography was performed identifying a bladder thickening of right posterior sidewall and an increased density of the adjacent fat. Second TURB was performed and a fetal bladder rhabdomyoma intermediate type was obtained. We performed another biopsy to confirm this rare pathology, with the same diagnosis. Subsequently, the patient continues with hematuria deciding on hemostatic radiotherapy (not candidate for cystectomy or arterial embolization). Currently, the patient is asymptomatic. DISCUSSION: Bladder rhabdomyomas are rare tumors, and, in fact, there have been only 5 papers published. Some cases are only isolation cited in the bladder mesenchymal tumors, and other polemic cases in which clinical and macroscopic characteristics remembered a rhabdomyosarcoma. The importance of this publication case is the macro- and microscopic images that can corroborate the final diagnosis, helping us to differentiate between rhabdomyoma, rhabdomyofibroma, or the malignant rhabdomyosarcoma, and shows the treatment possibilities of these tumors.

Progressive renal failure as the first manifestation of monoclonal light-chain deposition disease with rapid multiple organ involvement.

Light-chain deposition disease (LCDD) is an uncommon condition in which monoclonal light chains are deposited in different organs causing varying degrees of tissue damage. We report the case of a 56-year-old male with progressive renal failure as the first manifestation of LCDD without initial evidence of monoclonal immunoglobulin protein in either serum or urine. The patient later developed severe systemic disease with rapid multiple organ involvement. Finally, he died 2 months after diagnosis, despite steroid and cyclophosphamide therapy.

Relation between body fat and liver fat accumulation and cytokine pattern in non-alcoholic patients with chronic HCV infection.

BACKGROUND: Liver steatosis in chronic hepatitis C virus (HCV) infection is multifactorial. Therefore, there is not necessarily a relation between obesity and liver fat.On the other hand, body fat secretes cytokines, and cytokines and oxidative damage play important roles on progression of liver disease. METHODS: We analyzed the relationships between liver fat (assessed by histomorphometry) and trunk and subcutaneous fat (waist perimeter, triceps skinfold, BMI); the relationships between liver and body fat and cytokines (IL-6, TNF-alpha, IL-8, IFN-gamma, IL-4), adipokines (adiponectin and TIMP-1), and serum malondiladehyde and antioxidants (glutathione peroxidase and superoxide dismutase (SOD) activities); and the relationships of these data with histological changes in 40 HCV-infected non-alcoholic patients. RESULTS: Significant correlations were found between liver fat and waist perimeter and BMI, and between serum TIMP-1 and liver fat. Serum TIMP-1 was significantly related to body fat stores; serum IL-6 and IFN-gamma were related to histological inflammation. Patients with waist perimeter >102 cm (men) or 88 cm (women) showed increased liver fat. In 38.8% of non-obese patients, liver fat accumulation was intense. CONCLUSIONS: There is a relationship between visceral fat, serum TIMP-1 and liver steatosis. However, at least in some patients, factors different from mere adiposity play a role in liver steatosis.

Primary papillary psammomatous adenocarcinoma of the umbilicus.

The histological and ultrastructural features, as well as the immunoreactivity of one case of uncommon primary papillary and psammomatous adenocarcinoma of the umbilicus are studied in the present work. The observations have been undertaken in a nine-year follow-up, and have included the primitive tumour, two local recidives, and inguinal lymphatic metastasis on two occasions. Papillary structures, numerous psammoma bodies, as well as weak and focal positive reactions to CEA and cytokeratin were present in all the tumours. Since these features and their ultrastructural characteristics were identical to primary papillary serous neoplasias of the peritoneum and ovarium, the hypothesis of an origin in coelomic remnants is considered.

Contribution of the proximal and distal nerve stumps to peripheral nerve regeneration in silicone chambers.

The specific contribution of the proximal and distal nerve stumps across an 8 mm gap within silicone chamber regeneration models was studied. For this, proximal and distal (Group A), distal and distal (Group B) and proximal and proximal (Group C) nerve stumps were placed in opposite ends of silicone chambers. In all the groups, a tissue cable forms between the nerve stumps, demonstrating that, without distinction, proximal or distal stumps can stimulate the growth of other proximal or distal stumps. Furthermore, in Group B, the newly formed pseudo-nerve, in the absence of regenerating axons, contains a number of Schwann cells significantly similar to Group A, which confirms that proliferation and migration of Schwann cells do not require axonal presence or contact. Likewise, the findings demonstrate that, with the exception of the axons, the distal stump contributes to the peripheral nerve regeneration in the same way as the proximal stump. Finally, when proximal stumps are placed in both the opposite ends of the silicone chamber, Schwann cells and regenerating axons grow into the chamber gap from both inserts, and myelination also proceeds from both ends to the centre of the chambers.

Clinical and prognostic value of serum procollagen levels in chronic alcoholic liver disease.

Liver fibrogenesis involves the synthesis of collagen fibrils and proteoglycans by various types of liver cells, including Ito cells, transitional cells, myofibroblasts and hepatocytes. Synthesis of collagen fibrils follows a complex metabolic pathway with intermediate products such as type III procollagen (III-PC). Serum levels of III-PC may reflect the activity of the fibrogenetic process. We analysed the relationship between the serum levels of III-PC (N-terminal peptide) and diverse clinical, biochemical and histological parameters of 77 alcoholic patients (27 cirrhotics), comparing them with those of 15 age- and sex-matched controls. A highly significant difference was obtained between controls and patients (P less than 0.0001), but no differences were observed between cirrhotics and non-cirrhotics. Serum III-PC significantly correlated with clinical and biochemical data of liver function derangement (prothrombin activity, serum albumin, bilirubin, gynecomastia, ascites, encephalopathy, edema, splenomegaly); with the duration of ethanol addiction and with MCV. Sixty patients were followed up for a period ranging between 3 and 1056 days (mean = 356 days); 9 of them died. Patients with III-PC levels above 38 ng/ml had a significantly higher mortality (P = 0.006) than those with levels under 38 (log rank test). Thus, serum III-PC may be a useful tool in the clinical evaluation and prognostic assessment of patients with chronic alcoholic liver disease.

Sodium butyrate inhibits carcinoma development in a 1,2-dimethylhydrazine-induced rat colon cancer.

BACKGROUND: Butyric acid is one of the most important by-products of dietary fiber degradation. It is an important trophic agent for the intestinal mucosa under different experimental conditions. Data obtained from several in vitro studies strongly suggest that butyrate can be a potential therapeutic agent in controlling the growth of some cancer cells. However to date in vivo animal studies have failed to show conclusive results. METHODS: We evaluated the effects of intracecal administration of butyrate in an experimental model of colonic carcinogenesis induced by 1,2-dimethylhydrazine (DMH), administered at the site where it is naturally produced, the cecum. We studied the incidence of colon tumors and their main histologic features. RESULTS: Direct application of sodium butyrate significantly decreased the total number of tumors and the incidence of malignancies and carcinoma in the colon. CONCLUSIONS: Butyrate may inhibit the growth of tumors induced by DMH.

Case report. Pulmonary involvement in an adult male affected by type B Niemann-Pick disease.

We report the case of a 39-year-old male patient affected by type B Niemann-Pick disease, in whom pulmonary involvement became evident 15 years after the initial diagnosis. Pulmonary involvement was discovered incidentally during the evaluation of a dry cough and exertional dyspnoea which occurred in the context of an acute febrile, self-limiting illness. In this case, the pulmonary involvement is clinically mild, with minimal alteration of the diffusing capacity for carbon monoxide (DL(CO)), despite moderate fibrosis and widespread infiltration of both alveoli and interstitium by sea blue histiocytes.

Prognostic significance of Ki-67 nuclear proliferative antigen, bcl-2 protein, and p53 expression in follicular and diffuse large B-cell lymphoma.

We analyzed 104 patients with non-Hodgkin's lymphoma, follicular or diffuse large-B-cell-type lymphoma, in order to evaluate the correlation between clinical characteristics and immunohistochemical parameters. Immunostaining was performed by means of monoclonal antibodies against Ki-67, bcl-2, and p53 expression. Forty-nine of the patients showed follicular lymphoma. A high expression of bcl-2 was found in 93%, high expression of p53 in 57%, and low expression of Ki-67 in 96%. Follicular lymphoma grade III showed a p53 expression (p = 0.07) slightly higher than follicular lymphoma grades I and II, not reaching statistical significance. Follicular lymphoma grades I and II tended to express lower Ki-67 and higher levels of bcl-2 expression than grade III (p = 0.06). Fifty-five cases showed diffuse large-B-cell lymphoma. Among them, bcl-2 was absent in 39%, whereas p53 and Ki-67 expression were high in 38%. In the diffuse large-B-cell lymphomas, a high bcl-2 expression correlated with stages III and IV (p = 0.03) and involvement of more than one extranodal area (p = 0.03). High Ki-67 expression was also associated to extranodal involvement of more than one area (p = 0.03). Overall survival of patients did not show statistically significant differences regarding Ki-67, bcl-2, and p53 tumoral expression. Prognostic factors for overall survival in the multivariate analysis were age (p = 0.02) and LDH (p = 0.003). Time to progression was worse among follicular lymphoma with high p53 expression than with mild/moderate p53 expression (p = 0.009).

Relative and combined effects of ethanol and protein deficiency on gonadal function and histology.

The aim of the present study is to analyse the relative and combined effects of ethanol and protein deficiency on serum testosterone and LH, and on gonadal histology, in ethanol fed rats. The study was performed in 32 animals divided into four groups, fed with the Lieber & DeCarli control, 36% ethanol, 2% protein, and 36% ethanol 2% protein containing diets, respectively. Two months later, rats were anaesthetized with pentobarbital and sacrificed, and the right testes and epididymus were carefully removed. Both ethanol and protein deficiency independently lead to a decrease in serum testosterone levels, and to testicular atrophy, lowest testosterone levels and highest degrees of atrophy being observed in the rats receiving the 36% ethanol, 2% protein containing diet. Both serum testosterone and testicular size and weight significantly correlated with final weight and serum albumin. Hypospermia, atrophy of the seminiferous tubules, and reduced epididymal diameter were also observed in this last group of animals. Thus, protein deficiency may contribute to hypogonadism of alcoholics.

[Cytometric study of colonic tumors in a model of experimental colonic cancer. Impact of the diet]. / Estudio citométrico de tumores colónicos en un modelo de cáncer de colon experimental. Influencia de la dieta.

Butyrate is a short chain fatty acid, made up of four carbon atoms. Along with acetate and propionate, they are the main volatile fatty acids formed by the microbial fermentation of the carbohydrates of dietary fibre in the colon, mainly in the caecum. Additionally, they acidify the intracolonic pH, and they play an important role in the regulation of the absorption of water and sodium. On the other hand, they are, especially butyrate, preferred by the colon cell, as sources of energy alternative to glucose. Besides this, butyrate, in cellular cultures, is a known antineoplasic agent which is characterized by doubling the cellular duplication time for cells in the G1 phase, it increases the activity of certain enzymes, it stimulates the effects of interferon, it modifies the morphology of the cells, which in some cases leads to the reversion of the characteristic transformations of the cancerous cells, and it produces alterations in the chromatin, the nucleoli, elements of the cytoskeleton and the Golgi apparatus. Even though it is not known how it causes these actions, it is thought that the acetylization of histones which it produces, may be an important mechanism. We analyzed the effect of this substance in a colonic carcinogenesis model in Sprague-Dawley rats, in which the tumors were induced with the alkylating agent 1,2-dimethylhydrazide, observing the cytometric pattern of the tumors, and the possible differences between both groups. In one of them, sodium butyrate was continuously infused by means of a intrathecal catheter at a rhythm of 1.5 ml/hour during the tumoral induction which lasted four weeks.(ABSTRACT TRUNCATED AT 250 WORDS)

[Caroli's disease, congenital hepatic fibrosis and renal polycystosis: a little frequent association]. / Enfermedad de Caroli, fibrosis hepática congénita y poliquistosis renal: una asociación poco frecuente.

We report the case of a woman with Caroli's syndrome (Caroli's disease and congenital hepatic fibrosis) and adult polycystic kidney disease. Whereas the association of these two entities with autosomal-recessive-polycystic kidney disease has been widely reported, its association with adult-polycystic kidney disease is very infrequent.

[Acute perforated sigmoid diverticulitis in childhood]. / Diverticulitis aguda de sigma perforada en la infancia.

Colonic diverticulosis is a pathology of high incidence in normal population during the last decades of life. About 2-5 per 100 of patients admitted because of this disease are below 40 years old, and in this group of patients diverticulitis tends to be very aggressive with a high number of complications. We report a clinical case of acute perforation of sigmoid colon due to diverticulitis in a ten year old child who was treated surgically by means of resection of the sigma and primary end-to-end anastomosis. We did not find in the literature consulted any cases of this illness under twenty years of age.

[Inflammatory pseudotumor of the maxillary sinus: report of a new case and review of the literature]. / Pseudotumor inflamatorio de seno maxilar: presentación de un nuevo caso y revisión de la literatura.

A new case of inflammatory pseudotumor (IPT) of the maxillary sinus is presented. The clinical and histological features of this rare entity are discussed. Immunohistochemical studies support a possible myofibroblastic origin. Early diagnosis is important to prevent IPT, classified as a benign disease, from becoming a progressive destructive lesion.

Choroid plexus papilloma with stromal deposition of amyloid and elastic material.

Congophilic birefringent amyloid deposits, with immunostaining for transthyretin (TTR) and amyloid P, associated with numerous coarse, enlarged and thick elastic fibres, are reported in the stroma of two choroid plexus papillomas, a finding not previously described in choroid plexus tumours. TTR was expressed as aggregates of 'doughnut-shaped' bodies, in which the TTR-positive peripheral area encircled the elastic fibre (TTR-negative core). Ultrastructurally, the amyloid microfibrils surrounded the elastic fibres and appeared to continue into the microfibrillar mantle of the latter. The stromal TTR-amyloid deposits associated with abundant elastic fibres in tumours that occur in the choroid plexus may be related to the alteration (production/accumulation, insufficient breakdown and/or extracellular matrix modifications) of some of the choroid plexus functions (removal, target and source of polypeptides, including TTR synthesis) and may be of interest for future studies on choroid plexus polypeptide activity and on protein development into elastomeric and amyloidogenic microfibrils.