[Splenectomy in children. Review of 75 cases].
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Biomedical subjects
Publications and source records attributed to M Bravo.
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INTRODUCTION: Sotos syndrome is a form of infantile gigantism characterized by excessive body size from the time of birth, particular facies, acromegalic changes and signs of non-progressive cerebral involvement. The etiology is unknown. Diagnosis is based on somatometric data and the particular phenotype traits. Biochemical and endocrine studies are normal. Torticollis is a focal dystonia and therefore more common in adults. CLINICAL CASE: A 20 year old woman with macrosomic features since birth presented with: weight 104 kg, height 182 cm; prognathism, hypertelorism, a broad over hanging forehead with a high hair line; large ears, hands and feet; torticollis towards the right with elevation and anteroversion of the right shoulder which caused symptomatic scoliosis. She was bradypsychic and rather slow in speech. The complementary tests done (cerebral and cervical CT and MR, bone gammography, evoked potentials, EMG-ENG, sural nerve biopsy, biopsy of skin and muscle, EEG and hormone and biochemistry studies) were normal. The torticollis was treated with botulinus toxin and improved considerably, as did the scoliosis. CONCLUSIONS: To date, dystonia has not been described in association with Sotos syndrome. This may be a causal association, or even perhaps hereditary, since the patient's mother had dystonia (in the form of blepharospasm).
OBJECTIVES: We show the effectiveness of treatment with continuous intrathecal baclofen infusion in a case of hereditary generalized dystonia refractory to anticholinergics, tetrabenazine, pimozide, L-dopa, benzodiazepines and thalamotomy. CLINICAL CASE: A 26 years old female patient, when she was 11 years old began with torsion dystonia in her left feet, that progressively worsened to involve her entire body. She had painful spasms. She had four brothers, three of them with dystonia and one healthy. Her uncle grandfather had similar symptoms. Complementary explorations to reject secondary origin was negatives. She was treated with high and progressive dosages of anticholinergics, pimozide, tetrabenazine, benzodiazepines, L-dopa and thalamotomy without improvement. Underwent intrathecal baclofen test dosing, we used 25, 50, 100 micrograms/day, the last one with improvement during 10 hours. A pump was inserted with an initial dose of 220 micrograms/day. After pump insertion, baclofen dosage was gradually increased to 450 micrograms/day. She had a great improvement in right part of her body and less in her left body. Painful spasms had disappeared. CONCLUSION: We propose continuous baclofen intrathecal infusion pump for patients with severe torsion dystonia that not response to ordinal treatment.
A total of 103 clinical samples of carious dentin were used to study the antibacterial action of different dental resin adhesive materials (Gluma 2000, Syntac, Prisma Universal Bond 3, Scotchbond Multi-Purpose and Prime&Bond 2.0) glass ionomer cements (Ketac-Cem, Ketac-Bond, Ketac-Silver, Ketac-Fil) resin-modified glass ionomer cements (Fuji II LC, Vitremer and Vitrebond) and a compomer (Dyract). The agar plate diffusion method was used for the microbial cultures and a chlorhexidine control. The growth of the caries-producing microorganisms was effectively inhibited by the Vitremer and Vitrebond cements, and to a lesser extent by the Scotchbond Multi-Purpose adhesive system. Overall, there were statistically significant differences in the antibacterial activity of the products tested.
BACKGROUND: The present study aimed to analyze the pattern of p53 expression and its influence on survival in patients with oral squamous cell carcinoma (OSCC). MATERIALS AND METHODS: An immunohistochemical technique with BP53-12 antibody was performed on stored tissue from 78 patients with OSCC (intraoral cancer n=40; lip cancer n=38). The nuclear and cytoplasmic extension of p53 staining was assessed. Clinical and histopathological data were gathered and the patient survival was analyzed. RESULTS: 57.7% (n=45) of the OSCCs expressed p53, with nuclear expression in 52.6% (n=41) of cases and cytoplasmic expression in 24.4% (n=19). The OSCCs with extensive nuclear expression of p53 showed dissociated patterns of invasion of adjacent tissues (p<0.05). A greater extension of cytoplasmic expression of p53 most commonly appeared in tumors that were better-differentiated (p<0.005), more keratinized (p<0.01) and with less nuclear atypia (p<0.05). The parameters that significantly influenced survival of patients were tumor localization (p<0.01), size (p<0.0001), lymph node invasion (p<0.0001), clinical stage (p<0.0001), differentiation degree (p<0.01) and nucleargrade (p<0.01). CONCLUSION: The expression of p53 protein did not behave as a marker of prognostic value in patients with OSCC.
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Because dental fissures may serve as reservoirs for mutans streptococci, preventive measures should be taken to control microbial concentrations at these sites. This study estimated the influence that sealing permanent first molars would have on the levels of mutans streptococci and lactobacilli in saliva of healthy seven-year-old schoolchildren. Permanent first molars were sealed in 31 children without caries (NC group) and in 32 children with caries (C group). None of the children had caries in their permanent first molars. Conventional methods were used to count mutans streptococci and lactobacilli in saliva before applying the sealant and again at 4 and 12 weeks after application. Baseline counts of lactobacilli and mutans streptococci were significantly higher in the C group. A significant reduction in mutans streptococci was observed in the NC group at 4 and 12 weeks. It was concluded that fissure sealants in permanent first molars can help reduce salivary levels of mutans streptococci in children without caries.
The conventional intact PTH assays detect not only PTH 1-84 but also inactive fragments (as PTH 7-84) that accumulate in renal failure. There has been a recent development of a new PTH assay that measures only true 1-84 PTH (Whole PTH or CAP assay, Scantibodies). As 7-84 PTH fragment is antagonistic on bone effects of 1-84 PTH, Moniere-Faugere has suggested that 1-84/7-84 PTH ratio less than 1 is predictive of low turnover. We evaluated the usefulness of CAP assay and the 1-84/7-84 PTH ratio as markers of bone turnover in a groups of 24 patients in peritoneal dialysis (PD). Patients were classified as having low bone turn over if they had a Total PTH (similar to intact PTH) of less than 100 pg/ml. We also measured serum CrossLaps (CTX) as another serum resorption marker. Patients had a mean Whole PTH of 95.5 pg/ml and a mesan total PTH of 155.4 pg/l (range 9 to 900). Whole PTH represented 69.1% of total PTH. Fifteen patients (62.5%) had a total PTH of less than 100. These patients had a 1-84/7-84 relationship of 1.9 +/- 1.8 while 9 patients with Total PTH more than 100 had a relationship of 1.29 +/- 0.6 (p = NS). There was a tight correlation between Whole PTH and total PTH (r = 0.98; p < 0.0001) and with serum CTX (r = 0.78; p < 0.0001). We conclude that 1-84/7-84 ratio does not seem useful in the prediction of low bone turnover and that Whole PTH does not seem to be more useful than intact PTH in the prediction of bone turnover in this population. Future studies should correlate this markers with direct measurements of bone turnover in bone biopsies to demonstrate their usefulness in the prediction of the type of renal osteodystrophy.
BACKGROUND: Loss of expression of CD44 has been shown to be a factor of poor prognosis in some types of tumors. The purpose of this study was to analyze this event in relation to the survival of patients with laryngeal cancer. PATIENTS AND METHODS: The expression of adhesion molecule CD44 was studied in 137 patients with laryngeal cancer. Data were gathered on clinical (primary tumor location, pyriform sinus involvement and tongue base damage) and pathologic (T, N, differentiation, inflammatory response, tumor thickness, surgical margin involvement, and CD44 expression) parameters. Immunohistochemical studies were carried out using DF1485 anti-CD44 monoclonal antibody. RESULTS: In 29 tumors (21.1%) < 25%, in 18 (13.1%) 25%-49%, in 42 (30.6%) 50%-74%, and in 48 (35.0%) > or = 75% of the neoplastic cells expressed CD44. A Cox proportional risks multivariate analysis identified CD44 expression and surgical margin involvement as the parameters most associated with survival (p<0.001). CONCLUSION: The reduced expression of CD44 behaves as a marker of a poor laryngeal cancer prognosis.
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A girl presenting with an enlargement of the spleen, thrombocytopenia and microangiopathic hemolytic anemia at birth was studied, and a splenic hemangioma was diagnosed. Total splenectomy was performed with autotransplantation of a segment of normal splenic tissue. The patient has been followed up to age 4 years. She has not presented infections and has permanently shown normal hematological values, with evidence of good function and growth of the auto implanted spleen tissue. This experience supports the usefulness of autotransplantation of the spleen in newborns and infants aged less than 5 years that must undergo splenectomy for non malignant splenic lesions.
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