Self-esteem and quality of life in patients with neurogenic dysfunction and continent urinary diversion and/or procedures for anterograde enemas.

INTRODUCTION AND OBJECTIVE: The importance of evaluating the self-esteem and quality of life of patients with a chronic disease has become increasingly relevant. The study describes self-esteem and quality of life outcomes in patients with neurogenic dysfunction and continent urinary diversions and/or antegrade enema procedures. MATERIALS AND METHODS: Cross-sectional observational study, including patients with neurogenic dysfunction who required a continent urinary diversion and/or an antegrade enema conduit during their treatment. The self-esteem evaluation was done with the Rosenberg scale and the quality of life with the KIDSCREEN-27 questionnaire. VARIABLES MEASURED: socio-demographic, clinical and economic characteristics. Descriptive statistics were applied. RESULTS: The mean age of the 9 patients was 15.6 years, the mean time elapsed from surgery to application of the questionnaires was 60.9 ±â€¯37.1 months. With a perception of improvement in 8 of the 9 patients and a normal or higher self-esteem score in all cases. In the description of physical activity and health, it was found that 7 of the 9 young people presented a perception of good health in general. A considerable reduction in the use of diapers was achieved after the intervention, going from consuming 6.2 ±â€¯1.4 diapers per day to only 1.7 ±â€¯1.3 diapers per day. CONCLUSIONS: Despite the sequelae, comorbidities and procedures, the results of self-esteem and quality of life in patients with neurogenic dysfunction are high.

3D evaluation of maxillary arches in unilateral cleft lip and palate patients treated with nasoalveolar moulding vs. Hotz's plate.

To compare the three-dimensional changes occurring in the maxillary arch during the use of modified pre-surgical nasoalveolar moulding (PNAM) and Hotz's plate. A clinical trial including 32 children with unilateral cleft lip and palate (UCLP), 16 treated with Hotz's plate and 16 with PNAM, was performed. Impressions of the maxillary arches were taken: A. prior to pre-surgical orthopaedics, B. before cheiloplasty and C. after cheiloplasty. Models were digitised using a stereophotogrammetric instrument, and geodesic distances were calculated: anterior, canine and posterior widths of the arch, and lengths and cleft depths of the larger and shorter segments. The time and treatment effects were assessed by two-factor anova. A significant effect of treatment was found for cleft depth at the larger segment: children treated with Hotz's plate had significantly deeper cleft than children treated with PNAM. All distances significantly changed during time: the anterior and canine widths decreased, while the posterior width, the lengths and depths of the cleft segments increased. Significant treatment per time interactions was found. The anterior and canine widths reduced more with PNAM between time points A and B while Hotz's treatment was more effective between B and C. The shorter segment depth increased more between B and C with PNAM, and between A and B with Hotz's plate. During pre-surgical orthopaedics, therapy with PNAM obtained the best results in reducing the width at the anterior segment of the cleft. This treatment gave a lower increase in cleft depth than treatment with Hotz's plate.

[Autoamputated ovarian cysts. A clinical case]. / Autoamputación de quiste ovárico. Reporte de caso.

INTRODUCTION: Autoamputation refers to a situation in which a tubo-ovarian remnant is found free-floating in the abdomino-pelvis, which is a complication of fetal ovarian cyst causing ovarian torsion in uterus. There have been only 13 reported cases of ovarian autoamputation involving children under 1 years of age. CLINICAL CASE: We present the case of a 9-month old female with history of abdominal mass in the 26th week of gestation, who was referred to the pediatric surgery department with CT findings in an abdominal tumor and negative physical examination, in laparoscopic examination cystic mass is 6 cm in diameter and no left ovary. The histopathological examination confirmed an ovarian cyst with necrotic and hemorrhagic remaining. COMMENTATION: This case is reported because of the low incidence of pathology, exceptional presentation and the favorable outcome of the patient with implemented laparoscopic management.

Cytoskeletal control of vesicle transport and exocytosis in chromaffin cells.

Chromaffin cell exocytosis is a fascinating interplay between secretory vesicles and cellular components. One of these components is the cytoskeleton and its associated regulatory proteins. Transport of chromaffin secretory granules from their site of biosynthesis towards the active site of exocytosis requires both F-actin fine remodelling as well as microtubule trails. At least two molecular motors, myosins II and V, seem to play a crucial role in the control of F-actin dynamics and vectorial vesicle displacement respectively. Vesicle movement experiences spatial restrictions as they approach the cell cortical region, where the F-actin meshwork constitutes a barrier-limiting vesicle access to the plasmalemma. During secretion, cortical F-actin is locally disrupted providing access of vesicles to release sites on the plasmalemma. Removal of the stimulus restores cortical F-actin. Two pathways (Ca2+-scinderin and PKC-MARCKS) control F-actin changes during the secretory cycle . Furthermore, GTPases such as RhoA, that controls F-actin network integrity, and Cdc42 signalling which induces the formation of local actin filaments at active sites, provide additional evidence on the importance of F-actin as a key element in vesicle transport and in the exocytotic machinery of chromaffin cells.

Temperature and PMA affect different phases of exocytosis in bovine chromaffin cells.

Amperometry was used to study secretory kinetics of single bovine chromaffin cells stimulated by transient depolarizations at different temperatures. The initial rate of release was moderately enhanced when the temperature was raised from 18 to 22 and 37 degrees C. Secretion increased drastically at a later period, 5-10 s after the initiation of stimulus. Interestingly, incubation of the cells with phorbol 12-myristate 13-acetate (PMA) clearly enhanced fast secretory components. In addition, the rate of secretion of the slower component recruited by prolonged depolarizations (t > 30 s) was unaffected at the range of temperatures normally used in secretory experiments (22-37 degrees C). A 'counting events' analysis of secretion, which avoids the influence of event charge changes, showed specific increases in a population of vesicles fusing between 7 and 12 s over the same range of temperatures, and a marked increase in vesicles fusing during the initial phase (1-5 s), of PMA-treated cell secretion. An analysis of temperature influence on transient components released by high sucrose, the secretion elicited by cell permeabilization with digitonin, and studies of the individual characteristics of amperometric events, allow us to conclude that an increase in the size of a secondary-released vesicle population is the main factor contributing to temperature-dependent enhancement of secretion, in clear contrast to the enhancement of fast releasable pools caused by phorbol esters.

The F-actin cytoskeleton modulates slow secretory components rather than readily releasable vesicle pools in bovine chromaffin cells.

Adrenal chromaffin cells were used to test the role of the peripheral cytoskeleton of F-actin in controlling different vesicle pools. Phorbol 12-myristate 13-acetate and calyculin A, two substances affecting phosphorylation-dephosphorylation cycles, produced different degrees of F-actin reorganization, inducing the partial and the almost total disassembly of this structure, respectively, as visualized using rhodamine-phalloidin staining. Consequently, electron microscopy studies revealed the higher efficiency of calyculin-A over phorbol 12-myristate 13-acetate in promoting vesicle access to the plasmalemma boundary. Surprisingly, only the phorbol ester enhanced fast kinetics and the population of rapidly releasable vesicle pools as studied by single-cell amperometry, whereas both agents, as well as the F-actin severing compound, Latrunculin A, promoted an increase in the population of vesicles recruited in response to prolonged or repetitive stimulations. Taken together, our data support the notion that the F-actin peripheral barrier controls primary granule recruitment from reserve vesicle pools, whereas the phorbol ester effect on the rapidly releasable pools might be related to the alteration of late secretory stage through protein kinase C-dependent phosphorylation of an unidentified target.

A single amino acid near the C terminus of the synaptosomeassociated protein of 25 kDa (SNAP-25) is essential for exocytosis in chromaffin cells.

Amperometry in chromaffin cells expressing green fluorescent protein (GFP) fused to synaptosome-associated protein of 25 kDa (SNAP-25) have been used to test the involvement of single amino acids in exocytotic function, overcoming some of the limitations of studies based on Botulinum neurotoxin cleavage, as this occurs at defined sites of the protein. Constructs containing either the whole SNAP-25 polypeptide or several deleted forms lacking its C-terminal domain were heavily overexpressed in transfected cells. All GFP-fusions were located in both the cytoplasm and the plasma membrane. Although a construct containing complete SNAP-25 sustained normal secretion, removal of four or more amino acids of its C terminus greatly altered the overall rate and extent of exocytosis. Further mutational analysis proved that Leu203, the fourth residue from the C terminus, is critical for secretion. Kinetics of single granule fusions from cells expressing truncated forms showed slow onset and decay times when compared with control cells expressing full SNAP-25. Thus, these data provide direct evidence for the involvement of a specific residue of SNAP-25 in exocytosis and show that overexpression of GFP-SNAP contructs combined with single vesicle fusion measurements constitutes a powerful approach to dissect the structural elements playing a role in individual steps of the exocytotic cascade.

Symmetric lipomatosis of the tongue: report of a case and review of the literature.

Lipomatosis of the tongue is an extremely rare condition. To date, only 5 cases have been reported in the literature. An additional example, confirmed by biopsy, is presented. This appears to represent only the second instance of a nonoriental person who has been described with this condition.

The 26-mer peptide released from SNAP-25 cleavage by botulinum neurotoxin E inhibits vesicle docking.

Botulinum neurotoxin E (BoNT E) cleaves SNAP-25 at the C-terminal domain releasing a 26-mer peptide. This peptide product may act as an excitation-secretion uncoupling peptide (ESUP) to inhibit vesicle fusion and thus contribute to the efficacy of BoNT E in disabling neurosecretion. We have addressed this question using a synthetic 26-mer peptide which mimics the amino acid sequence of the naturally released peptide, and is hereafter denoted as ESUP E. This synthetic peptide is a potent inhibitor of Ca2+-evoked exocytosis in permeabilized chromaffin cells and reduces neurotransmitter release from identified cholinergic synapses in in vitro buccal ganglia of Aplysia californica. In chromaffin cells, both ESUP E and BoNT E abrogate the slow component of secretion without affecting the fast, Ca2+-mediated fusion event. Analysis of immunoprecipitates of the synaptic ternary complex involving SNAP-25, VAMP and syntaxin demonstrates that ESUP E interferes with the assembly of the docking complex. Thus, the efficacy of BoNTs as inhibitors of neurosecretion may arise from the synergistic action of cleaving the substrate and releasing peptide products that disable the fusion process by blocking specific steps of the exocytotic cascade.

Preferential localization of exocytotic active zones in the terminals of neurite-emitting chromaffin cells.

Amperometry using 2.5 microm radius carbon fiber electrodes was employed to study exocytotic catecholamine release from individual cultured bovine chromaffin cells. The secretory responses to either direct depolarization or nicotinic receptor stimulation were focal in nature in both round and neurite-emitting cells. In contrast to the random distribution of active sites found in round cells, bipolar and tripolar chromaffin cells had responsive zones preferentially located at neurite terminals as indicated by the lower probability of finding "silent" electrode positions and an increased nicotinic-receptor responsiveness when compared with the cell body. In agreement with these data we have observed a preferential deposition of dopamine-beta-hydroxylase into the neurite terminal plasmalemma after stimulation of intact cells. These observations might be of interest since the differences in the distribution of secretory "spots" between round and neurite-emitting chromaffin cells could be used to study the molecular factors determining active site localization.

A peptide that mimics the C-terminal sequence of SNAP-25 inhibits secretory vesicle docking in chromaffin cells.

Excitation-secretion uncoupling peptides (ESUPs) are inhibitors of Ca2+-dependent exocytosis in neural and endocrine cells. Their mechanism of action, however, remains elusive. We report that ESUP-A, a 20-mer peptide patterned after the C terminus of SNAP-25 (synaptosomal associated protein of 25 kDa) and containing the cleavage sequence for botulinum neurotoxin A (BoNT A), abrogates the slow, ATP-dependent component of the exocytotic pathway, without affecting the fast, ATP-independent, Ca2+-mediated fusion event. Ultrastructural analysis indicates that ESUP-A induces a drastic accumulation of dense-core vesicles near the plasma membrane, mimicking the effect of BoNT A. Together, these findings argue in favor of the notion that ESUP-A inhibits ATP-primed exocytosis by blocking vesicle docking. Identification of blocking peptides which mimic sequences that bind to complementary partner domains on interacting proteins of the exocytotic machinery provides new pharmacological tools to dissect the molecular and mechanistic details of neurosecretion. Our findings may assist in developing ESUPs as substitute drugs to BoNTs for the treatment of spasmodic disorders.

Rinofima; tratamiento con radiocirugía. Reporte de un caso / Rhinophyma; radiosurgery treatment. Report of one case

Comunicamos el caso de un paciente masculino de 72 años con diagnóstico de rinofima de varios años de evolución. El paciente fue tratado con radiocirugía con exlente resultado cosmético. Se presenta a la radiocirugía como un tratamiento que utiliza un flujo de electrones por alta frecuencia para incidir, abatir o coagular tejido

[Metastasizing pleomorphic adenomas. Clinical and cytometric analysis]. / Adénomes pléomorphes métastasants. Analyses cliniques et cytométriques.

Metastasizing pleomorphic adenoma is a rare lesion, histologically benign but with a malignant clinical behaviour. Two cases are reported with a flow cytometric study. Recurrent adenoma and its metastasis were aneuploid in one case.

Tongue necrosis as a complication of temporal arteritis.

Tongue necrosis is a rare complication in giant cell arteritis, an entity in which both temporal arteritis and rheumatic polymyalgia may be included as two different manifestations of the same pathologic process. The case of a 79-year-old patient who had tongue necrosis 3 hours after ingestion of 2 mg of ergotamine tartrate is presented. This complication was the basis for the diagnosis of temporal arteritis. We reviewed possible clinical manifestations of temporal arteritis and cases of tongue necrosis in the world literature. The possible existence of triggering factors that seemed to be present in 11 of the published cases was analyzed. In seven of these cases ingestion of ergotamine derivates had taken place.

[American Burkitt's lymphoma with mandibular involvement]. / Lymphome de Burkitt Américain de localisation mandibulaire.

Burkitt's tumor is a undifferentiated lymphoma of lymphocytes B with a characteristic histologic pattern often called as "starry sky". It is classified in two groups: endemic Burkitt's lymphoma (African form), and non-endemic Burkitt's lymphoma (American form). The extranodal involvement of the head and neck in the American type is uncommon, so the diagnosis can be delayed. The authors present in this paper a case of American Burkitt's lymphoma in a patient with gingival hyperplasia, and compare the epidemiological, clinical, radiographic, therapeutic and prognostic characteristics of the endemic and non-endemic forms of Burkitt's lymphoma.

Ruthenium red inhibits selectively chromaffin cell calcium channels.

The effect of Ruthenium red (RR) on ionic currents and catecholamine secretion was studied in chromaffin cells. This polycation inhibited 59 mM potassium-stimulated 45Ca2+ uptake in a concentration-dependent manner (IC50 = 5 +/- 0.2 microM). This effect was more evident at extracellular calcium concentrations over 1 mM and was not abolished by neuraminidase pretreatment. RR also inhibited potassium-stimulated catecholamine secretion (IC50 = 6 +/- 0.9 microM). These results were corroborated by patch-clamp in whole-cell recordings. RR inhibited chromaffin cell calcium currents (IC50 = 7 microM) without affecting significantly either sodium or potassium currents. Radioligand binding studies in adrenomedullary plasma membranes showed that RR inhibited [125I]omega-conotoxin GVIA binding but it had no effect on specific binding of [3H]nitrendipine. The effect of the RR on calcium currents was additive with the inhibitory effect observed with 10 microM nitrendipine. The residual dihydropyridine-resistant calcium current was inhibited with a potency similar to that determined under control conditions in the absence of nitrendipine. These results demonstrate that RR selectively inhibits calcium channels; however, this polycation was not selective for a particular calcium channel subtype.

[Temporomandibular compression due to condylar osteochondroma]. / Constriction temporo-mandibulaire par un ostéochondrome condylien.

We describe in this work one case of osteochondroma of the mandibular condyle. The first clinical sign was a limitation of the mouth opening. Based on this lesion, a review of the literature is made, and different treatment modalities are discussed.

[Combined epithelial odontogenic tumor]. / Tumeur odontogénique épithéliale "combinée".

The "combined epithelial odontogenic tumor" in an uncommon lesion characterized by the synchronous presentation of typical histological feature of the adenomatoid odontogenic tumour, and the Pindborg's tumor. In this work, the authors, describe a case of combined epithelial odontogenic tumor associated with an unerupted maxillary central incisor. The clinical and histological characteristics of this lesion point out that it could be primitively an adenomatoid odontogenic tumor, with several areas of calcifying epithelial odontogenic tumor.

The alpha 1-subunit of skeletal muscle L-type Ca channels is the key target for regulation by A-kinase and protein phosphatase-1C.

Despite the fact that the phosphorylation-mediated regulation of L-type Ca channels is viewed as a model of ion channel regulation, much remains to be learned about the protein phosphorylation and dephosphorylation reactions that underlie the regulation of the channels. The channel isoform most well studied biochemically is that expressed in skeletal muscle. The alpha 1- and beta-subunits of this channel isoform are substrates for protein kinase A, but it is unknown if phosphorylation or dephosphorylation of both subunits contributes to altered channel properties. Here, we report experiments in which the alpha 1- and beta-subunits were differentially phosphorylated by protein kinase A and dephosphorylated by protein phosphatase 1c under conditions that led to channel regulation. The results suggest that the alpha 1-subunit plays a key role in the phosphorylation and dephosphorylation-dependent regulation of the L-type Ca channels from skeletal muscle.

Solubilization, characterization and photoaffinity labeling of the mitochondrial dihydropyridine receptor from bovine adrenal medulla.

1. The mitochondrial dihydropyridine receptor was solubilized with Chaps at a detergent/protein ratio of 2.5, during 45 min at 4 degrees C. 2. From the rate constants of association (8.10 +/- 0.25 x 10(4) M-1 min-1) and dissociation (0.022 +/- 0.001 min-1) a Kd of 275 nM was calculated, while from saturation experiments a Kd of 270 +/- 30 nM and a density of receptors of 106 +/- 9 pmol/mg protein was obtained. 4. The solubilized receptors are heat-resistant, sensitive to the trypsin and to the reduction of disulfide bonds. 5. In native membranes, a polypeptide of 50 kDa was specifically photolabelled with [3H]Azidopine.