RESUMO
AIM: This cytohistopathological study was performed for a better knowledge of phenotypes derived from pluripotent stem cells, as well as for precise location of stem cells within the vascular niche in the brain. METHODS: We used light and transmission electron microscopy to demonstrate the presence of stem cells in the vascular wall of microvessels in the cerebral and cerebellar cortex, pia mater (considered by us a cordocytic-vascular tissue), adventitia of larger cortical arteries and veins, and around vessels. We investigated multiple vascular segments and brain tissue in a variety of clinical cases, such as cerebral tumors, cerebrovascular malformations, thromboses in the carotid system, and direct laceration. RESULTS: Our morphological and ultrastructural observations pointed out many changing phenotypes, as well as cell interrelationships within the vascular niche, both for repair processes when cordocytes cooperate with mesenchymal stem cells, and pathological processes such as atherogenesis, tumorigenesis, and neurotrauma. Our results underlie the important roles of cordocytes in their interrelations with precursor/stem cells in the arterial adventitia. CONCLUSION: The cells derived from pluripotent stem cells along different lineages have had different phenotypes as they derived from hematopoietic stem cells or mesenchymal stem cells, with or without epigenetic disregulations or depending on different microenvironments. Cell interactions, phenotypes, and underlying mechanisms, as well as biological responses to different small molecules or compounds, remain to be determined by future molecular insights within the vascular niche.
Assuntos
Encéfalo/ultraestrutura , Diferenciação Celular/fisiologia , Artérias Cerebrais/citologia , Nicho de Células-Tronco/fisiologia , Humanos , Células-Tronco Mesenquimais/citologia , Microscopia Eletrônica de Transmissão , Pia-Máter/ultraestruturaRESUMO
This study is to understand the nature and functional significance of the activated cell death programs and rehabilitation signs during late vascular changes after brain injury. We used light and transmission electron microscopy to describe changes of cells within the vascular endothelium and tunica media of the cortical arteries four weeks after craniocerebral traumatism. Within tunica media of the posttraumatic damaged artery, apoptotic and paraptotic phenotypes were identified as well as some early ultrastructural signs of smooth muscle cells regeneration, these cell highlighting a remarkable degree of plasticity. Surprisingly, some endothelial cells showed an extensive rough endoplasmic reticulum development, whereas other endothelial cells showed typical necrosis. In conclusion, two groups of suicidal cells apoptotic and paraptotic cells were encountered in the same lesional vascular wall after neurotrauma, showing also signs of cell regeneration. The pathophysiologic significance of the coexisting double cell death programs and cell regeneration seems to be in relation with late cell survival, after arterial damage when some cells disappear and other cells try to survive undergoing reversible injury.
Assuntos
Apoptose , Artérias Cerebrais/patologia , Endotélio Vascular/patologia , Regeneração , Túnica Média/patologia , Lesões Encefálicas/patologia , Artérias Cerebrais/lesões , Retículo Endoplasmático/metabolismo , Endotélio Vascular/lesões , Humanos , Masculino , Microscopia Eletrônica , Pessoa de Meia-Idade , Necrose , Túnica Média/lesõesRESUMO
INTRODUCTION: Dysfunctional voiding syndrome in children is characterized by a pattern of dysfunctional bladder emptying due to an active contraction of the external sphincter during micturition. Diagnosis is based on electromyographic and flowmetry results. The treatment is focused on relaxing the external sphincter during micturition where biofeedback is the treatment of choice. By the moment there are still centres without this possibility, alpha blockers are an alternative. OBJECTIVE: To determine the efficacy of alpha blockers as an alternative to biofeedback as a therapeutic possibility. MATERIAL AND METHODS: We included a total of 17 children with dysfunctional voiding syndrome and carried out a retrospective study. We registered age, symptoms at diagnosis, presence of associated urologic problems, flowmetry results pre and post-treatment, type of treatment used and its effectiveness comparing patients treated with alpha blockers and those who are starting to deal with biofeedback. RESULTS: There were 12 girls and 5 boys. The mean age at diagnosis was 4.9 years old, 88% of these children related enuresis, diurnal urinary incontinence and urgency, 57% of them had also urinary infections, 63% constipation, 36% had psychosocial problems. Ten patients were treated with alpha-antagonists: 6 with Tamsulosin and 4 with Doxazosin. They followed this treatment an average of 5.8 months, range between 2 and 12 months. Five patients were treated with biofeedback. All cases had an abnormal pelvic electromyography. Patients treated with alpha-blockers achieved a 70% of electromyographic improvement with a 70% of recurrence. In children treated with biofeedback we got improvement in 80% with no recurrence. After alpha blocker therapy, maximum flow rates and average flow values were better but not statistically significant, this difference was significant with biofeedback. A patient treated with Tamsulosin left treatment due to hypotension, 2 patients left Doxazosin because of dizziness. CONCLUSIONS: Alpha-blockers are effective in the treatment of dysfunctional voiding syndrome with a high percentage of recurrence. They can be an alternative to biofeedback but this one is the effective and definitive treatment.
Assuntos
Antagonistas de Receptores Adrenérgicos alfa 1/uso terapêutico , Doxazossina/uso terapêutico , Sulfonamidas/uso terapêutico , Transtornos Urinários/tratamento farmacológico , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos , Síndrome , Tansulosina , Transtornos Urinários/fisiopatologiaRESUMO
INTRODUCTION: Most children with anorectal malformations have some type of intestinal dysfunction. A correct follow up in this aspect after surgery affects their quality of life. MATERIAL AND METHODS: We gathered a sample of 20 children that were lost in their follow up after posterior sagittal anorectoplasty (PSARP). We got contact with them and they were interviewed and examined in our department. RESULTS: We collected 12 girls and 8 boys. Age range was between 3 and 14 years. 70% had good prognosis for continence (low fistula) and 30% poor prognosis (high fistula). 35% suffered from postoperative complications being the most frequent prolapse especially in high atresias. 85% had good rectal sensitivity, 15% had poor sensitivity that was directly related to incontinence. Anal tone was decreased in our exploration in 35% of patients which was not directly related to their continence. 65% suffered constipation with or without fecaloma. 5% of cases had intestinal hipermotility. The total incidence of fecal loose was 40%. More than a half (62,5%) kept on loosing stool despite treating their constipation or hipermotility, so we consider them true incontinents (no voluntary bowel movements). 67% of children with high fistula were true incontinents, just 7% of those with low fistula. Subjective quality of life in patients with soling was 6.4. In clean patients it was 9.3. Objective quality of life (Score/13) in dirty patients was 6.6. In Clean patients: 11.9. After our bowel management protocol we got 100% of patients clean during school time, thereby improving their quality of life. CONCLUSIONS: Incontinence determines the long-term quality of life in our patients in addition to the psycho-social consequences. They are clearly more frequent in patients with high fistula. Much assume incontinence as an unavoidable part of their disease so do not always demand treatment if they are not followed by a surgeon.
Assuntos
Anus Imperfurado/cirurgia , Qualidade de Vida , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , MasculinoRESUMO
An important goal of gene therapy is to be able to deliver genes, so that they express in a pattern that recapitulates the expression of an endogenous cellular gene. Although tissue-specific promoters confer selectivity, in a vector-based system, their activity may be too weak to mediate detectable levels in gene-expression studies. We have used a two-step transcriptional amplification system to amplify gene expression from lentiviral vectors using the human insulin promoter. In this system, the human insulin promoter drives expression of a potent synthetic transcription activator (the yeast GAL4 DNA-binding domain fused to the activation domain of the Herpes simplex virus-1 VP16 activator), which in turn activates a GAL4-responsive promoter, driving the enhanced green fluorescent protein reporter gene. Vectors carrying the human insulin promoter did not express in non-beta-cell lines, but expressed in murine insulinoma cell lines, indicating that the human insulin promoter was capable of conferring cell specificity of expression. The insulin-amplifiable vector was able to amplify gene expression five to nine times over a standard insulin-promoter vector. In primary human islets, gene expression from the insulin-promoted vectors was coincident with insulin staining. These vectors will be useful in gene-expression studies that require a detectable signal and tissue specificity.
Assuntos
Técnicas de Transferência de Genes , Vetores Genéticos , Células Secretoras de Insulina/metabolismo , Insulina/genética , Lentivirus/genética , Ativação Transcricional/genética , Animais , Linhagem Celular , Expressão Gênica , Proteínas de Fluorescência Verde/genética , Humanos , Ilhotas Pancreáticas/metabolismo , Camundongos , Especificidade de Órgãos , Fosfoglicerato Quinase/genética , Regiões Promotoras GenéticasRESUMO
INTRODUCTION: Helix valgus or procident ears is a common problem that affects about 5% of the population. The folds of the antehelix and the overdevelopment of the concha are the most commonly found anatomic alterations of the ear pavilion. In children this pathology usually causes anxiety and an emotional trauma that may interfere in their normal development. MATERIALS AND METHODS: There are a few tipes of techniques to correct helix valgus. We present the application of the technique in our service. We conduct the otoplastia with an outer puntiform technique which allows us to cut the cartilage partially from the outside. Next we fold from the rear the antehelix and hide the concha. RESULTS: We analysed 7 years of the application of this technique and we now present 87 otoplastias conducted to 44 children. The 97% of them were bilateral. No precocious complications have been observed after the surgery. All cases except for one of them have been bilateral. All the patients were satisfied with the aesthetic results. None of them showed relapse. In one case there was a hypertrophic scar that required cutting and in 2 of the cases there was a slight hypercorrection. CONCLUSIONS: Procident ears may occasion a psychological trauma in children. We believe that this technique, which is minimally invasive, provides very satisfactory aesthetic results, the puntiform scar being hardly noticed fifteen days before surgery. The patients need to stay in hospital for a short period, 24-48 hours, and complications are very rare, recidiva has not been described. We strongly recommend this technique for the correction of procident ears.
Assuntos
Orelha Externa/anormalidades , Orelha Externa/cirurgia , Procedimentos Cirúrgicos Minimamente Invasivos , Procedimentos de Cirurgia Plástica/métodos , Adolescente , Criança , Feminino , Humanos , MasculinoRESUMO
INTRODUCTION: Twenty per cent of the operated patients suffering from cryptorchidisim show no palpable testis in the physical check-up. The use of a non-palpable testis in the initial stages is considered to be controversial when deciding between a laparascopic or an inguinal approach. Our aim is to compare the results obtained with these two approaches and evaluate which one of them would be the most relevant as an initial option. MATERIALS AND METHODS: We examined the patients who have been subjected to surgical intervention for non-palpable testis in the last three years. We had a sample of 53 patients. Bilateral non-palpable testes were disregarded. In the case of 35 patients the initial approach was through the groins while in 18 of them the approach was laparascopic. Relevant data were recorded, such as the age of the patient, right or left side, surgical findings, need for a laparascopic or groin approach and associated hernia. RESULTS: The average age of the patients at the time of the surgical treatment was 3.7 years R (1-13 years). 42% of the testes were on the right side and 58% on the left. In an initial stage the inguinal approach was used with 35 patients, 2 of these requiring laparascopic exploration due to a non-concluding check-up; it was concluded that they were 2 cases of anorchia. An initial laparoscopic approach was used with 18 patients. Internal vessels in the canal were found which required an inguinal approach. In 12 patients normal or atrophic testes were observed. Testicular descent was achieved through the groin in 10 of them while in the remaining 2 a combined approach was adopted. CONCLUSIONS: 80% of the patients subjected to a first laparoscopic approach needed a groin approach later on. 11% of the patients subjected to a first inguinal approach required laparascopic examination. In view of the results obtained, we conclude that inguinal exploration diminishes the need for second procedures.
Assuntos
Criptorquidismo/cirurgia , Laparoscopia , Adolescente , Criança , Pré-Escolar , Virilha , Humanos , Lactente , Masculino , Estudos Retrospectivos , Procedimentos Cirúrgicos Urológicos Masculinos/métodosRESUMO
INTRODUCTION: Enuresis affects 15% of the children under 5. Possible etiopatogeny explaining the mechanism of production of nocturnal enuresis has been described, resulting in different terapeutical approaches; however, we cannot speak up to now of general guidelines for its treatment. MATERIALS AND METHODS: On the basis of 544 children who were treated in our hospital in the last 12 years, we analysed a sample of 124 patients corresponding to the last two years. We now present the therapeutical protocol used and analyse the results. In the initial therapeutical approach a distinction is made between monosymptomatic enuresis and eneuretic syndrome. RESULTS: We studied a total of 120 patients (89 boys and 31 girls). 63% of them showed monosymptomatic enuresis while 37% suffered from enuretic syndrome. In the case of 15% of them, this was associated with heavy sleep and difficulties to wake up. 92% was the general percentage of recovery. In the patients suffering from nonosymptomatic eneuresis, the problem was solved with desmopressin in 87% of them (demospressin on its own in 65% or in combination with oxibutine in the remaining 35%). In the group of enereutic symdrome, the problem was solved with oxibutine in 71% (on its own in 40% or in combination with desmopressin in 54%. CONCLUSIONS: The treatment and solution of enuresis improve the child's self-esteem and the anxiety caused in the family. A good medical history with a clear distinction between enuretic syndrome and monosyntomatic enuresis leads us to a suitable therapeutical approach for every patient, allowing us to find earlier the right treatment for every individual.
Assuntos
Enurese/diagnóstico , Enurese/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Diagnóstico Diferencial , Enurese/classificação , Feminino , Humanos , Masculino , SíndromeRESUMO
INTRODUCTION: The congenital obstruction of the pieloureteral junction (UPJ) is the most frequent cause of hydronephrosis in children. Sometimes, establishing the convenience of a surgical procedure is difficult, mainly if we consider the literature published on the spontaneous resolution of the slight, moderate and even severe hydronephrosis in newborns. OBJECTIVE: To determine the prognostic value of ultrasound in the evolution of the unilateral hydronephrosis, by assesing the size of the contralateral kidney. To verify if the "supranormal" renal function (SRF) is real or an artefact. PATIENTS AND METHODS: We have performed a descriptive observational study over a 10 years period (1995-2005). The study included all patients with the only diagnosis of obstructive unilateral hydronephrosis that underwent pieloplasty, and were controlled for 1 year period after the surgical treatment. RESULTS: Of the 66 patients in the study, 42 were boys (63.6%) and 24 girls (36.4%). After the first diuretic renogram (DR), in 57 of the patients (86.4%) the clearance half-time (T1/2) was over 20 minutes, in 6 cases (9.1%) it was 10-20 minutes and only in 3 cases it was shorter than 10 minutes. A similar differential renal function (FRD) was observed in the moderate and severe hydronephrosis. Supranormal function (FRD > 52%) was detected on DR in 11 patients, predominating in left hidronefrosis, and in more than 50% of the cases this value it did not agree with the renal function measured by dimercapto-succinic acid (DMSA), Pearson's correlation coefficient: 0.19. These kidneys experienced a greater reduction of the postoperative renal function. CONCLUSIONS: In the unilateral obstructive hydronephrosis, the healthy contralateral kidney experiences hypertrophy detectable by ultrasound, but these ecographic diameters are within the band of individual confidence of the healthy children of reference. The supranormal function exists, but in most cases it is an artefact.
Assuntos
Hidronefrose/diagnóstico por imagem , Pelve Renal , Obstrução Ureteral/diagnóstico por imagem , Diurese , Feminino , Humanos , Hidronefrose/etiologia , Lactente , Recém-Nascido , Masculino , Prognóstico , Renografia por Radioisótopo , Estudos Retrospectivos , Ultrassonografia , Obstrução Ureteral/complicaçõesRESUMO
OBJECTIVE: Retroperitoneoscopy has shown that US involution is not synonymous with complete regression of the dysplastic renal parenchyma in US-involuted multicystic dysplastic kidney (MCDK). The objective is to analyze the meaning of this results. PATIENTS AND METHODS: 16 patients (nine girls and seven boys) with unilateral MCDK that showed complete involution on ultrasonography, underwent prospectively a retroperitoneoscopic approach. US showed complete cysts involution at the mean age of 10 months (ranging from five to 22 months). All patients underwent a retroperitoneoscopic approach after US involution of the MCDK. The mean age of retroperitoneoscopy was 36 months (ranging from eight to 56 months). RESULTS: The retroperitoneoscopic approach revealed persistence of dysplastic renal tissue in 100% of the patients. The mean lenght of the renal renmant was 2 cm (ranging from 1 and 3.5 cm). All patients had a mean length of stay of less than 24 hours. Anatomo-pathological study of the samples showed a wide spectrum of dysplastic renal tissue and the absence of preneoplastic cells. CONCLUSIONS: Ultrasonography is our method of choice to follow up MCDK until cyst involution takes place. The presence of a dysplastic renal remnant which is not visible on US, requires an appropriate long-term follow up to screen for the growth of tumors. In our experience, retroperitoneoscopy allows the diagnosis and treatment of the displastic renal renmant in the same minimally invasive ambulatory procedure, avoiding long-term development of tumors. Overall, it is our responsibility to sufficiently inform to the family about the persistence of dysplastic renal remnant to facilitate their decision about the best treatment for their child.
Assuntos
Rim Displásico Multicístico , Feminino , Humanos , Lactente , Laparoscopia , Masculino , Rim Displásico Multicístico/diagnóstico , Rim Displásico Multicístico/diagnóstico por imagem , Rim Displásico Multicístico/cirurgia , Estudos Prospectivos , UltrassonografiaRESUMO
INTRODUCTION: C2 (2-hour post-absorption levels) monitoring of cyclosporine (CsA) seems to reduce the rate of acute rejection episodes (ARE) without increasing nephrotoxicity during the first months after transplant. There are a few reports on the impact of adopting this strategy in patients with stable renal transplants. We herein report a prospective trial in long-term renal transplant patients (>6 months) monitored by C0 or C3 who were switched to C2 monitoring. METHODS: Seventy-six (mean age = 43 +/- 11 years) kidney transplant patients (mean = 37 +/- 21 months after transplant) receiving CsA, steroids, and azathioprine were switched to C2 monitoring, seeking to achieve a target range of 800 +/- 100 ng/mL. The patients were followed for at least 6 months. RESULTS: At conversion the C2 values of 61% of the patients were above and 17% below the therapeutic range. Six months after conversion there was a significant reduction in BUN (29 +/- 11 vs 27 +/- 10, P < .01), Creatinine (Cr), cholesterol, and triglyceride levels were unchanged. Mean CsA dose was decreased 10% from 244 +/- 63 to 220 +/- 52 (P < .01), implying a net savings of 390 US dollars per patient per year. Among the group of patients who showed a high C2 level, there was also a reduction in BUN (30 +/- 12 vs 27 +/- 10, P < .01) and a nonsignificant decrease in Cr (1.53 +/- 0.6 vs 1.50 +/- 0.6). CONCLUSIONS: C2 monitoring in stable kidney transplant recipients is feasible and safe. The strategy results in reduced drug costs and improved renal function.
Assuntos
Ciclosporina/uso terapêutico , Imunossupressores/uso terapêutico , Transplante de Rim/fisiologia , Adulto , Nitrogênio da Ureia Sanguínea , Chile , Custos e Análise de Custo , Creatinina/sangue , Ciclosporina/economia , Ciclosporina/farmacocinética , Monitoramento de Medicamentos/métodos , Feminino , Hematócrito , Humanos , Imunossupressores/economia , Imunossupressores/farmacocinética , Testes de Função Renal , Transplante de Rim/imunologia , Metabolismo dos Lipídeos , Masculino , Taxa de Depuração Metabólica , Fatores de TempoRESUMO
INTRODUCTION: One-year serum creatinine (SCr) level has been used as a surrogate marker for graft survival in kidney transplantation. We evaluated the importance of different factors on this parameter, emphasizing the importance of adequate exposure to Cyclosporine (CyA). METHODS: Ninety-six consecutive renal transplant recipients who underwent transplantation between 1996 and 2002 were treated with CyA, steroids, and azathioprine. Univariate and multivariate regression analyses were performed for 1-year SCr, acute rejection episodes (ARE), and duration of delayed graft function (DGF). We considered adequate CyA levels within 1 week to be >250 ng/mL trough levels (38%) or 3 hour postdose level (C3) >1100 ng/mL (62%). RESULTS: Mean 1-year SCr was 1.52 +/- 0.5, ARE rate was 27%, and DGF rate was 31%. Overall, 53% of patients achieved adequate exposure to CyA at 1 week (68% on those monitored by C3). Univariate analysis identified female recipient gender, decreasing donor age, absence of ARE, and decreased DGF duration to yield lower 1-year SCr (P < .05). On multivariate analysis for donor age (lower), ARE rate, and duration of DGF (shorter) were the only factors considered to be significant for a lower 1-year SCr level. Multivariate analysis for ARE showed that adequate CyA exposure and lower HLA mismatch decreased ARE, whereas the ability to achieve adequate exposure to CyA and shorter cold ischemia time (CIT) correlated with a reduced incidence of DGF. CONCLUSIONS: One-year SCr level is affected primarily by the incidence of ARE, by donor age, and by duration of DGF. Adequate CyA exposure is related to lower ARE; however, its relation to DGF may be influenced by the reluctance to increase exposure on patients with a nonfunctioning graft.
Assuntos
Ciclosporina/uso terapêutico , Transplante de Rim/fisiologia , Creatinina/sangue , Ciclosporina/administração & dosagem , Esquema de Medicação , Feminino , Rejeição de Enxerto/epidemiologia , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/uso terapêutico , Transplante de Rim/imunologia , Masculino , Análise Multivariada , Resultado do TratamentoRESUMO
INTRODUCTION: Delayed graft function has been associated with worse long-term kidney allograft survival. Adequate diagnosis of the etiology of dysfunction is crucial, often requiring routine early biopsies. The aim of this article was to report the results and safety of early kidney allograft biopsies and how they influenced its management. METHOD: Between September 1994 and July 2004, 134 renal transplant recipients were prescribed cyclosporine (CsA; Neoral, Novartis, Chile), steroids, and a third agent (azathioprine in 92% of the graft recipients). Thirty-four patients (26%) had a kidney biopsy performed within the first week because of allograft dysfunction. RESULTS: The main diagnosis was acute tubular necrosis (ATN) in 22 patients (65%), whereas 6 (18%) were diagnosed with an acute rejection episode (ARE), allowing prompt initiation of therapy with reversal of rejection in 4 of them. Two patients (6%) showed signs of thrombotic microangiopathy (TMA) induced by CsA, which subsided following a switch from CsA to tacrolimus (Prograf Pharmainvesti, Chile). In 2 patients, the biopsy specimen showed signs of CsA nephrotoxicity that reverted following dose reduction. Finally, in 2 patients, the biopsy specimen showed chronic nephropathy of donor origin, which had not been previously recognized, resulting in graft loss. There was only one major complication related to the biopsy, intraperitoneal bleeding that required surgical treatment. CONCLUSIONS: Early allograft biopsy is safe and, in a significant number of cases (30%), it detects important allograft pathology (ARE, TMA, and drug toxicity), which when adequately and promptly treated may rescue the graft.
Assuntos
Transplante de Rim/patologia , Doença Aguda , Adulto , Biópsia , Feminino , Humanos , Transplante de Rim/fisiologia , Túbulos Renais/patologia , Masculino , Microcirculação/patologia , Necrose , Circulação Renal , Estudos Retrospectivos , Resultado do TratamentoRESUMO
The technical complexity of previous rheometers has tended to limit the availability of blood viscosity data obtained over a wide range of shear rates. However, an automated tube-type viscometer, the Rheolog, has been developed; it employs a disposable flow assembly and less than five minutes are required to obtain blood viscosity results over a shear rate range of 1-1500 s(-1). We have carried out validation studies of the Rheolog using normal human blood and have compared these results with those obtained by cone-plate and Couette viscometers; storage time and temperature effects were also evaluated. Replicate measurements indicated mean CV levels less than 5%, and were independent of hematocrit and shear rate. Rheolog blood viscosity data agreed closely with those from other viscometers: average Rheolog differences from mean cone-plate and Couette values were -0.3% at 28% hematocrit, -1.4% at 41% hematocrit (i.e., native), and 1.0% at 56% hematocrit. Storage at room temperature up to 8 hours and at 4 degrees C up to 4 days had minimal effects whereas notable changes were observed when stored for 3 hours at 37 degrees C. Our results indicate that, within the hematocrit and shear rate limits employed herein, the Rheolog provides rapid, accurate and reproducible blood viscosity data, and suggest its usefulness for both basic science and clinical studies.
Assuntos
Viscosidade Sanguínea , Hemorreologia/instrumentação , Adulto , Preservação de Sangue/métodos , Desenho de Equipamento , Hematócrito , Humanos , Reprodutibilidade dos Testes , Temperatura , Fatores de TempoRESUMO
The association of congenital cystic adenomatoid malformation with pulmonary sequestration is infrequent. Has been reported less than 60 cases. We describe two patients with this association. One of this with extralobar pulmonary sequestration, and the other with an intralobar one.
Assuntos
Sequestro Broncopulmonar/complicações , Sequestro Broncopulmonar/diagnóstico por imagem , Malformação Adenomatoide Cística Congênita do Pulmão/complicações , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico por imagem , Sequestro Broncopulmonar/cirurgia , Malformação Adenomatoide Cística Congênita do Pulmão/cirurgia , Humanos , Recém-Nascido , Masculino , Tomografia Computadorizada por Raios XRESUMO
INTRODUCTION: The nasal cleft cartilage tends to depression after primary rhinoplasty. Nasal stents are probe to be useful in the management of these patients in order to maintain the new morphology. AIM: The purpose of this paper is to present our experience with the use of postoperative nasal splinting in the management of cleft lip nasal deformity. PATIENTS AND METHODS: A nostril retainer was placed in 18 cleft patients (4 bilateral, 14 unilateral) during primary repair of the cleft lip nasal deformity from 2001 to 2004. The nasal morphology in the postoperative period was compared with that of 10 control patients who were operated on without nasal stenting in a previous period. Nostril retainers were left in place after the rhinoplasty using 4/0 poliglecaprone absorbable. Those sutures fixed the stent in place during three weeks after surgery. Results. Average follow-up was 18.5 months. Retention of the splint was 3.5 months mean time (range 4 weeks to 6 months). The methods employed for retention were multiple based upon parental preferences. Photogrammetric analysis showed relevant asymmetry of the nostrils in 10% of the splinted group as compared with 48% for controls (p<0.001). CONCLUSIONS: The use of nasal stents has been found effective postoperatively after cleft primary rhinoplasty, avoiding relapse of the nasal reconstruction and drop of the nasal ala. Retainers also allows airway patent and avoid surgical adhesions due to nasal secretions and scarring. Parental collaboration is needed.
Assuntos
Anormalidades Múltiplas/cirurgia , Fenda Labial/cirurgia , Nariz/anormalidades , Nariz/cirurgia , Rinoplastia , Stents , Desenho de Equipamento , Seguimentos , Humanos , Recém-Nascido , Rinoplastia/instrumentaçãoRESUMO
We report our experience of renal transplantation in three patients treated for Wilms' tumor (with lung metastasis in two of them), and review 26 previously reported cases in order to define the current indications of transplantation in this setting. Our patients, aged 5-12 years, were transplanted 13-95 months after completion of Wilms' tumor treatment. All three are alive and tumor-free, two with a functioning graft 20 and 97 months after transplantation. Two findings emerge from the review of the literature. First, posttransplant mortality is influenced by the delay between completion of tumor treatment and transplantation. Mortality reaches 79% when that delay is less than one year but falls to 27% when that delay exceeds one year. Second, the prognostic value of pretransplant metastasis depends on its location. All four patients with pretransplant abdominal metastasis died with active metastatic disease. By contrast, of three patients treated before transplantation for metastasis confined to the lung, two are alive and tumor free. We conclude that renal transplantation should be offered to patients successfully treated for Wilms' tumor for at least one year, even if the disease has been complicated by pulmonary metastasis. Several long-term survivors attest that the disease can be cured even under maintenance immunosuppression.
Assuntos
Neoplasias Renais/cirurgia , Transplante de Rim , Tumor de Wilms/cirurgia , Pré-Escolar , Feminino , Humanos , Lactente , Transplante de Rim/mortalidade , Neoplasias Pulmonares/secundário , Masculino , Tumor de Wilms/secundárioRESUMO
BACKGROUND: A defect in platelet function is the main determinant of the prolonged bleeding time in chronic renal failure (CRF). We previously reported a significant correlation between platelet abnormalities and elevated plasma markers of plasmin and thrombin generation. Our aim was to explore the effect of inhibiting both plasmin action with tranexamic acid (TA) and thrombin production with low molecular weight heparin (LMWH), on the bleeding time (BT) and platelet function in patients with CRF. METHODS: 37 patients with CRF (mean creatinine 8.6 +/- 4.4 mg/dl) under conservative treatment, with prolonged BT, entered this study and received TA during 6 days, with (n = 24) and without LMWH (n = 13). BT, platelet aggregation/secretion, platelet granule contents, von Willebrand factor and parameters of coagulation and fibrinolysis were recorded before and at the end of treatment. RESULTS: The BT was shortened in 26/37 (67%) patients. This effect was associated with significant improvement of platelet aggregation and secretion, with decrease to a normal range of fibrin/fibrinogen degradation products, mild increase in plasmin-antiplasmin complexes and pronounced reduction of circulating plasminogen. No differences were seen among patients with or without LMWH. No serious side effects or complications were observed. INTERPRETATION: These findings indicate that the activation of fibrinolysis plays a significant role in the defect of primary hemostasis in patients with CRF. Inhibition of plasmin activity with TA shortens the BT and improves platelet function in the majority of patients with severe disease.
Assuntos
Antifibrinolíticos/uso terapêutico , Fibrinólise/efeitos dos fármacos , Falência Renal Crônica/tratamento farmacológico , Ativação Plaquetária/efeitos dos fármacos , Ácido Tranexâmico/uso terapêutico , Antifibrinolíticos/farmacologia , Tempo de Sangramento , Hemostasia/efeitos dos fármacos , Humanos , Falência Renal Crônica/fisiopatologia , Ácido Tranexâmico/farmacologiaRESUMO
Plasma von Willebrand factor antigen, soluble thrombomodulin, and tissue factor were increased in 31 patients with severe chronic renal failure (creatinine clearance <20 ml/min) under conservative treatment, whereas plasminogen activator inhibitor antigen did not differ significantly from healthy controls. No correlation among plasma levels of these proteins was found. Three patterns of relationship between endothelial cell markers and hemostatic defects were identified: 1) Plasma thrombomodulin, a marker of endothelium damage, was found an independent predictor of bleeding time and platelet aggregation, and secretion defects, and was also related to the severity of renal failure; 2) von Willebrand factor antigen, an index of endothelial cell activation and secretion, was significantly correlated with intravascular markers of thrombin and plasmin generation and with platelet adenosine triphosphate content, but not with plasma creatinine levels; and 3) tissue factor and plasminogen activator inhibitor antigen levels were not statistically correlated with the diverse hemostatic defects. Activation of coagulation and fibrinolysis, secondary to endothelial cell activation, appearing early during the evolution of chronic renal failure, is pathogenically related to the platelet dysfunction, and probably to development of atherosclerosis and thrombotic events in this disease. The progression of chronic renal failure, through endothelial cell damage, would lead to aggravation of the platelet functional defect potentiating the hemorrhagic risk.
Assuntos
Endotélio Vascular/citologia , Hemostasia/fisiologia , Falência Renal Crônica/patologia , Uremia/patologia , Antígenos/sangue , Biomarcadores , Coagulação Sanguínea/fisiologia , Doença Crônica , Progressão da Doença , Fibrinólise/fisiologia , Humanos , Falência Renal Crônica/complicações , Falência Renal Crônica/fisiopatologia , Inibidor 1 de Ativador de Plasminogênio/imunologia , Trombomodulina/metabolismo , Tromboplastina/imunologia , Uremia/complicações , Uremia/fisiopatologia , Fator de von Willebrand/imunologiaRESUMO
Major vascular injury during laparoscopic procedures is a rare but catastrophic complication. We report a pediatric case of aortic laceration during the setup phase of diagnostic laparoscopy in a 5-year-old girl with recurrent abdominal pain. The Veress needle inserted below the umbilicus confirmed the proper placement by use of a saline-filled syringe. The abdomen was insufflated without difficulty. The first trocar was inserted at the same point as Veress needle. The video laparoscope was introduced, and a small amount of blood was seen in the abdomen. We converted the procedure to laparotomy immediately. There was a large retroperitoneal hematoma. The vascular laceration was identified at the origin of the iliac arteries. It was sutured with prolene 5/0. The girl was discharged without further complication on the 10th postoperative day. The incidence of major vascular injuries is 0.03% to 0.07%. The vessels most frequently involved are the aorta, the iliac arteries, the mesenteric vessels, and the vena cava. More than 400 cases have been reported in the literature, but only four of these involve pediatric patients. In the vast majority of cases, the complication took place during the setup phase of laparoscopy (75%), and were related to the introduction of either the Veress needle (30%) or the first umbilical trocar (43%), although the rate is opposite this in some studies.