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This work evaluates several co-composting scenarios based on the use of Arundo donax biomass (AD) as bulking agent for the co-composting of sewage sludge (MS) and agri-food sludge (AS), to manage these organic wastes and to produce balanced organic fertilizers by optimizing the process. For this, six piles were prepared in commercial composting conditions, using AD in a range of 40%-80% (on a dry weight basis). Physico-chemical and chemical parameters and the thermal behaviour were evaluated during the process, as were the physical and chemical parameters of the final composts. The proportion of AD in the mixtures has a significant effect on the development of the thermophilic stage of composting, showing the piles with higher proportion of AD a quicker organic matter degradation. In addition, the evolution of the thermal indices R1 and R2 was different depending on the origin of the sludge used, indicating an increase in the relative concentration of more recalcitrant materials in the piles prepared with AS. The estimation of the global warming potential showed that the use of higher proportion of AD in the composting mixture may be a strategy to mitigate the emission of greenhouse gases during the composting process. Moreover, the end-products obtained had an additional marketable value, with a balanced nutrient content and a good degree of maturity, which indicates the viability of the composting process as a method for the stabilization of these organic wastes.
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PURPOSE: Congenital diaphragmatic hernia (CDH) is one of the causes of respiratory failure in newborns due to lung hypoplasia and pulmonary abnormalities leading to pulmonary hypertension (PH). NAD(P)H oxidase (Nox) is a family of isoenzymes that generate reactive oxygen species (ROS) which can contribute to PH-induced vascular dysfunction. On the other hand, superoxide dismutase (SOD) 1-2 and catalase are the antioxidant enzymes that eliminate the excess of ROS in pulmonary vascular cells. Our aim is to examine whether PH-associated with CDH is due to a dysregulation of ROS production in lungs from CDH fetuses. METHODS: Pregnant rats received either 100 mg nitrofen or vehicle on E9.5. Fetuses were recovered on E21. (1) Nox activity, (2) H2O2 production and (3) mRNA levels of Nox1, Nox2, Nox4, SOD1, SOD2 and catalase were analyzed in fetal lungs. RESULTS: Nox activity and Nox1 and Nox2 mRNA levels were increased in the lungs of fetuses with CDH. However, there were no changes in H2O2 production and Nox4 mRNA levels. SOD1, SOD2 and catalase were decreased. CONCLUSIONS: The raised oxidative stress due to increase in ROS generation by Nox isoenzymes and dysfunction of antioxidant enzymes seems to be a potential mechanism responsible on PH-associated with CDH.
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Hérnias Diafragmáticas Congênitas/fisiopatologia , Hipertensão Pulmonar/fisiopatologia , Pulmão/fisiopatologia , Estresse Oxidativo/fisiologia , Animais , Modelos Animais de Doenças , Éteres Fenílicos , Ratos , Ratos Sprague-DawleyRESUMO
Ulcerative ileitis (UI) after restorative proctocolectomy (RPC) and ileoanal pullthrough procedure (IAPP) is a rare condition described as inflammation of the terminal ileum proximal to the anastomosis. It is mostly observed in ulcerative colitis (UC) and is designated as prepouch ileitis; sometimes with pouchitis, but not necessarily all the time. Its incidence in adults is less than 5%, but the information in children is limited. Pathogenesis is unknown, it has been considered as a disease by itself, independently on the underlying disease. It involves additional morbidity and impacts negatively on quality of life because there is no effective treatment so far. This study aims to review the cases of UI in our institution and to summarize their clinical features and management. A descriptive retrospective study of UI patients between 1990 and 2013 was conducted. Children with RPC and IAPP with clinical and pathological diagnosis of UI were included. Sociodemographic and clinical data, diagnostic, and therapeutic procedures were collected through medical records. UI was diagnosed in eight patients (six males) after RCP; four had UC, two had total colonic aganglionosis (TCA), and two had complex anorectal malformations (one cloacal exstrophy and one omphalopagus twin with bladder exstrophy). Different surgical techniques were used in each case: UC patients underwent IAPP, 50% with J-reservoir and 50% without it; posterior sagittal pullthrough was performed in those with anorectal malformations; one Soave and one Swenson procedure in those with TCA. In summary, three patients had reservoir and five did not. The median age at the IAPP and ostomy closure was 6 years (range 2 months-8 years) and 7 years (range 6 months-9 years), respectively. UI was found after a median of 23 months (range 1-48 months), all of them after digestive tract continuity was reestablished. The leading symptom was lower GI or gastrointestinal, both of them bleeding with abdominal pain, followed by abdominopelvic abscesses and malabsorption with weight loss. Pathology showed nonspecific inflammatory changes. Treatment included antibiotics, corticosteroids and/or immunosuppressive agents with variable response, requiring a new ileostomy in five cases. Ileitis disappeared after diversion. In our experience, UI after colectomy is not an exclusive feature of UC as has been previously described. Although it appears with pouchitis, the presence of a reservoir is not a must, suggesting that this is a different entity. No medical treatment has been really effective in our patients and diversion above this level stopped the process. Further studies on its pathogenesis and treatment strategies are necessary.
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Colite Ulcerativa/etiologia , Ileíte/etiologia , Complicações Pós-Operatórias/etiologia , Proctocolectomia Restauradora , Criança , Pré-Escolar , Colite Ulcerativa/diagnóstico , Colite Ulcerativa/terapia , Feminino , Humanos , Ileíte/diagnóstico , Ileíte/terapia , Lactente , Masculino , Complicações Pós-Operatórias/diagnóstico , Estudos Retrospectivos , Resultado do TratamentoRESUMO
To determine HRQOL after pediatric intestinal transplantation. Thirty-four IT survivors from 1999 to 2012 were asked to complete age-specific HRQOL non-disease-specific questionnaires: TAPQOL (0-4 yr), KINDL-R (5-7 yr; 8-12 yr; 13-17 yr), and SF-36v2 (>18 yr), all validated with Spanish population. Primary caregiver completed a SF-36 questionnaire and CBI. Thirty-one participants were included. Median age was 10.2 yr (1-29) and time after transplant 4.4 yr (0-13). Overall patient scores were 78.2 ± 10.6 (n = 8), 83.3 ± 9.7 (n = 6), 72.2 ± 9.21 (n = 6), 80.5 ± 12.4 (n = 7), and 82.2 ± 12.4 (n = 4) for each age group. Highest scores were obtained for vitality (group I), self-esteem (group IV), and physical and social functioning and emotions (group V). Lowest scores were obtained in appetite and behavior (I), family and school (III), and chronic disease perception (III, IV). No significant differences were found between caregivers and their children. CBI showed stress in 52%. SF-36 for caregivers was lower than general population. No significant differences were found depending on relevant clinical and sociodemographic data. HRQOL was acceptable and improved with age and time since transplantation. Parents had a slighter own QOL and worse perception of health than their children. When successful, intestinal transplantation allows a normal life in most patients and can be offered as an attractive option.
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Intestinos/transplante , Qualidade de Vida , Transplante/psicologia , Adolescente , Adulto , Cuidadores , Criança , Pré-Escolar , Feminino , Nível de Saúde , Humanos , Lactente , Masculino , Análise de Regressão , Espanha , Inquéritos e Questionários , Adulto JovemAssuntos
Doença de Hirschsprung/genética , Mutação , Proteínas Proto-Oncogênicas c-ret/genética , Feminino , Humanos , Masculino , Linhagem , GravidezRESUMO
Gastroesophageal reflux and intestinal distension have been described in survivors of congenital diaphragmatic hernia (CDH). Deficient enteric innervation demonstrated in experimental models is a likely explanation for these symptoms. This study aimed at further characterizing these anomalies and examining esophageal and intestinal motility in this condition. Pregnant rats received either nitrofen or vehicle on E9.5. Sections of E15, E18, and E21 esophagus and small bowel were stained for protein gene product 9.5, nicotinamide adenine dinucleotide phosphate (NADPH) diaphorase (NADPHd), and acetylcholinesterase (AChE). The proportion of neural tissue/muscle surface was measured and the NADPHd- and AChE-positive motor endplates (MEPs) were counted. E18 and E21 stomachs were stained for AChE, the ganglia were counted and measured. The peristalsis of the esophagus and small bowel was video recorded. The relative neural/muscle surface and the number of NADPHd- and AChE-positive MEPs were decreased on E15 and E18 in the esophagus and small bowel of embryos with CDH, but they tended to improve on E21. The number and the mean surface of stomach ganglia were smaller in E18 and E21 fetuses with CDH. Peristaltic movements were decreased in the esophagus and small bowel of animals with CDH. Deficient enteric innervation impaired gastrointestinal motility in experimental CDH. This could explain some long-term morbidity in the human condition.
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Sistema Nervoso Entérico/fisiopatologia , Esôfago/inervação , Hérnias Diafragmáticas Congênitas/fisiopatologia , Intestino Delgado/inervação , Peristaltismo/fisiologia , Estômago/inervação , Acetilcolinesterase/análise , Animais , Esôfago/fisiopatologia , Feminino , Intestino Delgado/fisiopatologia , Placa Motora/fisiologia , NADP/análise , NADPH Desidrogenase/análise , Éteres Fenílicos , Distribuição Aleatória , Ratos Sprague-Dawley , Estômago/fisiopatologia , Ubiquitina Tiolesterase/análiseRESUMO
BACKGROUND/AIM: Neither cervicotomy nor postero-lateral thoracotomy allow safe surgical access to the lower cervical spine and high posterior mediastinum with full control of the vascular and neural structures involved. We report our favorable experience with cervico-sternotomy for accessing this region. PATIENTS AND METHODS: Six patients were operated upon between 1998 and 2011 for either removal of huge cervico-thoracic neural ganglioneuromas (n = 2) or anterior arthrodesis for congenital (n = 2), neuropathic (n = 1) or osteolytic scoliosis (n = 1). In all cases, cervicotomy was followed by sternotomy, thymectomy, division of the innominate vein and dissection of jugular veins, carotid arteries and vagus nerves. RESULTS: The tumors measured 10.9 x 3.9 x 8.7 cm and 8 x 6 x 5 cm, and involved the paravertebral chain from the aortic arch to the base of the skull and from the left lung hilus to the thyroid region respectively. In the scoliosis patients, anterior vertebral fixation between C5 and T5 was readily feasible. Blood transfusion was avoided. Horner's syndrome and transient lymphedema were the only complications. Median operative time was 210 minutes (range 180-240 minutes) and median estimated blood loss was 2.7 cc/kg (0-13.8 cc/kg). Median hospital stay was 7 days (range 5-18 days). CONCLUSIONS: Cervico-sternotomy is an optimal approach for this anatomical region in children. It offers better exposure of the anterior cervico-thoracic spine and the thoracic inlet than cervicotomy or thoracotomy. Control of the nervous and vascular structures was safely achieved in all cases and postoperative discomfort was surprisingly limited.
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Vértebras Cervicais/cirurgia , Neoplasias de Cabeça e Pescoço/cirurgia , Escoliose/cirurgia , Fusão Vertebral , Esternotomia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , MasculinoRESUMO
PURPOSE: Embryonal sarcoma accounts for 6 % of liver tumors. This study reviews its features and the results of treatment in a referral center. METHODS: We retrospectively reviewed liver tumors treated between 1995 and 2011. PRETEXT staging and biopsy were used to tailor chemotherapy according to SIOP protocols. Radical surgery was performed when possible. Complications and cumulative survival were the outcome endpoints. RESULTS: Six out of 156 primary liver tumors (four males and two females) were sarcomas. The mean age at diagnosis was 81 ± 8.5 months. The most frequent finding was abdominal mass. Alfa-fetoprotein was normal. Imaging depicted heterogeneous tumors with septa, necrosis, and hemorrhagic areas. The diagnosis was ascertained by biopsy. Three tumors were located in the right lobe (PRETEXT II): two were bilobar (PRETEXT III) and one was in the left lobe (PRETEXT I). Two children had metastases at diagnosis and high-risk chemotherapy (vincristine, carboplatin, epirubicin) was administered with poor response. They died without operation 4 and 10 months later. Four patients with local disease underwent typical liver resections after chemotherapy (iphosphamide, vincristine, actinomycin D, and doxorrubicin). Overall actuarial survival at 70 months was 66.6 %. CONCLUSIONS: Extended and metastatic embryonal sarcoma do poorly whereas localized tumors amenable to complete surgical removal after chemotherapy can cure.
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Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/cirurgia , Sarcoma/tratamento farmacológico , Sarcoma/cirurgia , Adolescente , Antineoplásicos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Carboplatina/uso terapêutico , Criança , Pré-Escolar , Terapia Combinada , Dactinomicina/uso terapêutico , Doxorrubicina/uso terapêutico , Epirubicina/uso terapêutico , Feminino , Humanos , Ifosfamida/uso terapêutico , Lactente , Fígado/cirurgia , Masculino , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento , Vincristina/uso terapêuticoRESUMO
BACKGROUND AND AIMS: Gastroesophageal reflux (GER) is extremely frequent in patients with esophageal atresia and tracheoesophageal fistula (EA/TEF). It is often refractory to antireflux medication and requires antireflux surgery. The reasons for this close association, the dangers of persistent GER, and the indications, technical aspects, and pitfalls of antireflux operations in these patients are examined in the present study. METHODS: The literature and summary of a large institutional experience of the authors were reviewed. RESULTS: The esophagus is permanently defective in EA/TEF patients even when successful repair, sometimes under tension, has been achieved. Extrinsic and intrinsic innervations are abnormal and consequently, motor function and sphincters are defective. The result is that close to one half of these patients overall suffer chronic GER that leads often to Barrett esophagus. Fundoplication becomes necessary in more than 40% of them, particularly in cases of with refractory anastomotic stenoses and in those with pure and long-gap EA/TEF. In the long run, the risks of esophageal carcinoma are 50-fold higher in EA/TEF survivors than in the population at random. On the contrary, fundoplication is anatomically difficult to perform and it fails in one-fifth of these children due to the persistence of the conditions that facilitate GER in them. CONCLUSIONS: GER is extremely frequent in patients treated for EA/TEF because of serious structural and functional deficiencies. It is refractory to medical treatment and often requires antireflux surgery. However, the high rates of wrap failure invite close follow-up in all cases and reoperation or other measures whenever necessary.
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Atresia Esofágica/cirurgia , Fundoplicatura/efeitos adversos , Refluxo Gastroesofágico/etiologia , Humanos , Complicações Pós-OperatóriasRESUMO
Gastroesophageal reflux (GER) is almost constant in esophageal atresia and tracheoesophageal fistula (EA/TEF). These patients resist medical treatment and require antireflux surgery quite often. The present review examines why this happens, the long-term consequences of GER and the main indications and results of fundoplication in this particular group of patients. The esophagus of EA/TEF patients is malformed and has abnormal extrinsic and intrinsic innervation and, consequently, deficient sphincter function and dysmotility. These anomalies are permanent. Fifty percent of patients overall have GER, and one-fifth have Barrett's metaplasia. Close to 100%, GER of pure and long-gap cases require fundoplication. In the long run, these patients have 50-fold higher risk of carcinoma than the control population. GER in EA/TEF does not respond well to dietary, antacid, or prokinetic medication. Surgery is necessary in protracted anastomotic stenoses, in pure and long-gap cases, and when there is an associated duodenal atresia. It should be indicated as well in other symptomatic cases when conservative treatment fails. However, confection of a suitable wrap is anatomically difficult in this condition as shown by a failure rate of 30% that is also explained by the persistence for life of the conditions facilitating GER.
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Atresia Esofágica/complicações , Fundoplicatura , Refluxo Gastroesofágico/cirurgia , Fístula Traqueoesofágica/complicações , Refluxo Gastroesofágico/etiologia , Humanos , Resultado do TratamentoRESUMO
INTRODUCTION: Abdominal enteric duplications are found in 1 out of 4,500 autopsies, and only 4 to 5% of them are located in the duodenum, where they may be connected with the biliary or pancreatic ducts. The aim of this study was to describe the clinical features, management, and outcome of a large series of duodenal duplication cysts. MATERIALS AND METHODS: The charts of all patients treated at our institution between 1985 and 2011 were reviewed retrospectively with particular attention to imaging, surgical technique, pathology, and outcome. RESULTS: During that period, 11 cases (81.8% females) were treated. Out of the 11 patients, 8 were symptomatic (vomiting in 3, recurrent acute pancreatitis in 2, and abdominal pain in 3) and 3 were tentatively diagnosed prenatally as choledochal cysts. Median age at surgery was 2.3 years (0 to 13.7) and preoperative diagnosis was correct in five cases. Five cysts were developed intraluminally and three communicated with the biliary duct (one), pancreatic duct (one), or both (one). Surgical treatment consisted of complete resection (four cases, including one pancreaticoduodenectomy), partial removal including all mucosa (four cases), and internal marsupialization (three cases). In all cases, the ductal communications were divided and opened into the duodenal lumen. In six cases, ectopic gastric mucosa was found. All patients recovered uneventfully. CONCLUSION: Duodenal duplication cysts are rare and may have bizarre anatomical patterns due to biliopancreatic involvement. Optimal treatment is complete surgical removal, and, if this is not possible, partial removal including the mucosa or marsupialization are also good alternatives. In cases with biliary and pancreatic tract connections, these have to be taken down carefully and drained into the duodenum.
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Cistos/diagnóstico , Cistos/cirurgia , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Duodeno/anormalidades , Duodeno/cirurgia , Antro Pilórico/anormalidades , Antro Pilórico/cirurgia , Adolescente , Criança , Pré-Escolar , Cisto do Colédoco/diagnóstico , Cisto do Colédoco/cirurgia , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Pancreaticoduodenectomia/métodos , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Lung hypoplasia can be prevented in vitro by retinoic acid (RA). Recent evidence suggests that amniotic fluid stem (AFS) cells may integrate injured lungs and influence their recovery. We tested the hypothesis that AFS cells might improve lung growth and motility by paracrine mechanisms. Pregnant rats received either nitrofen or vehicle on E9.5. In vitro E13 embryonic lungs were cultured in the presence of culture medium alone or with RA, basophils, or AFS cells. In vivo green fluorescent protein-expressing (GFP(+)) rat AFS cells were transplanted in nitrofen-exposed rats on E10.5. E13 lung explants were cultured before analysis. The surface, the number of terminal buds, and the frequency of bronchial contractions were assessed. Protein gene product 9.5 (PGP 9.5) and α-actin protein levels were measured. The lung explants transplanted with AFS cells were stained for α-actin, PGP 9.5, and TTF-1. The levels of FGF-10, VEGFα, and TGF-ß1 secreted by the AFS cells in the culture medium were measured. Comparison between groups was made by ANOVA. In vitro, the surface, the number of terminal buds, and the bronchial peristalsis were increased in nitrofen+AFS cell explants in comparison with nitrofen-exposed lungs. While nitrofen+RA lungs were similar to nitrofen+AFS ones, basophils did not normalize these measurements. PGP 9.5 protein was decreased in nitrofen lungs, but after adding AFS cells, the value was similar to controls. No differences were found in the expression of α-actin. In vivo, the surface, number of terminal buds, and peristalsis were similar to control after injection of AFS cells in nitrofen-exposed rats. Colocalization with TTF-1-positive cells was found. The levels of FGF-10 and VEGFα were increased in nitrofen+AFS cell explants, while the levels of TGF-ß1 were similar to controls. Lung growth, bronchial motility, and innervation were decreased in nitrofen explants and rescued by AFS cells both in vitro and in vivo, similarly to that observed before with RA. The AFS cell beneficial effect was probably related to paracrine action of growth factor secretion.
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Líquido Amniótico/citologia , Pulmão/anormalidades , Pulmão/cirurgia , Transplante de Células-Tronco/métodos , Animais , Movimento Celular , Feminino , Hiperplasia/prevenção & controle , Pulmão/patologia , Microscopia Confocal , Praguicidas/toxicidade , Éteres Fenílicos/toxicidade , Gravidez , Distribuição Aleatória , Ratos , Ratos Sprague-Dawley , Tretinoína/farmacologiaRESUMO
PURPOSE: Our objective is to describe a new experimental model of amniotic band in rats and to show the initial morphological results. METHODS: The model was created by ligation of a hind limb with silk on day 17 of gestation; in selected fetuses ligation was withdrawn on day 19 of gestation. Fetuses were removed by cesarean section on day 21 of gestation, analyzing their weights and morphological characteristics of their hind legs. We used 10 pregnant SD rats, weighing 263 g (231-279). One hundred and thirteen fetuses were analyzed divided into 6 groups: fetuses from control rats (n=28), uterine opening and closing (n=7), band creation (n=28), band creation and subsequent removal (n=14) and not intervened fetuses of the litters in which the band was created (n=19) and that the band was created and removed (n=17). Comparisons between groups were made using parametric tests considering p<0.05 as a threshold for significance. RESULTS: Operated fetuses (band creation and band creation and removal) showed statistically significant differences in weight (mean=4.56 g and 4.4 g) and length of their hind limbs (mean=3.97 mm and 5.34 mm) compared to the rest of the groups. The rate of abortions in operated fetuses was 16.6% and the rate of amputations was 8.3%. DISCUSSION: A viable and reproducible experimental model of amniotic band is described, with potential applications in both the study of the disease and the role of fetal surgery thereon.
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Síndrome de Bandas Amnióticas/cirurgia , Modelos Animais de Doenças , Terapias Fetais/métodos , Aborto Espontâneo/epidemiologia , Amputação Traumática/epidemiologia , Animais , Feminino , Membro Posterior/embriologia , Gravidez , Ratos , Ratos Sprague-Dawley , Reprodutibilidade dos TestesRESUMO
PURPOSE: Our objective is to analyze the variables that influence the outcome of Small Bowel Transplantation (SBT) in rats in an experimental microsurgery program. The surgical technique and perioperative care are described in detail. METHODS: Retrospective study of the SBT in rats conducted in our experimental surgery laboratory from 2002 to 2010. The animals were divided into group A (those who survived more than 48 hours) and group B (those who died earlier without justificable cause). We compared in both groups: number of transplants performed by the surgeon, warm ischemia time, cold ischemia time and duration of the procedure. RESULTS: Five surgeons with different degrees of microsurgical training participated in the study. A total of 521 SBT were performed with an overall survival of 48%. The first successful transplant was performed after a median of 46 (25-68) transplants. Total procedure time (3.5 vs 2.9 hours) and warm ischemia time (51 vs 35 minutes) were higher in group B (p < 0.05). DISCUSSION: The number of transplants required for learning the technique is high. However, survival is acceptable when the time needed for vascular anastomosis is reduced. The SBT in rats is a valuable model for surgical training and research of the phenomena related to SBT.
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Intestinos/transplante , Animais , Modelos Animais , Transplante de Órgãos/métodos , Ratos , Ratos Endogâmicos BN , Ratos WistarRESUMO
BACKGROUND/AIM: Patients with esophageal atresia and tracheo-esophageal fistula (EA-TEF) have chronic respiratory tract disease and deficient tracheal innervation. This study tests the hypothesis in rats with EA-TEF that deficient lung innervation could be one of the causes of respiratory disease. MATERIAL AND METHODS: Pregnant rats were treated with either 2 mg/kg i.p. adriamycin or vehicle on E7, E8 and E9. Lungs and tracheas were retrieved on E15, E18 and E21 (term: E22). Innervation was examined by regular (PGP 9.5 and GDNF) and whole-mount (PGP 9.5 and α-actin) immunohistochemistry. PGP 9.5 and GDNF mRNA were measured by real-time, quantitative RT-PCR and the levels of PGP 9.5 protein by immunoblot. Embryonic lung primordia harvested on E13 were cultured for 72 h and airway peristalsis was assessed under an inverted microscope. PGP 9.5 expression was then examined in explants by whole-mount immunohistochemistry and RT-PCR. Values were compared with non-parametric tests. RESULTS: Neural networks were present in both EA-TEF and control fetuses on E15, E18 and E21, but the neural network was obviously disorganized in whole-mount immunohistochemistry of EA-TEF. The pan-neural marker PGP 9.5 protein was increased at term whereas the neural chemo-attractant GDNF protein was unchanged. PGP 9.5 mRNA significantly increased from subnormal levels on E15 to very increased ones on E18 compared with controls. GDNF mRNA levels followed the same trend. Airway peristalsis of explanted embryonal lungs was similar in both groups. The neural networks were underdeveloped in these primordia, as assessed by whole-mount PGP 9.5 immunohistochemistry and RT-PCR. CONCLUSIONS: The development of respiratory tract innervation in adriamycin-induced EA-TEF was delayed and abnormally controlled in rats compared with controls. However, these deficiencies were apparently compensated for at term and had no effect on airway peristalsis. The possible significance of innervation anomalies for respiratory sequelae in EA-TEF patients deserves further investigation.
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Atresia Esofágica/embriologia , Atresia Esofágica/patologia , Pulmão/embriologia , Pulmão/inervação , Rede Nervosa/embriologia , Doenças Respiratórias/etiologia , Animais , Doxorrubicina , Atresia Esofágica/induzido quimicamente , Atresia Esofágica/complicações , Atresia Esofágica/fisiopatologia , Feminino , Imuno-Histoquímica , Pulmão/anormalidades , Técnicas de Cultura de Órgãos , Gravidez , Ratos , Ratos Sprague-Dawley , Traqueia/inervaçãoRESUMO
AIM OF THE STUDY: Cystic fibrosis (CF) is a multisystemic disease, with some patients developing end-stage liver disease (ESLD), requiring liver transplantation (LT). These children usually present with severe mutations of the CFTR gene. Almost 100% of patients with severe mutations develop exocrine pancreatic insufficiency, leading later to endocrine insufficiency. Immunosuppression accelerates the development of insulin-dependent diabetes (IDD) in transplanted children with CF. Our aims were: (1) to analyze our experience with CF-related ESLD children who received LT, and the relationship to the development of IDD; (2) to report our preliminary results with en bloc liver-pancreas transplantation (CLPT). METHODS: 9 children (6M/3F) with CF and ESLD underwent LT between 1993 and 2010; median age and weight were 12.3 years (range: 5.4-17.0) and 36.7 kg (range: 14.2-58.5), respectively. 4 patients received a whole graft, 4 had reduced grafts (1 split) and 1 underwent CLPT. Immunosuppression followed the protocols at the time of transplantation. RESULTS: Liver function was restored in all patients and none of them needed re-transplantation. Median follow-up was 105 months (range: 4-206). 1 child died of respiratory failure at 23 months after transplantation while awaiting pulmonary transplantation. Survival (Kaplan-Meier) at 105 months was 87.5%. 4 children already had IDD before transplantation and 3 developed diabetes immediately after transplantation. 2 had not developed IDD at the end of the study: the youngest at the time of LT (5.4 years, follow-up 7.1 years) and the girl who had had CLPT and who recovered normal exocrine and endocrine pancreatic function after transplantation. CONCLUSIONS: LT is a realistic option to treat CF-related ESLD children. IDD is common in these patients. En bloc liver-pancreas transplantation is an appealing option, since it simultaneously restores exocrine function and prevents IDD. This procedure has clear technical advantages over simultaneous isolated liver and pancreas transplantation.
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Fibrose Cística/cirurgia , Transplante de Fígado/métodos , Transplante de Pâncreas/métodos , Adolescente , Criança , Pré-Escolar , Fibrose Cística/fisiopatologia , Feminino , Humanos , Testes de Função Hepática , Transplante de Fígado/mortalidade , Masculino , Transplante de Pâncreas/mortalidade , Análise de Sobrevida , Resultado do TratamentoRESUMO
INTRODUCTION: Children with PHACES syndrome (PS) and visual impairment or stridor show a dramatic and immediate response to propranolol. However, this beta-blocking drug could be responsible for an eventually increased risk of ischemic stroke due to the underlying cerebral vascular disease. To more accurately understand the effects of propranolol on brain vascularization, we examined PS patients treated with this drug for airway or visual complications using brain perfusion SPECT (Single Photon Emission Computed Tomography). In the past, this examination has been shown to be useful in the management of patients with different neurovascular disorders. METHODS: Clinical records and imaging studies were reviewed in 7 patients with a diagnosis of PS. All patients underwent magnetic resonance angiography (MRA), echocardiography, chest X-ray and ophthalmologic, neurological, and cardiologic assessments. They received 2-3 mg/kg/day propranolol in an attempt to treat stridor or avoid ophthalmologic occlusion. We performed SPECT after 3-6 months of treatment. RESULTS: SPECT showed a normal uptake in the frontal and temporal regions despite vascular abnormalities found with MRA imaging. Significant improvements of symptoms and in the volume of the hemangioma were noted in all cases without signs of a reduction of brain blood perfusion. CONCLUSIONS: Propranolol treatment was safe in our patients who did not show signs of perfusion changes. The high sensitivity for detecting functional impairment makes brain perfusion SPECT useful in the diagnosis and follow-up of patients with PS considered at risk of neurovascular impairment. Accurate knowledge of its pathophysiological basis, together with the appropriate technique and careful interpretation of reporting, will enhance the clinical use of brain SPECT in those patients.
Assuntos
Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/tratamento farmacológico , Encéfalo/diagnóstico por imagem , Anormalidades do Olho/diagnóstico por imagem , Anormalidades do Olho/tratamento farmacológico , Malformações Arteriovenosas Intracranianas/diagnóstico por imagem , Síndromes Neurocutâneas/diagnóstico por imagem , Síndromes Neurocutâneas/tratamento farmacológico , Propranolol/uso terapêutico , Acidente Vascular Cerebral/diagnóstico por imagem , Antagonistas Adrenérgicos beta/uso terapêutico , Encéfalo/anormalidades , Feminino , Humanos , Lactente , Masculino , Acidente Vascular Cerebral/prevenção & controle , Tomografia Computadorizada de Emissão de Fóton Único/métodosRESUMO
BACKGROUND AND AIMS: Esophageal atresia (EA) with or without tracheo-esophageal fistula (TEF) is a rare condition that can be nowadays succesfully treated. The current interest therefore is focused on the management of the difficult cases, on thoracoscopic approach, and on some aspects of the long-term results. METHODS: The current strategies for the difficult or impossible anastomoses in pure and long-gap EA, the introduction of thoracoscopic repair and the causes, mechanisms and management of post-operative gastro-esophageal reflux (GER) are reviewed. RESULTS: Methods of esophageal elongation and multi-staged repair of pure and long-gap EA allow anastomosis but with functional results that are often poor. Esophageal replacement with colon or stomach achieves at least similar results and often requires less procedures. Thoracoscopic repair is a promising adjunct, but the difficulties for setting it as a gold-standard are pointed out. GER is a part of the disease and its surgical treatment, that is often required, is burdened by high failure rates. CONCLUSIONS: EA with or without TEF can be successfully treated in most cases, but a number of unsolved issues remain and the current approach to difficult cases will certainly evolve in the future.