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1.
Pediatr Surg Int ; 30(6): 677-9, 2014 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24668180

RESUMEN

Allgrove syndrome is a rare genetic disorder typically manifested by alacrima, achalasia, and adrenal insufficiency, and is one of the rare causes of achalasia in infants. While the gold standard for achalasia treatment in adults is an esophageal myotomy with fundoplication, a standard treatment for infantile achalasia remains undetermined due to the low number of reported cases and rarity of the disease in this age group. We report a 7.7 kg infant with achalasia secondary to Allgrove syndrome who was successfully treated by Heller myotomy and simultaneous Toupet fundoplication. This case represents one of the smallest patients to ever be treated for achalasia and highlights the role that primary surgical therapy may have for the infantile variant of the disease.


Asunto(s)
Insuficiencia Suprarrenal/cirugía , Acalasia del Esófago/cirugía , Insuficiencia Suprarrenal/diagnóstico por imagen , Acalasia del Esófago/diagnóstico por imagen , Femenino , Fundoplicación , Humanos , Lactante , Recién Nacido , Laparoscopía , Radiografía
2.
Pediatr Surg Int ; 29(5): 437-43, 2013 May.
Artículo en Inglés | MEDLINE | ID: mdl-23411614

RESUMEN

PURPOSE: Our institution routinely utilizes needle core biopsy (NCB), instead of fine needle aspiration, in the evaluation of pediatric thyroid nodules. This practice initially arose from limited cytopathology services in our hospital. Given the lack of information regarding the utility of NCB in diagnosing pediatric thyroid neoplasms, we set out to review our institution's experience with this technique. METHODS: We performed a single institution retrospective chart review of all children who underwent thyroidectomy for primary thyroid pathology. RESULTS: Seventy-four patients, with a mean age of 12.9 ± 4.5 (SD) years, underwent partial or total thyroidectomy between 2002 and 2010. Seven of these patients had medically refractive hyperthyroidism. The remaining 67 patients had one or more thyroid nodules as identified by ultrasound. 24 (36 %) of these cases were malignant on final pathology. 14 (58 %) of the malignant cases were papillary thyroid carcinoma. 46 of the thyroid nodule cases underwent pre-operative NCB. Biopsy results for these patients were non-diagnostic in 6 (13 %), benign in 11 (24 %), atypical in 17 (37 %), and malignant in 12 (26 %). There were no complications arising from NCB. Sensitivity of NCB for diagnosing papillary carcinoma (PC) and follicular neoplasm was calculated at 0.88 (0.47-1.0, 95 % CI) and 0.84 (0.60-0.97, 95 % CI), respectively. Of the 28 patients not undergoing preoperative NCB, 12 underwent hemithyroidectomy, with one patient (8 %) requiring completion thyroidectomy for PC. Overall, the sensitivity of NCB in diagnosing PC and follicular thyroid neoplasms was 0.85 (0.55-0.99, 95 % CI), while the specificity was 0.63 (0.42-0.82, 95 % CI). CONCLUSIONS: Needle core biopsy appears to have a low rate of associated complications, and its sensitivity for diagnosing PC and follicular neoplasm is comparable to what has been reported for fine needle aspiration biopsy in a similar patient population.


Asunto(s)
Glándula Tiroides/patología , Neoplasias de la Tiroides/patología , Adolescente , Biopsia con Aguja Fina , Biopsia con Aguja Gruesa , Niño , Femenino , Humanos , Masculino , Estudios Retrospectivos , Sensibilidad y Especificidad , Neoplasias de la Tiroides/cirugía , Tiroidectomía
3.
Clin Obes ; 3(1-2): 7-11, 2013 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-25586387

RESUMEN

Although childhood obesity is common, many paediatric practitioners are not familiar with screening for its associated, serious comorbidities. We aimed to determine the adequacy of screening for nine well-recognized comorbidities in outpatients with severe morbid obesity (body mass index [BMI] ≥50 kg m(-2) ) seen in a large tertiary paediatric hospital. Patients with a BMI of ≥50 kg m(-2) seen at Texas Children's Hospital during calendar year 2009 were identified. Their medical records were reviewed for any documentation where hypertension, cardiac dysfunction, sleep apnoea, hepatosteatosis, diabetes, pseudotumour cerebri, dyslipidemia, orthopaedic issues and depression were noted and/or addressed as evidence of clinician awareness of these problems. We identified 123 patients seen at least once in 2009, with an average of 3.4 physician visits per patient and by an average of 2 different specialists. Hypertension screening was the most documented (91% of patients) and depression screening was the least documented (41%) in this patient cohort. Twelve patients (10%) had documented screening for all nine comorbidities. Overall, 55 patients (45%) had five or fewer of the nine comorbidities noted and/or addressed in the medical record. Adequate screening for comorbidities occurs in approximately half of children with severe morbid obesity, which means that many of these comorbidities are not being identified or treated. Educational programmes and new methodologies are needed to ensure comprehensive care of children with morbid obesity.

5.
J Perinatol ; 28(10): 665-74, 2008 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-18784730

RESUMEN

OBJECTIVE: Necrotizing enterocolitis (NEC) remains a major cause of neonatal morbidity and mortality. Some infants recover uneventfully with medical therapy whereas others develop severe disease (that is, NEC requiring surgery or resulting in death). Repeated attempts to identify clinical parameters that would reliably identify infants with NEC most likely to progress to severe disease have been unsuccessful. We hypothesized that comprehensive prospective data collection at multiple centers would allow us to develop a model which would identify those babies at risk for progressive NEC. STUDY DESIGN: This prospective, observational study was conducted at six university children's hospitals. Study subjects were neonates with suspected or confirmed NEC. Comprehensive maternal and newborn histories were collected at the time of enrollment, and newborn clinical data were collected prospectively, thereafter. Multivariate logistic regression analysis was used to develop a predictive model of risk factors for progression. RESULT: Of 455 neonates analyzed, 192 (42%) progressed to severe disease, and 263 (58%) advanced to full feedings without operation. The vast majority of the variables studied proved not to be associated with progression to severe disease. A total of 12 independent predictors for progression were identified, including only 3 not previously described: having a teenaged mother (odds ratio, OR, 3.14; 95% confidence interval, CI, 1.45 to 6.96), receiving cardiac compressions and/or resuscitative drugs at birth (OR, 2.51; 95% CI, 1.17 to 5.48), and having never received enteral feeding before diagnosis (OR, 2.41; 95% CI, 1.08 to 5.52). CONCLUSION: Our hypothesis proved false. Rigorous prospective data collection of a sufficient number of patients did not allow us to create a model sufficiently predictive of progressive NEC to be clinically useful. It appears increasingly likely that further analysis of clinical parameters alone will not lead to a significant improvement in our understanding of NEC. We believe that future studies must focus on advanced biologic parameters in conjunction with clinical findings.


Asunto(s)
Enterocolitis Necrotizante/etiología , Enfermedades del Prematuro/etiología , Nutrición Enteral , Enterocolitis Necrotizante/diagnóstico , Enterocolitis Necrotizante/terapia , Femenino , Humanos , Recién Nacido de Bajo Peso , Recién Nacido , Recien Nacido Prematuro , Enfermedades del Prematuro/diagnóstico , Enfermedades del Prematuro/terapia , Modelos Logísticos , Masculino , Valor Predictivo de las Pruebas , Estudios Prospectivos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Resultado del Tratamiento
6.
Am J Kidney Dis ; 38(3): 553-9, 2001 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-11532688

RESUMEN

Semipermanent venous catheters remain the most commonly used access for chronic hemodialysis (HD) in pediatric patients. The recent availability of Tesio catheters in 7 and 10 F has expanded available HD catheter options for children and adolescents. We report our experience with Tesio catheter survival, complications, and effect on dialysis adequacy in comparison to standard dual-lumen (DL) catheters in our pediatric HD patients. Demographic data were similar between the two groups. Overall actuarial survival was significantly longer for Tesio versus DL catheters (46% versus 0% at 1 year; P = 0.003). A comparison of smaller catheters (7 F Tesio catheter, 8 or 10 F DL catheter) showed that smaller Tesio catheters had a significantly longer survival (median survival, 244 versus 13 catheter-days; P < 0.01). Tesio 10 F catheters also survived significantly longer than the larger 11.5 and 12 F DL catheters (P < 0.02). Catheter sepsis occurred less frequently with Tesio catheters (one episode/20 catheter-months) than DL catheters (one episode/5 catheter-months) despite the longer duration of Tesio catheters. Adequate dialysis (single-pool Kt/V > 1.2) was delivered with the same frequency, but for a longer duration with Tesio catheters (76% +/- 32% over 100 monthly measurements versus DL catheter, 57% +/- 45% over 54 monthly measurements). Our clinical practice was to replace cuffed catheters when adequate dialysis could not be delivered. We conclude that Tesio catheters provide superior performance compared with DL catheters in terms of catheter survival, infection rates, and duration of adequate performance.


Asunto(s)
Catéteres de Permanencia , Fallo Renal Crónico/terapia , Diálisis Renal/instrumentación , Adolescente , Adulto , Cateterismo Periférico/efectos adversos , Cateterismo Periférico/métodos , Catéteres de Permanencia/efectos adversos , Niño , Falla de Equipo , Femenino , Humanos , Infecciones/etiología , Masculino , Factores de Tiempo
7.
J Pediatr Surg ; 36(5): 693-9, 2001 May.
Artículo en Inglés | MEDLINE | ID: mdl-11329568

RESUMEN

BACKGROUND/PURPOSE: Ovarian pathology, although rare in children, must be included in the differential diagnosis of all girls who present with abdominal pain, an abdominal mass, or precocious puberty. METHODS: To improve clinical appreciation of these lesions, the authors reviewed the presentation, evaluation, and outcome of all patients with ovarian pathology surgically treated at their institution since 1985. RESULTS: One hundred two girls (aged 9.8 +/- 5.5 years; range, 2 days to 20 years) underwent 106 separate ovarian operations (43 salpingo-oophorectomies, 21 oophorectomies, 33 ovarian cystectomies, and 9 ovarian biopsies). Of those presenting with acute abdominal pain (n = 59), 25 (42%) had ovarian torsion (14 associated with a mature teratoma), and only 1 (2%) had a malignant tumor. In contrast, of those presenting with an abdominal mass (n = 23), 6 (26%) had malignancies. There was no age difference between those with benign disease (9.9 +/- 5.6 years; n = 96) and those with malignant tumors (8.6 +/- 3.9 years, n = 10). Nine children had 10 operations for presumed malignant tumors (3 dysgerminomas, 2 immature teratomas with foci of yolk sac tumor, 2 juvenile granulosa cell tumors, 1 yolk sac tumor, and 1 Sertoli-Leydig cell tumor). These patients all had unilateral salpingo-oophorectomy, 4 had chemotherapy, and all are now disease free at 8.4 +/- 4.1 years follow-up. CONCLUSION: Ovarian pathology remains a rare indication for surgery in girls less than 20 years of age. Because most of these lesions are benign, ovarian-preserving operations should be performed whenever feasible.


Asunto(s)
Neoplasias Ováricas/cirugía , Ovariectomía/métodos , Ovariectomía/estadística & datos numéricos , Dolor Abdominal/etiología , Adolescente , Adulto , Distribución por Edad , Factores de Edad , Edad de Inicio , Biopsia , Niño , Preescolar , Diagnóstico Diferencial , Supervivencia sin Enfermedad , Trompas Uterinas/cirugía , Femenino , Estudios de Seguimiento , Hospitales Pediátricos , Humanos , Lactante , Recién Nacido , Epiplón/cirugía , Neoplasias Ováricas/complicaciones , Neoplasias Ováricas/diagnóstico , Resultado del Tratamiento
8.
J Pediatr Surg ; 36(1): 229-31, 2001 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-11150471

RESUMEN

BACKGROUND/PURPOSE: Lipoblastoma/lipoblastomatosis is a poorly understood and uncommon soft tissue tumor of infancy and early childhood. Twenty-four patients that presented to a single institution over a 15-year period were reviewed for clinical features and outcome. METHODS: A retrospective review was conducted. RESULTS: Twenty-five cases were identified; 1 chart was not available for review. Fourteen girls and 11 boys with a median age of 20 months (2 months, 10 years) presented with 26 separate tumors. Nineteen were focal and 7 diffuse. The tumors were located on the trunk (n = 13), leg (n = 6), arm (n = 3), and head and neck (n = 4). Five patients (all with diffuse type) had up to 3 recurrences. CONCLUSIONS: Patients with focal lipoblastoma are unlikely to require further surgery after initial resection. Patients with diffuse lipoblastoma (lipoblastomatosis) are likely to have recurrent disease (usually within 2 years) and should undergo close follow-up. Genetic analysis of the specimen will help exclude liposarcoma. Recurrent lesions are best imaged with magnetic resonance imaging to assess extent and plan reconstruction if necessary.


Asunto(s)
Lipoma/cirugía , Lipomatosis/cirugía , Neoplasias de los Tejidos Blandos/cirugía , Niño , Preescolar , Femenino , Humanos , Lactante , Lipoma/patología , Lipomatosis/patología , Masculino , Estudios Retrospectivos , Neoplasias de los Tejidos Blandos/patología , Resultado del Tratamiento
9.
Am J Surg ; 182(6): 729-32, 2001 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-11839348

RESUMEN

BACKGROUND: Omental infarction is a rare cause of abdominal pain, with fewer than 300 cases reported in the literature. Only 15% of reported cases occur in the pediatric population. We present our experience with 18 children diagnosed with omental infarction admitted to Texas Children's Hospital over a 15-year period. STUDY DESIGN: A retrospective review of clinical records, diagnostic images, and pathologic findings was carried out for all children diagnosed with isolated omental infarction from 1986 to 2000 in order to analyze presenting signs and symptoms, preoperative imaging, surgical management, and postoperative outcomes. RESULTS: Eighteen children were treated for isolated omental infarction. There were 12 boys and 6 girls with an average age of 7.5 years (range 2 to 13). All patients presented with acute onset of right lower quadrant pain. Only 5 of 18 (24%) had associated gastrointestinal symptoms. The average temperature at presentation was 99.4 degrees F (+/- 0.78). The average white blood cell count was 11.4 (+/- 4.4). Fourteen patients had ultrasonographys performed preoperatively: 6 of 14 incorrectly diagnosed appendicitis, 4 of 14 were nondiagnostic and 4 of 14 correctly diagnosed omental infarction. Two of 18 patients underwent computed tomography scans, which were diagnostic for omental infarction. Resection of the infarcted omentum was performed in all patients and appendectomy in 16 of 18. Thirteen patients underwent an open procedure, 5 were performed laparoscopically. There were no postoperative complications. All patients had resolution of pain postoperatively and were discharged an average of 3.0 (+/- 0.9) days after admission. CONCLUSIONS: Omental infarction is an uncommon cause of right lower quadrant pain in children and is often diagnosed as appendicitis preoperatively. Ultrasonography and computed tomography can be diagnostic. Surgical resection of the infarcted omentum results in immediate resolution of pain with no morbidity.


Asunto(s)
Dolor Abdominal/etiología , Infarto/complicaciones , Epiplón/irrigación sanguínea , Dolor Abdominal/diagnóstico , Adolescente , Apendicectomía , Apendicitis/diagnóstico , Temperatura Corporal , Niño , Preescolar , Errores Diagnósticos , Femenino , Humanos , Infarto/diagnóstico , Laparoscopía , Recuento de Leucocitos , Masculino , Estudios Retrospectivos
10.
J Pediatr Surg ; 35(11): 1531-6, 2000 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-11083416

RESUMEN

BACKGROUND/PURPOSE: To better define the indications for peritoneal drainage (PD) in premature babies with intestinal perforation, the authors reviewed their experience with this procedure in a tertiary neonatal intensive care setting. METHODS: The charts of all neonates who underwent PD as initial treatment for intestinal perforation between 1996 and 1999 were reviewed. Those patients with pneumatosis intestinalis on abdominal radiograph had perforated necrotizing enterocolitis (NEC) diagnosed; whereas, those infants with no pneumatosis had isolated intestinal perforation diagnosed. The clinical characteristics and outcomes of these 2 groups were compared. RESULTS: Twenty-one premature neonates had primary PD between 1996 and 1999, 10 for isolated intestinal perforation and 11 for perforated NEC. Patients with isolated intestinal perforation had lower birth weights (708 v 949 g; P < .05), were less likely to have started feedings (30% v 91%, P < .05), and the perforation developed at an earlier age (10.6 v 28.0 d, P < .05) compared with the patients who had perforated NEC. Only 2 of 10 infants with isolated perforation required subsequent laparotomy (at 10 weeks for stricture and 12 weeks for a persistent fistula). For these patients, the long-term survival rate was 90%. In contrast, 8 of 11 infants with perforated NEC required laparotomy, and although the 30-day survival rate was 64%, the long-term survival rate was only 27%. CONCLUSIONS: Peritoneal drainage provides successful and definitive treatment for most premature babies with isolated intestinal perforation. For neonates with perforation caused by NEC, peritoneal drainage may provide temporary stabilization, but most of these infants require subsequent laparotomy, and few survive.


Asunto(s)
Drenaje/métodos , Enterocolitis Necrotizante/terapia , Recién Nacido de muy Bajo Peso , Perforación Intestinal/terapia , Enterocolitis Necrotizante/complicaciones , Enterocolitis Necrotizante/mortalidad , Femenino , Humanos , Recién Nacido , Unidades de Cuidado Intensivo Neonatal , Perforación Intestinal/etiología , Perforación Intestinal/mortalidad , Masculino , Peritoneo/fisiopatología , Probabilidad , Estudios Retrospectivos , Medición de Riesgo , Sensibilidad y Especificidad , Tasa de Supervivencia , Resultado del Tratamiento
12.
Pediatrics ; 105(6): 1250-3, 2000 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-10835065

RESUMEN

PURPOSE: The purpose of this study was to determine indications for gallbladder surgery and risk factors for urgent surgery. METHODS: We reviewed all patients <19 years old, who underwent cholecystectomy between 1980 and 1996. RESULTS: There were 128 patients (mean age: 10 years). Fifty-two patients had an underlying hematologic disorder, 47 had another medical disorder, and 29 had no preexisting illness or identifiable risk factor for gallstone disease. Twenty-five percent (32/128) of cholecystectomies were performed urgently. Postoperative complications developed in 5 of 32 patients (16%) who underwent emergency surgery and 6 of 96 patients (6%) who underwent elective surgery. There were 3 deaths, all occurring in patients undergoing emergency cholecystectomy (odds ratio: 23). Furthermore, all who died had congenital heart disease (odds ratio: 183), making congenital heart disease an independent risk factor for gallstone-related mortality. CONCLUSIONS: Cholecystectomy is recommended when medically possible for children with underlying medical diseases. Patients with medical disorders that make them a high surgical risk can be followed clinically, realizing that if urgent surgery is necessary, the morbidity is relatively high. Those children with congenital heart disease and gallstones are at a prohibitively high risk for death after urgent cholecystectomy. For these patients, the risk of an elective cholecystectomy may be acceptable when weighed against the high risk of complications from their gallstones.


Asunto(s)
Colecistectomía , Colelitiasis/cirugía , Adolescente , Niño , Preescolar , Colelitiasis/complicaciones , Procedimientos Quirúrgicos Electivos , Urgencias Médicas , Femenino , Cálculos Biliares/cirugía , Cardiopatías Congénitas/complicaciones , Humanos , Lactante , Masculino , Complicaciones Posoperatorias
13.
J Pediatr Surg ; 35(2): 349-52, 2000 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-10693694

RESUMEN

BACKGROUND/PURPOSE: The T-tube ileostomy was first used at Texas Children's Hospital in 1959. The purpose of this study is to update the experience since the initial report of this technique in 1981. METHODS: A database of 448 patients with cystic fibrosis (CF) seen in the authors' institution was used to identify 83 patients (18.5%) who presented with meconium ileus. The clinic and hospital charts of these patients were reviewed retrospectively to identify patients who had undergone placement of a T-tube ileostomy. RESULTS: Surgery was performed in 60 of 83 patients for complications of meconium ileus or failure to evacuate the meconium after a contrast enema. Of these patients, 21 of 60 (35%) underwent placement of a T-tube ileostomy. An additional 8 patients were identified who underwent placement of a T-tube ileostomy but were not included in the CF database, for a total of 29 patients who have been treated with T-tube ileostomy since 1959 at Texas Children's Hospital. Five patients were excluded from analysis because of insufficient data or misdiagnosis. One of the 24 patients in the series died of complications of prematurity. A total of 20 of 23 patients had resolution of their meconium ileus after T-tube irrigation with n-acetylcysteine or pancreatic enzymes. Three patients required additional surgery to relieve persistent bowel obstruction. All patients had the T-tube removed within the first 8 weeks after surgery. Two patients required subsequent repair of an incisional hernia. There were otherwise no complications of this procedure, with an average follow-up of 11.5 years. CONCLUSION: In patients with uncomplicated meconium ileus unrelieved by contrast enema, the T-tube ileostomy is an effective and safe treatment.


Asunto(s)
Fibrosis Quística/complicaciones , Ileostomía/métodos , Obstrucción Intestinal/cirugía , Humanos , Recién Nacido , Obstrucción Intestinal/etiología , Meconio
14.
J Pediatr Surg ; 34(6): 959-61, 1999 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-10392913

RESUMEN

PURPOSE: The aim of this study was to compare three methods of postoperative feeding after pyloromyotomy for hypertrophic pyloric stenosis (HPS). METHODS: The authors reviewed retrospectively the charts of 308 patients who underwent pyloromyotomy for HPS from 1984 to 1997. Nineteen patients had prolonged hospitalization for other reasons and were excluded from the study, leaving 289 patients for analysis. All procedures were performed by a single group of pediatric surgeons. The individual preferences of these surgeons resulted in three different feeding schedules: R, strictly regimented (>12 hours nothing by mouth, then incremental feeding over > or =24 hours), I, intermediate (>8 hours nothing by mouth, then incremental feeding over <24 hours), or A, ad lib (< or =4 hours nothing by mouth, with or without a single small feeding, then ad lib feedings). RESULTS: Of the 289 patients, 248 (80.5%) were boys. The average age of the patients was 5.64 weeks (range, 1 to 21 weeks). A total of 265 of 289 (92%) were full term. Thirty-nine of 289 (13.5%) had a family history positive for pyloric stenosis. A total of 104 of 289 (36%) were first-born infants, 89 of 289 (31%) were second born. The diagnosis of pyloric stenosis was made by a combination of physical examination findings and diagnostic image for most patients. An "olive" was palpated in 60.6% of the patients. Sixty percent (60.4%) of patients had an upper gastrointestinal series performed, and 42.5% were examined by ultrasonography. Overall, 53% of the patients had postoperative emesis. Only 3.5% had emesis that persisted greater than 48 hours after surgery. Patients fed ad lib after pyloromyotomy had slightly more emesis (2.2 A v. 1.2 R, and 0.7 I episodes, P = .002), but tolerated full feedings sooner than patients fed with a regimented or intermediate schedule. No patient required additional therapy or readmission after tolerating two consecutive full feedings, suggesting that this might be a suitable discharge criterion for most patients with HPS.


Asunto(s)
Métodos de Alimentación , Cuidados Posoperatorios , Estenosis Pilórica/cirugía , Píloro/cirugía , Femenino , Humanos , Hipertrofia , Lactante , Recién Nacido , Masculino , Estenosis Pilórica/patología , Estudios Retrospectivos , Factores de Tiempo
15.
J Surg Res ; 85(1): 148-57, 1999 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-10383852

RESUMEN

BACKGROUND: Prenatal or neonatal hepatic gene delivery may result in more effective therapy for inborn errors of metabolism due to the immature immune system of the perinatal animal, and the ability to intervene prior to any significant cellular damage. Newborn New Zealand White rabbits have low serum levels of cholesterol at birth, with a significant and sustained rise of cholesterol while they are nursing. We used this physiologic hypercholesterolemia model to study the effect of adenovirus-mediated hepatic gene transfer of rat apolipoprotein B mRNA editing enzyme (Apobec1) on modulation of plasma cholesterol levels. METHODS AND RESULTS: Transcutaneous injection of recombinant adenovirus expressing Apobec1 (AvApobec1) into the liver of newborn rabbits in vivo resulted in efficient Apobec1 expression until Day 50, as detected by PCR-Southern blot analysis. By in vitro editing assay, liver extracts of AvApobec1-treated rabbits were found to have apoB mRNA editing activities of approximately 12, 15, and 15%, on Days 2, 10, and 20 after AvApobec1 administration, compared with 0% editing activity in AvLacZ control vector-injected animals. This physiological level of Apobec1 expression was associated with the production of apoB-48-containing lipoprotein particles from rabbit liver, with a concomitant 30% reduction in total plasma cholesterol compared to AvLacZ-treated or untreated control animals. CONCLUSION: Neonatal intrahepatic delivery of a first-generation adenoviral vector results in efficient gene transfer with little immune response, suggesting that repeated administration may be possible in the neonatal period.


Asunto(s)
Animales Recién Nacidos/sangre , Colesterol/sangre , Citidina Desaminasa/fisiología , Hígado/fisiología , Desaminasas APOBEC-1 , Adenoviridae/genética , Adenoviridae/inmunología , Envejecimiento/inmunología , Animales , Formación de Anticuerpos/fisiología , Anticolesterolemiantes/metabolismo , Citidina Desaminasa/genética , Citidina Desaminasa/inmunología , Técnicas de Transferencia de Gen , Vectores Genéticos/inmunología , Conejos , Ratas , Proteínas Recombinantes
16.
Fetal Diagn Ther ; 14(6): 323-7, 1999.
Artículo en Inglés | MEDLINE | ID: mdl-10640868

RESUMEN

BACKGROUND: Gene therapy for meconium ileus, or other genetic diseases involving the gastrointestinal epithelium, may be possible with prenatal delivery of the CFTR gene to the gastrointestinal tract. Although minimally invasive techniques will probably be used for any future therapy of gastrointestinal disease, it is important to first test this strategy with a reliable animal model. METHODS: The technique of orogastric fetal gene delivery was assessed using 7 pregnant rabbits (22 days' gestation n = 1, 25 days' gestation n = 4, 28 days' gestation n = 2). Four fetuses from each litter were given an adenoviral vector carrying a marker gene by instilling it into the posterior pharynx with an animal feeding needle (1 x 10(10) particles of ADV/RSV/LacZ suspended in 0.3 ml of saline), with the untreated fetuses serving as control animals. RESULTS: There were no recoverable fetuses from the does that had surgery at 22 and 28 days (n = 3) due to maternal death (n = 2) and premature delivery (n = 1). Among the 4 does that underwent hysterotomy at 25 days of gestation, 1 underwent cesarean section 2 days after fetal gene delivery and 3 delivered at term, 5 (n = 1) or 6 (n = 2) days following gene delivery. Eleven of the 16 experimental pups and 7 untreated control animals were collected alive, and were sacrificed at delivery for study. Nine of the 11 experimental pups (82%) showed positive blue (LacZ+) nuclei in the small intestine by X-gal staining. No positive cells were found in 7/7 control animals. The presence of the reporter gene LacZ was confirmed in 8/11 (73%) virus-treated pups by PCR with 5/5 control animals negative for LacZ by PCR. CONCLUSIONS: There was significant maternal and fetal loss related to anesthetic and husbandry issues when surgery was performed at 22 or 28 days of gestation. Based on these preliminary results, we conclude that orogastric gene delivery in the rabbit fetus at 25 days' gestation is an encouraging animal model to study fetal delivery to the gastrointestinal tract.


Asunto(s)
Feto/metabolismo , Técnicas de Transferencia de Gen , Intestino Delgado/embriología , Intestino Delgado/metabolismo , beta-Galactosidasa/genética , Adenoviridae/genética , Animales , ADN Recombinante/análisis , Femenino , Marcadores Genéticos , Terapia Genética , Vectores Genéticos , Edad Gestacional , Faringe/embriología , Reacción en Cadena de la Polimerasa , Embarazo , Conejos
17.
J Pediatr Surg ; 33(6): 874-9, 1998 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-9660219

RESUMEN

BACKGROUND/PURPOSE: For over 50 years there has been debate over how to manage the contralateral groin in children who present with a unilateral inguinal hernia. Many preoperative and intraoperative tools to diagnose a contralateral patent processus vaginalis or true inguinal hernia have been described. In 1992 laparoscopy was introduced as a new diagnostic test. Although multiple series have assessed this new tool, none of them have been able to statistically show that laparoscopy is effective in assessing the contralateral groin. By combining all published studies and using the technique of meta-analysis, intraoperative laparoscopy can be shown to be effective in diagnosing a contralateral patent processus vaginalis in children undergoing unilateral inguinal herniorrhaphy. METHODS: All available studies of children with a unilateral hernia who had exploration of the contralateral groin by laparoscopy were reanalyzed. Sensitivity and specificity of laparoscopy was determined using open exploration or development of a metachronous hernia as the gold standard. RESULTS: Nine hundred sixty-four patients were suitable for analysis. A contralateral hernia was seen on laparoscopy in 376 patients. All of these patients underwent open contralateral exploration. A patent processus vaginalis or true hernia sac was found in 373. The sensitivity of laparoscopy was 99.4% (95% confidence interval 97.87 to 99.91). Five hundred eighty-eight patients had a laparoscopy with negative results. Sixty-two of these patients then had open contralateral exploration. In one case, a patent processus vaginalis was found; the other 61 patients underwent exploration with negative results. In the remaining 526 laparoscopy-negative patients, follow-up (1 month to 3 years) was used to see if a contralateral hernia developed. A metachronous hernia developed in one of the 526 patients. The specificity of laparoscopy was 99.5% (95% confidence interval 98.39 to 99.87). Laparoscopy added an average of 6 minutes to the surgical time and was accurate regardless of the technique. There were two minor complications related to laparoscopy and no deaths. CONCLUSIONS: Laparoscopy may be the ideal tool to diagnose a contralateral patent processus vaginalis intraoperatively. It is sensitive, specific, fast, and safe. Although the presence of a patent processus does not imply that the patient will go on to develop a metachronous hernia, identifying and ligating a patent processus should certainly prevent the development of an indirect inguinal hernia.


Asunto(s)
Hernia Inguinal/diagnóstico , Laparoscopía , Niño , Hernia Inguinal/cirugía , Humanos , Ligadura , Estudios Retrospectivos , Sensibilidad y Especificidad
18.
Am J Surg ; 174(6): 741-4, 1997 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-9409609

RESUMEN

BACKGROUND: Inguinal herniorrhaphy is the most common general surgical procedure performed in children. The presence of a contralateral patent processus vaginalis forms the basis of the recommendation for contralateral exploration in patients undergoing unilateral herniorrhaphy. However, a patent processus vaginalis does not necessarily go on to become a clinically apparent inguinal hernia. METHODS: All published pediatric series, in which patients underwent unilateral inguinal hernia repair and were evaluated for the development of a metachronous hernia, were included. The incidence of and risk factors associated with development a metachronous hernia were evaluated with meta-analysis. RESULTS: There were 15,310 patients ranging in age from birth to 16 years, including premature infants. Of these, 1,062 patients (7%) developed a metachronous hernia. Gender and age were not risk factors. There was an 11% risk of metachronous hernia if the original hernia was on the left side, a risk that was 50% greater than if the original hernia was on the right. Of patients who developed a metachronous hernia, 90% did so within 5 years. The complication rate of metachronous hernia was 0.5%. CONCLUSION: There is no role for routine contralateral groin exploration. High-risk infants and children, especially those who undergo left inguinal herniorrhaphy, may benefit from contralateral groin exploration. If a patent processus vaginalis is found, it should be ligated. Patients who do not undergo contralateral groin exploration should be followed up for 5 years.


Asunto(s)
Hernia Inguinal/complicaciones , Hernia Inguinal/cirugía , Adolescente , Niño , Preescolar , Humanos , Lactante , Recién Nacido , Ligadura , Riesgo
19.
J Invest Surg ; 10(1-2): 41-6, 1997.
Artículo en Inglés | MEDLINE | ID: mdl-9100174

RESUMEN

The prevention of perinatal complications of congenital gastrointestinal (GI) diseases such as cystic fibrosis may require prenatal treatment. New Zealand White rabbits were evaluated as a potential animal model to study gastrointestinal anatomy and transit in the fetus. The lengths of the GI tract of fetuses at 21/31 and 28/31 days gestation were established, and gastric volume was measured. Gastric volume at 28 days gestation averaged 2.6 mL, adequate to permit instillation of a solution into the fetal stomach. A study was then carried out to establish gastric emptying and delivery of the solution into the small bowel. Using ultrasound guidance, the stomachs of 26 fetuses from 7 litters were punctured and 0.5 mL of dilute barium was injected. A cesarean section was performed 4 h later and the progression of barium though the GI tract was measured. In 18/26 (69.2%) of the fetuses barium was successfully delivered to the lumen of the stomach. In these 18 fetuses, barium progressed to the duodenum in 15 (58%), the jejunum in 13 (50%), and the ileum in 8 (31%). The stomach of the 3.5- to 4-week-old fetus is large enough to allow transuterine delivery of a solution of dilute barium. Gastric and intestinal motility in the 25-day-old rabbit fetus is coordinated and results in delivery of barium to the small bowel in 50% of animals successfully injected. The results suggest that the rabbit is an acceptable model for the study of gastrointestinal delivery of therapeutic drugs or genes to the fetus.


Asunto(s)
Feto/cirugía , Intestino Delgado/cirugía , Agujas , Diagnóstico Prenatal/métodos , Estómago/cirugía , Animales , Bario , Estudios de Evaluación como Asunto , Femenino , Enfermedades Gastrointestinales/diagnóstico por imagen , Enfermedades Gastrointestinales/patología , Enfermedades Gastrointestinales/cirugía , Embarazo , Conejos , Radiografía , Útero
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