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1.
Pediatr Surg Int ; 35(5): 619-623, 2019 May.
Artículo en Inglés | MEDLINE | ID: mdl-30761427

RESUMEN

PURPOSE: There is currently no gold standard for the type of analgesia or preferred circumcision technique in infants requiring circumcision after 1 month of age. Our study presents a modified Plastibell circumcision technique, which offers excellent surgical outcomes, and can be performed under local anesthesia until 6 months of age, thereby avoiding the risks of general anesthesia in delayed circumcision. METHODS: This is a retrospective case series of 508 consecutive male infants between 1 and 6 months of age, from one institution, who all underwent circumcision under local anesthesia, performed by the same pediatric surgeon, from 2013 to 2018. The study parameters included postoperative complications such as re-operation for control of hemorrhage, wound infection, circumcision revision, and urethral meatotomy. RESULTS: There were no re-operations for control of hemorrhage, no wound infections, and no circumcision revisions. One patient developed urethral meatal stenosis requiring urethral meatotomy. CONCLUSION: Our modified Plastibell circumcision technique under local anesthesia is a safe and reproducible alternative for infants between 1 and 6 months of age, whose parents desire circumcision and wish to avoid general anesthesia.


Asunto(s)
Anestesia Local/métodos , Circuncisión Masculina/instrumentación , Circuncisión Masculina/métodos , Anestesia General , Humanos , Lactante , Masculino , Ciudad de Nueva York , Estudios Retrospectivos , Resultado del Tratamiento
2.
Pediatr Surg Int ; 30(6): 681-4, 2014 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24781346

RESUMEN

Pyloric atresia with epidermolysis bullosa (EB) dystrophica is a rare entity that may not be immediately recognized. We describe the fourth confirmed case of pyloric atresia associated with the dystrophic subtype of EB diagnosed by standard pathologic measures, and discuss the clinical disease features and recent advances in the pathophysiology.


Asunto(s)
Epidermólisis Ampollosa Distrófica/diagnóstico , Obstrucción de la Salida Gástrica/congénito , Obstrucción de la Salida Gástrica/diagnóstico , Píloro/anomalías , Biopsia , Diagnóstico Diferencial , Epidermólisis Ampollosa Distrófica/fisiopatología , Resultado Fatal , Femenino , Obstrucción de la Salida Gástrica/fisiopatología , Humanos , Recién Nacido , Píloro/fisiopatología
3.
Blood ; 116(4): 661-70, 2010 Jul 29.
Artículo en Inglés | MEDLINE | ID: mdl-20363774

RESUMEN

Although platelets appear by embryonic day 10.5 in the developing mouse, an embryonic role for these cells has not been identified. The SYK-SLP-76 signaling pathway is required in blood cells to regulate embryonic blood-lymphatic vascular separation, but the cell type and molecular mechanism underlying this regulatory pathway are not known. In the present study we demonstrate that platelets regulate lymphatic vascular development by directly interacting with lymphatic endothelial cells through C-type lectin-like receptor 2 (CLEC-2) receptors. PODOPLANIN (PDPN), a transmembrane protein expressed on the surface of lymphatic endothelial cells, is required in nonhematopoietic cells for blood-lymphatic separation. Genetic loss of the PDPN receptor CLEC-2 ablates PDPN binding by platelets and confers embryonic lymphatic vascular defects like those seen in animals lacking PDPN or SLP-76. Platelet factor 4-Cre-mediated deletion of Slp-76 is sufficient to confer lymphatic vascular defects, identifying platelets as the cell type in which SLP-76 signaling is required to regulate lymphatic vascular development. Consistent with these genetic findings, we observe SLP-76-dependent platelet aggregate formation on the surface of lymphatic endothelial cells in vivo and ex vivo. These studies identify a nonhemostatic pathway in which platelet CLEC-2 receptors bind lymphatic endothelial PDPN and activate SLP-76 signaling to regulate embryonic vascular development.


Asunto(s)
Proteínas Adaptadoras Transductoras de Señales/fisiología , Plaquetas/fisiología , Lectinas Tipo C/fisiología , Vasos Linfáticos/embriología , Vasos Linfáticos/fisiología , Fosfoproteínas/fisiología , Proteínas Adaptadoras Transductoras de Señales/genética , Proteínas Adaptadoras Transductoras de Señales/metabolismo , Animales , Plaquetas/metabolismo , Vasos Sanguíneos/metabolismo , Células Cultivadas , Embrión de Mamíferos , Células Endoteliales/metabolismo , Células Endoteliales/fisiología , Endotelio Linfático/embriología , Endotelio Linfático/metabolismo , Endotelio Vascular/embriología , Endotelio Vascular/metabolismo , Humanos , Lectinas Tipo C/genética , Lectinas Tipo C/metabolismo , Vasos Linfáticos/metabolismo , Glicoproteínas de Membrana/metabolismo , Glicoproteínas de Membrana/fisiología , Ratones , Ratones Endogámicos C57BL , Ratones Transgénicos , Fosfoproteínas/genética , Fosfoproteínas/metabolismo , Unión Proteica , Transducción de Señal/genética , Transducción de Señal/fisiología
4.
Biol Blood Marrow Transplant ; 14(7): 729-40, 2008 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-18541191

RESUMEN

In utero hematopoietic stem cell transplantation (IUHCT) is a potential therapeutic alternative to postnatal hematopoietic stem cell transplantation (HSCT) for congenital hematologic disorders that can be diagnosed early in gestation and can be cured by HSCT. The rationale is to take advantage of normal events during hematopoietic and immunologic ontogeny to facilitate allogeneic hematopoietic engraftment. Although the rationale remains compelling, IUHCT has not yet achieved its clinical potential. Achieving therapeutic levels of engraftment by IUHCT alone remains challenging. However, considerable experimental progress has been made toward the clinical strategy of using IUHCT to induce donor-specific tolerance to facilitate a relatively nontoxic postnatal HSCT. Because donor specific tolerance induction requires relatively minimal engraftment, this strategy may hold the key to broad clinical application of IUHCT in the near future.


Asunto(s)
Terapias Fetales/métodos , Trasplante de Células Madre Hematopoyéticas/métodos , Animales , Modelos Animales de Enfermedad , Femenino , Enfermedades Fetales/terapia , Enfermedades Genéticas Congénitas/terapia , Supervivencia de Injerto , Humanos , Embarazo , Quimera por Trasplante/inmunología , Tolerancia al Trasplante/inmunología
5.
J Pediatr Surg ; 53(7): 1339-1344, 2018 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-29032983

RESUMEN

BACKGROUND: Complicated appendicitis is common in children, yet the timing of surgical management remains controversial. Some support initial antibiotics with delayed operation whereas others support immediate operation. While a few randomized trials have evaluated this question, they have been small, single-center trials with limited follow-up. We present a database analysis of outcomes in early versus late surgical management of complicated appendicitis with one-year follow-up. METHODS: We conducted a retrospective review of children with complicated appendicitis presenting between 2000 and 2013, utilizing a New York State database. We compare children undergoing later versus early appendectomy with a primary outcome measure of any complication within one year as determined from ICD-9 codes. RESULTS: 8840 children were included in the analysis, 7708 of whom underwent early appendectomy. Patients with late appendectomy were significantly more likely to have at least one complication when compared to those undergoing early appendectomy (34.6% vs 26.7%, p<0.01). CONCLUSIONS: We present the first population-level study evaluating early versus late appendectomy in children with complicated appendicitis with a one-year follow-up period. Children undergoing late appendectomy were more likely to have a complication than those undergoing early appendectomy. These data corroborated previous studies supporting early operative management. LEVEL OF EVIDENCE: This study provides level III evidence of a treatment study.


Asunto(s)
Antibacterianos/uso terapéutico , Apendicectomía , Apendicitis/diagnóstico por imagen , Apendicitis/cirugía , Complicaciones Posoperatorias/epidemiología , Análisis de Varianza , Apendicectomía/efectos adversos , Apendicitis/tratamiento farmacológico , Niño , Bases de Datos Factuales , Femenino , Estudios de Seguimiento , Humanos , Tiempo de Internación , Masculino , New York , Estudios Retrospectivos , Infección de la Herida Quirúrgica/epidemiología , Tiempo de Tratamiento , Espera Vigilante
6.
Surgery ; 136(3): 669-76, 2004 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-15349117

RESUMEN

BACKGROUND: Pancreatic duct cells (PDCs) are responsible for bicarbonate production by the pancreas. The trypsin-sensitive protease-activated receptor (PAR-2), also known as the trypsin receptor, is highly expressed in the pancreatic duct system and has been shown to regulate PDC ion transport. The possible role of this receptor on bicarbonate secretion, the central function of duct cells, is unknown. We hypothesize that PAR-2 may regulate pancreatic bicarbonate secretion during times of inappropriate pancreatic enzyme activation. METHODS: To study this hypothesis in vitro, explants of the bovine main pancreatic duct were isolated and maintained in primary culture. They were then mounted in Ussing chambers, and bicarbonate secretion was determined with an autoburette titration. The response to luminal or serosal trypsin (10 micromol/L) and the synthetic trypsin receptor activating peptide (TRAP) (30 micromol/L) on spontaneous and secretin-stimulated bicarbonate secretion (10 nmol/L) was examined. RESULTS: Serosal trypsin had no effect. Both luminal trypsin and TRAP significantly reduced the spontaneous bicarbonate secretion observed at luminal pH 7.4 (2.8 +/- 0.2 - 0.4 +/- 0.1 micromol/hr/cm(2) and 4.0 +/- 1.2 - 1.6 +/- 0.4 micromol/hr/cm(2), respectively) in a reversible manner. Baseline bicarbonate secretion at luminal pH 8.0 was reduced by trypsin and TRAP, but the increase in response to secretin stimulation observed with controls was unaffected. CONCLUSIONS: PAR-2 activation may be the mechanism by which pancreatic juice secretion is inhibited during pancreatitis. We suggest that pharmacologic activation of PAR-2 receptors could suppress pancreatic exocrine secretion and thus serve as a potential agent in the treatment and prevention of pancreatic fistulas.


Asunto(s)
Bicarbonatos/metabolismo , Conductos Pancreáticos/metabolismo , Receptor PAR-2/metabolismo , Animales , Bovinos , Células Cultivadas , Conductos Pancreáticos/citología , Conductos Pancreáticos/efectos de los fármacos , Receptor PAR-2/agonistas , Serina Endopeptidasas/farmacología , Tripsina/farmacología
7.
Mol Ther Methods Clin Dev ; 1: 14040, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-26015979

RESUMEN

Efficient gene transfer to muscle stem cells (satellite cells) has not been achieved despite broad transduction of skeletal muscle by systemically administered adeno-associated virus serotype 2/9 (AAV-9) in mice. We hypothesized that cellular migration during fetal development would make satellite cells accessible for gene transfer following in utero intravascular injection. We injected AAV-9 encoding green fluorescent protein (GFP) marker gene into the vascular space of mice ranging in ages from post-coital day 12 (E12) to postnatal day 1 (P1). Satellite cell transduction was examined using: immunohistochemistry and confocal microscopy, satellite cell migration assay, myofiber isolation and FACS analysis. GFP positive myofibers were detected in all mature skeletal muscle groups and up to 100% of the myofibers were transduced. We saw gestational variation in cardiac and skeletal muscle expression. E16 injection resulted in 27.7 ± 10.0% expression in satellite cells, which coincides with the timing of satellite cell migration, and poor satellite cell expression before and after satellite cell migration (E12 and P1). Our results demonstrate that efficient gene expression is achieved in differentiated myofibers and satellite cells after injection of AAV-9 in utero. These findings support the potential of prenatal gene transfer for muscle based treatment strategies.

9.
J Clin Invest ; 119(9): 2590-600, 2009 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-19652363

RESUMEN

The lack of fetal immune responses to foreign antigens, i.e., fetal immunologic tolerance, is the most compelling rationale for prenatal stem cell and gene therapy. However, the frequency of engraftment following in utero hematopoietic cell transplantation (IUHCT) in the murine model is reduced in allogeneic, compared with congenic, recipients. This observation supports the existence of an immune barrier to fetal transplantation and challenges the classic assumptions of fetal tolerance. Here, we present evidence that supports the presence of an adaptive immune response in murine recipients of IUHCT that failed to maintain engraftment. However, when IUHCT recipients were fostered by surrogate mothers, they all maintained long-term chimerism. Furthermore, we have demonstrated that the cells responsible for rejection of the graft were recipient in origin. Our observations suggest a mechanism by which IUHCT-dependent sensitization of the maternal immune system and the subsequent transmission of maternal alloantibodies to pups through breast milk induces a postnatal adaptive immune response in the recipient, which, in turn, results in the ablation of engraftment after IUHCT. Finally, we showed that non-fostered pups that maintained their chimerism had higher levels of Tregs as well as a more suppressive Treg phenotype than their non-chimeric, non-fostered siblings. This study resolves the apparent contradiction of induction of an adaptive immune response in the pre-immune fetus and confirms the potential of actively acquired tolerance to facilitate prenatal therapeutic applications.


Asunto(s)
Feto/inmunología , Trasplante de Células Madre Hematopoyéticas , Inmunidad Materno-Adquirida , Isoanticuerpos/metabolismo , Animales , Femenino , Rechazo de Injerto/inmunología , Tolerancia Inmunológica , Técnicas In Vitro , Activación de Linfocitos , Prueba de Cultivo Mixto de Linfocitos , Ratones , Ratones Endogámicos BALB C , Ratones Endogámicos C57BL , Ratones Transgénicos , Embarazo , Linfocitos T Reguladores/clasificación , Linfocitos T Reguladores/inmunología , Quimera por Trasplante/inmunología , Trasplante Homólogo
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