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1.
Am Surg ; : 31348241248788, 2024 Apr 22.
Artigo em Inglês | MEDLINE | ID: mdl-38648035

RESUMO

BACKGROUND: The minimum weight for enterostomy closure (EC) in infants remains debated with the current acceptable cut-off of >2 kg. As enterostomy-related complications or high enterostomy output (>30cc/kg/d) may prohibit a premature infant from reaching 2 kg, additional data is needed to evaluate the safety of EC in infants <2 kg. The objective of this study was to evaluate postoperative outcomes in low body weight (<2 kg) infants undergoing EC compared to larger infants. METHODS: We performed a multi-center retrospective analysis from 1/1/2012-12/31/2022 of all infants (age <1 year) who were <4 kg at time of EC. Primary outcomes included postoperative complications and 30-day mortality. Non-parametric analysis was performed using the Kruskal-Wallis one-way analysis of variance and chi-square tests. Univariable logistic regression was performed to identify factors associated with postoperative complications. RESULTS: Of 92 infants, 15 infants (16.3%) underwent EC at <2 kg, 16 (17.4%) at 2-2.49 kg, 31 (33.7%) at 2.5-2.99 kg, and 30 (32.6%) at ≥3 kg. Infants <2 kg at time of EC exhibited higher rates of hyperbilirubinemia (P = .030), neurologic comorbidities (P = .030), and high enterostomy output (P = .041). There was no difference in postoperative complications (P = .460) or 30-day mortality (P = .460) between the <2 kg group and larger weight groups. Low body weight was not associated with an increased risk for developing a postoperative complication (OR: 1.001, 95% CI: 1.001-1.001; P = .032). CONCLUSION: Our findings suggest that EC in infants <2 kg may be safe with comparable postoperative outcomes to larger weight infants. Thus, the timing of EC should be based on the infant's physiologic status, in contrast to a predetermined minimum weight cut-off.

2.
Elife ; 92020 08 07.
Artigo em Inglês | MEDLINE | ID: mdl-32762847

RESUMO

Mitochondrial Ca2+ uptake is mediated by an inner mitochondrial membrane protein called the mitochondrial calcium uniporter. In humans, the uniporter functions as a holocomplex consisting of MCU, EMRE, MICU1 and MICU2, among which MCU and EMRE form a subcomplex and function as the conductive channel while MICU1 and MICU2 are EF-hand proteins that regulate the channel activity in a Ca2+-dependent manner. Here, we present the EM structures of the human mitochondrial calcium uniporter holocomplex (uniplex) in the presence and absence of Ca2+, revealing distinct Ca2+ dependent assembly of the uniplex. Our structural observations suggest that Ca2+ changes the dimerization interaction between MICU1 and MICU2, which in turn determines how the MICU1-MICU2 subcomplex interacts with the MCU-EMRE channel and, consequently, changes the distribution of the uniplex assemblies between the blocked and unblocked states.


Assuntos
Canais de Cálcio/química , Canais de Cálcio/metabolismo , Cálcio/química , Cálcio/metabolismo , Proteínas de Ligação ao Cálcio/química , Proteínas de Ligação ao Cálcio/metabolismo , Proteínas de Transporte de Cátions/química , Proteínas de Transporte de Cátions/metabolismo , Humanos , Proteínas de Transporte da Membrana Mitocondrial/química , Proteínas de Transporte da Membrana Mitocondrial/metabolismo , Modelos Moleculares , Complexos Multiproteicos/química , Complexos Multiproteicos/metabolismo , Conformação Proteica , Multimerização Proteica
3.
Cell ; 177(5): 1252-1261.e13, 2019 05 16.
Artigo em Inglês | MEDLINE | ID: mdl-31080062

RESUMO

Mitochondrial calcium uptake is crucial to the regulation of eukaryotic Ca2+ homeostasis and is mediated by the mitochondrial calcium uniporter (MCU). While MCU alone can transport Ca2+ in primitive eukaryotes, metazoans require an essential single membrane-spanning auxiliary component called EMRE to form functional channels; however, the molecular mechanism of EMRE regulation remains elusive. Here, we present the cryo-EM structure of the human MCU-EMRE complex, which defines the interactions between MCU and EMRE as well as pinpoints the juxtamembrane loop of MCU and extended linker of EMRE as the crucial elements in the EMRE-dependent gating mechanism among metazoan MCUs. The structure also features the dimerization of two MCU-EMRE complexes along an interface at the N-terminal domain (NTD) of human MCU that is a hotspot for post-translational modifications. Thus, the human MCU-EMRE complex, which constitutes the minimal channel components among metazoans, provides a framework for future mechanistic studies on MCU.


Assuntos
Canais de Cálcio/metabolismo , Ativação do Canal Iônico/fisiologia , Complexos Multiproteicos/metabolismo , Multimerização Proteica/fisiologia , Canais de Cálcio/genética , Células HEK293 , Humanos , Complexos Multiproteicos/genética , Domínios Proteicos , Estrutura Secundária de Proteína
4.
Nature ; 562(7728): E25, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30108362

RESUMO

In this Article, ref. 15 has been replaced and references 32 to 50 have been renumbered online.

5.
Nature ; 559(7715): 570-574, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29995855

RESUMO

The mitochondrial calcium uniporter (MCU) is a highly selective calcium channel localized to the inner mitochondrial membrane. Here, we describe the structure of an MCU orthologue from the fungus Neosartorya fischeri (NfMCU) determined to 3.8 Å resolution by phase-plate cryo-electron microscopy. The channel is a homotetramer with two-fold symmetry in its amino-terminal domain (NTD) that adopts a similar structure to that of human MCU. The NTD assembles as a dimer of dimers to form a tetrameric ring that connects to the transmembrane domain through an elongated coiled-coil domain. The ion-conducting pore domain maintains four-fold symmetry, with the selectivity filter positioned at the start of the pore-forming TM2 helix. The aspartate and glutamate sidechains of the conserved DIME motif are oriented towards the central axis and separated by one helical turn. The structure of NfMCU offers insights into channel assembly, selective calcium permeation, and inhibitor binding.


Assuntos
Canais de Cálcio/química , Canais de Cálcio/ultraestrutura , Microscopia Crioeletrônica , Neosartorya/química , Sítios de Ligação , Cálcio/metabolismo , Canais de Cálcio/metabolismo , Humanos , Ativação do Canal Iônico/efeitos dos fármacos , Transporte de Íons/efeitos dos fármacos , Modelos Moleculares , Domínios Proteicos , Subunidades Proteicas/química , Subunidades Proteicas/metabolismo , Compostos de Rutênio/farmacologia , Solubilidade
6.
J Laparoendosc Adv Surg Tech A ; 27(4): 427-429, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28055335

RESUMO

INTRODUCTION: Conditions requiring an esophagectomy and esophageal replacement are rare in children. The preferred method and ideal replacement organ continue to be debated. We present long-term outcomes in children treated with esophagectomy and gastric pull-up. METHODS: We conducted a retrospective review of all the patients who underwent a esophagectomy and gastric pull-up at two major pediatric institutions from 2004 to 2015. Follow-up data were obtained for children when available, including any postoperative complications, need for dilation of strictures, and current feeding method. RESULTS: Minimally invasive procedures were performed on 7 patients (5 female and 2 male) with a median age of 3 years (range 2-20, standard deviation = 8). Three patients successfully underwent laparoscopic transhiatal esophagectomy and cervical gastric pull-up, and three patients successfully underwent combined laparoscopic and right thoracoscopic (Ivor-Lewis) esophagectomy and cervical gastric pull-up. We identified an additional 3 patients who had an open esophagectomy and gastric pull-up. Seven patients had tubularized gastric conduits, six without pyloroplasty and one with pyloroplasty. For those patients with tubularized conduits, the average time to achieve full oral feeds was 16 days, with 1 patient with pyloroplasty who took 27 days. Of the three whole-stomach conduits, one reached oral independence at 19 days and the other two had yet tolerated anything per os. Follow-up data were available for all patients. At the average 5 years follow-up (ranging from 1 month to 7 years), all but two were thriving well with full oral feeds. CONCLUSIONS: Minimally invasive esophagectomy and gastric pull-up is a good alternative in managing pediatric patients in need of esophagectomy and replacement; it offers acceptable early and long-term outcomes. Tubularized conduit appears to be superior to using the whole stomach and potentially avoids pyloroplasty. Ongoing study is needed to validate our findings.


Assuntos
Queimaduras Químicas/cirurgia , Atresia Esofágica/cirurgia , Estenose Esofágica/cirurgia , Esofagectomia/métodos , Esôfago/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Piloro/cirurgia , Estômago/cirurgia , Adolescente , Criança , Pré-Escolar , Acalasia Esofágica/cirurgia , Estenose Esofágica/induzido quimicamente , Estenose Esofágica/congênito , Esôfago/lesões , Feminino , Humanos , Laparoscopia/métodos , Masculino , Procedimentos Cirúrgicos Minimamente Invasivos , Pescoço , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Adulto Jovem
7.
Am Surg ; 82(10): 964-967, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27779984

RESUMO

The optimal management of pilonidal disease in the pediatric population is still debated. We conducted a retrospective review of patients 21 years old and younger who underwent surgical management for pilonidal disease between 2009 and 2013 at a single pediatric institution. Sixty patients (41.7% male) were included in the analysis, with a mean age of 15.0 years (range, 13-20). Twelve (20%) had a prior drainage procedure for pilonidal abscess before the definitive operative treatment. After excision to the presacral fascia, 36 (60%) had primary closure, 17 (28.3%) were left to heal by secondary intention, and 7 (11.7%) had flap closure. Overall recurrence rate was 41.7 per cent with 33.3 per cent in the primary, 58.8 per cent in the secondary, and 42.9 per cent in the flap group, respectively. Ten (16.7%) patients developed postoperative complications, which were similar among surgical groups, gender, and body mass index. The average length of stay was 0.67 (median 0, range, 0-5) days. Primary closure had the shortest length of stay (analysis of variance P = 0.04), and flap closure had no reoperations (analysis of variance P < 0.01). Pilonidal disease remains surgically challenging. Our data suggest that excision and primary closure is a better option in the pediatric population.


Assuntos
Seio Pilonidal/diagnóstico , Seio Pilonidal/cirurgia , Retalhos Cirúrgicos , Cicatrização/fisiologia , Adolescente , Fatores Etários , Análise de Variância , Criança , Estudos de Coortes , Feminino , Seguimentos , Humanos , Tempo de Internação , Masculino , Pediatria , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Resultado do Tratamento , Técnicas de Fechamento de Ferimentos/normas , Técnicas de Fechamento de Ferimentos/tendências , Adulto Jovem
8.
J Laparoendosc Adv Surg Tech A ; 26(6): 483-7, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27135255

RESUMO

INTRODUCTION: Achalasia is an uncommon disorder in children. Currently, there is no consensus regarding the optimal treatment for achalasia. We investigate the effectiveness of symptom relief in patients who underwent endoscopic treatments versus Heller myotomy (HM). METHODS: We conducted a retrospective review of all children (age 0-18 years) treated for achalasia at two pediatric hospitals from 2004 to 2014. Demographics, presenting symptoms, outcomes, and complications were analyzed. RESULTS: Twenty-three patients (61% male) were identified with a mean age at diagnosis of 11.6 ± 5.0 years. About 47.8% of the cohort had no comorbidities. Common presenting symptoms included weight loss/failure to thrive (87.0%), emesis (69.6%), and dysphagia (69.6%). Mean time from symptom onset to diagnosis was 18 ± 18.9 months. Nine patients underwent laparoscopic HM as their primary treatment, whereas 14 received esophageal dilatation (ED) as their first-line therapy. Patients who underwent ED as their initial treatment were younger (9.92 versus 15.6 years, P = .047). Patients who underwent HM were more likely to attain symptom resolution compared to those managed with ED alone (P = .004). Of the 14 patients who underwent ED initially, 10 subsequently required HM due to persistent symptoms. None of the 4 patients who underwent ED alone achieved long-term symptom relief and, on the average, required an increased number of procedures compared to their HM counterparts (5.25 versus 2.47, P = .010). There was a trend toward increased intraoperative mucosal perforation in patients who underwent preoperative ED and botulinum injections. CONCLUSION: Our data suggest that HM is superior to balloon dilatation or botulinum injection in children with achalasia. We conclude that HM should be recommended for newly diagnosed children with achalasia as a first-line therapy.


Assuntos
Toxinas Botulínicas/uso terapêutico , Dilatação , Acalasia Esofágica/terapia , Esfíncter Esofágico Inferior/cirurgia , Esofagoscopia , Laparoscopia , Fármacos Neuromusculares/uso terapêutico , Adolescente , Criança , Pré-Escolar , Dilatação/instrumentação , Dilatação/métodos , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Injeções , Masculino , Estudos Retrospectivos , Resultado do Tratamento
9.
mBio ; 6(2)2015 Mar 31.
Artigo em Inglês | MEDLINE | ID: mdl-25827420

RESUMO

UNLABELLED: Bacteria engage in chemical signaling, termed quorum sensing (QS), to mediate intercellular communication, mimicking multicellular organisms. The LuxR family of QS transcription factors regulates gene expression, coordinating population behavior by sensing endogenous acyl homoserine lactones (AHLs). However, some bacteria (such as Escherichia coli) do not produce AHLs. These LuxR orphans sense exogenous AHLs but also regulate transcription in the absence of AHLs. Importantly, this AHL-independent regulatory mechanism is still largely unknown. Here we present several structures of one such orphan LuxR-type protein, SdiA, from enterohemorrhagic E. coli (EHEC), in the presence and absence of AHL. SdiA is actually not in an apo state without AHL but is regulated by a previously unknown endogenous ligand, 1-octanoyl-rac-glycerol (OCL), which is ubiquitously found throughout the tree of life and serves as an energy source, signaling molecule, and substrate for membrane biogenesis. While exogenous AHL renders to SdiA higher stability and DNA binding affinity, OCL may function as a chemical chaperone placeholder that stabilizes SdiA, allowing for basal activity. Structural comparison between SdiA-AHL and SdiA-OCL complexes provides crucial mechanistic insights into the ligand regulation of AHL-dependent and -independent function of LuxR-type proteins. Importantly, in addition to its contribution to basic science, this work has implications for public health, inasmuch as the SdiA signaling system aids the deadly human pathogen EHEC to adapt to a commensal lifestyle in the gastrointestinal (GI) tract of cattle, its main reservoir. These studies open exciting and novel avenues to control shedding of this human pathogen in the environment. IMPORTANCE: Quorum sensing refers to bacterial chemical signaling. The QS acyl homoserine lactone (AHL) signals are recognized by LuxR-type receptors that regulate gene transcription. However, some bacteria have orphan LuxR-type receptors and do not produce AHLs, sensing them from other bacteria. We solved three structures of the E. coli SdiA orphan, in the presence and absence of AHL. SdiA with no AHL is not in an apo state but is regulated by a previously unknown endogenous ligand, 1-octanoyl-rac-glycerol (OCL). OCL is ubiquitously found in prokaryotes and eukaryotes and is a phospholipid precursor for membrane biogenesis and a signaling molecule. While AHL renders to SdiA higher stability and DNA-binding affinity, OCL functions as a chemical chaperone placeholder, stabilizing SdiA and allowing for basal activity. Our studies provide crucial mechanistic insights into the ligand regulation of SdiA activity.


Assuntos
Acil-Butirolactonas/metabolismo , Diglicerídeos/metabolismo , Escherichia coli Êntero-Hemorrágica/efeitos dos fármacos , Proteínas de Escherichia coli/metabolismo , Regulação Bacteriana da Expressão Gênica/efeitos dos fármacos , Percepção de Quorum , Transativadores/metabolismo , Transcrição Gênica/efeitos dos fármacos , Acil-Butirolactonas/química , Animais , Bovinos , Cristalografia por Raios X , Diglicerídeos/química , Escherichia coli Êntero-Hemorrágica/genética , Escherichia coli Êntero-Hemorrágica/fisiologia , Proteínas de Escherichia coli/química , Humanos , Modelos Moleculares , Conformação Proteica , Estabilidade Proteica , Transativadores/química
10.
J Laparoendosc Adv Surg Tech A ; 23(10): 876-80, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-24079961

RESUMO

BACKGROUND: Laparoscopic repair of congenital duodenal obstruction has become popularized over the past decade. Comparative data on outcomes, however, are sparse. We hypothesized that laparoscopic repair of congenital duodenal obstruction could be performed with similar outcomes to traditional open repair. PATIENTS AND METHODS: Medical records for all cases of congenital duodenal obstruction from 2005 to 2011 at three academic teaching hospitals were retrospectively reviewed. Patients were excluded from the analysis if they had confounding surgical diseases, did not have duodenoduodenostomy during the first hospital admission, had the repair performed before transfer from a referring hospital, or weighed less than 1.7 kg at the time of surgery. Analysis was performed as intention to treat, with laparoscopic converted to open cases included in the laparoscopic group. RESULTS: Sixty-four cases were included in the analysis (44 open, 20 laparoscopic). Baseline characteristics were similar between the two groups with the exception that the open group, on average, underwent repair later than the laparoscopic group (6 days versus 4 days, respectively). Seven laparoscopic cases were converted to an open procedure (35%), most commonly for difficulty in exposing the decompressed distal duodenum. Laparoscopic repair did take significantly longer than open repair (145 minutes versus 96 minutes, respectively), but clinical outcomes were similar. Complications were rare and were similar between methods of repair. Two patients in the laparoscopic group required subsequent open revision. CONCLUSIONS: Laparoscopic duodenoduodenostomy for congenital duodenal obstruction is a technically challenging procedure with a steep learning curve. Despite a relatively high conversion rate, clinical outcomes remained similar to the traditional open repair in selected patients.


Assuntos
Obstrução Duodenal/congênito , Obstrução Duodenal/cirurgia , Laparoscopia , Fatores Etários , Peso Corporal , Competência Clínica , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
11.
Pediatr Surg Int ; 29(7): 715-8, 2013 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-23728507

RESUMO

PURPOSE: Ovarian masses in the pediatric population are commonly resected with a three or four port laparoscopic approach. Single-incision laparoscopic (SIL) resection is an alternative approach. However, there is limited experience with this modality in ovarian mass resection. METHODS: We reviewed SIL ovarian mass resections performed by our group from 2010 to 2012. We evaluated patient demographics, surgery statistics, and hospital course. RESULTS: Six patients were identified with mean age of 14 years. Imaging studies showed cystic masses ranging 4-6 cm in five patients, and 20 cm in one patient. One patient presented with recurrent teratoma. Pathology revealed four benign teratomas, one benign cyst, and one serous cystadenoma. Average operating time was 75 min. All patients had an ovarian-preserving resection. Three patients had cyst spillage, including the one who presented with recurrence (this was the only patient with a subsequent recurrence). Hospital stay averaged 37 h. Narcotic use averaged 9.9 mg of morphine daily. All patients had excellent cosmetic results, and no postoperative complications. CONCLUSIONS: Ovarian cystic mass excision using the SIL approach carries a higher risk of tumor spillage. Although the incidence of malignancy is low, they cannot be conclusively excluded with our current preoperative evaluations. At this time, we recommend SIL resection only for simple cysts with low malignant potential; however, further experience with this procedure will likely improve the risk of tumor spillage in the future.


Assuntos
Cistadenoma Seroso/cirurgia , Laparoscopia/métodos , Cistos Ovarianos/cirurgia , Neoplasias Ovarianas/cirurgia , Teratoma/cirurgia , Adolescente , Criança , Feminino , Humanos , Tempo de Internação/estatística & dados numéricos , Resultado do Tratamento
12.
J Laparoendosc Adv Surg Tech A ; 23(2): 170-3, 2013 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-23327346

RESUMO

BACKGROUND: Placement of a primary gastrojejunal tube (GJT) can be technically challenging and often requires an open procedure to negotiate the tube past the duodenal sweep into the jejunum. The alternative approach is to first place a gastrostomy tube (GT), which is then changed to a GJT under endoscopic or fluoroscopic guidance after waiting 6-8 weeks to allow the stoma to mature. We report a case series of primary GJT placement using a combined laparoscopic-endoscopic approach. SUBJECTS AND METHODS: We retrospectively reviewed patients who underwent a combined laparoscopic-endoscopic primary GJT placement. Patients' demographics and relevant clinical information were analyzed. RESULTS: Six patients (4 male, 2 female) were identified. The median age at the time of operation was 30.2 months (range, 28 days-10 years). Five GJTs were successfully placed laparoscopically/endoscopically, and one procedure was converted to open. The mean operative time was 84 minutes (range, 63-102 minutes). Postoperative abdominal radiography confirmed post-pyloric tube position in all patients. Feedings were initiated on the first postoperative day. One intraoperative complication required conversion to an open procedure. No patients developed postoperative complications. CONCLUSIONS: Laparoscopic-endoscopic primary GJT placement is technically feasible and an excellent alternative in patients who require transpyloric feeding access.


Assuntos
Endoscopia Gastrointestinal , Nutrição Enteral , Intubação Gastrointestinal/métodos , Laparoscopia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Intubação Gastrointestinal/instrumentação , Jejuno , Masculino , Estudos Retrospectivos
13.
J Pediatr Surg ; 46(10): 1909-12, 2011 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22008326

RESUMO

PURPOSE: Our aim was to evaluate the outcomes of the single-incision laparoscopic (SIL) cholecystectomy compared with the standard 4-incision laparoscopic (SL) cholecystectomy. METHODS: A retrospective chart review of consecutive patients undergoing cholecystectomy using the SIL approach from January 2008 to September 2010 was performed. These patients were compared with a cohort who underwent an SL cholecystectomy from January 2007 to June 2009. Demographics, operative times, length of stay, blood loss, and intravenous narcotic use was obtained from the charts. A nonpaired Student's t test was used to determine statistical significance. RESULTS: We identified 40 patients in the SIL group and 68 in the SL group. Main diagnosis was cholelithiasis followed by gallstone pancreatitis and cholecystitis. The mean operative time for SIL cholecystectomies was 79.2 minutes vs 63 minutes in the SL group (P < .006). The average length of stay was 1.9 days in the SIL group vs 2.3 days in the SL group (P < .24). The mean intravenous narcotic use was 1 dose in the SIL group vs 2.9 doses in the SL group (P < .007). There were no intraoperative complications. At 1-month postoperative follow-up, all patients had satisfactory recovery. CONCLUSION: Single-incision laparoscopic cholecystectomy is a safe and feasible alternative to the standard laparoscopic approach in children, even in the setting of acute disease.


Assuntos
Colecistectomia Laparoscópica/métodos , Adolescente , Perda Sanguínea Cirúrgica , Criança , Colecistite/cirurgia , Colelitíase/cirurgia , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pancreatite/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
14.
J Laparoendosc Adv Surg Tech A ; 21(7): 647-9, 2011 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-21777064

RESUMO

INTRODUCTION: Laparoscopic adrenalectomy is now being recognized as the standard approach for adrenalectomy for benign lesions in adults. The published experience in children and adolescents has been limited to sporadic small case series. Therefore, we conducted a large multicenter review of children who have undergone laparoscopic adrenalectomy. METHODS: After Institutional Review Board's approval, a retrospective review was conducted on all patients who have undergone laparoscopic adrenalectomy at 12 institutions over the past 10 years. Operative times included unilateral adrenalectomy without concomitant procedures. RESULTS: About 140 patients were identified (70 males [50%]). Laterality included 76 (54.3%) left-sided lesions, 59 (42.1%) right, and 5 (3.6%) bilateral. Mean operative time was 130.2 ± 63.5 minutes (range 43-406 minutes). The most common pathology was neuroblastoma in 39 cases (27.9%), of which 23 (59.0%) had undergone preoperative chemotherapy. Other common pathology included 30 pheochromocytomas (21.4%), 22 ganglioneuromas (15.7%), and 20 adenomas (14.3%). There were 13 conversions to an open operation (9.9%). Most conversions were because of tumor adherence to surrounding organs, and tumor size was not different in converted cases (P=.97). A blood transfusion was required in 2 cases. The only postoperative complication was renal infarction after resection of a large neuroblastoma that required skeletonization of the renal vessels. At a median follow-up of 18 months, there was only one local recurrence, which was in a patient with a pheochromocytoma. CONCLUSIONS: The laparoscopic approach can be applied for adrenalectomy in children for a wide variety of conditions regardless of age with a 90% chance of completing the operation without conversion. The risk for significant blood loss or complications is low, and it should be considered the preferred approach for the majority of adrenal lesions in children.


Assuntos
Adrenalectomia/métodos , Laparoscopia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Adulto Jovem
15.
Pediatr Surg Int ; 27(7): 743-6, 2011 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-21336611

RESUMO

PURPOSE: We are reporting our experience so far with single incision laparoscopic cholecystectomy in children. METHODS: After the approval of the institutional review board, we performed a retrospective chart review of our single port cases from 01/2008 to 10/2009. We used operating room (OR) times, length of stay, as well as IV narcotic use as our outcome measures. Pertinent clinical data were extracted. The single port procedure was performed using a single infra-umbilical incision whereby three 5-mm ports were placed. RESULTS: We identified 25 patients in the single port group (20 females and 5 males). 23 patients in the study group underwent cholecystectomy without intra-operative cholangiogram and one patient had an intra-operative cholangiogram performed. This additional procedure did not add to the overall OR time significantly as compared to simple cholecystectomies. Average OR time was 97.5 min as compared to 71.4 min in the traditional 4-port group. Blood loss was reported as minimal for all cases in both the groups (5-25 ml). There were no intra-operative complications in either group. Mean length of stay was 1.47 days in the study group. All patients in the study group had minimal (1-3 doses) need for intravenous narcotics during their inpatient stay except for one patient, who required more. All patients in the study group had excellent cosmetic results on postoperative follow-up. CONCLUSION: Single incision laparoscopic cholecystectomy is safe and feasible to perform in pediatrics, even in the setting of acute disease.


Assuntos
Colecistectomia Laparoscópica/métodos , Doenças da Vesícula Biliar/cirurgia , Laparoscópios , Adolescente , Criança , Desenho de Equipamento , Feminino , Seguimentos , Humanos , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
16.
Am Surg ; 76(10): 1047-9, 2010 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-21105606

RESUMO

Laparoscopic cholecystectomy has become a standard procedure for treatment of gallbladder diseases. The operation is performed through a four-trocar technique. Single-incision laparoscopy (SIL) has recently gained popularity. The purpose of our study was to review our cases of SIL cholecystectomy and to evaluate the safety and feasibility of this technique. After the approval of the Institutional Review Boards, we performed a retrospective chart review of our SIL cholecystectomy cases performed between January 2008 and August 2009. Pertinent clinical data were extracted. The outcomes were reported as operating room time, intraoperative and postoperative complications, length of stay, and intravenous narcotic use. We identified 24 patients (19 females and five males) with a mean age of 15 years. Most patients (67%) had a diagnosis of symptomatic cholelithiasis. Two patients had gallstone pancreatitis, three had acute cholecystitis, and one had a hydropic gallbladder. Two patients had an intraoperative cholangiogram performed. Average operating room time was 97.5 +/- 34.5 minutes (range, 65 to 145 minutes). There were no intraoperative complications. All patients had minimal (one to three doses) need for intravenous narcotics. All patients have had excellent cosmetic results on postoperative follow-up. SIL cholecystectomy in children is safe and feasible, even in the setting of acute cholecystitis and the need for cholangiogram.


Assuntos
Colecistectomia Laparoscópica/métodos , Adolescente , Adulto , Colelitíase/cirurgia , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pancreatite/cirurgia , Estudos Retrospectivos , Adulto Jovem
17.
J Biol Chem ; 285(42): 32096-104, 2010 Oct 15.
Artigo em Inglês | MEDLINE | ID: mdl-20716526

RESUMO

Ectodomain shedding of transmembrane proteins may be regulated by their cytoplasmic domains. To date, the effecting cytoplasmic domain and the shed extracellular domain have been in the same polypeptide. In this study, shedding of GPIbα, the ligand-binding subunit of the platelet GPIb-IX complex and a marker for platelet senescence and storage lesion, was assessed in Chinese hamster ovary cells with/without functional GPIbα sheddase ADAM17. Mutagenesis of the GPIb-IX complex, which contains GPIbα, GPIbß, and GPIX subunits, revealed that the intracellular membrane-proximal calmodulin-binding region of GPIbß is critical for ADAM17-dependent shedding of GPIbα induced by the calmodulin inhibitor, W7. Perturbing the interaction between GPIbα and GPIbß subunits further lessened the restraint of GPIbß on GPIbα shedding. However, contrary to the widely accepted model of calmodulin regulation of ectodomain shedding, the R152E/L153E mutation in the GPIbß cytoplasmic domain disrupted calmodulin binding to GPIbß but had little effect on GPIbα shedding. Analysis of induction of GPIbα shedding by membrane-permeable GPIbß-derived peptides implicated the association of GPIbß with an unidentified intracellular protein in mediating regulation of GPIbα shedding. Overall, these results provide evidence for a novel trans-subunit mechanism for regulating ectodomain shedding.


Assuntos
Complexo Glicoproteico GPIb-IX de Plaquetas/química , Complexo Glicoproteico GPIb-IX de Plaquetas/metabolismo , Subunidades Proteicas/metabolismo , Proteínas ADAM/genética , Proteínas ADAM/metabolismo , Proteína ADAM17 , Sequência de Aminoácidos , Animais , Plaquetas/metabolismo , Células CHO , Calmodulina/genética , Calmodulina/metabolismo , Membrana Celular/química , Cricetinae , Cricetulus , Humanos , Mutagênese , Complexo Glicoproteico GPIb-IX de Plaquetas/genética , Estrutura Terciária de Proteína , Subunidades Proteicas/química , Subunidades Proteicas/genética
18.
Pediatr Surg Int ; 25(12): 1087-91, 2009 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-19707773

RESUMO

INTRODUCTION: Treatment of long-gap esophageal atresia (LEA) is a major challenge. Options for reconstruction include native esophagus, or replacement with stomach, colon, or small intestine. However, debate continues regarding the optimal conduit for esophageal replacement. METHODS: Medical records of patients with a diagnosis of esophageal atresia during a 20-year period were reviewed. RESULTS: Twenty-eight cases of LEA were identified. Ten patients underwent primary anastomosis either after serial pouch dilations (9/10) and/or after a lengthening procedure (2/10). Nine received colonic interpositions, and the remainder were reconstructed with a gastric tube (n = 3), or gastric interposition (n = 2). One patient died prior to repair, and two await definitive treatment. Repeat esophageal reconstruction was required in four patients because of conduit ischemia. Two ischemic events occurred in the colonic interposition group, and two in the native esophageal repairs. All patients, except one who relocated, received long-term follow-up (mean 4.2 years: range 0.5-11.5 years). CONCLUSIONS: Surgeon's expertise and patient's anatomy should be considered when selecting an appropriate operation for LEA. Although native esophagus is generally preferred, it is associated with a high rate of stricture. Although our study has a limited by numbers, we found that patients with gastric conduits had lower complication rates and no conduit ischemia. We suggest that gastric transposition may be favored as an initial reconstructive option.


Assuntos
Atresia Esofágica/cirurgia , Próteses e Implantes , Anastomose Cirúrgica , Colo/cirurgia , Feminino , Seguimentos , Humanos , Masculino , Complicações Pós-Operatórias , Estudos Retrospectivos , Estômago/cirurgia
19.
J Laparoendosc Adv Surg Tech A ; 19(1): 87-91, 2009 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-19196083

RESUMO

BACKGROUND: Choledochal cyst (CDC) is a rare biliary disorder. Surgical treatment consists of CDC excision and biliary-enteric reconstruction. Recently, some institutions have reported successful CDC excision by using minimally invasive techniques. In this study, we report our experience with the laparoscopic management of CDC, with a focus on key operative maneuvers that enhance the likelihood of successful excision. METHODS: Following institutional review board approval, we performed a retrospective review of patients who underwent the laparoscopic excision of CDC and Roux-en-Y hepaticojejunostomy. Between October 2003 and November 2007, we performed laparoscopic CDC excision in 9 patients (8 female and 1 male). Median age was 4 years (range, 8 months to 16 years). There were 7 type I and 2 type IV cysts, according to Todani's classification. Average cyst size was 4.4 cm (range, 1.3-8.5). The procedures were performed by utilizing four or five trochars. RESULTS: Six of 9 children presented with preoperative pancreatitis, 1 with abdominal pain, 1 with jaundice, and 1 was found incidentally. Three patients required the conversion to laparotomy due to dense adhesions, secondary to pancreatitis. Six patients underwent successful laparoscopic procedures, 5 had complete cyst excisions, and 1 underwent a proximal excision with distal mucosectomy. Of the 3 patients who required conversion, 2 underwent complete excisions; the other underwent a proximal excision, distal mucosectomy. There were no intraoperative complications. One patient had a postoperative bile leak that required an open hepaticojejunostomy revision. Eight patients had an uneventful recovery. Oral feedings were resumed within an average of 3.4 days (range, 2-9). Average time to discharge was 6.1 days (range, 5-12). Average follow-up time was 18 months (range, 4-48). No further laboratory abnormalities were detected in any of the patients. CONCLUSIONS: Laparoscopic resection of CDC and Roux-en-Y hepaticojejunostomy in children is an excellent treatment option. Preoperative pancreatitis may cause increased technical difficulty, necessitating a conversion. Proximal excision with distal mucosectomy


Assuntos
Cisto do Colédoco/cirurgia , Laparoscopia/métodos , Adolescente , Anastomose em-Y de Roux , Procedimentos Cirúrgicos do Sistema Biliar , Criança , Pré-Escolar , Coledocostomia , Feminino , Humanos , Lactente , Masculino , Complicações Pós-Operatórias , Estudos Retrospectivos , Resultado do Tratamento
20.
J Laparoendosc Adv Surg Tech A ; 19 Suppl 1: S197-200, 2009 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-19196095

RESUMO

Severe caustic burns to the esophagus may necessitate esophagectomy with replacement, if stictures form which are not amenable to dilation. Traditionally, the operation is performed via open transabdominal and/or thoracic approaches. Here we describe our minimally invasive approach, combining laparoscopy and thoracoscopy for esophagectomy, stomach tubularization and a cervical esophagogastrotomy. We successfully performed the procedure in a 4-year-old boy. There were no intraoperative complications, and his initial esophagram showed good patency with no leaks. However, one week postoperatively he was noted to have a retained foreign body and a minor anastomotic leak, which was most likely caused by the foreign body. He underwent a neck re-exploration,removal of the foreign body, and repair of the anastomotic leak. His subsequent hospital course was uneventful. He tolerated feedings and was discharged home on an unrestricted diet. The minimally invasive approach to esophagectomy and esophageal replacement is feasible and is an excellent option in select patients.


Assuntos
Esofagectomia/métodos , Esofagoplastia/métodos , Laparoscopia , Toracoscopia , Queimaduras Químicas/cirurgia , Pré-Escolar , Esôfago/lesões , Corpos Estranhos/cirurgia , Humanos , Masculino , Complicações Pós-Operatórias , Estômago/cirurgia
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