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1.
Pediatr Surg Int ; 39(1): 155, 2023 Mar 22.
Artigo em Inglês | MEDLINE | ID: mdl-36944730

RESUMO

PURPOSE: Thoracoscopic and open approaches for the management of congenital lung malformations (CLM) has been debated. The aim of this study is to compare 30-day outcomes for non-emergent lobectomies in children. METHODS: The National Surgical Quality Improvement Program-Pediatric database was queried for patients undergoing CLM resection from 2013 to 2020. Outcomes were compared by operative technique in an intention-to-treat model and then propensity matched. RESULTS: 2157 patients met inclusion criteria and underwent non-emergent pulmonary lobectomy for CLM. The intended operative approach was thoracoscopic in 57.7% of patients. Patients in the open group compared to the thoracoscopic were more likely to be born premature, have chronic lung disease, require preoperative oxygen support, and be ventilator dependent. After propensity matching, there was no statistically significant difference in 30-day mortality, unplanned readmission, and other complications between the thoracoscopic and open groups. Thoracoscopic approach was associated with a shorter length of stay. The proportion of cases approached via thoracoscopy increased over time from 48.8% in 2013 to 69.9% in 2020. CONCLUSIONS: This large multicenter retrospective matched analysis demonstrates thoracoscopic lobectomy in children has similar favorable 30-day outcomes and shorter length of stay for the non-emergent management of CLM, compared to open thoracotomy. LEVEL OF EVIDENCE: Level III.


Assuntos
Pneumopatias , Neoplasias Pulmonares , Anormalidades do Sistema Respiratório , Humanos , Criança , Estudos Retrospectivos , Pneumonectomia/métodos , Resultado do Tratamento , Pneumopatias/cirurgia , Pneumopatias/congênito , Pulmão/cirurgia , Pulmão/anormalidades , Toracotomia/métodos , Anormalidades do Sistema Respiratório/cirurgia , Tempo de Internação , Neoplasias Pulmonares/cirurgia , Cirurgia Torácica Vídeoassistida/métodos
2.
J Surg Res ; 249: 138-144, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-31954974

RESUMO

BACKGROUND: Trauma is the leading cause of death in pediatric patients over 1 y of age. Controversy exists regarding prehospital airway management for these patients, with some studies suggesting that endotracheal intubation in the field or at a referring hospital is associated with increased mortality and complication rate. These studies were largely performed at urban centers, and it is unclear whether the results apply to suburban/rural networks with longer transport times and more stops at referring hospitals. The purpose of this study is to evaluate differential outcomes in pediatric trauma patients who underwent endotracheal intubation at the scene of injury, referring hospital, or pediatric trauma center in a predominantly rural/suburban setting. MATERIALS AND METHODS: A retrospective review was performed evaluating trauma patients age 18 y or younger at a single institution over 10 y (2004-2014). Patients were selected who underwent endotracheal intubation and were classified based on location of intubation (scene, referring hospital, or trauma center). Fischer's exact test and t-tests were performed for comparison. Univariate and multivariate regression analyses were performed. RESULTS: 288 patients were identified. 155 (53.8%) were intubated at the scene of injury, 55 (19.1%) at a referring hospital, and 72 (25%) at the trauma center. Overall mortality was 21.9%, which was highest in the scene intubation group (29.7%) compared with the referring hospital (20%) and trauma center (5.6%) groups (P < 0.01). Patients intubated at the scene had higher Injury Severity Scores and lower Glasgow Coma Scale scores (P < 0.01). Duration of intubation was lowest in the trauma center group (P < 0.01). Complication rate was highest in the referring hospital group (P < 0.05). Multivariate analysis revealed that age, injury severity, and neurologic status were the key drivers of mortality rather than location of intubation. CONCLUSIONS: Mortality and duration of intubation were lowest in trauma patients intubated at a pediatric trauma center. However, location of intubation was not a significant independent predictor of mortality or complications on multivariate analysis, suggesting that age, injury severity, and neurologic status are the main indicators of prognosis in severe pediatric trauma.


Assuntos
Intubação Intratraqueal/estatística & dados numéricos , Serviços de Saúde Rural/estatística & dados numéricos , Serviços de Saúde Suburbana/estatística & dados numéricos , Transporte de Pacientes/estatística & dados numéricos , Centros de Traumatologia/estatística & dados numéricos , Ferimentos e Lesões/terapia , Adolescente , Criança , Pré-Escolar , Feminino , Escala de Coma de Glasgow , Pneumonia Associada a Assistência à Saúde/epidemiologia , Pneumonia Associada a Assistência à Saúde/etiologia , Humanos , Lactente , Recém-Nascido , Escala de Gravidade do Ferimento , Intubação Intratraqueal/efeitos adversos , Tempo de Internação/estatística & dados numéricos , Masculino , Estudos Retrospectivos , Fatores de Tempo , Estenose Traqueal/epidemiologia , Estenose Traqueal/etiologia , Resultado do Tratamento , Ferimentos e Lesões/diagnóstico , Ferimentos e Lesões/mortalidade
3.
J Surg Res ; 245: 217-224, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31421366

RESUMO

BACKGROUND: Gastroschisis is an increasingly common congenital abdominal wall defect. Due to advances in neonatal critical care and early surgical management, mortality from gastroschisis and associated complications has decreased to less than 10% in most series. However, it has been recognized that the outcome of gastroschisis has a spectrum and that the disorder affects a heterogeneous cohort of neonates. The goal of this study is to predict morbidity and mortality in neonates with gastroschisis using clinically relevant variables. METHODS: A multicenter, retrospective observational study of neonates born with gastroschisis was conducted. Neonatal characteristics and outcomes were collected and compared. Prediction of morbidity and mortality was performed using multivariate clinical models. RESULTS: Five hundred and sixty-six neonates with gastroschisis were identified. Overall survival was 95%. Median hospital length of stay was 37 d. Sepsis was diagnosed in 107 neonates. Days on parenteral nutrition and mechanical ventilation were considerable with a median of 27 and 5 d, respectively. Complex gastroschisis (atresia, perforation, volvulus), preterm delivery (<37 wk), and very low birth weight (<1500 g) were associated with worse clinical outcomes including increased sepsis, short bowel syndrome, parenteral nutrition days, and length of stay. The composite metric of birth weight, Apgar score at 5 min, and complex gastroschisis was able to successfully predict mortality (area under the curve, 0.81). CONCLUSIONS: Clinical variables can be used in gastroschisis to distinguish those who will survive from nonsurvivors. Although these findings need to be validated in other large multicenter data sets, this prognostic score may aid practitioners in the identification and management of at-risk neonates.


Assuntos
Gastrosquise/mortalidade , Sepse/epidemiologia , Síndrome do Intestino Curto/epidemiologia , Índice de Apgar , Estudos de Viabilidade , Feminino , Gastrosquise/complicações , Gastrosquise/terapia , Idade Gestacional , Humanos , Recém-Nascido de Baixo Peso , Recém-Nascido , Recém-Nascido Prematuro , Tempo de Internação/estatística & dados numéricos , Masculino , Nutrição Parenteral/estatística & dados numéricos , Prognóstico , Respiração Artificial/estatística & dados numéricos , Estudos Retrospectivos , Medição de Risco/métodos , Fatores de Risco , Sepse/etiologia , Síndrome do Intestino Curto/etiologia , Taxa de Sobrevida
4.
Crit Care Med ; 47(4): 566-573, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30664526

RESUMO

OBJECTIVES: This study sought to examine mortality, health-related quality of life, and physical function among sepsis survivors who developed chronic critical illness. DESIGN: Single-institution, prospective, longitudinal, observational cohort study assessing 12-month outcomes. SETTING: Two surgical/trauma ICUs at an academic tertiary medical and level 1 trauma center. PATIENTS: Adult critically ill patients that survived 14 days or longer after sepsis onset. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Baseline patient characteristics and function, sepsis severity, and clinical outcomes of the index hospitalization were collected. Follow-up physical function (short physical performance battery; Zubrod; hand grip strength) and health-related quality of life (EuroQol-5D-3L, Short Form-36) were measured at 3, 6, and 12 months. Hospital-free days and mortality were determined at 12 months. We compared differences in long-term outcomes between subjects who developed chronic critical illness (≥ 14 ICU days with persistent organ dysfunction) versus those with rapid recovery. The cohort consisted of 173 sepsis patients; 63 (36%) developed chronic critical illness and 110 (64%) exhibited rapid recovery. Baseline physical function and health-related quality of life did not differ between groups. Those who developed chronic critical illness had significantly fewer hospital-free days (196 ± 148 vs 321 ± 65; p < 0.0001) and reduced survival at 12-months compared with rapid recovery subjects (54% vs 92%; p < 0.0001). At 3- and 6-month follow-up, chronic critical illness patients had significantly lower physical function (3 mo: short physical performance battery, Zubrod, and hand grip; 6 mo: short physical performance battery, Zubrod) and health-related quality of life (3- and 6-mo: EuroQol-5D-3L) compared with patients who rapidly recovered. By 12-month follow-up, chronic critical illness patients had significantly lower physical function and health-related quality of life on all measures. CONCLUSIONS: Surgical patients who develop chronic critical illness after sepsis exhibit high healthcare resource utilization and ultimately suffer dismal long-term clinical, functional, and health-related quality of life outcomes. Further understanding of the mechanisms driving the development and persistence of chronic critical illness will be necessary to improve long-term outcomes after sepsis.


Assuntos
Estado Terminal/epidemiologia , Indicadores Básicos de Saúde , Qualidade de Vida , Sepse/epidemiologia , Sobreviventes/estatística & dados numéricos , Adulto , Idoso , Estudos de Coortes , Estado Terminal/terapia , Feminino , Nível de Saúde , Humanos , Unidades de Terapia Intensiva , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Sepse/psicologia , Sepse/terapia , Sobreviventes/psicologia
5.
Biochim Biophys Acta Mol Basis Dis ; 1863(10 Pt B): 2564-2573, 2017 10.
Artigo em Inglês | MEDLINE | ID: mdl-28115287

RESUMO

Early host recognition of microbial invasion or damaged host tissues provides an effective warning system by which protective immune and inflammatory processes are initiated. Host tissues responsible for continuous sampling of their local environment employ cell surface and cytosolic pattern recognition receptors (PRRs) that provide redundant and overlapping identification of both microbial and host alarmins. Microbial products containing pathogen-associated molecular patterns (PAMPs), as well as damage-associated molecular patterns (DAMPs) serve as principle ligands for recognition by these PRRs. It is this interaction which plays both an essential survival role in response to infection and injury, as well as the pathologic role in tissue and organ injury associated with severe sepsis and trauma. Elucidating the interaction between ligands and their respective PRRs can provide both a better understanding of the host response, as well as a rational basis for therapeutic intervention. This article is part of a Special Issue entitled: Immune and Metabolic Alterations in Trauma and Sepsis edited by Dr. Raghavan Raju.


Assuntos
Receptores de Reconhecimento de Padrão/imunologia , Sepse/imunologia , Transdução de Sinais/imunologia , Ferimentos e Lesões/imunologia , Animais , Humanos , Receptores de Reconhecimento de Padrão/metabolismo , Sepse/metabolismo , Sepse/mortalidade , Sepse/patologia , Ferimentos e Lesões/metabolismo , Ferimentos e Lesões/mortalidade , Ferimentos e Lesões/patologia
6.
Crit Care Med ; 45(12): 1989-1996, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28837430

RESUMO

OBJECTIVE: To determine the incidence and risk factors of chronic critical illness after severe blunt trauma. DESIGN: Prospective observational cohort study (NCT01810328). SETTING: Two level-one trauma centers in the United States. PATIENTS: One hundred thirty-five adult blunt trauma patients with hemorrhagic shock who survived beyond 48 hours after injury. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Chronic critical illness was defined as an ICU stay lasting 14 days or more with evidence of persistent organ dysfunction. Three subjects (2%) died within the first 7 days, 107 (79%) exhibited rapid recovery and 25 (19%) progressed to chronic critical illness. Patients who developed chronic critical illness were older (55 vs 44-year-old; p = 0.01), had more severe shock (base deficit, -9.2 vs -5.5; p = 0.005), greater organ failure severity (Denver multiple organ failure score, 3.5 ± 2.4 vs 0.8 ± 1.1; p < 0.0001) and developed more infectious complications (84% vs 35%; p < 0.0001). Chronic critical illness patients were more likely to be discharged to a long-term care setting (56% vs 34%; p = 0.008) than to a rehabilitation facility/home. At 4 months, chronic critical illness patients had higher mortality (16.0% vs 1.9%; p < 0.05), with survivors scoring lower in general health measures (p < 0.005). Multivariate analysis revealed age greater than or equal to 55 years, systolic hypotension less than or equal to 70 mm Hg, transfusion greater than or equal to 5 units packed red blood cells within 24 hours, and Denver multiple organ failure score at 72 hours as independent predictors of chronic critical illness (area under the receiver operating curve, 0.87; 95% CI, 0.75-0.95). CONCLUSIONS: Although early mortality is low after severe trauma, chronic critical illness is a common trajectory in survivors and is associated with poor long-term outcomes. Advancing age, shock severity, and persistent organ dysfunction are predictive of chronic critical illness. Early identification may facilitate targeted interventions to change the trajectory of this morbid phenotype.


Assuntos
Doença Crônica/epidemiologia , Estado Terminal/epidemiologia , Unidades de Terapia Intensiva/estatística & dados numéricos , Centros de Traumatologia/estatística & dados numéricos , Ferimentos não Penetrantes/epidemiologia , Adulto , Fatores Etários , Idoso , Transfusão de Sangue/estatística & dados numéricos , Doença Crônica/mortalidade , Estado Terminal/mortalidade , Infecção Hospitalar/epidemiologia , Feminino , Humanos , Hipotensão/epidemiologia , Escala de Gravidade do Ferimento , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Insuficiência de Múltiplos Órgãos/epidemiologia , Alta do Paciente , Prognóstico , Estudos Prospectivos , Fatores de Risco , Índice de Gravidade de Doença , Choque Hemorrágico/epidemiologia , Ferimentos não Penetrantes/mortalidade
8.
Surg Open Sci ; 19: 158-161, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38745564

RESUMO

Objective: A pediatric surgery fellow is often regarded as a cornerstone of an academic children's hospital due to the need for their clinical services with overnight coverage being an important aspect of the care provided. There is little known about the objective sleep patterns and work-related communications of a pediatric surgery fellow during overnight home call. The aim of this study is to better understand the sleep patterns and interruptions of an on-call pediatric surgery fellow. Design: A prospective observational study of 60 call nights and 60 non-call nights of a pediatric surgery senior fellow was performed from September 2022 to February 2023. Setting: An academic Children's Hospital. Participant: An ACGME-accredited clinical pediatric surgery fellow. Results: On average, the pediatric surgery fellow spent 6.9 and 5.8 total hours in bed and asleep each night, respectively. The total sleep time was less for call nights compared to non-call nights (5.4 versus 6.3 h, p < 0.0001). The mean number of work-related communications per 12-hour night shift was four. The majority of communications were regarding new consults (63.8 %). The pediatric surgery fellow spent an average of 5.9 min per communication and approximately 23.8 min total during each 12-hour night shift. Approximately half of these communications occurred during sleep hours. Conclusions: This study reveals overall sleep duration was below recommended levels. There were significant alterations in sleep patterns during call nights. Work-related communications further compounded sleep disturbances. Further research and interventions in this area are warranted.

9.
Am J Case Rep ; 25: e942203, 2024 Feb 27.
Artigo em Inglês | MEDLINE | ID: mdl-38412145

RESUMO

BACKGROUND Cloaca malformations result from a disproportion of apoptosis, cell growth, and maturation. The range of cloacal malformations is extensive and diverse, with a lack of a straightforward classification system. Cloacal dysgenesis sequence (CDS), also known as urorectal septum malformation sequence, is a rare cloaca variant described as the absence of a perineal orifice. Prenatal magnetic resonance imaging and antenatal ultrasounds may reveal a cloacal malformation; however, many patients are not diagnosed with cloacal malformation until birth. CASE REPORT We present a case of a female neonate delivered by a 23-year-old G2P1T1A0L0 mother who had received comprehensive prenatal care. During pregnancy, bilateral multicystic dysplastic kidneys were identified prenatally, leading to the in utero placement of a vesicoamniotic shunt. The physical exam revealed a distended abdomen with reduced abdominal musculature and laxity, ascites, a vesicoamniotic shunt in place, absent urethra, ambiguous genitalia with no vaginal opening, no perineal opening, and clubfoot. Abdominal radiograph showed findings consistent with significant abdominal ascites. An exploratory laparotomy was performed that included diverting colostomy, mucous fistula creation, tube vaginostomy, removal of the vesicoamniotic shunt, and suprapubic tube placement. The patient recovered well from this operation with no complications. CONCLUSIONS CDS is an uncommon condition in pediatric patients, and although sonographic findings can reveal urinary tract abnormalities, prenatal imaging might not always identify CDS. Our case underscores the uniqueness of the case and the significance of early detection and immediate medical and surgical intervention.


Assuntos
Anormalidades Múltiplas , Ascite , Recém-Nascido , Animais , Feminino , Gravidez , Humanos , Criança , Adulto Jovem , Adulto , Cloaca/diagnóstico por imagem , Cloaca/cirurgia , Cloaca/anormalidades , Ultrassonografia Pré-Natal/métodos , Anormalidades Múltiplas/diagnóstico por imagem , Anormalidades Múltiplas/cirurgia , Vagina/diagnóstico por imagem , Vagina/cirurgia , Vagina/anormalidades
10.
Am J Case Rep ; 24: e938249, 2023 Jan 17.
Artigo em Inglês | MEDLINE | ID: mdl-36647328

RESUMO

BACKGROUND Juvenile-type granulosa cell tumors (JGCTs) are a rare subtype of sex cord stromal tumor with a characteristic histology that is commonly found in the first 3 decades of life. It most commonly presents with symptoms of hyperestrogenism, which may present as precocious pseudopuberty or as menstruation-related symptoms, allowing for early detection of the tumor. CASE REPORT We present the case of a 12-year-old girl who presented to her primary care provider (PCP) with secondary amenorrhea with intermittent abdominal pain, who underwent an ultrasound for further evaluation, which revealed a large incidental pelvic mass. She was admitted to the Emergency Department (ED) and had findings of galactorrhea and hyperprolactinemia on examination. Imaging studies demonstrated a large ovarian mass measuring 15.0×9.0×18.8 cm that was resected, and subsequent pathology results showed JGCT stage 1A. CONCLUSIONS Prognosis of granulosa cell tumors (GCT) largely depends on its initial size, stage at diagnosis, residual tumors after surgery, and the subtype of GCT. If the patient is of reproductive age, fertility-sparing surgical options must be considered and patients must be regularly monitored for recurrence. JGCTs can present with minimal to no symptoms of precocious puberty in young girls but may present with amenorrhea, which may be considered normal for their developmental age. Although JGCTs are rare, they are important to include in differential diagnoses of younger female patients with abdominal pain, especially if accompanied by hormonal irregularities.


Assuntos
Galactorreia , Tumor de Células da Granulosa , Hiperprolactinemia , Neoplasias Ovarianas , Feminino , Humanos , Adolescente , Gravidez , Criança , Tumor de Células da Granulosa/complicações , Tumor de Células da Granulosa/diagnóstico , Tumor de Células da Granulosa/cirurgia , Neoplasias Ovarianas/complicações , Neoplasias Ovarianas/diagnóstico , Neoplasias Ovarianas/patologia , Amenorreia/complicações , Hiperprolactinemia/complicações , Dor Abdominal/etiologia
11.
Semin Pediatr Surg ; 32(3): 151313, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37276781

RESUMO

Unfortunately, we are all too familiar with the statement: "Necrotizing enterocolitis remains the leading cause of gastrointestinal surgical emergency in preterm neonates". It's been five decades since the first animal models of necrotizing enterocolitis (NEC) were described. There remains much investigative work to be done on identifying various aspects of NEC, ranging from the underlying mechanisms to treatment modalities. Experimental NEC is mainly focused on a rat, mouse, and piglet models. Our aim is to not only highlight the pros and cons of these three main models, but to also present some of the less-used animal models that have contributed to the body of knowledge about NEC. Choosing an appropriate model is essential to conducting effective research and answering the questions asked. As such, this paper reviews some of the variations that come with each model.


Assuntos
Enterocolite Necrosante , Animais , Camundongos , Ratos , Modelos Animais de Doenças , Enterocolite Necrosante/diagnóstico , Enterocolite Necrosante/etiologia , Enterocolite Necrosante/terapia , Suínos
12.
J Surg Case Rep ; 2023(5): rjad233, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-37192875

RESUMO

Cardiac tamponade is a known life-threatening complication of extracorporeal membrane oxygenation (ECMO), often secondary to hemopericardium from major vascular or cardiac perforation. We present the unique case of a neonate with a milky pericardial effusion causing tamponade after ECMO cannulation, managed successfully with pericardial window. Understanding ECMO physiology and its effect on the classic presentation of tamponade is critical and can prevent delays in diagnosis. While hemopericardium is most commonly seen in these cases, findings of a non-bloody, milky effusion should prompt further workup for infection, chylopericardium or total parenteral nutrition-associated pericardial effusion, as the appropriate management can mitigate immediate and potential long-term sequelae.

13.
J Robot Surg ; 17(5): 2427-2433, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37452975

RESUMO

Robotic-assisted surgery (RAS) has a variety of theoretical advantages, including tremor filtration, optimal visualization, and improvement of surgeon ergonomics. Though it has achieved wide application in pediatric urology, the majority of pediatric general surgeons do not employ RAS. This study reports our institution's experience with RAS on a pediatric general surgery team. Following IRB approval, a retrospective review of all pediatric patients at our academic children's hospital who underwent RAS between 2017 and 2022 for pediatric general surgical conditions was performed. Patient demographics, operation performed, operating time, complications, and recovery were evaluated. A total of 159 children underwent RAS, increasing from 10 patients in 2017 to 59 patients in 2022. The median age and weight were 15.3 years and 76.4 kg, and 121 (76.1%) were female. The application of RAS was successful in all cases. There were no intraoperative complications and no conversions to an open approach. Eleven patients (6.9%) had unplanned presentations to the emergency department within 30 days. Five of these patients (3.1%) required admission to the hospital. This study demonstrates that the application of RAS in an academic pediatric general surgery practice is feasible and safe. The application of RAS to pediatric general surgery should continue to increase as operative teams increase their experience and comfort.Level of evidence Level IV.


Assuntos
Laparoscopia , Procedimentos Cirúrgicos Robóticos , Robótica , Cirurgiões , Humanos , Criança , Feminino , Masculino , Procedimentos Cirúrgicos Robóticos/métodos , Estudos Retrospectivos , Complicações Intraoperatórias
14.
Am J Case Rep ; 24: e938248, 2023 Mar 24.
Artigo em Inglês | MEDLINE | ID: mdl-36959724

RESUMO

BACKGROUND Lowe syndrome, also known as oculocerebrorenal syndrome, is a rare genetic condition caused by an X-linked mutation of the OCRL1 gene, with an estimated prevalence in the general population of approximately 1 in 500 000. It is a multisystem disorder most commonly affecting the eyes, central nervous system, and kidneys. These commonly manifest as congenital cataracts, intellectual disability, and proximal renal dysfunction (Fanconi-type). Epidermal lesions are an uncommon manifestation of this condition, and the association is not completely understood. CASE REPORT Here we present a case of a 9-year-old boy with Lowe syndrome who presented with multiple cystic masses found in the perianal region. An excision was then performed to remove the masses and found that the lesions were epidermal cysts, which are infrequently found in Lowe syndrome. After excision, the patient recovered uneventfully without complications. CONCLUSIONS While epidermal cysts are an uncommon manifestation that have been documented, our case remains unique given the location and associated symptoms of the lesions. At presentation, the constellation of pain and perianal masses was concerning for a malignant etiology. However, after diagnostic imaging was performed, these lesions were found to be epidermal cysts, an infrequent manifestation of Lowe syndrome. Few previous case reports described cystic lesions in association with Lowe syndrome, and none, to our knowledge, have described multiple symptomatic perianal lesions. This case is important to consider because epidermal cystic lesions can be found with this presentation and should be considered on differential diagnoses for dermatologic findings in Lowe syndrome patients.


Assuntos
Cisto Epidérmico , Deficiência Intelectual , Síndrome Oculocerebrorrenal , Síndrome WAGR , Masculino , Humanos , Criança , Síndrome Oculocerebrorrenal/complicações , Síndrome Oculocerebrorrenal/diagnóstico , Síndrome Oculocerebrorrenal/genética , Cisto Epidérmico/complicações , Cisto Epidérmico/diagnóstico , Cisto Epidérmico/cirurgia , Mutação , Deficiência Intelectual/complicações , Síndrome WAGR/complicações
15.
J Pediatr Surg ; 58(6): 1133-1138, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-36914464

RESUMO

PURPOSE: This study describes the job market from the perspective of recent pediatric surgery graduates. METHODS: An anonymous survey was circulated to the 137 pediatric surgeons who graduated from fellowships 2019-2021. RESULTS: The survey response rate was 49%. The majority of respondents were women (52%), Caucasian (72%), and had a median student debt burden of $225,000. Considering job opportunities, respondents strongly emphasized camaraderie (93%), mentorship (93%), case mix (85%), geography (67%), faculty reputation (62%), spousal employment (57%), compensation (51%), and call frequency (45%). 30% were satisfied with the employment opportunities available, and 21% felt strongly prepared to negotiate for their first job. All respondents were able to secure a job. Most jobs were university-based (70%) or hospital employed (18%) positions where surgeons covered median of two hospitals. 49% wanted protected research time, and 12% of respondents were able to secure substantial, protected research time. The median compensation for university-based jobs was $12,583 below the median AAMC benchmark for assistant professors for the corresponding year of graduation. CONCLUSION: These data highlight the ongoing need for assessment of the pediatric surgery workforce and for professional societies and training programs to further assist graduating fellows in preparing to negotiate their first job. TYPE OF STUDY: Survey LEVEL OF EVIDENCE: Level V.


Assuntos
Internato e Residência , Especialidades Cirúrgicas , Criança , Humanos , Masculino , Feminino , Emprego , Bolsas de Estudo , Inquéritos e Questionários , Hospitais
16.
Semin Pediatr Surg ; 31(6): 151230, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36446303

RESUMO

Abdominal wall defects are one of the most frequently encountered human congenital anomalies. They are seen in as many as 1 in 2,000 live births with evidence to suggest that their incidence is increasing. While often discussed together abdominal wall defects consist mainly of two entities namely gastroschisis and omphalocele. There are marked differences in their theories of embryo-pathogenesis, clinical presentation/anatomy and overall outcomes. There is no clear consensus explaining the precise embryological mechanisms leading to the development of abdominal wall defects. Many clinicians and embryologists have attempted to explain the genesis of congenital abdominal wall defects because of failure of progression of various phases of normal embryonic development. This review summarizes the mechanisms involved in normal and abnormal development of the ventral abdominal wall leading to the development of gastroschisis and omphalocele.


Assuntos
Parede Abdominal , Gastrosquise , Hérnia Umbilical , Gravidez , Feminino , Humanos , Gastrosquise/diagnóstico , Gastrosquise/complicações , Parede Abdominal/anormalidades
17.
Front Surg ; 8: 613605, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33718427

RESUMO

Purpose: Numerous definitive surgical techniques exist for the treatment of pilonidal disease with varied recurrence rates and wound complications. Due to the wide array of techniques and lack of consensus on the best approach, we proposed to study our experience treating pilonidal disease in adolescents and young adults. Methods: A retrospective analysis was conducted of patients 10-24 years old treated at a tertiary medical center from 2011 to 2016. Data including demographics, management, and outcomes were collected and analyzed. Primary outcome was recurrence of disease. Results: One hundred and thirty three patients with pilonidal disease underwent operative management. Fifty one percent underwent primary closure and 49% healed by secondary intention with no significant difference in recurrence rates (primary 18%, secondary 11%; p = 0.3245). Secondary healing patients had significantly lower wound complication rates (primary 51%, secondary 23%; p = 0.0012). After accounting for sex, race, weight, and operative technique, age was predictive of disease recurrence with an adjusted odds ratio (OR) of 0.706 (0.560-0.888; p = 0.003). Age and sex were both predictive of wound complications. Older patients had decreased risk of wound complication (adjusted OR 0.806, 95% CI 0.684-0.951; p = 0.0105), and male patients had increased risk of wound complication (adjusted OR 2.902, 95% CI 1.001-8.409; p = 0.0497). Conclusion: In summary, there is no significant difference in the recurrence rates between operative techniques for pilonidal disease. Older patients have decreased risk of recurrence following intervention. Wound complication rates are lower in patients undergoing secondary healing, though this may be better explained by differences in age and sex. Additional research investigating newer, minimally-invasive techniques needs to be pursued.

18.
J Trauma Acute Care Surg ; 90(2): 257-267, 2021 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-33214489

RESUMO

BACKGROUND: The genomic/cytokine "storm" after severe trauma is well described. However, the differing composition, magnitude and resolution of this response, and its relationship to clinical outcomes remain unclear. METHODS: This is a secondary analysis of a prospective longitudinal cohort study of severely injured trauma patients in hemorrhagic shock. Peripheral blood sampling was performed at 0.5, 1, 4, 7, 14, and 28 days after injury for measurement of circulating immune biomarkers. K-means clustering using overall mean and trajectory slope of selected immunologic biomarkers were used to identify distinct temporal immunologic endotypes. Endotypes were compared with known clinical trajectories defined as early death (<14 days), chronic critical illness (CCI) (ICU length of stay of ≥14 days with persistent organ dysfunction), and rapid recovery (RAP) (ICU length of stay of <14 days with organ recovery). RESULTS: The cohort included 102 subjects enrolled across 2 level 1 trauma centers. We identified three distinct immunologic endotypes (iA, iB, and iC), each with unique associations to clinical trajectory. Endotype iA (n = 47) exhibited a moderate initial proinflammatory response followed by a return to immunologic homeostasis, with a primary clinical trajectory of RAP (n = 44, 93.6%). Endotype iB (n = 44) exhibited an early hyperinflammatory response with persistent inflammation and immunosuppression, with the highest incidence of CCI (n = 10, 22.7%). Endotype iC (n = 11) exhibited a similar hyperinflammatory response, but with rapid return to immunologic homeostasis and a predominant trajectory of RAP (n = 9, 81.8%). Patients with endotype iB had the highest severity/duration of organ dysfunction and highest incidence of nosocomial infections (50%, p = 0.001), and endotype iB was the predominant endotype of patients who developed CCI (10 of 13 patients, 76.9%; p = 0.002). CONCLUSION: We identified three distinct immunologic endotypes after severe injury differing the magnitude and duration of the early response. The clinical trajectory of CCI is characterized by an endotype (iB) defined by persistent alteration in inflammation/immunosuppression and is associated with poor clinical outcomes. LEVEL OF EVIDENCE: Prognostic, level III.


Assuntos
Síndrome da Liberação de Citocina/imunologia , Endofenótipos , Choque Hemorrágico/imunologia , Ferimentos não Penetrantes/imunologia , Biomarcadores/sangue , Estudos de Coortes , Infecção Hospitalar/imunologia , Infecção Hospitalar/terapia , Síndrome da Liberação de Citocina/terapia , Seguimentos , Humanos , Terapia de Imunossupressão , Tempo de Internação , Estudos Longitudinais , Estudos Prospectivos , Choque Hemorrágico/terapia , Síndrome de Resposta Inflamatória Sistêmica/imunologia , Síndrome de Resposta Inflamatória Sistêmica/terapia , Centros de Traumatologia , Resultado do Tratamento , Ferimentos não Penetrantes/terapia
19.
Artigo em Inglês | MEDLINE | ID: mdl-20460167

RESUMO

Ion regulation is a biological process crucial to the survival of mosquito larvae and a major organ responsible for this regulation is the rectum. The recta of anopheline larvae are distinct from other subfamilies of mosquitoes in several ways, yet have not yet been characterized extensively. Here we characterize the two major cell types of the anopheline rectum, DAR and non-DAR cells, using histological, physiological, and pharmacological analyses. Proton flux was measured at the basal membrane of 2%- and 50%-artificial sea water-reared An. albimanus larvae using self-referencing ion-selective microelectrodes, and the two cell types were found to differ in basal membrane proton flux. Additionally, differences in the response of that flux to pharmacological inhibitors in larvae reared in 2% versus 50% ASW indicate changes in protein function between the two rearing conditions. Finally, histological analyses suggest that the non-DAR cells are structurally suited for mediating ion transport. These data support a model of rectal ion regulation in which the non-DAR cells have a resorptive function in freshwater-reared larvae and a secretive function in saline water-reared larvae. In this way, anopheline larvae may adapt to varying salinities.


Assuntos
Anopheles/fisiologia , Proteínas de Insetos/metabolismo , Reto/fisiologia , Salinidade , Adaptação Psicológica/efeitos dos fármacos , Adaptação Psicológica/fisiologia , Animais , Anopheles/anatomia & histologia , Anopheles/citologia , Anopheles/efeitos dos fármacos , Membrana Celular/efeitos dos fármacos , Membrana Celular/metabolismo , Água Doce , Proteínas de Insetos/análise , Transporte de Íons/efeitos dos fármacos , Larva/anatomia & histologia , Larva/citologia , Larva/efeitos dos fármacos , Larva/fisiologia , Proteínas de Membrana/análise , Proteínas de Membrana/metabolismo , Microeletrodos , Bombas de Próton/efeitos dos fármacos , Bombas de Próton/metabolismo , Prótons , Reto/citologia , Reto/efeitos dos fármacos , Reto/metabolismo , Água do Mar , Cloreto de Sódio/farmacologia
20.
J Pediatr Surg ; 55(7): 1280-1285, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31472924

RESUMO

BACKGROUND/PURPOSE: The optimal method to repair gastroschisis defects continues to be debated. The two primary methods are immediate closure (IC) or silo placement (SP). The purpose of this study was to compare outcomes between each approach using a multicenter retrospective analysis. We hypothesized that patients undergoing SP for ≤5 days would have largely equivalent outcomes compared to IC patients. METHODS: Gastroschisis patient data were collected over a 7-year period. The cohort was separated into IC and SP groups. The SP group was further stratified based on time to closure (≤5 days, 6-10 days, >10 days). Characteristics and outcomes were compared between groups. Multivariate logistic regression was also performed. RESULTS: 566 neonates with gastroschisis were identified including 224 patients in the IC group and 337 patients in the SP group. Among SP patients, 130 were closed within 5 days, 140 in 6-10 days, and 57 in >10 days. There were no significant differences in mortality, sepsis, readmission, or days to full enteral feeds between IC patients and SP patients who had a silo ≤5 days. IC patients had a significantly higher incidence of ventral hernias. Multivariate analysis revealed time to closure as a significant independent predictor of length of stay, ventilator duration, time to full enteral feeds, and TPN duration. CONCLUSIONS: Our data show largely equivalent outcomes between patients who undergo immediate closure and those who have silos ≤5 days. We propose that closure within 5 days avoids many of the risks commonly attributed to delay in closure. LEVEL OF EVIDENCE: Level II retrospective study.


Assuntos
Procedimentos Cirúrgicos do Sistema Digestório/métodos , Gastrosquise/cirurgia , Feminino , Seguimentos , Gastrosquise/mortalidade , Humanos , Lactente , Recém-Nascido , Masculino , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Resultado do Tratamento
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