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1.
Pediatr Radiol ; 2024 Jun 22.
Artigo em Inglês | MEDLINE | ID: mdl-38907841

RESUMO

We describe a case of anaphylaxis during administration of intravenous (IV) ferumoxytol as a magnetic resonance imaging (MRI) contrast agent in a 4-year-old patient with complicated past medical history including YARS genetic mutation with resultant liver failure and deceased donor liver transplantation, stage IV chronic kidney disease (CKD), and hypertension. The patient was noted to have labored breathing 4 min after initiation of ferumoxytol infusion and was subsequently rapidly intubated and returned to the intensive care unit (ICU) for monitoring. Anaphylactic reactions to therapeutic doses of ferumoxytol led to issuance of a black box warning by the FDA in 2015. Adverse reactions to lower-dose ferumoxytol used in diagnostic imaging, however, are rare and there has been a paucity of documented anaphylactic reactions in the literature.

2.
Pediatr Radiol ; 52(7): 1207-1223, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35166890

RESUMO

The fetal circulation is characterized by the presence of three physiological vascular shunts - the ductus arteriosus, the foramen ovale and the ductus venosus. Acting in concert, these shunts preferentially stream blood flow in a pattern that maximizes efficiency of blood oxygenation by the maternofetal unit. Shortly following the transition to extrauterine life, a quick and predetermined succession of events results in closure of these embryological structures with consequent establishment of postnatal vascular flow patterns. While this transition is often seamless, the physiological shunts of the fetus occasionally fail to regress. Such failure to regress can occur in isolation or in association with other congenital malformations. This failed regression challenges the circulatory physiology of the neonate and might have implications for the optimum functioning of several organ systems. When symptomatic, these shunts are treated. Interventions, when undertaken, might be medical, endovascular or surgical. The radiologist's role continues to expand in the assessment of these shunts, in providing a roadmap for treatment and in prompt identification of treatment-related complications. This review is to familiarize radiologists with the embryology, pre- and post-treatment imaging appearances, and associated complications of persistent fetal vascular shunts.


Assuntos
Permeabilidade do Canal Arterial , Canal Arterial , Canal Arterial/fisiologia , Hemodinâmica/fisiologia , Humanos , Recém-Nascido , Radiologistas
3.
Pediatr Radiol ; 52(10): 1998-2008, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35953542

RESUMO

Amid the coronavirus disease 2019 (COVID-19) pandemic, numerous publications of imaging findings in children have surfaced in a very short time. Publications discuss populations of overlapping age groups and describe different imaging patterns. We aim to present an overview of the quantity and type of literature available regarding COVID-19 chest imaging findings in children according to a 2020 publication timeline. We conducted a systematic review using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline. We searched terminology related to COVID-19, chest, children and imaging modalities in PubMed and Embase. The included papers were published online in 2020 and described imaging findings specific to children and reported five or more cases. Two researchers reviewed each abstract to determine inclusion or exclusion, and a radiologist reconciled any disagreements. Then we reviewed full articles for the main analysis. Eligible study designs included original articles, case series (≥5 cases), systematic reviews and meta-analyses. We excluded non-English manuscripts, retracted articles, and those without available full text. The remaining articles were distributed to four pediatric radiologists (on the Society for Pediatric Radiology Thoracic Committee), who summarized chest imaging findings. Eighty-two articles were included in the final analysis - 28% in radiology journals and 71% in non-radiology journals; 71% contained original data and 29% were review-style papers. There was a disproportionate contribution of review-style papers in April (55%), considering the paucity of preceding publications with original data in March (5 papers). June had the highest number of publications (n=14), followed by April (n=11) and July (n=11). Most (52%) original papers were from China and most individual pediatric imaging descriptions were from China (57%), while the majority of review papers (83%) were international. Imaging descriptions were available for 2,199 children (1,678 CT descriptions and 780 chest radiography descriptions). Findings included a 25% normal CT scan reports vs. 40% normal chest radiography reports. Ground-glass opacification was the most common CT finding (33%) and was reported in only a minority of chest radiographs (9%). A significant amount of information on pediatric COVID-19 chest imaging has become rapidly available over a short period. Most publications in 2020 were original articles, but they were published more often in non-radiology journals. A disproportionate number of review articles were published early on and were based on little original pediatric imaging data. CT scan reports, which represent the standard, outnumbered radiographic reports and indicated that ground-glass opacification is the main imaging finding and that only a quarter of scans are normal in children with COVID-19.


Assuntos
COVID-19 , Criança , China , Humanos , Pandemias , SARS-CoV-2 , Tomografia Computadorizada por Raios X/métodos
4.
Breast J ; 27(7): 573-580, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-33738890

RESUMO

BACKGROUND: Breast cancer mortality rates are 39% higher in the African-American (AA) women compared to White-American (WA) women despite the advances in overall breast cancer screening and treatments. Several studies have undertaken to identify the factors leading to this disparity in United States with possible effects of lower socioeconomic status and underlying aggressive biology. METHODS: A retrospective analysis was done using a prospectively maintained database of a metropolitan health system. Patients were selected based on diagnosis of early-stage breast cancer between 10/1998 and 02/2017, and included women over age of 18 with clinically node-negative disease. Patients were then stratified by phenotype confirmed by pathology and patient-identified race. RESULTS: A total of 2,298 women were identified in the cohort with 39% AA and 61% WA women. The overall mean age at the time of diagnosis for AA women was slightly younger at 60 years compared to 62 years for WA women (p = 0.003). Follow-up time was longer for the WA women at 95 months vs. 86 months in AA women. The overall 5-year survival was analyzed for the entire cohort, with the lowest survival occurring in patients with triple-negative breast cancer (TNBC). Phenotype distribution revealed a higher incidence of TNBC in AA women compared to WA women (AA 16% vs. WA 10%; p < 0.0001). AA women also had higher incidence of HER2 positive cancers (AA 16.8% vs. WA 15.3%; p < 0.0001). WA women had a significantly higher distribution of Non-TNBC/HER2-negative phenotype (AA 55% vs. WA 65%; p < 0.0001). Furthermore, a subgroup analysis was done for a sentinel lymph node (SLN) negative cohort that showed higher rates of grade 3 tumors in AA (AA 35% vs. WA 23%; p < 0.0001); and higher rates of grade 1 and grade 2 tumors in WA (30% vs. 21% and 44% vs. 40%). Despite higher grade tumors in AA women, five-year overall survival outcomes in SLN-negative cohort did not differ between AA and WA women when stratifying based on tumor subtype. CONCLUSION: Breast cancer survival disparities in AA and WA women with SLN-negative breast cancer are diminished when evaluated at early-stage cancers defined by SLN-negative tumors. Our evaluation suggests that when diagnosed early, phenotype does not contribute to racial survival outcomes. The lower survival rate in AA women with breast cancer may be attributed to later stage biology between the two races, or underlying socioeconomic disparities.


Assuntos
Neoplasias da Mama , Neoplasias de Mama Triplo Negativas , Negro ou Afro-Americano , Feminino , Humanos , Fenótipo , Estudos Retrospectivos , Estados Unidos/epidemiologia , População Branca
5.
Pediatr Radiol ; 51(8): 1518-1525, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-33666732

RESUMO

Increasing volume and complexity of cases in academic radiology and the drive toward pediatric sub-specialization have threatened knowledge assimilation for radiologists. There is a clear need for a system that retrieves vetted information from the excess available on the internet. Partnered with an interdisciplinary team from the Graduate School of Education, the authors created the first comprehensive learning management system (LMS) for radiology, implemented in the reading room to augment image interpretation and point-of-care education. The LMS supports quantitative analysis using a robust analytics platform to evaluate user statistics, facilitating improved quality of patient care by revolutionizing the way radiologists assimilate knowledge. This integration promises to enhance workflow and point-of-care teaching and to support the highest quality of care.


Assuntos
Radiologia , Criança , Currículo , Humanos , Aprendizagem , Radiografia , Radiologistas , Radiologia/educação
6.
Catheter Cardiovasc Interv ; 95(6): 1158-1162, 2020 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-31957932

RESUMO

Stent angioplasty of patent ductus arteriosus has been shown to be a viable alternative to operative shunt placement in cyanotic neonates. With broader implementation of this strategy, novel complications are bound to arise. We present a series of cases evaluated for ductal stent angioplasty in which a dilated and torturous ductus arteriosus compressed the left mainstem bronchus. After reviewing our recent experience with ductal stenting and isolated Blalock-Taussig shunts, our best estimate of the incidence of bronchial compression by the dilated ductus is 4.6% (3/64, 95% confidence interval 1.0-12.9%). Awareness of the airway and other nonvascular contents of the thorax is an important consideration prior to ductal stenting.


Assuntos
Obstrução das Vias Respiratórias/etiologia , Brônquios , Permeabilidade do Canal Arterial/complicações , Obstrução das Vias Respiratórias/diagnóstico por imagem , Obstrução das Vias Respiratórias/fisiopatologia , Angioplastia/efeitos adversos , Angioplastia/instrumentação , Procedimento de Blalock-Taussig , Brônquios/diagnóstico por imagem , Brônquios/fisiopatologia , Tomada de Decisão Clínica , Permeabilidade do Canal Arterial/diagnóstico por imagem , Permeabilidade do Canal Arterial/fisiopatologia , Permeabilidade do Canal Arterial/terapia , Feminino , Humanos , Recém-Nascido , Cuidados Paliativos , Fatores de Risco , Stents , Resultado do Tratamento
7.
Ann Surg ; 270(3): 484-492, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31356281

RESUMO

OBJECTIVE: To investigate subtype-specific risk of germline alleles associated with triple negative breast cancer (TNBC) in African ancestry populations. BACKGROUND: Breast cancer (BC) mortality is higher in African American (AA) compared to White American (WA) women; this disparity is partly explained by 2-fold higher TNBC incidence. METHODS: We used a surgically maintained biospecimen cohort of 2884 BC cases. Subsets of the total (760 AA; 962 WA; 910 West African/Ghanaian; 252 East African/Ethiopian) were analyzed for genotypes of candidate alleles. A subset of 417 healthy controls were also genotyped, to measure associations with overall BC risk and TNBC. RESULTS: TNBC frequency was highest in Ghanaian and AA cases (49% and 44% respectively; P < 0.0001) and lowest in Ethiopian and WA cases (17% and 24% respectively; P < 0.0001). TNBC cases had higher West African ancestry than non-TNBC (P < 0.0001). Frequency of the Duffy-null allele (rs2814778; an African ancestral variant adopted under selective pressure as protection against malaria) was associated with TNBC-specific risk (P < 0.0001), quantified West African Ancestry (P < 0.0001) and was more common in AA, Ghanaians, and TNBC cases. Additionally, rs4849887 was significantly associated with overall BC risk, and both rs2363956 and rs13000023 were associated with TNBC-specific risk, although none as strongly as the Duffy-null variant. CONCLUSIONS: West African ancestry is strongly correlated with TNBC status, as well as germline variants related to BC risk. The Duffy-null allele was associated with TNBC risk in our cohort.


Assuntos
Negro ou Afro-Americano/genética , Suscetibilidade a Doenças/epidemiologia , Mutação em Linhagem Germinativa/genética , Receptor ErbB-2/genética , Neoplasias de Mama Triplo Negativas/genética , Adulto , África Subsaariana/etnologia , Idoso , Estudos de Casos e Controles , Bases de Dados Factuais , Feminino , Gana/etnologia , Humanos , Incidência , Internacionalidade , Pessoa de Meia-Idade , Receptores de Estrogênio/genética , Receptores de Progesterona/genética , Medição de Risco , Neoplasias de Mama Triplo Negativas/etnologia , Neoplasias de Mama Triplo Negativas/patologia , Estados Unidos
8.
Radiology ; 291(3): 774-780, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30938628

RESUMO

Background The Fontan operation is performed for surgical palliation of single ventricle physiology. This operation is usually preceded by a superior cavopulmonary connection (SCPC); lymphatic abnormalities after SCPC may be demonstrated at MRI and prior to the Fontan operation. Purpose To determine if the degree of neck and thoracic lymphatic abnormalities at T2-weighted MRI in patients after superior cavopulmonary connection (SCPC) correlated with surgical outcomes from the Fontan procedure. Materials and Methods Patients for whom SCPC was performed for palliation of single ventricle disease who underwent chest MRI between July 2012 and May 2015 at a single institution were retrospectively reviewed. T2-weighted images were scored as lymphatic type 1 (little or no T2 mediastinal and supraclavicular signal) to type 4 (T2 signal into both the mediastinum and the lung parenchyma). Fontan takedown, duration of post-Fontan hospitalization and pleural effusion, postoperative plastic bronchitis, need for transplant, and mortality were tabulated. The relationship between lymphatic type and clinical outcomes was evaluated by using analysis of variance (ANOVA), the Kruskal-Wallis H test, and the Fisher exact test. Results A total of 83 patients (mean age, 7.9 years ± 2.6) were evaluated. Among these 83 patients, 53 (64%) were classified with type 1 or 2 lymphatic abnormalities, 17 (20%) with type 3, and 12 (16%) with type 4. The rate of failure of Fontan completion was higher in patients with type 4 than in type 1 or 2 (54% vs 2%, respectively; P = .004). Need for cardiac transplant (one of 13 [8%]) and death (three of 13 [23%]) occurred only in type 4. Median postoperative length of stay was longer for patients with type 4 than for those with types 1 or 2 (29 days vs 9 days, respectively; P < .01). Conclusion Greater MRI-based severity of lymphatic abnormalities in patients prior to planned Fontan procedure was associated with failure of Fontan completion and longer postoperative stay. © RSNA, 2019 Online supplemental material is available for this article.


Assuntos
Técnica de Fontan , Anormalidades Linfáticas/diagnóstico por imagem , Complicações Pós-Operatórias/diagnóstico por imagem , Criança , Pré-Escolar , Técnica de Fontan/efeitos adversos , Técnica de Fontan/mortalidade , Técnica de Fontan/estatística & dados numéricos , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Humanos , Tempo de Internação , Anormalidades Linfáticas/etiologia , Sistema Linfático/diagnóstico por imagem , Sistema Linfático/patologia , Imageamento por Ressonância Magnética , Pescoço/diagnóstico por imagem , Pescoço/patologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Tórax/diagnóstico por imagem , Tórax/patologia , Resultado do Tratamento
9.
Eur Radiol ; 29(10): 5190-5196, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30887210

RESUMO

OBJECTIVES: To describe the technique and report on our initial experience with the use of intrahepatic dynamic contrast magnetic resonance lymphangiography (IH-DCMRL) for evaluation of the lymphatics in patients with hepatic lymphatic flow disorders. METHODS: This is a retrospective review of the imaging and clinical findings in six consecutive patients undergoing IH-DCMRL. The technique involves injection of a gadolinium contrast agent into the intrahepatic lymphatic ducts followed by imaging of the abdomen and chest with both heavily T2-weighted imaging and dynamic time-resolved imaging. RESULTS: In six consecutive patients, IH-DCMRL was technically successful. There were four patients with protein-losing enteropathy (PLE) and two with ascites in this study. In the four patients with PLE, IH-DCMRL demonstrated hepatoduodenal connections with leak of contrast into the duodenal lumen not seen by conventional lymphangiography. In one patient with ascites, IH-DCMRL demonstrated lymphatic leakage into the peritoneal cavity not seen by intranodal lymphangiography. In the second patient with ascites, retrograde lymphatic perfusion of mesenteric lymphatic networks and nodes was seen. Venous contamination was seen in two patients. No biliary contamination was identified. There were no short-term complications. CONCLUSIONS: IH-DCMRL is a cross-sectional technique which successfully evaluated hepatic lymphatic flow disorders and warrants further investigation. KEY POINTS: • Intrahepatic dynamic contrast magnetic resonance lymphangiography (IH-DCMRL) is a new imaging technique to evaluate hepatic lymphatic flow disorders such as protein-losing enteropathy. • In comparison to conventional liver lymphangiography, IH-DCMRL offers a 3D imaging technique and better distal lymphatic contrast distribution and does not use ionizing radiation.


Assuntos
Fígado/irrigação sanguínea , Doenças Linfáticas/diagnóstico , Vasos Linfáticos/patologia , Linfografia/métodos , Angiografia por Ressonância Magnética/métodos , Compostos Organometálicos/farmacologia , Adolescente , Adulto , Criança , Pré-Escolar , Meios de Contraste/farmacologia , Estudos Transversais , Feminino , Humanos , Imageamento Tridimensional , Lactente , Fígado/diagnóstico por imagem , Masculino , Estudos Retrospectivos , Adulto Jovem
10.
J Surg Res ; 234: 54-58, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30527498

RESUMO

BACKGROUND: Little is known about general surgery trainees' education regarding management of breast problems. We sought to measure the impact of a dedicated breast surgery rotation on American Board of Surgery In-Service Examination (ABSITE) scores and operative volumes. METHODS: A breast surgery rotation was implemented at our program in July 2016. We obtained the January 2017 ABSITE scores for postgraduate year (PGY) 1-3 residents, and obtained the case volumes for PGY 1-3 residents during the years 2015-2016 and 2016-2017. RESULTS: We compared the performance on total questions and skin, soft tissue, and breast questions between the residents who had the breast rotation before the ABSITE to those that had it after. There was no difference in the average overall percentage (70.2% versus 71.7%, P = 0.55) or in the average skin, soft tissue, and breast percentage (70% versus 71.4%, P = 0.72). A postgraduate year-to-year comparison showed an increase in average total major cases among the PGY-1 residents (93.8 versus 166.8, P = 0.02), and an increase in average breast cases among the PGY-1 (17.8 versus 27 cases, P < 0.01) and PGY-2 (27.3 versus 47.7 cases, P = 0.02) years. There was an increase in the proportion of complex breast cases performed by PGY-3 residents (23.2% versus 33.1%, P = 0.01). CONCLUSIONS: A dedicated breast surgery rotation did not detract from the nonbreast general surgery educational experience of junior residents (as measured by ABSITE scores), and it increased the case volume of breast as well as total major cases among junior residents. A breast surgery rotation is valuable for strengthening surgical case volumes.


Assuntos
Mama/cirurgia , Cirurgia Geral/educação , Internato e Residência , Avaliação Educacional , Humanos
11.
Pediatr Radiol ; 48(10): 1381-1387, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-30121791

RESUMO

Whether employed in a private practice, a community or academic setting, or even a hybrid practice, radiologists fill many roles in the course of a day. Teaching is one of the most important roles in the dissemination of knowledge and the continued renewal of the health, vibrancy and advancement of our field. In this article the author reviews some of the various facets of what radiology teaching and learning look like in the current era. Highlighted topics include workstation teaching, workshop and small group education, interdisciplinary activities and mentorship.


Assuntos
Modelos Educacionais , Pediatria/educação , Radiologia/educação , Ensino/tendências , Currículo , Docentes de Medicina , Humanos , Tutoria , Materiais de Ensino
12.
Pediatr Radiol ; 48(11): 1658-1671, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30194461

RESUMO

Congenital aberrant hepatic vascular communications result from intrahepatic or extrahepatic errors in vascular development or involution during the transition from fetal to newborn hepatic circulation. These hepatic vascular shunts can be fortuitously discovered and asymptomatic, or can cause symptoms of varying severity, often presenting diagnostic dilemmas. Some hepatic vascular shunts resolve spontaneously while others require interventional radiologic or surgical closure. Affected patients are often first studied with real-time and Doppler ultrasound, so radiologists should familiarize themselves with the expected ultrasound findings of these vascular shunts for effective diagnosis, triage and management. In this review, the authors focus on ultrasound and Doppler findings of hepatic vascular shunts with underlying embryology, clinical features and management strategies. Broadly, these aberrant hepatic vascular communications include portosystemic venous shunts (which can be intra- or extrahepatic and include persistent patent ductus venosus), arterioportal, arteriovenous or mixed shunts.

13.
J Ultrasound Med ; 35(7): 1465-73, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27229130

RESUMO

OBJECTIVES: Neonates in the neonatal intensive care unit often require considerable support with endotracheal tubes, umbilical arterial and venous catheters, and peripherally inserted central venous catheters. Support device evaluation with radiography exposes neonates to ionizing radiation. This study evaluated the effectiveness of sonographic localization for endotracheal tubes, umbilical arterial and venous catheters, and peripherally inserted central venous catheters. METHODS: This blinded prospective Institutional Review Board-approved, Health Insurance Portability and Accountability Act-compliant study with informed consent compared sonography to radiography for endotracheal tube, umbilical arterial and venous catheter, and peripherally inserted central venous catheter localization. Participants were consecutively recruited NICU patients of any weight, gestation, and chronologic age who had an endotracheal tube, umbilical arterial catheter, umbilical venous catheter, or peripherally inserted central venous catheter placed or adjusted and had subsequent radiographic confirmation within 24 hours. Sonographic evaluation was obtained as soon as possible, without prior review of the radiograph, and results were compared. RESULTS: Thirty sonographic studies were performed in 25 patients (14 male and 11 female), for a total of 50 lines and tubes: 18 umbilical venous catheters, 12 umbilical arterial catheters, 11 peripherally inserted central venous catheters, and 9 endotracheal tubes. Forty-nine support devices (98%) were visualized with sonography, all concordant with radiography. Forty-four were correctly positioned, and 6 were malpositioned. Sonography identified the location of umbilical venous catheters in all 18 cases (100%), umbilical arterial catheters in all 12 (100%), peripherally inserted central venous catheters in 10 (91%), and endotracheal tubes in 9 (100%). CONCLUSIONS: The effectiveness of sonography was excellent for evaluation of umbilical arterial and venous catheters, endotracheal tubes, and peripherally inserted central venous catheters. These results support the goal of further point-of-care training and accreditation to use sonography as a primary modality for complete evaluation of NICU support devices.


Assuntos
Cateterismo/instrumentação , Cuidados Críticos/métodos , Unidades de Terapia Intensiva Neonatal , Intubação Intratraqueal/instrumentação , Ultrassonografia de Intervenção/métodos , Cateterismo/métodos , Cateterismo Venoso Central/instrumentação , Cateterismo Venoso Central/métodos , Cateterismo Periférico/instrumentação , Cateterismo Periférico/métodos , Feminino , Humanos , Recém-Nascido , Masculino , Projetos Piloto , Sistemas Automatizados de Assistência Junto ao Leito , Estudos Prospectivos , Método Simples-Cego , Veias Umbilicais/diagnóstico por imagem , Dispositivos de Acesso Vascular
14.
Pediatr Radiol ; 46(5): 666-73, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-26795619

RESUMO

BACKGROUND: Functional MR urography (fMRU) provides comprehensive functional data that can be subject to variability. To interpret the results of fMRU, it is essential to know the intra- and inter-observer variability of the measured parameters. OBJECTIVE: To define the range of variability in fMRU, particularly that of the differential renal function based on volume (volumetric differential renal function) and Patlak differential renal function measurements in children. MATERIALS AND METHODS: We included 15 fMRU studies, 10 of non-duplicated and 5 of unilateral duplex kidneys. We recruited six observers with a range of fMRU experience, including two MRI technologists, one resident, one fellow, one pediatric radiologist and one pediatric urologist. The observers underwent intensive training in using the Children's Hospital of Philadelphia (CHOP)-fMRU freeware for analysis. They conducted the fMRU analysis on each case twice, at least 1 week apart. Mean and standard deviation were calculated for each set of absolute volume, absolute Patlak, volumetric differential renal function and Patlak differential renal function. We calculated the statistical significance of these deviations using the student's t-test. We also calculated interclass correlations for intra-observer and inter-observer agreement of both volume and Patlak measurements using SPSS software. RESULTS: Intra- and inter-observer variability did not differ significantly, measuring 6% and 4% for relative volume (volumetric differential renal function: P > 0.05) and 5% and 3% for relative function (Patlak differential renal function: P > 0.05). Absolute values of parameters showed more variability than the relative values. Intra- and inter-observer agreement was well above 0.90 (P < 0.001) for all volume measures except for duplex upper pole intra-observer measurements (0.80, P < 0.01). Intra- and inter-observer agreement for Patlak values were also above 0.90 (P < 0.001) except for duplex upper pole measurements, which were 0.54 (P = 0.13) and 0.81 (P < 0.01), respectively. CONCLUSION: Functional MRU analysis using CHOP-fMRU software is reproducible, with overall intra- and inter-observer variability rates of 5% for volumetric differential renal function and 4% for Patlak differential renal function. There was higher variability in volume and function measurements between upper and lower pole moieties of duplicated kidneys and for absolute volume and function values overall. A range of 45-55% for relative values of volumetric differential renal function and Patlak differential renal function could serve as the normal range.


Assuntos
Nefropatias/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Testes de Função Renal , Masculino , Variações Dependentes do Observador , Estudos Prospectivos , Reprodutibilidade dos Testes , Software , Urografia
15.
Pediatr Radiol ; 46(4): 483-9, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26691156

RESUMO

BACKGROUND: Hypoplastic left heart syndrome (HLHS) is the third most common cause of critical congenital heart disease in newborns, and one of the most challenging forms to treat. Secondary pulmonary lymphangiectasia has been recognized in association with HLHS, an appearance described on fetal MRI as the "nutmeg lung." OBJECTIVE: To investigate the association of fetal nutmeg lung with HLHS survival. MATERIALS AND METHODS: A retrospective search of the fetal MRI database was performed. The nutmeg lung pattern was defined as T2 heterogeneous signal with tubular structures radiating peripherally from the hila. Postnatal echocardiograms and charts were reviewed. RESULTS: Forty-four fetal MR studies met inclusion criteria, of which 4 patients (9%) had the nutmeg lung pattern and 3 of whom also had restrictive lesions. Mortality in this nutmeg lung group was 100% by 5 months of age. Of the 40 patients without nutmeg lung, mortality/orthotopic heart transplant (OHT) was 35%. Of these 40 patients without nutmeg lung, 5 had restriction on echo, 3 of whom died/had OHT before 5 months of age (60% of patients with restriction and non-nutmeg lung). There was a significantly higher incidence of restrictive lesions (P = 0.02) and mortality/OHT (P = 0.02) in patients with nutmeg lung compared to those without. CONCLUSION: The nutmeg lung MR appearance in HLHS fetuses is associated with increased mortality/OHT (100% in the first 5 months of life compared to 35% with HLHS alone). Not all patients with restrictive lesions develop nutmeg lung, and outcome is not as poor when restriction is present in isolation. Dedicated evaluation for nutmeg lung pattern on fetal MR studies may be useful to guide prognostication and aid clinicians in counseling parents of fetuses with HLHS.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Pneumopatias/congênito , Linfangiectasia/congênito , Diagnóstico Pré-Natal/estatística & dados numéricos , Causalidade , Pré-Escolar , Comorbidade , Feminino , Mortalidade Fetal , Humanos , Síndrome do Coração Esquerdo Hipoplásico/embriologia , Lactente , Recém-Nascido , Pulmão/diagnóstico por imagem , Pulmão/embriologia , Pneumopatias/diagnóstico por imagem , Pneumopatias/mortalidade , Linfangiectasia/diagnóstico por imagem , Linfangiectasia/mortalidade , Imageamento por Ressonância Magnética/estatística & dados numéricos , Masculino , Pennsylvania/epidemiologia , Prognóstico , Fatores de Risco , Taxa de Sobrevida
16.
J Pediatr Surg ; 59(3): 509-514, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37875379

RESUMO

STUDY OBJECTIVE: The study objective was to apply a previously created composite score for the prediction of adnexal torsion in children and adolescents to a larger group of heterogeneous patients to test its validity and to refine this scoring system to more accurately predict adnexal torsion. METHODS: This was a retrospective chart review at a tertiary care children's hospital and its affiliates. Participants were female patients 0-20 years of age who underwent surgery for suspected adnexal torsion from 2016 to 2019. Data were collected from outpatient, emergency department, and inpatient notes, radiographic data, and operative reports. The primary outcome was the accuracy and predictive value of the total composite score (TCS) to identify or exclude adnexal torsion. RESULTS: Of the 291 patients with suspected adnexal torsion who went to the operating room during the study period, 168 (57.8 %) had confirmed adnexal torsion. The accuracy of the TCS, which included presence of vomiting, adnexal volume, and adnexal ratio, in predicting or excluding torsion was 83.6 % for all patients, 92.1 % for premenarchal patients, and 81.3 % for menarchal patients. A variation of the TCS that included only vomiting and adnexal volume had an accuracy of 85.8 %. CONCLUSIONS: Our study demonstrates the accuracy and reproducibility of a previously published composite score to predict adnexal torsion in children and adolescents. It also further refines this score to a potentially more clinically useful tool. Future studies are needed to prospectively evaluate these composite scores and their implementation in clinical settings. LEVEL OF EVIDENCE: III.


Assuntos
Doenças dos Anexos , Criança , Humanos , Feminino , Adolescente , Masculino , Doenças dos Anexos/diagnóstico por imagem , Doenças dos Anexos/cirurgia , Torção Ovariana , Estudos Retrospectivos , Anormalidade Torcional/diagnóstico , Anormalidade Torcional/cirurgia , Reprodutibilidade dos Testes , Vômito
17.
J Pediatr Surg ; 2024 Mar 29.
Artigo em Inglês | MEDLINE | ID: mdl-38649311

RESUMO

BACKGROUND: Isolated fallopian tube torsion (IFTT) is a rare form of adnexal torsion that is more difficult to diagnose, which may lead to delays in treatment. Our objectives were to identify clinical and radiologic factors associated with surgically-confirmed IFTT and compare them with those of patients without torsion and with adnexal torsion (AT) in a large pediatric population. METHODS: We conducted a retrospective chart review of all patients who underwent surgery for suspected adnexal torsion from 2016 to 2019. Torsion was determined intraoperatively, with IFFT defined as those with only tubal but no ovarian torsion and AT defined as those with ovarian torsion, with or without involvement of the ipsilateral fallopian tube. Clinical and radiologic variables were compared between patients with IFTT and those without torsion and with AT using descriptive statistics. A previously-described composite score to predict torsion based on the presence of vomiting and adnexal volume (VVCS) was calculated for each patient. RESULTS: Of 291 patients who underwent surgery for suspected torsion, 168 had confirmed torsion: 33 (19.6%) IFTT and 135 (80.4%) AT. Patients with IFTT were more likely to be younger (12.8 vs. 14.2 years, P = 0.02), premenarchal (29.0% vs. 10.7%, P = 0.009), experience nausea (90.6% vs. 70.9%, P = 0.02) and vomiting (81.3% vs. 32.8%, P < 0.001), have a paratubal cyst on imaging (18.8% vs. 2.5%, P = 0.003), and have larger adnexal volume (143.3 vs. 64.9 ml, P < 0.001) than those without torsion. Higher BMI (26.6 vs. 22.9 kg/m2, P = 0.03), a paratubal cyst on imaging (18.8% vs. 1.5%, P < 0.001), presence of arterial (65.5% vs. 44.1%, P = 0.04) and venous Doppler flow (79.3% vs. 55.9%, P = 0.02), and radiologic impression indicating lack of torsion (37.9% vs. 16.8%, P = 0.04) were more common in IFTT than AT. The accuracy of the VVCS in predicting torsion for the IFFT group was 83.9%. CONCLUSIONS: IFTT has a similar clinical presentation to AT but with a higher likelihood of a paratubal cyst and preserved Doppler flow on imaging. IFTT should be strongly considered in patients who present with pain, nausea, and vomiting and have an adnexal mass separate from the ovary on imaging, regardless of Doppler flow. LEVEL OF EVIDENCE: Level II. TYPE OF STUDY: Prognosis study.

18.
Mol Biol Evol ; 29(2): 825-35, 2012 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-21998276

RESUMO

Thermophily is thought to be a primitive trait, characteristic of early forms of life on Earth, that has been gradually lost over evolutionary time. The genus Bacillus provides an ideal model for studying the evolution of thermophily as it is an ancient taxon and its contemporary species inhabit a range of thermal environments. The thermostability of reconstructed ancestral proteins has been used as a proxy for ancient thermal adaptation. The reconstruction of ancestral "enzymes" has the added advantages of demonstrable activity, which acts as an internal control for accurate inference, and providing insights into the evolution of enzymatic catalysis. Here, we report the reconstruction of the structurally complex core metabolic enzyme LeuB (3-isopropylmalate dehydrogenase, E. C. 1.1.1.85) from the last common ancestor (LCA) of Bacillus using both maximum likelihood (ML) and Bayesian inference. ML LeuB from the LCA of Bacillus shares only 76% sequence identity with its closest contemporary homolog, yet it is fully functional, thermophilic, and exhibits high values for k(cat), k(cat)/K(M), and ΔG(‡) for unfolding. The Bayesian version of this enzyme is also thermophilic but exhibits anomalous catalytic kinetics. We have determined the 3D structure of the ML enzyme and found that it is more closely aligned with LeuB from deeply branching bacteria, such as Thermotoga maritima, than contemporary Bacillus species. To investigate the evolution of thermophily, three descendents of LeuB from the LCA of Bacillus were also reconstructed. They reveal a fluctuating trend in thermal evolution, with a temporal adaptation toward mesophily followed by a more recent return to thermophily. Structural analysis suggests that the determinants of thermophily in LeuB from the LCA of Bacillus and the most recent ancestor are distinct and that thermophily has arisen in this genus at least twice via independent evolutionary paths. Our results add significant fluctuations to the broad trend in thermal adaptation previously proposed and demonstrate that thermophily is not exclusively a primitive trait, as it can be readily gained as well as lost. Our findings also demonstrate that reconstruction of complex functional Precambrian enzymes is possible and can provide empirical access to the evolution of ancient phenotypes and metabolisms.


Assuntos
3-Isopropilmalato Desidrogenase/genética , Bacillus/enzimologia , Bacillus/genética , Evolução Molecular , 3-Isopropilmalato Desidrogenase/metabolismo , Adaptação Fisiológica/genética , Sequência de Aminoácidos , Bacillus/metabolismo , Proteínas de Bactérias/genética , Proteínas de Bactérias/metabolismo , Teorema de Bayes , Evolução Biológica , Temperatura Alta , Funções Verossimilhança , Filogenia
19.
Otolaryngol Head Neck Surg ; 169(3): 496-503, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-36808755

RESUMO

OBJECTIVE: To validate and compare novel ultrasound scoring systems for dermoid cysts and thyroglossal duct cysts among pediatric patients. STUDY DESIGN: Retrospective study. SETTING: Tertiary care children's hospital. METHODS: An electronic medical record query of patients younger than 18 years old who underwent primary excision of a neck mass between January 2005 and February 2022, who underwent preoperative ultrasound, and had final histopathologic diagnosis of either thyroglossal duct cysts or dermoid cysts. This generated 260 results, of which 134 patients met the inclusion criteria. Charts were reviewed for demographic data, clinical impressions, and radiographic studies. Radiologists blindly reviewed ultrasound for SIST score (septae + irregular walls + solid components = thyroglossal), and 4S algorithm (Septations, depth relative to Strap muscles, Shape, Solid parts). Statistical analyses were performed to determine the accuracy of each diagnostic modality. RESULTS: Of 134 patients, 90 (67%) had a final histopathologic diagnosis of thyroglossal duct cysts, and 44 (33%) were dermoid cysts. The accuracy of clinical diagnosis was 52%, and preoperative ultrasound report accuracy was 31%. The 4S and SIST accuracies were each 84%. CONCLUSION: Both the 4S algorithm and SIST score provide increased accuracy of diagnosis relative to standard preoperative ultrasound assessment. Neither scoring modality was determined to be superior. Further research is warranted in improving the accuracy of preoperative assessments for pediatric congenital neck masses.


Assuntos
Cisto Dermoide , Cisto Tireoglosso , Criança , Humanos , Adolescente , Estudos Retrospectivos , Cisto Tireoglosso/diagnóstico por imagem , Cisto Tireoglosso/cirurgia , Cisto Dermoide/diagnóstico por imagem , Cisto Dermoide/cirurgia , Ultrassonografia
20.
J Pediatr Surg ; 58(4): 767-773, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-36008196

RESUMO

BACKGROUND: Adnexal torsion is a gynecologic emergency in children and adolescents but remains a challenging diagnosis, with no consistent clinical or radiologic diagnostic criteria. Our objective was to identify risk factors associated with adnexal torsion in premenarchal and menarchal patients with surgically confirmed torsion compared with those without torsion. METHODS: We conducted a retrospective chart review of all patients who underwent surgery between January 2016 and December 2019 for possible adnexal torsion. Data on demographics, clinical characteristics, radiologic variables, and operative findings were compared using descriptive statistics. Independent predictors of torsion were then examined in multivariate logistic regression models. RESULTS: Of the 291 patients who underwent surgery, 168 (57.7%) had torsion. Patients with torsion were younger than those without torsion (11.9 vs. 14.2 years, P < .01). Vomiting was significantly associated with torsion for all patients (P < .001). Large adnexal volume and absent arterial Doppler flow were associated with torsion for the total population and menarchal subgroup. A logistic regression model for the total population that controlled for age and menarchal status found that vomiting (adjusted odds ratio [aOR] 5.92, 95% confidence interval [CI] 2.87-12.22), highest adnexal volume category (aOR 4.92, 95% CI 2.25-10.75), and absent arterial Doppler flow (aOR 2.674, 95% CI 1.28-5.60) were associated with torsion. CONCLUSIONS: Vomiting, enlarged adnexal volume, and absent arterial Doppler flow were associated with adnexal torsion. However, no single risk factor accurately diagnosed torsion, and multiple factors should be interpreted together. LEVEL OF EVIDENCE: Study of Diagnostic Test, Level II.


Assuntos
Doenças dos Anexos , Criança , Feminino , Humanos , Adolescente , Doenças dos Anexos/diagnóstico por imagem , Doenças dos Anexos/cirurgia , Doenças dos Anexos/complicações , Torção Ovariana , Estudos Retrospectivos , Anormalidade Torcional/diagnóstico por imagem , Anormalidade Torcional/cirurgia , Anormalidade Torcional/complicações , Vômito/etiologia
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