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1.
Artículo en Inglés | MEDLINE | ID: mdl-38698908

RESUMEN

Eosinophilic esophagitis (EoE) is a chronic inflammatory condition characterized by an intense infiltration of eosinophils into the esophageal epithelium. When not adequately controlled, eosinophilic inflammation can lead to changes in components of the extracellular matrix (ECM) of the lamina propria. Particularly, alterations to the collagen fiber matrix can lead to lamina propria fibrosis (LPF), which plays an important role in the fibrostenotic complications of EoE. Current approaches to assess LPF in EoE are prone to inter-observer inconsistencies and provide limited insight into the structural remodeling of the ECM. An objective approach to quantify LPF can eliminate inter-observer inconsistencies and provide novel insights into the fibrotic transformation of the lamina propria in EoE. Second harmonic generation (SHG) microscopy is a powerful modality for objectively quantifying disease associated alterations in ECM collagen structure that is finding increasing use for clinical research. We used SHG with morphometric analysis (SHG-MA) to characterize lamina propria collagen fibers and ECM porosity in esophageal biopsies collected from children with active EoE (n = 11), inactive EoE (n = 11), and non-EoE (n = 11). The collagen fiber width quantified by SHG-MA correlated positively with peak eosinophil count (r = 0.65, p < 0.005) and histopathologist scoring of LPF (r = 0.52, p < 0.005) in the esophageal biopsies. Patients with active EoE had a significant enlargement of ECM pores compared to inactive EoE and non-EoE (p < 0.005), with the mean pore area correlating positively with EoE activity (r = 0.76, p < 0.005) and LPF severity (r = 0.65, p < 0.005). These results indicate that SHG-MA can be utilized to objectively characterize and provide novel insights into lamina propria ECM structural remodeling in children with EoE, which could aid in monitoring disease progression.

3.
Ann Surg ; 279(3): 528-535, 2024 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-37264925

RESUMEN

OBJECTIVE: The purpose of this study was to describe management and outcomes from a contemporary cohort of children with Wilms tumor complicated by inferior vena caval thrombus. BACKGROUND: The largest series of these patients was published almost 2 decades ago. Since then, neoadjuvant chemotherapy has been commonly used to manage these patients, and outcomes have not been reported. METHODS: Retrospective review of 19 North American centers between 2009 and 2019. Patient and disease characteristics, management, and outcomes were investigated and analyzed. RESULTS: Of 124 patients, 81% had favorable histology (FH), and 52% were stage IV. IVC thrombus level was infrahepatic in 53 (43%), intrahepatic in 32 (26%), suprahepatic in 14 (11%), and cardiac in 24 (19%). Neoadjuvant chemotherapy using a 3-drug regimen was administered in 82% and postresection radiation in 90%. Thrombus level regression was 45% overall, with suprahepatic level showing the best response (62%). Cardiopulmonary bypass (CPB) was potentially avoided in 67%. The perioperative complication rate was significantly lower after neoadjuvant chemotherapy [(25%) vs upfront surgery (55%); P =0.005]. CPB was not associated with higher complications [CPB (50%) vs no CPB (27%); P =0.08]. Two-year event-free survival was 93% and overall survival was 96%, higher in FH cases (FH 98% vs unfavorable histology/anaplastic 82%; P =0.73). Neither incomplete resection nor viable thrombus cells affected event-free survival or overall survival. CONCLUSIONS: Multimodal therapy resulted in excellent outcomes, even with advanced-stage disease and cardiac extension. Neoadjuvant chemotherapy decreased the need for CPB to facilitate resection. Complete thrombectomy may not always be necessary.


Asunto(s)
Neoplasias Renales , Oncología Quirúrgica , Trombosis de la Vena , Tumor de Wilms , Humanos , Niño , Neoplasias Renales/cirugía , Vena Cava Inferior/cirugía , Tumor de Wilms/cirugía , Tumor de Wilms/tratamiento farmacológico , Trombosis de la Vena/patología , Trombectomía/métodos , Estudios Retrospectivos , Nefrectomía/métodos
4.
Ann Surg ; 279(3): 536-541, 2024 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-37487006

RESUMEN

OBJECTIVE: To determine the impact of nodal basin ultrasound (US) surveillance versus completion lymph node dissection (CLND) in children and adolescents with sentinel lymph node (SLN) positive melanoma. BACKGROUND: Treatment for children and adolescents with melanoma are extrapolated from adult trials. However, there is increasing evidence that important clinical and biological differences exist between pediatric and adult melanoma. METHODS: Patients ≤18 years diagnosed with cutaneous melanoma between 2010 and 2020 from 14 pediatric hospitals were included. Data extracted included demographics, histopathology, nodal basin strategies, surveillance intervals, and survival information. RESULTS: Of 252 patients, 90.1% (n=227) underwent SLN biopsy (SLNB), 50.9% (n=115) had at least 1 positive node. A total of 67 patients underwent CLND with 97.0% (n=65/67) performed after a positive SLNB. In contrast, 46 total patients underwent US observation of nodal basins with 78.3% (n=36/46) of these occurring after positive SLNB. Younger patients were more likely to undergo US surveillance (median age 8.5 y) than CLND (median age 11.3 y; P =0.0103). Overall, 8.9% (n=21/235) experienced disease recurrence: 6 primary, 6 nodal, and 9 distant. There was no difference in recurrence (11.1% vs 18.8%; P =0.28) or death from disease (2.2% vs 9.7%; P =0.36) for those who underwent US versus CLND, respectively. CONCLUSIONS: Children and adolescents with cutaneous melanoma frequently have nodal metastases identified by SLN. Recurrence was more common among patients with thicker primary lesions and positive SLN. No significant differences in oncologic outcomes were observed with US surveillance and CLND following the identification of a positive SLN.


Asunto(s)
Melanoma , Ganglio Linfático Centinela , Neoplasias Cutáneas , Adulto , Humanos , Adolescente , Niño , Melanoma/diagnóstico por imagen , Melanoma/cirugía , Melanoma/patología , Neoplasias Cutáneas/diagnóstico por imagen , Neoplasias Cutáneas/cirugía , Ganglio Linfático Centinela/patología , Recurrencia Local de Neoplasia/patología , Escisión del Ganglio Linfático , Biopsia del Ganglio Linfático Centinela , Estudios Retrospectivos
5.
Clin Transl Gastroenterol ; 15(2): e00665, 2024 02 01.
Artículo en Inglés | MEDLINE | ID: mdl-38112293

RESUMEN

INTRODUCTION: Biochemical alterations in the esophagus of patients with eosinophilic esophagitis (EoE) are poorly understood. We used Raman spectroscopy through a pediatric endoscope to identify key Raman features reflective of the esophageal biochemical composition to differentiate between children with EoE from non-EoE controls and between children with active (aEoE) and inactive EoE (iEoE). METHODS: Spectral measurements were obtained using a customized pediatric endoscope-compatible fiber-optic Raman probe in real time during an esophagogastroduodenoscopy. Chemometric analysis was performed to identify key Raman features associated with EoE. Pearson correlation analysis was used to assess relationship between the key Raman features and EoE activity indices. Their diagnostic utility was ascertained using the receiver operator characteristic curve analysis. RESULTS: Forty-three children were included in the study (EoE = 32 [74%] and non-EoE control = 11 [26%]; aEoE = 20 [63%] and iEoE = 12 [37%]). Raman intensities assigned to lipids, proteins, and glycogen:protein ratio accurately distinguished children with EoE from those without EoE and aEoE from iEoE. They significantly correlated with EoE activity indices. The Raman peak ratio for lipids had 90.6% sensitivity, 100% specificity, and an area under the curve of 0.95 to differentiate children with EoE from non-EoE controls. The glycogen:protein ratio had 70% sensitivity, 91.7% specificity, and an area under the curve of 0.75 to distinguish children with aEoE from iEoE. DISCUSSION: Real-time intraendoscopy Raman spectroscopy is an effective method for identifying spectral markers reflective of the esophageal biochemical composition in children with EoE. This technique may aid in the diagnosis and monitoring of EoE and help to elucidate EoE pathogenesis.


Asunto(s)
Enteritis , Eosinofilia , Esofagitis Eosinofílica , Gastritis , Humanos , Niño , Esofagitis Eosinofílica/diagnóstico , Esofagitis Eosinofílica/patología , Espectrometría Raman , Glucógeno , Lípidos
6.
J Pediatr Pharmacol Ther ; 28(6): 504-508, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-38130344

RESUMEN

OBJECTIVE: Proton pump inhibitors (PPIs) are commonly used to manage children with upper gastrointestinal symptoms and without a formal diagnosis. We investigated the effect of PPIs on esophageal mucosal transcriptome and active microbiota in children with normal esophagi. Furthermore, we examined whether the differences in host esophageal mucosal gene expression were driven by an underlying esophageal epithelial cell type composition. METHODS: Using metatranscriptomics, the host transcriptional and active microbial profiles were captured from 17 esophageal biopsy samples (PPI naïve [PPI-], n = 7; PPI exposed [PPI+], n = 10) collected from children without any endoscopic and histologic abnormalities in their esophagus (normal esophagus). Deconvolution computational analysis was performed with xCell to assess if the observed epithelial gene expression changes were related to the cell type composition in the esophageal samples. RESULTS: The median (IQR) age of our cohort was 14 years (12-16) with female (63%) preponderance. Both groups were similar in terms of their demographics and clinical features. Compared with PPI-, the PPI+ had upregulation of 27 genes including the MUC genes. The cell type composition was similar between the PPI- and PPI+ groups. Prevotella sp and Streptococcus sp were abundant in PPI+ group. CONCLUSIONS: In children with normal esophagus, PPI exposure can be associated with upregulation of esophageal mucosal homeostasis and epithelial cell function genes in a cell-type independent manner, and an altered esophageal microbiome. Additional studies are warranted to validate our findings and to investigate the causal effect of PPIs on the normal esophageal epithelium and microbial communities.

7.
Cancers (Basel) ; 15(19)2023 Oct 09.
Artículo en Inglés | MEDLINE | ID: mdl-37835596

RESUMEN

The association between Hashimoto's thyroiditis (HT) and pediatric thyroid cancer is controversial. Most studies examining this connection have been based on adults, and larger studies in children are lacking. We performed a retrospective study of all sequential pediatric patients who underwent a thyroidectomy for a neoplasm at our institution over a twenty-year period in order to explore the link between HT and pediatric thyroid cancer. A total of 153 patients, median age 16.5 (interquartile range [IQR] 14.2-18.3) years, underwent thyroid surgery for a neoplasm. Patients were mainly female (80%) and White (84%). Median follow-up was 58.6 (IQR 20.7-105.4) months. Thirty-five (23%) patients had HT. Patients who underwent thyroid surgery and had HT were more likely to harbor a malignant neoplasm (p = 0.05); specifically, papillary thyroid carcinoma (PTC, p = 0.02). There was a difference in the distribution of HT among the subtypes of PTC (p = 0.03). Despite this, there was no difference in terms of survival between patients with/without HT. In conclusion, children with a thyroid malignancy, specifically, PTC, are more likely to have HT. The association between HT and pediatric PTC appears to be subtype-specific but does not seem to affect patient survival.

8.
Am J Surg Pathol ; 47(9): 977-989, 2023 09 01.
Artículo en Inglés | MEDLINE | ID: mdl-37357941

RESUMEN

A recent increase in reports of severe acute hepatitis of unknown etiology in children is under investigation. Although adenovirus has been frequently detected, its role remains unclear, and systematic histopathologic analysis is lacking. We conducted a retrospective study of 11 children hospitalized between October 2021 and May 2022 with unexplained acute hepatitis and concurrent adenovirus infection. Liver biopsies collected shortly after admission demonstrated moderately to severely active hepatitis in 8/11 (73%) cases, characterized by marked portal mixed inflammation, moderate-to-severe interface activity, and milder lobular inflammation. Clusters of plasma cells were present in 6/11 (55%) cases, mimicking autoimmune hepatitis. Semiquantitative scoring of 17 discrete histologic features found that greater degrees of portal inflammation, interface activity, bile duct injury, bile ductular reaction, lobular inflammation, Kupffer cell activation, and hepatocyte focal necrosis were significantly more common in these cases in comparison to the control group of unexplained acute severe hepatitis without adenovirus infection. Liver biopsy immunohistochemistry was negative for adenovirus in all cases. Polymerase chain reaction testing of liver tissue was positive for the enteric adenovirus serotypes 41 (species F) in 10/11 (91%) cases. An immunoprofile study of hepatic infiltrating lymphocytes in 1 patient revealed the presence of large numbers of CD3 + and CD4 + lymphocytes. Nine patients received supportive treatment without steroids and recovered without the need for liver transplantation. In summary, liver injury in children with severe acute hepatitis and adenovirus infection is characterized by a hepatitic pattern that resembles severe autoimmune hepatitis and may represent an immune-mediated process associated with viral infection.


Asunto(s)
Infecciones por Adenoviridae , Hepatitis Autoinmune , Humanos , Niño , Hepatitis Autoinmune/patología , Estudios Retrospectivos , Hígado/patología , Inflamación/patología , Infecciones por Adenoviridae/complicaciones , Infecciones por Adenoviridae/patología , Linfocitos T CD4-Positivos
9.
Am Surg ; 89(9): 3745-3750, 2023 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-37150742

RESUMEN

BACKGROUND: Neuroblastomas are the most common extracranial solid malignancy in children with variable manifestations and complications depending on the presence of paraneoplastic syndromes. MATERIALS AND METHODS: We performed a single institution retrospective cohort study of all patients less than 18 years old diagnosed with neuroblastoma or ganglioneuroblastoma between January 2002 and July 2022. Patients were identified through the pathology and cancer registry and cross-referenced with pediatric records. Patient demographics, clinical presentation, treatment, and outcomes were collected. A univariate descriptive analysis of the collected data was conducted. RESULTS: In our study period, 130 children were diagnosed with neuroblastoma, and 15 were diagnosed with ganglioneuroblastoma. There were 12 children with a paraneoplastic syndrome identified, 8 with NBL and 4 with ganglioneuroblastoma (GNBL). The average age at diagnosis was 22 months. All but 1 underwent resection prior to treatment of paraneoplastic syndrome, and 4 children required neoadjuvant therapy. Neurological complications were the most common with 10 children (83%). The average time from symptom onset to diagnosis was 0.7 months. Eight children had complete resolution of their symptoms after treatment and resection, 2 children recently started treatment within a year, 1 had partial resolution, and 1 died during treatment. The presence of tumor-infiltrating lymphocytes occurred in 4 children with neurologic paraneoplastic syndromes. Six children had neuropil rich tumors. CONCLUSION: The histological profile of paraneoplastic syndromes of neuroblastoma and ganglioneuroblastoma and their treatment across a single institution can be highly variable. The presence of tumor-infiltrating lymphocytes and neuropil may have an impact on paraneoplastic pathology.


Asunto(s)
Ganglioneuroblastoma , Enfermedades del Sistema Nervioso , Neuroblastoma , Síndromes Paraneoplásicos , Humanos , Niño , Lactante , Adolescente , Ganglioneuroblastoma/complicaciones , Ganglioneuroblastoma/diagnóstico , Ganglioneuroblastoma/cirugía , Estudios Retrospectivos , Neuroblastoma/complicaciones , Neuroblastoma/terapia , Neuroblastoma/patología , Síndromes Paraneoplásicos/terapia , Síndromes Paraneoplásicos/complicaciones
10.
Front Oncol ; 13: 1091274, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37007076

RESUMEN

Introduction: Wilms Tumor (WT), or nephroblastoma, is the most common pediatric kidney cancer. Most WTs display a "favorable" triphasic histology, in which the tumor is comprised of blastemal, stromal, and epithelial cell types. Blastemal predominance after neoadjuvant chemotherapy or diffuse anaplasia ("unfavorable" histology; 5-8%) portend a worse prognosis. Blastema likely provide the putative cancer stem cells (CSCs), which retain molecular and histologic features characteristic of nephron progenitor cells (NPCs), within WTs. NPCs arise in the metanephric mesenchyme (MM) and populate the cap mesenchyme (CM) in the developing kidney. WT blastemal cells, like NPCs, similarly express markers, SIX2 and CITED1. Tumor xenotransplantation is currently the only dependable method to propagate tumor tissue for research or therapeutic screening, since efforts to culture tumors in vitro as monolayers have invariably failed. Therefore, a critical need exists to propagate WT stem cells rapidly and efficiently for high-throughput, real-time drug screening. Methods: Previously, our lab developed niche conditions that support the propagation of murine NPCs in culture. Applying similar conditions to WTs, we assessed our ability to maintain key NPC "stemness" markers, SIX2, NCAM, and YAP1, and CSC marker ALDHI in cells from five distinct untreated patient tumors. Results: Accordingly, our culture conditions maintained the expression of these markers in cultured WT cells through multiple passages of rapidly dividing cells. Discussion: These findings suggest that our culture conditions sustain the WT blastemal population, as previously shown for normal NPCs. As a result, we have developed new WT cell lines and a multi-passage in vitro model for studying the blastemal lineage/CSCs in WTs. Furthermore, this system supports growth of heterogeneous WT cells, upon which potential drug therapies could be tested for efficacy and resistance.

11.
JAMA Otolaryngol Head Neck Surg ; 149(5): 464-465, 2023 05 01.
Artículo en Inglés | MEDLINE | ID: mdl-36892838

RESUMEN

An 11-year-old girl with no significant medical history was referred to the pediatric otolaryngology department for a painless, left-sided neck mass that had been slowly enlarging for 8 months. What is your diagnosis?


Asunto(s)
Cuello , Humanos , Niño , Diagnóstico Diferencial
12.
J Pediatr Surg ; 58(4): 702-707, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36670003

RESUMEN

BACKGROUND: The precision of minimally invasive surgery (MIS) to resect benign ovarian and paratubal masses while preserving adnexa in children is unclear. This study evaluated the integrity of laparoscopy to spare adnexa while resecting benign pathologies in children. METHODS: The institutional pathology database was queried to identify patients aged 18 years and younger having any ovarian or tubal lesion resected at a comprehensive children's hospital between 2006 and 2021. Adnexa-sparing surgery was defined as preserving both the ovary and tube from which the lesion was resected. Postoperative ultrasounds were reviewed to document ovarian follicles, blood flow, volumes, and lesion recurrence. RESULTS: Adnexal preservation was implemented in 168 of 328 pathological resections. MIS approach was used in 149 cases. Median age was 13.4 [11.0-15.3]. Among sparing surgeries, MIS associated with benign pathology, shorter operative time (median minutes: 78 MIS [59-111.5]; 130 open [92.8-149.8]; 174 MIS-to-open [132.8-199.5]; p = 0.010), and reduced hospital stay (median days: 1 MIS (Lindfors, 1971; Lovvorn III et al., 1998) [1-2]; 2 open [2-2.9], 2 MIS-to-open [1-3.3]; p = 0.001). Postoperative ovarian volume ipsilateral to the MIS procedure (median, 7.6 ml [4.3-12.1]) was relatively smaller than the contralateral ovary (median, 9.1 ml [5.5-15.0]). Blood flow was documented to the ovary in 70/94 (74.4%) of patients after MIS adnexal-sparing surgery. Distinct follicles were detected in 48/74 (64.8%) of post-menarchal patients after MIS adnexal-sparing surgery. Five ovarian cysts recurred. CONCLUSIONS: MIS preserves adnexa reliably, with postoperative ovarian follicles and blood flow detected in most patients, and maintains ∼80% of contralateral volume, while achieving definitive tumor resection. LEVEL OF EVIDENCE: III.


Asunto(s)
Laparoscopía , Quistes Ováricos , Femenino , Niño , Humanos , Adolescente , Anexos Uterinos/cirugía , Quistes Ováricos/cirugía , Laparoscopía/métodos , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Estudios Retrospectivos
13.
Am Surg ; 89(5): 1449-1456, 2023 May.
Artículo en Inglés | MEDLINE | ID: mdl-34855532

RESUMEN

BACKGROUND: Solid pseudopapillary tumors (SPTs) of the pancreas arise rarely in children, are often large, and can associate intimately with splenic vessels. Splenic preservation is a fundamental consideration when resecting distal SPT. Occasionally, the main splenic vessels must be divided to resect the SPT with negative margins, but the spleen can be preserved if the short gastric vessels remain intact (ie, Warshaw procedure). The purpose of this study was to evaluate outcomes of distal pancreatectomy (DP) for SPT in children and to highlight 2 cases of splenic preservation using the Warshaw procedure. METHODS: Patients 19 years and younger who were treated at a single children's hospital between July 2004 and January 2021 were examined. Patient characteristics were collected from the electronic medical record. A pediatric radiologist calculated SPT and pre- and post-operative (ie, non-infarcted) splenic volumes. RESULTS: Eleven patients received DP for SPT. Six DPs were performed open and 5 laparoscopically. The spleen was preserved in 3 open and 4 laparoscopic DPs. A laparoscopic Warshaw procedure was performed in 2 patients. Laparoscopic resection associated with less frequent epidural use (P = .015), shorter time to full diet (P = .030), and post-operative length of stay (P = .009), compared to open resection. Average residual splenic volume after the laparoscopic Warshaw procedure was 70% of preoperative volume. DISCUSSION: Laparoscopic DP for pediatric SPT achieved similar oncologic goals to open resection. Splenic preservation was feasible with laparoscopy in most cases and was successfully supplemented with the Warshaw procedure, which has not been previously reported for SPT resection in children.


Asunto(s)
Laparoscopía , Neoplasias Pancreáticas , Humanos , Niño , Neoplasias Pancreáticas/cirugía , Neoplasias Pancreáticas/patología , Páncreas/cirugía , Bazo/cirugía , Pancreatectomía/métodos , Laparoscopía/métodos , Resultado del Tratamiento
14.
Clin Exp Dermatol ; 47(12): 2234-2241, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-35988043

RESUMEN

BACKGROUND: Psoriasis is a chronic inflammatory skin disease associated with several important medical comorbidities. There are scant data available on the comorbidities of patients with psoriasis in South America. AIM: To examine the comorbidity profile of adult patients with psoriasis in Chile and its association with severity of psoriasis. METHODS: This was a multicentre, cross-sectional study involving 16 hospitals and clinics in Chile, which used a 48-item questionnaire to study clinician- and patient-reported outcomes and comorbidities. Inferential analyses were performed by psoriasis severity, using Fisher exact test, Student t-test and multivariable logistic regression. RESULTS: In total, 598 adult patients with psoriasis were included (51.1% male; mean age 49.2 ± 15.1 years); 48.5% mild and 51.4% moderate to severe; Psoriasis Area and Severity Index 11.6 ± 11.5; body surface area 14.7 ± 18.2%. Plaque psoriasis was the most common phenotype (90.2%), followed by guttate (13.4%). Psoriatic arthritis occurred in 27.3% of patients. Comorbidities were reported in 60.2% of all patients with psoriasis. Frequent concomitant diseases were obesity (25.3%), hypertension (24.3%), Type 2 diabetes mellitus (T2DM) (18.7%), dyslipidaemia (17.4%), metabolic syndrome (16.7%) and depression (14.4%). After adjustment, significant associations were found between moderate to severe psoriasis and obesity, T2DM and nonalcoholic fatty liver disease (NAFLD) compared with mild psoriasis. CONCLUSIONS: We report a large study of comorbidities, including depression, dyslipidaemia, T2DM and NAFLD, in people with psoriasis in Chile. The prevalence of comorbidities with psoriasis in Chile appears similar to that found in Western countries, and emphasizes the importance of assessing patients with psoriasis for risk factors for and presence of, comorbid disease in a multidisciplinary setting.


Asunto(s)
Diabetes Mellitus Tipo 2 , Dislipidemias , Enfermedad del Hígado Graso no Alcohólico , Psoriasis , Masculino , Femenino , Humanos , Enfermedad del Hígado Graso no Alcohólico/epidemiología , Chile/epidemiología , Diabetes Mellitus Tipo 2/epidemiología , Estudios Transversales , Psoriasis/epidemiología , Comorbilidad , Obesidad/epidemiología , Atención a la Salud
15.
Pediatr Dev Pathol ; 25(4): 458-465, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35452319

RESUMEN

PURPOSE AND CONTEXT: Civatte bodies (CB) are associated with cutaneous and mucosal lichen planus in adults. They are a distinct feature of Lichen Esophagitis Pattern, which is not well described in children. We characterized clinicopathologic associations of archival esophageal CB at our Children's Hospital to determine whether lichen planus or Lichen Esophagitis Pattern occurs in children. METHOD: Pathology records were queried for pediatric esophageal biopsy diagnoses containing "CB," "apoptosis, "necrosis," or "dyskeratosis." Cases with concurrent eosinophilic/acute esophagitis were excluded. H&E slides and clinical reports were reviewed. KEY RESULTS: Biopsies with CB or similar were identified from 19 patients and had been termed "dyskeratotic cells" in 8 reports. Patients had variable age and presenting symptoms, male predominance (74%), and frequent clinical history of polypharmacy (47%), Crohn disease (42%), and/or celiac disease (21%). Civatte bodies were prominent in the distal esophagus (95%), as few isolated cells (63%), and with variable chronic inflammation (absent, pauci-inflammatory, and lichen planus-like in approximately one-third of cases each). CONCLUSIONS: We show that esophageal CB from pediatric patients are under-recognized and may have different features and implications compared to Lichen Esophagitis Pattern in adults. Recognition and documentation of pediatric esophageal CB is needed to understand their clinical significance.


Asunto(s)
Esofagitis Eosinofílica , Liquen Plano , Líquenes , Adulto , Biopsia , Niño , Enteritis , Eosinofilia , Esofagitis Eosinofílica/diagnóstico , Femenino , Gastritis , Humanos , Liquen Plano/complicaciones , Liquen Plano/diagnóstico , Liquen Plano/patología , Masculino
16.
Am Surg ; 88(9): 2241-2243, 2022 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-35471864

RESUMEN

This case details the presentation and surgical management of a 15-year-old male patient with multiple endocrine neoplasia syndrome type 1 (MEN1) who required distal pancreatectomy for multiple nonfunctional pancreatic tumors. An intraoperative ultrasound was utilized to allow for proper location of the distal pancreatectomy, as well as visualization of the splenic vessel relationships and to ensure all lesions were contained within the specimen. Pathology demonstrated 5 well-differentiated neuroendocrine tumors with no evidence of malignancy. This case utilized innovative technology and a multidisciplinary approach in a challenging case to achieve a safe minimally invasive resection. The use of ultrasound intraoperatively provided confidence that all lesions had been identified, as well as demonstration of safe planes separate from the nearby vasculature.


Asunto(s)
Neoplasia Endocrina Múltiple Tipo 1 , Tumores Neuroendocrinos , Neoplasias Pancreáticas , Adolescente , Humanos , Laparoscopía/métodos , Masculino , Neoplasia Endocrina Múltiple Tipo 1/diagnóstico por imagen , Neoplasia Endocrina Múltiple Tipo 1/patología , Neoplasia Endocrina Múltiple Tipo 1/cirugía , Tumores Neuroendocrinos/diagnóstico por imagen , Tumores Neuroendocrinos/patología , Tumores Neuroendocrinos/cirugía , Pancreatectomía/métodos , Neoplasias Pancreáticas/diagnóstico por imagen , Neoplasias Pancreáticas/patología , Neoplasias Pancreáticas/cirugía , Ultrasonografía Intervencional/métodos
17.
Pediatr Surg Int ; 38(6): 817-824, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35338382

RESUMEN

PURPOSE: The majority of pediatric patients with choledochal cysts (CDC) are symptomatic prior to undergoing CDC excision. This study investigated the impact of surgical timing of CDC excision on postoperative outcomes among children. METHODS: We performed a retrospective review of 59 patients undergoing open CDC excision with Roux-Y hepaticojejunostomy between 2000 and 2020. Patients were grouped based on whether they underwent an electively scheduled or urgent CDC excision, as defined as CDC excision within the same admission due to CDC-related symptoms. Patient characteristics and perioperative data were compared between the two groups. RESULTS: Patients who underwent an elective surgery were older, had more Todani-type 1 CDC, and had decreased postoperative hospital length of stay and opioid use compared to patients who underwent CDC excision within the same admission due to CDC-related symptoms. No significant differences emerged regarding postoperative complications. Multivariable analysis showed that elective cyst excision (HR = 0.55, p = 0.04; HR = 0.59, p = 0.008) and type 1 CDC (HR = 0.32, p = 0.03; HR = 0.12, p < 0.001) were independently associated with decreased opioid use and postoperative hospital length of stay. CONCLUSIONS: Elective CDC excision is associated with shortened hospital stay and decreased opioid use among children compared to patients who undergo a CDC excision during the same admission for CDC-related symptoms.


Asunto(s)
Quiste del Colédoco , Laparoscopía , Analgésicos Opioides , Anastomosis en-Y de Roux , Niño , Quiste del Colédoco/diagnóstico , Quiste del Colédoco/cirugía , Humanos , Laparoscopía/efectos adversos , Complicaciones Posoperatorias/etiología , Estudios Retrospectivos , Resultado del Tratamiento
18.
Am J Ophthalmol Case Rep ; 25: 101274, 2022 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-35106404

RESUMEN

PURPOSE: To report a case of orbital rhabdomyosarcoma and highlight the treatment approach to the dilemma of a residual mass. OBSERVATIONS: An eleven-year-old boy was diagnosed with Stage 1, Group III embryonal rhabdomyosarcoma in the orbit. After completing a 24-week treatment regimen of chemotherapy and radiation, imaging showed a large persistent mass with erosion through the medial wall. It was uncertain whether the erosion was due to radiation osteonecrosis or to advancing tumor, creating a treatment dilemma for the providers. A repeat biopsy was planned. During the procedure, the mass was completely excised due to ease of removal, and the biopsy showed completely treated tumor. MRI surveillance at four years follow up showed that the patient remains tumor-free. CONCLUSIONS AND IMPORTANCE: Rhabdomyosarcoma was once a disease with a very poor outcome, but advances in imaging, chemotherapy, and radiation therapy have improved the prognosis of these patients. What was once a surgical disease treated with morbid resection is now predominantly a medical disease diagnosed with biopsy and treated with chemotherapy and radiation. However, such patients may have a residual mass after completing treatment. This situation presents a challenge, as it may not be clear whether the persistent mass is active tumor or treated tumor. This report describes the presentation and management of such a case in the orbit and demonstrates that a residual orbital mass may remain and represent completely treated tumor.

19.
Rev. argent. salud pública ; 14 (Suplemento COVID-19), 2022;14: 1-5, 02 Febrero 2022.
Artículo en Español | LILACS, ARGMSAL, BINACIS | ID: biblio-1379694

RESUMEN

INTRODUCCIÓN: Frente al aumento de casos de COVID-19 en el municipio bonaerense de Quilmes, y considerando las características sociodemográficas y habitacionales de su población, se puso en funcionamiento el Centro de Aislamiento Sanitario en la Universidad Nacional de Quilmes (CAS-UNQ) desde el 22 de abril hasta el 15 de noviembre de 2020. El objetivo de este trabajo fue describir el funcionamiento del CAS-UNQ y su contribución al abordaje de la primera ola de COVID 19 en el municipio de Quilmes de la Provincia de Buenos Aires. MÉTODOS: La organización general del CAS-UNQ estuvo a cargo del equipo de salud, que desplegó las estrategias necesarias para la atención de todas las personas alojadas. Las personas ingresadas se encontraban cursando cuadros leves de la enfermedad y no podían realizar el aislamiento en su domicilio. Se establecieron tres líneas de trabajo: atención médica, acompañamiento psicológico y abordaje social con perspectiva de derechos. RESULTADOS: Se aisló a 954 personas (407 mujeres adultas, 362 varones adultos y 185 niños menores de 13 años). El máximo de personas aisladas de forma simultánea fue de 120. La media de edad de internación fue de 27 años. DISCUSIÓN: La puesta en funcionamiento del CASUNQ fue una herramienta efectiva tanto para cortar cadenas de contagios en entornos de rápida propagación del virus como para implementar acompañamientos integrales de salud y acceso a derechos de la población.


Asunto(s)
Salud Pública , COVID-19
20.
Rev. argent. salud publica ; 14(supl.1): 52-52, feb. 2022. graf
Artículo en Español | LILACS-Express | LILACS | ID: biblio-1394712

RESUMEN

RESUMEN INTRODUCCIÓN: Frente al aumento de casos de COVID-19 en el municipio bonaerense de Quilmes, y considerando las características sociodemográficas y habitacionales de su población, se puso en funcionamiento el Centro de Aislamiento Sanitario en la Universidad Nacional de Quilmes (CAS-UNQ) desde el 22 de abril hasta el 15 de noviembre de 2020. El objetivo de este trabajo fue describir el funcionamiento del CAS-UNQ y su contribución al abordaje de la primera ola de COVID 19 en el municipio de Quilmes de la Provincia de Buenos Aires. MÉTODOS: La organización general del CAS-UNQ estuvo a cargo del equipo de salud, que desplegó las estrategias necesarias para la atención de todas las personas alojadas. Las personas ingresadas se encontraban cursando cuadros leves de la enfermedad y no podían realizar el aislamiento en su domicilio. Se establecieron tres líneas de trabajo: atención médica, acompañamiento psicológico y abordaje social con perspectiva de derechos. RESULTADOS: Se aisló a 954 personas (407 mujeres adultas, 362 varones adultos y 185 niños menores de 13 años). El máximo de personas aisladas de forma simultánea fue de 120. La media de edad de internación fue de 27 años. DISCUSIÓN: La puesta en funcionamiento del CASUNQ fue una herramienta efectiva tanto para cortar cadenas de contagios en entornos de rápida propagación del virus como para implementar acompañamientos integrales de salud y acceso a derechos de la población.


INTRODUCTION: In response to the increase of COVID-19 cases in the city of Quilmes (Buenos Aires Province), and considering the sociodemographic and housing characteristics of its population, a Sanitary Isolation Center at the National University of Quilmes (CASUNQ) was put into operation from April 22 to November 15, 2020. The objective of this work was to describe the operation of CAS-UNQ and its contribution to addressing the first wave of COVID 19 in Quilmes. METHODS: The general organization if the CAS-UNQ was conducted by a health team, which deployed the necessary strategies to take care of all the people housed there. Those who came to CAS-UNQ were experiencing mild illness and could not be isolated at home. Three lines of work were established: medical care, psychological support and social approach with a rights perspective. RESULTS: A total of 954 people were isolated (407 women, 362 men, and 185 children under 13 years of age). The maximum number of people isolated simultaneously was 120. The mean age of hospitalization was 27 years. DISCUSSION: The implementation of the CAS-UNQ was an effective tool both to cut chains of infections in environments of rapid spread of the virus and to carry out comprehensive health monitoring and access to rights of the population.

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