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1.
J Hand Surg Glob Online ; 5(3): 363-367, 2023 May.
Article in English | MEDLINE | ID: mdl-37323966

ABSTRACT

Disseminated coccidioidomycosis is a rare cause of flexor tendon tenosynovitis, particularly in the pediatric population. We present the case of a 2-month-old male infant with disseminated coccidioidomycosis of the right index finger, which was initially treated with debridement and long-term antifungal therapy. Six months after discontinuing antifungal medications and at the age of 2 years, the patient presented with relapse of coccidioidomycosis of his right index finger. Serial debridement and long-term antifungal therapy resulted in disease quiescence. The relapse of pediatric coccidioidomycosis tenosynovitis using surgical management with accompanying magnetic resonance imaging, histopathology, and intraoperative findings is described herein. Coccidioidomycosis should be considered for the differential diagnosis of pediatric patients who have traveled to or live in endemic areas and present with indolent hand infections.

2.
Pediatr Dev Pathol ; 20(6): 522-525, 2017.
Article in English | MEDLINE | ID: mdl-28590786

ABSTRACT

Crigler-Najjar syndrome is a hereditary unconjugated hyperbilirubinemia. Two forms of the disease are recognized. Type I is more severe and results in kernicterus if left untreated, and Type II is less severe and responds to phenobarbital. While Crigler-Najjar syndrome is thought by many to have normal liver histology, few reports of the liver pathology exist. Herein, we present a 19-year-old patient with Crigler-Najjar who underwent liver transplantation. The liver showed marked canalicular cholestasis with portal and variable, delicate, bridging fibrosis. Correlation of the patient's genetic test results and clinical phenotype is presented.


Subject(s)
Crigler-Najjar Syndrome/pathology , Heterozygote , Liver Cirrhosis/etiology , Liver/pathology , Crigler-Najjar Syndrome/diagnosis , Crigler-Najjar Syndrome/genetics , Crigler-Najjar Syndrome/surgery , Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Genetic Markers , Glucuronosyltransferase/genetics , Humans , Liver Cirrhosis/diagnosis , Liver Cirrhosis/pathology , Liver Transplantation , Male , Point Mutation , Receptors, Cell Surface/genetics , Sphingomyelin Phosphodiesterase/genetics , Young Adult
3.
Am J Surg Pathol ; 41(1): 128-133, 2017 01.
Article in English | MEDLINE | ID: mdl-27684993

ABSTRACT

Mesenteric tumor deposits (MTDs) are not included in the American Joint Committee on Cancer (AJCC) staging system for midgut small intestinal neuroendocrine tumors (NETs). We examined the prognostic significance of MTDs associated with midgut NETs. Hematoxylin and eosin slides from 132 resected jejunal/ileal NETs were reviewed for AJCC tumor stage, lymph node (LN) metastasis, MTDs, and hepatic metastases. MTDs were defined as discrete irregular mesenteric tumor nodules discontinuous from the primary tumor. Clinical or pathologic evidence of metastases and survival data were abstracted from electronic medical records. The cohort included 72 male and 60 female patients with a median age of 60 years. LN metastasis, MTDs, and liver metastasis were present in 80%, 68%, and 58% of patients, respectively. Female sex and presence of MTDs were independent predictors of liver metastasis. The odds ratio for hepatic metastasis in the presence of MTDs was 16.68 (95% confidence interval [CI], 4.66-59.73) and 0.81 (95% CI, 0.20-3.26) for LN metastasis. Age, MTDs, and hepatic metastasis were associated with disease-specific survival (DSS) in univariate analysis. Primary tumor histologic grade, pT3/T4 stage, and LN metastasis were not associated with DSS. Multivariate analysis of liver metastasis-free survival stratified by tumor grade showed that MTDs were associated with adverse outcomes. The hazard ratio for MTDs was 4.58 (95% CI, 1.89-11.11), compared with 0.98 (95% CI, 0.47-2.05) for LN metastasis. MTDs, but not LN metastasis, in midgut NETs are a strong predictor for hepatic metastasis and are associated with poor DSS.


Subject(s)
Intestinal Neoplasms/pathology , Mesentery/pathology , Neuroendocrine Tumors/pathology , Adult , Aged , Aged, 80 and over , Disease-Free Survival , Female , Humans , Intestinal Neoplasms/mortality , Intestine, Small/pathology , Kaplan-Meier Estimate , Liver Neoplasms/mortality , Liver Neoplasms/secondary , Lymphatic Metastasis/pathology , Male , Middle Aged , Neuroendocrine Tumors/mortality , Prognosis , Proportional Hazards Models , Young Adult
4.
Hum Pathol ; 46(10): 1427-36, 2015 Oct.
Article in English | MEDLINE | ID: mdl-26243671

ABSTRACT

Phenotypic differences between otherwise similar tumors arising from different gynecologic locations may be highly significant in understanding the underlying driver molecular events at each site and may potentially offer insights into differential responses to treatment. In this study, the authors sought to identify and quantify phenotypic differences between ovarian clear cell carcinoma (OCCC) and endometrial clear cell carcinoma (ECCC) using a proteomic approach. Tissue microarrays were constructed from tumor samples of 108 patients (54 ECCCs and 54 OCCCs). Formalin-fixed samples on microarray slides were analyzed by matrix-assisted laser desorption/ionization mass spectrometry, and 730 spectral peaks were generated from the combined data set. A linear mixed-effect model with random intercept was used to generate 93 (12.7%) peaks that were significantly different between OCCCs and ECCCs at the fold cutoffs of 1.5 and 0.667 and an adjusted P value cutoff of 1.0 × 10(-10). Liquid chromatography-tandem mass spectrometry was performed on selected cores from each group, and peptides identified therefrom were compared with lists of statistically significant peaks from the aforementioned linear mixed-effects model to find matches within 0.2 Da. A total of 53 candidate proteins were thus identified as being differentially expressed in OCCCs and ECCCs, 45 (85%) of which were expressed at higher levels in ECCCs than OCCCs. These proteins were functionally diverse and did not highlight a clearly dominant cellular theme or molecular pathway. Although ECCCs and OCCCs are very similar, some phenotypic differences are demonstrable. Additional studies of these differentially expressed proteins may ultimately clarify the significance of these differences.


Subject(s)
Adenocarcinoma, Clear Cell/classification , Biomarkers, Tumor/analysis , Endometrial Neoplasms/classification , Ovarian Neoplasms/classification , Proteomics/methods , Adenocarcinoma, Clear Cell/metabolism , Adult , Aged , Aged, 80 and over , Algorithms , Chromatography, Liquid , Endometrial Neoplasms/metabolism , Female , Humans , Immunohistochemistry , Middle Aged , Ovarian Neoplasms/metabolism , Phenotype , Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization , Tandem Mass Spectrometry , Tissue Array Analysis
6.
Clin Infect Dis ; 59(6): 845-50, 2014 Sep 15.
Article in English | MEDLINE | ID: mdl-24917656

ABSTRACT

BACKGROUND: Heartland virus (HRTV) is a tick-borne phlebovirus recently described in Missouri that is associated with fever, leukopenia, and thrombocytopenia. The virus has also been detected in Ambylomma americanum ticks. METHODS: Here we report the first fatal case of HRTV disease in an 80-year-old Tennessee resident. He was hospitalized with fever, confusion, leukopenia, and thrombocytopenia and developed multiorgan failure and hemorrhage. A tick-borne illness was suspected and testing for ehrlichiosis was negative. He died on hospital day 15, and autopsy specimens were tested for various pathogens as part of an unexplained death evaluation. RESULTS: HRTV antigens were detected in postmortem spleen and lymph nodes by immunohistochemistry, and HRTV was detected in premortem blood by reverse transcription polymerase chain reaction and by isolation in cell culture. CONCLUSIONS: This case demonstrates that HRTV infection can cause severe disease and death and expands the geographic range of HRTV within the United States.


Subject(s)
Phlebotomus Fever/virology , Phlebovirus , Aged, 80 and over , Fatal Outcome , Humans , Male , Phlebotomus Fever/diagnosis , Phlebotomus Fever/therapy , Phlebovirus/classification , Pulmonary Disease, Chronic Obstructive/complications , Risk Factors , Serotyping , Tennessee
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