Abstract:
:We report the premortem and postmortem morphologic and histologic features and biochemical findings of a patient with long-chain 3-hydroxyacyl-CoA dehydrogenase (L-CHAD) deficiency and compare these with those described in previously reported cases of L-CHAD deficiency. In addition to chronic nonketotic hypoglycemia, hypotonia, and liver failure, this patient had chronic hemolytic anemia and delayed central nervous system myelination. These features have not been previously documented in L-CHAD deficiency.
journal_name
Arch Pathol Lab Medjournal_title
Archives of pathology & laboratory medicineauthors
Amirkhan RH,Timmons CF,Brown KO,Weinberger MJ,Bennett MJsubject
Has Abstractpub_date
1997-07-01 00:00:00pages
730-4issue
7eissn
0003-9985issn
1543-2165journal_volume
121pub_type
杂志文章abstract::Just as electronic health records are transforming the practice of medicine and health care information management, practicing in the era of the electronic health record offers opportunities, if not imperatives, for pathologists to take on new and "transformative" professional and leadership roles for the organization...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:10.5858/arpa.2013-0714-SO
更新日期:2015-03-01 00:00:00
abstract:CONTEXT:Lesions associated with monoclonal light and heavy chains display a variety of glomerular, tubular interstitial, and vascular manifestations. While some of the entities are well recognized, including light and heavy chain deposition diseases, AL (light chain) and AH (heavy chain) amyloidosis, and light chain ("...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章,多中心研究
doi:10.5858/arpa.2013-0493-OA
更新日期:2014-10-01 00:00:00
abstract::A case of cigarette smoking-related polyclonal B lymphocytosis, with binucleated cells, was observed. The condition was persistent for two years. We indicated the abnormal ultrastructural features and suggest that this unexplained syndrome could be a premalignant state. ...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1987-11-01 00:00:00
abstract:CONTEXT.—:Pathologist interobserver discordance is significant in grading of prostate cancer, limiting reliability. Diagnostic reproducibility may be improved with digital images, but adoption faces workflow, cost, and quality challenges. A novel digital method using an alternative tissue processing approach and novel ...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:10.5858/arpa.2020-0037-OA
更新日期:2020-09-29 00:00:00
abstract:CONTEXT:Impairment of venous outflow manifests as zone 3 sinusoidal dilatation and congestion (SDC) in liver biopsy. However, the finding of SDC is not specific for venous outflow impairment.Objectives.-To determine the specificity of SDC in liver biopsies for venous outflow impairment and to seek an explanation for SD...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章,评审
doi:10.1043/1543-2165(2004)128<901:SDACIL>2.0.CO;2
更新日期:2004-08-01 00:00:00
abstract::We report two unusual manifestations of multiple myeloma in a 71-year-old man. Late in the course of his disease, myeloma cells were observed in the patient's urinary sediment. At post mortem, the source of the cells was found to be a plasmacytoma of the urinary bladder. To our knowledge, this appears to be the first ...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1985-09-01 00:00:00
abstract:CONTEXT:Dysregulation of mediators of apoptosis is associated with carcinogenesis. For biliary duct cancers, p53 gene mutation is an important contributor to carcinogenesis. Mutations in the p53 gene affect transcription of the Fas gene, resulting in lack of Fas expression on cell membrane. It has been previously shown...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:10.1043/1543-2165(2005)129<481:ROAIC>2.0.CO;2
更新日期:2005-04-01 00:00:00
abstract::We report a case of focal infection of the brain with cytomegalovirus (CMV) in a 14-year-old boy with metastatic lymphoepithelioma (nasopharyngeal carcinoma). Cytomegalovirus-bearing subependymal glia were only seen in the fourth ventricle, in close proximity to tumor cells, these were associated with an intense infla...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1979-07-01 00:00:00
abstract::Early proliferative coronary atherosclerosis was produced in swine by feeding them a high-fat, high-cholesterol diet for 17 months, at which time one group of animals was killed (reference group), while the remainder was transferred for 12 months to a moderate diet that resulted in serum cholesterol levels of about 19...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1978-06-01 00:00:00
abstract::We describe a case of granulomatous encephalitis caused by Bipolaris (Drechslera) hawaiiensis in an immunocompetent patient. An 18-year-old man with a seven-month history of seizures and right leg weakness was found by computed tomographic scan to have a left frontoparietal enhancing lesion. Biopsy of the lesion revea...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1986-12-01 00:00:00
abstract::Discrepancies between the hematocrit values measured by automated methods and those measured by centrifugation have discouraged the use of the centrifuged microhematocrit to calibrate automated hematology analyzers. The discrepancy appears to be due to changes in the internal viscosity of red blood cells and can be el...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1987-04-01 00:00:00
abstract::Coccidioidomycosis has been reported in almost every tissue of the human body. The gastrointestinal tract and breast have been uniquely spared from this fungal infection. Despite the exceedingly large number of screening mammographies that are performed every year, to our knowledge subcutaneous breast tissue involveme...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1991-10-01 00:00:00
abstract::Adult rhabdomyoma is a benign, highly differentiated tumor of skeletal muscle. We observed transverse tubules and triads beside myofilaments in a rhabdomyoma from the submandibular area of a 66-year-old woman. Several types of muscle cells were present. In one, mitochondria abounded; in another, muscle filaments predo...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1981-11-01 00:00:00
abstract::Desmoplastic small round cell tumor typically presents with abundant desmoplastic stroma containing nested primitive round cells bearing a polyphenotypic immunohistochemical profile. Lesions with minimal classic morphology pose a formidable diagnostic challenge. The current case represents one such example, arising as...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:10.1043/1543-2165(2007)131[646:EMDSRC]2.0.CO;2
更新日期:2007-04-01 00:00:00
abstract::We describe the case of a 31-week fetus who died in utero with an invasive retroperitoneal kaposiform hemangioendothelioma. This rare vascular neoplasm usually presents as a localized violaceous skin lesion in infants and behaves in a benign fashion; however, kaposiform hemangioendothelioma may present as an invasive ...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:10.1043/1543-2165(2004)128<678:KHAWNF>2.0.CO;2
更新日期:2004-06-01 00:00:00
abstract:CONTEXT:Clear cell chondrosarcoma (CCC) is commonly considered to be a low-grade subtype of chondrosarcoma. However, a few cases of CCC behave as high-grade lesions (with early metastases or multiple/synchronous locations). OBJECTIVE:To investigate morphologic features that can help predict the aggressiveness of these...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:10.1043/1543-2165(2006)130[1673:ACCCDD]2.0.CO;2
更新日期:2006-11-01 00:00:00
abstract::Herein, we report a case of cutaneous angiosarcoma in a 35-year-old, morbidly obese woman. The tumor arose in the most dependent portion of the lower abdominal panniculus and showed typical changes of chronic lymphedema. The patient underwent a radical resection of her lower abdominal wall panniculus, which showed a m...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:10.1043/0003-9985(2001)125<0531:CACMO>2.0.CO;2
更新日期:2001-04-01 00:00:00
abstract::Although mucoepidermoid carcinoma is the most common primary malignancy of the salivary glands, the sclerosing morphologic variant of this tumor is extremely rare, with only 6 reported cases. As its name suggests, sclerosing mucoepidermoid carcinoma is characterized by an intense central sclerosis that occupies the en...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章,评审
doi:10.1043/1543-2165(2004)128<1046:SMCOTP>2.0.CO;2
更新日期:2004-09-01 00:00:00
abstract::Previous immunohistochemical studies of cardiac myxomas are few in number, limited in scope and, in part, discrepant. We studied the immunoreactivity of five cardiac myxomas for factor VIII-related antigen, Ulex europaeus agglutinin I, smooth-muscle myosin, alpha 1-antitrypsin, and alpha 1-antichymotrypsin. Positive s...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1986-02-01 00:00:00
abstract::In this report, we describe a highly unusual case of glandular malignant peripheral nerve sheath tumor presenting as a neck mass in a previously healthy 29-year-old man. Grossly, the tumor was found to arise from a swollen peripheral nerve trunk. The tumor was largely composed of spindle cells that demonstrated marked...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章,评审
doi:10.1043/0003-9985(2000)124<1364:GMPNST>2.0.CO;2
更新日期:2000-09-01 00:00:00
abstract:CONTEXT:No study has evaluated radiology/pathology correlation of percentage viable tumor (PVT) estimates in ablated hepatocellular carcinoma (HCC) to examine the reliability of radiologic estimates. OBJECTIVE:To determine how well interdisciplinary PVT estimates correlate and identify pathologic factors that influenc...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:10.5858/arpa.2012-0126-OA
更新日期:2013-03-01 00:00:00
abstract::Since it is believed that "a picture is worth a thousand words," incorporation of computer-generated line art was used as a adjunct to gross description in surgical pathology reporting in selected cases. The lack of an integrated software program was overcome by using commercially available graphic and word processing...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1993-01-01 00:00:00
abstract::Cytogenetic studies from the peripheral blood of a patient with malignant lymphoma and rheumatoid arthritis who was treated with intra-articular gold Au 198 revealed mosaicism with a normal female metaphase and a 43-chromosome metaphase. The abnormal cell line showed six missing normal chromosomes and three morphologi...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1980-09-01 00:00:00
abstract::A 72-year-old patient had a meningotheliomatous meningioma that invaded through the skull and into temporalis muscle. One year following craniotomy for removal of the neoplasm, he developed headaches, diplopia, and proptosis of the left eye. Biopsy of the orbital contents revealed a malignant supporting tissue neoplas...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1976-12-01 00:00:00
abstract::We have developed an inexpensive, microcomputer-based, multiuser hard disk system for surgical pathology that uses a commercially available word processor and data base manager, linked together by several BASIC programs written by us. System functions include keyboard input of patient demographic data and specimen inf...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1985-01-01 00:00:00
abstract::Diffuse persistent glomerular immaturity and focal proximal tubular ectasia were seen in bilateral open renal biopsy specimens for an infant with fluid and salt depletion and slowly progressive renal failure. Subsequently, diffuse tubulopapillary renal adenoma subtotally replaced each kidney, thereby, necessitating re...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1979-04-01 00:00:00
abstract::Rhabdoid tumor of the thyroid gland is a very rare neoplasm, characterized by significant metastatic potential. All of the 6 cases reported in the recent literature had poor outcomes. We report an additional case involving, to our knowledge, the oldest patient reported so far. A 67-year-old woman had a nodular goiter ...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:10.1043/1543-2165(2005)129
更新日期:2005-03-01 00:00:00
abstract::Evidence is presented that basophilic radial hairlines in the calcified layer of articular cartilage are in vivo microcracks. They were present in three of 25 normal patellas from subjects below the age of 50 years, in 20 of 25 recently fractured hips (average patient age, 77.3 years), and in 16 of 25 osteoarthritic t...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
doi:
更新日期:1993-02-01 00:00:00
abstract:CONTEXT.—:The diagnostic distinction of pulmonary neuroendocrine (NE) tumors from non-small cell lung carcinomas (NSCLCs) is clinically relevant for prognostication and treatment. Diagnosis is based on morphology and immunohistochemical staining. OBJECTIVE.—:To determine the diagnostic value of insulinoma-associated p...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章,评审
doi:10.5858/arpa.2019-0250-OA
更新日期:2020-09-01 00:00:00
abstract::Nephrogenic adenoma, also referred to as nephrogenic metaplasia, is an uncommon benign lesion of the urothelial tract, characterized by a circumscribed proliferation of tubules, cysts, and papillae lined by cells with low cuboidal to columnar epithelium. The diagnostic features that are useful in the recognition of th...
journal_title:Archives of pathology & laboratory medicine
pub_type: 杂志文章
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更新日期:2010-10-01 00:00:00