Abstract:
:Glycogen branching enzyme deficiency/Andersen disease can manifest with a spectrum of clinical phenotypes, making the diagnosis difficult. An 11-year-old Pakistani boy presented with a history of progressive weakness and delayed milestones. Echocardiography showed features of dilated cardiomyopathy. He was suspected to have congenital myopathy and was evaluated further. Muscle biopsy showed subsarcolemmal accumulation of basophilic material, which stained positively with Periodic acid-Schiff reagent (diastase-resistant). Ultrastructural examination revealed accumulation of structurally abnormal forms of filamentous glycogen, confirming the diagnosis as Andersen disease. As histopathological and immunohistochemical evaluation of muscle biopsies is not always diagnostic, ultrastructural examination may serve as a valuable adjunct in difficult cases.
journal_name
Ultrastruct Patholjournal_title
Ultrastructural pathologyauthors
Kakkar A,Sharma MC,Nambirajan A,Sarkar C,Suri V,Gulati Sdoi
10.3109/01913123.2015.1014612subject
Has Abstractpub_date
2015-01-01 00:00:00pages
293-7issue
4eissn
0191-3123issn
1521-0758journal_volume
39pub_type
杂志文章abstract:BACKGROUND:Proteinuria is an uncommon clinical manifestation of IgA nephropathy and is usually seen in cases with severe lesions like endocapillary proliferation. However, it is occasionally seen even with cases with mild glomerular manifestations and may even be of nephrotic range. PREDICTOR:Podocyte foot process eff...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.3109/01913123.2014.960543
更新日期:2015-04-01 00:00:00
abstract::We report here ultrastructural and immunohistochemical studies of neuroblastic differentiation in the retrospective (n = 17) and prospective (n = 26) series of primitive neuroectodermal tumors (PNETs). By electron microscopy, neuritelike structures containing parallel-oriented microtubules, adhesive plaque junctions, ...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.3109/01913129509064217
更新日期:1995-05-01 00:00:00
abstract::A metastasizing carcinoid of the gastric stump found 25 years after Billroth II gastric resection for duodenal ulcer is described. Electron microscopy and optical endocrine cell staining proved the tumor to be composed of enterochromaffinlike (ECL) cells. This unusual combination further shows that, at variance with m...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.3109/01913129109021888
更新日期:1991-05-01 00:00:00
abstract::The aim of this study was to examine the effect of the different protein supports in the cryopreservation solution on improving human ovarian tissue preservation after frozen-thawed procedures. Biopsies of ovarian cortical tissue were obtained from 14 subjects. All specimens were cryopreserved using a slow freezing/ra...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.1080/01913120600820187
更新日期:2006-07-01 00:00:00
abstract::A slowly growing tumor in the right shoulder of a 38-year-old white male, which felt like a superficial cystic mass, was studied. The spindle cells, which represented the main component of the tumor, were arranged in a typical storiform pattern and were positive for CD34 and focally for CD117. The pigmented cells were...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.1080/01913120309935
更新日期:2003-05-01 00:00:00
abstract::By means of optical and electron microscopy and by atomic absorption spectrophotometry in a graphite chamber, this study evaluated the effect of temperature (22-35 degrees C) on lesions in the kidney, liver, and brain, and on concentrations of lead caused by the administration of 2 and 5 mg/kg/IP of lead acetate to Sw...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.3109/01913129609016324
更新日期:1996-05-01 00:00:00
abstract::Recently, interest has been increasing for human decellularized matrices, due to their ability to reduce numerous side effects related to hernia repair. To date, only animal studies investigated the biological interaction post-implant of human decellularized matrices for soft tissue repair. Therefore, the aim of this ...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.1080/01913123.2017.1365788
更新日期:2018-01-01 00:00:00
abstract::Electron-microscopic analysis of the ultrastructure of the Krebs-2 carcinoma ascites cells in the first 90 min immediately after their exposure to fragmented double-stranded DNA has been performed. Morphological attributes of the treated cancer cells indicate the induction in these cells of destructive processes of pr...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.1080/01913123.2019.1575499
更新日期:2019-01-01 00:00:00
abstract::Two problematic spindle cell sarcomas involving upper jawbones in two adult male patients have been studied by histology, immunohistochemistry, and transmission electron microscopy, and respectively graded as low-grade malignancy and high-grade malignancy. While any single methodological study did not allow confident ...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.1080/019131201317101261
更新日期:2001-09-01 00:00:00
abstract::The electron microscope has been used with great skill in many aspects of the acquired immunodeficiency syndrome. It has played a critical role in classifying the human immunodeficiency virus, in characterizing the morphogenesis and gene products of the virus, and in elucidating the host cell targets and interactions....
journal_title:Ultrastructural pathology
pub_type: 杂志文章,评审
doi:10.3109/01913129209074561
更新日期:1992-01-01 00:00:00
abstract::As You Like It, Part 3, is a continuation of the lectures given by the author (Ultra Path VIII and Ultra Path IX). It is a critical historical review of topics of interest to electron microscopists, attempting to show what went wrong and perhaps also why. The topics chosen this time demonstrate the prominent role elec...
journal_title:Ultrastructural pathology
pub_type: 历史文章,杂志文章,评审
doi:10.1080/019131201300343874
更新日期:2001-05-01 00:00:00
abstract::Pleomorphic liposarcoma (PL) is the least common subtype of liposarcoma, displaying a lipoblastic, malignant fibrous histiocytoma (MFH)-like and, less frequently, an epithelioid growth pattern. The epithelioid morphology in PL is still underrecognized and may closely simulate other epithelial neoplasms, mainly adrenal...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.1080/01913120290104575
更新日期:2002-09-01 00:00:00
abstract::Dysembryoplastic neuroectodermal tumor (DNT) is a rare brain neoplasm. Although the tumor pathology is relatively well charactererized, its full clinicopathological spectrum is still discussed, and ultrastructural data on it are very limited. Here, the authors describe detailed ultrastructural pathology of 7 cases of ...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.1080/019131201753343494
更新日期:2001-11-01 00:00:00
abstract::This study was aimed at identifying ultrastructural abnormalities that may be present in the duodenum of patients with chronic renal failure who were undergoing hemodialysis and that may have an effect on the normal absorptive function of the duodenum. Duodenal biopsy specimens from ten patients and four controls were...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.3109/01913129009050868
更新日期:1990-01-01 00:00:00
abstract::Recent studies suggest that nerve growth factor (NGF) protects olfactory cells and axons from injury in vitro. Eighteen Wistar-Albino rats randomly divided into three groups: control group, diabetic group without NGF, and diabetic with NGF. Intranasal NGF (6 µg/day) was administered over a 5-day period. At the end of ...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.1080/01913123.2018.1463337
更新日期:2018-05-01 00:00:00
abstract::This report ultrastructurally illustrates the giant lysosome-related organelles in the various cellular components of a renal biopsy from a 10 year-old female affected by the Chédiak-Higashi syndrome. Albeit similar observations have been made and reported in animal models of the syndrome, to the author's knowledge, t...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.3109/01913123.2011.584656
更新日期:2011-08-01 00:00:00
abstract::This study evaluated, by SEM, the morphology of human primary teeth roots. Twenty-four teeth were divided into 3 groups: pulp vitality (group I) and pulp necrosis without (group II) and with apical periodontitis (group III). Roots were analyzed by the presence of periodontal ligament (PDL) fibers and resorption areas....
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.3109/01913120903275642
更新日期:2009-10-01 00:00:00
abstract::A case of myoepithelial carcinoma arising in a benign myoepithelioma of the minor salivary gland in a 71-year-old patient is reported. The tumor presented initially on the palate and had been diagnosed as "benign lesion" 40 years before. It recurred 22, 36, and 40 years after initial presentation, and a similar histop...
journal_title:Ultrastructural pathology
pub_type: 杂志文章,评审
doi:10.3109/01913129609016309
更新日期:1996-03-01 00:00:00
abstract::Pulmonary blastomas are rare neoplasms typically occurring in patients of pediatric age, clinically characterized by fever, respiratory distress, and radiologic findings of a pulmonary cystic and/or solid mass with partial or complete obliteration of emithorax. Their behavior is aggressive and outcome is poor due to f...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.1080/01913120500323456
更新日期:2005-11-01 00:00:00
abstract::Finger pulp biopsies of 31 patients suffering from Raynaud's syndrome (RS) were investigated by light and electron microscopy. The patients suffered from various underlying diseases. Interest was focused on the vascular changes and nerve and connective tissue alterations, especially the elastic fiber changes. In the d...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.3109/01913128209016642
更新日期:1982-04-01 00:00:00
abstract::Adult neuroblastoma is an uncommon tumor and it is rare for one to present as a primary bone lesion. We report the case of a 23-year-old man in whom the tumor initially appeared in the right scapula. Light microscopic, immunocytochemical, and electron microscopic findings were typical of neuroblastoma. ...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.3109/01913128709048440
更新日期:1987-01-01 00:00:00
abstract::Transmission electron microscopy has played a key role in our understanding of the human immunodeficiency virus and the opportunistic infections that accompany HIV disease. This paper describes features of HIV production; HHV-8, the virus that is associated with Kaposi sarcoma; Trachipleistophora anthropophthera, a ne...
journal_title:Ultrastructural pathology
pub_type: 杂志文章,评审
doi:10.1080/01913120290104502
更新日期:2002-07-01 00:00:00
abstract::A technique for immunoelectron microscopy is described whereby tissue is embedded in large (6 x 12 mm) plastic blocks and reembedded ("popped off") directly from semithin (3-microns) sections for thin sectioning. This technique is particularly useful in locating relatively sparse cells or tissue structures. Three acry...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.3109/01913129209074550
更新日期:1992-01-01 00:00:00
abstract::The clinicopathologic, immunocytochemical, and electron microscopic features of a case of meningeal melanocytoma arising in the phylum terminale are reported. Meningeal melanocytoma is an uncommon tumor that must be distinguished from metastatic or primary malignant melanoma, meningeal melanocytic nevi, pigmented meni...
journal_title:Ultrastructural pathology
pub_type: 杂志文章,评审
doi:10.3109/01913129809103356
更新日期:1998-07-01 00:00:00
abstract::The aim of this study was to investigate the morphologic and ultrastructural features of biofilms of slow and fast-growing mycobacteria in different stress conditions, presence and absence of oleic acid albumin dextrose catalase (OADC) enrichment and at different temperatures: 30, 37 and 42 °C. Four hundred mycobacter...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.3109/01913123.2014.950780
更新日期:2015-02-01 00:00:00
abstract::An intestinal-type epithelium is often present at columnar-lined esophagus, gastroesophageal junction or within the so-called short segment Barrett's esophagus, but ultrastructural study failed to detect enterocytes in columnar-lined esophagus. The authors have analyzed the intestinal aspects present in areas of colum...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.1080/01913120252959281
更新日期:2002-03-01 00:00:00
abstract::As the name suggests, the histogenesis of the granular cell tumor is uncertain, but this intriguing neoplasm has occasioned much speculation and controversy since its description in 1926 as a myoblastoma. Though ubiquitous in its anatomic location, it is usually small, solitary, benign, and readily recognized by routi...
journal_title:Ultrastructural pathology
pub_type: 杂志文章,评审
doi:10.1080/019131299281545
更新日期:1999-07-01 00:00:00
abstract::A 15-year-old girl presented with a small, indolent mass near the knee joint. Light microscopy revealed a peculiar myxoid plexiform tumor composed of cytologically bland cells. Interpretation of the initial biopsy material was controversial. Subsequent immunohistochemical studies revealed tumor cells to be strongly re...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.3109/01913129309027771
更新日期:1993-05-01 00:00:00
abstract::Electrophoresis revealed two cases of malignant lymphoma that each contained three M-proteins (IgM lambda.lgG kappa.lgG lambda and IgM lambda.IgM kappa.lgG kappa) in the sera. To determine cellular origin of each M-protein, atypical lymphoid and plasmacytoid cells of both cases were examined by electron microscopy. At...
journal_title:Ultrastructural pathology
pub_type: 杂志文章,评审
doi:10.3109/01913129809032277
更新日期:1998-11-01 00:00:00
abstract::Electron microscopic investigation of biopsy materials from Kaposi sarcoma (KS) skin lesions of 2 African AIDS patients occasionally revealed fungal cells within the tumor tissue. The spherical cells were surrounded by a triple-layered cell wall and were not encapsulated. The ultrastructural characteristics of the cel...
journal_title:Ultrastructural pathology
pub_type: 杂志文章
doi:10.1080/01913120500520960
更新日期:2006-05-01 00:00:00