scholarly journals Carney Triad in an Adult with Aggressive Behavior: The First Case in Korea

2009 ◽  
Vol 50 (5) ◽  
pp. 709 ◽  
Author(s):  
Hye-Jong Song ◽  
Kyoung-Mee Kim ◽  
Dong Il Choi ◽  
Cheol Keun Park
Keyword(s):  
Aggressive Behavior ◽  
Carney Triad ◽  
First Case

scholarly journals Case Report: Five Adult Cases of H3K27-Altered Diffuse Midline Glioma in the Spinal Cord

2021 ◽  
Vol 11 ◽  
Author(s):  
Quanquan Gu ◽  
Yajing Huang ◽  
Hao Zhang ◽  
Biao Jiang
Keyword(s):  
Spinal Cord ◽  
Case Report ◽  
Aggressive Behavior ◽  
Progression Free Survival ◽  
Central Canal ◽  
Rapid Progression ◽  
High Grade ◽  
First Case ◽  
Diffuse Midline Glioma

BackgroundDiffuse midline glioma with H3K27-altered (DMG-H3K27a) is a novel tumor entity of the pediatric-type diffuse high-grade tumor in the latest WHO CNS 5. It mostly affects children and is only rarely found in adults. The tumor has a high level of aggressiveness, with a rapid progression and bad prognosis. In adults, the spinal cord is the most common site of DMG-H3K27a. Rare adult cases of primary DMG-H3K27a in the spinal cord were reported in this study, together with clinico-histopathologico-radiographic data.MethodsFrom January 2016 to December 2020, we conducted a retrospective study of five adults with primary DMG-H3K27a in the spinal cord, analyzing their clinical, pathohistological, and radiographic datasets from the first diagnosis to follow-up.ResultsAll five patients were diagnosed for the first time and were given full treatment. In three of the five patients, post-operative follow-up revealed tumor recurrence. The longest survival of the five patients was 45 months at the time of report submission, while the longest progression-free survival (PFS) following surgery was 20 months. Immunohistochemical studies showed the tumors featured aggressive behavior (grade 4) and were positive for the H3K27M mutation. The radiographic appearances were varied, but they were all initially mistaken as benign. DMG-H3K27a in the spinal cord was characterized by isointense/hyperintense on T1WI and isointense/hyperintense on T2WI, as well as cystic necrosis and peripheral spinal cord edema, as well as central canal enlargement and other types of enhancement.ConclusionThis is the first case report focusing on adult DMG-H3K27a of the pediatric-type diffuses high-grade gliomas in the spinal cord. In our cases, we discovered the following: 1) adults had a better prognosis with a longer PFS compared with prior pediatric reports; 2) despite aggressive behavior under the microscope, radiographic appearances of the tumors were less aggressive; and 3) adjuvant treatment, including TCM, may have played a role in the prognosis.

IGH-Mediated Translocations, Recurrent in Classic Hodgkin Lymphoma, Frequently Correlate with an Aggressive Behavior

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 2922-2922
Author(s):  
Iwona Wlodarska ◽  
Daan Dierickx ◽  
Ursula Pluys ◽  
Kathleen Doms ◽  
Carlos Graux ◽  
...  
Keyword(s):  
Aggressive Behavior ◽  
B Cell ◽  
Hodgkin Lymphoma ◽  
Lymphoblastic Leukemia ◽  
Cell Phenotype ◽  
First Case ◽  
Classic Hodgkin Lymphoma ◽  
Cell Acute Lymphoblastic Leukemia ◽  
Bcl2 Protein

Abstract Genetic studies of classic Hodgkin lymphoma (cHL) are hampered by a low content of neoplastic Reed-Stenberg cells (RSCs) in tumor mass. Therefore, cytogenetic features of cHL remain poorly characterized. Previous interphase FISH (iFISH) studies, however, suggest that RSCs which carry 'crippling' IGH mutations and lost B cell phenotype, are recurrently featured by t(IGH)-related rearrangements (PMID: 17079453, 18940474). Recently, we have identified 14q32/IGH aberrations in karyotypes of two patients with nodular sclerosis HL (NSHL) analyzed at time of relapse. The first case showed the classic follicular lymphoma-associated t(14;18)(q32;q21)/IGH-BCL2 rearrangement which correlated with expression of the BCL2 protein (as demonstrated by immunohistochemistry). The second case revealed inv(14)(q11q32)/IGH-CEPBE, originally identified in B cell acute lymphoblastic leukemia. To identify additional cHL cases with 14q32/IGH aberrations and check whether these rearrangements correlate with clinical outcome, we screened by RSC-focused iFISH: (i) 46 relapsed cHL, (ii) 40 cHL with a long-term remission, and (iii) 60 prospective cases. Altogether, IGH rearrangements were detected in 28 patients, including 17 representing the former group (35.4%), four cases representing the second group (10%) and seven prospective cases (11.6%). FISH pattern of LSI IGH suggested a reciprocal t(14q32/IGH) in 20 cases, nonreciprocal t(14q32/IGH) in four cases, inv(14q32/IGH) or ins(14q32/IGH) in three cases and interstitial del(14q32/IGH) in one case. Twenty six cases were further analyzed by iFISH with break-apart (BA) assays for BCL2, BCL3, BCL6 and MYC. In three cases with a presumed inv(14)/ins(14), CEPBE BA was applied. Altogether, we have identified the IGH-BCL2 rearrangement in two cases, including the index case with t(14;18), and the IGH-CEPBE rearrangement in the case with inv(14). All the remaining cases showed a non-rearranged status of the examined loci. The cases were additionally examined with the JAK2/PDLs (9p24) probes; amplification of signals was observed in six cases (21.5%) and FISH pattern suggestive of the PDLs rearrangement was found in two cases (7%). The majority of patients (18/28) with t(14q32/IGH) were diagnosed with NSHL. There were 18 male and 10 female patients in age ranging from 18 to 91 years (mean 50). Patients presented with both early (10/26) and advanced (16/26) stage (two not staged). All were treated with chemotherapy with or without radiotherapy, except for two patients who were subjected to a palliative treatment. After induction therapy, complete and partial remission was achieved in 17 and 5 patients, respectively. Thirteen patients are alive (11 without disease and 2 with disease); 13 patients died, including 11 whose death was related with a disease, and two are lost for follow up. In summary, we confirmed that chromosomal aberrations involving 14q32/IGH occur recurrently in cHL. These rearrangements rarely target BCL2 and CEPBE, and do not affect BCL3, BCL6 and MYC, the oncogenes frequently affected in B-NHL. Interestingly, 17 out of 21 (81%) patients with t(14q32/IGH) and long follow up showed an aggressive behavior. Our study allows a new insight into the pathogenesis of cHL and might help in further stratification of cHL patients. Disclosures Graux: Amgen: Honoraria; Celgene: Honoraria; Novartis: Honoraria.

Hemoglobin crystals of the red blood cells in the human renal cortex: electron microscopic observations of the renal lithiasis

1978 ◽  
Vol 36 (2) ◽  
pp. 480-481
Author(s):  
Kosuke Ueda ◽  
Hiroto Washida ◽  
Nakazo Watari
Keyword(s):  
Red Blood Cells ◽  
Blood Cells ◽  
Renal Cortex ◽  
Uranyl Acetate ◽  
Osmic Acid ◽  
Renal Lithiasis ◽  
Electron Microscopic ◽  
Hemoglobin C ◽  
First Case ◽  
Ultrathin Sections

IntroductionHemoglobin crystals in the red blood cells were electronmicroscopically reported by Fawcett in the cat myocardium. In the human, Lessin revealed crystal-containing cells in the periphral blood of hemoglobin C disease patients. We found the hemoglobin crystals and its agglutination in the erythrocytes in the renal cortex of the human renal lithiasis, and these patients had no hematological abnormalities or other diseases out of the renal lithiasis. Hemoglobin crystals in the human erythrocytes were confirmed to be the first case in the kidney.Material and MethodsTen cases of the human renal biopsies were performed on the operations of the seven pyelolithotomies and three ureterolithotomies. The each specimens were primarily fixed in cacodylate buffered 3. 0% glutaraldehyde and post fixed in osmic acid, dehydrated in graded concentrations of ethanol, and then embedded in Epon 812. Ultrathin sections, cut on LKB microtome, were doubly stained with uranyl acetate and lead citrate.

Electron-diffraction studies in synthetic polymer materials

1983 ◽  
Vol 41 ◽  
pp. 362-365
Author(s):  
D.T. Grubb
Keyword(s):  
Electron Diffraction ◽  
Synthetic Polymer ◽  
Polymer Materials ◽  
Crystallographic Structure ◽  
High Dose ◽  
Electron Dose ◽  
Dose Rates ◽  
First Case ◽  
Diffraction Patterns ◽  
The Many

Diffraction studies in polymeric and other beam sensitive materials may bring to mind the many experiments where diffracted intensity has been used as a measure of the electron dose required to destroy fine structure in the TEM. But this paper is concerned with a range of cases where the diffraction pattern itself contains the important information.In the first case, electron diffraction from paraffins, degraded polyethylene and polyethylene single crystals, all the samples are highly ordered, and their crystallographic structure is well known. The diffraction patterns fade on irradiation and may also change considerably in a-spacing, increasing the unit cell volume on irradiation. The effect is large and continuous far C94H190 paraffin and for PE, while for shorter chains to C 28H58 the change is less, levelling off at high dose, Fig.l. It is also found that the change in a-spacing increases at higher dose rates and at higher irradiation temperatures.

Small-Angle Electron Scattering (SAES) of Polymers

1985 ◽  
Vol 43 ◽  
pp. 78-79
Author(s):  
Ralph Oralor ◽  
Pamela Lloyd ◽  
Satish Kumar ◽  
W. W. Adams
Keyword(s):  
Electron Scattering ◽  
Small Angle ◽  
Angular Resolution ◽  
Structural Features ◽  
Objective Lens ◽  
High Angular Resolution ◽  
Push Button ◽  
First Case ◽  
Diffraction Mode ◽  
Very High

Small angle electron scattering (SAES) has been used to study structural features of up to several thousand angstroms in polymers, as well as in metals. SAES may be done either in (a) long camera mode by switching off the objective lens current or in (b) selected area diffraction mode. In the first case very high camera lengths (up to 7Ø meters on JEOL 1Ø ØCX) and high angular resolution can be obtained, while in the second case smaller camera lengths (approximately up to 3.6 meters on JEOL 1Ø ØCX) and lower angular resolution is obtainable. We conducted our SAES studies on JEOL 1ØØCX which can be switched to either mode with a push button as a standard feature.

Impairment Tutorial: Functionally Limiting Upper Extremity Pain: Controversies in Impairment Rating

2003 ◽  
Vol 8 (5) ◽  
pp. 4-12
Author(s):  
Lorne Direnfeld ◽  
James Talmage ◽  
Christopher Brigham
Keyword(s):  
Upper Extremity ◽  
Radicular Pain ◽  
Illness Behavior ◽  
Orthopedic Surgeon ◽  
First Case ◽  
Clinical Presentations ◽  
Whole Person ◽  
Physical Examinations ◽  
Upper Extremity Impairment ◽  
The Individual

Abstract This article was prompted by the submission of two challenging cases that exemplify the decision processes involved in using the AMA Guides to the Evaluation of Permanent Impairment (AMA Guides). In both cases, the physical examinations were normal with no evidence of illness behavior, but, based on their histories and clinical presentations, the patients reported credible symptoms attributable to specific significant injuries. The dilemma for evaluators was whether to adhere to the AMA Guides, as written, or to attempt to rate impairment in these rare cases. In the first case, the evaluating neurologist used alternative approaches to define impairment based on the presence of thoracic outlet syndrome and upper extremity pain, as if there were a nerve injury. An orthopedic surgeon who evaluated the case did not base impairment on pain and used the upper extremity chapters in the AMA Guides. The impairment ratings determined using either the nervous system or upper extremity chapters of the AMA Guides resulted in almost the same rating (9% vs 8% upper extremity impairment), and either value converted to 5% whole person permanent impairment. In the second case, the neurologist evaluated the individual for neuropathic pain (9% WPI), and the orthopedic surgeon rated the patient as Diagnosis-related estimates Cervical Category II for nonverifiable radicular pain (5% to 8% WPI).

Histological verification of non-specific aorta-arteritis (Takayasu's arteritis) using percutaneous transluminal atherectomy

VASA ◽  
2004 ◽  
Vol 33 (4) ◽  
pp. 247-251 ◽  
Author(s):  
Zeller ◽  
Koch ◽  
Frank ◽  
Bürgelin ◽  
Schwarzwälder ◽  
...  
Keyword(s):  
Clinical Diagnosis ◽  
Axillary Artery ◽  
Takayasu’S Arteritis ◽  
Giant Cells ◽  
Interventional Therapy ◽  
Takayasu's Arteritis ◽  
New Device ◽  
First Case ◽  
Anti Inflammatory ◽  
Percutaneous Transluminal

Diagnosis of non-specific aorto-arteritis (NSAA, Takaysu's arteritis) is typically based on clinical and investigational parameters. We report here about two patients with clinically suspected diagnosis of a Takayasu's arteritis already under anti-inflammatory therapy in whom percutaneous transluminal atherectomy of subclavian and axillary artery stenoses was performed to relief the patients from symptoms – intermittent dyspraxia of the arms – and to verify the clinical diagnosis by histology. In the first case aorto-arteritis could be histologically confirmed through the analysis of plaque material including media structures excised from the subclavian and axillary arteries using a new device for atherectomy. The biopsy showed diffuse inflammation and granulomatous lesions with giant cells typically for Takayasu's disease. In the second patient, biopsy showed no acute or chronic inflammatory signs but only atherosclerotic lesions. Percutaneous transluminal atherectomy is therefore not only an interventional but also a diagnostic tool and should be used in every case of interventional therapy of suspected aorto-arteritis to make the clinical diagnosis and as a major consequence the initiation of an aggressive anti-inflammatory medical therapy more reliable.

Endoscopic mucosal resection for recurrent gastric antral vascular ectasia

VASA ◽  
2008 ◽  
Vol 37 (3) ◽  
pp. 289-292 ◽  
Author(s):  
Katsinelos ◽  
Chatzimavroudis ◽  
Katsinelos ◽  
Panagiotopoulou ◽  
Kotakidou ◽  
...  
Keyword(s):  
Endoscopic Mucosal Resection ◽  
Argon Plasma Coagulation ◽  
Argon Plasma ◽  
Deficiency Anemia ◽  
Gastric Antral Vascular Ectasia ◽  
Therapeutic Approaches ◽  
Plasma Coagulation ◽  
Vascular Ectasia ◽  
Mucosal Resection ◽  
First Case

Gastric antral vascular ectasia (GAVE) is an overt or occult source of gastrointestinal bleeding. Despite several therapeutic approaches have been successfully tested for preventing chronic bleeding, some patients present recurrence of GAVE lesions. To the best of our knowledge, we report the first case, of a 86-year-old woman who presented severe iron-deficiency anemia due to GAVE and showed recurrence of GAVE lesion despite the intensive argon plasma coagulation treatment. We performed endoscopic mucosal resection of bleeding GAVE with resolution of anemia.

Adult-to-Adult Living-Donor Liver Transplantation

Swiss Surgery ◽  
2003 ◽  
Vol 9 (5) ◽  
pp. 227-236 ◽  
Author(s):  
Majno ◽  
Mentha ◽  
Berney ◽  
Bühler ◽  
Giostra ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Living Donor ◽  
Living Donor Liver Transplantation ◽  
Therapeutic Option ◽  
Donor Liver ◽  
Donor Liver Transplantation ◽  
Multidisciplinary Program ◽  
First Case ◽  
Living Donor Transplantation ◽  
The Right

Living donor liver transplantation is a relatively new procedure in which the right side of the liver is harvested in a healthy donor and transplanted into a recipient. After the first case in 1994, over 3000 cases have been done worldwide. This review summarizes the reasons why the procedure is needed, describes its main technical aspects, highlights the boundaries in which it can be done safely, summarizes the current experience worldwide and describes the main points of the program in our unit. We argue that living-donor transplantation is a viable alternative to a long time on the waiting list for several patients, and it can be performed safely and successfully provided that all precautions are undertaken to minimize the risks in the donor and to increase the chances of a good outcome in the recipients. If these prerequisites are met, and within the framework of a structured multidisciplinary program, we believe that living-donor liver transplantation should be funded by health insurances as a recognized therapeutic option.

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