scholarly journals Pathogenesis and Classification of Paracocidioidomycosis: New Insights From Old Good Stuff

2020 ◽  
Author(s):  
Gil Benard ◽  
Gilda Del Negro
Keyword(s):  
Natural History ◽  
Case Reports ◽  
Case Series ◽  
Current View ◽  
Clinical Classification ◽  
Chronic Form

Abstract Different classifications of paracoccidioidomycosis emerged since its discovery in 1908, culminating in the proposition of a simplified and consensual one in 1987. However, by revisiting these classifications, case-reports or case-series from which the authors based their own, we found many patients who never truly fitted, neither in the 1987’s classification nor in the correspondent natural history/pathogenesis view. Herein, the concepts of paracoccidioidomycosis-infection, primary pulmonary paracoccidioidomycosis (PP-PCM) and other subclinical forms of PCM are reassessed. A classification is proposed to encompass all these subtle but distinct outcomes. We suggest a continuum between the PP-PCM and the overt chronic form of disease, and not the current view of quiescent foci, frozen in time and suddenly reactivated for unknown reasons. Failure to fully resolve the infection since its initial stages is a conceivable hypothesis for the chronic form. The proposed clinical classification might offer new insights to better characterize and manage PCM patients.

Sporadic Schwannomatosis: A Systematic Review Following the 2005 Consensus Statement

2018 ◽  
Vol 79 (05) ◽  
pp. 408-415 ◽  
Author(s):  
Jan Victor ◽  
Thibault Poujade ◽  
Nadine Hollevoet ◽  
Gregoire Chick
Keyword(s):  
Systematic Review ◽  
Consensus Statement ◽  
Case Reports ◽  
Case Series ◽  
Spinal Tumors ◽  
The Mean ◽  
Tumor Distribution ◽  
Key Aspects ◽  
Number Of Individuals

Aims To identify the frequency of reports of sporadic schwannomatosis, the types of patients affected, and the nerves affected. Patients and Methods We identified all case reports and case series that reported on patients with sporadic schwannomatosis according to established criteria. Results The initial search yielded 1,597 studies, of which 15 were included. A total of 38 of 55 individuals met the inclusion criteria. The mean age of the patients was 48 years; 41% were male. Thirty-three patients had peripheral nerve tumors, and 17 had spinal tumors. Twelve had tumors in both locations. Tumor distribution was unilateral in 25 of 30 cases (83.3%) and segmental (limited to one limb or five or fewer contiguous segments of the spine) in 28 of 38 cases (73.7%). Conclusion This systematic review quantified the number of individuals who meet the criteria for sporadic schwannomatosis and better described this population to facilitate the classification of neurofibromatosis in regard to the 2005 consensus statement. Unilateral or segmental distribution of nerve tumors are key aspects when dealing with multiple noncutaneous schwannomas without involvement of the vestibular nerve.

Clinical Classification and Prognosis of Patients with Multiple Sclerosis in Northern China

2013 ◽  
Vol 680 ◽  
pp. 606-610
Author(s):  
Xue Mei Han ◽  
Ying Chang ◽  
Zhao Shi Zheng ◽  
Song Yan Liu
Keyword(s):  
Quality Of Life ◽  
Multiple Sclerosis ◽  
Nervous System ◽  
Natural History ◽  
Northern China ◽  
Natural Course ◽  
Neurological Deficits ◽  
Clinical Classification

Objective: To study the clinical classification of multiple sclerosis (MS) by analyzing the features of the magnetic resonance imaging (MRI) on brain and spinal cord and surveying the natural history. Methods: MRI examination was conducted on 132 clinically diagnosed multiple sclerosis patients and their quality of life and disability rate were graded. Results: The general distribution of lesions in the nervous system was obtained. In northern China, relapsing-remitting MS (RRMS) by natural course classification and simple brain MS by lesion distribution classification lead to fewer neurological deficits with a relatively top life quality. Meanwhile, there is a tendency for the correlation between the pathogenetic locations of MS and its natural course prognosis. Conclusion: To improve the understanding of the prognosis of MS and promote the diagnosis ability to guide MS treatment. Multiple sclerosis (MS) is a common immune-mediated chronic disease of the central nervous system with the demyelination of white matter as the main pathological feature. The incidence of MS has a significant regional difference and studies on the natural history classification and lesions distribution classification of MS patients in northern China are not sufficient. In this study, natural history and lesions distribution of MS patients were classified by MRI approach and the impacts of each MS classification on neurological deficits and quality of life were observed. In this thesis, features and prognosis of MS classifications in northern China are discussed for the purpose of improving the level of diagnosis and treatment.

scholarly journals “When should primary angiitis of the central nervous system (PACNS) be suspected?”: literature review and proposal of a preliminary screening algorithm

2020 ◽  
Vol 41 (11) ◽  
pp. 3135-3148 ◽  
Author(s):  
Cristina Sarti ◽  
Antonella Picchioni ◽  
Roberta Telese ◽  
Marco Pasi ◽  
Ylenia Failli ◽  
...  
Keyword(s):  
Case Reports ◽  
Case Series ◽  
Neurological Deficits ◽  
Preliminary Screening ◽  
Screening Algorithm ◽  
Level Data ◽  
Primary Angiitis ◽  
The Central Nervous System ◽  
Selection Of

Abstract Background Primary angiitis of the CNS (PACNS) is a process causing variously combined neurological disturbances. Its rarity and kaleidoscopic presentation make it difficult to diagnose and even to suspect. Objective (1) To provide an up-to-date review on PACNS and (2) to create a preliminary screening algorithm based on clinical and radiological first-level data, useful to suspect PACNS and guide further investigations. Methods Review of PUBMED case series on PACNS, published from 2002 to 2017, collection of frequencies of clinical and neuroimaging features and calculation of median values. Classification of features as “major” or “minor” if frequency was higher or lower than median value. Combination of features in sets of criteria represented by all possible combinations of major and minor clinical and neuroradiological features. Application of criteria to published PACNS case reports and selection of the ones best identifying patients with definite PACNS. Results We reviewed 24 case series. “Major” clinical features were headache, stroke, cognitive impairment, focal neurological deficits; “minor” were seizures, altered consciousness, psychiatric disorders. “Major” neuroradiological features were multiple parenchymal lesions, parenchymal/meningeal contrast enhancement, magnetic resonance angiography vessel abnormalities, vessel wall enhancement; “minor” were parenchymal/subarachnoid hemorrhage, single parenchymal lesion. The selected sets of criteria able to identify all PACNS patients were (1) one clinical (major/minor) + one major neuroradiological feature; and (2) Two clinical (≥ 1 major) + one minor neuroradiological feature. Conclusion Our review provides a detailed clinical/neuroradiological picture of PACNS. The proposed algorithm should be regarded as a preliminary screening tool to move the first steps towards PACNS diagnosis that needs validation.

scholarly journals Congenital Rhabdomyosarcoma Presenting as a Neck Mass at Birth

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Leah E. Waldman ◽  
Alex K. Williamson ◽  
John B. Amodio ◽  
Lee Collins
Keyword(s):  
Malignant Tumor ◽  
Gene Fusion ◽  
Soft Tissues ◽  
Case Reports ◽  
Pediatric Population ◽  
Case Series ◽  
Neck Mass ◽  
Gene Rearrangements ◽  
Prognostic Information

Rhabdomyosarcoma is a malignant tumor of the soft tissues which preferentially affects the pediatric population. Neonatal rhabdomyosarcoma is rare, and much of the published literature concerning this entity consists of isolated case reports and small case series. Recent work involving the classification of rhabdomyosarcoma has helped to delineate prognostic information based on gene rearrangements. Here, we present a case of congenital rhabdomyosarcoma seen in utero which manifested as a neck mass at birth and was found to harbor a favorable gene fusion.

Morphological Typing of Endometrial Adenocarcinoma as the Basis of the Natural History of the Tumor.

1974 ◽  
Vol 60 (2) ◽  
pp. 163-170 ◽  
Author(s):  
Marcello Filotico ◽  
Mario Trabucco ◽  
Francesco Faggiano ◽  
Giovanni Mannarini ◽  
Silvana Grasso
Keyword(s):  
Natural History ◽  
Endometrial Adenocarcinoma ◽  
Case Series ◽  
Endocrine Treatment ◽  
Morphological Criterion ◽  
History Of ◽  
Type 3

Current ideas on Müllerian tumors of the ovary are applied to the classification of endometrial adenocarcinomas, which are divided into four main histotypes: Type 1 or homologous or endometrioid (65 % of cases); Type 2 or Fallopianlike (15.8 %); Type 3 or cervical-like (13.4%) and Type 4 or unclassifiable (5%). This classification aims to provide a useful morphological criterion for forecasting the sensitivity of endometrial adenocarcinomas to endocrine treatment with progestational agents. Type 1 accounts for about 80 % of all adenocarcinomas of the endometrium in the fifth decade of life, which is supposed to be the period most exposed to estrogenic stimulation. These adenocarcinomas represent only 14 % of all endometrial adenocarcinomas. The above results are based on a 155-case series.

scholarly journals The Natural History of Severe Acute Respiratory Syndrome Coronavirus 2–Related Multisystem Inflammatory Syndrome in Children: A Systematic Review

2020 ◽  
Author(s):  
Stephen C Aronoff ◽  
Ashleigh Hall ◽  
Michael T Del Vecchio
Keyword(s):  
Systematic Review ◽  
Natural History ◽  
Severe Acute Respiratory Syndrome ◽  
Myocardial Dysfunction ◽  
Case Reports ◽  
Clinical Manifestations ◽  
Gastrointestinal Symptoms ◽  
Case Series ◽  
History Of ◽  
Inflammatory Syndrome

Abstract Background The clinical manifestations and natural history of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)–related multisystem inflammatory syndrome in children (MIS-C) are poorly defined. Using a systematic review of individual cases and case series and collating elements of the clinical course, the objective of this study was to provide a detailed clinical description and natural history of MIS-C. Methods Case reports and series of MIS-C were recovered from repeated MEDLINE searches, a single EMBASE search, and table of contents reviews of major general medicine and pediatric journals performed between June 3 and July 23, 2020. Fever, evidence of inflammation, and evidence of organ dysfunction were required for inclusion. Results MEDLINE and EMBASE searches produced 129 articles, and 10 articles were identified from journal contents or article bibliographies; 16 reports describing 505 children with MIS-C comprise this review. Thirty-two children (14.7%) had negative results for SARS-CoV-2 by nucleic acid and/or antibody testing. The weighted median age was 9 years (6 months to 20 years). Clinical findings included fever (100%), gastrointestinal symptoms (88.0%), rash (59.2%), conjunctivitis (50.0%), cheilitis/ “strawberry tongue” (55.7%), or extremity edema/erythema (47.5%). Median serum C-reactive protein, ferritin, fibrinogen, and D-dimer concentrations were above the normal range. Intravenous gammaglobulin (78.1%) and methylprednisolone/prednisone (57.6%) were the most common therapeutic interventions; immunomodulation was used in 24.3% of cases. Myocardial dysfunction requiring ionotropic support (57.4%) plus extracorporeal membrane oxygenation (5.3%), respiratory distress requiring mechanical ventilation (26.1%), and acute kidney injury (11.9%) were the major complications; anticoagulation was used commonly (54.4%), but thrombotic events occurred rarely (3.5%). Seven (1.4%) children died. Conclusions MIS-C following SARS-CoV-2 infection frequently presents with gastrointestinal complaints and/or rash; conjunctivitis, cheilitis, and/or extremity changes also occur frequently. Serious complications occur frequently and respond to aggressive supportive therapy.

Intramedullary spinal cord cavernous malformations

2010 ◽  
Vol 29 (3) ◽  
pp. E14 ◽  
Author(s):  
Bradley A. Gross ◽  
Rose Du ◽  
A. John Popp ◽  
Arthur L. Day
Keyword(s):  
Spinal Cord ◽  
Natural History ◽  
Vascular Malformations ◽  
Case Reports ◽  
Case Series ◽  
Motor Deficits ◽  
Intramedullary Spinal Cord ◽  
Cavernous Malformations ◽  
Management Algorithm ◽  
Surgical Data

Although originally the subject of rare case reports, intramedullary spinal cord cavernous malformations (CMs) have recently surfaced in an increasing number of case series and natural history reports in the literature. The authors reviewed 27 publications with 352 patients to consolidate modern epidemiological, natural history, and clinical and surgical data to facilitate decision making when managing these challenging vascular malformations. The mean age at presentation was 42 years without a sex predilection. Thirty-eight percent of the cases were cervical, 57% thoracic, 4% lumbar, and 1% unspecified location. Nine percent of the patients had a family history of CNS CMs. Twenty-seven percent of the patients had an associated cranial CM. On presentation 63% of the patients had motor deficits, 65% had sensory deficits, 27% had pain, and 11% had bowel or bladder dysfunction. Presentation was acute in 30%, recurrent in 16%, and progressive in 54% of cases. An overall annual hemorrhage rate was calculated as 2.5% for 92 patients followed up for a total of 2571 patient-years. Across 24 reviewed surgical series, a 91% complete resection rate was found. Transient morbidity was seen in 36% of cases. Sixty-one percent of patients improved, 27% were unchanged, and 12% were worse at the long-term follow-up. Using this information, the authors review surgical nuances in treating these lesions and propose a management algorithm.

Cannabis-associated arteritis

VASA ◽  
2010 ◽  
Vol 39 (1) ◽  
pp. 43-53 ◽  
Author(s):  
Grotenhermen
Keyword(s):  
Systematic Review ◽  
Case Reports ◽  
Case Series ◽  
Cardiovascular Effects ◽  
Causative Factor ◽  
Cannabis Use ◽  
Thromboangiitis Obliterans ◽  
Pathological Features ◽  
Scientific Support ◽  
Clinical And Pathological Features

Background: To investigate the hypothesis that cases of arteritis similar to thromboangiitis obliterans (TAO) and associated with the use of cannabis were caused by cannabis or THC (dronabinol), or that cannabis use is a co-factor of TAO. Patients and methods: A systematic review on case reports and the literature on so-called cannabis arteritis, TAO, and cardiovascular effects of cannabinoids was conducted. Results: Fifteen reports with 57 cases of an arteritis associated with the use of cannabis and two additional case series of TAO, in which some patients also used cannabis, were identified. Clinical and pathological features of cannabis-associated arteritis do not differ from TAO and the major risk factor of TAO, tobacco use, was present in most, if not in all of these cases. The proposed pathophysiological mechanisms for the development of an arteritis by cannabis use are not substantiated. Conclusions: The hypothesis of cannabis being a causative factor or co-factor of TAO or an arteritis similar to TAO is not supported by the available evidence. The use of the term “cannabis arteritis” should be avoided until or unless more convincing scientific support is forthcoming.

Thrombosis in Cancer Patients Treated with Hematopoietic Growth Factors - A Meta-Analysis

1996 ◽  
Vol 75 (02) ◽  
pp. 368-371 ◽  
Author(s):  
T Barbul ◽  
G Finazzi ◽  
A Grassi ◽  
R Marchioli
Keyword(s):  
Cancer Patients ◽  
Case Reports ◽  
Meta Analysis ◽  
Case Series ◽  
Hematopoietic Growth Factors ◽  
Patients With Cancer ◽  
Cytotoxic Treatment ◽  
Pertinent Data ◽  
Thrombotic Complications ◽  
Vascular Occlusions

SummaryHematopoietic colony-stimulating factors (CSFs) are largely used in patients with cancer undergoing cytotoxic treatment to accelerate neutrophil recovery and decrease the incidence of febrile neutropenia. Clinical practice guidelines for their use have been recently established (1), taking into account clinical benefit, but also cost and toxicity. Vascular occlusions have been recently reported among the severe reactions associated with the use of CSFs, in anedoctal case reports (2, 3), consecutive case series (4) and randomized clinical trial (5, 6). However, the role of CSFs in the pathogenesis of thrombotic complications is difficult to ascertain, because pertinent data are scanty and widely distributed over a number of heterogenous investigations. We report here a systematic review of relevant articles, with the aims to estimate the prevalence of thrombosis associated with the use of CSFs and to assess if this rate is significantly higher than that observed in cancer patients not receiving CSFs.

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