scholarly journals Diagnosis and treatment of Rosai-Dorfman disease of the spine: a systematic literature review

2021 ◽  
Vol 10 (1) ◽  
Author(s):  
Pan-pan Hu ◽  
Feng Wei ◽  
Xiao-guang Liu ◽  
Zhong-jun Liu

Abstract Purpose To review and summarize the clinical features, diagnosis, treatment strategies, and prognosis of spinal Rosai-Dorfman disease (RDD). Methods RDD is also termed as sinus histiocytosis with massive lymphadenopathy. We searched the databases of PubMed, Elsevier ScienceDirect, SpringerLink, and OVID. The keywords were Rosai-Dorfman disease and spine/central nervous system. Research articles and case reports with accessibility to full texts regarding spinal RDD were eligible for the inclusion. A total of 62 articles were included, and they contained 69 cases. We extracted the information of interest and analyzed them using SPSS statistics package. Results The average age was 33.1 ± 18.3 years. The ratio of males to females was 1.9/1. Overall, 63 cases presented with spine-related symptoms. A total of 27 cases (39.1%) had multi-organ lesions, and 12 cases had records of massive lymphadenopathy. Among 47 cases who first manifested spine-related symptoms, 93.6% were preoperatively misdiagnosed. The disease had a predilection for cervical spine (38.8%) and thoracic spine (40.3%). 62.9% of lesions were dura-based. Surgery remained the mainstream treatment option (78.8%), with or without adjuvant therapies. Total lesion resection was achieved in 34.8% of cases. The rate of lesion recurrence/progression was 19.5%, which was marginally lower for total resection than for non-total resection. Conclusion Spinal RDD has no pathognomonic clinical and imaging features. Most cases first present with spine-relevant symptoms. Massive lymphadenopathy is not common, but a tendency for multi-organ involvement should be considered. Spinal RDD has a high recurrence rate; thus, total resection is the treatment of choice. Adjuvant therapies are indicated for multi-organ lesions and residual lesions. A wait and watch strategy is recommended for asymptomatic patients. Herein, a workflow of diagnosis and treatment of the spinal RDD is established.

2019 ◽  
Vol 24 (5) ◽  
pp. 378-389 ◽  
Author(s):  
Jenna W. Bartlett ◽  
Pamela L. Walker

Calcium channel blockers (CCBs) are commonly prescribed cardiovascular medications used in several disease states including hypertension, coronary artery disease, and atrial fibrillation. Inadvertent exposure or intentional overdose of CCBs may result in hypotension, bradycardia, dysrhythmias, conduction disturbances, and hyperglycemia. In the most severe cases, CCB toxicity can lead to rapid cardiovascular collapse. Given the risk of significant morbidity and mortality associated with CCB toxicity, it is important that health care professionals are able to recognize and treat patients who present with a potentially toxic ingestion. Due to the paucity of literature in managing pediatric patients with severe CCB toxicity, treatment strategies for pediatric patients are mostly limited to case reports and extrapolation from expert consensus recommendations for adults. All pediatric patients with a potentially toxic CCB ingestion should be evaluated in the emergency department. Activated charcoal may be considered for asymptomatic patients presenting within an hour of ingestion. Symptomatic patients should be placed under cardiac monitoring and treatments to stabilize the patient's hemodynamics should not be delayed. Traditional first-line IV therapies include small boluses of fluids, calcium, and vasopressors. High-dose insulin has been proposed to independently increase inotropy and improve CCB-induced hypoinsulinemia and insulin resistance that results from CCB inhibition of insulin release from pancreatic β-islet cells. High-dose insulin is recommended as first-line therapy for adults and shows promising efficacy and safety in several pediatric case reports. Intravenous lipid emulsion may be considered in patients who are refractory to first-line therapies, although the data for pediatric patients are extremely limited.


2001 ◽  
Vol 61 (3) ◽  
pp. 243-247 ◽  
Author(s):  
Asma El Kohen ◽  
Xavier Planquart ◽  
Zitouna Al Hamany ◽  
Laurent Bienvenu ◽  
Mohamed Kzadri ◽  
...  

2021 ◽  
Vol Volume 9 (upjohns/volume9/Issue2) ◽  
pp. 47-50
Author(s):  
N. Vishnu S. Reddy

ABSTRACT Rosai-Dorfman disease (RDD) is a rare histiocytic disorder commonly manifested as histiocytic proliferation of lymph nodes and extra nodal tissues initially described as a separate entity by Rosai and Dorfman under the term sinus histiocytosis with massive lymphadenopathy (SHML) in 1969. The treatment strategies can be variable and based on the severity of disease, v ital organ invol vement and pos s ible complications. The first case was a 47 years old female presenting with nasal obstruction and submandibular lymphadenopathy since few days. The second case was a 8 year old girl presented with stridor and lymphadenopathy in the submandibular region. In both the cases spontaneous resolution was expected and intervention was done only if there werecomplications. Patients were followed up every three months for two years and both the cases have resolved completely without any sequelae of the disease.Here we present two cases of Rosai-Dorfman disease presented to our hospital, this case series adds to the growing knowledge of Rosai-Dorfman di sease, along with under s tanding of pathophysiology, clinical diagnosis, treatment strategies and recovery options in cases where complications occurred.


2020 ◽  
Vol 1 (1) ◽  
pp. 58-68
Author(s):  
Ayesha Aftab ◽  
Syed Babar Jamal ◽  
Syeda Marriam Bakhtiar

Background: Obesity is an emerging pandemic considered to be an outcome of change in lifestyle owing to more processed food and the use of mechanical locomotives. Obesity has not only appeared as a problem in the esthetic appearance of an individual rather is a serious health issue due to its associations with various chronic diseases such as coronary and cardiovascular problems, hypertension, osteoarthritis, type-II diabetes mellitus, hyperlipidemia, and certain cancers. It is estimated that 30 percent of the world’s population, i.e. approximately 2.1 billion people, are victims of obesity. In addition to environmental causes, various genes and a group of genes are reported to be increasing the suceptibility of obesity. Objective: Pakistan is a heterogeneous population, an amalgam of various races, therefore, narrowing down the list of obesity-associated genes and their functional variance could help molecular biologists to select potential SNPs in the Pakistani population for molecular diagnosis and treatment. Method: The extraction of a set of obesity-associated genes has been performed by using Polysearch2. SNPs for each gene are retrieved from dbSNP. RegulomeDB and SNPinfo tools have been used for the functional analysis of SNPs retrieved against the Pakistani population. For the prediction of potential deleterious SNPs, SIFT, Polyphen-2, MUTTASTER, MUTASSESSOR, and LRT (likelihood ratio test) are utilized. Functional analysis of potential deleterious SNPs has been performed by studying protein stability and mapping of identified SNPs to protein structure. For the protein stability analysis, I-Mutant and SNPs3D have been used. Results: Four genes FTO, POMC, LEPR, and MC4R and further analysis revealed 3 deleterious SNPs in FTO, 4 in POMC, 1 in LEPR, and 1 in MC4R. Conclusion: This research was designed to identify obesity-associated genes and the most impactful deleterious SNPs in these genes. These findings will be helpful for the molecular biologists and pharmacists to design better and focused diagnosis and treatment strategies.


Cardiology ◽  
2021 ◽  
pp. 1-8
Author(s):  
Nurdan Erol

Myocardial bridging (MB) is a congenital anomaly where a coronary artery branch or group of branches extends inside a tunnel consisting of myocardium. Although it is mostly considered “benign,” it is reported that MB may lead to significant cardiac problems and sudden cardiac deaths. While it is a congenital anomaly, its symptoms usually arise at further ages rather than childhood. The literature on MB in children is in the form of case reports or small case series. This is why pediatric cases are assessed in the light of information obtained from adults. This review compiled the literature on MB in adults and children and compared it, as well as discussing questions arising regarding the clinic, diagnosis, and treatment of MB.


2015 ◽  
Vol 21 (6) ◽  
pp. 769-773 ◽  
Author(s):  
Xianli Lv ◽  
Zhongxue Wu

Objective The purpose of this study is to describe anatomic variations of the internal jugular vein (IJV), inferior petrosal sinus (IPS) and their confluence pattern and implications in IPS catheterization. The anatomic route of IPS after going out of the cranium and its confluence patterns with IJV and will supply knowledge about typing of IPS-IJV junction. Method A review of the literature was performed. Results There might be different routes for entering the intracranial segment of the IPS and multislice spiral computed tomography (MSCT) is effective in identifying the confluences of the IPS with the IJV and their courses. It is important to find the confluence of IPS with IJV for diagnosis and treatment of intracranial lesions via venous route. Meanwhile, IPS diameter at the confluence can significantly affect success of catheterization. Conclusion The classification and the theory of the development of the caudal end of the IPS may be useful in establishing treatment strategies that involve endovascular manipulation via the IPS.


Neurosurgery ◽  
2016 ◽  
Vol 79 (5) ◽  
pp. E634-E638 ◽  
Author(s):  
Marcus D. Mazur ◽  
Philipp Taussky ◽  
Joel D. MacDonald ◽  
Min S. Park

Abstract BACKGROUND AND IMPORTANCE: As the use of flow-diverting stents (FDSs) for intracranial aneurysms expands, a small number of case reports have described the successful treatment of blister aneurysms of the internal carotid artery with flow diversion. Blister aneurysms are uncommon and fragile lesions that historically have high rates of morbidity and mortality despite multiple treatment strategies. We report a case of rebleeding after treatment of a ruptured blister aneurysm with deployment of a single FDS. CLINICAL PRESENTATION: A 29-year-old man presented with subarachnoid hemorrhage and a ruptured dorsal variant internal carotid artery aneurysm. Despite a technically successful treatment with a single FDS, a second catastrophic hemorrhage occurred during the course of his hospitalization. CONCLUSION: This case highlights the risk of hemorrhage during the period after deployment of a single FDS. Ruptured aneurysms, especially of the blister type, are at risk for rehemorrhage while the occlusion remains incomplete after flow diversion.


2019 ◽  
Vol 153 (9) ◽  
pp. e52
Author(s):  
Marta Custódio ◽  
Isa Silva ◽  
Anabela Salgueiro Marques

2016 ◽  
Vol 12 (6) ◽  
pp. 855-870 ◽  
Author(s):  
Chanaka D Wickramasinghe ◽  
Kim-Lien Nguyen ◽  
Karol E Watson ◽  
Gabriel Vorobiof ◽  
Eric H Yang

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