Kasabach-Merritt Syndrome

2002 ◽  
Vol 6 (4) ◽  
pp. 335-339 ◽  
Author(s):  
Sheilagh Maguiness ◽  
Lyn Guenther

Background: Kasabach-Merritt syndrome (KMS) is a consumptive coagulopathy associated with the presence of a large vascular lesion. It is often a frustrating condition to treat and it carries a high mortality rate. There are currently no known treatment guidelines. Kasabach-Merritt syndrome is associated with kaposiform hemangioendothelioma (KHE) and tufted angioma (TA); these lesions, when associated with KMS, are locally invasive, aggressive vascular tumors. Treatment options include supportive care, local therapies, and drug and surgical management. In most recent case reports, a multimodal approach to therapy is taken. Objective and Conclusion: The objective of this article is to provide a comprehensive review of KMS and give an up-to-date summary of treatment options. The clinical presentation, laboratory findings, vascular pathology, and pathophysiology will also be discussed.

1992 ◽  
Vol 26 (11) ◽  
pp. 1452-1455 ◽  
Author(s):  
Jeffrey T. Moss ◽  
James P. Wilson

OBJECTIVE: To review the epidemiology, clinical presentation, risk factors for transmission, and pathogenesis of leishmaniasis, as well as current treatment options for this disease. DATA SOURCES/DATA SELECTION: We reviewed unclassified medical-threat briefing material, subject-matter reviews, and case reports from the world's infectious disease literature. We concentrated on literature pertaining to the pathogenesis and management of leishmaniasis indigenous to Southwest Asia. DATA EXTRACTION: Data from subject reviews published in the English language were evaluated. Case reports and clinical trials provided supplemental data on evolving theories and management options. DATA SYNTHESIS: The clinical presentation of leishmaniasis is highly variable. Management relies heavily upon the use of parenteral antimonial drugs. Although these agents are effective in most cases, toxicity and the emergence of resistance limit the usefulness of standard therapies. Alternative treatment modalities include heat, surgical curettage, ketoconazole, metronidazole, pentamidine, rifampin, amphotericin B, aminoglycosides, allopurinol, and immunotherapy. CONCLUSIONS: Although the number of reported cases of leishmaniasis in the US has generally been low, there is a possibility that more cases may be reported in the future because of the large number of military personnel returning to this country from endemic areas. Medical personnel, particularly those working in governmental institutions, should be familiar with the pathogenesis of this unusual infection as well as potential treatment options.


2017 ◽  
Vol 107 (5) ◽  
pp. 461-466
Author(s):  
Georgios Kotzias ◽  
Modupe Agunbiade ◽  
Leon Isaac ◽  
Morteza Khaladj

Lymphangioma-like Kaposi's sarcoma (LLKS) is a rare histologic variant of KS. Kaposi's sarcoma is also known as human herpesvirus type 8. The clinical presentation of the LLKS lesion is highly unusual and similar to that of classic KS but with multinodular vascular tumors and lymphedema. We present a 63-year-old native Haitian man with multiple slowly progressive exophytic ulcerated lesions covering more than 60% of his left lower extremity with no systemic involvement. Much confusion surrounded the clinical presentation of these wounds, and we postulated several differential diagnoses. Herein we describe the evaluation, clinical appearance, and progression of LLKS. Due to the rarity of LLKS, treating physicians need to be aware of the clinical presentation and diagnostic criteria of this variant. Despite being incurable, early diagnosis of LLKS can lead to long-term treatment options and a major reduction in symptoms.


Haemangioma of the penis (HOP) is a rare benign vascular lesion which can affect the penis of males including infants, children, and adults of up to the over 80 year-olds. HOP does manifest as: asymptomatic lump or lesion on the penis; localised pain or tenderness anywhere within the penis; a tumoral mass anywhere on the penis; pain in the penis on erection; curvature of penis on erection; bright red compressible papule or papules on penis; nodules on penis; plaques on penis; ulceration on penis; haemorrhage on penis. HOP may be associated erectile dysfunction in some cases and could affect voiding if it is associated with the urethral meatus. HOP which tends to be solitary in most cases may mimic other diseases of the penis as well as may be associated with haemangioma in the scrotum and perineal region occasionally. Diagnosis can be established by the clinical features and this can be reaffirmed by radiology imaging features with the use of Doppler ultrasound scan, CT scan and MRI scan which show low-flow within the lesion. Majority of the lesions tend to be superficial and not associated with the corpora cavernosa and spongiosum. There is no consensus opinion on the best treatment options hence various treatment options for HOP have been successfully utilized including: complete surgical excision, sclerotherapy, laser treatment, cryotherapy, and other therapies. Most cases of HOP do not recur but following sclerotherapy and laser treatment of large HOPs repeat procedures tend to be required and there may be residual / recurrent lesion that may need to be treated again. Deep and large HOPs may require complete excision with insertion of tunica graft. Very large HOPs could be more effectively treated by one stage complete surgical excision but the choice of the patient is important. The diagnosis of the lesion can be confirmed by the histopathology features of the lesion and positive immunohistochemistry staining for CD31 (strong), Factor VIII, and HHF35 (myopericytes) and minimal staining for CD34. HOP needs to be carefully differentiated from epithelioid angiosarcoma of the penis and epithelioid haemangioendothelioma. A multi-centre trial of various treatment options for HOP would be required to provide treatment guidelines for HOP taking into consideration the size of the lesion and availability of the various treatment options.


2021 ◽  
Vol 1 (1) ◽  
pp. 135-150
Author(s):  
Elie Al Zaghrini ◽  
Nancy Emmanuel ◽  
Victor Zibara ◽  
Wael Terro ◽  
Samia Hanna

Objective: The purpose of this article is to review the cases of myocarditis in COVID-19 patients and synthesize the current understanding regarding the presentation, diagnosis, and management of myocarditis in the setting of COVID-19 disease. Background: The novel coronavirus disease has shown serious implications for the cardiovascular system, including acute myocardial injury, arrhythmias, venous thromboembolism, and myocarditis. Several cases of myocarditis in COVID-19 patients have been reported since the disease's emergence at the end of 2019. The diagnostic approach and management have been variable. The purpose of this narrative review is to gather the most reliable published material regarding myocarditis in COVID-19 and present it as an overview to simplify the current understanding we have of this disease. Methods: We screened PubMed, Scopus, and Embase. We then selected peer-reviewed and pre-print articles published in English that were related to the involvement of the cardiovascular system in COVID-19, with a focus on myocarditis. We included case reports describing myocarditis in COVID-19 patients and summarized their clinical presentation, diagnosis, and management. References of the selected articles were also screened, and some were included when relevant. Discussion: This article is subdivided into sections that discuss the clinical presentation of COVID-19 myocarditis and move on to various diagnostic approaches and management options. Each subsection presents a brief literature review followed by a summary and interpretation of what was found in the reported cases. Conclusion: After noticing the involvement of the cardiovascular system in COVID-19 patients, specifically through myocarditis, we present this narrative review to provide the medical community with a unified article regarding the current understanding of myocarditis in COVID-19 patients. This article further stresses the necessity of establishing proper treatment guidelines for COVID-19 myocarditis.


Author(s):  
Louise Shewan ◽  
Michael Henein ◽  
Andrew Coats

<p class="normal">This special issue includes expert reviews and original research on Takotsubo syndrome (TTS) including the history of its identification and the unravelling of the pathophysiology of the different variants of the syndrome. The role of neurological stress or catecholaminergic overload are reviewed, as is the role of CNS disorders triggering TTS. Clinical presentation patterns of TTS and the most useful diagnostic tests are reviewed, including original research into a novel clinical score the ‘GET QT' score to help in rapid differentiation of TTS from STEMI. There is also a review of TTS in the intensive care unit setting.  The mechanisms of TTS including the role and assessment of the microcirculation in generating the classical myocardial dysfunction are reviewed in detail, as are the pathophysiological pathways of recurrent TTS. Lastly Singh, Akashi and Horowitz review the emerging treatment options for TTS. They issue also includes multiple elegant and fascinating case reports. TTS has come of age and now urgently adequate sized RCT’s.  </p>


2020 ◽  
Vol 13 (9) ◽  
pp. e235065
Author(s):  
Ariyati Yosi ◽  
Abdullah Afif Siregar ◽  
Yasmine Siregar ◽  
Widyaningsih Oentari

Kasabach-Merritt phenomenon (KMP) is consumptive coagulopathy in large vascular lesion. This condition is specific in kaposiform haemangioendothelioma (KHE) and tufted angioma, associated with high mortality rate. This condition often involves the limbs, trunk and retroperitoneum. The clinical features that are usually found are purpura, oedema, induration and ecchymosis. A boy baby aged 9 months presented with a history of violaceous tumour that appeared on his left leg at birth that became enlarged since 6 months ago that caused discrepancy between both of his legs. The patient has his left leg massaged through alternative treatment, causing it became bruised and wounded. Blood examination showed normocytic normochromic anaemia with severe thrombocytopenia and high D-dimer. The patient was diagnosed with KMP with cellulitis and treated with oral corticosteroid, propranolol and antibiotics. Diagnosis of KMP can be made through clinical and laboratory findings. Combination therapy with corticosteroid and propranolol can be considered for KMP treatment.


PEDIATRICS ◽  
2000 ◽  
Vol 105 (Supplement_2) ◽  
pp. 242-245
Author(s):  
Thomas B. Newman ◽  
M. Jeffrey Maisels

The publication of guidelines calling for less aggressive treatment of jaundice in newborns has been followed by a reappearance of case reports of kernicterus. These case reports illustrate important issues for writers and consumers of practice guidelines. One issue is the particular salience of identified patients with bad outcomes, and their potentially disproportionate influence on decision-makers. A second issue is whether, when good evidence of treatment benefit is lacking, policymakers should recommend what has traditionally been done, recommend less treatment, or not make recommendations at all. Finally, the cases raise the question of whether treatment guidelines should be more conservative than their authors actually believe is necessary, to take into account the likelihood that they will not be closely followed. We believe that case reports can serve as an important early warning system, but policymakers should be aware of their potentially disproportionate influence. In the long run, patients and clinicians will be best served by guidelines that summarize and acknowledge the limitations of existing evidence, that allow a wide range of treatment options when evidence is weak, and that recommend what the guideline authors actually believe should be done. In the short run a period of readjustment may be required, however, as clinicians become accustomed to guidelines written to be followed, rather than bent.neonatal jaundice, bilirubin, kernicterus, practice guidelines.


2021 ◽  
Vol 1 (1) ◽  
Author(s):  
Hitesh Garg ◽  
◽  
Betina Chandolia ◽  

Essential tremor (ET) is the most prevalent movement disorder globally and is ten times more prevalent than Parkinson's. It is considered one of the most common movement disorders with various treatment options reported in clinical practice but uncertainty about the most robust one. The International Parkinson and Movement Disorder Society recently reviewed the clinical studies of essential tremors and updated the treatment guidelines. Mild to moderate tremor severity can sometimes be managed with occupational treatment such as speech therapy or adaptation. In contrast, the first-line pharmacological treatments include symptomatic treatment with propranolol, primidone, and topiramate. Botulinum toxin is preferred for selected cases, whereas invasive treatments should be considered a severe essential tremor treatment option. Focused ultrasound thalamotomy is attracting a lot of attention as a new therapy for essential tremors. Misdiagnosis among tremor syndromes is another point of concern and impacts both clinical care and research. A new neurophysiological measure, known as the tremor stability index, is introduced recently to attain diagnostic accuracy between Parkinson's disease tremor and essential tremor. Also, ET-Plus is a newly suggested term for the classification of tremor in the recent Consensus statement, which acknowledges the presence of additional neurological signs in patients with ET. This review includes in detail defining ET, hypothesis about ET, clinical features along with points to consider while differentiating between ET and Parkinson's Disease (PD), evaluation of ET based on laboratory findings, treatment procedures, measures to reduce misdiagnosis between ET and PD, and reality about the new term ET-Plus syndrome.


2019 ◽  
Vol 12 (4) ◽  
pp. 140-144
Author(s):  
Benjamin Marlow ◽  
Kate Parker ◽  
Samantha Hodges

Partial or complete failure of tooth eruption may be due to several causes, including primary failure of eruption (PFE), and an accurate diagnosis is essential for appropriate management. This article reviews PFE and the possible treatment options. Case reports of two patients diagnosed with PFE are presented and their management discussed. CPD/Clinical Relevance: Primary failure of eruption can be difficult to diagnose and differentiate from other causes of failure of eruption. This paper highlights the clinical presentation of PFE through a review of the literature and by illustration with two clinical cases.


2012 ◽  
Vol 21 (3) ◽  
pp. 75-84
Author(s):  
Venkata Vijaya K. Dalai ◽  
Jason E. Childress ◽  
Paul E Schulz

Dementia is a major public health concern that afflicts an estimated 24.3 million people worldwide. Great strides are being made in order to better diagnose, prevent, and treat these disorders. Dementia is associated with multiple complications, some of which can be life-threatening, such as dysphagia. There is great variability between dementias in terms of when dysphagia and other swallowing disorders occur. In order to prepare the reader for the other articles in this publication discussing swallowing issues in depth, the authors of this article will provide a brief overview of the prevalence, risk factors, pathogenesis, clinical presentation, diagnosis, current treatment options, and implications for eating for the common forms of neurodegenerative dementias.


Sign in / Sign up

Export Citation Format

Share Document