Osier Weber Rendu Syndrome: A Systemic Angiodysptasia

1995 ◽  
Vol 112 (5) ◽  
pp. P37-P37
Author(s):  
Robert I. White ◽  
Douglas A. Ross ◽  
William H. Saunders

Educational objectives: To screen patients with epistaxis for life-threatening brain and pulmonary complications associated with Osier Weber Rendu syndrome and to have further knowledge of genetics and family history necessary to advise and treat these patients.

Blood ◽  
2006 ◽  
Vol 107 (9) ◽  
pp. 3492-3494 ◽  
Author(s):  
Shigesaburo Miyakoshi ◽  
Masahiro Kami ◽  
Koichiro Yuji ◽  
Tomoko Matsumura ◽  
Masaaki Takatoku ◽  
...  

Bortezomib is a novel proteasome inhibitor with significant antimyeloma activity. Its frequent adverse effects are manageable, including gastrointestinal symptoms, peripheral neuropathy, and thrombocytopenia. Severe lung toxicity has not previously been reported. Between June 2004 and September 2005, 13 Japanese patients with multiple myeloma were treated with bortezomib in Toranomon Hospital, Juntendo University School of Medicine, and Jichi Medical School. Four of them developed severe pulmonary complications, and 2 died of respiratory failure without progression of underlying disease. To our knowledge, this is the first report on life-threatening pulmonary adverse effects after bortezomib therapy. Previous clinical studies on bortezomib, mostly in the United States and Europe, have shown low incidences of pulmonary adverse effects. Our study suggests that bortezomib can cause serious lung injury, and that its incidence might vary among different ethnicities. Clinicians need to be alert to the possibility.


1994 ◽  
Vol 52 (3) ◽  
pp. 410-413 ◽  
Author(s):  
Luiz Augusto F. Andrade ◽  
Sonia Maria D. Brucki

Cephalic tetanus is a localized form of tetanus. As in generalized forms , trismus is a prominent feature of the disease, leading to considerable difficulty in feeding, swallowing of the saliva and mouth hygiene. These difficulties often precede respiratory problems and aspiration bronchopneumonia is a frequent life-threatening complication. Muscle relaxants other than curare drugs may show a limited benefit for relieving trismus. Tetanospasmin, the tetanic neurotoxin, and botulinum toxin share many similarities, having a closely related chemical structure, an origin from related microorganisms (Clostridium tetani and Clostridium botulinum, respectively), and presumably, the same mechanisms of action in the neuron. The difference between the two lies in their peculiar neurospecificity, acting in different neurons. Injection of minute doses of botulinum toxin in the muscles involved in focal dystonias or other localized spastic disorders have proved to be very effective in these conditions. We describe the use of botulinum toxin A in the successful treatment of trismus in a patient suffering from cephalic tetanus. We believe that this form of treatment may be of value in lowering the risk of pulmonary complications in tetanic patients.


2017 ◽  
Vol 5 (2) ◽  
pp. 139-140
Author(s):  
Sanjith Saseedharan ◽  
Edwin Pathrose ◽  
AM Argikar

Cough, as a symptom by default gets the focus of a physician towards the chest ruling out the causes could be of the same. However an incessant cough causing life threatening extra pulmonary complications is a rarity. We present a case of an elderly female who presented with a cough with expectoration but ended up requiring strict hemodynamic monitoring due to a rectus sheath hematoma.Bangladesh Crit Care J September 2017; 5(2): 139-140


Thorax ◽  
2017 ◽  
Vol 73 (3) ◽  
pp. 270-276 ◽  
Author(s):  
Rachel M Scott ◽  
Elizabeth P Henske ◽  
Benjamin Raby ◽  
Philip M Boone ◽  
Rosemary A Rusk ◽  
...  

One in 10 patients suffering from primary spontaneous pneumothoraces has a family history of the disorder. Such familial pneumothoraces can occur in isolation, but can also be the presentation of serious genetic disorders with life-threatening vascular or cancerous complications. As the pneumothorax frequently precedes the more dangerous complications by many years, it provides an opportunity to intervene in a focused manner, permitting the practice of precision medicine. In this review, we will discuss the clinical manifestations and underlying biology of the genetic causes of familial pneumothorax.


2015 ◽  
Vol 6 (4) ◽  
pp. 6-10
Author(s):  
I. S Skopets ◽  
N. N Vezikova ◽  
I. M Marusenko ◽  
O. Yu Barysheva

A number of studies demonstrate that patients with traditional risk factors (TRF) have not only increases primary risk of atherothrombotic events, but are also associated with many complicates and poor prognosis.Purpose: assessment of TRF effect on the incidence of complications and outcomes in patients with acute coronary syndrome (ACS).Materials and methods: in 255 patients hospitalized with ACS were retrospective determined the TRF prevalence, frequency of the complications and correlation between the presence of TRF and the risk of complications and long-term prognosis (follow-up 1 year).Results: patients had TRF very often, 80% patients had more than 3 TRFs. The presence of some TRFs (smoking, abdominal obesity, family history) was associated with a significantly increased risk of complications in patients with ACS, including life-threatening. Effect of TRF on long-term prognosis was not determined.Conclusion: the findings suggest the need to evaluation TRF not only in primary preventive and also to improve the effectiveness of risk stratification in patients with ACS.


2018 ◽  
Vol 26 (1) ◽  
pp. 87-94 ◽  
Author(s):  
Ion Antohe ◽  
Angela Dăscălescu ◽  
Cătălin Dănăilă ◽  
Mihaela Zlei ◽  
Iuliu Ivanov ◽  
...  

Abstract Background: Acute basophilic leukemia is a rare subtype of acute myeloid leukemia, as categorized by the 2008 World Health Organization classification of myeloid neoplasms. Acute basophilic leukemia diagnosis requires thorough morphological, cytochemical, immunophenotypic, molecular, and cytogenetic studies and exclusion of other hematological neoplasms associating basophilia. The disease course is defined by histamine driven, occasionally life-threatening respiratory, cardiovascular, cutaneous or digestive complications, as well as primary refractoriness to standard therapy. Clinical presentation: We herein report a case of a 63-year-old asthmatic female patient diagnosed with acute basophilic leukemia, associated with previously unpublished cytogenetic features and FLT-3 ITD mutation, pulmonary leukostasis and spontaneous pulmonary capillary leak syndrome, which worsened immediately following chemotherapy initiation. Respiratory complications were successfully managed, but recrudesced upon emergence of refractory disease and were ultimately fatal. We highlight the likelihood of pulmonary complications induced by basophil degranulation and tumor lysis in hypercellular acute basophilic leukemia and the potential benefit of histamine receptor blockade in this setting.


Author(s):  
Victor F. Seabra ◽  
Bertrand L. Jaber

Since its first successful performance in 1945, haemodialysis has become a widely performed routine and safe procedure. However, despite significant improvements in the dialysis equipment, staff training, and patient monitoring, acute complications can occur during the therapy, ranging from mild to life-threatening. This chapter reviews selected acute complications that are encountered during or are directly related to the haemodialysis procedure, including cardiovascular, neuromuscular, haematological, and pulmonary complications, technical malfunctions, dialysis reactions (including anaphylactic and anaphylactoid reactions), and other complications such as post-dialysis fatigue, pruritus, priapism, and hearing and visual loss.


2010 ◽  
Vol 2010 ◽  
pp. 1-9 ◽  
Author(s):  
Thorsten Brenner ◽  
Johann Motsch ◽  
Jens Werner ◽  
Lars Grenacher ◽  
Eike Martin ◽  
...  

Metastatic liver resection following cytoreductive chemotherapy is an accepted treatment for oligometastatic tumor diseases. Although pulmonary complications are frequently reported in patients undergoing liver surgery including liver transplantation, life-threatening acute respiratory failures in the absence of aspiration, embolism, transfusion-related acute lung injury (TRALI), pulmonary infection, or an obvious source of systemic sepsis are rare. We performed an extensive clinical review of a patient undergoing metastatic liver resection who had a clinical course compatible to an acute respiratory distress syndrome (ARDS) without an obvious cause except for the surgical procedure and multiple preoperative chemotherapies. We hypothesize that either the surgical procedure mediated by cytokines and tumor necrosis factor or possible toxic effects of oxygen applied during general anesthesia were associated with life-threatening respiratory failure in the patient. Discrete and subclinical inflammated alveoli (probably due to multiple preoperative chemotherapies with substances at potential risk for interstitial pneumonitis as well as chest radiation) might therefore be considered as risk factors.


2019 ◽  
Vol 7 (3) ◽  
pp. 341-346
Author(s):  
Svetlan Dermendjiev ◽  
Vesela Slavcheva Blagoeva

BACKGROUND: Angioedema (AE) is acute oedema of the skin and mucous surfaces, involving the respiratory and gastrointestinal tracts. AE could be a life-threatening medical condition. Regardless of its growing clinical importance, many aspects of its aetiology and pathogenesis remain poorly understood. Its incidence, demographic characteristics, diagnosis and therapy, need further investigation. AIM: This study reports our experience with angioedema concerning its social and demographic characteristics, aetiology, clinical features, diagnosis and treatment outcomes. Study design: Eighty-eight patients with AE were enrolled. The study is a retrospective analysis of patients treated in our Clinics. METHODS: All participants were asked on a voluntary basis to fill out a specially designed questionnaire on the day of their discharge. Other important data sources included: patients’ history and notes during the hospital stay, information from previous admissions, etc. Parametric and non-parametric statistical methods were used for data processing. Data analysis was performed using SPSS (SPSS Inc., IBM SPSS Statistica) version 20.0 RESULTS: Based on our results, AE affects more frequently patients over 50 years of age, regardless of their sex. Urban residents prevail, among them - more commonly working women. Non-steroidal anti-inflammatory drugs (NSAIDs), hormones and antibiotics were among the most common triggers – in 8%, 4.5% and 11.4% of the cases respectively. In 9.09% of the patients, food-induced AE was seen, the most common foods reported, were – nuts, eggs and egg products. The common sites of involvement were periorbital area and lips. In only 9.1% of the patients, oedema progressed to spread to the upper respiratory tract. Cardiac conditions were the most frequent underlying disorders – 33%, of the patients, auto-immune thyroiditis was the second most common-14.8%, followed by musculo- skeletal disorders (10.2%) and diabetes (4.5%) Family history of allergy was seen in 8.4% of the patients, the most frequent allergic disorder, reported, was asthma. In patients with HAE, family history was present in 2.9% of the patients. CONCLUSIONS: All patients received therapy with steroids and antihistamines, resulting in resolution of symptoms and no invasive procedures were necessary. Based on our results, the diagnosis of AE is often difficult and delayed and requires specialist evaluation. If recognised on time and adequately treated, the outcomes are favourable.


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