Lemierre syndrome.

Lemierre's syndrome is rare and characterized by an oropharyngeal infection with thrombophlebitis of the internal jugular vein (IJV). Septic microemboli can spread to the lungs or abdomen. This case describes a patient who presented with a sore throat, unilateral swelling on the neck and diffuse abdominal pain. Imaging showed a thrombus in the right IJV. The patient developed septic shock and was treated with antibiotics, anticoagulants, and intensive care support. The most common causative organism is Fusobacterium necrophorum. Early diagnosis and treatment are crucial for reducing mortality.

[Idiopatic subglottic trancheal stenosis].

Subglottic stenosis (SGS) is a rare condition. The aetiology of SGS can be congenital, iatrogenic, idiopathic or caused by infectious and vascular diseases. In this case report, a 49-year-old woman was admitted to the hospital due to acute respiratory insufficiency. During intubation attempt SGS was found, and acute tracheotomy was performed. Biopsies from SGS and serology did not support an underlying vascular disease and the patient was diagnosed with an idiopathic SGS. SGS is a rare cause of acute respiratory insufficiency but is important to consider as a differential diagnosis.

Correction: Genetic susceptibility to angiotensin-converting enzyme-inhibitor induced angioedema: A systematic review and evaluation of methodological approaches.

[This corrects the article DOI: 10.1371/journal.pone.0224858.].

Genetic susceptibility to angiotensin-converting enzyme-inhibitor induced angioedema: A systematic review and evaluation of methodological approaches.

Angiotensin-converting enzyme (ACE) converts angiotensin I to angiotensin II which causes vasoconstriction. ACE inhibitors reduce blood pressure by inhibiting ACE. A well-known adverse drug reaction to ACE inhibitors is ACE inhibitor-induced angioedema (ACEi-AE). Angioedema is a swelling of skin and mucosa, which can be fatal if the airway is compromised. We have performed a systematic review of the evidence suggesting that genetic polymorphisms are associated with ACEi-AE and evaluated the methodological approaches of the included studies. The Cochrane Database of Systematic Reviews, Google Scholar, and PubMed were searched. Studies investigating the association between genetic markers and ACEi-AE were included. The Q-genie tool was used to evaluate the quality of the study methodologies. Seven studies were included. With the exception of one whole genome study, all of the included studies were candidate gene association studies. Study quality assessment scores ranged from 36 to 55. One study was found to be of good quality, suggesting that the detected associations may be unreliable. The inferior quality of some studies was due to poor organization, lack of analyses and missing information. Polymorphisms within XPEPNP2, BDKRB2-9/+ 9 and neprilysin genes, were reported to be associated with increased risk of ACEi-AE. However, due to low quality, these associations need to be confirmed in larger studies.

Recurrent angioedema associated with pharmacological inhibition of dipeptidyl peptidase IV.

Angioedema (AE) of the upper airways is a severe and potentially life-threatening condition. The incidence has been increasing in the past two decades, primarily due to increased use of medications inhibiting the degradation of vasoactive peptides. Acquired angioedema related to angiotensin-converting enzyme inhibitors (ACEI-AAE) is well known, but other pharmaceutical agents also affect the degradation of bradykinin and substance P. We present a middle-aged man with recurrent episodes of severe AE of the oral cavity, hypopharynx and larynx due to pharmacological inhibition of dipeptidyl peptidase IV.

The Use of Plasma-Derived Complement C1-Esterase Inhibitor Concentrate (Berinert®) in the Treatment of Angiotensin Converting Enzyme-Inhibitor Related Angioedema.

Angioedema of the upper airways is a severe and potentially life-threatening condition. The incidence has been increasing in the past two decades, primarily due to pharmaceuticals influencing the generation or degradation of the vasoactive molecule bradykinin. Plasma-derived C1-esterase inhibitor concentrate is a well-established treatment option of hereditary and acquired complement C1-esterase inhibitor deficiency, which are also mediated by an increased level of bradykinin resulting in recurrent angioedema. We here present a case of severe angiotensin converting enzyme-inhibitor related angioedema (ACEi-AE) of the hypopharynx that completely resolved rapidly after the infusion of plasma-derived C1-inhibitor concentrate adding to the sparse reports in the existing literature.

[Hypercalcaemia can be the only initial symptom of sarcoidosis].

Sarcoidosis is an ordinary systemic inflammatory disease that can affect many organs. It is characterized by development of non-necrotizing granulomas. It commonly affects lungs (80%) while hypercalcaemia is found in 5% and reflects an activation of T-cells within the granulomas that causes an overproduction of 1,25-dihydroxyvitamin D3. We describe a case where hypercalcaemia was the only symptom in a patient with extrapulmonary sarcoidosis, and the investigation was misled by different serologic findings though PET-CT showed fludeoxyglucose uptake in the biceps femoris muscles. The diagnosis was made after a biopsy.