The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up‐to‐date consensus recommendations for the management of HAE. In the development of this update and revision of the guideline, an international expert panel reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in June 2016 in Vienna. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies. The goal of this guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1‐inhibitor (type 1) and HAE with dysfunctional C1‐inhibitor (type 2). The key clinical questions covered by these recommendations are: (1) How should HAE‐1/2 be defined and classified?, (2) How should HAE‐1/2 be diagnosed?, (3) Should HAE‐1/2 patients receive prophylactic and/or on‐demand treatment and what treatment options should be used?, (4) Should HAE‐1/2 management be different for special HAE‐1/2 patient groups such as pregnant/lactating women or children?, and (5) Should HAE‐1/2 management incorporate self‐administration of therapies and patient support measures?     

Combined use of prostate-specific antigen derivatives decreases the number of unnecessary biopsies to detect prostate cancer.

The authors evaluated the prostate cancer detection rate in Turkish patients with prostate-specific antigen (PSA) levels of 4 ng/ml to 10 ng/ml and who had normal digital rectal examination (DRE) findings. They also aimed to evaluate the value of PSA density and percent free PSA in minimizing unnecessary prostate biopsies for these PSA ranges. This prospective study included 134 consecutive men referred for early prostate cancer detection or lower urinary tract symptoms. All men underwent transrectal ultrasound with systematic sextant needle biopsies. The ability of PSA density and percent free PSA to improve the power of PSA in the detection of prostate cancer was evaluated with statistical analyses as well as receiver operating characteristics curves. Among the 134 men, 124 (92.5%) had a benign histology and 10 (7.5%) had cancer diagnosed on the initial biopsies. Despite the disappointing results in regard to the sensitivity and specificity of PSA derivatives alone, the combination of PSA density and percent free PSA significantly increased the area under the curve compared with the use of each test alone. To increase the specificity of PSA in this patient population, the authors recommend combining two PSA derivatives in deciding whether to perform a biopsy. In a PSA range of 4 ng/ml to 10 ng/ml and with normal DRE, a percent free PSA < 21% and a PSA density > 0.18 yields highest specificity with 90% sensitivity.     

Nasopharyngeal tuberculosis

Nasopharyngeal tuberculosis is a rare disorder. It usually occurs via hematogenous and lymphatic spread from coexistent pulmonary or systemic tuberculosis, and by airway during respiration or bacillary expectoration. MATERIAL: we present a case of nasopharyngeal tuberculosis in a 50-year-old female patient presenting with a cervical mass and unilateral otitis media with effusion. The diagnosis was made by PCR and by isolation of Mycobacterium Tuberculosis on Loveinstein-Jensen cultures. CONCLUSION: Nasopharyngeal tuberculosis may present with cervical mass, unilateral otitis media with effusion, and may mimic nasopharyngeal carcinoma. Furthermore, nasopharyngeal carcinoma may harbor foci of granulomatous reaction, and this feature may confuse the pathologist. However PCR carries the advantage to detect the DNA of Mycobacterium tuberculosis before its growth on culture. Even though it is rare, this entity must be considered in the differential diagnosis of the nasopharyngeal masses.     

Investigation of anterior segment structures of the eye in Behçet’s disease using in vivo confocal microscopy

Graefe's Archive for Clinical and Experimental Ophthalmology - We sought to investigate alterations in the corneal subbasal nerve plexus and endothelium in patients with Behçet’s...     

Vertical mattress suture technique: an alternative vascular anastomosis

The objective of this study was to introduce the vertical mattress (VM) suture technique and compare its patency and postoperative healing with other commonly used suture techniques. Thirty-two Sprague-Dawley rats were randomly assigned to one of four suture techniques to be applied to both femoral arteries: continuous (C), interrupted (I), VM, and VM with flap (VMF) sutures. The operating time and the degree of postanastomotic leakage were recorded. The milking test was employed 5 and 15 minutes and 14 days after the operation to attain patency. Blood flow velocity was determined using ultrasonography at 1 hour and on days 1 and 14 postoperation. Rats then were sacrificed, and anastomotic lining tissues were harvested for histopathologic examination. Anastomoses by C and I were executed in 12.06 and 18.31 minutes. Modified suture techniques took longer to execute anastomosis (23.09 minutes for VM and 32.44 minutes for VMF). VM suture technique was superior to other suture techniques in terms of anastomotic leakage, quality of filling, and blood flow velocity. Also, absence of the luminal closure or constriction, intimal injury and hyperplasia, foreign body inflammation, and inversion were noted in histopathology. In conclusion, the VM suture technique could be considered for microvascular surgery.     

Retrochorionic hematoma in congenital afibrinogenemia: resolution with fibrinogen concentrate infusions

Without treatment, pregnancies in patients with congenital afibrinogenemia terminate in miscarriage at 5-6 weeks of gestation. Animal model studies have suggested that implantation site bleeding contributes to miscarriage in afibrinogenemia; however, retrochorionic hematoma in human congenital afibrinogenemia has not been previously observed. A patient with congenital afibrinogenemia receiving fibrinogen prophylaxis developed a retrochorionic hematoma in the first trimester. With continuous intensified fibrinogen concentrate replacement the hematoma resolved over 6 weeks, and the patient delivered a healthy infant. Median fibrinogen levels in the first trimester were 48 mg/dL and in second and third trimester 44 mg/dL. Median fibrinogen levels under 60 mg/dL may be adequate to maintain pregnancy in patients with congenital afibrinogenemia, although it is possible that higher levels might reduce the risk of hemorrhagic events.