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Hereditary Angioedema (HAE) is a rare disease and for this reason proper diagnosis and appropriate therapy are often unknown or not available for physicians and other health care providers. For this reason we convened a group of specialists that focus upon HAE from around the world to develop not only a consensus on diagnosis and management of HAE, but to also provide evidence based grades, strength of evidence and classification for the consensus. Since both consensus and evidence grading were adhered to the document meets criteria as a guideline. The outcome of the guideline is to improve diagnosis and management of patients with HAE throughout the world and to help initiate uniform care and availability of therapies to all with the diagnosis no matter where the residence of the individual with HAE exists.
Cylindera (Ifasina) thitarooae Wiesner and Hori, new species, Jansenia phyuae Wiesner and Hori, new species, and the previously unknown male of Jansenia myanmarensis Wiesner, 2004 (Coleoptera: Cicindelidae) are described. Cylindera (I.) thitarooae may be recognized by shape of labrum and aedeagus. Jansenia phyuae may be recognized by its elytral contour and shape of elytral maculation.
Patients with neuroendocrine tumors (NET) often go through a long phase between onset of symptoms and initial diagnosis. Assessment of time to diagnosis and pre-clinical pathway in patients with gastroenteropancreatic NET (GEP-NET) with regard to metastases and symptoms. Retrospective analysis of patients with GEP-NET at a tertiary referral center from 1984 to 2019; inclusion criteria: Patients ≥18 years, diagnosis of GEP-NET; statistical analysis using non-parametrical methods. Four hundred eighty-six patients with 488 tumors were identified; median age at first diagnosis (478/486, 8 unknown) was 59 years; 52.9% male patients. Pancreatic NET: 143/488 tumors; 29.3%; small intestinal NET: 145/488 tumors, 29.7%. 128/303 patients (42.2%) showed NET specific and 122/486 (25%) patients other tumor-specific symptoms. 222/279 patients had distant metastases at initial diagnosis (187/222 liver metastases). 154/488 (31.6%) of GEP-NET were incidental findings. Median time from tumor manifestation (e.g., symptoms related to NET) to initial diagnosis across all entities was 19.5 (95% CI: 12–28) days. No significant difference in patients with or without distant metastases (median 73 vs 105 days, P = .42). A large proportion of GEP-NET are incidental findings and only about half of all patients are symptomatic at the time of diagnosis. We did not find a significant influence of the presence of metastases on time to diagnosis, which shows a large variability with a median of <30 days.
Prothyma (Genoprothyma) thandamoeae Wiesner, Phyu and Hori, new species, Prothyma (Genoprothyma) sotai Wiesner, Phyu and Hori, new species, and Prothyma (Genoprothyma) asamii Wiesner, Phyu and Hori, new species (Coleoptera: Cicindelidae) are described from Myanmar. A key to all members of the genus known to occur in Myanmar is given.
Thopeutica (Thopeutica) petertaylori Medina, Cabras and Wiesner (Coleoptera: Cicindelidae), new species, is described from the Cagan river, New Bataan, Mindanao. It is characterized by the metallic ground colour of the elytra, prominent middle tooth in the labrum, and bottle-shaped aedeagus with apical hook.
Introduction: Scarce data exist for therapy regimens other than somatostatin analogues (SSA) and peptide receptor radiotherapy (PRRT) for siNET. We analyzed real world data for differences in survival according to therapy. Patients and methods: Analysis of 145 patients, diagnosed between 1993 and 2018 at a single institution, divided in treatment groups. Group (gr.) 0: no treatment (n = 10), gr 1: TACE and/or PRRT (n = 26), gr. 2: SSA (n = 32), gr. 3: SSA/PRRT (n = 8), gr. 4: chemotherapy (n = 8), gr. 5: not metastasized (at diagnosis), surgery only (n = 53), gr. 6 = metastasized (at diagnosis), surgery only (n = 10). Results: 45.5% female, median age 60 years (range, 27–84). A total of 125/145 patients with a resection of the primary tumor. For all patients, 1-year OS (%) was 93.8 (95%-CI: 90–98), 3-year OS = 84.3 (CI: 78–90) and 5-year OS = 77.5 (CI: 70–85). For analysis of survival according to therapy, only stage IV patients (baseline) that received treatment were included. Compared with reference gr. 2 (SSA only), HR for OS was 1.49 (p = 0.47) for gr. 1, 0.72 (p = 0.69) for gr. 3, 2.34 (p = 0.19) for gr. 4. The 5 y OS rate of patients whose primary tumor was resected (n = 125) was 73.1%, and without PTR was 33.3% (HR: 4.31; p = 0.003). Individual patients are represented in swimmer plots. Conclusions: For stage IV patients in this analysis (limited by low patient numbers in co. 3/4), multimodal treatment did not significantly improve survival over SSA treatment alone. A resection of primary tumor significantly improves survival.
The Australian endemic staphylinoid plant bug genus Carvalhoma Slater & Gross, 1977 is revised. The genus is redescribed and its systematic position within Cylapinae is discussed. Carvalhoma malcolmae Slater & Gross, 1977 and C. taplini Slater & Gross, 1977 are redescribed. Three species, C. ovatum sp. nov., C. parvum sp. nov., and C. weiri sp. nov., are described as new to science. A key to species, digital habitus images, SEM images, drawings of male and female genitalia and distribution map are provided.
Cylindera (Cylinderina) sierramadrensis Wiesner and Dheurle, new species (Coleoptera: Cicindelidae) is described from Luzon. Re-descriptions of the other seven species of this subgenus (C. (C.) nanula (Horn, 1937), C. (C.) nana (Schaum, 1862), C. (C.) pseudonana (Horn, 1924), C. (C.) werneri Wiesner, 1988, C. (C.) rothschildi (Horn, 1896), C. (C.) genieri Cassola and Werner, 2003, C. (C.) vandenberghei Dheurle, 2016) are provided together with a key and pictures of their habitus, labrum and aedeagus.
Naviauxella varians Wiesner and Constant, n. sp. (Coleoptera: Cicindelidae), is described. Neocollyris (Pachycollyris) bipartita unicolor (Horn, 1935), Therates laotiensis Sawada and Wiesner, 1999, Cosmodela duponti duponti (Dejean, 1826) and Cylindera (Ifasina) somnuki Naviaux, 1991 are recorded for the first time from Cambodia. Five tiger beetle species are recorded for the first time from Kampong Speu province, two for the first time from Ratanakiri province, and one each the first time from Pursat province and Preah Vihear province.