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https://www.readbyqxmd.com/read/28329588/ustekinumab-for-treatment-of-cutaneous-crohn-s-disease
#1
Rana Abdat, Alina Markova, Francis A Farraye, Michael K Lichtman
Cutaneous Crohn disease (CD) affecting the vulva, perineum, and perianal skin, is a rare entity, which may accompany or precede gastrointestinal CD. Vulvar involvement, if untreated, may ultimately require extensive surgery including vulvectomy to gain control of the disease. Both gastrointestinal and cutaneous CD respond to biologics, which block TNF. In addition, ustekinumab, which targets both IL-12 and IL-23 cytokines, is effective in patients with gastrointestinal CD who fail TNF blockade. However, it is unclear if ustekinumab is effective for cutaneous CD...
October 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28321087/myelodysplastic-syndrome-diagnosed-on-the-occasion-of-fournier-s-gangrene
#2
Masaaki Adachi, Kimiyoshi Mitsuhashi, Hiroyuki Matsuda, Junko Watanabe, Katsuya Nakanishi
Fournier's gangrene (FG) is a fulminant infective necrotizing fasciitis, which includes the genital, perineal, and perianal regions. A 77-year-old man had previously been diagnosed as having diabetes mellitus (DM) and was treated with pioglitazone (15 mg) and miglitol (150 mg). He developed sudden perineal discomfort, fever with painful penile, and scrotal edema, subsequently leading to urinary retention. According to physical examination and CT scan results for the swollen penis and scrotum, he was diagnosed with FG...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28319427/new-horizons-in-the-imaging-of-perianal-crohn-s-disease-transperineal-ultrasonography
#3
Cristina Bezzio, Robert V Bryant, Gianpiero Manes, Giovanni Maconi, Simone Saibeni
Perianal disease, most commonly manifest as fistula or abscess formation, affects up to 40% of patients with Crohn's disease. Perianal Crohn's disease is disabling, associated with poor outcomes, and represents a therapeutic challenge for physicians. Correct diagnosis and classification of perianal disease is the first crucial step for appropriate multidisciplinary management. Areas covered: A literature search was performed of the PubMed database using the terms "transperineal ultrasonography", "transperineal ultrasound", "perianal disease", "perianal fistula", "perianal abscess", "magnetic resonance", "endoanal ultrasonography", "endoscopic ultrasound" in combination with "Crohn's disease"...
March 20, 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28293746/long-standing-crohn-s-disease-and-its-implication-on-anal-squamous-cell-cancer-management
#4
Amy L Lightner, Sara B Moncrief, Thomas C Smyrk, John H Pemberton, Michael G Haddock, David W Larson, Eric J Dozois, Kellie L Mathis
BACKGROUND: Anal squamous cell carcinoma (ASCC) is rare, accounting for only 1% of gastrointestinal malignancies. We sought to better understand management strategies for ASCC in the setting of Crohn's disease (CD). METHODS: A retrospective chart review from 2001 to 2016 was conducted using ICD-9/10 codes for CD (555.9/K50) and ASCC (154.3/C44.520). Adult patients with a diagnosis of CD at the time of ASCC diagnosis were included. RESULTS: Seven patients (five female) were included with a median age of 50 years...
March 14, 2017: International Journal of Colorectal Disease
https://www.readbyqxmd.com/read/28291131/study-of-selected-brca1-brca2-and-pik3ca-mutations-in-benign-and-malignant-lesions-of-anogenital-mammary-like-glands
#5
Anastasia M Konstantinova, Ksenya V Shelekhova, Evgeny N Imyanitov, Aglaya Iyevleva, Denisa Kacerovska, Michal Michal, Dmitry V Kazakov
Anogenital mammary-like glands (AGMLGs) are nowadays considered a normal component of the anogenital area. Lesions involving AGMLGs are histopathologically very similar to their mammary counterparts, but the information on molecular biological mechanisms in these vulvar/perianal tumors is scarce. Mutations in the PI3K-AKT cascade have been found in hidradenoma papilliferum. The authors studied selected BRCA1, BRCA2, and PIK3CA mutations in series of benign and malignant neoplasms thought to be associated with AGMLGs, including 9 cases of primary extramammary Paget disease, 3 different cases of mammary-type carcinoma (adenoid cystic like, tubulolobular, and invasive ductal like), and 5 cases of hidradenoma papilliferum...
March 9, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28290676/biochemical-characterization-of-ap-lyase-and-m-6-a-demethylase-activities-of-human-alkb-homolog-1-alkbh1
#6
Tina A Müller, Michael A Tobar, Madison N Perian, Robert P Hausinger
Alkbh1 is one of nine mammalian homologs of Escherichia coli AlkB, a 2-oxoglutarate-dependent dioxygenase that catalyzes direct DNA repair by removing alkyl lesions from DNA. Six distinct enzymatic activities have been reported for Alkbh1, including hydroxylation of variously methylated DNA, mRNA, tRNA, or histone substrates along with the cleavage of DNA at apurinic/apyrimidinic (AP) sites followed by covalent attachment to the 5'-product. The studies described here extend the biochemical characterization for two of these enzymatic activities using human ALKBH1: the AP lyase and 6-methyl adenine DNA demethylase activities...
March 14, 2017: Biochemistry
https://www.readbyqxmd.com/read/28289985/perianal-lesions-in-children-an-updated-review
#7
Michelle Xu, Hannah Liu, Sharon Glick, Amor Khachemoune
Perianal lesions in children are common reasons for dermatology clinic visits and a well-defined approach to diagnosis and management is helpful to the practicing clinician. In this article, we review and update various etiologies of perianal lesions in the pediatric population, including infectious, papulosquamous, vascular, and neoplastic. We provide a standard initial approach to diagnosis and updates on current management. Infectious etiologies of perianal lesions discussed in this article include fungal, bacterial, parasitic, and viral...
March 13, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28289934/anoperineal-disease-in-hidradenitis-suppurativa-mr-imaging-distinction-from-perianal-crohn-s-disease
#8
Louise Monnier, Anthony Dohan, Nedjoua Amara, Anne-Marie Zagdanski, Moustapha Drame, Philippe Soyer, Christine Hoeffel
OBJECTIVE: To determine whether Hidradenitis suppurativa (HS)-related anoperineal disease can be distinguished from Crohn's disease (CD) using MRI. METHODS: Pelvic MRI of 23 HS and 46 CD patients with anoperineal disease between 2007 and 2014 were independently analysed by two radiologists. For diagnosis of HS, sensitivity, specificity and positive likelihood ratios (LRs) of inflammatory features and of their anatomical distribution were calculated and compared to those of CD...
March 13, 2017: European Radiology
https://www.readbyqxmd.com/read/28284459/unexpected-presentation-of-perianal-granular-cell-tumor-in-a-young-woman
#9
Laila Rashidi, Darren Pollock, Sarah Bastawrous
Granular cell tumors originate from Schwann cells of the soft tissues and are rarely observed in the perianal region. Often, correct diagnosis can be challenging owing to nonspecific clinical symptoms and imaging characteristics, as well as its ability to mimic other malignant lesions histologically. We describe the case of a previously healthy 36-year-old woman who presents with a slow growing, painless lump in her perianal region who underwent surgical excision and was found to have a granular cell tumor on microscopic evaluation...
January 14, 2017: Current Problems in Diagnostic Radiology
https://www.readbyqxmd.com/read/28281511/-efficacy-of-adalimumab-for-crohn-s-disease-in-real-clinical-practice
#10
O V Knyazev, A V Kagramanova, I N Ruchkina, N A Fadeeva, A A Lishchinskaya, O N Boldyreva, E Yu Zhulina, P L Shcherbakov, N V Orlova, M V Kirova, A I Parfenov
AIM: To evaluate the efficacy and safety of adalimumab (ADA) in patients with Crohn's disease (CD) treated at the Department of Inflammatory Bowel Diseases, Moscow Clinical Research and Practical Center, and to determine the predictors of a therapy response. SUBJECTS AND METHODS: All the patients with CD treated with ADA were followed up for at least 6 months or until the drug was discontinued. Therapeutic effectiveness was evaluated at 4 weeks and 6 months after the initiation of treatment and at the end of a follow-up...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28277336/development-and-function-of-immune-cells-in-an-adolescent-patient-with-a-deficiency-in-the-interleukin-10-receptor
#11
Sharon Veenbergen, Marieke A van Leeuwen, Gertjan J J Driessen, Rogier Kersseboom, Lilian F de Ruiter, Rolien H C Raatgeep, Dicky J Lindenbergh-Kortleve, Ytje Simons-Oosterhuis, Katharina Biermann, Dicky J J Halley, Lissy de Ridder, Johanna C Escher, Janneke N Samsom
OBJECTIVE: Monogenic defects in the interleukin-10 (IL-10) pathway are extremely rare and cause infantile-onset inflammatory bowel disease (IBD)-like pathology. Understanding how immune responses are dysregulated in monogenic IBD-like diseases can provide valuable insight in "classical" IBD pathogenesis. Here, we studied long-term immune cell development and function in an adolescent IL-10 receptor (IL10RA)-deficient patient who presented in infancy with severe colitis and fistulizing perianal disease and is currently treated with immune suppressants...
March 8, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28276118/malignant-anal-sac-melanoma-in-dogs-eleven-cases-2000-to-2015
#12
A Vinayak, C B Frank, D W Gardiner, K M Thieman-Mankin, D R Worley
OBJECTIVES: To report the signalment, clinical presentation, treatments pursued and outcomes of dogs with malignant anal sac melanoma. METHODS: Medical records from five institutions from January 2000 through December 2015 were reviewed and dogs with cytologically- or histologically-confirmed malignant anal sac melanoma were identified. Signalment, clinical signs, staging, cytology, histopathologic analysis, surgical and non-surgical treatments were extracted from the medical records...
April 2017: Journal of Small Animal Practice
https://www.readbyqxmd.com/read/28270879/anal-neuroendocrine-tumor-masquerading-as-external-hemorrhoids-a-case-report
#13
Muhammad Khan, Ahmed Dirweesh, Chikezie Alvarez, Herbert Conaway, Robert Moser
Neuroendocrine tumors in gastrointestinal (GI) tract are a rare source of GI malignancy with an estimated incidence of 2.5 - 5 per 100,000 people per year and the prevalence of 35 per 100,000. In the GI tract, they are located in decreasing order of frequency in appendix, ileum, rectum, stomach, and colon. Those found in the anal region represent just 1% of all malignancies of the anal canal. Their clinical presentation can be widely varying, sometimes being found incidentally with metastatic disease and an unknown primary source...
February 2017: Gastroenterology Research
https://www.readbyqxmd.com/read/28267044/mutations-in-interleukin-10-receptor-and-clinical-phenotypes-in-patients-with-very-early-onset-inflammatory-bowel-disease-a-chinese-veo-ibd-collaboration-group-survey
#14
Zhiheng Huang, Kaiyue Peng, Xiaoqin Li, Ruiqin Zhao, Jieyu You, Xiuyong Cheng, Zhaoxia Wang, Ying Wang, Bingbing Wu, Huijun Wang, Huasong Zeng, Zhuowen Yu, Cuifang Zheng, Yuesheng Wang, Ying Huang
BACKGROUND: Interleukin-10 (IL10) signaling plays an important role in the pathogenesis of very early onset inflammatory bowel disease (VEO-IBD) in children. However, little is known about the role of the IL10 axis in children with VEO-IBD in China. METHODS: The Chinese VEO-IBD Collaboration Group was created to collect clinical and genetic data from patients deficient in IL10 and the IL10 receptor. High-throughput sequencing was performed to identify mutations in these genes...
April 2017: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/28257985/microbiological-aspects-of-fournier-s-gangrene
#15
Tuncay Yilmazlar, Baris Gulcu, Ozgen Isik, Ersin Ozturk
BACKGROUND: Fournier's gangrene (FG) is a devastating disease that is characterized by necrotizing fasciitis of the perineal, genital, or perianal region. Broad-spectrum antibiotics are the key component of its treatment. However, there is paucity of data regarding the optimal empirical antibiotherapy for FG. MATERIALS AND METHODS: Data from patients who underwent surgery for FG between January 2007 and December 2012 were retrieved from a prospectively collected departmental FG database...
February 28, 2017: International Journal of Surgery
https://www.readbyqxmd.com/read/28256718/lipocalin-2-is-expressed-by-activated-granulocytes-and-keratinocytes-in-affected-skin-and-reflects-disease-activity-in-acne-inversa-hidradenitis-suppurativa
#16
K Wolk, J Wenzel, A Tsaousi, E Witte-Händel, N Babel, C Zelenak, H-D Volk, W Sterry, S Schneider-Burrus, R Sabat
BACKGROUND: Acne inversa (AI)/Hidradenitis suppurativa is a chronic inflammatory disease characterized by painful axillary, inguinal, and perianal skin lesions with deep-seated nodules, abscesses, and fistulae. OBJECTIVES: This study aimed at the identification and characterization of key players in AI pathogenesis. METHODS: Epidemiologic and anamnestic data as well as blood and skin samples of AI patients were collected. Healthy participants and psoriasis patients served as controls...
March 3, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28255911/solitary-rectal-ulcer-syndrome-in-children-a-case-series-study
#17
K Kowalska-Duplaga, I Lazowska-Przeorek, K Karolewska-Bochenek, M Woynarowski, G Czaja-Bulsa, A Stawarski, S Pieczarkowski, E Hapyn, J Jozefczuk, B Korczowski, A Szaflarska-Poplawska, A Banaszkiewicz
Information on solitary rectal ulcer syndrome (SRUS) in children is limited and based on case reports only. This study was undertaken with the objective of describing the clinical history, symptoms, diagnostic work-up, and treatment of a large case series of pediatric patients with SRUS. The study was multi-center and retrospective. All pediatric endoscopists in Poland were invited to participate in the study and were asked to look through their endoscopic databases to identify SRUS cases from the last 10 years...
March 3, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28255623/anal-canal-duplication-and-triplication-a-rare-entity-with-different-presentations
#18
P Palazon, V Julia, L Saura, I de Haro, M Bejarano, C Rovira, X Tarrado
Anal canal duplication (ACD) is the rarest of gastrointestinal duplications. Few cases have been reported. Most cases present as an opening in the midline, posterior to the normal anus. The aim of our revision is to contribute with eight new cases, some of them with unusual presentations: five presented as the typical form, one with a perianal nodule, and two presented as two separate orifices (anal canal triplication). Complete excision was performed in all patients with no complications. ACD is the most distal and the least frequent digestive duplication...
March 2, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28251356/midterm-outcomes-of-injectable-bulking-agents-for-fecal-incontinence-a-systematic-review-and-meta-analysis
#19
REVIEW
K D Hong, J S Kim, W B Ji, J W Um
BACKGROUND: Various bulking agents have been used to treat fecal incontinence. While short-term outcomes are attractive, there is still a lack of long-term data. The aim of this systematic review and meta-analysis was to investigate the midterm outcomes of treatment with injectable bulking agents and to identify predictive factors for improvement in incontinence. METHODS: PubMed, EMBASE, Web of Science, and Cochrane Library databases were searched using the terms injection, bulking agents, and fecal incontinence...
March 1, 2017: Techniques in Coloproctology
https://www.readbyqxmd.com/read/28229030/retroperitoneal-necrotizing-fasciitis-masquerading-as-perianal-abscess-rare-and-perilous
#20
Anandhi Amaranathan, Ashok Kumar Sahoo, Deepak Barathi, Gomathi Shankar, Sarath Chandra Sistla
Necrotizing fasciitis is one of the uncommon presentations of a rapidly spreading subcutaneous tissue infection. Although the actual cause is unclear in many cases, most of them are due to the rapid proliferation of microorganisms. Retroperitoneal necrotizing fasciitis is extremely rare. It is a potentially lethal infection that requires immediate and aggressive surgical care. Early diagnosis is the key to a better prognosis. The possibility of retroperitoneal necrotizing fasciitis should be suspected in patients with symptoms of sepsis that are disproportionate to clinical findings...
January 17, 2017: Curēus
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