Vol 3-2 Mini Review

Postorgasmic Illness Syndrome: What do we know so far?

Tan Vu Le1, Hoang Minh Tue Nguyen1, Wayne J.G. Hellstrom1*

1Tulane University School of Medicine, Department of Urology, New Orleans LA, USA

Post-orgasmic illness syndrome (POIS) is rare condition that is characterized by transient flu-like symptoms and cognition disorders that occur shortly after ejaculation and last for 2-7 days. There are about 50 cases of POIS in the literature. The prevalence and incidence of POIS are still unknown due to a paucity of studies. The exact pathogenesis of POIS remains unknown; the most acceptable hypothesis is an autoimmune/allergic process. We made a literature search via PubMed for publications from 2002 to 2018 with the “post orgasmic illness syndrome” medical subject heading term to analyze current data regarding symptoms, burden, pathophysiology, and to discuss potential management options for POIS. POIS is diagnosed by five preliminary diagnostic criteria. It is categorized into two types: primary and secondary. There is the concomitance between POIS and PE. The autoimmune/allergy hypothesis is the most accepted hypothesis explaining POIS pathogenesis. A competing hypothesis involves a disorder of endogenous μ-opioid receptors. Patients with POIS have been symptomatically treated with antihistamines, selective serotonin reuptake inhibitors, and benzodiazepines. A trial of hyposensitization therapy with autologous semen was successful. A trial of nonsteroidal anti-inflammatory medication helped in a single case report but failed to successfully treat other patients with POIS.

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Vol 3-2 Short Report

Clinical diagnostic pearls in Familial Dysautonomia

Natan Gadoth1*

1The Sackler faculty of medicine, Tel-Aviv University and Maynei Hayeshua Medical Center, Bnei Barak, Israel

Rare genetic disorders are usually a diagnostic challenge mainly due to the complexity of the clinical presentation which is frequently changing with age and progression of the particular disorder. Familial Dysautonomia is a rare and complex multisystem disorder with peculiar but distinct clinical features . Some of those features are unique to this particular syndrome and the one who is familiar with them can quite easily reach the diagnosis without the need for sophisticated laboratory work-up. In this short review those “clinical pearls” will be described in some detail.

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Vol 3-2 Commentary

Commentary: Individuals affected by Eosinophilic Gastrointestinal Disorders Have Complex Unmet Needs and Experience Barriers to Care

Girish Hiremath1*, Evan S. Dellon2

1Division of Pediatric Gastroenterology, Hepatology and Nutrition, Monroe Carell Jr. Children’s Hospital at Vanderbilt, Nashville TN 37232, USA

2Division of Gastroenterology and Hepatology, University of North Carolina School of Medicine, Chapel Hill, NC 27514, USA

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Vol 3-2 Review Article

Inflammatory Breast Cancer: A Panoramic Overview

Sangjucta Barkataki1,2, Madhura Joglekar-Javadekar1,2, Patti Bradfield3, Thomas Murphy1, Diana Dickson-Witmer2,4 and Kenneth L. van Golen1,2*

1The University of Delaware Department of Biological Sciences, Newark, DE, USA

2The Center for Translational Cancer Research, Newark, DE, USA

3The Inflammatory Breast Cancer Foundation, Newark, DE, USA

4The Breast Center at the Helen F. Graham Cancer Center, Christiana Care Health System, Newark, DE 19716-2500, USA

Inflammatory breast cancer (IBC) is a unique breast cancer with a highly virulent course and low 5- and 10-year survival rates. Although IBC only accounts for 1-5% of breast cancers it is estimated to account for 10% of breast cancer deaths annually in the United States. The accuracy of diagnosis and classification of this unique cancer is a major concern within the medical community. Multimodality treatment includes preoperative chemotherapy, mastectomy, and radiation therapy is the therapeutic mainstay and has been shown to improve prognosis. The potential for inaccurate diagnosis and misclassification in cases of IBC is increased by many factors. This includes the misleading initial symptoms of IBC. The early signs of IBC will present in women who have inflammation of the skin of the affected breast, as well as red or purple coloration of the inflamed area. Molecular studies have shown unique signature genes that are hallmarks of IBC. The current article reviews multiple aspects of primary inflammatory breast cancer.

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Vol 3-2 Commentary

Commentary: Quadriparesis Caused by Lead Poisoning Nine Years after a Gunshot Wound with Retained Bullet Fragments: A Case Report

Emma Nally1,2, Robert D. Bunning1*

1MedStar National Rehabilitation Hospital, 102 Irving St., NW, Washington, DC, 20010, USA

2Georgetown University Hospital, Department of Rehabilitation Medicine, Washington, DC, USA

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Vol 3-3 Mini Review

Geographic Diversity in Atypical Hemolytic Uremic Syndrome (aHUS): The Genetic Background of aHUS Cohort in Japan

Yoichiro Ikeda*, Yoko Yoshida, Yuuka Sugawara, Masaomi Nangaku

Division of Nephrology and Endocrinology, The University of Tokyo, 7-3-1, Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan

Atypical hemolytic uremic syndrome (aHUS) is a rare disease caused by the dysfunction of the alternative pathway of the complement system, which leads to the spontaneous activation of the complement system in the circulating plasma or cell surface. Recently our group published the cohort analysis of aHUS in Japan (n=118). Through the study, we revealed the followings; 1) the genetic background of aHUS in Japan was different from that in Western countries, 2) the most frequent genetic mutation detected in this study was I1157T in C3 (n=24), which was associated with superior renal outcome in spite of frequent replases, 3) Anti-CFH antibody positive aHUS had an excellent renal outcome, 4) 44% cases presented nephrotic syndrome, 5) only 12 % developed end stage renal disease (ESRD) and 6) there were 13 cases that discontinued eculizumab treatment and were followed up. These findings might help establishing the robust evidence for the optimal treatment of aHUS.

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