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Volumen 7, Asunto 2 (2017)

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Peripheral Necrosis in an Ebola Survivor Case

Thakor Hitendrasinh G, MarkAlain Déry, Jeffery Gilbert, Chad K Bush and Mohamed Alieu Shour

The most common symptoms in the present outbreak in West Africa region are fever, fatigue, vomiting, diarrhoea, loss of appetite, headache, abdominal pain and myalgias. However, a case of peripheral necrosis was observed in Sierra Leone where the tips of the fingers of both the hands get necrosis in a survivor of Ebola infection. The present case study discusses the findings and the pathology of the same.

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Lyell’s Syndrome: Effectiveness of a Diagnostic Management and Early Treatment

Amine Salmi

Lyell’s syndrome or toxic epidermal necrolysis is a bullous dermatosis, of etiology, very often medicated. It is an acute necrosis of the epidermis, with a clinical appearance of extensive burns. At this table are associated mucosal damage and, frequently, a multiorgan making poor prognosis. We report in this paper a case of toxic epidermal necrolysis, which illustrates the importance of early treatment in an intensive care unit, whose foundations are based on fluid and electrolyte intake, infection prevention and its treatment with appropriate antibiotics.

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Rare Coagulation Factor Deficiencies Associated with Congenital Abnormalities

Baris Malbora, Murat Derbent and Namik Ozbek

Among bleeding disorders, hemophilia A, hemophilia B and von Willebrand diseases are the most commonly occurring, whereas deficiencies of other coagulation factors are rare worldwide. Unlike hemophilias, which are X-chromosome linked disorders, the inheritance pattern of the rare coagulation factor deficiencies is generally autosomal recessive. In literature, association of these factor deficiencies and congenital abnormalities are especially rare. In this study, we present our cases having both rare factor deficiencies and congenital abnormalities, including Cenani-Lenz syndrome and Duane retraction syndrome.

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Hypophosphataemic Rickets Due to Parenteral Ferrous Carboxymaltose in a Young Man with Crohn Disease and Iron Deficiency: A Case Report and Review of Literature

Michael Reyes and Terrence Diamond

We present the case of recurrent fractures and hypophosphataemic rickets that occurred in a young man who received parenteral ferrous carboxymaltose (FCM) for chronic iron deficiency associated with Crohn disease. The effect of FCM on bone and mineral metabolism and the frequency of this disorder are discussed.

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Anaphylaxis as a Manifestation of Systemic Mastocytosis: A Case Report and Review of Literature

Elie Haddad, Carmen Joukhadar, Nabil Chehata and Roy Nasnas

Introduction: Anaphylaxis is defined as “a serious, life-threatening generalized or systemic hypersensitivity reaction” and “a serious allergic reaction that is rapid in onset and might cause death”. The determination of different mast cells (MC) mediators, such as serum and/or plasma histamine and tryptase, is proposed for the diagnosis of anaphylaxis. There are differences in the clinical presentation of anaphylaxis in patients with indolent systemic mastocytosis versus idiopathic anaphylaxis. Case presentation: This is the case of a 72-year-old female patient presenting with acute bacterial pyelonephritis treated with intra venous antibiotics. She developed a facial flush then a diffuse flush with hypoxia and respiratory arrest. The patient recovered with non-invasive ventilation without the need for intubation. A full blood panel returned normal and two bone marrows biopsies were performed. The second one showed the presence of a perivascular infiltrate mast cell, consistent with systemic mastocytosis. Discussion: All aspects of systemic mastocyosis including the classification and the main clinical manifestations as well as the diagnosis were concisely reviewed. Then supportive and immunomodulator treatment were differentiated. Conclusion: Patients suffering of systemic mastocytosis typically present anaphylactic symptoms primarily involving the cardiovascular system in the absence of urticaria and/or angioedema, which makes such clinical presentation highly suspicious. Patients suffering of c-MCD (Cutaneous-mast cell disease) need assistance in identifying their triggers and education on proper avoidance.

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Dilemma on Classifying Diabetes Mellitus in Adolescence: A Case Report and Review of Literature

Che-Ming Chiang, Li-Wei Wu, Wei-Liang Chen, Chung-Ching Wang, Shih-Yao Liu, Chien-Hsing Lee, Kao-Hsian Hsieh, Chih-Chien Wang and Chien-Ming Lin

Diabetic ketoacidosis (DKA) is one of the most serious complications of diabetes mellitus (DM) that is prone to occur in children with type 1 DM (T1DM). We report a 17-year-old overweight girl who was treated with multiple oral antihyperglycemic drugs under the initial impression of type 2 DM (T2DM). Nevertheless, severe euglycemic DKA happened after the additional prescription of sodium-glucose cotransporter 2 inhibitors for two months. Consequently, T1DM was diagnosed on the basis of insulinopenic status in glucagon test and the presence of islet autoantibodies. This case highlighted the importance of precise classification before diabetes treatment in adolescence. A literature review is aimed to help clinicians differentiate various types of DM more specifically in clinical practice

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Case of Bilateral Primary Testicular Lymphoma in a Chronic Renal Disease Patient

Maheshgir S Gosavi

Lymphoma constitutes about 5% of testicular neoplasms and aggressive form of extanodal lymphoma. A case of bilateral scrotal swelling in a known case of Chronic renal disease of which on USG showed multiple hypoechoic lesions on further histopathological study was diagnosed as primary diffuse primary large B-cell NHL and later treated with chemotherapy.

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Brugada Syndrome: Management and Anaesthetic Implications: A Case Report

Nuria Esteve, Vicente Cuquerella, Marta Resalt and Ana Perez

Brugada syndrome (BrS) is a genetically determined disorder and has an autosomal dominant pattern of transmission, with incomplete penetrance and variable expression. These alterations may predispose to the development of reentrant arrhythmias, and therefore cause ventricular tachycardia and ventricular fibrillation. Patients may be asymptomatic, but they are prone to develop ventricular tachycardia or ventricular fibrillation and sudden death. Many events that occur during general anesthesia, such as drugs, temperature changes, and heart rate variations, may precipitate lethal arrhythmias in these patients. Thus, an individual anesthetic plan, taking into consideration the drugs that may induce arrhythmias, must be arranged before the surgery. Furthermore, it is essential to provide an exhaustive monitoring during the entire perioperative period. We describe a case of general anesthesia in a patient with Brugada syndrome who was diagnosed with right colon neoplasia and programmed for scheduled right hemicolectomy by laparotomy.

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Metastatic Colon Cancer from Asymptomatic Gastric Stump Cancer: Report of a Rare Case

Lei-Ying Huang, Wen-Yih Liang, Jeng-Kai Jiang and Hung-Hsin Lin

Background: Herein we present a rare case of metastatic tumor in transverse colon originating from an asymptomatic gastric stump cancer.

Case presentation: A 55-year-old woman underwent partial gastrectomy and pyloroplasty for the gastrointestinal stromal tumor in the stomach in 2006. She developed abdominal fullness and a reduced appetite in April 2015. Colonoscopy and computerized tomography indicated primary transverse colon cancer with obstruction and multiple liver metastases. She underwent an emergency extended right hemicolectomy, and pathological analysis revealed a secondary colon cancer. A series of examinations after an operation, including gastric endoscopy with biopsy, and pathology examination including immunohistochemical studies, indicated a metastatic adenocarcinoma from a gastric stump.

Conclusion: This case may provide physicians with additional knowledge on rare presentations of gastric stump.

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Successful Treatment With Lymphovenous Anastomosis for Lower Extremity Edema Secondary to Lipiodol Lymphangiography

Shoichi Sasaki, Yasutoshi Suzuki, Kohei Umekawa, Takashi Kurabayashi and Hirotaka Asato

Background: Chylothorax is one of the complications after thoracic surgery and treated by conservative or surgical means. Lipiodol lymphangiography is one of the options and it causes obliteration of chylous leak by inflammatory manner. In this article, we describe a case of lymphedema of the bilateral lower extremities occurs after lipiodol lymphangiography and it is treated successfully by lymphovenous anastomosis. Case presentation: A 67-year-old man presented with refractory chylothorax after subtotal esophagectomy and thoracic lymph node dissection. His chylothorax developed 4-month later of subtotal esophagectomy and was refractory to the conservative treatment (i.e. tube thoracostomy). He was referred to our department to treat chylothorax. We chose lipiodol lymphangiography as the treatment. Lymphatic duct of left foot was detected with indocyanine green and exposed to inject lipiodol into lymphatic duct directly. Chylothorax improved immediately after lipiodol lymphangiography and his edema of right lower extremity emerged 22-month later of lipiodol lymphangiography. We considered that his lower extremity edema was caused by lipiodol lymphangiography and performed lymphovenous anastomosis. Lymphovenous anastomosis was performed at the proximal of right thigh and the dorsum of the foot. At six-month later of lymphovenous anastomosis, we revealed that his right lower extremity had become thinner significantly, nevertheless laterality remained. Conclusion: To our best knowledge, this is the first report of lymphedema of the bilateral lower extremities after lipiodol lymphangiography for chylothorax. Lymphovenous anastomosis is a treatment option for such condition.

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A Case of Pregnancy Complicated with Type 1 Hereditary Angioedema (HAE 1)

Junko Ushijima, Shigetane Sasaki, Isao Horiuchi, Hiroko Masuko, Tomoyuki Kuwata, Tisio Demitsu and Kenjiro Takagi

Hereditary angioedema (HAE) is an inherited autosomal dominant trait and symptoms of HAE include sudden onset of edema in various organs, such as subcutaneous edema, laryngeal edema and gastrointestinal tracts mucosa. We have experienced a case of a pregnant woman diagnosed with HAE and successfully managed her pregnancy. The patient was 37 years old, gravida 1, primiparous. She had experienced two attacks of angioedema during pregnancy but her delivery course was not affected by her HAE. The frequency of attacks during pregnancy had decreased compared with before and after pregnancy. Treatment using human C1-inactivator concentrate was as effective for attack suppression during pregnancy as it had been in her non-pregnant state.

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Severe Calcification in the Abdominal Pocket of an Implantable Pulse Generator (IPG) in a Patient on Long-Term Hemodialysis

Sven Bamps, Joost Dejaeger, Ben Sprangers and Tom Theys

Introduction: Ectopic mineralization has been described in patients with end-stage renal failure managed with long-term hemodialysis. This study reports the first case of severe focal ectopic calcification in an implantable abdominal pulse generator pocket. The patient has a medical history of long-term hemodialysis and failed back surgery syndrome. The failed back surgery syndrome is treated with a spinal cord stimulator and an implantable pulse generator (IPG).

Case report: Severe ectopic calcification of an implantable abdominal pulse generator pocket was observed in a 58-year-old female patient undergoing IPG relocation. The patient had a medical history of long-term hemodialysis for end-stage renal failure. She is also known with a spinal cord stimulator and an IPG for failed low back surgery syndrome. Microbiological study was negative and histopathological analysis confirmed the presence of focal calcifications in the implantable abdominal pulse generator pocket and on the IPG itself.

Discussion: Extra-cellular matrix (ECM) calcification is highly prevalent in patients with end-stage renal failure. The exact mechanism remains unclear. Physiopathological disturbance in the calcium and phosphate homeostasis finally and a pro-inflammatory environment result in soft tissue.

Conclusion: This is to our knowledge the first case reported in the literature of dystrophic severe focal ectopic calcification in the abdominal pocket of an implantable pulse generator (IPG) in a patient on long-term hemodialysis. Pain physicians should be aware of this phenomenon.

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