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Volumen 7, Asunto 8 (2017)

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Patients with Behcet’s Disease and Cardiovascular Risk Factors are at Risk for Early Cardiovascular and Renal Disease

Elon HCVD, Houwen TBVD, Kappen JH, Daele PV, Caliskan K, Hagen MMV and Laar JAMV

We present a case of a 39-year-old Turkish female patient with a mild clinical phenotype of Behçet’s disease (BD), but experiencing severe cardiac and renal disease, probably enhanced by microvascular atherosclerosis and hypertension. In this case, several cardiovascular risk factors like positive family history, hypertension and smoking were present. Up until recently cardiovascular risk factors were relatively neglected in BD, however recent reports suggest that traditional risk factors are an independent predictor of early coronary artery disease-related morbidity in young BD patients. With this case report we want to stress the fact that severe atherosclerotic complications occur even in young BD patients with longstanding stable, mild disease. This is probably due to chronic inflammation process in chronic diseases like BD, which is a known factor in the initiation and progression of the atherosclerotic process. Therefore, a tight, careful risk factor management should be implied during the follow-up of BD patients.

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Sarcoidosis and Collagenous Colitis– Important Clinical Association or Coincidence?

Aleksandar Gavrić, David Drobne, Nina Zidar and Borut Štabuc

We present a case of a 57-year-old woman with two rare concomitant diseases; sarcoidosis and collagenous colitis. Patient was admitted to our hospital with the symptoms of watery diarrhea that intermittently lasted for years because of delayed diagnosis. Despite increasing awareness of microscopic colitis, the delayed diagnosis remains an important problem. Diagnosis was quickly confirmed with flexible proctosigmoidoscopy. Rectal biopsies were sufficient for diagnosis. Symptoms improved dramatically the second day of the induction therapy with budesonide. Causal relationship between sarcoidosis and microscopic colitis is not yet confirmed, and to our knowledge, this is the first such case report.

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An Infertility SRY-Negative 46, XX Male Detected by Quantitative Fluorescent Polymerase Chain Reaction

Cao Thi Tai Nguyen, Nguyen Trung Kien, Vu Thi Nhuan, Nguyen Dac Khoa, Nguyen Chung Vieng, Nguyen Thi Bich Ngoc, Nguyen Phan Vinh, Trinh Minh Thiet, Cao Luon Binh and Trinh Thi Bich Lien

Taking a frequency of 1 in 20,000–25,000 males, De la Chappelle syndrome (46, XX male) is a rare syndrome which exists in different clinical categories with ambiguous genitalia or partially to fully mature male genitalia, in combination with complete or incomplete masculinization. A case of SRY-negative XX male is reported with complete masculinization but with infertility. The patient was mature enough to have full genitalia with descended but small testes and no signs of undervirilization. There were 14 markers in the quantitative fluorescent polymerase chain reaction (QF-PCR): amelogenin gene, presented on X and Y chromosomes allows for the determination of the Y/X ratio (AMEL marker), TAF9B gene presented on chromosomes X and 3 permits the determination of X/chromosome 3 ratio (TAF9B marker or T3 marker), ten non- polymorphic Y-specific markers (SRY, sY84 and sY86 in AZFa region, sY127 and sY134 in AZFb region, sY254, sY255, sY1191, sY1192 and sY1291 in AZFc region), and co-amplification of DAZ/DAZL and CDY2/CDY1 fragments that permit determination of the DAZ, CDY1 and CDY2 gene copy number. The study resulted that there were only two peaks of markers: AMELX/Y marker produced a peak area ratio of 1:0 and T3 with the ratio of 2:2, indicating the presence of two X chromosomes in this case. The absence of the Y chromosome was confirmed when repeating peripheral blood culture for chromosome: there are 46 chromosomes with XX constitution. The first reported case in Mekong Delta, Vietnam is reported of a SRY-negative 46, XX male with normal male phenotype and infertility.

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A Rare Case of Spontaneous Pneumocephalus as a Complication of Nontraumatic Nasal Liquorrhea

Shelesko EV, Chernikova NA and Zaitsev OS

Pneumocephalus is the ingress of air into the intracranial cavity. Pneumocephalus is associated with several etiological factors, such as head injuries, surgical interventions, infections and neoplasms. With spontaneous nasal liquorrhea, pneumocephalus is extremely rare, since a defect must be large in order to cause it. Clinical implications of pneumocephalus depend on location and volume of air in the cranial cavity. The most common and described symptoms are headache, “splashing sound”, rhinorrhea and otorrhea, meningism, dysfunction of cerebrospinal nerves, hemiparesis, optic disc edema, epileptic seizures, collaptoid states, psychiatric symptoms. For treatment of pneumocephalus, both conservative and surgical methods are used. The choice of tactics depends on type, etiology and volume of the air that has entered the cavity of the skull.

In this article, we describe the case of effective treatment of spontaneous nasal liquorrhea, complicated by pneumocephalus and meningitis.

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Arcuate Line Hernia Initially Missed Getting Complicated: A Case Report

Matthias Hugot, Emilie Nicodème- Paulin and Alexandre Stolz

The arcuate line hernia is an, usually asymptomatic, ascending protrusion of intra peritoneal structure under the fold of Douglas, classified into three types depending on its severity and the degree of complication. We report a case of a 64-year-old Caucasian woman whose diagnosis of arcuate line hernia was initially missed at a computed tomography and diagnosed 3 weeks later on the repeat computed tomography scan when an intrahernian necrosis of the epiploic fat appeared. The present case discusses the classical anatomical and radiological features. The therapeutic approach is based on laparoscopic surgery.

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Chronic Pain Treated Successfully with Novel Taping Therapy: A New Approach to the Treatment of Pain

Hwa Soo Hwang, Bong Hyo Lee and Emily Bien

Objective: We report four cases of obstinate pain treated successfully using a taping therapy, to inform readers a novel approach to the treatment of pain.

Cases: (a) Pain lasted for 26 years at forehead with no special sign under imaging examination; (b) pain had been around the styloid process of the 5th metatarsal after ankle sprain that occurred 3 months ago. Physical therapy worked for ankle pain but not for styloid process pain; (c) low back pain lasted for several months especially while sit on a floor; (d) heel pain lasted for 1 year and was diagnosed with plantar fasciitis.

Results: (a) Pain was not ameliorated with treatment of sternocleidomastoid. Tape was attached on the local then the pain dramatically decreased; (b) tape was attached on dorsal around the styloid process following the patient’s statement but produced no effect. After additional examination, tape was attached on the sole and the pain was markedly reduced; (c) tape was attached at sacrum following painful response. However, the first treatment’s effect did not recur during following treatments. After additional examination, painful response was found at the coccyx, not sacrum, as if the pain shifted. After attaching at coccyx, the pain disappeared; (d) tape was attached around heel following finger pressure examination. After additional self-treatments for 2 weeks, the pain disappeared.

Conclusion: Finger pressure eliciting painful response is useful for examination and evaluating the effect of treatment in chronic local pain. Regulating patient’s bioelectric currents can be a novel strategy for treatment of pain.

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Myxoid Endometrial Stromal Sarcoma: A Case Report and Literature Review

Young-Mee Lim, Gina Nam, Heejin Bang, Sewha Kim, Min-Sun Cho, Seung-Cheol Kim and Yun Hwan Kim

Myxoid type of endometrial stromal sarcoma (ESS) is a very rare tumor of uterine malignancies. Although several cases about pathologic features for the diagnosis of ESS have been reported, it remains still difficult to find a study of the clinical course, management, and outcome of ESS. Here we report a postmenopausal woman with myxoid ESS of low grade type who had aggressive clinical features. Although adequate progestin therapy was provided followed by total abdominal hysterectomy and bilateral salpingo-oophorectomy, they failed to prevent early recurrence of ESS. Following cytoreductive surgery and chemotherapy were also proved to be refractory to control the rapid progression of ESS.

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A Case of Pott’s Disease: Cold Abscess in a Haitian Woman Presenting with Chronic Back Pain

Sahar T, Heela A, Tarundeep G, Alexa K, Zaid S and William L

Background: Tuberculous spondylitis is a bacterial infection caused by Mycobacterium tuberculosis. This bacteria usually attacks the lungs but can attack any part of the body such as the kidney, spine, and brain. Spine being the most frequently affected site, Spinal TB patients typically present with back pain but other constitutional or pulmonary symptoms may be absent, rendering the diagnosis difficult. Mycobacterium tuberculosis can remain dormant for decades after the initial infection and doesn’t always cause infections.

Case: We report a case of a 23-year-old Haitian woman who presented with increased swelling in right lower flank and vague lower back pain for two years before being diagnosed with tuberculous spondylitis.

Conclusion: Physicians in the USA often miss opportunities to identify patients with tuberculous spondylitis considering its endemic in various countries around the world. Symptoms may be vague and screening tests have limitations. We review current strategies for diagnosis, and treatment of tuberculous spondylitis in immigrant patients who have lived in endemic areas and discuss the clinical features and management the infection.

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A Severe Case of Recurrent External Endometriosis in An Ovariectomized Young Woman

Mykhailo VM, Marina YE, Ioannis PK, Antonio M, Andrea T and Ospan AM

This is a case of a 35-year-old ovariectomized woman with recurrent external endometriosis. During 15 years she has undergone 3 laparotomies, excisions of 159 recurrent abdominal wall endometriotic lesions and 12 vulvar, bilateral labia majora and pubis enodmetriotic lesions. Letrozole (7.5 mg/daily) treatment provided some relief of acute pain.

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Toxic Leucoencephalopathy Due to CHOP Therapy in a Patient with NonHodgkin’s Lymphoma

Chetanya Malik, Aditya Kutiyal, Sanjay Pandit, Sandeep Garg and Naresh Gupta

Toxic leucoencephalopathy is damage to cerebral white matter due to exposure to cranial irradiation, environmental toxins, drugs of abuse and chemotherapeutic agents. It results in wide variety of clinical manifestations like personality change, inattention, memory loss, abulia, stupor, coma and death. It leads to white matter demyelination which is visible on T2 weighted MRI as hyperintensity. We report a rare case of a 14-year-old boy with large B cell lymphoma who developed toxic leucoencephalopathy due to exposure to CHOP regimen.

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Fracture Dislocations of the Coccyx: A Case Series and Literature Review

Kamal Hamoud and Janan Abbas

Study design: A case report and literature review.

Objective: To present a rare case series of dislocation of the mid-coccygeal and sacro-coccygeal joints in a young adult and a baby, who were treated conservatively.

Summary of background data: Dislocations of the mid-coccygeal or the sacro-coccygeal joints are infrequent injuries and are rarely reported. The treatment for these disorders is usually conservative.

Methods: Detailed description of the posterior dislocation of the mid-coccygeal joint in a 19-year-old patient and a complete anterior dislocation (Salter-Harris type I) of the sacro-coccygeal joint in a 2 year and 8 months baby and management of both cases are presented, with review of the relevant literature.

Results: A conservative treatment was performed in both cases. Clinical and radiological results at sixteen months after the injury in the first case, and five months in the second, revealed excellent results, with good healing of the dislocation and in normal alignment.

Conclusion: Fracture dislocation of the mid-coccygeal and sacro-coccygeal joints in the young adults and pediatric population could be treated conservatively, as the potential of healing and remodelling is great. Closed reduction should not be attempted.

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A Case of Co-Infection with Orientia Tsutsugamushi, Acute Hepatitis B, and Mycoplasma Pneumoniae in a Child with Fever and Systemic Rash

Eun Lee and Nae-Yun Heo

Background: Systemic rash combined with prolonged fever requires a differential diagnosis of possible diseases including infectious diseases. Although scrub typhus can cause co-infection with leptospirosis, co-infection with Orientia tsutsugamushi and Mycoplasma pneumoniae is thought to be rare, with only one case reported in a young adult. However, to date, there have been no reports of simultaneous co-infection with three pathogens, including Orientia tsutsugamushi, hepatitis B, and Mycoplasma pneumoniae.

Case: We report a child simultaneously co-infected with Orientia tsutsugamushi, acute hepatitis B, and Mycoplasma pneumoniae, which made her prolonged fever and systemic rash with mild itching. On day 2 of hospitalization, an eschar on the left inguinal area became prominent and antibodies against Orientia tsutsugamushi showed levels = 1:1520. Owing to an elevation in the levels of liver enzymes at admission, IgM for hepatitis B surface antigen was positive and IgG for hepatitis B surface antigen was negative. Following two days of tetracycline administration, the fever subsided and the systemic area skin rashes gradually improved.

Conclusion: Proper evaluation based on the presenting symptoms during the illness is important to ensure that the differential diagnosis is not overlooked.

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Proliferative Glomerulonephritis with Monoclonal IgG Deposits Associated with Membrano-Proliferative Features: Case Report

Ma?gorzata W?growska- Danilewicz and Marian Danilewicz

Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a recently described entity. It is featured by glomerular nonorganized monoclonal immunoglobulin G deposits. Monoclonal IgG deposits are associated with glomerular proliferative lesions, mimicking different types of immune-complex glomerulonephritis. We report two classic cases of PGNMID which fulfilled all criteria of this disease. We conclude that recognition of proliferative glomerulonephritis with monoclonal IgG deposits requires routine immunostaining for light chain and IgG isotype.

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Anti-Cancer Drug Nab-Paclitaxel May Exacerbate Corneal Epithelial Disorder

Yuka Hosotani, Hiroto Ishikawa, Kumiko Miyanaga and Fumi Gomi

Purpose: To report 2 cases of corneal disorder after cancer treatment with nab paclitaxel.

Methods: Retrospective review of 2 cases.

Case presentation: Case 1 was a 55-year-old female who presented with blurred vision. She underwent cancer treatment in the form of combination therapy with nab-paclitaxel, trastuzumab and pertuzumab for breast cancer 2 weeks before. An ophthalmoscopic examination showed an atypical corneal epithelium invasion in both eyes. The corneal disorder was resolved following discontinuation of nab-paclitaxel. Case 2 had a pancreatic cancer and she presented with an atypical corneal epithelium invasion in both eyes after treatment with S-1 and gemcitabine hydrochloride. The corneal disorder might be caused by S-1, so S-1 treatment was replaced by nab-paclitaxel. The corneal disorder improved temporarily, but it worsened 3 months after the administration of nab-paclitaxel. Finally, the corneal disorder was resolved following the discontinuation of nab-paclitaxel as well as in case 1.

Conclusion: Our 2 cases suggest that cancer treatment including nab-paclitaxel may cause severe corneal disorders. Clinicians should consider the possibility of the corneal disorder being caused by the cancer therapy.

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Laparoscopy Assisted Transduodenal Resection of a Periampullary Tumor at the Inferior Duodenal Angulus

Masahiro Sakon, Yasushi Sekino, Hitoshi Seki, Ayako Seki, Yasuhiro Munakata and Osamu Hasebe

Background: Duodenal adenoma is a rare lesion that requires resection because of its malignant potential. Minimally invasive surgery is desirable for such lesions.

Case summary: A 52-year-old man underwent esophagogastroduodenoscopy, which revealed a 30-mm 0-IIalike lesion at the inferior duodenal angulus. Examination of the biopsy specimen revealed a Group IV adenoma. The tumor was treated by laparoscopic transduodenal ampullectomy, which was performed under general anesthesia and via 5 trocars. After laparoscopic mobilization of the duodenum and pancreas head, the duodenum was externalized through a mini-laparotomy. A longitudinal incision was placed in the duodenum, and the tumor was excised extracorporeally under direct vision; submucosal dissection was achieved with an electrocautery device. The mucosal defect and duodenal wall were closed with interrupted sutures. The tumor measured 26 mm × 25 mm and was diagnosed histologically as a tubular adenoma with low grade atypia. The surgical margin was adenomanegative. The postoperative course was uneventful.

Conclusion: From our experience in this case, we deem laparoscopy-assisted transduodenal ampullectomy to be a feasible and safe procedure for a periampullary duodenal tumor.

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