Management of a rare case of arrhythmogenic right ventricular dysplasia in pregnancy: a case report

<p>Abstract</p> <p>Introduction</p> <p>Arrhythmogenic right ventricular dysplasia is a heritable disease of the heart muscle characterized by fibrofatty degeneration of cardiomyocytes. Patients present with ventricular arrhythmias or congestive heart failure, and sometimes sudden cardiac death occurs. Prenatal diagnosis has become possible with the detection of mutations, but, to the best of our knowledge, no case of prenatal diagnosis has been reported previously. There is little information about the management of arrhythmogenic right ventricular dysplasia in pregnancy, and the preferred mode of delivery is not certain; therefore, we present the case of a patient with arrhythmogenic right ventricular dysplasia and discuss the prenatal diagnosis, patient management and prognosis in pregnancy.</p> <p>Case presentation</p> <p>A 26-year-old Caucasian woman who presented to our hospital with heart palpitations was diagnosed with arrhythmogenic right ventricular dysplasia, and, after three years of follow up with anti-arrhythmic drugs, she wanted to conceive. During pregnancy, she ceased taking her medication. She tolerated pregnancy very well but her cardiac symptoms recurred after her 30th week of pregnancy. She delivered a baby via cesarean section under general anesthesia in her 38th week of pregnancy. She was discharged without any medications and continued lactation for six months.</p> <p>Conclusion</p> <p>Patients with mild to moderate arrhythmogenic right ventricular dysplasia tolerate pregnancy and breastfeeding very well, but patients with end-stage arrhythmogenic right ventricular dysplasia should be discouraged from conception.</p

Results of midtrimester emergency cercllage

İstanbul Bilim Üniversitesi, Tıp Fakültesi.OBJECTIVE: To evaluate the outcomes of midtrimester emergency cerclage and to find out,the contributing factors. STUDY DESIGN: Twenty-five patients presenting with cervical dilation and effacement with the membranes at the level of the external os or prolapsed into the vagina were included in the study. At the time of the cervical cerclage placement, gestational age ranged from 14-26 weeks. A good outcome was defined as the take-home baby rate, but also the interval between the time of the cervical cerclage placement and delivery and the gestational age at delivery were discussed. RESULTS: The mean gestational age at the time of the cervical cerclage placement was 21.2 +/- 2.73 weeks, the mean gestational age at delivery was 29.4 +/- 5.37 weeks, and the mean birth weight was 1,511 g. Eighteen patients had McDonald type cerclage, and 7 patients had both McDonald and Saling type cerclages. The overall take-home baby rate was 64%. Patients presenting with membranes at the level of external os had a take-home baby rate of 68.8%, whereas patients presenting with membranes prolapsed into the vagina had a take-home baby rate of 31.3%. CONCLUSION: Cervical dilation at midtrimester has a poor outcome which can be improved with emergency cerclage. Patients presenting with membranes prolapsed into the vagina have a decreased success rate. Total closure of the cervix may improve results. (J Reprod Med 2013;58:143-148

Relationship of endometrial thickness with metabolic parameters and sex-steroids in postmenopausal women

İstanbul Bilim Üniversitesi, Tıp Fakültesi.Amaç: Bu çalışma postmenopozal kadınlarda seks steroidleri ve metabolik parametreler ile endometrial kalınlık arasındaki bağlantıyı araştırmak amacıyla planlandı Gereç ve Yöntem: 2010-2012 yılları arasında prospektif bir çalışmaya dahil olan hastaların verileri değerlendirildi. Postmenopozal hastaların endometrial kalınlık ölçümleri, hormonal ve metabolik parametreleri ile karşılaştırıldı. Bulgular: Çalışmaya 54 hasta dahil edildi. Endometrial kalınlığı 5mm'nin altında olan olgularda ortalama FSH değeri 83.8±27.7 mIU/ml, endometrial kalınlığı 5mm ve üzerinde olan olgularda ise ortalama FSH değeri 52.4±16.7mIU/ml olarak bulunmuştur (p=0.043). Endometrial kalınlık ile seks steroidleri, vücut kitle indeksi (VKİ) ve metabolik parametreler arasında bir ilişki saptanmamıştır.Purpose: The aim of this study was to understand the relationship between endometrial thickness and metabolic parameters and sex-steroids in postmenopausal women. Methods: We used data of patients attending a prospective study between 2010 and 2012. We compared endometrial thickness of postmenopausal women with hormonal and metabolic parameters. Results:We included 54 patients in this study. Mean FSH level of patients with an endometrial thickness <5mm was 83.8±27.7 mIU/ml, mean FSH level of patients with an endometrial thickness ≥ 5mm was 52.4±16.7mIU/ml (p=0.043). We did not detect any other relationship between endometrial thickness and sex-steroids, body mass index (BMI) and metabolic parameters

Polikistik over sendromlu hastalarda koroner kalp hastalıklarının belirteci olarak c-reaktif protein ve lipoprotein-a bakılması

İstanbul Bilim Üniversitesi, Tıp Fakültesi.OBJECTIVE: The aim of this study was to investigate the risk factors of coronary heart disease, CRP and Lipoprotein-a in polycystic ovary syndrome patients. MATERIAL AND METHODS: Prospectively collected data of polycystic ovary syndrome patients (n=62) and control group (n=40) were compared. RESULTS: PCOS patients had higher HOMA-IR, CRP, DHEAS, free testosterone, FAI, LH and prolactin levels when compared to the control group. Lipoprotein-a levels did not differ between the groups. The obese PCOS group had statistically significantly higher fasting blood glucose, total cholesterol, triglyceride, free testosterone, insulin, CRP and HOMA-IR and statistically significantly lower HDL and SHBG when compared to normal weight PCOS persons. Fasting blood glucose, total cholesterol, LDL, SHBG, CRP, Lipoprotein-a, FSH, LH, TSH, DHEAS and prolactin levels did not differ between the normal weight and obese control groups. CONCLUSION: CRP levels increase in polycystic ovary syndrome patients and can be used as a marker of coronary heart disease. Future studies can be directed at treatments to decrease CRP levels, including antiinflammatory treatments.Amaç: Bu çalışmanın amacı polikistik over sendromlu hastalarda koroner kalp hastalıkları risk faktörlerini, CRP ve Lipoprotein-a araştırmaktı. Gereç ve Yöntemler: Prospektif olarak polikistik over sendromlu (n=62) ve kontrol grubunun (n=40) kan örnekleri çalışıldı. Bulgular: Polikistik over sendromlu hastalarda HOMA-IR, CRP, DHEAS, serbest testosteron, FAI, LH and prolaktin seviyeleri yüksek, FSH ve SHBG seviyeleri ise düşük bulundu. Lipoprotein-a gruplarda farklı değildi. Obez polikistik over sendromlu hastalarda açlık kan şekeri, total kolesterol, trigliserit, serbest testosteron, insulin, CRP ve HOMAIR daha yüksek, HDL ve SHBG daha düşük bulundu. Açlık kan şekeri, total kolesterol, LDL, CRP, SHBG, Lipoprotein-a, FSH, LH, TSH, DHEAS ve prolaktin seviyeleri obez ve normal kilolu kontrol gruplarında benzerd

Achondrogenesis type Ia (Houston-Harris): 2D/3D/4D ultrasonographic prenatal diagnosis: Case report

İstanbul Bilim Üniversitesi, Tıp Fakültesi.Tip IA akondrogenezi, şiddetli mikromeli, vertebra korpuslarında ossifikasyon olmaması ve multipl kırıklar olan kaburgalarla karakterize ölümcül bir kondrodistrofidir. Yirminci gebelik haftasında kliniğimize konsülte edilen hastanın 2 boyutlu ultrasonografik incelemesi bu nadir anomaliyi ortaya koymuştur. Üç ve 4 boyutlu ultrasonografi görüntülemeleri olguyu spesifik tanısına yaklaştırdı. Hastanın aydınlatılmış onamı ile yapılan gebelik terminasyon sonucunda tanı postnatal olarak doğrulandı. Son yıllarda sonografi alanındaki gelişmeler doğrultusunda iskelet displazilerinin tanınması erken dönemlerde mümkün olmaktadır. Erken ve spesifik prenatal tanı, ileri haftalara taşınması muhtemel olumsuzlukları önlerken, genetik danışmanlığa yön vermesi nedeni ile de önemlidir. Görüntülemedeki teknik üstünlüklerinin yanı sıra 3 boyutlu ultrasonografinin anormal bulguların doktor ve aile tarafından çok daha çabuk ve açık olarak anlaşılmasını sağladığını düşünüyoruz.Type IA achondrogenesis is a lethal chondrodystrophy characterized by severe micromelia, absent mineralization of vertebral bodies, and multiple rib fractures. 2-dimensional ultrasound evaluation of the patient who was consulted with our clinic at 20 weeks of gestation revealed this extremely rare fetal anomaly. 3-and 4-dimensional ultrasound imaging approximated the case to its specific diagnosis. The final diagnosis was postnatally confirmed at pregnancy termination that was preceded with patient's informed consent. Early identification of skeletal dysplasias becomes easier according to the developments in sonography. Early and specific prenatal diagnosis avoids the possible problems that could come up against at further weeks of gestation, and guides the genetic counseling, as well. We think, 3-dimensional ultrasound, besides its technical superiority at imaging, makes it quicker and more definite to understand the abnormal findings both by the doctor and the family

Endocervical glandular involvement, multicentricity, and extent of the disease are features of high-grade cervical intraepithelial neoplasia

İstanbul Bilim Üniversitesi, Tıp Fakültesi.This study aimed to evaluate the rate of endocervical glandular involvement, positive surgical margins, multicentricity, and disease extent between low-grade and high-grade cervical intraepithelial lesions after loop electrosurgical excision procedure (LEEP). Pathology medical records of patients who underwent LEEP were reviewed retrospectively. Patients with negative LEEP results were excluded. Loop electrosurgical excision procedure reports of patients with cervical intraepithelial neoplasia (CIN) 1, 2, and 3 were compared. There was no statistically significant difference between patients with CIN 1 (n=24), CIN 2 (n=27), and CIN 3 (n=64) when age and surgical margin positivity were considered. Endocervical glandular involvement, multicentricity, and disease extent were higher in patients with CIN 3 (P=.001, P=.002, and P=.001, respectively). In conclusion, we recommend that patients with endocervical glandular involvement, lesions involving more than two-thirds of the LEEP specimen, and multicentricity be followed up more closely

Three-step approach versus see-and-treat approach in patients with cytological abnormalities

İstanbul Bilim Üniversitesi, Tıp Fakültesi.The aim of this study was to compare the results of see-and-treat procedure with the classical three-step procedure in terms of initial cytology and LEEP reports. We searched the pathology charts of patients that had LEEP were searched retrospectively and then they were divided into 2 groups according to the presence or absence of a cervical biopsy before LEEP. There were 116 patients in the study. Of the patients with ASCUS/LSIL cytology and a positive cervical biopsy 48.4% had CIN 2-3 at LEEP, in contrast only 19% of the patients without a prior cervical biopsy had CIN 2-3 at LEEP (p=0.031); there was no statistically significant difference between the 2 procedures in patients with a HSIL and ASC-H smear result (p=0.726 and p=1.0 respectively). In conclusion patients with ASC-H and HSIL cytology see-and-treat approach seems more advantageous, avoids delay in treatment, noncompliance and risk of skipping lesions at biopsy

Vajenin düşük-dereceli fibromiksoid sarkomu: Bu konumda daha önce bildirilmemiş bir tümör

İstanbul Bilim Üniversitesi, Tıp Fakültesi.Bu rapor vajen duvarından kaynaklanan ilk düşük-gradeli fibromiksoid sarkom (DGFMS) olgusunu sunmakta ve vajen duvarından kaynaklanan kitlelerin ayırıcı tanısına dikkat çekmektedir. Karın ağrısı nedeniyle acil bölümüne başvuran hastaya Douglas boşluğunu dolduran ovaryan kitle tanısı konmuştur. Hastanemizde yapılan laparoskopide kitlenin kaynağının vajen arka duvarı olduğu görüldü. Jelatinöz yapıdaki kitlenin vajinal eksizyonundan sonra patolojik tanı nadir bir tümör olan DGFMS olarak geldi. Biz de vajinal DGFMS’nin ayırıcı tanısını tartıştık.This report presents the first case of low-grade fibromyxoid sarcoma (LGFMS) arising from the vaginal wall (a rare soft-tissue sarcoma of subfascial planes) and draws attention to differential diagnosis of masses arising from the vaginal wall. A patient presenting with abdominal pain to emergency department was diagnosed to have an ovarian mass filling the Douglas space. At laparoscopy, the origin of the mass was identified as the posterior vaginal wall. After vaginal excision of the gelatinous mass, pathologic diagnosis revealed a rare tumor, LGFMS. We discussed the differential diagnosis of vaginal LGFMS

Management and Prognosis of Cesarean Scar Pregnancy; Case RSezaryen Skar Gebeliğinde Tanı, Yönetim ve Prognoz; Vaka Sunumu

Giriş: Artan sezaryen oranlarıyla birlikte sezar- yene bağlı gelişen komplikasyonlarda da artış ol- maktadır. Sezaryen komplikasyonlarından biri olan sezaryen skar gebeliği de erken tanı konmadığında ölümle sonuçlanabilmektedir. Olgu: 34 yaşında sezaryen skar gebeliği tanısı ko- yulan bir hastada, gestasyonel kesenin eksize edile- rek tedavi edildiği bir olguyu sunmak istiyoruz. Sonuç: Sezaryen skar gebeliği tanı konulmadığında yüksek mortalite oranına sahiptir. Erken tanı ile fer- tilite koruyucu girişimler yapılarak başarıyla tedavi edilebilmektedir. Anahtar Kelimeler: Sezaryen, skar, ektopik gebelikIntroduction: Increasing rate of ceseraen delivery causes increasing rate in the complications of this procedure. One of the complications of cesarean se- ction is cesarean scar pregnancy (CSP), which is a life threatining condition if not diagnosed early. Case: 34 year old multiparous women with CSP with favorable outcome by surgical excision of the gestational sac was presented. Conclusion: CSP may be a life threatining conditi- on and management by fertility preserving procedu- res is feasible with the early diagnosis

The utility of transrectal ultrasound in adolescents when transabdominal or transvaginal ultrasound is not feasible

İstanbul Bilim Üniversitesi, Tıp Fakültesi.Study Objective: To present the indications and diagnosis in adolescents undergoing transrectal ultrasound (RU). Design: Retrospective chart review. Participants: Adolescents presenting to gynecology clinic between January 1, 2005 and December 31, 2012. Main Outcome Measures: Detection of RU, transvaginal, and transabdominal (AU) ultrasound indications, and final diagnosis. Results: The main indications for RU were menstrual abnormalities, pelvic pain-dysmenorrhea, and vulvovaginitis. When compared according to final diagnosis adolescents with vulvovaginitis (13.9%) and amenorrhea (8.3%) were evaluated more with RU. Conclusion: RU is highly acceptable and it provides images superior to AU. It can be used in adolescents to visualize the pelvic organs and to exclude genital abnormalities and mass lesions