Netherton syndrome-A therapeutic challenge in childhood.

Key Clinical Message: High-dose intravenous immunoglobulin exhibits great potential in the treatment of Netherton syndrome.

Abstract: Netherton syndrome (NS) is a rare autosomal recessive genodermatosis (OMIM #256500) characterized by superficial scaling, atopic manifestations, and multisystemic complications. It is caused by loss-of-function mutations in the SPINK5 gene, which encode a key kallikrein protease inhibitor.

Elevated IgE Levels-An Allergy or an Underlying Inborn Error of Immunity in Children with Recurrent Infections?

Elevated immunoglobulin E (IgE) is a hallmark of allergic diseases. However, high IgE levels also occur in a number of other infectious and noninfectious diseases. In most cases, elevated IgE levels indicate allergy, eczema, or chronic skin infection.

Preventable Hazards from in Vitro Fertilization - A Case Series of CF Patients from Bulgaria.

Pre-implantation genetic diagnosis (PGD) is not often performed when donor gametes are used, due to its high cost. This is with the presumption that the donors are healthy. We report on five cases of babies with confirmed cystic fibrosis (CF), being the result from in vitro fertilization (IVF) with donor (4 cases) or own gametes (one case).

[BENEFITS AND RISKS AT IMPLEMENTATION OF PROPHILACTIC VACCINES FOR CERVICAL CANCER].

The aim of this review is to present the benefits and risks of the implementation of prophylactic vaccines for cervical cancer. The classical understanding of human papilloma virus (HPV) infection and its role for the cervical oncogenesis, as well as, the place of prophylactic HPV vaccines are discussed. Results concerning the effectiveness of vaccines 10 years after their introduction and data about their safety are presented.

Ipsilateral in-breast tumor recurrence after breast conserving therapy: true recurrence versus new primary tumor.

Purpose: To classify ipsilateral in-breast cancer recurrences (IBCR) in patients treated with conservative surgery and radiation therapy, either as new primary tumor (NP) or true recurrence (TR) and to assess the prognostic and therapeutic importance of this classification.

Methods: The records of 107 patients treated for local tu- mor recurrence after breast-conserving therapy (BCT) at the National Cancer Center, Sofia, between March 1999 and May 2011 were retrospectively analysed. The patients'primary tumors were up to 2 cm in size.

[Breast cancer developing during pregnancy--clinical cases and review of the literature].

The goal of the present paper is to discuss diagnosis, treatment approaches and histopathologic characteristics of breast cancer developing during pregnancy, based on our results and published literature data. We retrospectively evaluated clinical and pathologic features of tumors, treatment methods, decisions related to pregnancy and final outcome by eight pregnant patients with breast cancer. The patients' age varied from 26 to 36 years.

Children, care, career - a cross-sectional study on the risk of burnout among German hospital physicians at different career stages.

Background: With the increasing number of female medical students physicians' need for work-life balanced hospital jobs rises at all career stages. The Working Time Act (Arbeitszeitgesetz, ArbZG), an implementation of the European Working Time Directive into German law in 2004, should have improved the general conditions for creating flexible work. Nevertheless, the vast majority of female physicians still report an incompatibility of work and family.

[New colposcopic terminology: Rio de Janeiro--2011].

The purpose of this work is to review the new colposcopic classification of the International Federation for Cervical Pathology and Colposcopy (IFCPC) from 2011 and the possibilities for its application in diagnostic and treatment processes and research. It fulfills the necessity for a modern and widely applicable nomenclature of the findings and it is based on the latest knowledge in this area. Colposcopic terminology of the vagina, as part of the pathology of the lower genital tract, is included as well, while the vulva and perineum terminology is not yet finally adopted.

[Rare clinical case of inflammatory macromastia in childhood].

AThe present and rare case is of a patient who developed macromastia in childhood. Usually, gigantomastia can occur as a complication due to pregnancy or it may occur in puberty, which is known as virginal breast hypertrophy, the second of which is more frequent. In our case, during the first decade of life, her breasts rapidly increased and reached gigantic sizes over a short period of time.

[Pregnancy and malignant diseases. Part V. Some rare gynecological tumors during pregnancy].

Neoplasia of the vagina, endometrium and fallopian tube have low frequency in pregnant women and in the literature have been reported occasionally. Symptoms of cancer during pregnancy is often ignored by patients and physicians, leading to delayed diagnosis. This publication examines some characteristics of clinical course and management of cases of pregnancy combined with such tumors.

[Dysgerminoma of the ovary. Presentation of case report].

Germ-cell tumors account for about 20% of all ovarian tumors and the dysgerminoma is the most common in this group. The treatment is surgical, followed by chemotherapy and radiotherapy in greater stage than IA. The aim of this article is to present a case report of 18-years-old patient with a left ovary dysgerminoma who had undergone a surgery--left-side ovariectomy.

Cervical cancer screening practices in central and eastern Europe in 2012.

The burden of cervical cancer in central and eastern Europe is generally higher compared to western or northern Europe due to a history of mostly opportunistic cervical cancer screening practices and due to the strong influence of political and economic changes in post-communist transition. This article describes the current cervical cancer screening practices, organizational plans for the future, and main obstacles that need to be overcome in 16 countries in central and eastern Europe: Albania, Bosnia and Herzegovina, Bulgaria, Croatia, the Czech Republic, Estonia, Hungary, Latvia, Lithuania, Montenegro, Poland, Romania, Serbia, Slovakia, Slovenia and The former Yugoslav Republic of Macedonia. Unfortunately, only a few countries have managed to establish an organized and well-functioning cervical cancer screening program in recent years, whereas most countries in the region are still struggling with implementation-related issues of organized cervical cancer screening.

Less work: more burnout? A comparison of working conditions and the risk of burnout by German physicians before and after the implementation of the EU Working Time Directive.

Objectives: The present study is a 10-year comparison (1997 vs. 2007) of occupational and health aspects before and after the implementation of the European Working Time Directive on German hospital physicians. A major focus is whether the changes in working conditions are accompanied by a lower risk for burnout.

[Application of the RNA-based HPV test in the diagnostics of cervical epithelial changes].

Unlabelled: Our purpose was to investigate the diagnostic value of the RNA-based HPV test (NucliSENS EasyQtm HPV) which utilizes NASBA technology for determination of the expression of E6/E7 of five high-risk HPV types (16, 18, 31, 33 u 45).

Methods: Two hundred women aged 16 to 61 (mean 34.44) were prospectively studied.

[Pregnancy and malignant diseases. Part III--. Vulvar cancer].

Vulvar cancer is among the rarest gynecological tumors. It is extremely rare in pregnant women. In the scientific literature, data has been recorded for no more than 50 cases of vulvar cancer during pregnancy.

[Pregnancy and malignant diseases. Part III--Ovarian cancer].

The frequency of ovarian tumors during pregnancy is from 1:81 to 1:328 and most of them are functional cysts. Ovarian cancer varies from 1:10 000 to 1:25 000 pregnancies. It presents 2-5% of all ovarian tumors in pregnant women.

[Pregnancy and malignant diseases. Part II--cervical cancer].

The malignancies of the uterine cervix are the most common neoplasia during pregnancy. Cancer in situ varies from 0, 11 to 0, 55% and invasive cervical cancer is found in around 0, 03% of pregnant women. The summarized data by different authors shows that the cervical cancer can be found in the wide range between 1:250 to 1:13000 pregnancies (mean 1:2000 to 1:3000).

Is cervical cancer screening necessary or the "Paradox" example of Bulgaria.

Purpose: The present study aimed at analyzing the results of cervical screening carried out in Bulgaria, its effect on basic epidemiological indices, age-related incidence of cervical cancer and their dynamics. It also highlighted the way in which the suspension of organized screening has influenced the epidemiological data for cervical cancer.

Methods: Analyses were carried out, based on data from the National Cancer Register, the Central Institute of Statistics and the Oncological Dispensaries.

[Prophylaxis and screening of precancer and cancer of the uterine cervix in the XXI century].

At the beginning of the XXI century a tendency of promotion of new methods aimed at modernization and optimization of cervical cancer screening is outlined. In the present review the diagnostic confidence of the cytological method as well as the new technologies (thin prep technique, automated screening, HPV typization, genetic markers and other modern digital visualization methods) is discussed. Each of these methods has its own priorities and disadvantages and the aim of future investigations should be to choose the most appropriate screening scheme, in order to improve the control of this disease.

[A case of 11 operation because of genitourilal abnormalites and cervical carcinoma].

A case of young woman with 11 operation because of genitourinal abnormalities and cancer of the uterine cervix. The patient was with bladder exstrophy and 2nd stage cervical cancer, treated operatively and with chemotherapy. Because local recurrence she was operated 3 times.

[Therapeutic results in patients with endometrial cancer].

The aim of the present study is to analyze the patients with endometrial cancer, treated at Gynecology Clinic of the National Oncology Hospital, Sofia according to the stage of the disease, histological type, differentiation, the peculiarities of the spreading and metastases, the therapeutic methods applied and the obtained results. For the period 2002-2004, 152 women with endometrial cancer were treated at the clinic. Predominate the patients with first stage cancer 117 (77%) and with endometroid adenocarcinoma/adenoacantoma 116 (76.

[Clinical study on the effect of vaginal administration of micronized progesterone at dysfunctional uterine bleeding in premenopause].

The aim of the present study was to establish the effectiveness of vaginal administration of micronized progesterone at 37 premenopausal women aged between 40-49 years (mean 44.5) with dysfunctional uterine bleeding. All women were subject of classical dilatation and curettage and after that micronized progesterone (Utrogestan) was applied vaginally 3 tablets 2 times per day, starting at 14 day of the menstrual cycle for 12 days.

Five-year overall survival and prognostic factors in patients with cervical cancer in Bulgaria.

Purpose: To perform a population-based analysis on the 5-year survival rate and to analyse the significance of various prognostic factors for survival in patients with cervical cancer in Bulgaria for the period 1993-2002.

Patients And Methods: A total of 9,457 women were analyzed using the data of the National Cancer Registry. Their mean age was 51.