No abstract

Juvenile ovarian granulosa cell tumor is a sex chord stromal tumor derived from granulosa cells. It rarely occurs in children. Although it is usually classified as a benign tumor in children due to its good prognosis after surgical intervention, it is a malignant tumor and can be deadly, especially if recurrence occurs. We report two rare cases. The first one is a five-year-old girl with a malignant juvenile ovarian granulosa cell tumor stage 3 FIGO, presenting with abdominal pain in the inferior part of the abdomen and very early pubertal development. On physical examination, she was at stage two puberty. The second case is a 12 years old girl presenting with two periods monthly. An 8.3/5.4cm mass was found at the pelvic region on ultrasound examination. Exploratory laparoscopy with histological examination led to the diagnosis of stage 2A FIGO, combined juvenile and adult ovarian granulosa cell tumor. Conclusion: Precocious puberty accompanying abdominal pain or more than a period in a month is a pointer to juvenile and adult ovarian granulosa cell tumor requiring immediate investigations and patient management. Early diagnosis with the assistance of inhibin test and a FISH test of p53(17p13) aids better management of patients with Juvenile ovarian granulosa cell tumor, preventing tumor recurrence for a favorable outcome. Juvenile and adult ovarian granulosa cell tumors in pediatric female patients can present in different forms but gearing towards menstruation abnormalities.

Introduction. Chronic rhinosinusitis, a very common inflammatory condition, is a main public health issue affecting the quality of life. Furthermore, some patients do not respond to either medical or surgical intervention, which could be explained by the presence of the bacterial biofilm in the rhinosinusal zone. Objectives. The aim of this study is to evaluate the influence of endoscopic sinus surgery on olfactory impairment caused by chronic sinusitis with and without nasal polyps (CRSNP and CRS), by testing the olfactory function and potential dysfunction before and after endoscopic sinus surgery. Another objective is to find a connection between the percentage of coverage with bacterial biofilm of the nasal mucosa from patients with CRSNP and CRS and to evidence the fountain of infection role of the bacterial biofilm, while demonstrating that antibiotic therapy is not efficient once the bacterial biofilm is formed in the nasal sinuses. Materials and methods. We investigated 123 patients with CRSNP and CRS, which underwent functional endoscopic sinus surgery (FESS). The olfactory function was tested pre and post FESS. We also analyzed and compared the scores of endoscopic images of Lund-Kennedy and CT staging scale of LundMackey for the two study groups. The degree of olfactory rehabilitation in patients with CRSNP and CRS was evaluated performing smell diskettes test. The presence of bacterial biofilm on the surface of the nasal mucosa extracted during FESS from patients with CRSNP and CRS was examined with the electronic microscope and the percentage of coverage with bacterial biofilm was measured with Carnoy software. Results. Bacterial biofilm was present in a higher percentage in patients with CRS vs. CRSNP. By comparing the level of olfactory function, significant improvement was found after FESS intervention in both study groups. Postoperatively, Lund-Kennedy scores decrease significantly for the whole group (Z = -9.66 at p < 0.001, d Cohen = 4.40), indicating the major role of surgery in the treatment of CRS. The decrease in Lund-Kennedy score values is also significant for each group. In the case of subjects diagnosed with CRS, the mean values decrease from 6.57 (preoperative) to 0.90 (postoperative), respectively Z = -6.779 to p < 0.001. In the case of subjects diagnosed with CRSNP, the mean values decrease from 9.03 (preoperative) to 1.44 (postoperative), Z = -6.927 at p < 0.001. Out of the total number of patients included in the study, 59 patients tested positive with the Prick test for dust and mites and more than half of the patients with positive allergy test were from the lot diagnosed with CRSNP. Conclusions. FESS plays an important role in the improvement of olfactory function in patients with CRS. Bacterial biofilm was present in both study groups but in higher percentage in the CRS group and was found in lower percentage in the group with positive allergy tests. The CRSNP group presented a higher positive result regarding the allergy Prick test and a lower percentage of coverage with bacterial biofilm of the nasal mucosa. In conclusion, chronic rhinosinusitis with or without nasal polyps that is refractary to antibiotic therapy should be directed to the ENT department in order to receive surgical therapy in order to improve olfactory function.

Objective: Allogeneic Hematopoietic Stem Cell Transplantation (HSCT) is the treatment of choice in patients with Fanconi Anemia (FA). The aim of our study is to evaluate the impact and benefits of allogenic matched donor HSCT in a case of a 12 year-old girl with FA, who displayed good clinical evolution following 2 months post-transplantation. Patients and Methods: In the pre-transplant phase, reference blood samples from the donor and recipient were collected on EDTA. The DNA from blood samples was extracted using an automated Maxwell® 48 RSC instrument (Promega, USA) with the Maxwell® RSC Whole blood DNA kit (Promega, USA). For DNA quantification, the PowerQuant System kit (Promega, USA) was used with the ABI 7500 Real-time PCR system (Applied Biosystems, USA). The amplification of the short tandem repeat markers was performed using the 24plex Investigator QS kit (Qiagen, Germany) on a ProFlex PCR System. Furthermore, the PCR products were separated and detected on an ABI 3500 Genetic Analyzer (Applied Biosytems, USA). Results: After 30 days of the transplantation, a Complete Chimerism (CC) was achieved with a full replacement by donor derived hematopoietic cells. After 60 days of the transplantation, the CC status was maintained with improvement of hematological findings. Conclusion: In FA, chimerism monitoring after HSCT provides useful informations of engraftment or possibility of post-transplantation complications such as graft versus host disease.