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Cappellini M.D.,University of Milan | Bejaoui M.,Center National Of Greffe Of Moelle Osseuse | Agaoglu L.,Istanbul University | Canatan D.,Suleyman Demirel University of Turkey | And 14 more authors.
Blood | Year: 2011

Patients with β-thalassemia require lifelong iron chelation therapy from early childhood to prevent complications associated with transfusional iron overload. To evaluate long-term efficacy and safety of once-daily oral iron chelation with deferasirox, patients aged ≥ 2 years who completed a 1-year, phase 3, randomized trial entered a 4-year extension study, either continuing on deferasirox (deferasirox cohort) or switching from deferoxamine to deferasirox (crossover cohort). Of 555 patients who received ≥ 1 deferasirox dose, 66.8% completed the study; 43 patients (7.7%) discontinued because of adverse events. In patients with ≥ 4 years' deferasirox exposure who had liver biopsy, mean liver iron concentration significantly decreased by 7.8 ± 11.2 mg Fe/g dry weight (dw; n = 103; P < .001) and 3.1 ± 7.9 mg Fe/g dw (n = 68; P < .001) in the deferasirox and crossover cohorts, respectively. Median serum ferritin significantly decreased by 706 ng/mL (n = 196; P < .001) and 371 ng/mL (n = 147; P < .001), respectively, after ≥ 4 years'exposure. Investigator-assessed, drug-related adverse events, including increased blood creatinine (11.2%), abdominal pain (9.0%), and nausea (7.4%), were generally mild to moderate, transient, and reduced in frequency over time. No adverse effect was observed on pediatric growth or adolescent sexual development. This first prospective study of long-term deferasirox use in pediatric and adult patients with β- thalassemia suggests treatment for ≤ 5 years is generally well tolerated and effectively reduces iron burden. This trial was registered at www.clinicaltrials-.gov as #NCT00171210. © 2011 by The American Society of Hematology.


PubMed | Athens General Hospital G Gennimatas, University of Thessaly, Athens Naval & VA Hospital, University of Ioannina and 5 more.
Type: Review | Journal: Clinical endocrinology | Year: 2016

Growth hormone (GH) and insulin-like growth factor I (IGF-I) are the principal biomarkers used to assess disease activity in acromegaly, and any discrepancy between them renders interpretation of results inconclusive. Purpose of this study was to assess the frequency of this discrepancy and identify parameters that might affect its occurrence.A systematic review of MEDLINE and Scopus was performed (1987-2013) followed by a meta-analysis to address the frequency of discrepant results between GH and IGF-I levels. Meta-regression and subgroup analyses were performed assessing the effects of the year of publication, the different types of GH testing and GH assays used, as well as the impact of treatment with somatostatin analogues (SSAs) on the occurrence of this discrepancy.The analysis retrieved 39 eligible studies totalling 7071 patients. The pooled discordance rate between GH and IGF-I was 257% (95% CI: 223-294), and the predominant format was that of elevated IGF-I with normal GH levels (153%, 95% CI: 125-187). No significant correlation between the discordance rate and the year of publication was shown; whereas, the use of ultrasensitive GH assays resulted in higher discordance rates (307%, 95% CI: 259-359 vs 198%, 95% CI: 141-272, P = 004) as did treatment with SSAs (325%, 95% CI: 278-374) vs (216%, 95% CI: 178-256, P = 0001).Discrepancy between GH and IGF-I results is encountered in a quarter of treated patients with acromegaly, especially when using ultrasensitive GH assays or in patients receiving SSAs, a fact that the clinician should take into consideration when making clinical decisions.


Venizelos I.,Hippokration General Hospital of Thessaloniki | Papathomas T.G.,Hippokration General Hospital of Thessaloniki | Papathanasiou M.,Aristotle University of Thessaloniki | Cheva A.,Hippokration General Hospital of Thessaloniki | And 2 more authors.
Survey of Ophthalmology | Year: 2010

Castleman disease is a quite uncommon lymphoproliferative disorder usually occurring in the lymph nodes. Rarely, Castleman disease develops in an extranodal anatomic location. We report on the first biopsy-proven case of multicentric plasma cell type of Castleman disease involving the orbital areas in a human herpes virus 8 (HHV-8)-unassociated/ human immunodeficiency virus (HIV)-seronegative 70-year-old man suffering from Parkinson disease. The diagnosis was established on the basis of morphologic, immunophenotypic, and molecular findings of a lymph node and orbital soft tissue biopsy. We additionally provide a review of all previously published cases of Castleman disease with an orbital involvement, discussing the distinctive characteristics and potential associations with regard to their counterparts at other sites. Although Castleman disease involving the orbit is an exceptionally rare occurrence that may present initially with ocular signs and symptoms, this should be included in the complete differential diagnosis of orbital mass lesion. © 2010 Elsevier Inc.


PubMed | Hippokration General Hospital of Thessaloniki, Aristotle University of Thessaloniki and Chelsea and Westminster Hospital NHS
Type: Journal Article | Journal: Quantitative imaging in medicine and surgery | Year: 2016

Posterior reversible encephalopathy syndrome (PRES) is a rare clinical-radiological entity characterised by seizures, severe headache, mental status instability and visual disturbances. Hypertension is typically present. We report a case of a 13-year old boy with Burkitt lymphoma/leukaemia, who presented with posterior leukoencephalopathy 24 hours after intrathecal methotrexate (MTX) infusion. The child presented with headache, seizures, elevated blood pressure and gradual deterioration of his neurological status. Midazolam, dexamethazone and furosemide were initiated leading to reduction of cerebral oedema and clinical improvement. A thorough literature review is discussed in this report. Pathophysiology of leukoencephalopathy remains unclear. It develops within 5-14 days after intrathecal MTX and resolves within a week usually without permanent neurological sequelae. Broad use of MRI has led to an increasing number of identified cases of PRES. Treatment approach is mainly to manage the underlying cause of PRES. Prognosis is generally benign; however delayed diagnosis and improper management may result in permanent brain insult.


PubMed | Hippokration General Hospital of Thessaloniki and Aristotle University of Thessaloniki
Type: Journal Article | Journal: International journal of clinical pediatric dentistry | Year: 2016

Ewings sarcoma (ES) is an uncommon aggressive bone malignancy that mainly affects children and adolescents. Mandible involvement is quite rare and usually represents metastasis from another skeletal site. Combined therapy including wide surgical resection and preoperative and postoperative chemotherapy has been demonstrated as the mainstay of therapeutic approach. As improved therapeutic modalities have significantly increased survival over the last decades, functional and esthetic reconstruction of the postmandibulectomy defect is usually necessary for patients total rehabilitation. We report a case of ES of the mandible with special consideration to the postresection spontaneous structural and functional regeneration of the mandible.Chatzistefanou I, Kabesi S, Paraskevopoulos K, Koliouskas D, Antoniades K. Ewings Sarcoma of Mandible: An Impressive Case of Spontaneous Mandible Regeneration. Int J Clin Pediatr Dent 2016;9(3):273-277.


Polyzos S.A.,Harvard University | Makras P.,VA General Hospital | Efstathiadou Z.,Hippokration General Hospital of Thessaloniki | Anastasilakis A.D.,424 General Military Hospital
Expert Opinion on Investigational Drugs | Year: 2015

Introduction: Intermittent parathyroid hormone (PTH) administration, acting through multiple signaling pathways, exerts an osteoanabolic effect on the skeleton that surpasses the effect of other antiosteoporotic agents. However, its efficacy is limited by the coupling effect and relatively common adverse events. Thus, the development of more sophisticated PTH receptor analogs seems imperative. Areas covered: In this review, the authors summarize the role of PTH signaling pathway in bone remodeling. The authors also summarize investigational analogs targeting this pathway, which may be potential treatments for osteoporosis. Expert opinion: β-arrestins are multifunctional cytoplasmic molecules that are decisive for regulating intracellular PTH signaling. Recently, in preclinical studies, arrestin analogs have achieved the anabolic bone effect of PTH without an accompanying increase in bone resorption. However, it is not yet known whether these analogs have adverse effects and there are no clinical data for their efficacy to date. On the other hand, several molecules derived either from PTH and PTH-related protein (PTHrP) molecules have been developed. Alternative routes of PTH 1-34 delivery (oral, transdermal), the PTH analog ostabolin and the N-terminal PTHrP analogs PTHrP 1-36 and abaloparatide, have recently been or are currently being tested in clinical trials and are more likely to become available for use in the near future. © 2015 Informa UK, Ltd.


Mantadakis E.,Democritus University of Thrace | Farmaki E.,Hippokration General Hospital of Thessaloniki | Buchanan G.R.,University of Texas Southwestern Medical Center
Journal of Pediatrics | Year: 2010

Objective: To determine the incidence of immune thrombocytopenic purpura (ITP) after measles-mumps-rubella (MMR) immunization compared with natural measles and rubella, its clinical course and outcome, and the risk of recurrence after repeat MMR vaccination. Study design: We performed a systematic review of the Ovid MEDLINE (1950 to present) bibliographic database. We selected studies that reported cases of thrombocytopenia in a known number of children who were immunized with MMR vaccine before development of ITP. We also extracted data from the same and other studies regarding bleeding manifestations and the resolution of MMR-associated thrombocytopenia or thrombocytopenic purpura within 6 months. Finally, we studied the risk of ITP recurrence after MMR immunization or reimmunization. Results: On the basis of 12 studies, the incidence of MMR-associated ITP ranged from 0.087 to 4 (median 2.6) cases per 100 000 vaccine doses. Severe bleeding manifestations were rare, and MMR-associated thrombocytopenia resolved within 6 months from diagnosis in 93% of the children. MMR vaccination of unimmunized patients with ITP and revaccination of patients with prior ITP did not lead to recurrence of thrombocytopenia. Conclusions: MMR-associated ITP is rare, self-limited, and non-life threatening, and susceptible children with ITP should be immunized with MMR at the recommended ages. © 2010 Mosby, Inc. All rights reserved.


Efstathiadou Z.A.,Hippokration General Hospital of Thessaloniki | Sapranidis M.,Hippokration General Hospital of Thessaloniki | Anagnostis P.,Hippokration General Hospital of Thessaloniki | Kita M.D.,Hippokration General Hospital of Thessaloniki
Head and Neck | Year: 2014

Background Pituitary tumors, paragangliomas, and Cowden syndrome do not usually occur together. Methods The synchronous presentation of papillary thyroid carcinoma and neck paraganglioma was revealed in a 43-year-old woman who had been diagnosed with a microprolactinoma one decade before and now presented with a constellation of characteristics that are components of Cowden syndrome, specifically macrocephaly, multiple skin papules, fibrocystic mammary disease, and uterine leiomyofibroma. Results Germline mutation analysis of phosphatase and tensin homolog (PTEN), succinate dehydrogenase subunit B (SDHB), succinate dehydrogenase subunit C (SDHC), and succinate dehydrogenase subunit D (SDHD) was performed with revelation of 3 polymorphic sites in introns 1, 4, and 8 of the PTEN gene and 1 polymorphic site in exon 1 of the SDHB gene, but absence of known pathogenic mutations. Conclusion The coexistence of Cowden-like syndrome, neck paraganglioma, and pituitary adenoma is described for the first time, and could represent a novel genetic syndrome with an as yet unidentified common genetic basis. © 2013 Wiley Periodicals, Inc.


Smaropoulos E.,Hippokration General Hospital of Thessaloniki | Romeos S.,Hippokration General Hospital of Thessaloniki | Dimitriadou C.,Hippokration General Hospital of Thessaloniki
Wounds UK | Year: 2011

Aims: To compare honey-based ointments and dressings for the treatment of paediatric partial-thickness burns and skin lesions with results from cases treated with conventional methods. Methods: Eight children (eight months-13 years) were included in the study when admitted to the emergency room (ER). Five had partial-thickness burns (2-35% total body surface area [TBSA]). The three others had necrotic ulcers, circular skin lesions and deep cervical trauma. After stabilising the patients in the ER, honey-based products were applied. Parents were given instructions on how to apply the honey ointment and dressings at home. Patients were followed up weekly on an out-patient basis. Similar cases treated with povidone iodine were collated from hospital archives for comparison. Results: All honey cases had complete epithelialisation by the end of the second week, without any adverse effects. The povidone iodine group needed on average three weeks for epithelialisation and had prolonged hospitalisation. The home care by the parents provided stress-free healing for both children and parents. Conclusions: The honey-based therapy indicates a fast (19 days vs 25-31 days), cost-effective and patient-friendlier treatment method compared to povidone iodine. Conflict-of-interest: None.


Patsiaoura K.,Hippokration General Hospital of Thessaloniki | Anagnostou E.,Hippokration General Hospital of Thessaloniki | Benis N.,Hippokration General Hospital of Thessaloniki
Auris Nasus Larynx | Year: 2010

Intramuscular myxoma is an uncommon benign soft tissue neoplasm and a distinct histopathological entity within the heterogeneous group of myxomas. The most common localizations are the gluteal muscles and the muscles of the thigh. We report on a case of a myxoma within the mimetic muscles of the nasal and mouth region in a 52-year-old man. The tumor was surgically removed and the patient is free of recurrence or complications 8 months after treatment. The clinical and pathologic features of this lesion are presented. The head and neck region is an uncommon site of presentation for intramuscular myxoma with only 13 documented cases in the available literature. To the very best of our knowledge, there is no report of this neoplasm located within the nasal and oral mimetic muscles. © 2009 Elsevier Ireland Ltd. All rights reserved.

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