Increased Bone Marrow Iron Scores Are Strongly Correlated With Elevated Serum Ferritin Levels and Poorer Survival in Patients With Iron Overload That Underwent Allogeneic Hematopoietic Stem Cell Transplantation: A Single Center Experience.

BACKGROUND: Iron overload is one of the most significant problems as a leading cause of death in patients with leukemia and those who underwent allogeneic hematopoietic stem cell transplantation (alloHSCT). METHODS: In the current study, we retrospectively evaluated the bone marrow iron scores (BMIS) in patients who underwent alloHSCT (n = 125). The first available bone marrow biopsy specimens prior to the alloHSCT procedure or date of hospitalization (control group) were assessed in a blinded fashion using a standardized scoring system. RESULTS: A total of 125 patients were enrolled in the study. Seventy-six (60.8%) of the patients were male, and 49 (39.2%) were female. The median level of pre-transplant serum ferritin was 1023.00 ng/mL (range, 393.80-1627.50 ng/mL). The majority of the patients were diagnosed with acute leukemia (83; 66.4%) and lymphomas (20; 16.0%). The median time for neutrophil engraftment was 14.00 days (range, 13.00-16.00 days) and 11.00 days (range, 10.00-14.00 days) for platelet engraftment. The peri-transplant mortality was similar to international mortality rates (3; 2.4%). The overall survival and disease-free survival were strongly correlated with the degree of BMIS, and both were significantly poorer in patients with high bone marrow iron content (P < .001 and P = .012, respectively). CONCLUSION: The validation of BMIS for risk stratification in patients who undergo alloHSCT may predict posttransplant outcomes.

Metaplastic Conditions in The Bladder in Patient with Epidermolysis Bullosa.

Epidermolysis bullosa is a rare inherited muco-cutaneous disorder that sometimes presentes with genitourinary involvement. Herein we report the case of an 11-year-old girl with a history of junctional epidermolysis bullosa who was admitted with urological symptoms. On cystoscopy, suspected bullous bladder lesions were observed. Mesonephroid, intestinal and squamous metaplasia is reported here for the first time.

Metaplastic Conditions in The Bladder in Patient With Epidermolysis Bullosa

ABSTRACT Epidermolysis bullosa is a rare inherited muco-cutaneous disorder that sometimes presents with genitourinary involvement. Herein we report the case of an 11-year-old girl with a history of junctional epidermolysis bullosa who was admitted with urological symptoms. On cystoscopy, suspected bullous bladder lesions were observed. Mesonephroid, intestinal and squamous metaplasia is reported here for the first time.

Role of cone-beam computed tomography in the evaluation of a paradental cyst related to the fusion of a wisdom tooth with a paramolar: A rare case report.

Fusion is an abnormality of tooth development defined as the union of two developing dental germs, resulting in a single large dental structure. This irregular tooth morphology is associated with a high predisposition to dental caries and periodontal diseases. As a result of recurring inflammatory periodontal processes, disorders such as periodontal pocket, pericoronitis, and paradental cysts may develop. A rare mandibular anatomic variation is the retromolar canal, which is very significant for surgical procedures. The fusion of a paramolar and mandibular third molar associated with a paradental cyst co-occurring with the presence of a retromolar canal is rare, and the aim of the present study is to describe the evaluation of this anatomical configuration using cone-beam computed tomography.

Odontogenic tumors: a collaborative study of 218 cases diagnosed over 12 years and comprehensive review of the literature.

OBJECTIVES: The objective of this study was to analyze the frequency and distribution of odontogenic tumors (OTs) in the Cappadocia region of Turkey, and to compare the findings with those reported in the literature. STUDY DESIGN: The records of the Oral and Maxillofacial Surgery and Pathology Departments at Erciyes University, with histologic diagnosis of odontogenic tumors (based on the World Health Organization classification, 2005), over a 12-year period, were analyzed. The relative frequency of different types of tumors was also analyzed and compared with the literature. RESULTS: OTs in the present study constituted 2.74% of all the 7,942 registered biopsies. A total of 218 cases of OTs were collected and reviewed. Of these, (94.04%) were benign and (5.96%) were malignant. The mandible was the most commonly affected anatomic location, with 170 cases (77.9%). Ameloblastoma with a predilection for the posterior mandible was the most frequent odontogenic tumor (30.28%), followed by keratocystic odontogenic tumor (19.5%), odontoma (13.4%), and odontogenic myxoma (8.5%). CONCLUSIONS: OTs are rare neoplasms and appear to show geographic variations in the world. In Cappadocia, Turkey, they are more common in the mandible, with ameloblastoma followed by keratocystic odontogenic tumors with the incidences observed in the present study being similar to those of previous studies from Asia and Africa, and in contrast to those reported from American countries.

A c.1244G>A (p.Arg415Gln) mutation in SH3BP2 gene causes cherubism in a Turkish family: Report of a family with review of the literature

OBJECTIVES: The present study was aimed at advancing the understanding of the pathogenesis of cherubism by presenting a case study based on history, physical examination, typical radiological features, molecular and histopathological laboratory tests and a review of the literature. Study DESIGN: This study began with a 7-year-old boy who was referred due to mandibular overgrowth. A panoramic radiograph revealed multilocular radiolucent lesions of the upper/lower jaws suggestive of cherubism. Overall, a total of four family members were tested for SH3BP2 mutations, namely two siblings and their parents. Both siblings had been clinically diagnosed with cherubism; however, the parents were clinically normal. Peripheral blood was collected from all participants and genomic DNA sequencing was carried out. RESULTS: A missense mutation was found in the two affected siblings and their asymptomatic mother. The mutation was a 1244 G>A transversion which resulted in an amino acid substitution from arginine to glutamine (p.Arg415Gln) in exon 9. CONCLUSIONS: The present study emphasized the importance of further clinical and molecular investigation even when only a single case of cherubism is identified within a family. Genotype-phenotype association studies in individuals with cherubism are necessary to provide important insights into the molecular mechanisms associated with this disease

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A c.1244G>A (p.Arg415Gln) mutation in SH3BP2 gene causes cherubism in a Turkish family: report of a family with review of the literature.

OBJECTIVES: The present study was aimed at advancing the understanding of the pathogenesis of cherubism by presenting a case study based on history, physical examination, typical radiological features, molecular and histopathological laboratory tests and a review of the literature. STUDY DESIGN: This study began with a 7-year-old boy who was referred due to mandibular overgrowth. A panoramic radiograph revealed multilocular radiolucent lesions of the upper/lower jaws suggestive of cherubism. Overall, a total of four family members were tested for SH3BP2 mutations, namely two siblings and their parents. Both siblings had been clinically diagnosed with cherubism; however, the parents were clinically normal. Peripheral blood was collected from all participants and genomic DNA sequencing was carried out. RESULTS: A missense mutation was found in the two affected siblings and their asymptomatic mother. The mutation was a 1244 G>A transversion which resulted in an amino acid substitution from arginine to glutamine (p.Arg415Gln) in exon 9. CONCLUSIONS: The present study emphasized the importance of further clinical and molecular investigation even when only a single case of cherubism is identified within a family. Genotype-phenotype association studies in individuals with cherubism are necessary to provide important insights into the molecular mechanisms associated with this disease.

[Retrospective evaluation of the alveolar echinococcosis cases between 1980-2010 in Erciyes University Hospital]. / Erciyes Üniversitesi Hastanesinde 1980-2010 Yillari Arasinda Alveoler Ekinokokkozis Saptanan Olgularin Retrospektif Degerlendirilmesi.

OBJECTIVE: Alveolar echinococcosis is an uncommon parasitic disesae confined to the Northern Hemisphere. There is limited data regarding the incidence of the disease in Kayseri. METHODS: Clinicopathologic features of the cases with the diagnosis of alveolar echinococcosis reviewed between 1980-2010. RESULTS: Twenty-nine cases of alveolar echinococcosis were found. There were no significant distribution differences during the study period. 28 of the 29 cases were localised in the liver, whereas one case was localised in the omentum. Sixteen of the 29 cases were male (55%) and 13 were female (45%). The age distribution of these cases varied between 33 and 80. Thirteen cases resided in Kayseri, 2 cases resided in Erzurum, 1 each case resided in Adana, Ardahan, Kars, Nigde, Nevsehir and Yozgat. We could not obtain information from the remaining 8 cases. Abdominal pain was the main symptom in 8 cases, jaundice in 2 cases and fatigue and fever in one case on admission. One case was detected incidentally. All of the cases were diagnosed by histologic examination. CONCLUSION: The data about the alveolar echinococcosis is limited due to its low prevelance. Alveolar echinococcosis cases were detected in Kayseri with a lower incidence than in the East Anatolian region. This report will add data about the incidence of the alveolar echinococcosis.