Gürses Şahin

EXPRESSION OF MATRIX METALLOPROTEINASE-9 (MMP-9) AND TISSUE INHIBITOR OF MATRIX METALLOPROTEINASE (TIMP-1) IN TISSUES WITH A DIAGNOSIS OF CHILDHOOD LYMPHOMA

Matrix metalloproteinases (MMP) are enzymes involved in the reconfiguration of the microenvironment by means of degrading the extracellular matrix and have more than 20 subgroups containing zinc. Proteins that serve as the inhibitors of these enzymes are called tissue inhibitors of matrix metalloproteinase (TIMP). These enzymes have been shown to be active in a wide range of processes, from wound recovery to fetus development, heart diseases, and spread of malignant diseases. The aim of this study was to investigate whether there is a relationship between the type, stage, and prognosis of childhood lymphoma subjects and matrix metalloproteinase type-9 (MMP-9) and its inhibitor, tissue inhibitor of matrix metalloproteinase type-1 (TIMP-1). Paraffin blocks of childhood patients diagnosed with non-Hodgkin lymphoma (n = 23), Hodgkin lymphoma (n = 14), or reactive lymphadenopathy (n = 12) were retrospectively immunohistochemically stained with MMP-9 and TIMP-1 stains and whether there was a relationship between the degree of staining and the type, tumor stage, and prognosis of the disease was investigated. Moderate and high degrees of MMP-9 staining were detected in 94.6% of the lymphoma patient tissues and a slight TIMP-1 staining was detected in 21.6% of the lymphoma patient tissues. No relationship was observed between the degree of these staining patterns and the type, tumor stage, and prognosis of the disease. This study indicates that the equilibrium between MMP-9 and TIMP-1 is important in lymphomas in addition to all the physiological and pathologic events although MMP-9 and the TIMP-1 staining patterns are not related to the tumor stage, prognosis, and type of the disease. Larger series of patients are needed to determine the prognostic value of MMP-9 and TIMP-1 in childhood lymphoma.

The Correlation Between Pre-treatment Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Parameters and Clinical Prognostic Factors in Pediatric Hodgkin Lymphoma

Objective: To compare standardized uptake values (SUV) derived from pre-treatment 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) imaging and clinical prognostic factors in pediatric patients with Hodgkin lymphoma (HL). Methods: Pre-treatment FDG PET/CT findings of 28 children with HL were evaluated in this retrospective study. Metabolic tumor volume (MTV), SUVmax normalized by weight (SUVweight), lean body mass (SUVlbm), body surface area (SUVbsa) and plasma glucose levels of tumors (SUVglucose) were calculated using pre-treatment FDG PET/CT scan images. These metabolic parameters were correlated with clinical factors [age, sex, number of lymph node groups, presence of splenic involvement, bulky mediastinal disease, Ann Arbor stage, serum white blood cell (WBC) count, erythrocyte sedimentation rate (ESR), serum albumin and hemoglobin levels]. Results: SUVbsa, SUVlbm, SUVweight, SUVglucose and MTV were higher in patients with stage III-IV disease, bulky tumor and ≥3 lymph node groups (p<0.05). SUVbsa and SUVglucose were higher in patients with splenic involvement (p<0.05). There was no significant correlation between these metabolic parameters and sex, ESR, levels of albumin and WBC (p>0.05). SUVbsa and SUVlbm were higher in patients with anemia (p<0.05). Additionally, significant increases were detected in SUVweight, MTV, and SUVglucose with increasing age (p=0.005, p=0.027, and p=0.009, respectively). SUVbsa and SUVlbm had no significant correlation with age (p>0.05). Conclusion: Metabolic parameters derived from pre-treatment FDG PET/CT may have an important role in predicting high-risk disease in patients with HL. Also, SUVbsa and SUVlbm may be better markers than SUVweight in the quantitative evaluation of FDG PET/CT scans in pediatric patients.

Kikuchi–Fujimoto disease triggered by Salmonella enteritidis in a child with concurrent auto-immune thyroiditis and papilloedema

Abstract Kikuchi–Fujimoto disease (KFD) is a histiocytic necrotising lymphadenitis characterised by painful cervical lymphadenopathy, fever, malaise and weight loss. Infections, auto-immune pathogenesis and a genetic association have been implicated. A 12-year-old boy presented with a 1-month history of fever, abdominal pain, constipation and weight loss, and a painful lymph node was detected in the right axilla. Chest CT demonstrated multiple lymph nodes, especially in the left mediastinum. Salmonella enteritidis group D was detected in a blood culture and he was treated with ceftriaxone, followed by meropenem. An axillary lymph node biopsy demonstrated necrotising histiocytic lymphadenitis and KFD was diagnosed. He was discharged 35 days after admission. He was re-admitted 3 weeks later with recurrence of symptoms and headache and was found to have papilloedema of the left eye and auto-immune thyroiditis. Intravenous immunoglobulin (IVIG) 400 mg/kg/day was administered for 5 days. The fever and papilloedema slowly resolved and, subsequently, the thyroiditis, and he has remained well on follow-up. This is the first report of an association of S. enteritidis infection and papilloedema with KFD. IVIG may be required in prolonged or recurrent cases and in those with an auto-immune association.

Epidemiological features and risks of hemangiomas

Şahin G, Düzcan-Kilimci D, Tanyıldız HG. Epidemiological features and risks of hemangiomas. Turk J Pediatr 2017; 59: 664-669. Hemangiomas are the most common benign tumors of childhood. The known risk factors in developing hemangiomas are female gender, prematurity and low birth weight. Our study was designed to investigate prenatal and natal risk factors in addition to the above factors that would potentially affect development of hemangiomas. Two hundred forty hemangioma patients and 100 healthy controls were included in the study. Both groups were interviewed for age, gender, prenatal characteristics (presence of maternal pregnancy hypotension, hypertension and hyperemesis, parental smoking habits) as well as natal characteristics (delivery, birth weight, gestational age) and maternal-paternal age. Hemangiomas were mostly localized in the head and neck. Risk of developing hemangioma was higher in girls and in firstlings. History of hyperemesis during pregnancy increased incidence of hemangioma. Post-term delivery was found to be protective against developing hemangioma. We are in the opinion that there is a need for wider studies with a bigger sample size so as to detect other risk factors.

Clinical and epidemiological characteristics of children with germ cell tumors: a single center experience in a developing country

İncesoy-Özdemir S, Ertem U, Şahin G, Bozkurt C, Yüksek N, Ören AC, Balkaya E, Alkan A. Clinical and epidemiological characteristics of children with germ cell tumors: A single center experience in a developing country. Turk J Pediatr 2017; 59: 410-417. Germ cell tumor (GCT) is a rare malignancy accounting for 2-3% of all pediatric tumors. The overall survival rate of children and adolescents with GCT is more than 80% after adopting combined therapy. The aim of this study is to review clinical presentation, management, and outcome in a single-center series with extracranial GCT. Clinical characteristics, pathologic presentations, and survival outcomes of 101 children with GCT, treated at our hospital from 1988 to 2011, were analyzed. Sixty-two of patients were female and 39 of them were male. Fifty-eight (57%) patients had gonadal tumor (24 testicular, 34 ovarian), 43 (43%) extragonadal. Histologically, teratomas were found most frequently (26 mature, 10 immature), followed by yolk sac tumors (n: 33), mixed malignant tumors (n: 13), embryonal carcinoma (n: 10), disgerminoma (n: 8) and seminoma (n: 1). Twenty-six patients were diagnosed as mature teratoma and we excluded them in the evaluation of staging and survival. Five-year overall and relaps-free survival were 80.3% (mean follow-up time: 215.8 months) and 73.4% (mean follow-up time: 176.2 months), respectively. Five-year survival rates were 93.2% and 90.2% in malign GCTs diagnosed after 1999.

Ewing`s sarcoma of the mandible misdiagnosed as periodontal inflammation: report of three cases

Balkaya E, Bozkurt C, Aksu AE, Özmen S, İncesoy-Özdemir S, Şahin G. Ewing`s sarcoma of the mandible misdiagnosed as periodontal inflammation: Report of three cases. Turk J Pediatr 2017; 59: 704-707. Ewing`s sarcoma (ES) is the second most common childhood primary malignant tumor of the bone. The most popular locations of ES are long bones and pelvis. The involvement of the mandible is very rare in childhood. In last 10 years, we met with three cases of ES of the mandible in our department. Initially the patients had symptoms similar to periodontal inflammation. The involvement of the mandible might be considered with periodontal inflammation in its initial stages, what frequently leads to delayed treatment. Although this tumor has an aggressive clinical behavior and rapid growth, early diagnosis can reduce patient`s morbidity and mortality and thus it is important to distinguish from periodontal inflammation.

Vitamin D Receptor (vdr) Polymorphisms in Pediatric Patients Presenting With Hodgkin’s Lymphoma

Vitamin D receptor (VDR) polymorphisms are found more commonly in some tumor types than in healthy individuals, suggesting that some polymorphisms (Cdx2, Fok1, Bsm1, Apa1, Taq1) contribute to tumor development. There is no previous report on VDR polymorphism in Hodgkin’s lymphoma (HL) patients. VDR polymorphism patterns in 95 pediatric HL cases with 100 healthy controls were compared. No statistically significant difference was found between the patient group and control group in terms of Cdx2, Fok1, Bsm1, Apa1, and Taq1 polymorphisms (P>0.5). Our findings suggest that VDR polymorphisms may not play a role in HL development.

Ağır beslenme bozukluğu ile başvuran birincil polidipsi olgusu

Primary polydipsia is a clinical status with excessive fluid consumption without any physiological need A seventeen month old male infant with severe malnutrition and developmental retardation was found to have polyuria and polydipsia in the follow up The urine density was found to be 1001 Na was found to be 124 mEq L and the serum and urine osmolarity was found to be low The patient was diagnosed with primary polydipsia and was treated by gradual water restriction This case is presented to emphasize that primary polydipsia changes nutritional habits which may lead to malnutrition and developmental retardation Turk Arch Ped 2013; 48: 251 254