Biomedical subjects

Stavroula Kostaridou

Publications and source records attributed to Stavroula Kostaridou.

6 recordsLinked to original sources

PubMed2005

Abdominal lymphadenopathy in beta-thalassemia: MRI features and correlation with liver iron overload and posttransfusion chronic hepatitis C.

OBJECTIVE: The objective of our study was to describe the MRI features of abdominal lymphadenopathy in patients with beta-thalassemia major and investigate the relation of abdominal lymphadenopathy with the severity of iron overload and posttransfusion chronic hepatitis C. MATERIALS AND METHODS: Abdominal MRI studies of 60 consecutive patients with beta-thalassemia major, performed for quantification of liver iron overload at a single institution, were retrospectively studied for the presence of lymph nodes and their distribution, size, and number. The signal intensity ratios of liver, spleen, and the largest lymph node to the right paraspinous muscle (L/M, S/M, and LN/M, respectively) were calculated on T1-weighted gradient-echo images. MRI findings for the lymph nodes were compared with the histologically assigned activity level of chronic hepatitis C that was available in 17 patients who had undergone liver biopsy within 1 month of the MRI examination. RESULTS: Hypointense abdominal lymph nodes larger than 7 mm were seen in 19 (32%) of 60 thalassemic patients in perihepatic and paraortic distributions. Lymphadenopathy was related to both the severity of hepatic siderosis, as expressed by the L/M values, and the presence of chronic hepatitis C, given that 18 (95%) of the 19 thalassemic patients with lymphadenopathy had chronic hepatitis C. Moreover, thalassemic patients with a moderate or severe level of hepatic inflammation presented with abdominal lymphadenopathy more frequently than those with mild hepatic inflammation. CONCLUSION: The development of hypointense abdominal lymphadenopathy in patients with beta-thalassemia major who have received multiple transfusions depends both on the severity of liver iron overload and on the presence and the activity level of coexistent chronic hepatitis C.

Abdomen↗

PubMed2005

Recurrent torticollis secondary to Langerhans cell histiocytosis: a case report.

Torticollis is a common clinical sign encountered by pediatricians and orthopaedic surgeons in a wide spectrum of childhood conditions ranging from benign to life-threatening. We report the case of a child with recurrent torticollis caused by Langerhans Cell Histiocytosis (LCH). The patient was a 1-year-old boy with recurrent torticollis, followed by a painless swelling over the right temporal bone. The diagnosis was confirmed by an open biopsy of the calvarial lesion. As LCH is a very rare cause of torticollis it was not considered in the initial differential by the primary care physicians and the diagnosis was delayed about 4 months. The patient received chemotherapy with steroids and etoposide for 52 weeks. He showed complete regression of the sign and imaging tests at the end of treatment were normal. No relapse of symptoms occurred during a follow-up period of 2 years. The rarity of this disease as well as the site and form of presentation are emphasised to alert physicians for an early diagnostic evaluation, which is important to prevent neurological lesions and other late complications.

Adrenal Cortex Hormones↗

PubMed2004

Non-Hodgkin lymphoma in a child with Williams syndrome.

Non-Hodgkin lymphoma in an 8-year-old boy with Williams syndrome is reported. Molecular DNA analysis showed a maternal deletion at 7q11.23, the locus of elastin and several other genes, including the BCL7B gene, involved in early development. To our knowledge, this is the second reported case of a lymphoma in a Williams syndrome patient and the first in a child.

Burkitt Lymphoma↗

PubMed2004

Ineffective erythropoiesis underlies the clinical heterogeneity of congenital dyserythropoietic anemia type II (CDA II).

BACKGROUND: Congenital dyserythropoietic anemia type II (CDA II) is an autosomal recessive disease, and is the most common CDA. The qualitative and quantitative defects of erythropoiesis in CDA II, as estimated by the hematological and biochemical parameters at the time of diagnosis, may not reflect the heterogeneity of the disease course of each patient. METHODS: Three pediatric patients with CDA II are herein presented, having an heterogeneous clinical course. In addition to bone marrow morphology, Ham test and SDS-PAGE of erythrocyte membrane protein, the present study also examined erythroid marrow activity, by measuring serum Erythropoietin (Epo), soluble Transferrin Receptors (sTfR) concentrations and Reticulocyte Production Index (RPI). RESULTS: All patients demonstrated the same pattern of ineffective erythropoiesis, having increased Epo (350-389 IU/L), sTfR concentrations (6.2-7.8 mg/L) and low RPI values (0.8-1.3). Erythron expansion was expressed by RPI values nearly twofold higher than normal values in parallel to raised sTfR and high Epo production. Despite the same degree of ineffective erythropoiesis seen in all patients, the severity of the clinical course was diverse in terms of frequency and clinical relevance of transfusion needs. CONCLUSION: An analysis of the parameters expressing ineffective erythropoiesis in CDA II can provide a better understanding of the degree of dyserythropoiesis, however it is not useful for predicting the clinical course of disease in patients, because the underlying genetic heterogeneity of this disorder remains obscure.

Anemia, Dyserythropoietic, Congenital↗

PubMed2004

Decrease of CD4+ and B-lymphocyte populations is not associated with severe infectious complications in children with acute lymphoblastic leukemia during maintenance.

The suppression of lymphopoiesis and immune competence during the maintenance phase in children with acute lymphoblastic leukemia (ALL) and the occurrence of infectious complications remain an unexplored area. In this study we assessed lymphocyte subpopulation disturbances during maintenance for childhood ALL along with the incidence, type, and severity of infections that occur during that period in the absence of neutropenia. Twenty-eight children (13 boys, 15 girls) with ALL aged 3-14 years (median 7 years) and treated according to the ALL-BFM 90/95 protocol were studied during maintenance for ALL. Complete white blood cell (WBC) counts and peripheral blood lymphocyte (PBL) analyses were performed. Major lymphocyte subsets (CD19+, CD3+CD4+, CD3-CD8+, CD3-CD16+CD56+, CD45RA-, CD45RO+) and markers of T-cell activation (CD25, CD38, CD69, HLA-DR) were analyzed with flow cytometry. Serum immunoglobulin G (IgG), IgA, and IgM levels were measured by a nephelometric assay. All infectious episodes during the study period were recorded in detail. Additionally, 41 age-matched immunocompetent children were used as controls. Absolute WBC counts (median, 3627/microL) and PBL counts (median, 1206/microL) were significantly below the age-adjusted control values (7400/microL and 2673/microL, respectively; P < .0001). B-lymphocyte, total CD4+, and memory CD4+ (CD4+CD45RO+) subsets were also significantly decreased (33/microL versus 377/microL [P < .0001], 531/microL versus 1045/microL [P < .01], and 80/microL versus 299/microL [P < .001], respectively). Significantly lower immunoglobulin levels were found in all patients. Twenty-two of the 28 patients presented with 74 episodes of a variety o minor infections (mostly respiratory viral [39], skin [7], and gastrointestinal [3]), none demanding prolonged hospital treat ment. Our findings demonstrate a profound immunosuppression throughout maintenance therapy in children with ALL tha has no major clinical impact in terms of increased incidence or severity of systemic infections.

Adolescent↗

PubMedDate not supplied

Nasopharyngeal carcinoma in childhood and adolescence: a single institution's experience with treatment modalities during the last 15 years.

Pediatric nasopharyngeal carcinoma (NPC) represents a locally advanced undifferentiated tumor with widely varying epidemiological features and with a high cure rate when combined modality treatment is provided. Both local and systemic treatment is necessary, and additional treatment with biologic modifiers seems promising. In this study, clinical experience and therapeutic results of 10 children with newly diagnosed NPC, treated in a single pediatric hematology/oncology institution in Athens over a period of 15 years, are analyzed. Results from Greece on NPC in young patients are reported for the first time. Ten patients (6 male, 4 female) 7-14 years old (median = 12.5) with a nasopharyngeal tumor were retrospectively evaluated. Disease extent was staged according to the TNM system. EBV-DNA, EBERs, and LMP-1 from paraffin-embedded tissues were studied in 8 patients. All patients received both local and systemic treatment. All cases were classified as type WHO-3. The presence of EBV-DNA and expression of EBER 1-2 mRNAs was demonstrated in the 8 tumors examined, while LMP-1 protein was expressed in 4/8 of the studied cases. Disease stage was III in 4 and IV in 6 patients. Time from the onset of symptoms to diagnosis ranged from 4 to 24 weeks (mean 8 weeks). All patients received preradiation chemotherapy and radiotherapy, and 5/10 received postradiation chemotherapy due to either resistant or advanced disease. In 9/10 patients, complete locoregional control was achieved. In addition to chemotherapy and radiotherapy the latest patient of this series was treated with recombinant IFN-beta (10(5) IU/Kg i.v. 3 times a week) for 6 months and at 18 months remains in continuous complete remission. One patient was lost to follow-up 3 years after cessation of treatment while remaining in complete remission. Of the remaining 9 patients, 7 are alive for a median follow-up of 54 months (range 18-186); 5/7 are free of disease, and 2/7 are with disease but stable. The median time for first relapse was 17 months. The data confirm the good results of combined chemo-radiotherapy treatment for high-risk NPC in young patients. The documented EBV latency underlying this tumor, which possibly critically mediates its pathogenesis, justifies the use of biological modifiers with antiviral and immunoregulatory activity, like the IFNs, which may offer better therapeutic results in the future.

Adolescent↗