Rare presentation of renal failure related to tumor lysis syndrome

Background: Tumor lysis syndrome (TLS) which mostly occurs in lymphoproliferative malignancies after the start of chemotherapy is an oncologic emergency. Manifestations of metabolic imbalance including increasing hyperkalemia, hyperphosphatemia, hyperuricemia and hypocalcemia are common presentation of TLS. Case report: We present two cases of spontaneous TLS; a rare presentation of TLS before cytotoxic chemotherapy. These cases were admitted with presentation of TLS without any history of chemotherapy with mediastinal mass in chest X-ray (CXR) and subsequent diagnosis of lymphoblastic lymphoma and T-cell acute lymphocytic leukemia (ALL). After several hemodialysis sessions, their conditions were improved and they underwent chemotherapy. Conclusions: It was found that the presentation of mediastinal mass in cases of lymphoma and acute leukemia might be associated with TLS before chemotherapy. In addition, it is important to pay attention to CXR, when we face to a patient with acute renal failure related to TLS

A rare case of Kikuchi-Fujimoto disease (case report)

Background: Kikuchi disease is a rare, benign, self-limited disease characterized primarily by fever and cervical lymphadenopathy. Diagnosis is based on excisional biopsy and pathologic study. We report a case of an atypical axillary lymph node enlargement. Case report: This patient was a 12-year-old boy with clinical characteristics including axillary lymph adenopathy, fever and fatigue. He became asymptomatic after excisional biopsy. Histologic study reported necrotizing lymphadenitis without neutrophils (Kikuchi disease). Conclusions: These findings are important for diagnosis, because of the disease rarity, clinical features (such as lymphadenopathy, prolonged fever) and unidentified etiology

A rare presentation of actinomycosis: case report

Background: Anaerobic, nonsporulating, Gram-positive bacteria groups called actinomyces organisms are responsible for the so called actinomycosis. This chronic disease is rare in children and has tendency to mimic many other diseases. It also has wide variety of manifestations and non-specific symptoms. As a result, it is difficult to diagnose before the biopsy and microscopic examination. Although infection may involve any organ in the body, the significant sites of actinomyces infection include cervicofacial, abdominal, pelvic and pulmonary tissues. Case report: Here, we describe one case of unusual presentation; an 11-year-old girl with a soft tissue mass in the left lower lateral chest wall which was finally diagnosed actinomycosis based on the pathological findings. Conclusions: Actinomycosis may rarely present with chest wall mass

The effect of using partnership care model on the quality of life in the school-age children with β-thalassemia

زمینه و هدف: تالاسمی از شایع ترین بیماری های ژنتیک است که بر کیفیت زندگی اثر دارد. با توجه به لزوم یافتن بهترین روشهای اداره تالاسمی از طریق درگیری تمام ارکان مراقبتی، تحقیق حاضر با هدف تعیین تأثیر مدل مراقبت مشارکتی بر کیفیت زندگی کودکان سن مدرسه مبتلا به تالاسمی انجام گرفته است. روش بررسی: در این پژوهش تجربی 72 کودک مبتلا به تالاسمی ماژور به صورت در دسترس انتخاب و به صورت تصادفی در دو گروه آزمون و شاهد قرار گرفتند. پرسشنامه های دموگرافیک و کیفیت زندگی عمومی کودک (گزارش کودک و والدین) (Peds-QOL)، قبل از مداخله در هر دو گروه تکمیل و مدل مراقبت مشارکتی بر اساس مراحل انگیزش، آماده سازی، درگیرسازی و ارزشیابی، در گروه آزمون و با حضور مراقبت دهنده اصلی در منزل به مدت 2 ماه اجرا شد. میانگین نمرات کیفیت زندگی 3 ماه بعد از مداخله با اطلاعات قبل از مداخله به کمک آزمون های آماری کای دو، فیشر و t مستقل و t زوجی مقایسه گردید. یافته‌ها: نتایج نشان داد که تفاوت معنی‌داری در کیفیت زندگی کودکان بین دو گروه قبل از مداخله وجود ندارد، میانگین نمره کیفیت زندگی پس از مداخله در همه ابعاد (گزارش والدین و کودک) به جز عملکرد جسمی والدین در گروه آزمون بالاتر از گروه شاهد بود (001/0

Complications of patients with thalassemia major and intermedia in a selected Iranian population

Background: Due to anemia in thalassemia major (TM) and thalassemia intermedia (TI) patients, bone changes occur, especially in the broad bones like jaw and skull, which are the main sites of hematopoiesis. Therefore, the dentist should be aware of the disease to prevent complications. The aim of this study was to evaluate the radiographic findings of the jaw and teeth in TM and TI patients and to compare the two groups. Methods: 50 TM patients and 50 TI patients in Amirkola Thalassemia Center, whose thalassemia were definitively diagnosed by a hematologist, were selected as the study group and the control group consisted of 50 healthy individuals. In patients` panoramic radiographs, dental anomalies (microdontia, root shortening etc.) and bone disorders (bone marrow hyperplasia, maxillary sinus invisibility etc.) were assessed. A p<0.05 was considered. Results: Dental anomalies were (42.84) in TI patients and (23.46) in the control group, the difference was significant. Dental anomalies in TM patients were (38.76) and in the control group (23.46) and a significant difference was observed (p<0.001). Bone disorders were (47.94) in TI patients and in the control group (32.64). Bone disorders in TM patients were (44.88) and in the control group was (32.64) that showed a significant difference. Conclusion: This study showed that in thalassemia patients, bone and dental disorders frequency were higher than healthy individuals. Bone disorders were also more common than dental anomalies. Dental and bone disorders were more common in TI

Complications of patients with hematologic malignancies in a selected Iranian population

Background: Hematologic malignancies in childhood and their treatments can cause dental anomalies and jaw bone abnormalities; therefore, the aim of this study was to assess the prevalence of these disorders in children. Methods: This cross-sectional study was conducted on all children diagnosed with hematologic malignancies that referred to in Amirkola Children’s Hospital from 2011 to 2018. All of children underwent clinical evaluation in a Dental Radiology Clinic and panoramic imaging was performed. The radiographs were examined for dental anomalies and jaw bone abnormalities. The data were analyzed by descriptive analysis using chi-square, Mann-Whitney and Kruskal-Wallis with a 95 confidence interval. Results: The study population consisted of 32 children and 9.46 of these patients showed intraoral signs in clinical evaluations. Panoramic radiographs indicated dental anomalies in 12 (63.9) and jaw bone abnormalities in 17 (89.4) patients. The most common dental anomaly and bone abnormality were taurodontism and changes in lamina dura, respectively. By measuring the mandibular cortical index (MCI), it was revealed that 13 had osteopenia, in which 4 of them had severe osteopenia (osteoporosis). The statistical analysis demonstrated that there was a significant difference between the incidence of osteoporosis and changes in lamina dura with the gender (p<0.05). Conclusion: Due to early onset of osteopenia and osteoporosis and the presence of dental and bone abnormalities in half of study population, early assessment of their clinical and radiographic condition can be helpful in the prevention and the early treatment of their oral complications

Relationship between β-Thalassemia minor and Helicobacter pylori infection

Background: Until now, no study has been reported investigating the association between β-thalassemia minor and Helicobacter pylori (H. pylori) infection. This study was designed to compare H. pylori infection rate between β-thalassemia minor patients and healthy controls. Methods: A number of 100 β-thalassemia minor patients (50 males, 50 females) and 100 gender-matched healthy controls were prospectively recruited in this study in a period of 3 months. The study population consisted of the people who referred to a health center in Babol, North of Iran, for premarital counseling. H. pylori status was assessed by measuring the anti-H. pylori IgG antibodies using enzyme-linked immunosorbent assay. Demographic information and informed consent were collected from all participants. Results: The overall H. pylori infection rate was 43%. The infection was significantly more prevalent in thalassemia patients (53%) than in the controls (33%) in both univariate (OR=2.29, 95% CI: 1.3-4.06) and multivariable analyses (OR=2.05, 95% CI: 1.12-3.76). Age was the only significant factor which was positively correlated with the infection in β-thalassemia minor cases (OR=1.11, 95% CI: 1.02-1.2). Gender, blood groups, residency, and education level were not related to the infection. Conclusions: According to the results, it can be concluded that β-thalassemia minor patients are possibly more susceptible to H. pylori infection than healthy people. Further studies are needed to discover more about the exact mechanisms of increased susceptibility to H. pylori infection in β-thalassemia minor patients

Therapeutic Effects of Recombinant Growth Hormone in Improving the Growth of Thalassemia Major Patients: A Retrospective Study

Background and purpose: Beta thalassemia major is one of the most common inherited blood diseases and patients with this condition require regular blood transfusions. The resulting iron overload deposition causes endocrine gland disorders, including growth hormone (GH) abnormality. In this study, we examined the effects of recombinant growth hormone (rGH) in these patients. Materials and methods: This retrospective study was performed in thalassemia major patients in Amirkola Thalassemia Center, using the medical records (2011-2018), to evaluate the therapeutic effects of rGH on  growth velocity of six short stature patients aged 7-15 years (four boys and two girls). On the basis of abnormal or normal response to GH provocation test, the patients were included in GH deficiency (GHD) group (n=3) and idiopathic short stature (ISS) group (n=3), respectively. The data were analyzed in SPSS-18. Results: The mean age of patients was 11.00 ± 2.75 years old. The mean values for growth velocity before and after the treatment were 4.00± 0.86 and 7.83 ± 1.25 cm/year in GHD group (P=0.057) and 4.06 ± 0.81 and 6.00 ± 1.50 cm/year in ISS group, respectively (P=0.023). Conclusion: In this study, the positive therapeutic effects of rGh in improvement of growth velocity were seen in thalassemia major patients (both GHD and ISS groups)

The effects of beta-carotene and vitamin E on erythrocytes lipid peroxidation in beta-thalassemia patients

<strong>BACKGROUND</strong>: Thalassemia is the most common hereditary disease in the world. Thalassemic erythrocytes are exposed to higher oxidative stress and lipid peroxidation. The aim of this study was to investigate the effects of beta-carotene and vitamin E on erythrocytes lipid peroxidation in beta-thalassemia patients.<br /> <strong>METHODS</strong>: A prospective double-blind, placebo-controlled study of the effect of beta-carotene and vitamin E on lipid peroxidation in erythrocytes membranes was performed on 120 beta-thalassemia major patients in four groups. The patients were supplemented for 4 weeks as follows: group 1 with beta-carotene (13 mg/day), group 2 with vitamin E (550 mg/day), group 3 with beta-carotene plus vitamin E and group 4 with placebo. We prepared all capsules for 4 roups in the same shape and color. Measurements of serum beta-carotene and vitamin E were performed by high performance<br /> liquid chromatography. After preparation of ghost cells from blood specimens, malondialdehyde (MDA) was determined as index of lipid peroxidation in erythrocytes membranes before and after treatment. RESULTS: The levels of serum beta-carotene and vitamin E were significantly lower and MDA concentrations in erythrocytes membranes were significantly higher in beta-thalassemia patients compared to controls (P<0.001). In groups that treated with vitamin supplements for 4-weeks, lipid peroxidation rates were significantly reduced after treatment (P<0.001), but in placebo group there was not significant difference (P>0.05). <br /> <strong>CONCLUSIONS</strong>: Our findings provide evidence that an oral treatment with beta-carotene and vitamin E can significantly reduce lipid peroxidation of erythrocytes membranes and could be useful in management of beta-thalassemia major patients. <strong>KEYWORDS</strong>: Beta-thalassemia major, beta-carotene, vitamin E, malondialdehyde, lipid peroxidation.<br /&gt

Isolated thrombocytopenia report of a rare presentation of childhood Systemic Lupus Erythematosus

Background: Systemic lupus erythematosus (SLE) has various presentations in children. Hematologic abnormalities is common in childhood onset of SLE, however, isolated thrombocytopenia is relatively rare. Thus, we present a child with isolated thrombocytopenia as a rare presentation of SLE. Case presentation: A 12-year-old boy with chief complaints of loss of appetite, weight loss, decreased platelet count (8000/µL) and lymph node enlargement was referred to our hospital. Biopsy of lymph node showed reactive lymphadenopathy. Investigations regarding infectious disease was negative. Platelet count remained low after low dose steroidned therapy. Antinuclear antibody (ANA) and anti-double stranded DNA antibody screening tests were positive with titer of 1/62 and 1/54, respectively. Therefore, juvenile SLE was considered as the final diagnosis and raising the dose of prednisolone to 2mg/kg/day was associated with increasing platelet count to 40000/µL and a week later to 96000/µL. Conclusion: The findings of this study indicate that in cases with isolated thrombocytopenia refractory to conventional dose of steroids, SLE should be considered. This study justifies serum ANA and anti DNA assessment in children with thrombocytopeni