Cerebellar neuroblastoma in 2.5 years old child

Neuroblastoma is the third most common malignancy of childhood, after leukemia and brain tumors. Only 2% of all neuroblastoma occur in the brain. Primary cerebellar neuroblastoma is an specific subset of Primitive Neuroectodermal Tumors [PNET]. Meduloblastoma is a relatively common and well-established entity, consisting of primitive and multipotential cells that may exhibit some evidence of neuroblastic or gliad differentiation. But cerebellar neuroblastoma with ultrastractural evidence of significant neuroblastic differentiation is extremely rare. We report a rare case of neuroblastoma in the cerebellum. A 2.5-year-old Iranian boy presented with vomiting and nausea in the morning and ataxia. CT scan showed a tumor mass in the cerebellum and the report of radiologist was medulloblastoma. Light microscopic assay showed a small cell neoplasm with lobules of densely packed cells [lobulated pattern] and better differentiated cells. Neuron-Specific Enolase was positive. Pathologic diagnosis confirmed the existence of cerebellar neuroblastoma. Chemotherapy followed surgical removal. No relapse occurred 12 months after surgery

Unusual presentation of a patient with hemoglobin constant spring and immune hemolytic anemia

Hemoglobin Constant Spring [Hb CS], an abnormal Hb characterized by elongated alpha -globin chain resulting from mutations of the termination codon in the alpha 2 - globin gene, is the most common nondelitional alpha -thalassemic mutation and is an important cause of HbH like disease in Southeast Asia. Case Report: A 9- years-old female with immune hemolytic anemia and splenomegally and abnormal hemoglobin in Hb electrophoresis is reported. The first presentation of our patient was weakness and dark urine. She had a hemolytic anemia with normal MCV and positive direct coombs. In Hb electrophoresis, she had abnormal hemoglobin near the A2 region and slow moving component. PCR testing showed homozygous mutation in codon 142. Hb CS-containing RBCs have membrane pathology and these pathology lead to destruction of her RBCs in reticuloendotelial system and she had a RBC sick syndrome resemble thalassemia intermedia. Our study showed that the five-day massage therapy is a safe technique mothers can perform for stable preterm infants to facilitate weight gain in neonate

Frequency of iron deficiency anaemia and beta Thalassemia minor among adolescent school girls in Ahvaz, Iran

To investigate the frequency of causes of anaemia among adolescent school girls in Ahvaz in 2009. A cross-sectional study was conducted on 208 Adolescent girls aged 15-19 years from high schools in Ahvaz. Blood samples were taken from all patients and were sent to laboratory for determination of complete blood count [CBC] test, serum ferritin [SF], hemoglobin electrophoresis [Hb F, Hb A2] was done for all of the anaemic cases. Iron deficiency anaemia was defined as a situation where Hb is less than 12 g/dl and ferritin is less than 12 ng/ml. Beta-thalassemia were identified by high performance liquid chromatography [HPLC] analysis of hemoglobin and Hb A2>3.4. Of the 208 patients, 37 cases [17.8%] were anemic and Iron deficiency anaemia was found in only 11 from 208 children [5.3%], minor beta thalassemia was found in only five of 208 children [2.4%], mixed beta thalassemia and iron deficiency anaemia was found in four of 208 [1.9%] and other patient with anaemia 19 patient [9.1%] minor alpha thalassemia was considered, anybody of students were macrocytic anaemia. The results suggest that thalassemia and iron deficiency anaemia may be major contributing factors to the occurrence of anaemia in this area among schoolgirl population

Clinical characteristics and the prognosis of childhood rhabdomyosarcoma in 60 patients treated at a single institute

Rhabdomyosarcoma [RMS] is the most frequent soft tissue sarcoma in children. The aim of study was to retrospectively review the treatment results of childhood rhabdomyosarcoma and identify prognostic factors. 60 children with rhabdomyosarcoma treated between 1996 and 2002 in Shafa Hospital were reviewed. The data were analyzed for clinico-epidemiological factors. Age, gender, race, histology type, primary site, tumor size and intergroup rhabdomyosarcoma study [IRS] group were evaluated. The primary site of involvement was orbit in 6 cases [10%] head and neck nonparameningial in 12 cases [20%], parameningial region in 12 cases [20%]. The histological findings were as follows: 12 cases [72.5%] for embryonal, 6 cases [10%] for alveolar and 11 cases [17. 5%] for botryoid type. With respect to the IRS III [15%] were group II, 32 [52.5%] were group III and 24 cases [40%] were group IV. The 5-year survival rate was 47.9%. Primary tumor site [P=0.0003], and histology [P=0.05] were associated significantly with survival after recurrence. Among the variables, age, gender, regional lymph node involvement, and IRS group did not affect 5-year survival but the type and time of recurrence [P=0.0002], and its relation with therapy [P=0.0001] were associated with survival. This study showed that overall survival for rhabdomyosarcoma is dependent on histological subtype, primary site, disease group, duration of disease before treatment. The outcome for infant with RMS is less satisfactory than older children and the patients aged 1-9 years had the best 5 year survival

Serum zinc level in thalassemia major

To compare serum zinc level between Thalassemia Major [TM] patients and normal population at Shafa Hospital in South West of Iran. A total of 25 male and 36 female of TM patients were enrolled in this study. Out of 61 patients thirty were treated by deferroxamine [DFO] and 31 were on the combination of DFO and deferiprone [DEF] protocol therapy. Sixty normal subjects of the matching age and gender were recruited as controls. From each patient and control group 2 ml of blood was taken in fasting condition. Cell blood count and serum zinc were carried out for both thalassemia patients and normal subjects. The mean age of patients and control group was 15 +/- 5years. Mean serum zinc level was 68.97 +/- 21.12microg/dl, 78.10 +/- 28.50 microg/dl, and 80.16 +/- 26.54 microg/dl in the TM with DFO, TM with DFO + DEF combination protocol and control group respectively. There was no significant correlation between patients and control group. However 50 percent of TM with DFO, 38.7 percent of TM with DFO + DEF and 32.8 percent of control group had hypozincemia. Nearly 40 to 50 percent of TM patients and one third of normal subjects are suffering from hypozincemia. This study shows that low level of serum zinc is a health problem in both thalassemia patients and normal population in South West of Iran

[Elucidation of alpha-thalassemia mutations in Khuzestan, Iran]

Thalassemia is one of the most common hemoglobin disorders, in which alpha-thalassemia appears in affected individuals with hypochromic microcytic anemia. Because of the absence of the mutation detection capabilities in the most health care centers in Iran, the most patients with alpha-thalassemia misdiagnosed as beta silent carriers. Until now, no inclusive research has been done to expose the patterns of it in Khuzestan. Therefore, in the present study we decided to clarify the prevalence of the mutations in alpha -Thalassemia in Khuzestan. One hundred and fourteen patients from Khuzestan were selected among the patients, referred to Kariminejad and Najmabadi Pathology and Genetics Center, between 1998-2006, showing the signs of hypochromic microcytic anemia and normal HbA2 levels. First of all, they were all tested for 3 common alpha -thalassemia mutations,-3.7, -4.2, --MED, by gap-PCR amplification method. Alpha-Globin Strip Assay of the nine mutation panel and DNA sequencing was used to determine other major contributes in Khuzestan. We could detect alpha -thalassemia mutations for 84.2% of tested individuals. 79.2% had cis-type alpha -thalassemia, 13.5% trans-type and 7.3% was a carrier for two cis-type mutations. In total, 72.9% were silent carrier, 19.8% alpha -thalassemia trait, and 7.3% had alpha -thalassemia major. As an early report 27.4% of the tested alleles were found to be mutated. The - alpha[3.7] single gene deletion was the most frequent alpha -globin mutation in our population representing 55.2% of alpha -thalassemia mutations in Khuzestan. Twelve other mutations [--MED, alpha[PA2[GAA]], - alpha[4.2], alpha[cd19], alpha[-5nt], alpha[cd14], alpha[PA1[AAG]], alpha[CS], anti 3.7 triplication, alpha[St], alpha[cd21], alpha[cd59]. We strongly recommend screening for the identified ten common mutations to improve the molecular diagnosis of anemia

Monitoring of serum nitric oxide in patients with acute leukemia

Nitric oxide [NO] is a molecule required for many physiological functions, produced from L-arginine by NO synthases [NOS]. It is a free radical, producing many reactive intermediates that account for its bioactivity. Sustained induction of the inducible form of NOS [iNOS] in chronic inflammation may be mutagenic, through NO-mediated DNA damage or hindrance to DNA repair, and thus potentially carcinogenic. Due to the short half-life of NO, usually its end products [nitrate or nitrite] are measured as an index of NO production. There is evidence that expression of iNOS in tumor cells, including acute myeloid leukemia and chronic lymphocytic leukemia increased. In this study, the levels of nitrate and nitrite [nitric oxide products] in the serum of patients with acute leukemia were determined. The serum levels of these compounds were measured in 40 acute leukemia patients. The results of serum nitrite and nitrate of patients were compared with corresponding values obtained in 40 healthy volunteers. These results indicate that patients with acute leukemia had a significant increase in the serum level of nitrite and nitrate