Iranian Journal of Pediatric Hematology and Oncology
Volume:2 Issue: 3, Summer 2012

Computed tomography (CT)-guided biopsy of the lung is a well-established method of diagnosis. The aim of this study is to determine the success rate of fluoroscopic CT (FCT) and conventional CT (CCT) in needle navigation biopsies from the lung lesions.

A total of 78 patients were prospectively enrolled to receive CT-guided biopsy with (group I, n=23) or without (group II, n=55) fluoroscopic guidance. The mean age was 13.5, and the outcome measurements were success and complication rate of procedure.

The success rate was increased in the FCT group (87%) as compared with that of CCT group (80%, p=0.012). The complications (pneumothorax and bleeding) were not significantly different between the two groups (0 versus 8 cases, p=0.097).

The obtained results revealed that the FCT facilitates the CT-guided biopsy procedures and it will reduce the complications by allowing real-time visualization of the needle tip from skin entrance to the target point.

Bone mineral density (BMD) may be occurred following treatment of Acute lymphoblastic Leukemia (ALL). 25-hydroxyvitamin D insufficiency has been described in these patients. In this Randomized Control Trial (RCT) we assessed the effectiveness of oral vitamin D administration after one year of treatment to protect bone density.

Twenty-four survivors of ALL (17 males and 7 females), who had completed their treatment with oral vitamin D supplement, at least 1 yr previously, and Twenty-five (20 males and 5 females) control group were examined with dual energy x-ray absorptiometry of the total body and L2–L4 vertebrae and neck of femur.

Average of BMD (g/cm2) was significantly increased in oral vitamin D supplemented children (p=0.038) but average of Z-score decrease (p=0.006). Osteoporosis in this group was 4.2% and in control group, 40.9%.

Oral vitamin D supplementation to ALL children during 1 year did not show impact on Z-score and BMD (g/cm2). In the other hand osteoporosis decreased in this group.

A higher prevalence of congenital heart defects (CHD) in neuroblastoma patients in general population is reported in some publications, however, some authors did not find such an association. The evidence for this relation comes from the observation that, the neural crest cells accompanies to the development of the heart and neuroblastoma as well.

We prospectively investigated this relation for the first time, in a cohort of 114 patients with CHD. Echocardiographic evaluations were made as two-dimensional morphological examination and the patients with CHD were evaluated with through physical examination especially for the stigmata of neuroblastoma, telecardiogram and abdominal ultrasonography.

In a period of 6 months, 114 children were diagnosed as CHD. Ventricular septal defect (VSD), atrial septal defect (ASD), pulmonary stenosis (PS) and tetrology of Fallot (TOF) were the most common diagnoses. We did not find any evidence for the association between CHD and neuroblastoma.

The possible association between the two entities was generally investigated by retrospective evaluation of echocardiographic studies in neuroblastoma patients. However, according to our preliminary results the presence of CHD is not a predilection for neuroblastoma. Detailed laboratory and radiological studies for neuroblastoma is not necessary in patients with CHD.

Myocardial iron overload is the leading cause of death in patients with beta-thalassemia major.Combined therapy with deferiprone(DFP) and desferrioxamine (DFO)were suggested to be more effective than deferasirox(DFX) for removing heart iron. Deferasirox has recently been made available but its long-term efficacy on cardiac function has not yet been established. Our study aimed to compare the effectiveness of deferiproneanddesferrioxaminewith deferasiroxon ventricular function in thalassemia major patients.

In this clinical trialstudy,72thalassemia major (TM) patients were randomised to receive either deferipronecombined with desferrioxamineanddeferasirox, and thencardiac function were evaluated. Data were analysed for left ventricular ejection fractions(LVEF)at baselinebyechocardiograpy, following 12 months of treatment.

72 TM patientswere enrolled in this study lasting 12 months,36 TMwere placed on DFP/DFO (DFP,50–86 mg/kg body weight; DFO, 24–52 mg/kg body weigh),36received DFX(range 18–40 mg/kg body weight).In 36 patients receiving combined therapy, left ventricular ejection fractionincreased from 59.3+/-5.7% to 63.7+/-5.1% (p=0.001) over 12 months [baseline LVEF values 56-61%]. deferasirox showed no change in LVEF (p = 0.93).We found improvement of left ventricular ejection fractionsin the deferiprone combined with desferrioxamine versus the deferasirox group (P = 0.008).

The patients treated with combined therapy with deferiprone and desferrioxamineshowed better systolic ventricular function compared to the patients treated with deferasirox.The patients treated with combined therapy with deferiprone and desferrioxamine showed better systolic ventricular function compared to the patients treated with deferasirox.

Polymorphism A1298C of the methylenetetrahydrofolate-reductase (MTHFR) gene has been implicated in spontaneous abortion. In this study, we determined the allele and genotype frequencies of this polymorphism in recurrent spontaneous abortion (RSA) and implantation failure after in vitro fertilization (IVF).

We performed a case–control study on 60 women with RSA and 72 women with implantation failure after IVF (both of group have a problem in embryo implantation so, each other compare to health group) and 60 fertile women to investigate the association between MTHFR A1298G, and pregnancy loss by polymerase chain reaction restriction fragment length polymorphism (PCR-RLFP) technique.

Among the RSA patients 29 (72.5%) were heterozygote and 7 (17.5%) of them were homozygote for MTHFR mutation. In addition, 46 (63.9%) of IVF failure patients were heterozygote and the frequency of homozygote was 17 (23.6%). While in the control group 28 (56.0%) were heterozygote but none of them were homozygote. So the mutation rate of MTHFR in patients with abortion was statistically different from that in controls. Also significant difference was found in the frequencies of MTHFR between the patients and IVF failure group (p <0.001).

Our study revealed that the genotypes of MTHFR A1298C were significantly associated with increased risk of implantation failure of abortion and IVF failure.

Pain is described as the fifth vital sign, and inadequate pain management is linked to numerous immediate and long-term negative outcomes. Venipuncture is one of the most painful medical procedures in children. Distraction is one of the main effective ways to relieve pain. Reducing patients’ pain sensation maybe feeling is important for all nurses for many reasons. Unnecessary pain can damage the nurse-patient relationship, whereas the knowledge of alternative techniques can improve patient care and satisfaction.

Forty patients (6–12 years) suffering from thalassemia and requiring venipuncture were randomized into distraction group (n=20, regular breathing exercise) and control group (n=20, without any intervention). The pediatric pain behavioral symptoms and Numeric Pain Rating Scale were used to assess pain caused by venipuncture.

The mean of pain score based on the numerical scale was 5.60 ± 3.13 in the control group and 1.85±1.42 in breathing exercises and the mean score of behavioral pain symptoms was 3.80±2.80 in the control group and 0.96±0.75 in breathing exercise group. Results showed a significant difference between the mean of pain scores (based on numeric scale and pain behavior scale). (p≤0.001) Conclusion& Distraction: demonstrated to be effective in reducing pain. This intervention requires minimum effort and time and may be a cost-effective and convenient nursing intervention that could be used easily in clinical settings.

Hypothyroidism usually appears in the second decade of life and is thought to be associated with iron overload in patients with thalassemia major. This study aimed to evaluate thyroid dysfunctions in patients with beta-thalassemia major. This research is a descriptive – cross sectional study, carried out in 2009 to assess thyroid function in 100 patients with beta thalassemia major at the ages between 2-18 years. The study was carried out retrospectively and 100 medical records from 400 samples of thalassemia major patients, under regular care of Sarvar Clinic, were assessed. Thyroid function and iron load status were evaluated by measuring the serum total triiodothyronine (T4), thyroid-stimulating hormone (TSH) and ferritin levels from the serum of patients, admitted to the Sarvar Clinic. TSH and T4 concentrations were estimated by enzyme-linked immunosorbent assay (ELISA). Primary hypothyroidism was defined by a TSH level >4µIU/ml. Results were analyzed by descriptive statistical methods, with the help of SPSS software. Subclinical hypothyroidism was seen in 7% patients. All of them had normal T4 levels with elevated TSH levels, consistent with a diagnosis of subclinical hypothyroidism. Mean age of hypothyroid patients was 10.2 ± 2.5 years. Frequency of hypothyroidism was associated with increased serum ferritin levels (p=0.037). Subclinical hypothyroidism occurs in a significant proportion of thalassaemia major patients in the absence of obvious clinical signs of hypothyroidism. Regular follow-up for early detection and timely treatment of such complications could improve the quality of life of these patients.

In Acute Myeloid Leukemia (AML), malignant clones of immature myeloid cells (primarily blasts) proliferate, replace bone marrow, circulate in blood and invade other tissues. The unique presentation of unilateral ptosis and unilateral eyelid swelling in AML has been reported during the present study. Case Report: A 7-year-old girl was encountered with progressively increasing unilateral right eyelid swelling and ipsilateral ptosis. There was no systemic manifestation of leukemia. Computed tomographic image reported right orbit anteromedial mass. A high total leucocytic count in cell blood count with low platelet, the bone marrow aspirate and biopsy showing leukemic blast cells confirmed the diagnosis of AML. After diagnosis, an orbital MRI was done, which revealed a mass lesion, antromedial of right orbit, related to soft-tissue tumor. The patient was treated by chemotherapeutic drugs associated with intravenous steroids.

Unilateral ptosis and eyelid swelling is an initial manifestation of AML that has not been reported previously. A peripheral blood smear with bone marrow aspirate and biopsy helps in the early detection of AML. We should consider AML in any orbital mass with unknown origin.