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In the present research, we reported an acute myeloid leukemia subject. The patient was treated with Cytarabine 200 mg/m² per day with continuous IV infusion on days 1, 2, 3, 4, Etoposide 100 mg/m² per day with continuous IV infusion on days 1, 2, 3, 4, Idarubicin 12 mg/m² as a 4-hour IV infusion on days 2, 4, 6, and Mitoxantrone 10 mg/m2 as a 30-minute IV infusion on days 1, 2, 3. During treatment, the patient suffered from 3 life-threatening complications caused by chemotherapy drugs include sinusoidal obstruction syndrome (SOS), refractory thrombocytopenia, and pericardial effusion with pericardiocentesis limitation. To this end, we tried three novel approaches for patient management by Corticosteroid and Colchicine. Intravenous immunoglobulin infusion and oral prednisolone (2 mg/kg/d) were not effective in thrombocytopenia. However, eltrombopag (25 mg/kg/d) response was dramatic. Moreover, pericardial effusion and respiratory distress during the first chemotherapy course were managed with Lasix (2 mg/kg/d) and Spironolactone (25 mg/d); meanwhile, the massive pericardial effusion during the second chemotherapy course was managed with Colchicine (0.5 mg/d) through the mentioned treatment. Fortunately, these approaches were effective and life-saving. [ABSTRACT FROM AUTHOR]

Thalassemia syndromes are the most prevalent monogenic hemoglobinopathy in the world. In Iran, thalassemia is a public health problem because this country has been located on the thalassemia belt. In recent decades, considering that the life expectancy of patients with thalassemia has dramatically improved, some unrecognized complications have emerged in these individuals. One of these complications is a hypercoagulable state that may lead to thromboembolic events (TEE). The TEE may involve any organ in the body, including the central nervous system. Ischemic cerebrovascular events in thalassemic patients have been divided into two categories, namely overt stroke and silent cerebral infarcts (SCI). Overt stroke often develops in patients with beta-thalassemia major; however, patients with thalassemia intermedia usually suffer from SCI. This review article discusses brain vascular involvement. [ABSTRACT FROM AUTHOR]

Cardiac disease is the main cause of death in both forms of thalassemia; thalassemia major (TM) and thalassemia intermedia (TI). Pulmonary hypertension (PH) is one of the cardiopulmonary morbidities with high mortality that, if not treated, may trigger right-sided heart failure and premature death. PH is defined as a mean pulmonary artery pressure of =25 mmHg at rest or =30 mmHg during exercise. The prevalence of PH is known to be higher in TI than in TM. Moreover, the pathophysiology of PH in thalassemia appears to be sophisticated and complex. Risk factors for occurrence of PH consists of non-transfusion dependent thalassemia (NTDT), sub-optimally transfused transfusion dependent thalassemia (TDT), splenectomy, thrombocytosis, anemia, NRBC = 300 × 106, iron accumulation, history of thrombosis and older age. Other parameters which aggravate the risk of PH include hemolysis, oxidative stress, hypoxemia, alteration of erythrocyte membrane, decline of nitric oxide biological availability, arginine abnormal regulation and arginase excess. The screening method for PH is Doppler echocardiography but the gold standard for detection of PH is right heart catheterization (RHC). Current medical therapeutic options in PH comprise hydroxyurea, L-Carnitine, sildenafil, calcium channel antagonists, endothelin 1-receptor blockers and prostacyclin agonists. The only curative surgical method for the refractory and severe cases of PH is pulmonary endarterectomy. In this article, the etiology, pathophysiology, diagnostic methods and novel therapies of thalassemia associated PH are discussed. [ABSTRACT FROM AUTHOR]

Neuroblastoma is an extracranial solid tumor that is most commonly observed in children, yet it is rare to have brain metastasis in neuroblastoma during primary involvement or relapse. In this article, we report a successful treatment of high risk neuroblastoma with primary leptomeningeal metastasis based on developing countries facilities and explain combination chemotherapy (a classic regimen with salvage regimen based on temozolamid and methotroxate intratechal chemotherapy and localized radiation therapy) as a multimodal therapy. [ABSTRACT FROM AUTHOR]

In the recent decades, the survival of children with cancer has improved dramatically. This improvement is due to use of different chemotherapeutic agents, but the majority of these drugs have kidney toxicities. Deterioration of renal function due to antineoplastic agents is the main limiting factor for administration of these drugs. Chemotherapeutic agents could result in an acute or chronic injury in glomeruli or tubules. Overall childhood cancer survivors have a nine-fold higher risk of developing kidney function impairment when compared with their sibling. In this review, the nephrotoxicity due to some chemotherapeutic drugs such as platinum agents, ifosfamide, methotrexate(MTX), doxorubicin, gemcitabine and imatinib have been discussed. [ABSTRACT FROM AUTHOR]

Improved survival among transfusion dependent thalassemia patients in recent years has led to the manifestation of morbidities such as renal dysfunction. Renal injury is still an underestimated complication in ß thalassemia major patients. Chronic anemia, iron overload due to repeated transfusion, and specific iron chelators are the main factors in pathogenesis of renal dysfunction in ß thalassemia. Early identification of this morbidity allows us to delay the progression of kidney damage and therefore reduce renal impairment. In recent decades, novel biomarkers for early recognition of renal dysfunction have been studied in thalassemic patients, such as cystatin C, beta 2 microglobulin, alpha 1 microglobulin, N-acetyl beta-D-glucosaminidase (NAG), neutrophil gelatinase associated lipocaline (NGAL), kidney injury molecule 1 (KIM-1), liver type fatty acid binding protein (LFABP), and retinol binding protein (RBP). In this review, renal aspects of thalassemia with focus on novel biomarkers were discussed. [ABSTRACT FROM AUTHOR]

Sickle cell disease (SCD) is a common hemoglobinopathy in the world with progressive multi organ failure. Many organs have been affected in this disease, but sickle cell nephropathy (SCN) is a major complication which affects the quality of life in these patients. SCN has a wide range of renal manifestations such as asymptomatic microalbuminuria, hyposthenuria, hematuria, frank proteinuria, nephrotic syndrome and end stage renal disease (ESRD). Nowadays novel biomarkers such as N-acetyl-beta-D-glucosaminidase (NAG), kidney injury molecule-1 (KIM-1) and transforming growth factor ß (TGF-ß) has allowed the early detection of the kidney involvement in sickle cell disease. The angiotensin converting enzyme (ACE) inhibitor drugs such as captopril or enalapril and also ACE receptor blockers (losartan) have beneficial effects in albuminuria or proteinuria. Also endothelin-1 (ET-1) receptor antagonists have a promising role in glomerular injury in SCD. In sickle patients who develop ESRD, renal replacement therapy can be life- saving. In recent years, kidney transplantation is the only curative treatment for advanced chronic kidney disease in these patients. [ABSTRACT FROM AUTHOR]

Background: Hepatic tumors are rare in children comprising only 1% - 4 % of all childhood solid tumors. Primary sarcomas of the liver are extremely rare and represent approximately 0.2 % of all liver tumors. Undifferentiated embryonal sarcoma of liver is an aggressive tumor with an unfavorable prognosis. Here we reported a case of undifferentiated embryonal sarcoma of liver in a 9-year-old boy who was misdiagnosed as hydatid cyst of liver. Case Report: An 8-year-old male patient was referred to our hospital following unsuccessful surgery for presumed hydatid disease of the right lobe of liver. Computed tomography (CT) and ultrasonography showed a large hepatic mass. Following the surgery, our patient received adjuvant chemotherapy. At the time of writing, it has been one year after the termination of adjuvant chemotherapy. Follow up imaging studies, including ultrasonography, CT scan, and MRI show no evidence of recurrence. Conclusion: Here we reported a case of undifferentiated embryonal sarcoma of liver in a 9-year-old boy mistaken for hydatid cyst of liver. Based on our observation, undifferentiated embryonal sarcoma of liver should be considered as a differential diagnosis among patients suspected of hydatid cyst of liver. [ABSTRACT FROM AUTHOR]

Background: The present study was conducted to determine the utility of Score for Neonatal Acute Physiology II (SNAP II) and Score for Neonatal Acute Physiology with Perinatal Extension II (SNAPPE II) scoring systems as predictors of neonatal mortality rate, and to compare the predictive value of these two methods. Methods: In this prospective study data were gathered from infants admitted to the neonatal intensive care unit (NICU) of Imam Hossein Medical Center, Tehran, Iran, from March 2015 to December 2015. In addition to demographic data, Apgar score at 5 minutes after birth, initial and final diagnosis, SNAP II, and SNAPPE II were recorded within 24 hours after admission to the NICU. Results: One hundred ninety-one newborn infants entered into the study. Birth weight (2555 ± 722 g in survival group versus 1588 ± 860 g in expired group, P < 0.001), and Apgar score more than 7 at 5 minutes after birth (99.4% in survival group versus 57.1% in expired group, P < 0.001) were significantly related to the mortality rate. By analyzing the data using logistic regression, it was found that SNAP II (area under the curve [AUC] = 0.992; 95% CI: 0.98-1) and SNAPPE II (AUC = 0.994; 95% CI: 0.984-1) had better value for predicting the patients' survival compared to Apgar score at 5 minutes after birth (AUC = 0.711; 95% CI: 0.568-0.855). There was no statistically significant difference in predictive value of SNAP II and SNAPPE II methods (P > 0.99). Conclusion: According to our findings SNAP II and SNAPPE II are useful tools in predicting the mortality rate among Iranian neonates admitted to NICU. Although there was no significant difference between SNAP II and SNAPPE II, both methods had a much better predictive value compared to Apgar score at 5 minutes after birth. [ABSTRACT FROM AUTHOR]

Background: In recent years, the success in management of thalassemic patients, has allowed for some previously unrecognized complications including renal abnormalities to emerge. This prospective study aimed to investigate kidney iron overload by means of MRI T2* and also renal function based on laboratory tests for early markers of glomerular and tubular dysfunction among adult Iranian transfusion-dependent thalassemia major patients. Subjects and Methods: Two-hundred and two patients with transfusion-dependent ß-thalassemia major were included in this study in Zafar Adult Thalassemia Center, Tehran, Iran. For all patients, kidney MRI T2* as well as evaluation of BUN, creatinine, uric acid, calcium, phosphorus, sodium (Na), potassium (K), total protein, albumin, cystatin C, serum ferritin ß2-microglobulin, NAG (N-acetyl-beta-D-Glucosaminidase), and urine protein were performed. Results: One-hundred and fourteen female and 88 male transfusion-dependent ß-thalassemia major patients with mean age of 30.1 ± 9.4 participated in the present study. We found that 77.7% of our patients had kidney hemosiderosis based on MRI T2*. Also, 67 patients (33.2%) had elevation of serum cystatin C, and 104 patients (51.5%) had reduced estimated glomerular filtration rate (e-GFR). Increased urinary excretion of NAG and hypercalciuria were found in 50% and 79.2% of participants, respectively. Conclusion: Renal hemosiderosis and asymptomatic renal dysfunction are prevalent among transfusiondependent ß-thalassemia major patients which necessitate regular screening with early markers of glomerular and tubular dysfunction. Further studies in order to investigate the correlation between renal hemosiderosis and early markers of kidney dysfunction among these patients are recommended. [ABSTRACT FROM AUTHOR]

Introduction: To evaluate the accuracy of transcutaneous bilirubin measurement in a large population of newborn infants, before and during the phototherapy. Patients and Methods: Asingle Bilicheck instrument was used for transcutaneous measurements. A photo-opaque patch was positioned over the measurement site prior to starting phototherapy. Transcutaneous bilirubinometry was conducted on an unpatched area of the forehead skin and on the nearby site covered by the photo-opaque patch. Readings were obtained from patched and unpatched areas and simultaneous total serum bilirubin concentrations were compared. Results: We studied 134 term and 36 preterm newborns. Pre-phototherapy measurements showed a strong correlation (r: 0.929, P < 0.001, Limit of agreement: -1.8 to 3.1) between Bilicheck and serum bilirubin readings. Post-phototherapy correlation between Bilicheck and serum bilirubin readings was (r: 0.921, P < 0.001, LOA: -1.8 to 2.8) among term and (r: 0.887, P = 0.001, LOA: -1.4 to 2.7) among preterm neonates in patched areas. These correlations were (r: 0.666, P < 0.001, LOA: -1.7 to 7.3) among term and (r: 0.756, P < 0.001, LOA: -0.5 to 5.3) preterm neonates post-phototherapy in unpatched areas. Conclusion: BiliCheck can be safely used for the evaluation of bilirubin levels in preterm and term newborn infants under phototherapy. BiliCheck is slightly less reliable among preterm newborns. [ABSTRACT FROM AUTHOR]

Introduction: Major thalassemic patients need lifelong transfusions. The consequence of these repeated transfusions is iron accumulation in different organs. The main aim of the present study was to investigate the correlation between heart, liver and pancreas hemosiderosis in thalassemic patients from Iran. Methods: This cross-sectional study was conducted on 164 major thalassemia patients at Zafar Adult Thalassemia Center, a referral thalassemia center in Tehran, Iran, from May to November 2014. All patients were on regular blood transfusion at 2-4 week intervals to keep their hemoglobin at a level of 7-9 gr/dL before each transfusion. Demographic data were gathered from patients' history. MRI T2* of liver, heart and pancreas were performed for all patients. Results: There was a moderate correlation between pancreatic T2* and cardiac T2* relaxation times (r = 0.42, P < 0.001), a weak to moderate correlation between T2* of pancreas and liver (r = 0.41, P < 0.001), and a weak correlation between T2* relaxation times of heart and liver (r = 0.31, P< 0.001). Conclusion: Poor correlation between liver and heart, as well as a weak to moderate correlation between pancreas and liver T2* relaxation times indicate that relying on liver MRI T2* to predict the exact condition of pancreas or heart iron overload might not be a reliable approach in thalassemia major patients. Our findings suggest the advantage of using pancreas and heart MRI T2* as a non-invasive method for estimation of iron overload instead of relying on liver MRI T2*. [ABSTRACT FROM AUTHOR]

Background: Proteus syndrome is a very rare condition with less than 100 confirmed cases reported worldwide. We report a case of Proteus syndrome in a two-year-old male who has hemophilia A comorbidity. Case Presentation: A two-year-old male patient was admitted with the chief complaint of severe bleeding in mouth cavity after trauma for two weeks. At admission he was found to have petechiae on buccal mucosa and fecal discoloration due to GI bleeding. We noted multiple abnormalities in his musculoskeletal system and skin. He had lymph edema in left leg, hemihypertrophy, macrodactyly in both foots and macrocephaly. With the history of severe bleeding and recurrent blood product transfusion, we suspected a hemorrhagic disorder. The reduced level of Factor VIII activity confirmed the diagnosis of hemophilia A. Considering patient's various musculoskeletal abnormalities according to the diagnostic criteria and after ruling out similar disorders the diagnosis of Proteus syndrome was established. Conclusion: Because of the variability of clinical features, Proteus syndrome can be confused with other disorders of multiple tissue overgrowth. Our case of Proteus syndrome, who had hemophilia A comorbidity outlines the challenges in diagnosis of such rare combination of diseases. [ABSTRACT FROM AUTHOR]

Objective: Acute seizure attack is a stressful experience both for health care personnel and parents. These attacks might cause morbidity and mortality among patients, so reliable methods to control the seizure preferably at home should be developed. This study was performed to measure the time needed to control seizure attacks using intranasal midazolam compared to the common treatment (intravenous diazepam) and to evaluate its probable side effects. " Methods: This study was conducted as a not blind randomized clinical trial among 60 patients coming to™ Imam Ali Hospital, Zahedan, Iran. The patients were 2 months to 15 years old children coming to our emergency department suffering from an acute seizure episode. Intranasal midazolam was administered 0.2 mg/kg equally dropped in both nostrils for case group and intravenous diazepam was administered 0.3mg/kg via IV line for control group. After both treatments the time needed to control the seizure was registered by the practitioner. Pulse rate and Ch saturation were recorded at patients' entrance and in minutes 5 and 10 after drug administration. Findings: The time needed to control seizure using intranasal midazolam (3.16±1.24) was statistically shorter than intravenous diazepam (6.42+2.59] if the time needed to establish IV line in patients treated by intravenous diazepam is taken into account (P<0.001). The readings for O2 saturation or heart rate did not indicate a statistically significant difference between two groups of patients either at entrance or 5 and 10 minutes after drug administration. Conclusion: Considering the shorter time needed to control acute seizure episodes compared to intravenous diazepam and its safety record, intranasal midazolam seems to be a good candidate to replace diazepam, as the drug of choice, in controlling this condition. [ABSTRACT FROM AUTHOR]

Background: Improved survival in thalassemic patients has lead to the manifestation of morbidities such as renal dysfunction. This involvement suggests the need for a reliable and non-invasive method to assess the degree of kidney iron overload. We conducted the present study to evaluate the relationship between serum ferritin levels, liver, heart, and kidney MRI gradient echo (T2*) relaxation times in thalassemic patients, as a step to evaluate the feasibility of using MRI T2* to assess the degree of kidney iron overload. Methods: This was a prospective study of 120 (60 males, 60 females) regularly transfused thalassemic patients (mean age: 25.9 ± 9 years) who suffered from major and intermediate thalassemia. Patients attended an adult thalassemia clinic located in Tehran, Iran. Cardiac, hepatic and renal MRI T2* were performed. Serum ferritin levels were measured. Results: Our results indicated a moderate correlation between kidney MRI T2* relaxation time and serum ferritin (r = -0.446, P < 0.001). Kidney MRI T2* relaxation time weakly correlated with liver MRI T2* relaxation time (r = 0.388, P < 0.001) and cardiac MRI T2* relaxation time (r = 0.338, P = 0.023). Discussion: The moderate correlation between kidney MRI T2* relaxation time and serum ferritin, and its weak correlation with liver and heart T2* relaxation times indicate that relying on liver and heart MRI T2*, as well as serum ferritin levels to predict the exact condition of kidney iron overload might not be a reliable approach. Our findings suggest the use of kidney MRI T2* as a noninvasive method for evaluating renal iron overload in thalassemic patients. Further studies to investigate the relation between kidney MRI T2* relaxation times and renal function, as well as the cost benefit of using this method, are suggested. [ABSTRACT FROM AUTHOR]

Background: In recent years, the success in management of thalassemic patients, has allowed for some previously unrecognized complications including renal abnormalities to emerge. This prospective study aimed to investigate kidney iron overload by means of MRI T2* and also renal function based on laboratory tests for early markers of glomerular and tubular dysfunction among adult Iranian transfusion-dependent thalassemia major patients. Subjects and Methods: Two-hundred and two patients with transfusion-dependent β-thalassemia major were included in this study in Zafar Adult Thalassemia Center, Tehran, Iran. For all patients, kidney MRI T2* as well as evaluation of BUN, creatinine, uric acid, calcium, phosphorus, sodium (Na), potassium (K), total protein, albumin, cystatin C, serum ferritin β2-microglobulin, NAG (N-acetyl-beta-D-Glucosaminidase), and urine protein were performed. Results: One-hundred and fourteen female and 88 male transfusion-dependent β-thalassemia major patients with mean age of 30.1 ± 9.4 participated in the present study. We found that 77.7% of our patients had kidney hemosiderosis based on MRI T2*. Also, 67 patients (33.2%) had elevation of serum cystatin C, and 104 patients (51.5%) had reduced estimated glomerular filtration rate (e-GFR). Increased urinary excretion of NAG and hypercalciuria were found in 50% and 79.2% of participants, respectively. Conclusion: Renal hemosiderosis and asymptomatic renal dysfunction are prevalent among transfusion- dependent β-thalassemia major patients which necessitate regular screening with early markers of glomerular and tubular dysfunction. Further studies in order to investigate the correlation between renal hemosiderosis and early markers of kidney dysfunction among these patients are recommended.

Introduction: Major thalassemia patients need lifelong transfusions. The consequence of these repeated transfusions is iron accumulation in different organs. The main aim of the present study was to investigate the correlation between heart, liver and pancreas hemosiderosis in thalassemic patients from Iran., Methods: This cross-sectional study was conducted on 164 major thalassemia patients at Zafar Adult Thalassemia Center, a referral thalassemia center in Tehran, Iran, from May to November 2014.  All patients were on regular blood transfusion at 2-4 week intervals to keep their hemoglobin at a level of 7-9 gr/dL before each transfusion. Demographic data were gathered from patients' history. MRI T2* of liver, heart and pancreas were performed for all patients., Results: There were a moderate correlation between pancreatic T2* and cardiac T2* relaxation times (r = 0.42, P < 0.001), a moderate correlation between T2* of pancreas and liver (r = 0.41, P < 0.001), and a weak correlation between T2* relaxation times of heart and liver (r = 0.31, P < 0.001)., Conclusion: Poor correlation between liver and heart, as well as a weak to moderate correlation between pancreas and liver T2* relaxation times indicate that relying on liver MRI T2* to predict the exact condition of pancreas or heart iron overload might not be a reliable approach in thalassemia major patients. Our findings suggest the advantage of using pancreas and heart MRI T2* as a non-invasive method for estimation of iron overload instead of relying on liver MRI T2*.

Purpose: To investigate the efficiency of partial radiofrequency ablation of the spleen in patients with thalassemia major and intermedia., Materials and Methods: Partial radiofrequency ablation of the spleen was performed in 19 thalassemic patients (10 females, nine males) with a mean age of 12.7 years (age range, 7-30 years). This group of patients consisted of 11 patients with thalassemia major and nine with thalassemia intermedia. The procedure was performed under intravenous sedation and was complete in 29-35 min., Results: The ratio of the ablated volume to the whole spleen ranged from 5.3% to 23% (mean, 9.83 ± 5.56%). A significant increase was found in the platelet count after radiofrequency ablation of the spleen (P = 0.002). No statistically significant difference was found in hemoglobin levels (P = 0.171) or transfusion intervals (P = 0.054) before and after radiofrequency ablation. Additionally, no statistically significant relationship was observed between the ablation ratio and hemoglobin levels (P = 0.233) and between the ablation ratio and transfusion interval (P = 0.822). No major complication occurred due to this interventional procedure., Conclusion: A single percutaneous radiofrequency ablation of the spleen reduces thrombocytopenia in thalassemic patients with splenomegaly but does not change the hemoglobin levels or transfusion intervals.