ACUTE LYMPHOBLASTIC LEUKEMIA WITHOUT CIRCULATING BLASTS PRESENTING AS SEVERE HYPERCALCEMIA

Hypercalcemia complicating malignancy is a rare complication in pediatric age group. In this article, we present a case with acute lymphoblastic leukemia presenting as severe hypercalcemia. A 10 years old girl presented with an acute onset of fever, nausea, vomiting, loss of weight, costovertebral pain and frequency. She was admitted with a presumptive diagnosis of acute pyelonephritis. Her examination showed mild hepatosplenomegaly. In laboratory studies she had sever hypercalcemia. Despite the absence of circulating blast, bone marrow aspiration was diagnostic of acute lymphoblastic leukemia. The hypercalcemia was initially treated with intravenous hydration and furosemide but the serum calcium levels normalized only after the beginning of specific chemotherapy. Hypercalcemia represents an emergency in children, and acute leukemia must be considered in differential diagnosis even when there are no circulating blasts

Prognostic factors in children with acute lymphoblastic leukemia: a ten year study

Background: Acute lymphoblastic leukemia (ALL) is the most common cancer in the pediatric population. With modern treatments, the chance of the complete recovery is nearly 100%. The most important prognostic factors are appropriate treatment protocol and determination of patient risk factors based on clinical, morphological, immunological and cytological characteristics. In this study we reviewed frequency of these factors, like as age, gender, the primary white blood cell number, sub- group on the base of FAB classification, immunophenotype and the clinical progress. Methods: In this retrospective study, we reviewed 877 pediatric patients with the diagnosis of ALL between the years of 1994 and 2004. In these patients the age, gender, primary WBC count, sub-group based on the FAB classification, immunophenotype and the clinical progress in 177 patient with acute lymphoblastic leukemia at Imam Khomeini Hospital between the years of 1994 to 2004 were determined. Results: Of these patients, 1.6% was younger than one year, 24.8% more than ten years old and 73.6% were between the ages of one and ten years; 63.8% were male. WBC counts were above 50,000/ul in 28.8% of the patients. FAB classifications included L1 in 80.2%, L2 in 17.5% and L3 in 2.3% of the patients. Immunophenotypes included pre-B cell in 63.8%, early pre-B cell in 23.1%, T cell in 12.3% and mature B cell in 0.8% of the patients. Marker CD10+ was detected in 88.1% of the B cell cases. In this study group, 74% of the patients recovered, 16.3% died and 16.5% relapsed.Conclusions: The prevalence of FAB-L1 and pre-B cell cases in this study is greater than a previous study, while the prevalence of FAB-L2 and early pre-B cell cases is less than that of the previous study

PREVALENCE OF AUTOANTIBODIES TO THYROID PEROXIDASE AND AUTOIMMUNE THYROID DISEASE IN GIRLS WITH TURNER’S SYNDROME

Patients with Turner’s syndrome (TS) are at an increased risk of developing autoimmune thyroid disease (ATD). The aim of this study was to determine the frequency of anti-thyroid peroxidase (anti-Tpo) antibodies and ATD in children and adolescent girls with TS. It also assessed the influence of karyotype on the development of thyroid disease. Sixty eight patients with TS were compared with 68 age matched healthy unrelated girls in this study. They were screened for anti-Tpo antibodies, free T4 and TSH levels. Sign and symptoms of hypothyroidism and hyperthyroidism and the presence of goiter were also investigated. Anti-Tpo antibodies were found in 18 (26.4%) TS patients and 1 (1.4%) patient in the control group (P < 0.001), evenly distributed between the karyotypes 45X, 46X, isoXq and mosaicism. Out of 68 TS patients, 8 (11.7%) had visible goiter. Subclinical hypothyroidism and hypothyroidism both occurred in 2 patients (5.9%). These patients were characterized by higher levels of anti-Tpo antibodies. Visible goiter was found in 3 (4.4%) subjects of the control group, but all of them were euthyroid. We found that younger patients were more likely to be anti-Tpo negative (P < 0.001). Our data demonstrated a high frequency of ATD in a representative sample of Iranian girls with TS which is in accordance with previous observations. Regular follow up assessment of thyroid autoantibodies and thyroid function in patients with TS is recommended for timely diagnosis of thyroid dysfunction and treatment

Prevalence of dyslipidemia in children and adolescents with diabetes mellitus type I

Methods: 128 patients with type I diabetes attending a single pediatric endocrine clinic underwent anthropometric and biochemical assessment. Anthropometric measurements followed WHO criteria. Blood samples were analyzed for glycated hemoglobin (HbA1C), cholesterol (chol), triglycerides (TG), low density lipoprotein (LDL) and high density lipoprotein (HDL), and blood pressure was recorded. Findings: Patients' mean age was 12.6 ± 4.1 years. Patients' mean age at the onset of diabetes was 7.1 ± 2.8 years. Mean duration of diabetes was 6.9 ± 3.2 years. 48.5% of patients had some form of dyslipidemia. 21.4% had isolated hypertriglyceridemia, 11.6% isolated hypercholesterolemia and 15.5% mixed hyperlipidemia. Factors associated with dyslipidemia included longer duration of diabetes, higher mean age, higher mean HbA1C (p 0.001). Hypertriglyceridemia was more frequent in female patients and subjects with higher BMI (p<0.05). The mean value of TG 199.9 ± 74.1 mg/dl, TC 178.5 ± 29 mg/dl and LDL 141.2± 37 were significantly higher in patients with poor metabolic control (mean value of HbA1C 9.3 ± 1.8) than the diabetic patients with better control (mean value of HbA1C 7.1 ± 0.77), TG 156.8 ± 55.9 mg/dl; TC 143.5 ± 37.6 mg/dl and LDL 108± 21.2. Conclusion: Our findings indicated that type I diabetic patients with poor metabolic control are at higher risk of developing dyslipidemia. However, given the well documented problems of lifestyle regulation and compliance in optimizing control especially in this age group, we need to develop alternative and simple interventional strategies to improve outcome. Monitoring of lipids should be extended and yearly screening of patients for dyslipidemia recommended

Prevalence of hyperuricemia in children with leukemia and lymphoma with respect to prophylactic treatment

Background: Hyperuricemia is one of the oncologic emergency that occurs most often in patients with hematologic disorders particularly leukemia and high-grade lymphoma. This study was conducted in order to determine the prevalence of hyperuricemia with respect to prophylactic treatment (in particular allopurinol) in patients with lymphoproliferative disease in the pediatric hematologic ward of Imam Khomeini Hospital, Tehran. Methods: In this retrospective cross-sectional study, 316 children (75 females, 241 males) under the age of 12 years participated. Among the subjects, 66 patients (20.9%) had lymphoma and 250 patients (79.1%) had leukemia. Results: Of the 56 (17.7%) patients diagnosed with hyperuricemia, 13 with lymphoma (19.7%) and 43 (17.2%) with acute lymphoblastic leukemia, 52 patients showed hyperuricemia after induction of chemotherapy (p&lt;0.001). Hyperuricemia was more prevalent in patients with more advanced disease (50.9% in stage IV, p&lt;0.001). Hyperuricemia was more frequent in male patients (p&lt;0.001). Among the 217 patients who had received prophylaxis (hydration, alkalization, allopurinol), 19 (8.7%) subjects had hyperuricemia compare to 37.3% in the group of patients who did not receive prophylactic treatment (p&lt;0.001). Conclusion: From the literature reviewed, a recombinant form of the urate oxidase enzyme (rasburicase) is a safe and effective alternative to allopurinol to rapidly control plasma uric acid concentrations in patients with hematologic malignancy at high risk for tumor lysis during induction of chemotherapy. In this respect, we recommend a prospective study to compare allopurinol and rasburicase in children with leukemia and lymphoma

Evaluation of the cause and predisposing factors in neonatal mortality by using international coding diseases version 10 in Valiasr Hospital

Background: According to the fact that the reduction of Neonatal Mortality Rate is correspondent to improvements in health and hygienic status of the society, understanding the major neonatal mortality causes will help the society to plan better prenatal and neonatal care systems. Methods: On this retrospective study, the major causes of the mortality of the dead neonates were extracted from files of Vali-Asr hospital according to the International Coding of Disease Ver 10 (ICD10). Data were analyzed in proportion to gestational age , birth weight , gender and neonatal age. Findings: Mortality rate was higher during the first week of life (78%). Generally five main causes of death were; prematurity, respiratory distress syndrome, intra cerebral hemorrhage, multiple congenital anomalies and air leak syndromes. It was also revealed that the causes of death of low birth weight neonates were similar to those of premature neonates (respiratory distress syndrome, prematurity, intra cerebral hemorrhage, septicemia and air leak syndromes). Similarly there was no difference between the causes the of death in mature neonates and appropriate for growth age neonates. The main causes of death in these two groups were; congenital disease, infections (septicemia and Pneumonia), asphyxia, diffuse intra vascular coagulation, intra cerebral hemorrhage, meconium aspiration and complications of pregnancy. Data analysis indicated significant relationships between death and gestational age, neonatal age and birth weight (P=0.001) but it was not related to gender. Conclusions: With due attention to the easy application of ICD 10 for determining the major and underlying causes of death of neonates and the fact that autopsy is not routinely applicable in neonatology wards, routine using of ICD 10 for classifying the causes of death in death certificates is highly recommended

Pediatric septic arthritis: a 10- year epidemiologic study in Imam Khomeini Hospital Complex

&quot;n Normal 0 false false false EN-US X-NONE AR-SA MicrosoftInternetExplorer4 /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0in 5.4pt 0in 5.4pt; mso-para-margin:0in; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:Arial; mso-bidi-theme-font:minor-bidi;} Background: Septic Arthritis is an acute infection of intra-arthicular space. Delay in diagnosis and in appropriate treatment may lead to prolongation of treatment duration and poor outcome. We decided to evaluate clinical aspects of this disease in our department pediatric department, Imam Khomeini Hospital complex, Tehran, Iran during a 10 years period.&quot;n&quot;nMethods: In a retrospective cross-sectional study, 60 patients with age from 1 month to 14 year-old during 1996-2005 were evaluated. The demographics characteristics, clinical observations (signs &amp;amp; symptoms) and paraclinic values were gathered from medical records and analyzed with statistical tests. &quot;n&quot;nResults: Pain and Swelling was seen in all, joint limited motion in 80% (50 cases) claudicating in 64% (38 cases), fever in 80% (48 cases), monoarticular disease in 80% (48 cases) and polyarticular one in 20% (12 cases) of them. Hip was most Common involved joint 62% (37 cases), elevated ESR was seen in all patients. CRP was positive in 85% (51 cases). Leuckocytosis was found in 65/8% (17 cases) of cases. In infectious cases, Staphylococcus aurous was responsible organism in 65.6% (16 cases), klebsiella in 12.4% (3 cases), Streptococcus pneumonia in 12.3% (3 cases), group b streptococcus in 4.1% (1 case), Hemophilus Influenza type b in 4.1% (1 case) of study patients. With therapy ESR was normalized in 64% (39 cases) and CRP was normalized in all patients. &quot;n&quot;nConclusions: In general, all children with complaint of fever, pain, and limited joint motion or claudicating should be suspected for septic arthritis

Clinical heterogeneity and chromosome breakage in Iranian patients suspicious of Fanconi anemia

Background: Fanconi anemia (FA) is a rare autosomal recessive disorder characterized by short stature, skeletal anomalies, increased incidence of solid tumors and leukemia, and bone marrow failure (aplastic anemia). FA has been reported in all races and ethnic groups and affects men and women in an equal proportion. The frequency of FA has been estimated at approximately 1 per 360,000 live births. In some populations, including Ashkenazi Jews, Turks, Saudi Arabians and Iranians, this frequency appears to be higher, probably as a result of the founder effect and consanguineous marriage. Because of extensive genetic and clinical heterogeneity (the age of onset, clinical manifestations and survival), diagnosis of FA on the basis of clinical data alone is unreliable and its molecular diagnosis is difficult. The diagnosis of FA exploits the hypersensitivity of FA lymphocytes and fibroblasts to bifunctional alkylating agents such as mitomycin C (MMC), diepoxybutane (DEB) and nitrogen mustard and differentiates it from idiopathic aplastic anemia. In this study, in addition to the patients&apos; clinical profiles, a cytogenetic test using MMC was implemented for an accurate diagnosis of Fanconi anemia.Methods: In this study, the lymphocytes of 20 patients referred for FA, and those of their normal sex-matched controls, were treated with three different concentrations of mitomycin C (20, 30, 40 ng/ml). Slides were prepared and solid stained. In order to determine the number and kind of chromosome abnormalities, 50 metaphase spreads from each culture were analyzed. Clinical information was obtained from patient files.Results: Five patients manifested increased chromosome breakage with MMC, confirming the FA diagnosis. Two different concentrations of MMC (30, 40 ng/ml) were most effective.Conclusion: The chromosomal breakage test is important for the accurate diagnosis of Fanconi anemia. DNA crosslinking agents used to treat idiopathic aplastic anemia may be lethal for patients with FA. Thus, aplastic anemia patients with unknown etiology, infants with congenital abnormalities involved in FA and siblings of FA patients should also be cytogenetically tested.&quot;n&amp;nbsp