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Background: The present study was carried out on the human population of Kashmir valley to evaluate the status of the biochemical parameters of infected population.

Materials and Methods: Blood samples were collected from 514 individuals, 298 (57.97%) males and 216 (42.02%) females, 187 (36.38%) were found sero-positive for human toxocariasis.

Results: Alkaline phosphatase level was found higher in infected children and adults than in uninfected population. Serum bilirubin concentration was not affected by Toxocara infection. The mean value of serum creatinine in uninfected persons was similar to that of infected persons. There was no effect of Toxocara infection on blood urea level in infected persons. The mean value of blood urea in uninfected persons and infected persons was normal. The blood glucose level was not affected by Toxocara infection.

Conclusion: From above results alkaline phosphatase level was only found to be affected in Toxocara infection.

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This study was undertaken to assess the frequency of decreased bone mineral density and its risk factors as well as its impact on the quality of life during childhood among hemophiliac patients. Materials and Methods: Thirty seven children with severe hemophilia A and B, referred to Mofid Children’s Hospital during 2010, were selected. For all patients the joint score, body mass indexes, bone mineral density, the level of inhibitor antibodies were measured. Short forms of Haeamo-QoL questionnaire were used to assess their quality of life. Data were statistically analyzed using Kolmogorov-Smirnov Z, Mann-Whitney, T-test, Fisher’s eact test, and χ² test. Results: In this study the overall prevalence of low bone density was 35%. Factors that were significantly associated with the frequency and severity of decreased bone density were age, presence of inhibitor antibodies, and reduced joint range of motion. Total quality of life score, and the sub scores of “viewpoint” and “others” as well as the “attitude” were decreased significantly in patients with decreased bone density. Conclusion: According to our findings there is a high prevalence of low bone density among hemophiliac patients. The body mass index should be maintained by appropriate nutrition and exercise to prevent loss of bone density in patients with hemophilia. Prophylaxis regimen in early childhood and regular monitoring of inhibitor antibody development are advised for early detection and management of this complication. Key words: Hemophilia, antibody, body mass index, bone mineral density, quality of life

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Background: Hb A2 is elevated in subjects with beta thalassemia minor but small percent of carriers have normal Hb A2 with elevated levels of HbF (2-10%). This type of thalassemia is called delta beta thalassemia, and can be missed in pre-marriage hematologic consults or screening which leads to increased risk of child birth with beta thalassemia major. Materials and Methods: In this prospective descriptive study, 17768 subjects participated from January 2007 to January 2008. Complete blood count was checked for subjects. If MCV was below 80 fl or MCH was below 27pg, HbA2 was checked with column chromatography. If HbA2 was higher than 3.4 %, subject was labelled as beta thalassemia minor. If HbA2 was normal, 45 days of iron therapy were started for patients whom serum iron, serum ferritin and total iron-binding capacity were in favour of iron deficiency anemia. CBC was rechecked after iron therapy and if MCV or MCH still was below normal range, HbF was checked using alkaline denaturation method as well as Hb electrophoresis. Patients with HbF in the range of 2-10% were diagnosed as delta beta thalassemia minor. Results: 17768 subjects were recruited in this study and 1326 subjects (7.4%) were diagnosed as beta thalassemia minor. 1133 of 1326 thalassemia minor subjects (85.4%) had MCV below 70 fl and 193 subjects (14.6%) had MCV between 70 fl and 80 fl. Three subjects from 17768 (0.01%) had normal HbA2 with HbF between 2% and 10% (delta beta thalassemia). Conclusion: It is very important to use Hb electrophoresis and check HbF by alkaline denaturation method for all people coming for pre-marriage hematologic consultation to detect delta beta thalassemia or other hemoglobinopathies. Keywords: Delta-Beta Thalassemia, screening, consultation, Iran.

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Autoimmune lymphoproliferative Syndrome (ALPS) is a rare inherited disorder of apoptosis. It usually presents with chronic lymphadenopathy, splenomegaly, and symptomatic cytopenia in a child. Herein, we report a 14-year-old boy with symptoms misdiagnosed as hemophagocytic lymphohistiocytosis who was treated before ALPS was diagnosed for the patient. This case should alert pediatricians to consider ALPS in differential diagnosis of a child with lymphadenopathy, splenomegaly, and cytopenia.

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Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is one of the most known enzyme defects in Iran with various genetic mutations. We aimed to study the predisposing factors of hemolysis in children with G6PD deficiency.
Methods: This study was done during 2007-2012 in two referral centers of Mofid Children’s Hospital and Baqiyatallah Hospital, Tehran, Iran. The hospital records of the patients were fully reviewed and questionnaires for each patient were filled for the date of admission, initial symptoms, initial laboratory results, family history and history of any drug consumption, infection or fava bean ingestion. 
Results: Medical records of 192 children with mean age of 4.2 years (1 month to 14 years) were extracted. 68.2% of the cases were male. Hemolytic crises were significantly more common in spring which is the peak time for fava bean consumption and occurred more frequently in those with a family history of G6PD deficiency especially in females. The most common initial symptoms were jaundice (71%), dark color urine (49%), fever (34.4%), and pallor (24.5%), followed by abdominal pain (16.7%). Fava bean intake (93%) was the first etiological agent triggering hemolysis followed by infectious agents and drug consumption. Initial hemoglobin level was significantly lower in male patients.
Conclusion: Regarding the high prevalence of G6PD deficiency in Iran, we should emphasize on education of parents and physicians about the disease and prevention of fava bean ingestion in people with G6PD deficiency.

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Macrophage activation syndrome (MAS) is a rare feature of rheumatic disorders in children and adolescence and its presentation as the first symptom of rheumatic disorders is very infrequent.
A 9-year-old girl, in whom MAS developed, was admitted to our Hospital in Tehran, Iran. She suffered from high grade fever and rash followed by multiple joint swelling months afterwards. Bone marrow aspiration and biopsy showed normocellular marrow with a cellularity of 90%. Benign-looking macrophages were remarkably increased; many of them showed hemophagocytic features. According to the presentation of long-standing fever and observation of “hemophagocytic macrophage” in bone marrow, MAS was diagnosed for the patient. Additionally, due to recurrent joint swelling in following months, she was diagnosed to be affected by “Juvenile Idiopathic Arhtritis” complicated by MAS.
MAS is a rare complication of rheumatic disorders which should be considered as the first presentation of rheumatic disorders in children specifically in those presenting with high fever, hepatosplenomegaly, lymphadenopathy and severe cytopenia. 

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Detection of ABO blood groups through agglutination is the basis of pre-transfusion testing. However, weak agglutination reactions may be obtained with reagent antibodies and are a result of weak expression of A and B antigens on red blood cell surface which may cause a discrepancy in blood group typing. Here, we report a donor which showed discrepancy between red blood cells (forward) and serum (reverse) typing. A detailed analysis revealed the blood type as a variant of blood group B. Subgroup of B is a very rare phenotype of blood. This is the first case ever detected in Kurdistan blood transfusion Center.

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Background: Frequent blood transfusions lead to various complications in patients with thalassemia major. Heart problems caused by iron deposition in the myocardium are the major cause of death in patients with thalassemia major. Early diagnosis of cardiac dysfunction in patients with thalassemia major undergoing frequent blood transfusion is very important. We aimed to investigate the correlation between serum Ferritin level, serum Troponin T (TropT), cardiac T2* MRI and echocardiographic findings in patients with thalassemia major.
Methods: This cross-sectional study was conducted on all children >5 years old with thalassemia major admitted to Amirkabir Hospital, Arak, Iran, during 2016-2017. serum Ferritin level and Troponin T, echocardiography, and cardiac T2*MRI were analyzed in all participants.
Results: We found no correlation between serum ferritin and Trop T levels. However, there was a significant correlation between serum Trop T and serum ferritin with cardiac T2*MRI and also between serum Trop T and echocardiographic parameters (positive correlation with E/A ratio and functional shortening); and a negative correlation with left ventricular ejection fraction (LVEF). Moreover, there was no correlation between cardiac T2* MRI and echocardiographic findings.
Conclusion: Our results showed a positive correlation between serum troponin T with E/A size and FS and a negative correlation with LVEF and it was an important prognostic factor in early stage of cardiac damage in patients with thalassemia major. Moreover, there was a negative correlation between results of T2* MRI and serum ferritin and troponin T.

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Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease with diverse clinical manifestations involving multiple organ systems. Peritoneal involvement as a sign of gastrointestinal disease in SLE is a rare condition. Massive ascites as the initial manifestation of SLE is an extremely rare situation. Here, we report a 13-year-old female with bile-stained vomiting, ascites and signs of pseudo-obstructions with a past medical history of immune thrombocytopenic purpura (ITP) who was later diagnosed to have SLE.

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Hemangiomas are defined as soft tissue lesions in the maxillofacial or oral region. Hemangiomas of salivary glands constitute 30% of the non-epithelial tumors in major salivary glands.  Benign tumors in salivary glands are located 85% in parotid gland and 13% in submandibular gland. We present a case of submandibular hemangioma in an infant patient that had some complications and a challenging diagnosis. A 3- month-old female patient presented a giant hemangioma located in the submandibular, preauricular and right malar region with purplish color that during hospitalization had a cardiorespiratory arrest as a severe complication of the disease.

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Chordomas are rare tumors from primitive notochord. They are usually located at sacrococcygeal region when it affects the spine. We present a case of giant chordoma in the thoracolumbar spine in Peru. A 43-year-old woman came to the hospital in Huanuco due to back pain and sensorymotor alterations in the lower limbs. Imaging confirmed an unresectable tumor involving the spine. Two years later, she came back with paraplegia, weight loss, severe anemia and complicated urinary tract infection. Magnetic resonance imaging confirmed a mass measuring 15×15×14 cm in T11, T12 and L1 which histopathology was consistent with chordoma. Chordoma is a rare tumor which should be included in the differential diagnosis of spinal tumors. An earlier diagnosis leads to more options of treatments and longer survival.

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Background: Cancer is a public health emergency. It has a high mortality rate despite numerous studies on pharmaceutical therapies. Chimeric antigen receptor-natural killer (CAR-NK) cells are promising immunotherapy that could be used to treat cancer, especially leukemia. However, the evidence is still unclear. Thus, this systematic review aims to summarize the evidence regarding the use of CAR-NK cells as a therapy for leukemia.
Method: This systematic review was conducted in accordance with the preferred reporting items for systematic reviews and meta-analysis (PRISMA) statement guidelines. The literature search was done using PubMed, ProQuest, ScienceDirect, and EBSCOHost with “chimeric antigen receptor”, “natural killer cell”, and “leukemia” as the primary keywords until 20 March 2020. Data collection and extraction were done by three independent reviewers. Extending a risk-of-bias approach to address in-vitro studies for assessing the risk of bias was utilized in the quality assessment of the studies.
Results: The search strategy identified 221 studies. Three relevant articles met our inclusion criteria. All the included studies had a low risk of bias. The main findings from available data were as follows: (a) cytotoxicities of CAR-NK cells were found highest in cell lines expressing antigen for CAR (CD19+ cancer cells) (b) CAR-NK cells had low cytotoxicities against cells that didn’t express antigen for CAR (e.g. SR-91); (c) all studies did not result in aberrant growth of the transduced CAR-NK cells.
Conclusion: The use of CAR-NK cells showed promising results in treating leukemia based on its cytotoxicity against CD19+ cancer cell lines.

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Iron overload can adversely affect thyroid and parathyroid function in patients with transfusion-dependent thalassemia. Iron deposition in both glands or the pituitary gland, which controls thyroid function, can lead to their destruction and dysfunction. Hypothyroidism can cause symptoms such as fatigue, weight gain, and depression, while hypoparathyroidism can cause symptoms such as numbness and tingling in the hands and feet, muscle cramps, and seizures. Regular thyroid and parathyroid function monitoring is essential in thalassemia patients to detect any dysfunction early and provide appropriate treatment. Treatment may include medications to replace thyroid hormone or calcium and vitamin D supplements to manage hypoparathyroidism. A comprehensive approach to managing endocrine complications in thalassemia patients can improve outcomes and quality of life for these individuals. To provide professional healthcare members with clear and concise recommendations for diagnosing and treating hypothyroidism and hypoparathyroidism in transfusion dependent thalassemia patients, a practical national guideline should be developed.

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