Patient Safety Domains in Primary Healthcare: A Systematic Review.

Background: Healthcare systems should ensure the provision of quality services to patients without harming them. However, the provision of services is occasionally accompanied by harm or complications, most of which are preventable. Most studies have focused on secondary healthcare rather than primary healthcare (PHC). Thus, this study aimed to identify various dimensions and components of patient safety in PHC worldwide. Methods: This systematic review study was conducted in November 2022 based on PRISMA reporting guidelines. Studies were retrieved from PubMed, Scopus, Cochrane Library, Web of Science, and EMBASE and searched for English documents using the keywords "patient safety" and "PHC" from 2000 to 2022. Finally, two reviewers extracted the data independently and analyzed using thematic content analysis. Results: Overall, 23 out of the initially 4937 identified articles were selected for the final analysis based on the inclusion and exclusion criteria. Most of these studies used a qualitative-quantitative approach (61.9%, seven studies for both), and 64% had been conducted in European countries. Eventually, five dimensions and 22 components were identified for patient safety in PHC, including management measures, quality management, resources and technology, documents, and patient-related factors. Conclusion: The patient safety dimensions and components identified in this research can help develop a clear definition of patient safety and its assessment standards and criteria in PHC. Considering that most previous studies on patient safety in PHC were conducted in European and developed countries, it is suggested that researchers conduct more studies in developing countries to fill this research gap.

Delivering Primary Health Care (PHC) Services for Controlling NCDs During the COVID-19 Pandemic: A Scoping Review.

Non-communicable diseases (NCDs) are the leading cause of death worldwide. NCDs also increase mortality from COVID-19 and primary health care (PHC) services are an important component in the prevention and control of long-term NCDs. The main goal of the present study was to review primary healthcare services for the NCDs patients via primary healthcare network during COVID-19 pandemic. In this scoping review, Search engines including PubMed, Scopus, and Science-direct up to 1st February 2022 were searched to identify studies regarding primary care services for NCDs patients via primary health care during COVID-19 pandemic. A total of 42 studies met the inclusion criteria and were included in our analysis. 24 studies were about the status and changes of primary health services for NCDs patients in PHC settings, while 18 studies focused on adaptive strategies used during COVID-19 in different countries including United States, Canada, United Kingdom, Portugal, Georgia, South Africa, Thailand, Mexico, India, Kenya, Guatemala and Saudi Arabia. These strategies included remote monitoring, follow up, consultation, empowerment and educational services as well as home visiting Disruption of NCDs services in PHC during the COVID-19 pandemic was observed in different countries, which highlights the urgency of attention of researchers and policy-makers to development of appropriate and adaptive policies to improve PHC service coverage and its quality during the pandemics.

Examining the care of noncommunicable diseases at the first level of providing services during the COVID-19 pandemic.

Aim: Providing services for patients with noncommunicable diseases is one of the main responsibilities of health systems. During the COVID-19 pandemic, the care of these patients faced problems. This study investigates the ways of providing optimal care to patients during pandemics like COVID-19. Subject and methods: This study was conducted in 2021 in Tehran province using an analytical cross-sectional method. Six hundred participants were selected for the study. In order to examine the challenges and solutions for receiving services, a questionnaire was completed and its reliability and validity were checked; finally, a telephone interview was completed over a period of 3 months. Results: Among study participants, 68.2% were female, and the highest percentage was in the age group of 50-60 years. Fifty-four percent were illiterate or had primary education, 48.8% had diabetes, 42.8% had high blood pressure, and 8.3% had both diseases. Forty-three percent of the interviewees had not used health care services during the COVID-19 pandemic, the main reason for which was the fear of contracting COVID-19. The outbreak of coronavirus disease had affected the care of noncommunicable diseases for 63% of the interviewees. Conclusion: The fundamental need for changes in the health system was revealed by the COVID-19 pandemic. The need for flexibility in the health system will inevitably arise when similar cases occur, and policymakers and managers should consider necessary measures in this regard. The use of new technologies is one of the ways to replace traditional models.

Vitamin D abnormalities of children with recurrence of malignancy and comparison with newly diagnosed patients.

Background: Vitamin D (Vit-D) is a necessary ingredient for human growth and its deficiency may increase the risk of cancer and its recurrence. The main purpose of this research was to assess the levels of Vit-D in children with recurrence of malignancy and compare it with new cases of malignancy and the control group. Methods: The status of 25(OH) Vit-D was determined utilizing the HPLC method in 47 patients with recurrence of malignancy (group A), 50 children with new malignancy (group B) and 49 normal healthy siblings of the two groups as a control (group C). Results: Vit-D was low (<30 ng/ml) in the 92% of patients with recurrence of malignancy, which was a significant difference compared to groups B (60%) and C (45%). Vit-D insufficiency (10-30 ng/dl) in group A was also higher than the other two groups. The mean levels of Vit-D in patients with recurrence were significantly lower than the new cases and controls. Low Vit-D (<30 ng/ml) in group A in both male and female, and also in all ages (<6 and ≥ 6 years) was higher than groups B and C. Also, low Vit-D in terms of the type of malignancy in group A was higher than group B only in leukemic patients while this was not different for non-leukemic patients in these two groups. Conclusion: Results of this study showed an increased prevalence of low Vit-D in children with recurrence of malignancies. Therefore, it may increase the risk of recurrence of malignancies in children.

Spontaneous Regression of Diffuse Large B-cell Lymphoma in a Patient with Ataxia-Telangiectasia.

Ataxia-telangiectasia (AT) is a type of primary immunodeficiency characterized by an autosomal recessive mode of inheritance and usually presents with progressive cerebellar ataxia in early life. This complex disease is associated with humoral and cellular immune dysfunction and other features including characteristic oculocutaneous telangiectasia and increased predisposition to cancers, particularly lymphoma and leukemia. An 11-year-old Iranian girl presented with primary immunodeficiency and was diagnosed as having AT according to her clinical manifestations and molecular findings. She had a history of two types of non-Hodgkin's lymphoma and showed spontaneous regression of her diffuse large B-cell lymphoma without any specific treatment. Gene mutations and dysfunction in patients with AT result in different manifestations including abnormal development of the thymus, immunodeficiency, increased susceptibility to malignancies, and increased radiosensitivity. No standard treatment is available for these patients. The use of immunotherapeutic strategies in patients with primary immune deficiency disease-associated tumors is potentially important.

Therapeutic potential of silymarin as a natural iron-chelating agent in ß-thalassemia intermedia.

Abnormal iron accumulation in vital organs is one of the major complications of ß-thalassemia intermedia (ß-TI). Silymarin, a flavonolignan isolated from Silybum marianum, significantly decreases the serum ferritin levels of ß-TI patients. This finding suggests silymarin as a safe and effective natural iron-chelating agent for the treatment of iron-overloaded conditions.

A comparative study of the status of supportive-palliative care provision in Iran and selected countries: Strengths and weaknesses.

BACKGROUND: Terminally, illnesses such as cancer, AIDS, dementia, and advanced heart disease will require special supportive and palliative care, although a few numbers of these patients are provided with these services. The aim of the present study was to perform a comparative study of supportive-palliative care provision in selected countries. MATERIALS AND METHODS: This research was a descriptive comparative study that its research population was the frameworks of palliative and supportive care provision in Egypt, Turkey, America, Australia, Canada, the Netherlands, and China. These frameworks were compared across six dimensions of service receivers, financing, providers, service provider centers, type of services provided, and training. Data collection tool has included the checklist and information sources, documents, evidence, articles, books, and journals collected through the Internet and organizations related to the health information of selected countries and by the library search. Data were investigated and analyzed using the data collection tool and checklists. FINDINGS: The findings showed that the developed countries having decentralized trusteeship structure had a more favorable status in palliative and supportive care provision. The type of services provided was a combination of mental, psychological, social, spiritual, financial, and physical and communication services. Provider centers included hospital, the elderly, and cancer and charity centers. CONCLUSION: Regarding the investigation and recognition of the status of supportive-palliative care provision, it was observed that the provision of these services was a concern of the selected countries, but they did not have a defined model or pattern to provide these services. Therefore, it is suggested that each country takes a step to redesign and define frameworks and structures in the evolution of supportive-palliative cares in accordance with the particular conditions, indigenous culture, religion, and other effective cases of that country and pays special attention to the role and position of supportive-palliative cares.

Investigating the challenges and barriers of palliative care delivery in Iran and the World: A systematic review study.

Palliative care and terminal patients care centers have an important role in improving the physical and psychological state of the patient and their families and increasing their satisfaction and care providers. A literature search of online databases (PubMed, Scopus, Web of science, Cochrane library, and Google Scholar) was searched from January 1, 2000, to the end of April 2019, by using the appropriate English keywords. Furthermore, IranMedex, Barkat, and Magiran databases were searched for the Persian articles. We used Standards for Reporting Qualitative Research checklist to evaluate the articles quality. From 1328 articles, 166 were reviewed in depth with 13 satisfying our inclusion criteria. The findings of this study revealed a wide range of barriers and challenges to palliative care delivery. The identified barriers were: Cultural, social and organizational barriers, lack of resources, equipment and financing, attitudes and cultures, barriers related to the patient and the patient's family, related barriers providers, time and money, education, communication challenges, policies, insurance problems, safety, and crisis management. The results of the studies showed that there are various barriers and challenges such as economic, cultural, social, organizational, and communication related to palliative care. Given the identified barriers and challenges, it is suggested that to improve the delivery of palliative care, the health system policy-makers and planners consider a resource-appropriate and culturally appropriate framework for palliative care delivery.

Unusual sites of bone involvement in Langerhans cell histiocytosis: a systematic review of the literature.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare disease that originates from the uncontrolled proliferation and accumulation of bone marrow-derived immature myeloid dendritic cells. Dendritic cells are a type of histiocyte that play an important role in the human immune system and are found in the bone, skin, stomach, eyes, intestines, and lungs. OBJECTIVE: This systematic review aimed to collect and report published case reports of rare bone disease caused by LCH to avoid misdiagnoses or delays in diagnosis. METHODS: We systematically searched Scopus, PubMed, Embase, and Web of Sciences from August 1, 2000 to December 31, 2019. Studies reporting cases of LCH with rare bone involvement were included. RESULTS: We identified 60 articles including 64 cases. Of the identified cases, 31 (48.4%) involved children, and 33 (51.6%) involved adults. Additionally, 46.9% (30 individuals) were from Asian countries. The mean age of the children was 7.6 ± 4.3 years and that of the adults was 36 ± 12 years. The findings indicated that unifocal bone involvements were the most prevalent form of the disease (68.7%), and, overall, the skull and chest wall were the most commonly affected bones in both adults and children. The spine and long bones were the second most commonly affected bones in children, and the spine and jaw were the second most commonly affected bones in adults. Pain and swelling were the most frequent presenting signs among the investigated cases, and loss of consciousness, myelopathy, nerve palsy, visual loss, torticollis and clicking sounds were rare signs. Osteolytic lesions were the most frequent radiologic feature (62.5%), and intracranial hemorrhage, fluid-fluid level, dura and intracranial extension and pathologic fractures were rare radiological features. Total excision, curettage and observation in the unifocal group of patients and systemic chemotherapy in the other groups (i.e., multifocal and multisystem) were the most frequent management approaches. The recovery rates of the unifocal and multifocal groups were 77.3% and 81.8%, respectively, while that of the multisystem group was 55.5%. The rates of recurrence and mortality in the multisystem group were 11% and were higher than those in the other groups. CONCLUSIONS: LCH is a rare disease that can affect any organ in the human body. However, bone is the most commonly involved organ, and rare bone involvements may be the first or only symptom of the disease due to the rarity of such lesions; a lack of familiarity with them may result in misdiagnosis or delayed diagnosis.

Bernard-Soulier syndrome or idiopathic thrombocytopenic purpura: A case series.

BACKGROUND: Bernard-Soulier syndrome (BSS) is a rare, autosomal recessive platelet function disorder which is commonly mistaken for idiopathic thrombocytopenic purpura (ITP).The report includes seven cases of BSS that have been diagnosed and treated as ITP for a long time. METHODS: Between 2006 and 2016, data of seven BSS patients who have long been diagnosed and treated as ITP were collected and analyzed. RESULTS: Two patients were males and 5 were females. The patient's age range was between one day and four years at the onset of symptoms. Easy bruising, nose bleeds and mucocutaneous bleeding were the most frequent symptoms. Bleeding attacks of the gum, gastrointestinal tract and menorrhagia also occurred and in one case bleeding in the injection site of the first vaccination was reported. In 6 patients, parents were relatives and in three cases, there was a family history of low platelet counts. Variable thrombocytopenia, prolonged bleeding time (BT), and large platelets with increased bone marrow megakaryocyte were seen in all cases. Most patients were treated with steroids, Intravenous immunoglobulin (IVIG), and some with IV anti-D, Azathioprine, Danazol, Rituximab. Splenectomy was performed in one case. In supplementary tests the platelet aggregation to ristocetin was absent and GPIb expression level by flow cytometry method was lower than 10%. CONCLUSION: BSS should always be considered in differential diagnosis of ITP especially in persistent and refractory ITP.

The impact of accreditation on nurses' perceptions of quality of care in Iran and its barriers and facilitators.

BACKGROUND: Iran national hospital accreditation was initiated as a government and mandatory program in 2012. The aim of this study was to examine the impact of accreditation on nurses' perceptions of quality of care and to determine those barriers and facilitators needed for effective implementation of accreditation. METHODS: A cross-sectional survey conducted in 43 tertiary public hospitals in 5 metropolises, Iran, which successfully passed national accreditation surveys. Participants included nurses with at least 5-year work experience. Overall response rate was 76%, with 1312 of 1706 valid responses included in the data analysis. A questionnaire was applied using a 5-point Likert scale ranging from 1 "strongly disagree" to 5 "strongly agree". In addition, the questionnaire included 2 open-ended questions allowing the respondent to identify barriers and facilitators to the process of for improving accreditation implementation. The relationship between the quality of results and the independent variables was tested using multiple regression analysis. RESULTS: The scales measuring benefits of accreditation had the highest mean score followed by strategic quality planning, education and training, and staff involvement. Regression analysis indicated that leadership, commitment, and support; education and training; rewards and recognition; and staff involvement were factors affecting quality results. Barriers encountered included financial and capital resources, staff, institutional, and patients. Hospital accreditation has a positive impact on quality of care. CONCLUSIONS: The findings of this study provide valuable information to policymakers and hospital managers on which to base the process of accreditation and its requirements, and to help reap its benefits.

The Effect of Soy Nut Compared to Cowpea Nut on Body Weight, Blood Cells, Inflammatory Markers and Chemotherapy Complications in Children with Acute Lymphoblastic Leukemia: A Randomized Controlled Clinical Trial.

No previous studies were found to examine the effect of soy as a whole food on patients with leukemia. The present randomized controlled clinical trial studied the effect of soy nut on children with B-cell acute lymphoblastic leukemia (ALL) who were in the maintenance phase of chemotherapy. The eligible patients were randomized to receive 30 g/day soy or cowpea nut powder for 12 weeks. Dietary intake, physical activity, anthropometric measurements, complete blood count, serum albumin, serum highly sensitive C-reactive protein (hs-CRP), and Tumor necrosis factor alpha (TNF-α) as well as chemotherapy side effects were assessed at the start and the end of the study. In total 29 and 27 children completed the study (aged 6.34 ± 2.44 and 5.85 ± 2.35 years) in soy and cowpea nut groups, respectively. The total energy and protein intake, and physical activity as well as body weight, body mass index, number of red blood cells, hemoglobin and hematocrit levels, and fatigue were significantly improved in the soy nut group compared to patients who consumed cowpea nut (P < 0.05). Soy nut intake might improve the nutritional status, anemia, and fatigue in children with ALL. Studies targeting blood cell fractions and disease recurrence are highly recommended.

Assessment of Patient Safety Culture in Iranian Academic Hospitals: Strengths and Weaknesses.

BACKGROUND: In the relevant literature, the establishment of a patient safety culture (PSC) is a vital step in providing quality service to patients. The goal of this study was to establish a baseline for PSC in Iranian academic hospitals and to determine its strength and weaknesses. METHODS: A survey was distributed in 26 academic hospitals linked to the Iran and Tehran Universities of Medical Sciences, of which 18 participated. The questionnaire, the Hospital Survey on Patient Safety Culture (HSOPSC), was used to collect the data. The questionnaire contained 14 dimensions and 43 items, and the questions were scored using a 5-point Likert scale in which 1 = strongly disagree and 5 = strongly agree.The results were expressed in the percentage of positive responses (%), or the percentage of positive responses (e.g., agree, strongly agree) to positively worded items (e.g., "Hospital units work well together to provide the best care for patients") in relation to the percentage of negative responses (e.g., disagree, strongly disagree) to negatively worded items (e.g., "When an event is reported, it feels like the person is being written up, not the problem"). To score each item, the following formula was applied: % of Positive Responses = [(No. of Agree + No. of Totally Agree) / Total No. of Subjects Responding to the Question)] × 100). For the negatively worded items, the answers were reversed. To score each dimension, a composite mean score of positive responses were estimated. The same methodology was applied to the scoring of the PSC of each hospital. (i.e., the composite mean score of positive responses on 12 dimensions was obtained). Note that the data reported in the tables of this manuscript represent percentages, and for ease of communication, (%) are omitted from the stated tables. In the text, however, we have used percent wherever needed.To categorize the hospitals' safety culture from very weak to very strong, a range of 0.0% to 100% positive responses on the PSC questionnaire was divided by 5 ((100% - 0.0%) / 5 = 20%), and 5 equally spaced intervals were defined (i.e., 0.0%-20.0%, very weak; 21.0%-40.0%, weak; 41.0%-60.0%, intermediate; 61.0%-80.0%, strong; 81.0%-100.0%, very strong).The convenience sampling method was used to select the samples. For the purpose of this research, 35 questionnaires were randomly distributed in each hospital: 15 among nurses and assistant nurses (as the frontline personnel having the most direct contact with patients) and 20 among other clinical and paraclinical personnel as well as other groups (physicians, residents, interns, ward secretaries, laboratory personnel, radiology personnel, and managers). The questionnaires were distributed and collected by the hospitals' head nurses. The data were analyzed using a one-sample and independent t test. RESULTS: The mean score of the hospitals on the PSC ranged from 42.74 to 67.22, 12 of 18 hospitals obtained an "intermediate" ranking position, and 6 obtained a "strong" ranking position on the PSC. The overall mean (SD) score for the entire hospital for PSC was 56.74 (18.41). No significant difference was observed between the Iran and Tehran universities of medical sciences hospitals for the PSC. However, significant differences were observable at the dimension and item levels.A hospital's strengths lie in the "organizational learning-continuous improvement," "teamwork within hospital units," and "hospital management support for patient safety" dimensions. Dimensions including "feedback and communication concerning errors," "communication openness," "staffing," and "nonpunitive response to error" were shown to be weak points of all of the hospitals in this study. CONCLUSIONS: The PSC of the investigated hospitals is not at an ideal level and is in need of serious improvement, particularly in the dimensions of feedback and communication regarding errors, communication openness, staffing, and nonpunitive response to error. The same conditions hold true for other Iranian hospitals (i.e., the Afshar and Sadoughi hospitals in the city of Yazd, Iran), and American hospitals were used for comparison purposes in this paper.

The relationship between iron bone marrow stores and response to treatment in pediatric acute lymphoblastic leukemia.

Iron is an intracellular element whose accumulation in the body is associated with tissue damage. This study examines the effect of iron on pediatric acute lymphoblastic leukemia (ALL) and its "response to treatment." At the end of the first year of treatment, bone marrow iron store (BMIS) was evaluated in children with ALL and the relationship between iron store and minimal residual disease was investigated. Moreover, the 3-year disease-free survival (3-DFS) of patients was determined. Patients' BMIS were compared with that of subjects with normal bone marrow. The study examined 93 children, including 78 Pre-B and 15 T-cell ALL patients. BMIS did not differ between the children with ALL and those with no evidence of cancer. BMIS was increased in 26.6% of patients at the end of the first year of treatment. Drug resistance and BM relapses were more prevalent in cases with high BMIS in both Pre-B and T-cell groups. Bone marrow iron store is not considered a risk factor for childhood ALL. However, high levels of BMIS are associated with poor response to treatment and the risk of relapse. Bone marrow iron store control during treatment can therefore help achieve better outcomes and improve the chances of recovery.

EBV lymphoproliferative-associated disease and primary cardiac T-cell lymphoma in a STK4 deficient patient: A case report.

RATIONALE: Primary cardiac lymphoma (PLC) is an extremely uncommon malignancy. PCL is more common in secondary immunodeficient patients. In this report, we describe a unique case of PLC who had been diagnosed as a STK4 deficient patient. This case is the first Primary immunodeficiency (PID) patient developing PCL in the world. PATIENT CONCERNS: An eleven-year-old girl, a known case of PID, was referred to the pediatric cardiology department because of chest pain and dyspnea. Her CXR revealed cardiomegaly without mediastinal involvement and the echocardiography showed a mild pericardial effusion and cystic-shape echogenic masses. DIAGNOSES: After a period of missed follow up, she presented with respiratory distress following with syncope at the clinic because of a pressure effect of a large mass on the right ventricular outflow tract (RVOT) .An emergency operation was done for debulking of the tumors and resolving of RVOT obstruction. Biopsy and immunohistochemical staining was revealing "T-cell lymphoma", non-Hodgkin's type. INTERVENTIONS: Chemotherapy was done with cyclophosphamide, methotrexate, adriamycine, vincristine, hydrocortisone and allopurinol. OUTCOMES: The tumors shrank after chemotherapy initiation and she stayed stable for almost one month. Finally, she developed sever thrombocytopenia during her chemotherapy and died because of lung hemorrhage two months after her operation. LESSONS: Although PCL is very rare, it must be considered in the differential diagnosis of intracardiac mass or refractory pericardial effusions, especially in PIDs which are widely known for developing EBV-associated diseases such as lymphoma.

Investigation of FANCA gene in Fanconi anaemia patients in Iran.

BACKGROUND & OBJECTIVES: Fanconi anaemia (FA) is a syndrome with a predisposition to bone marrow failure, congenital anomalies and malignancies. It is characterized by cellular hypersensitivity to cross-linking agents such as mitomycin C (MMC). In the present study, a new approach was selected to investigate FANCA (Fanconi anaemia complementation group A) gene in patients clinically diagnosed with cellular hypersensitivity to DNA cross-linking agent MMC. METHODS: Chromosomal breakage analysis was performed to prove the diagnosis of Fanconi anaemia in 318 families. Of these, 70 families had a positive result. Forty families agreed to molecular genetic testing. In total, there were 27 patients with unknown complementary types. Genomic DNA was extracted and total RNA was isolated from fresh whole blood of the patients. The first-strand cDNA was synthesized and the cDNA of each patient was then tested with 21 pairs of overlapping primers. High resolution melting curve analysis was used to screen FANCA, and LinReg software version 1.7 was utilized for analysis of expression. RESULTS: In total, six sequence alterations were identified, which included two stop codons, two frames-shift mutations, one large deletion and one amino acid exchange. FANCA expression was downregulated in patients who had sequence alterations. INTERPRETATION & CONCLUSIONS: The results of the present study show that high resolution melting (HRM) curve analysis may be useful in the detection of sequence alteration. It is simpler and more cost-effective than the multiplex ligation-dependent probe amplification (MLPA) procedure.

Vitamin D and Bone Minerals Status in the Long-term Survivors of Childhood Acute Lymphoblastic Leukemia.

BACKGROUND: Low vitamin D and diminished bone minerals with the potential for fractures are one of the nonapparent late effects of acute lymphoblastic leukemia (ALL). Chemotherapy and radiation were known as two important risk factors. We evaluated these late effects in ALL survivors who were treated with chemotherapy or chemo plus cranial radiation therapy. METHODS: In a case-control study, 33 of ALL survivors who were treated with chemotherapy (Group A), and 33 subjects who were treated with chemoplus cranial radiation (Group B) were compared against 33 matched age, sex, and pubertal stage of their healthy siblings (Group C). Standard anthropometric data were collected as well as Tanner staging for puberty, number of fractures since treatment, serum calcium (Ca), phosphorus (P), magnesium (Mg), alkaline phosphatase, parathyroid hormone, and 25-hydroxyvitamin D (25(OH) D). The independent t-test, one-way ANOVA, Chi-square test, and Tukey's test were used to analyze the data. RESULTS: The findings indicated that the mean serum levels of 25(OH) D in ALL survivors (i.e. Groups A and B) with age mean score of 11.2 years and 12.3 years, average treatment length: 3.25 years and average time after treatment completion: 4 years, was lower compared to the controls group (12.94 ± 6.69, 14.6 ± 8.1, 20.16 ± 10.83, respectively, P < 0.001) but no significant difference was observed between Group A and B in this regard (P > 0.05). Other clinical and laboratory parameters had no significant differences between the survivors and control. Vitamin D deficiency (<20 ng/ml) was observed in 27% of group A and 24% of group B and vitamin D insufficiency (20-30 ng/ml) in 72.7% and 69.6% survivors of Group A and B and 48.5% of controls group (P = 0.003). CONCLUSIONS: ALL treatment is associated with the increase in prevalence of vitamin D insufficiency in the childhood ALL survivors and since the low vitamin D level potentially increases the risk of low bone density, subsequent malignancies, and cardiovascular disease in the survivors, close follow-up of such patients are highly recommended to prevent the stated complications.

Evaluating the safety and efficacy of silymarin in ß-thalassemia patients: a review.

ß-Thalassemia (ß-thal) is a type of hereditary anemia affecting hemoglobin (Hb) synthesis causing severe chronic anemia in homozygous patients. Regular blood transfusions are the mainstay treatment for this type of anemia. In turn, this leads to iron overload which is responsible for the formation of reactive oxygen species (ROS), oxidative stress and organ damage. Deferoxamine (DFO) is the standard of treatment for iron overload but regular painful subcutaneous administration of this medication prevents optimal compliance. Oral chelators, such as deferasirox (DFX) and deferiprone (DFP), are also effective and safe. Deferiprone is most effective in combination therapy with DFO rather than monotherapy; however, DFX is very expensive and the cost is a significant new burden for patients. Recently, researchers have proposed an iron chelating effect for silymarin that is a flavonoid extract from the milk thistle plant. This extract has different properties and has long been used for its antioxidant and hepatoprotective effects. In this review we assess different aspects of silymarin's potential effects and compare them to the profile of thalassemic patients.

Post transplant anaplastic large T-cell lymphoma.

Post transplant lymphoproliferative disorders (PTLD) are a heterogeneous group of lymphoid proliferation that ranges from polyclonal hyperplasia to monoclonal malignant lymphoma. We report a 13-year-old boy who was diagnosed with PTLD in February 2007 after 3 1/2 years of deceased renal transplantation. We treated him with an adapted ACVBP (doxorubicin, cyclo-phosphamide, vincristine, bleomycin and prednisone) regimen. He responded well to the chemo-therapy without deterioration of graft function.

The metabolic syndrome in survivors of childhood acute lymphoblastic leukemia in Isfahan, Iran.

BACKGROUND: To determine the prevalence of metabolic syndrome in survivors of childhood leukemia in Isfahan, Iran. METHODS: During a 4-year period (2003 to 2007), 55 children (33 male and 22 female) diagnosed with ALL at Unit of Hematology/ Oncology, Department of Pediatrics, Isfahan University of Medical Science, were enrolled in this cross-sectional study. Metabolic syndrome was defined using the modified version of Adult Treatment Panel (ATP III) crite-ria. Insulin resistance was defined based on the homeostasis model assessment index (HOMA-IR). RESULTS: The mean age of participates was 10.4 years (range 6-19 years) and the mean interval since completion of chemotherapy was 35 months. Twenty percent (11/55) of survivors (10 male, 1 female) met criteria for diagnosis of metabolic syndrome. Obesity was observed in one forth of patients and nearly 3/4 of obese patients had metabolic syndrome. High serum insulin levels were found in 16% of participants and in 63% of obese survivors. The mean insulin levels in survivors with metabolic syndrome was three-times more than those without (28.3 mu/l vs. 9.57 mu/l, p = 0.004). Insulin resistance was detected in 72.7% of survivors with metabolic syndrome and it was positively correlated with serum triglycerides (0.543, p ≤ 0.001), systolic and diastolic BP (0.348, p = 0.01 and 0.368, p = 006 respectively), insulin levels (0.914, p < 0.001) and blood sugar (0.398, p = 003). CONCLUSIONS: The prevalence of metabolic syndrome in survivors of childhood leukemia in Iran is higher than developed countries. Nearly all of the obese patients had metabolic syndrome. Weight control and regular physical exercise are recommended to the survivors.