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.
Human rotavirus is the most important cause of severe diarrhoea in infants and young children worldwide. We describe the aetiology of viral diarrhoea and the characteristics of rotavirus infection in Shahrekord, Iran. Two hundred and fifty nine children <5 years old admitted to Hajar Hospital, 245 children with acute diarrhoea attending primary health centres in Shahrekord, and 114 children hospitalised for elective surgery were selected from October 2001 to August 2002. Stool samples were screened for enteric viruses using EM. Rotaviruses were characterised using ELISA, reverse transcription-polymerase chain reaction (RT-PCR), and electropherotyping. One hundred and eighty six viruses were identified, of which 146 (78%) were rotavirus. The second most frequent virus was coronavirus, followed by calicivirus. Rotaviruses exhibited a marked seasonal variation, being most frequently isolated from November to February (50% of rotavirus recovered) and affected mostly children <2 years old. The RT-PCR successfully typed 139 of the 146 (95%) rotavirus G types and 124 (85%) P types. The most frequent P type was, P[8] in 108 (74%), P[4] in 16 (11%), and was P non-typeable in 22 (15%). Among the G types, G1 was identified in 120 (82%), G2 in 19 (13%), and was G-non-typeable in 7 (5%). Our results are the first report of rotavirus genotypes affecting Iranian children. The most frequent G and P types (G1, G2, P[8], and P[4]) are similar to those reported from around the world and will be covered by existing rotavirus vaccines targeting G types G1-G4.
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.
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.
β-Thalassemia (β-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.
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.
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.
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.
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