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Background: The aim of this study was to assess the impact of serum panel reactive antibodies (PRA) on the outcomes of allogeneic hematopoietic stem cell transplantation (HSCT) in pediatric thalassemia patients. Methods: A total of 73 pediatric patients with thalassemia were included in this single‐center study. Pre‐transplant PRA levels were evaluated, and the patients were divided into two groups: PRA‐negative (group 1; n = 44) and PRA‐positive (group 2; n = 29). Patient characteristics, including age, gender, donor type, stem cell source, and HLA compatibility, were analyzed. Transplant outcomes, including engraftment, transfusion requirements, and transplant‐related complications, were compared between the two groups. Further subgroup analysis was performed based on MFI values. Results: At the time of transplantation, patients in group 1 were younger than those in group 2 (p =.008). The number of fully matched donors within the family (MSD and MFD) was significantly higher in group 1 (p =.049). Additionally, Rh blood group incompatibility was higher in group 2 (p =.03). There was no statistically significant difference in the engraftment days of neutrophils, platelets, and erythrocytes between the two groups. The frequency of poor graft function and graft failure was higher in the group 2, but there was no statistically significant difference. Post‐transplant transfusion requirements for platelets and red blood cells were significantly higher in the group 2 (p <.001). Transplant‐related complications such as VOD, PRES, and aGvHD were more common in the group 2, but no statistical significance was detected. Conclusions: Serum PRA in pediatric thalassemia patients may impact the outcomes of HSCT. PRA‐positive patients had higher rates of blood product transfusion requirements. Although poor graft function, graft failure, and post‐transplant complications were more common in the group 2, statistical significance was not observed. Identifying patients with high PRA levels can assist in optimizing transplant strategies and post‐transplant care, leading to improved outcomes for the patients. [ABSTRACT FROM AUTHOR]

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The article presents a study which described the treatment of a patient stricken with infantile osteopetrorickets and was treated with haploidentical bone marrow transplantation. The patient is actually suffering from a combination of two ailments, osteopetrosis which is remedied by bone marrow transplantation and rickets wherein a high dose of calcitrol and recombinant interferon gamma are used as treatment.

Captopril and enalapril are the most commonly used angiotensin converting enzyme inhibitors in several cardiac diseases in children. On the other hand, the intrinsic renin-angiotensin system in the bone marrow might affect the growth of hematopoietic colonies and cellular production, proliferation and differentiation in physiological and pathological states. Starting with the hypothesis that inhibition of the renin-angiotensin system may have some effects on the hematopoietic system, including morphological changes within the granulocytes, we thus aimed to investigate prospectively whether the use of angiotensin converting enzyme inhibitors has any effect on the morphology, and especially segmentation, of neutrophils in peripheral blood. A total of 40 children with various heart diseases receiving either of two angiotensin converting enzyme inhibitors (captopril or enalapril) aged between 2 to 16 years were enrolled, and 40 healthy age- and sex-matched children were enrolled as controls. Complete blood count, peripheral blood smear, liver and renal function tests, and measurement of serum alkaline phosphatase, ferritin, vitamin B12 and folate levels were performed in all cases. Peripheral blood smears were viewed by two pediatric hematologists in a blinded manner. Neutrophil hypersegmentation was described as presence of five or more neutrophils with five well-separated lobes or at least one neutrophil with six or more lobes among 100 segmented neutrophils. The number of patients with neutrophil hypersegmentation in the study group was significantly higher than in the control group, and the mean lobe count in the study group was significantly higher than in the control group. Neutrophil hypersegmentation, as detected in patients using angiotensin converting enzyme inhibitors in the present study, has not been reported previously. Further studies aiming to explain the pathophysiological mechanism(s) underlying neutrophil hypersegmentation in patients receiving angiotensin converting enzyme inhibitors are needed. [ABSTRACT FROM AUTHOR]

Helicobacter pylori gastritis has been associated with autoimmune disease, including immune thrombocytopenic purpura (ITP). The most recent reports also have supported this association in adults. ITP in children differs from that in adults in terms of clinical picture and mechanisms of thrombocytopenia. The authors report a case of a 12-year-old boy with chronic ITP, in whom they detected H. pylori infection and observed a complete platelet recovery after the eradication of H. pylori. [ABSTRACT FROM AUTHOR]

The article presents a case report of an 18 month-old boy with skeletal anomalies, assumed to have congenital nephrogenic diabetes insipidus (CNDI). The patient has been identified to have vasopressin V2 receptor (AVPR2) mutation, one of the two molecules which causes CNDI. Several laboratory tests were also performed including a genetic analysis in both the mother and the patient. Further examinations indicated an X-linked recessive noninvasive diagnostic imaging (NDI) without association with the skeletal anomalies.

AIM: Chronic systemic diseases in childhood have negatively affecting the quality of life and debilitating effects for both children and parents. In our study, we investigated depression, anxiety and hopelessness situations at parents of children with these diseases. METHOD: The study was done at parents of children diagnosed with malignancy or chronic disease in GATA Department of Pediatrics Heath and Disease, Pediatric Hematology and Oncology Clinics. Beck Depression Scale, Beck Anxiety Scale and Beck Hopelessness Scale were applied to the participants. RESULTS: Parents of children, who are followed due to malignancy or chronic disease in department of pediatrics heath and disease, pediatric hematology and oncology clinics, constituted the study group. 60 mothers and 51 fathers as study group and 64 mothers and 45 fathers as control group were enrolled in the study between 1st July 2009 and 1st June 2010. The mean age of the parents in study group was 35.7±5.1 and 33,3 5,6 age in control group. The depression score was significantly higher statistically in study group (p=0.035). No difference was fond for the anxiety and hopelessness scores between the groups (p=0.064 and p=0.695 respectively). There was no difference for depression, hopelessness and anxiety scores between mothers and fathers of the children (p=0.217, p=0.447, p=0.102, respectively). CONCLUSION: Without gender discrimination the parents of children with malignancy and chronic disease are in the risk group for depression. It is necessary to support the parents both socially and psychologically. [ABSTRACT FROM AUTHOR]

This study was performed to investigate the platelet aggregation alterations in platelet-rich plasma (PRP) samples of children with Helicobacter pylori (H pylori) infection. Platelet aggregation induced by adenosine diphosphate (ADP), collagen, ristocetin, or epinephrine was studied with photometric aggregometry in 30 patients before and after eradication therapy and in a control group including 15 children. The pretreatment mean maximum aggregation values and slope were significantly lower (P < .0001) in the study group at 10 μmol/L concentrations of ADP (ADP-like defect). The maximum aggregation values and slope revealed no significant differences (P > 0.05) between the study group after therapy and the control group. We concluded that H pylori infection may cause dysfunction of platelets in children and can be reversed by H pylori eradication therapy. Further studies should be carried out to determine the mechanisms of platelet dysfunction in children with H pylori infection.

There has been little or no evidence of brainstem auditory evoked potentials (BAEPs) among infants with iron deficiency (ID) that is not severe enough to cause anemia. To our knowledge, the effect of ID on auditory functions and/or potentials has not been investigated previously, though it seems reasonable that it should be associated with BAEP measures intermediate between those observed in iron deficiency anemia (IDA) and in iron sufficiency, considering the role of iron in myelin formation and maintenance. We therefore aimed in this study to investigate the effect of ID on BAEPs by comparing three groups of infants with ID, IDA and iron sufficiency (control) both before and after iron treatment (in iron-deficient groups). Three groups of infants (IDA, n = 25; ID, n = 24; Control, n = 44) were compared on the basis of hematological laboratory parameters and BAEP measurements both at entry into and after (12 weeks treatment with oral iron in IDA and ID groups) the study. BAEP measurements recorded at 85 dB both at entry into and after the study were not significantly different among the groups, although a sufficient response to iron treatment was achieved in iron-deficient groups (Group I and Group II). The only positive finding determined in our study was a slight decrease in latencies obtained at the end of the study when compared to the pre-study values in all three groups of the study in accordance with the expected age-dependent developmental changes. Although no negative electrophysiological effect of ID on brainstem auditory functions was found in the present study, further longer term (late childhood or adult) studies are necessary to elucidate the relationships among anemia (maybe other than IDA), ID and auditory functions, and clinical implications of hearing loss (if any) should be questioned.

Immune thrombocytopenic purpura in childhood is characterized by a typical history of acute development of purpura and bruising in an otherwise healthy child. In children it usually follows a viral infection (eg, mumps, rubella) or immunization. We report for the first time a child with acute lymphoblastic leukemia who developed immune thrombocytopenic purpura due to mumps during the maintenance phase of acute lymphoblastic leukemia treatment.