Childhood-onset Takayasu arteritis and immunodeficiency: case-based review.

Takayasu arteritis (TAK) has been rarely reported in patients with immunodeficiency. In this review, we present two cases with childhood-onset TAK (c-TAK) and primary immunodeficiency while reviewing similar cases in the literature. We reviewed the data for our two pediatric patients with c-TAK and primary immunodeficiency. We also reviewed the literature for patients with c-TAK and immunodeficiency from the inceptions of the databases up to November 2021. A 14-year-old patient had lipopolysaccharide-sensitive beige-like anchor (LRBA) deficiency, and a 16-year-old had X-linked severe combined immunodeficiency (X-linked SCID). During the follow-up, they developed findings suggestive of vasculitides such as hypertension, elevation in acute phase reactants, weakness, and weight loss. Thoracoabdominal computed tomography angiography revealed findings consistent with vasculitis involving the aorta and its major branches. Patients were diagnosed with c-TAK, and corticosteroids were given to both patients in the treatment. We identified 11 articles describing 17 TAK patients with immunodeficiency in our literature search. Two of the patients with c-TAK were infected with human immunodeficiency virus (HIV), another patient had Wiskott-Aldrich syndrome, and the other had idiopathic CD4 + T lymphocytopenia. Nine adult patients with TAK were infected with HIV, three patients had common variable immunodeficiency disorder (CVID), and the other had STAT1 gain-of-function mutation. Clinicians should consider that immunodeficiencies may be accompanied by vasculitic conditions such as TAK. Hypertension, increased inflammatory markers, and constitutional symptoms may be red flags for the development of TAK.

Pediatric bone marrow transplantation: Psychopathologic features in recipients along with siblings.

OBJECTIVE: To analyze the development of psychopathology in recipients along with their donor and nondonor siblings and the relationship with the bone marrow transplantation (BMT) process. METHODS: All children were interviewed using the Kiddie Schedule for Affective Disorders and Schizophrenia to assess psychopathology. The depression and anxiety symptoms and self-esteem of children and adolescents were evaluated using the Children's Depression Inventory, State-Trait Anxiety Inventory for Children, State-Trait Anxiety Inventory, and Rosenberg Self-Esteem Scale. RESULTS: In this study, the depressive symptom level was found significantly higher in the donor group compared with the nondonor group. State anxiety symptoms were higher in the BMT group (P < .05). There were no significant differences in trait anxiety symptoms. Self-respect was higher in children in the donor group compared with those in the BMT group (P < .05). During the transplant process, children with bone marrow transplants had a higher prevalence of depression, anxiety disorder, and attention-deficit/hyperactivity disorder, and nondonor siblings had a higher prevalence of depressive disorder, anxiety disorder, and attention-deficit/hyperactivity disorder compared with society in general. CONCLUSION: Physicians should deal with the family as a whole, not just their patient, and should be aware of the psychiatric risk of other siblings during the assessment.

Structural investigation of donor age effect on human bone marrow mesenchymal stem cells: FTIR spectroscopy and imaging.

Stem cell studies hold enormous potential for development of new therapies for tissue regeneration and repair. Bone marrow mesenchymal stem cells (BM-MSCs) can differentiate into a variety of non-hematopoietic tissues and contribute maintenance of healthy hematopoiesis by providing supportive cellular microenvironment into BM. Here, we investigated age-related differences in BM-MSCs by using attenuated total reflection Fourier transform infrared (ATR-FTIR) spectroscopy and FTIR imaging together with hierarchical clustering as a novel methods to clarify global alterations in the structure and function of macromolecules in characterized BM-MSCs of different aged donors. The results may contribute to identification of age-related new molecular marker(s) to determine the effects of donor age on MSCs. The spectral results reflected that there were significant increases in the concentration of saturated lipids, proteins, glycogen, and nucleic acids in children and adolescent group BM-MSCs when compared to the infants and early and mid adults. The concentration of mentioned macromolecules in adult (early and mid) BM-MSCs were significantly lower than the concentrations in the children and adolescents. These results were attributed to the increase in the proliferation activity in younger BM-MSCs. The distribution of macromolecules into the cells was shown as in the form of chemical maps by FTIR imaging, and the results are in agreement with the ATR-FTIR spectroscopy results. The cellular activity degree was determined by the thiazolyl blue tetrazolium bromide (MTT) proliferation assay to support ATR-FTIR spectroscopy results. BM-MSCs of five different age groups were discriminated by making the hierarchical cluster analysis where the spectral data according to alterations in structure and composition of macromolecules were considered.