Asako Hiraoka

Significance of human neutrophil antigen-2a (NB1) expression and neutrophil number in pregnancy

BACKGROUND: Expression of human neutrophil antigen‐2a (HNA‐2a) is greater in women than in men. The size of the HNA‐2a‐positive neutrophil population increases with pregnancy.

A possible role for maternal HLA antibody in a case of alloimmune neonatal neutropenia

BACKGROUND: Alloimmune neonatal neutropenia (ANN) is caused by a reaction of maternal alloantibodies with paternally inherited antigens on the fetal neutrophils. While human neutrophil antigens (HNA) antibodies are found in half of ANN cases, specific antibodies have not been defined in the remaining cases.

Efficacy of prophylactic use of Trimethoprim-Sulfamethoxazole in autoimmune neutropenia in infancy

Purpose Most children with autoimmune neutropenia (AIN) have a benign clinical course because of the spontaneous resolution of neutropenia. The authors observed the clinical course of AIN in infancy accompanied by the prophylactic use of trimethoprim–sulfamethoxazole (TMP-SMX) during neutropenia. Patients and Methods Eight infants with AIN were followed by serial tests for antineutrophil antibodies and management of infectious complications. Results The spontaneous disappearance of antineutrophil antibodies that preceded the normalization of the neutrophil count was found in all patients. Until the resolution of neutropenia, TMP-SMX was administered in five patients, resulting in a reduction in the incidence of infection with no adverse effects. Conclusions These observations demonstrate the possibility of the safety and usefulness of TMP-SMX treatment in patients with AIN.

Monitoring neutrophil engraftment in allogeneic stem cell transplantation by flow cytometric analysis of neutrophil‐specific antigens NA1 and NA2

Neutrophil‐specific antigen (NA) expression on neutrophils was analysed in 18 Japanese children before and after allogeneic stem cell transplantation (allo‐SCT) with myeloablative regimen. Donor–recipient NA‐incompatibility was present in one of eight NA1/NA2 heterozygous patients and eight of 10 NA1/NA1 or NA2/NA2 homozygous patients. After allo‐SCTs from NA‐incompatible donors, a neutrophil recipient‐to‐donor conversion was confirmed in all cases. Conversion to donor NA type was complete before the absolute neutrophil count reached 0·1 × 109/l. These observations indicate that flow cytometric analysis of NA antigens is a simple and useful method for monitoring neutrophil engraftment in NA‐incompatible allo‐SCT.

Successful bone marrow transplantation in chronic granulomatous disease

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Dizygotic twins with neonatal alloimmune neutropenia associated with maternal anti-human neutrophil antigen-1b antibody

organs, including cataracts, heart and lung disorders, gastrointestinal problems, and central nervous dysfunction. And the endocrinological abnormalities, hypogonadism and hyperinsulinemia, are well described. Additionally, both abnormal glucocorticoid metabolism and poor adrenocortical reactivity to adrenocorticotropic hormone (ACTH) have been reported as adrenocortical dysfunctions. DM1-associated hyperkalemia has been reported in only a few cases of adult MD. Misra et al. reported three MD patients with hyperkalemia suggestive of hyperreninemic hypoaldosteronism. In those patients replacement therapy with a mineral corticoid (fludrocortisone) was ineffective. Although the mechanisms of adrenal hyporesponsiveness and resistance to exogenous mineral corticoid are unclear, abnormal DMPK activity may influence the expression of cytokines, ACTH, and atrial natriuretic hormone, and may be related to adrenocortical disorders. Abnormalities of skeletal muscle ionic channels and pumps during MD are well known. Especially with regard to potassium control, increases in the apamin-sensitive small-conductance Ca2+-activated K channel (SK3) and decreases in the Na+-K+ pump have been discovered. These complexes are thought to influence the stability of the resting membrane potential and to assist in depolarization. Furthermore, the Na+-K+ pump on red blood cell membranes is thought to be dysfunctional in MD. Thus, the abnormalities of these channels and pumps with regard to potassium could potentially induce hyperkalemia. In the current case, the same mechanism was thought to play an important role in the occurrence of hyperkalemia. Furthermore, hyperkalemia was improved, but not completely, at discharge. We considered the possibility that the function of channels or pumps had improved with the passing of time, but not completely. Although hyperkalemia is not common in CMD, and the mechanism of this unusual electrolyte abnormality has not been clearly defined, critical symptomatic hyperkalemia with atrial flutter and atrial fibrillation has been reported during adult MD. Based on this experience, hyperkalemia may also complicate CMD.