scholarly journals Creating Awareness for Primary Immunodeficiencies in Japan

2021 ◽  
Vol 12 ◽  
Author(s):  
Hidetoshi Takada

Primary immunodeficiency (PID) is primarily characterized by susceptibility to infectious diseases. In addition, patients with some type of PID are prone to develop autoimmune, autoinflammatory, or malignant diseases. Therefore, the term, inborn errors of immunity (IEI), has been more used rather than PID. In recent years, the number of diseases which belong to PID has been increasing. There were approximately 110 diseases in the report of International Union of Immunological Societies in 1999. Since then, the number increased to 430 diseases in the latest IUIS report in 2019. We conducted PID nationwide survey in Japan for 3 times in the last 15 years. These studies were focused on incidence and complications of PID, the clinical course of viral infection, and methods to prevent infectious diseases in PID patients. For the awareness of PID, it is essential to know the general and fundamental information of PID patients. Needless to say, we need it to offer appropriate medical services for PID patients. Moreover, chances to provide answers to the questionnaires and seeing the results of the analysis should contribute to the awareness of PID among doctors. In this review, I am going to summarize the results of 3 nationwide survey in Japan, and pick up interleukin-1 receptor-associated kinase 4 (IRAK4) deficiency as an example for creating awareness for its appropriate management.

2009 ◽  
Vol 41 (1) ◽  
pp. 89-96 ◽  
Author(s):  
Anna G. Orr ◽  
Anup Sharma ◽  
Nikolaus B. Binder ◽  
Andrew H. Miller ◽  
Bradley D. Pearce

PEDIATRICS ◽  
1956 ◽  
Vol 18 (1) ◽  
pp. 160-162 ◽  
Author(s):  
ROBERT J. HAGGERTY ◽  
R. CANNON ELEY

In the past 2 years we have seen 2 patients at the Children's Medical Center, who, while receiving cortisone therapy, developed varicella and died following a state of shock within a few days after the appearance of the vesicles. Post-mortem examinations of these patients revealed hemorrhagic vesicles throughout all viscera. In 1 case the virus of varicella was grown from vesicle fluid, heart blood, and lung. Since fatalities from varicella in childhood are so rarely encountered, even by physicians with vast experience in the field of infectious diseases, and since the clinical course of the infection in these two patients was so fulminating, the possible relationship of cortisone administration was raised.


PEDIATRICS ◽  
1985 ◽  
Vol 75 (2) ◽  
pp. 280-283
Author(s):  
ELIZABETH H. DANISH ◽  
BEVERLY B. DAHMS ◽  
MARY L. KUMAR

Virus-associated hemophagocytic syndrome, first described by Risdall and co-workers in 1979,1 is a rare histiocytic proliferative syndrome characterzed by fever, hepatosplenomegaly, pancytopenia, and erythrophagocytosis by histiocytes that appear benign by histologic criteria. The clinical course and pathologic findings may be identical with another histiocytic disorder, familial erythrophagocytic lymphohistiocytosis, which occurs predominantly in infants. Diagnosis of virus-associated hemophagocytic syndrome depends entirely on evidence of concurrent viral infection, usually of the herpes group. Epstein-Barr virus has been associated with this syndrome in the few cases reported in children without underlying disease, whereas cytomegalovirus (CMV) has been implicated in immunosuppressed patients. We report a case of fatal CMV-associated hemophagocytic syndrome which occurred in a previously healthy infant.


2018 ◽  
Vol 24 (6) ◽  
pp. 710-717 ◽  
Author(s):  
Osamu Kanauchi ◽  
Akira Andoh ◽  
Sazaly AbuBakar ◽  
Naoki Yamamoto

Recently, the risk of viral infection has dramatically increased owing to changes in human ecology such as global warming and an increased geographical movement of people and goods. However, the efficacy of vaccines and remedies for infectious diseases is limited by the high mutation rates of viruses, especially, RNA viruses. Here, we comprehensively review the effectiveness of several probiotics and paraprobiotics (sterilized probiotics) for the prevention or treatment of virally-induced infectious diseases. We discuss the unique roles of these agents in modulating the cross-talk between commensal bacteria and the mucosal immune system. In addition, we provide an overview of the unique mechanism by which viruses are eliminated through the stimulation of type 1 interferon production by probiotics and paraprobiotics via the activation of dendritic cells. Although further detailed research is necessary in the future, probiotics and/or paraprobiotics are expected to be among the rational adjunctive options for the treatment of various viral diseases.


2021 ◽  
Vol 22 (2) ◽  
pp. 60-63
Author(s):  
A. A. Beltikova ◽  
◽  
A. A. Zasypkina ◽  
O. A. Lyubimtseva ◽  
L. V. Khanipova ◽  
...  

Aim. The objective is to explore the incidence rate and the specifics of the clinical course of herpesviral meningitis in children and adults. Materials and methods. The post-hoc analysis of 23 patients’ records has been carried out. The patients were admitted to State Budgetary Healthcare Institution of the Tyumen Region, Clinical Hospital for Infectious Diseases, City of Tyumen, during the period of 2018-2019 diagnosed with meningitis of herpesviral etiology. When analysing the data the following aspects were taken into account: age, sex, etiological agent, complaints at admission, results of clinical examination and laboratory findings. Results. Twenty-three (23) cases of herpesviral meningitis were registered during the period of two (2) years; mixed infection was detected in every fifth patient. Epstein Barr viral meningitis was diagnosed in most cases and was detected 3.5 times oftener in adults than in children. Herpes Simplex Virus meningitis was the second one in terms of detection rate and it affected only adults. Varicella Zoster Virus meningitis was detected two times rarer than Herpes Simplex Virus and only in adults. The herpesviral meningitis caused by human herpes virus type 6 was detected with the same rate as Varicella Zoster meningitis but was registered among children only. Cytomegaloviral meningitis was detected in one adult who was affected by severe immune deficiency. Conclusion. The detected specifics of the incidence of the herpesviral meningitis with respect to a patient’s age will enable a practitioner to suggest the possible meningitis etiology and to prescribe appropriate treatment thus improving the prognosis for the disease.


Sign in / Sign up

Export Citation Format

Share Document