Pediatrics international : official journal of the Japan Pediatric Society
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Juvenile fibromyalgia (JFM) is a disease in which patients complain of acute and chronic severe pain, an overt primary cause for which cannot be found or surmised. Although patients with JFM mainly complain of systemic pain or allodynia in the medical interview and physical examination, the concept of the disease is the total sum of painful illness, chronic fatigue, hypothermia and many other autonomic symptoms and signs. Many issues are interacting including individual traits (personality, temperament, sensitivity, memory of pain; age: early adolescence), individual states (self-esteem, anxiety, developmental level), and external stressors (parent especially mother, school environment). ⋯ Psychological support is advocated. Although the exact number of patients with JFM is still to be elucidated, it seems to be growing because pediatric rheumatologists in Japan encounter children with a wide variety of musculoskeletal pains. This guideline describes how to diagnose JFM in children and how to treat them appropriately.
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A significant proportion of cases of tracheobronchial foreign body aspiration due to life-threatening condition is observed during childhood. The aim of the present study was to describe our experience with the diagnosis and treatment of foreign body aspirations during childhood and review published literature. ⋯ Tracheobronchial foreign body aspiration is a significant cause of childhood morbidity and mortality. Early diagnosis and treatment is of utmost importance. Rigid bronchoscopy under general anesthesia should be performed in all patients suspected of foreign body aspiration, which could minimize mortality and morbidity if performed by experienced personnel with safe methods.
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Hemophagocytic lymphohistiocytosis (HLH) is a multisystem inflammatory disorder due to cytokine overproduction from excessively activated lymphocytes and macrophages. HLH has been divided into two subgroups: primary HLH and secondary HLH. Primary HLH includes PRF1, UNC13D, STX11, STXBP2, RAB27A, LYST, SH2D1A and XIAP gene mutations; and secondary HLH is associated with infections, malignancies and autoimmune diseases. ⋯ Analysis of clinical phenotypes of XLP patients suggests that XLP predominantly shows familial HLH phenotypes, whereas some XLP patients present sporadic HLH. For many decades, clinicians and investigators have been concerned with possible XLP in young boys presenting with Epstein-Barr-virus-associated HLH. This review aims to describe the new knowledge about XLP and to draw the attention of the pediatrician to XLP, which should be differentiated from other forms of HLH.
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Several therapies including immunosuppressive agents have been shown to be effective and safe for frequently relapsing nephrotic syndrome/steroid-dependent nephrotic syndrome (FRNS/SDNS) and steroid-resistant nephrotic syndrome in children. It is evident, however, that a substantial number of children are still refractory to treatment despite these therapies. Rituximab is a chimeric monoclonal antibody, which inhibits CD20-mediated B-cell proliferation and differentiation. ⋯ The Research Group of Childhood-onset Refractory Nephrotic Syndrome (RCRNS) conducted a randomized, double-blind, placebo-controlled, multi-center clinical trial (RCRNS-01) and an open-label, multi-center, pharmacokinetic clinical trial (RCRNS-02). These two trials were investigator-initiated, registration-directed clinical trials designed to apply Ministry of Health, Labour and Welfare approval for the use of rituximab for childhood-onset refractory FRNS/SDNS in Japan. RCRNS-01 could be the first study to clarify whether rituximab is effective and safe for childhood-onset refractory FRNS/SDNS.
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Bronchoscopies are performed in childhood for diagnostic reasons (e.g. evaluation of stridor, unexplained cough, possible malformations) and therapeutic reasons (e.g. foreign body removal, management of the difficult airway). ⋯ Although inspection of the pediatric airways has become a well-accepted routine procedure with a high diagnostic yield, and bronchoscopies are well tolerated, it is important that the most appropriate means of access to the airways is chosen according to the indications and the age of the child.