Hiroshi Igarashi

High serum levels of M-CSF and G-CSF in Kawasaki disease.

To examine any role of macrophage colony‐stimulating factor (M‐CSF), in the immune responses in Kawasaki disease (KD), we serially assayed M‐CSF and several related cytokines using ELISA. In 10 paediatric patients with KD the level of M‐CSF was significantly higher in the acute phase than in the convalescent phase (1476.1 ± 443.6 v 805.0 ± 184.7 U/ml). Higher levels of serum granulocyte colony‐stimulating factor (G‐CSF) and interleukin‐6 were also found in the acute phase. These results suggest that M‐CSF, G‐CSF and interleukin‐6, derived from monocytes as monokines or derived from vascular endothelial cells, might play an important role in the acute phase of KD.

Hypertrophic cardiomyopathy in congenital myotonic dystrophy

Involvement of the cardiac conduction system is a common clinical feature in myotonic dystrophy, whereas the association of primary myocardial abnormalities has rarely been reported. A patient with a severe form of congenital myotonic dystrophy who developed fatal left ventricular hypertrophy at 3 months of age and died at 2 years of age is reported. Serial ultrasonographic studies revealed progressive left ventricular hypertrophy accompanied by outflow obstruction of the left ventricle. Southern analysis for the myotonin kinase gene revealed a 5.8 kb expansion of CTG repeats in addition to a fragment of normal length. The degree of expansion was much greater than those of other reported patients with congenital myotonic dystrophy. These findings suggest that left ventricular hypertrophy represents an extreme level of myocardial damage in myotonic dystrophy and that this damage may be related to the larger size of the CTG repeats.

Increased serum granulocyte colony-stimulating factor correlates with coronary artery dilatation in Kawasaki disease.

Abstract. In acute-phase Kawasaki disease, neutrophils cause injury to the coronary arterial endothelium through the production of elastase. Previous research has demonstrated the modulation of neutrophil function and kinetics, such as development and maturation, by granulocyte colony-stimulating factor (G-CSF). To examine the correlation between G-CSF and cardiac complications in Kawasaki disease, functional activity of serum G-CSF and cytokines was measured by enzyme-linked immunosorbent assay in 30 patients with acute-phase Kawasaki disease aged 2 months to 5 years. The mean serum G-CSF was higher in the 1st week of Kawasaki disease than during weeks 2 to 4, and G-CSF was significantly higher in patients with coronary artery dilatation (CAL) than in those without. There was no significant difference in the activity of other cytokines studied or white blood cell counts between the patients with CAL. Conclusion: granulocyte colony-stimulating factor is correlated with coronary artery dilatation in acute-phase Kawasaki disease and increased neutrophil function may contribute to the pathogenesis of coronary arterial endothelial injury in these patients.

Case of Kawasaki disease in NICU

The incidence of Kawasaki disease (KD) in infants younger than 3 months of age is quite low. 1,2 This may be due to the unknown protective effects of transplacental immunity or reduced exposure to infectious agents in younger infants. There are no previous reports of KD in neonatal intensive care units (NICU) due to the isolated environment or the very young age of infants being cared for in NICU. Intravenous gamma globulin (IVGG) has become a treatment option to prevent the development of coronary artery aneurysm, however, some patients are resistant to IVGG. A one-monthold infant developed persistent fever and generalized skin rash in the Jichi Medical School NICU and was diagnosed as having KD. The infant’s coronary arteries were affected despite treatment with IVGG and ulinastatin. This is the first reported case of KD in an NICU.

A Case of Kawasaki Disease in NICU

Abstract[Background] Kawasaki disease (KD) in children younger than 3 months of age is rarely observed. The rarity of disease in early infants may be due to protective effects of transplacental immunity and/or low level of exposure to infectious agent. We report a case of an infant who developed KD in a neonatal intensive care unit (NICU). [Case report] A male infant was born at 31 weeks of gestation; weighed 1772g, and admitted to our NICU. At 84 days of age, he presented with high fever, exanthema and dyspnea. He was diagnosed with pneumonia and treated with intravenous antibiotics and gammaglobulin (150 mg/kg/day, 3 consecutive days). The high fever lasted even after improvement of respiratory lesions, which were followed by bilateral conjectival injection, bright red lips and edematous changes in palms and soles. He was diagnosed with KD at 93 days of age (9 days of illness). Right and left coronary arteries were dilated up to 2 mm. He was treated with high-dose gammaglobulin (1500 mg/kg/dose), however, fever and other symptoms persisted. He developed pericardial effusion and bilateral coronary arterial aneurysms (4.5 mm), despite 4 additional gammaglobulin injections (1000 mg/kg/dose, respectively). Beginning at 24 days of illness, he was treated with intravenous ulinastatin (15000 U/kg/day) for 5 days, and fever and all other symptoms disappeared. He was discharged at 63 days of illness. Follow-up cardiac catheterization and angiography at 2 months after discharge confirmed bilateral coronary arterial aneurysms. [Discussion] The present case developed KD even in extremely isolated environment as NICU. One possible explanation is that premature infants receive insufficent passive immunity from their mothers. In this infant, ulinastatin therapy was effective. Thus this treatment should be considered for young infants who are resistant to intravenous gammaglobulin therapy

Efficacy of a leukotriene receptor antagonist for pediatric cedar pollen allergy complicated by asthma

Leukotriene receptor antagonists (LTRAs) are identified as a monotherapy for asthma and allergic rhinitis; however, their use in children for treatment of these diseases has not been examined. Accordingly, the present study investigated the efficacy of pranlukast dry syrup for children with both pollinosis and asthma. The subjects were children receiving treatment for asthma who were also diagnosed with cedar pollen allergy. Patients were divided into a group that received continuous treatment with pranlukast (group A; n=20) and a group that commenced add-on treatment for pollinosis following the onset of symptoms (group B; n=20). Patients in group B were randomly allocated to subgroup B1 (add-on treatment with pranlukast dry syrup) or subgroup B2 (add-on treatment with a second-generation antihistamine). In both groups, nasal and ocular symptoms were evaluated every day and recorded in a diary. Exacerbation of nasal obstruction was demonstrated in group B; however, not in group A. There was a significant difference in symptoms observed between the two groups during the late peak pollen period (P<0.05). The incidence of nasal obstruction (defined as a nasal obstruction score ≥3 or use of a nasal steroid spray) was significantly lower in group A compared with group B (P<0.05). The maximum scores for sneezing and nasal obstruction during the late peak of the pollen season were lowest in group A, followed by subgroup B1 and subgroup B2. The present study demonstrated that long-term administration of LTRA for the management of asthma may improve nasal symptoms of pollinosis during the pollen season in children with pollinosis and asthma.