Effect of CpG Oligodeoxynucleotides on Airways of Mice with Established Airways Inflammation

PURPOSE: Airways eosinophilia and increased IgE, characteristic features of asthma, result from a predominant Th2 response. In this study, we investigated the effect of CpG oligodeoxynucleotides (ODNs) on the inhibition of airways eosinophilia in mice with established airway inflammation. We also investigated the immunological mechanisms involved. METHODS: Groups of BALB/c mice were sensitized intradermally with ovalbumin(OVA). At week 10, airway inflammation was induced by intranasal challenge of the mice with OVA. At week 14, the mice were challenged intranasally again with OVA in the presence and without the presence of CpG ODNs. Mice with saline administration served as negative controls. Bronchoalveolar lavage fluids(BALF) were obtained and eosinophils were counted. Th1 and Th2 cytokines in the spleen cell cultures were measured by ELISA. Serum OVA-specific IgE and IgG2a antibodies were also measured by ELISA. RESULTS: BALF eosinophils were significantly inhibited in the CpG ODNs-treated mice(P<0.01). IgE and IgG2a levels increased significantly in both CpG ODNs-treated and untreated groups as compared to the negative control group; there was, however, no significant difference between the two groups four days after intranasal administration of CpG ODNs. Cytokine analysis revealed decreased production of IL-4, IL-5, and IL-13 and increased production of IL-12 in the CpG ODNs-treated group as compared to the untreated group. Interestingly, IFN-gamma levels were not upregulated in the CpG ODNs-treated group. CONCLUSION: CpG ODNs vaccination is a potentially useful approach for reversing airways eosinophilia in mice with established airways inflammation.

Two Cases of Leigh Disease in Siblings

Leigh disease is a familial and degenerative disorder characterized by focal, bilateral, and usually symmetric lesions of the both gray and white matter in the brain and the spinal cord. The clinical course is variable, but in most cases, the prognosis is poor with subacute progression leading to death within months or years of life. The pathogenesis was known as mitochondrial enzyme defects of the respiratory chain system. We experienced 2 cases of Leigh diseases in a brother and sister. The brother had general weakness at 43 months of life and the sister had ataxic gait and tachypnea at 34 months of life. Their MRI revealed low signal intensity in the midbrain and pons at T1 weighted imaging. They died at 43 months and 41 months of life, respectively. We report these cases with a brief review of the related literature.

A Case of Aseptic Meningitis Following Administration of Intravenous Immunoglobulin in A Patient with Idiopathic Thrombocytopenic Purpura

Intravenous immunoglobulin(IVIG) has been widely used to treat idiopathic thrombocytopenic purpura in childhood. Aseptic meningitis has been reported as a rare complication of IVIG therapy. This report is on an 11 year-old boy with ITP who suffered from aseptic meningitis following the administration of IVIG. He was given 1 g/kg of IVIG for 2 days, and on the fourth day after the administration of IVIG, he experienced headache, vomiting and fever. Cerebrospinal fluid showed 400/mm3 white cells with 96% segmented neutrophils and 1% lymphocytes. The symptoms subsided within 8 days of admission.

A Case of Hypoglossia-Hypodactylia with t(3q;19p)

Hypoglossia-hypodactylia syndrome is a congenital disease which is characterized by severe micrognathia, hypoglossia and various anomalies of extremities. This was first reported as 'aglossia congenita' back in 1932 by Rosenthal, and has been reported many times ever since, but has never been reported in Korea. Hall first used the term 'hypoglossia-hypodactylia syndrome', and classified it as one of the oromandibular-limb hypogenesis syndromes. According to the studies, most of the cases are sporadic, and this is known to be due to the dominant mutant gene. Etiology is still unknown, but a number of theories have been proposed, such as intrauterine damage and vascular distruptive mechanism. We report a case on hypoglossia-hypodactylia syndrome in a male neonate with karyotype showing 46,XY,t(3;19)(q22;p12) with the review of the associated literatures.