Comparison of the seroconversion rate after primary hepatitis B vaccination and after revaccination of non-responders in full-term infants according to mother's HBsAg seropositivity / 소아과

PURPOSE: This study aimed to identify the true extent of non-responsiveness in full-term infants born from HBsAg-negative or HBsAg-positive mothers and vaccinated against hepatitis B virus (HBV) at 0, 1, and 6 months of age and to evaluate the effect of revaccination among non-responders. METHODS: The study included 716 full-term infants born in 2004-2007. Of 716, 662 infants (A group) were born to HBsAg- negative mothers and 54 infants (B group: 50, except HBsAg-positive infants) were born to HBsAg-positive mothers. All infants were administered DNA recombinant vaccines at 0, 1, and 6 months of age. B group infants received hepatitis B immunoglobulin at birth. Anti-HBs titers were tested at 7-12 and 9-15 months in A and B groups, respectively. Three revaccination doses were administered to non-responders whose anti-HBs titers were under 10 mIU/ml; revaccinated infants were retested at 1-3 months after last vaccination. The association between HBeAg seropositivity of mother and the failure of HBV immunoprophylaxis was evaluated. RESULTS: The seroconversion rates after primary hepatitis B vaccination were higher in A group (94.1%) than in B group (78%, P<0.001). The seroconversion rates were high in revaccinated infants (A group non-responders: 96.9%, B group non- responders: 87.5%). The failure of HBV immunoprophylaxis was significantly associated with maternal HBeAg seropositivity (P<0.001). CONCLUSION: The seroconversion rates after primary hepatitis B vaccination were low in B group infants. Revaccination of non-responders in B group was very effective. Therefore, anti-HBs testing and revaccination of B group is very important. Revaccination of non-responders in A group was also very effective. Thus, testing the immune status of infants born to HBsAg-negative mothers even after primary hepatitis B vaccination should be considered. However, to realize this, further studies on the cost-effectiveness of anti-HBs testing in healthy full-term infants are necessary.

Incidence of hearing loss and importance of risk factors in the neonatal intensive care unit / 소아과

PURPOSE: To assess the incidence of neonatal hearing loss in a neonatal intensive care unit and the relative importance of risk factors for hearing imparement in a neonatal intensive care unit which the Joint Committee on Infant Hearing(JCIH) had recommended. METHODS: One thousand, two hundred and one newborns admitted to the Good Moonhwa Intensive Care Unit from May 2003 to December 2005 were assesed using the automated auditory brainstem response(AABR). The screening was performed on those aged more than 36 weeks and weighing more than 2,200 g. We divided the infants into two groups, 'pass' and 'refer'. The 'refer' group were retested one month later, and if classified as 'refer' during the retest, were referred to a hearing impairment clinic. RESULTS: From the 1,201 neonates, 1,187(98.8 percent) passed the test and 14(1.2 percent) failed. 293(24.4 percent) of the 1,201 neonates had a risk factor for hearing impairment; 282(96.2 percent) passed the test and 11(3.8 percent) failed. The group with risk factors were shown to have a higher incidence of hearing loss(P<0.001). The neonates in the refer group were shown to have a higher incidence of ototoxic drugs(P<0.001), low birth weight(<1,500 g)(P<0.001) and craniofacial anomalies(P=0.007). On the other hand, there were no statistical differences between the pass and refer groups in congenital infection, hyperbilirubinemia, bacterial meningitis, low Apgar scores, prolonged mechanical ventilation and syndromes known to include hearing loss. CONCLUSION: In order to identify hearing-impaired infants within an appropriate period, neonatal hearing screening tests and identification of the risk factors for neonatal hearing loss are important.

A Case of Short Arm Deletion and Long Arm Duplication at Chromosome 3 / 소아과

The long arm duplication of chromosome 3 was reported for the first time in 1966 by Falek et al., and Hirschhorn et al. came to identify the duplication of 3q21--> qter region in 1973. In most cases of duplication 3q syndrome patients, pure duplication of 3qter is believed to be rare and is often reported accompanied with deletion of another segment of the chromosome. Approximately 75 percent of parents of the patient in the meantime have been demonstrated to have unbalanced translocations or inversions of the chromosome. Partial deletion of the distal part of the short arm of chromosome 3 was first reported by Verjaal and De Nef in 1978 and terminal deletion of chromosome 3 (3p25- qter) has been observed in most cases. In karyotyping of chromosomes of immature infants showing the manifestations of flat occiputs, low set ears, hypertelorism, broad nasal roots, thin lips, web necks, hypotonia, hypertrichosis skin, cryptorchidism etc, we experienced a case diagnosed as 46, XY, rec (3) dup (3) (q21) del (3) (p25) inv (3) (p25q21).

Investigation of Automated Neonatal Hearing Screening for Early Detection of Childhood Hearing Impairment / 소아과

PURPOSE: Early diagnosis of congenital hearing loss through the neonatal hearing screening test minimizes language defect. This research intends to identify frequency of congenital hearing loss in infants through neonatal hearing screening test with the aim of communicating the importance of hearing test for infants. METHODS: From May 20, 2003 to May 19, 2004, infants were subjected to Automated Auditory Brainstem Response test during one month of birth to conduct the test with 35 dB sound. Infants who passed the 1st round of hearing test, were classified into 'pass' group whereas those who did not were classified into 'refer' group. Infants who did not 'pass' in the hearing test conducted within one month of birth were subjected to re-test one month later, and if classified as 'refer' during the re-test, they were subjected to the diagnosis for validation of hearing loss by requesting test to the hearing loss clinic. RESULTS: There was no difference among the 'pass' and 'refer' group in terms of form of childbirth, weight at birth and gestational age. In the 1st test, total of 45 infants were classified into 'refer' group. Six among 35 who were subjected to re-test (17%) did not pass the re-test, and all were diagnosed with congenital hearing loss. This corresponds to 0.35% (3.5 per 1, 000) among total number of 1, 718 subjects. CONCLUSION: In our study the congenital hearing loss tends to be considerably more frequently than congenital metabolic disorder. Accordingly, newly born infants are strongly recommended to undergo neonatal hearing screening test.

Modulation of Eosinophilia and Cytokines of Bronchoalveolar Larvage Fluid(BALF) by CpG Oligodeoxynucleotides(ODN) in a Mouse Model of Established Airway Inflammation / 소아알레르기및호흡기학회지

PURPOSE: Asthma is an inflammatory disease of the airways that is induced by Th2 cytokines and inhibited by Th1 cytokines. Oligodeoxynucleotides containing a CpG motif(CpG ODN), as potent inducers of Th1 immunity, are considered promising candidates for immune modulation in asthma. In this study we wanted to investigate the effect of CpG ODN on eosinophilia and cytokines of BALF in a mouse model established airway inflammation and the optimal route(systemic vs mucosal) of CpG ODN. We examined the difference of immunologic responses between CpG ODN and corticosteroids. METHODS: Female BALB/c mice, induced pulmonary allergic inflammation, were treated intranasally or intraperitoneally with CpG ODN and Dexamethasone. Allergen-specific antibody responses, cytokines(IL-4, IL-5, IL-12), and eosinophilic inflammation of the airways were investigated on BALF and splenocyte. RESULTS: CpG ODN effectively induced IL-12 and inhibited IL-4 and IL-5 as well as eosinophilic inflammation when CpG ODN was administered intranasally or intraperitoneally with allergen challenge. Therapy with corticosteroides, while effective inhibiting IL-5 generation, did not induced IL-12 in BALF. CONCLUSION: Systemic or mucosal administration of CpG ODN effectively stimulated the production of Th1 cytokines and suppressed eosinophilic airway inflammation in contrast of corticosteroids and control ODN. Thus, CpG ODN vaccination is a potentially useful approach for immunomodulation of established airway inflammation in a mouse model of asthma.

Chondrodysplasia Punctata(Rhizomelic Type) Associated with Pneumothorax

Chondrodysplasia punctata is a rare congenital syndrome caused by a peroxisomal dysfunction. Chondrodysplasia punctata is classified into four main types-Coradi-Hunermann's type, rhizomelic type, X-linked dominant form and X-linked recessive form. A male patient with this condition was born at 39 weeks gestation, the pregnancy being complicated by polyhydroamnios, breech presentation, and anomalies of congenital limbs. At delivery, there was no activity and no initial crying. Physical examination revealed a flat nose, a short neck, scaled ichthyolytic skin, and bilaterally symmetrical shortening of the upper and lower extremities. Choromosomal analysis revealed a 46, XY karyotype. Radiologic examination disclosed stippling of the cartilage on the epiphyseal regions of the long bones, paravertebral regions, carpal bones and tarsal bones. In additions, a chest x-ray showed right pneumothorax. Chest and endotracheal tubes were inserted. However, the patient died due to respiratory failure at 19 days of life. We report a case of rhizomelic type of chondrodysplasia punctata assocoated with pneumothorax with a brief review of the related literatures.

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.

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.