Prevalence and allergens of allergic rhinitis in children and adolescents in Gwangju

PURPOSE: Allergic rhinitis (AR) is one of the most common childhood diseases in Korea. In this study, we aimed to investigate the prevalence and allergens of childhood AR in Gwangju. METHODS: From April 2013 to September 2013 in Gwangju, skin prick test and questionnaire survey were performed targeting 2,330 children in total (350 kindergartners, 930 elementary school students, 589 middle school students, and 461 high school students). RESULTS: The overall prevalence of AR was 23.5% (female 21.3%, male 26.1%). According to age groups, prevalence of AR was 14.9% (female 10.7%, male 19.8%) in kindergartners, 24.5% (female 18.8%, male 29.4%) in elementary school students, 23.3% (female 18.1%, male 27.6%) in middle school students, 26.2% (female 27.7%, male 23.2%) in high school students. The most common allergen was Dermatophagoides pteronyssinus (79.7%), followed by Dermatophagoides farina (72.2%), birch (14.0%), alder (12.0%), Japanese hop (9.2%), Alternaria (8.9%), cat fur (7.2%), Japanese cedar (6.9%), ryegrass (6.6%), willow (6.0%), pine (5.4%), oak (4.9%), mugwort (4.3%), orchard grass (4.0%), sheep fescue (3.7%), fat hen (3.4%), ragweed (3.4%), and maple (3.4%). CONCLUSION: This research figures out the prevalence and the detailed allergens of AR in Gwangju children. We suggest that more vegetation data of Japanese cedar should be surveyed in recent future.

Aseptic Meningitis Associated with Intravenous Immunoglobulin Therapy for Myasthenia Gravis : A Report of Two Cases / 대한소아신경학회지

Intravenous immunoglobulin (IVIG) is widely used to treat various neurologic or autoimmune disorders in childhood. Aseptic meningitis has been reported as a rare complication of IVIG therapy. We describe two girls, aged 2 and 8 years, with myasthenia gravis who suffered from aseptic meningitis after an administration of high-dose IVIG. Any other etiologic agent were identified in neither case. Both patients recovered completely within a few days and left no sequelae. To our knowledge, these are the first such reported cases in children with myasthenia gravis.

A Case of Mollaret's Meningitis Associated with Benign Pineal Cyst / 대한소아신경학회지

Mollaret's meningitis is a recurrent aseptic meningitis with characteristic clinical features and Mollaret cells in cerebrospinal fluid(CSF). We describe a case of Mollaret's meningitis in a 3-year-old boy who presented with three episodes of aseptic meningitis within a 4-month period. Each episode was characterized by sudden onset of meningeal irritation followed by spontaneous remission in several days. He was free of neurological symptoms between the episodes. In the acute phase of each episode, his CSF showed polymorphonuclear pleocytosis with normal protein and glucose concentrations. In addition, some epithelial cell clusters in the CSF were evident during the third episode. No pathogenic microorganisms were identified in the CSF or the blood cultures. Brain MRI revealed a benign pineal cyst, 0.8 cm in diameter, and epithelial cell clusters were supposed to represent ruptured cystic walls and recurrent episodes of aseptic meningitis were triggered by spontaneous rupture of the cyst. Our case appears to support "spontaneous rupture of epidermoid cysts in the central nervous system" as one of the etiologies of Mollaret's meningitis.

A Case of Langerhans Cell Histiocytosis Preceding with Central Diabetes Insipidus / 대한소아내분비학회지

A 3-year-old girl presented with polydipsia and polyuria for last 2 years. Her fluid intake was 7~8 L/day, and urinalysis showed low osmolality and specific gravity. Central diabetes insipidus (DI) was diagnosed by a water deprivation test. Intranasal 1-desamino-8-D-arginine vasopressin relieved her symptoms and normalized urinary concentrations. A T1-weighted MRI scan revealed a symmetrical thickening of the central part of the pituitary stalk. Six months after the diagnosis of central DI, she developed papular skin lesions on her forehead. The lesions were surgically removed, and histologically classified as Langerhans cell histiocytosis (LCH). We concluded that thickening of the central part of the pituitary stalk might represent the first manifestation of LCH clinically presenting with central DI. In children with central DI, special attention should be paid to the appearance of the pituitary stalk using MRI for the various manifestations of LCH in the central nervous system.

Congenital insensitivity to pain with anhidrosis: a case report

Congenital insensitivity to pain with anhidrosis (CIPA) is a very rare genetic disorder of the peripheral nervous system characterized by recurrent episodes of unexplained fever, generalized anhidrosis, insensitivity to pain and temperature, and accompanied by self-mutilating behavior and mental retardation. We report on a 16 month-old boy with CIPA who exhibited these characteristic clinical features. A sural nerve biopsy revealed markedly reduced numbers of unmyelinated and small myelinated fibers, consistent with the characteristic features of CIPA.

Bilateral Cortical Dysplasias : MRI Findings and Clinical Aspects / 대한소아신경학회지

PURPOSE: Cerebral cortical dysplasias are one of the important causes for epileptic seizures and developmental disabilities in children, particularly in diffuse or bilateral cases. These developmental malformations are generally regarded as a group of neuronal migration disorders, however, the classification system and pathogenetic mechanisms of cortical dysplasias are not yet entirely clear. Even a novel entity, congenital bilateral perisylvian syndrome, characterized by speech delay, pseudobulbar Palsy, intractable seizures, and bilateral perisylvian abnormalities on imaging studies, have rather diverse figures on morphology and symptomatology than initially considered. We have studied the clinical features and correlations of clinical outcomes 3nd magnetic resonance imaging(MRI) findings of bilaterally involved cortical dysplasias. METHODS: 20 cases of bilateral cortical dyspalsias were grouped into three categories on the basis of MRI findings; centroparietal dysplasias(6 cases), diffuse dysplasias with (5) or without white matter lesions(4), and schizencephaly(5). EEGs, motor and language development, epilepsy, and outcomes were reviewed in each groups. RESULTS: Language delay(100%), motor developmental delay(94.7%), motor deficit(65%), epilepsy(40%) were the main reasons for their initial hospital visits. A meaningful word expression was possible at the mean age of 2 years and 2 months(1 to 4 years of age). Hypotonia and spastic motor paralysis were evident in all the cases of diffuse dysplasia with white matter lesions and the schizencephaly groups. All but one case of centroparietal dysplasia showed motor developmental delay. Epilepsies were developed in 8 cases at the mean age of 5 years and 5 months(2 months to 12 years of age) and the seizures were relatively well controlled with anticonvulsants. EEG findings were variable; normal, focal or diffuse abnormalities. High amplitude diffuse fast activities were only noted in the diffuse dysplasia group. CONCLUSION: The severity of neurological deficits and developmental delay had correlated to the size of cortical abnormalities. The epilepsies were relatively well controlled during childhood and the EEG finding of high amplitude diffuse fast activities was specific for the diffuse cortical dysplasias. Bilateral centroparietal dysplasias should be included on consideration of the causes for developmental aphasia.

Reversible brain MRI Finding in Hypertensive Encephalopathy with Poststreptococcal Acute Glomerulonephritis / 대한소아신경학회지

Hypertensive encephalopathy is characterized by an acute, severe rise in blood pressure associated with headache, nausea, vomiting, altered mental status, and focal neurologic deficits, and rapid improvement after control of blood pressure. An eight-year old boy had been admitted with symptoms of red urine, fever, headache, convulsion, and visual blurring of vision. Blood pressure was 140/90mmHg, and CSF exam revealed WBC 8/mm3, glucose 83mg/dL, protein 106mg/dL. Serum C3 was 8mg/dL, C4 17mg/dL, ASO 1,024 Todd units. Brain CT showed non specific findings, however, MRI revealed symmetric high signal intensity lesions on T2WI over the parieto-occipital areas bilaterally. These lesions had been completely resolved on the following MRI 4 weeks later.

High Dose of Amphotericin B in Intralipid Emulsion-based Delivery System in Immunocompromised Children with Invasive Fungal Infections

PURPOSE: Fungal infections are an important cause of morbidity and mortality in patients with hematologic malignancies. The therapy of choice in documented or suspected invasive fungal infections has been intravenous Amphotericin B (AmB). Adverse effects such as fever, chils, thrombophlebitis, nausea or vomiting are common. A more serious adverse effect is potential renal impairment. As AmB administration mixed with Intralipid (AmB/Intralipid) was reported to decrease AmB toxicity without a concomitant loss of antifungal efficacy, we studied the efficacy and side effects of long-term administration of AmB/Intralipid in leukemic children with invasive fungal diseases. METHODS: AmB/Intralipid was administered in seven leukemic children (male, 3; female, 4) who had invasive fungal infections between July 1994 and March 1997. RESULTS: AmB/Intralipid was administered at a mean concentration of 1.45mg/kg/day for a mean of 58.1 days with cumulative dose of 3.01g. Excluding 2 patients who succumbed to the underlying leukemia, 4 out of 5 remaining patients remained free of both fungal infection and leukemia. Chills associated with AmB/Intralipid were found 13 times in 4 patients. One patient could not continue the administration because of the chills on the 45th day of AmB/Intralipid. Renal and hepatic impairment greater than Grade II toxicity was found in each case, respectively. The other 6 patients showed mild elevation from the baseline, but remained within the normal limits. CONCLUSION: Long-term, high-dose AmB/Intralipid therapy can be safely and effectively used in immunocompromised children with invasive fungal infections.

A Case of Moyamoya Syndrome Associated with Hereditary Spherocytosis / 대한소아신경학회지

Moyamoya is a chronic cerebrovascular disease characterized by progressive stenosis or occlusion of the terminal parts of both intermal carotid arteries with telangiectatic vascular network of collateral circulation at the base of the brain and leptomeningeal arteries. The etiology and pathophysiology of this disease are still unknown. Although the idiopathic presentattion is the commonest, moyamoya disease has also been reported in several hereditary or acquired clinical conditions including neurofibromatosis, sickle cell anemia, tuberculous meningitis, atherosclerosis, and following radiation therapy to the head. The term moyamoya disease should be reserved for those cases in which the characteristic angiogrphic pattern is idiopathic; moyamoya syndrome is used when the underlying condition is known. We have experienced a case of coexistence of moyamoya syndrome and hereditary spherocytosis in a 6-year-8-month-old girl who presented with right-sided hemiparesis and pallor. A cerebral angiogram revealed occlusion of proximal portion of left middle cerebral artery and abnormal collateral network. The peripheral blood smear and osmotic fragility test disclosed hereditary spherocytosis. To our knowledge, the coexistence of moyamoya syndrome and hereditary spherocytosis has not been documented. We report here the case and the brief review of related literatures. Further studies are needed to clarify the intimate relationship between the two diseases.

A Clinical Study of Fanconi's Anemia / 대한소아혈액종양학회지

BACKGROUND: Fanconi's anemia(FA) is an autosomal recessive disease characterized by aplastic anemia and congenital malformations. As up to 30% of patients have no physical stigmata, the modern diagnosis of FA rests on chromosomal breakage of patient's cells induced by chemical clastogens such as diepoxybutane(DEB) or mitomycin-C(MMC). METHODS: We reviewed the clinical manifestations, laboratory findings, diagnostic methods, treatment and outcome of 6 patients diagnosed to have a FA at the Chonnam University Hospital for the last 6 years. RESULTS: Six cases(16.2 %) were found to have FA among 37 aplastic children who were diagnosed during the same period. The mean age at diagnosis was 6.3 years which was the usual onset of hematologic findings. All patients had features of aplastic anemia, and had one or more anomalies, such as low birth weight, hyperpigmentation, cafeau-lait spots, mental retardation, developmental delay, peculiar face(broad nasal bases, epicanthal folds, micrognathia), polydactyly, microcephaly, short stature, and dislocation of hip. We found increased breaks in cultured cells with DEB and MMC in 5 cases tested. The median duration of follow-up was 30 months. Oxymetholone and prednisolone treatment was partially beneficial in three cases. Immunosuppressive treatment with ALG/ATG was not successful in two cases tried. Four cases are living now, without transfusion in three. Two patients were died of disseminated fungal infection and transplant-related problems, respectively. CONCLUSIONS: Fanconi's anemia should be sought carefully in any patients with aplastic anemia because the prognosis, treatment modality, and the approach to bone marrow transplantation are quite different when the hematologic disorder is inherited rather than acquired.

Disseminated Fusarial Infections in Two Children with Acute Leukemia / 대한소아혈액종양학회지

Disseminated Fusarium infection has rarely been encountered in neutropenic cancer patients. The clinical features include fever, positive blood cultures, severe myalgias, disseminated ecthyma gangrenosum-like skin lesions, ocular symptoms and multi-organ-system involvement. The mortality rate reported so far exceeded 70% despite antifungal treatments. We report here the first two cases of disseminated fusarial infections in Korea. The first patient contracted pneumonia during severe neutropenic period following consolidation chemotherapy for acute myelogenous leukemia(AML). Fusarium oxyshrum was cultured from bronchoalveolar lavage fluid. The patient remains free of fungemia after successful antifungal treatment of high-dose amphotericin B and terbinafine. The second patient had relapsed AML and suffered from disseminated fusarial infection involving lungs with characteristic skin lesions. The patient succumbed to refractory leukemia and the fungal infection. A brief review of the literature ensues with the case report.

Benefits of High Dose Intravenous Gammaglobulin in Children with Severe Guillain-Barr Syndrome / 대한소아신경학회지

To know the efficacy of intravenous gammaglobulin(IVGG) treatment and the resulting outcome at 18 months for acute Guillain-Barr syndrome (GBS) in children, clinical courses of 27 GBS patients who had been treated with IVGG in a dose of 1g/kg/day oyer 2 consecutive days were investigated. One died with respiratory failure and 8 patients received assisted ventilatory care with improvement. All consecutively treated patients responded well to IVGG. The mean plateau period in 26 recovered patients was 8.5 days. At the 6th month evaluation, only one failed to reach grade 2 on GBSSG scales. At the 18th month evaluation, minor motor symptoms such as easy fatigability or mild tremor on writing were remained in 9 patients. 9 patients showed mild to moderate febrile response to IVGG infusion, but no significant side effects were noted. IVGG can be chosen as an initial treatment for acute GBS in children because of its rapid effect, relative safety even in unstable patients and convenience of administration.

Four Cases of Steroid-Induced Lipodystrophy

No abstract available.