A Case of Barley Anaphylaxis due to Infant's Weaning Food / 소아알레르기및호흡기학회지

Barley is not a rare dietary content in Asian food but very few cases of anaphylactic reactions to barley have been reported. Furthermore, most reports are in adult patients with allergic reactions to beer containing barley components or occupational asthmatics known as 'baker's asthma'. We present a case of 11-month old boy who experienced an episode of anaphylaxis with generalized urticaria, periorbital swelling, wheeze and dyspnea after eating baby food containing barley, chestnut, glutinous rice, nonglutinous rice, within 30 minutes. Laboratory tests demonstrated high total serum IgE (531 kU/L) and revealed high level of IgE sensitizations to wheat (64.3 kU/L), barley (35.2 kU/L), rice (1.39 kU/L). Using the IgE-immunoblot assay, 7, 14, 25, and 26 kDa of IgE binding bands were identified and high degree of IgE cross-reactivity between barley and wheat extracts were revealed using IgE-immunoblot inhibition and ELISA-inhibition tests in this case.

Acute Optic Neuritis in Children : Clinical Features, Visual Outcome, Recurrence Rate, and Subsequent Evolution into Multiple Sclerosis / 대한소아신경학회지

PURPOSE: Acute optic neuritis is an inflammatory optic neuropathy, and rarely occurs in children. Childhood-onset optic neuritis is different from adult-onset optic neuritis in terms of clinical and prognostic aspects. We investigated the clinical characteristics and visual prognosis related to the steroid therapy in children with acute optic neuritis. In addition, we evaluated the recurrence rate of acute optic neuritis and subsequent evolution into multiple sclerosis. METHODS: The medical records of ten children, who were previously admitted for acute optic neuritis at the Department of Pediatrics of Ajou University Medical Center from January 1995 to April 2004, were retrospectively reviewed. Nine patients were treated with intravenous methylprednisone pulse therapy, 0.5-1.0 g/day, for 3-5 days followed by an oral prednisone therapy. To 30 months after discharge, we followed up a long-term visual prognosis, recurrence rate and subsequent evolution into multiple sclerosis. RESULTS: There were 4 boys and 6 girls and the mean age of onset was 10 years. Bilateral optic neuritis was noticed in 8 children and 9 children presented with papillitis. Etiology of acute optic neuritis was idiopathic in 50% of patients. Of the remaining cases, it was related with acute disseminated encephalomyelitis(30%), sinusitis(10%), and neuromyelitis optica(10%). Orbital MRI showed optic nerve enhancement and/or enlargement and optic disc protrusion in 15 of the 18 examined eyes. Visual evoked potential (VEP) was abnormal in 12 of the 16 examined eyes; P100 response was totally attenu ated in 6 eyes, and in the others the amplitude of P100 response was normal but the latency was delayed. On follow-up VEP at 1 month after steroid pulse therapy, the amplitude of P100 response was normalized in all, but the latency was still delayed in 60% of the patients. A short-term visual acuity after treatment with steroid was normally recovered in 14 of 15 involved eyes. Acute optic neuritis recurred in one of the nine patients who were treated with high dose methylprednisone pulse therapy, but there was no subsequent development of multiple sclerosis during the follow up. CONCLUSION: The most common cause of acute optic neuritis in children is idiopathic, and presents as bilateral papillitis. On this study, a short-term visual prognosis after high dose methylprednisone pulse therapy is extremely good, but there was no significant difference in long-term visual prognosis at 6 months after treatment with steroid. Acute optic neuritis in children does not usually recur, and does not evolve into multiple sclerosis.

Review of Chromosomal Analyses Performed in a Single Hospital / 소아과

PURPOSE: Chromosomal aberration has an effect on the lives of both patients and families. Peripheral blood chromosomal analysis is useful to make a diagnosis and counsel the patient with chromosomal abnormality. We studied the cases with peripheral blood chromosomal analyses and investigated the positivity of peripheral blood chromosomal analysis. METHODS: We analyzed 650 patients with chromosomal analyses in Ajou University Hospital during the past 10 years. We used a high-resolution banding technique to diagnose the chromosomal aberrations. RESULTS: The male to female ratio of the patients was 1.1 : 1. Among 650 patients, 108 patients had chromosomal aberrations(16.6%). Fifty seven out of 313 patients with the congenital anomalies or dysmorphologies had chromosomal aberrations. twenty six out of 166 patients with hematologic disorders had chromosomal aberrations. Fifteen out of 109 patients with developmental delay had chromosomal aberrations. Four out of 24 patients with seizures had chromosomal aberrations. Six out of 39 patients with the aberrant perinatal histories had chromosomal aberrations. CONCLUSION: Peripheral blood chromosomal analysis is an important diagnostic method in managing patients with chromosomal aberrations. Further advanced chromosomal staining and molecular genetic methods will raise the detection rate of chromosomal aberration and be useful in managing patients with chromosomal abnormalities.

A Four-year Follow-up Case of Oligomeganephronia Detected Early by School Screening Urinalysis

Oligmeganephronia is congenital hypoplasia of kidney with renal pathology showing very small number of nephrons with compensatory hypertrophy of the remaining glomeruli. A 7- year-old girl was referred to our nephrology clinic due to hematuria detected on school screening urinalysis and diagnosed as chronic renal failure and oligomeganephronia on renal biopsy. We are reporting the clinical and histomorphometric changes for the four years follow-up with review of literatures.

A Case of Homocystinuria Complicated with Systemic Deep Vein Thrombosis and Venous Infarction of Thalamus / 대한소아신경학회지

Homocystinuria is an autosomal recessive inherited disorder of methionine metabolism. The most common cause of homocystinuria is cystathione-beta-synthase deficiency, which has the characteristic clinical features such as ectopia lentis, Marfanoid skeletal changes, mental retardation, and vascular thromboembolic events such as deep vein thrombosis, cerebral infarction, pulmonary embolism, and myocardial infarction. The thromboembolic vascular events occur in 20-50% of untreated patients with homocystinuria at the age of 15, and could be associated with vasculopathy related mortality in 20% of untreated patients before the age of 30. Therefore, homocystinuria is one of the important cause of stroke in children and young adults. Only 2 cases of homocystinuria were reported in Korea; one without vasculopathy and the other with cerebral infarction. Homocystinuria complicated with systemic deep vein thrombosis is first reported in Korea. We report a 13 year old female with homocystinuria complicated with severe systemic deep vein thrombosis and venous infarction of both thalami due to thrombosis of vein of Galen and internal cerebral vein.