Severe dapsone hypersensitivity syndrome in a child.

Dapsone (4,4'-diaminodiphenylsulfone, DDS), a potent anti-inflammatory agent, is widely used in the treatment of leprosy and several chronic inflammatory skin diseases. Dapsone therapy rarely results in development of dapsone hypersensitivity syndrome, which is characterized by fever, hepatitis, generalized exfoliative dermatitis, and lymphadenopathy. Here, we describe the case of an 11-year-old Korean boy who initially presented with high fever, a morbilliform skin rash, generalized lymphadenopathy, hepatosplenomegaly, and leukopenia after 6 weeks of dapsone intake. Subsequently, he exhibited cholecystitis, gingivitis, colitis, sepsis, aseptic meningitis, disseminated intravascular coagulation, syndrome of inappropriate antidiuretic hormone secretion, pneumonia, pleural effusions, peritonitis, bronchiectatic changes, exfoliative dermatitis, and acute renal failure. After 2 months of supportive therapy, and prednisolone and antibiotic administration, most of the systemic symptoms resolved, with the exception of exfoliative dermatitis and erythema, which ameliorated over the following 4 months. Agranulocytosis, atypical lymphocytosis, aseptic meningitis, and bronchiectatic changes along with prolonged systemic symptoms with exfoliative dermatitis were the most peculiar features of the present case.

Clinical characteristics of children with 2009 pandemic influenza A (H1N1) admitted in a single institution.

PURPOSE: This study aims to investigate the clinical characteristics of children diagnosed with the novel influenza A (H1N1) in the winter of 2009 at a single medical institution. METHODS: Out of 545 confirmed cases of influenza A (H1N1) in children, using the real time RT-PCR method at Kosin University Gospel Hospital from September to December of 2009, 149 patients and their medical records were reviewed in terms of symptoms, laboratory findings, complications and transmission within a family. RESULTS: Median age of subjects was 7 years (range: 2 months-18 years). New cases increased rapidly from September to reach a peak in November, then declined rapidly. Most frequently observed symptoms were fever (96.7%), cough (73.2%), rhinorrhea (36.9%) and sore throat (31.5%). Average body temperatures on the 1st, 2nd and 3rd hospital day were 38.75±0.65℃, 38.08±0.87℃ and 37.51±0.76℃, respectively. Complete blood counts and biochemical tests performed on the first admission day showed within the reference values in most cases. Of the 82 patients with simple chest radiography, 18 (22%) had pneumonic lesions; multi-focal bronchopneumonia in eleven, single or multi-segmental lobar pneumonia in five, and diffuse interstitial pneumonia in two patients. All of the 149 patients improved from their symptoms and discharged within 9 days of admission without any late complication. CONCLUSION: Children with 2009 pandemic influenza A (H1N1) at our single institution displayed nonspecific symptoms and laboratory findings, resembling those of common viral respiratory illnesses, and did not appear to develop more severe disease.

Differential gene expression profiles of the olfactory bulb after nasal obstruction in neonatal rats.

OBJECTIVE: Microarray technique is a useful tool to identify functional gene candidates. In this study, we evaluated the gene expression profiles in the olfactory bulbs of normal rats and naris-occluded rats using the gene microarray technique. STUDY DESIGN AND METHODS: To induce atrophic change in the olfactory bulb, we performed a unilateral nasal obstruction by electronic cauterization on postnatal day 1 rats. Differential gene expression profiles of the nasal obstruction group and the normal control group at postnatal day 35 were analyzed with a DNA microarray. RESULTS: Microarray revealed 41 genes that were upregulated at least 2-fold in the nasal obstruction group compared with the control group. Among these upregulated genes, increased expression levels of 20 functional genes were confirmed by semiquantitative reverse transcription-polymerase chain reaction. CONCLUSION: This study examines candidate genes associated with the development, apoptosis, and signal transduction of the olfactory bulb. These results may explain the fact that blockage of airflow by inflammation and nasal polyps causes deprivation of olfactory functions in vivo.

Increased expression of glycoprotein 340 in the ethmoid sinus mucosa of patients with chronic sinusitis.

OBJECTIVES: To investigate the expression and distribution of glycoprotein 340 (gp340), a secretory glycoprotein, in normal human sinus mucosa and inflammatory sinus mucosa and evaluate the possible effects of gp340 on the development of chronic sinusitis. Glycoprotein 340 was identified as a key element in the innate host defense mechanism on many mucosal surfaces and is directly involved in defense functions aimed at clearing gram-positive and gram-negative bacteria. DESIGN: Prospective study. SETTING: Tertiary academic institution. PATIENTS: Normal sinus mucosa was obtained from the ethmoid sinus mucosa of 8 patients with blowout fractures undergoing endoscopic reduction. Inflammatory sinus mucosa was taken from 25 patients with chronic polypoid sinusitis during endoscopic sinus surgery. INTERVENTION: Semiquantitative reverse transcription-polymerase chain reaction (RT-PCR), immunohistochemical analysis, and Western blotting were performed. MAIN OUTCOME MEASURES: The expression level and distributional pattern of gp340 in normal and inflammatory sinus mucosa were analyzed. RESULTS: Transcripts of the gp340 gene were detected in all human sinus tissues analyzed by RT-PCR. Immunohistochemical analysis revealed that gp340 is mainly localized in submucosal gland of both normal and inflammatory sinus mucosa. Semiquantitative RT-PCR and Western blot analysis showed the increased expression levels of gp340 in the inflammatory sinus mucosa compared with the normal sinus mucosa. CONCLUSION: These results suggest that gp340 may play a constitutive role in nasal defense and may be up-regulated in response to inflammation, participating in antimicrobial defense in chronic sinusitis.

Over-expression of neuropeptide urocortin and its receptors in human allergic nasal mucosa.

OBJECTIVES: Urocortin (UCN) is a member of the corticotropin releasing factor (CRF) neuropeptide family. UCN act as locally expressed proinflammatory factor and induce mast cell degranulation, cytokine secretion, and trigger vascular permeability, which are mediated by CRF receptors in peripheral tissues. Considering its functional roles, UCN and its receptors may play a role in the pathogenesis of allergic nasal mucosa. Therefore, we investigated the expression profile and distribution of UCN and CRF receptors in normal and allergic nasal mucosa. METHODS: Reverse transcriptase-polymerase chain reaction, immunohistochemistry, and Western blotting were applied to the normal and allergic nasal mucosa. RESULTS: The expression levels of UCN and CRF receptors were increased in allergic nasal mucosa in comparison with normal nasal mucosa. In normal nasal mucosa, UCN and CRF receptors were restricted to the vascular endothelium of submucosal cavernous sinusoids where faint staining was found. However, in allergic nasal mucosa, UCN was expressed in small vessels distributed in lamina propria and the vascular endothelium of cavernous sinusoid located in submucosa. Many scattered positive cells were also found in allergic nasal mucosa, probably UCN-positive leukocytes. CRF receptors were also localized in the vascular endothelium of small vessels and cavernous sinusoid. CONCLUSIONS: These results indicate that UCN may play a role in the regulation of vascular swelling in normal nasal mucosa. Moreover, in allergic nasal mucosa, increased expression levels of UCN and its receptors may contribute to increased mucosal swelling and vascular permeability, playing an important role in the pathogenesis of allergic rhinitis.

D2-40 immunohistochemical assessment of lymphangiogenesis in normal and edematous sinus mucosa and nasal polyp.

OBJECTIVE: The lymphatic system plays an important role in the maintenance of tissue fluid homeostasis, which facilitates interstitial protein transport. Until recently, the lymphatic system of the sinonasal mucosa has been relatively poorly studied. The authors aimed to investigate the distributional and quantitative changes of the lymphatic vessels in inflammatory sinus mucosa and nasal polyps in comparison with healthy sinus mucosa using D2-40 antibody. METHODS: Immunohistochemistry and Western blotting with D2-40 antibody were applied to normal and edematous ethmoid sinus mucosa and nasal polyps. The number, size, and length densities of lymphatic vessels were evaluated using tissue sections and whole mount preparations. RESULTS: Most lymphatic vessels in normal and edematous ethmoid sinus mucosa were distributed in the subepithelial layer. Some of these vessels were dilated, whereas others were compressed and had a slit-like lumen. No D2-40 positive vessels were found in samples of nasal polyps. Lymphatic vessels showed no statistically significant difference in their number, size, or length density between normal and edematous ethmoid sinus mucosa. Western blot also showed no differences in their expression levels. CONCLUSION: These findings indicate that lymphangiogenesis does not occur in edematous ethmoid sinus mucosa, which may not reuptake interstitial fluid efficiently in inflammatory conditions, resulting in the formation of mucosal edema in chronic inflammation.