Methylprednisolone pulse therapy for massive lymphadenopathy in a child with intravenous immunoglobulin-resistant Kawasaki disease.

Kawasaki disease (KD) is an acute febrile multi-system vasculitis of unknown etiology. The diagnosis is based on clinical features. We describe a case of intravenous immunoglobulins (IVIG)-resistant KD presenting with persistent fever and massive cervical lymphadenopathy associated with mild respiratory distress. The symptoms resolved after methylprednisolone pulse therapy. High-dose pulse steroid may be an alternative therapeutic option in KD which presents with possible life-threatening complications or failure to respond to high-dose IVIG infusion.

Cyclosporin A therapy in a case with hyperimmunoglobulin E and nephrotic syndrome.

We describe the beneficial effects of treatment with cyclosporin A in a 10-year-old boy with hyperimmunoglobulin E and refractory nephrotic syndrome. The patient was initially resistant to steroid therapy with prednisolone alone. Additional therapy with cyclosporin A was then prescribed, effectively reducing levels of serum immunoglobulin E and preventing flare up of nephrotic syndrome.

Risk factors of retinopathy of prematurity in premature infants weighing less than 1600 g.

There are many risk factors contributing to retinopathy of prematurity (ROP). Some are still controversial, including the use of glucocorticoid and intraventricular hemorrhage. Hence, a retrospective study was performed to evaluate the association between the suspected risk factors and ROP in a medical center in southern Taiwan. One hundred fifty-nine infants with birth body weight < 1600 g admitted to our neonatal intensive care unit before the 29th day of life were enrolled into this study. Clinical data were analyzed by means of logistic regression. The prevalence of ROP in all infants (birthweight < 1600 g) is 36.48% (58 of 159) and 59.46% (22 of 37) in extremely low birthweight infants (birthweight < or = 1000 g). One infant with gestational age 32 weeks and birthweight 1420 g developed stage III ROP. Logistic regression revealed six factors to be significant variables. Birthweight < or = 1000 g, intraventricular hemorrhage, sepsis, and use of glucocorticoid or dopamine were risk factors associated with higher incidence of ROP. Supplementation of vitamin E was shown to relate to lower incidence of ROP. This study confirms several risk factors recognized in previous statistical analyses. Sepsis is the most significant factor contributing to ROP. Vitamin E was proven to be effective in prophylaxis of development of ROP. The possibility of development of ROP could not be excluded in infants with gestational age > 32 weeks.

Henoch-Schonlein purpura with hemorrhagic bullae in children: report of two cases.

Henoch-Schonlein purpura (HSP) is the most common form of acute vasculitis primarily affecting children. Clinical features include skin rashes, arthritis, abdominal pain and nephritis. Skin biopsy on immunofluorescence often reveals granular depositions of immunoglobulin A (IgA) and C3 within the walls of the dermal vessels as well as in the connective tissue of the upper dermis. The diversity of skin rashes produces confusion in diagnosis of HSP, especially in the presence of bullous lesions. Bullous lesions are very rare in children with HSP, whereas they often appear in adults with HSP. We report 2 cases of HSP in whom hemorrhagic bullae manifested predominantly. In our report, the skin biopsies of both patients revealed typical leukocytoclastic vasculitis without IgA and complement depositions on direct immunofluorescence studies. Dramatic improvement of clinical symptoms and signs was observed within a few days after corticosteroids were administered. There was neither recurrence nor nephritis in these 2 patients.

Altered p38 mitogen-activated protein kinase expression in different leukocytes with increment of immunosuppressive mediators in patients with severe acute respiratory syndrome.

Severe acute respiratory syndrome (SARS) has spread to a global pandemic, especially in Asia. The transmission route of SARS has been clarified, but the immunopathogenesis of SARS is unclear. In an age-matched case-control design, we studied immune parameters in 15 SARS patients who were previously healthy. Plasma was harvested for detection of virus load, cytokines, and nitrite/nitrate levels, and blood leukocytes were subjected to flow cytometric analysis of intracellular mitogen-activated protein kinases (MAPKs) in different leukocytes. Patients with SARS had significantly higher IL-8 levels (p = 0.016) in early stage, and higher IL-2 levels (p = 0.039) in late stage than normal controls. Blood TNF-alpha, IL-6, and IL-10, and nitrite/nitrate levels were not significantly elevated. In contrast, TGF-beta and PGE(2) levels were significantly elevated in SARS patients. Five of the 15 SARS patients had detectable coronaviruses in blood, but patients with detectable and undetectable viremia had no different profiles of immune mediators. Flow cytometric analysis of MAPKs activation by phospho-p38 and phospho-p44/42 (extracellular signal-regulated kinase) expression showed that augmented p38 activation (p = 0.044) of CD14 monocytes associated with suppressed p38 activation (p = 0.033) of CD8 lymphocytes was found in SARS patients. These results suggest that regulation of TGF-beta and PGE(2) production and MAPKs activation in different leukocytes may be considered while developing therapeutics for the SARS treatment.

Liver abscess in children: a single institutional experience in southern Taiwan.

Liver abscess is uncommon in children. The purpose of this study is to evaluate the predisposing factors, pathogens, duration of hospitalization, and the managements of liver abscess in children. From 1986 to 2001, fifteen children were admitted to our hospital under the diagnosis of liver abscess. Thirteen cases were older than 8 years old and two were younger than one year old. Fever (15/15, 100%) and abdominal pain (13/15, 87%) were the most common symptoms. Twelve patients (80%) had prolonged fever (fever for 7 days or longer before diagnosis). Eleven (73%) cases were cryptogenic in origin. Most of the microorganisms were obtained solely from cultures of pus. Klebsiella pneumoniae was the most common organism isolated (6/15, 40%). Beside administration of antibiotics, percutaneous catheter drainage (PCD) was performed in 11 patients (73%); only one underwent surgical intervention due to poor response to PCD management. All of our patients were surviving after at least one year follow-up. In conclusion, liver abscess should be suspected in the patients with prolonged fever of unknown origin and abdominal pain. PCD combined with adequate antibiotics were sufficient for therapy of liver abscess in most cases. K. pneumoniae was the most common isolated pathogen in southern Taiwan.