A Clinical Study of Intravenous Gamma Globulin Re-treatment in Kawasaki Disease

PURPOSE: Intravenous gamma globulin(IVGG) treatment has reduced symptoms and complications in Kawasaki disease(KD). However, fever persisted in 20-30% of the patients, and there are no reliable data on the indication and dosage of IVGG re-treatment. Therefore, we tried to reveal the effectiveness of IVGG re-treatment and to find risk factors in predicting the re-treatment. METHODS: Among 57 patients with typical KD, 47(82.5%) patients were put into group A, which improved after the treatment with standard 2g/kg of IVIG. 10(17.5%) patients were put into group B, which retreated with 1-2g/kg of IVIG due to persistent fever for at least 3 days after standard IVIG therapy. We compared clinical symptoms, laboratory findings and echocardiograms between group A and B, retrospectively. RESULTS: All patients in group B responded IVGG re-treatment and no considerable side effects. The total duration of the fever was significantly longer(P<0.001) and the initial and peak levels of CRP and the peak levels of ESR were significantly higher(P<0.01) in group B compared to group A. Even though leukocytosis, cervical lymphadenitis and coronary artery aneurysm were more frequent, and the levels of serum lipids at admission were lower in group B, without significance. CONCLUSION: IVGG re-treatment appeared to be effective in the treatment of refractory KD, but could not reduce the incidence of coronary artery aneurysm. We concluded it was difficult to predict risk factors for IVGG re-treatment from these data. Further studies are needed to determine the indication and appropriate dosage of IVGG re-treatment.

A Case of Nonsyndromic Paucity of Interlobular Bile Ducts in Down Syndrome

The nonsyndromic paucity of interlobular bile ducts, which belongs to intrahepatic biliary atresia, is characterized by conjugated hyperbilirubinemia, suggesting cholestasis in newborn infants it has little relationship with extrahepatic congenital abnormalities. Pathologic findings through percutaneous liver biopsy show portal changes(duct paucity and fibrosis) and lobular changes(cholestasis, giant cell transformation, extramedullary hematopoiesis and perisinusoidal fibrosis). The overall incidence of intrahepatic biliary atresia may be as rare as 1 in 50,000 to 75,000 live births. Puri et al first described intrahepatic biliary atresia in Down syndrome in 1975 and Kahn et al revealed 17 cases of nonsyndromic paucity of interlobular bile ducts, including 2 cases of Down syndrome in 1986. The only treatment available in patients, with intrahepatic biliary atresia, is symptomatic because it is not necessary to operate. The prognosis of these patients remains quite varied; approximately half of patients will later develop cirrhosis with portal hypertension and will die from liver failure in the first year of life. We report this case with the review of the associated literatures.

Distribution and Expression of Kainate(KA) Receptor Subunits in Moderate Hypoxic Newborn Piglet Brain

PURPOSE: The mechanism of hypoxic damage is mainly intracellular influx of calcium ions through the glutamate ionotropic receptor. This study was performed to determine alterations in distribution and expression of kainate receptor subunits after 1 hour of moderate hypoxia in the newborn piglet brain, as in a condition of mild to moderate perinatal hypoxic-ischemic encephalopathy. METHODS: Ten newborn piglets were ventilated at PaO2 over 80mmHg for 30min. Thereafter, the control group(n=5) was ventilated with 21% oxygen, and hypoxic group(n=5) with 6% oxygen at PaO2 below 25mmHg for 1 hour. Concentrations of protein, ATP and phosphocreatine were determined. The proteins were immunostained with anti-rat GluR6/7 and anti-rat KA2 antibody. RESULTS: Hypoxia(PaO2 20+/-1mmHg) and acidosis(pH 7.06+/-0.09) developed significantly in the hypoxic group compared to the control group(PaO2 104+/-4mmHg, pH 7.44+/-0.03, respectively, Phippocampus, thalamus, hypothalamus>basal ganglia, cerebellum>white matter, and KA2 subunits were ordered : hippocampus, basal ganglia>cerebral cortex>thalamus, cerebellum>hypothalamus, white matter. The distribution of the subunits between the hypoxic group and control group were similar. CONCLUSION: Cerebral cortex, hippocampus and basal ganglia may be the most vulnerable to excitotoxic injury. Kainate receptor subunits did not change after 1 hour of moderate hypoxia.

Distribution and Expression of Kainate(KA) Receptor Subunits in Moderate Hypoxic Newborn Piglet Brain

PURPOSE: The mechanism of hypoxic damage is mainly intracellular influx of calcium ions through the glutamate ionotropic receptor. This study was performed to determine alterations in distribution and expression of kainate receptor subunits after 1 hour of moderate hypoxia in the newborn piglet brain, as in a condition of mild to moderate perinatal hypoxic-ischemic encephalopathy. METHODS: Ten newborn piglets were ventilated at PaO2 over 80mmHg for 30min. Thereafter, the control group(n=5) was ventilated with 21% oxygen, and hypoxic group(n=5) with 6% oxygen at PaO2 below 25mmHg for 1 hour. Concentrations of protein, ATP and phosphocreatine were determined. The proteins were immunostained with anti-rat GluR6/7 and anti-rat KA2 antibody. RESULTS: Hypoxia(PaO2 20+/-1mmHg) and acidosis(pH 7.06+/-0.09) developed significantly in the hypoxic group compared to the control group(PaO2 104+/-4mmHg, pH 7.44+/-0.03, respectively, Phippocampus, thalamus, hypothalamus>basal ganglia, cerebellum>white matter, and KA2 subunits were ordered : hippocampus, basal ganglia>cerebral cortex>thalamus, cerebellum>hypothalamus, white matter. The distribution of the subunits between the hypoxic group and control group were similar. CONCLUSION: Cerebral cortex, hippocampus and basal ganglia may be the most vulnerable to excitotoxic injury. Kainate receptor subunits did not change after 1 hour of moderate hypoxia.

A Case of Parry-Romberg Syndrome in Neonate

Parry-Romberg syndrome(Progressive hemifacial atrophy), described in the last century by Parry(1825) and Romberg(1846), is a very rare disorder characterized by a slowly progressive and self-limited unilateral(rarely bilateral) atrophy of the faces affecting variably the skin, subcutaneous fat tissues, musculature, connective tissue, cartilage and bones. And this disorder is usually accompanied by contralateral Jacksonian epilepsy, trigerminal neuralgia, and changes in the eyes and hair. The onset is slow and progressive, starting at 5-15 years of age and lasting from 2-10 years, ending with the face being "burned out". There are a few cases of this disease which presented during the neonatal period. This disorder seems to affect females more than males, and its etiology and incidence has yet to be determined. Trauma, infection with a slow virus, sympathetic dysfunction, immunological abnormality and cranial vascular malformation are proposed causes. No typical or consistent neuropathologic findings occur. No specific treatment for the syndrome exists; however, various reconstructive surgical procedures can have in reasonably good cosmetic effects, as well as antiinflammatory or immunosuppressive treatment. We report a case of Parry-Romberg syndrome, which was presented at 1 month of age, and has progressd to contralateral hemiparesis.

Retrospective 3-year Clinical Study of Enterobacter Bacteremia in Neonatal Intensive Care Unit

PURPOSE: Enterobacter is one of the important organisms in neonatal intensive care unit. We reviewed the clinical characteristics, underlying diseases, invasive procedures during admission, mortality and antibiotic sensitivity of Enterobacter infection in NICU. METHODS: We retrospectively reviewed 21 neonatal patients whose blood cultures yielded Enterobacter between June 1994 and June 1997 at Dongguk University Hospital. RESULTS: Blood cultures were positive in 62 from 2,025 neonates and 21 was Enterobacter. The clinical spectrums were diverse such as sepsis (85%), pneumonia, disseminated intravascular coagulopathy and necrotizing enterocolitis. The underlying conditions upon admission were composed of prematurity (38%), hyaline membrane disease (38%), jaundice, sepsis and pneumonia. The procedures used during admission were endotracheal intubation (57%), mechanical ventilation (57%), umbilical vessel catheterization, gastric tube inserition, total parenteral nutrition and exchange transfusion. The antibiotic sensitivity was as follows : ampicillin (0%), ceftriaxone (0%), amikacin (55%), gentamicin (85%), ciprofloxacin (100%), imipenem (100%). Overall mortality was 26.5%. Mortality was significantly high in cases of leukopenia (P< or = 0.01), thrombocytopenia (P< or = 0.01) and use of inappropriate antibiotics (P< or = 0.01). CONCLUSION: Enterobacter is an important organism in the cause of nosocomial infection in NICU and has a high rate of mortality. Enterobacter infection was associated with prolonged hospitalization, invasive procedures and preceding antibiotics. Commonly used antibiotics such as penicillin and cephalosporin would be inappropriate for the treatment of Enterobacter infection. We consider the use of gentamicin or imipenem to be far more effective in the initial therapy of Enterobacter infection.