A Case of Chronic Pneumonitis of Infancy in Pediatric Interstitial Lung Disease

The interstitial lung diseases(ILD) comprises a large, heterogeneous group of disorders characterized by derangement of alveolar walls and alveolar capillary unit causing restrictive pulmonary dysfunction and disordered gas exchange. It is rare, especially in children, and the opinions on investigation and treatments are controversial. The classification of adult ILD is applied to that of children. But considering occurrence during the developing state of lung and immune systems, we should be more cautious because the clinical manifestations and prognosis will be different from those of adults. In 1995, Katzenstein named a unique type of ILD, chronic pneumonitis of infancy (CPI) for the first time. CPI is characterized by marked alveolar septal thickening, striking type II pneumocyte hyperplasia, and eosinophilic alveolar exudate containing numerous macrophages. We report a 14 month-old girl, who had continuing respiratory difficulty and cyanosis after upper respiratory infection, could not sustain oxygen saturation and was finally diagnosed as CPI by histologic examination.

A Case of Acute Necrotizing Encephalopathy

This disease predominantly affects infants and young children living in Japan and Taiwan, and manifests itself as acute encephalopathy following viral infections. The hallmark of this encephalopathy is multifocal, symmetric brain lesions affecting bilateral thalamus, brainstem tegmentum, cerebral periventricular white matter and cerebellar medulla, which can be visualized by computed tomography and magnetic resonance imaging. Both the gray and white matter are involved, with neuropathological evidence of local breakdown of the blood-brain barrier. The prognosis was poor in the 1980s, but has improved recently. A characteristic combination of focal neurologic signs is often recognized as the sequelae. We experienced a case of a 7-month-old male infant who had been transferred to our hospital for comatous mentality and clonic seizure of both arms and legs. We report this case with a review of the related literatures.

A Clinical Study of Adenoviral Respiratory Infection in Children

PURPOSE: Adenoviral respiratory infection can develop a life threatening condition similar to severe bacterial pneumonia. Despite adequate intensive care, this infection progresses to acute respiratory distress syndrome and causes permanent lung damage in some patients. In this study, we analyzed clinical features and long-term follow-up clinical data of this infection in children. METHODS: Forty-seven cases of inpatients were diagnosed as adenoviral respiratory tract infection by viral culture of nasal aspirates or histopathological diagnosis at Samsung Medical Center during the period from February 1995 to July 1998. We reviewed medical records retrospectively. RESULTS: During this study, 1301 cases of acute respiratory tract infection were investigated, of which 47 cases were confirmed as adenoviral infection. Age ranged from 2 months to 6 years. Initial symptoms were productive cough(100%), fever(91%), dyspnea(62%), diarrhea(40%), and conjunctival injection(30%). Thirty-nine cases(83%) were pneumonia and 7 cases(15%) were bronchiolitis. The radiologic findings were pneumonic consolidation(60%), effusion(36%), infiltration(19 %), and atelectasis(13%). Five cases developed acute respiratory distress syndrome and 3 cases expired. In 31 cases of recovered patient, follow-up evaluation was done at a minimum of 3 months(mean duration 9.9+/-9.3 months). Under the follow-up evaluation, 13 patients(42%) showed physical and radiologic findings that consisted with bronchiolitis obliterans or bronchiectasis. CONCLUSION: Because of severe clinical manifestations and pulmonary complications, early diagnosis, adequate management, and long-term follow-up are needed for adenoviral respiratory tract infection.

A Case of Branchio-Oto-Renal Syndrome

Branchio-oto-renal(BOR) syndrome is an autosomal dominant disorder. The pathophysiology of this syndrome is unknown. BOR syndrome has a wide range of clinical manifestations affecting the branchial, auditory and renal systerns. Associated abnormalities of the face, lacrimal duct, palate and ureters have also been described. However, the major clinical findings associated and/ or ear pits, and renal anormaly. We experienced a case of a 15-day-old rnale newborn who had visited our hospital for deformed auricle and atresia of external auditory canal found at birth. We report this case with a review of the related literatures.

Egg white-specific IgG western blotting in children with atopic dermatitis / 천식및알레르기

BACKGROUND AND OBJECTIVE: Specific IgE to food allergen is associated with atopic dermatitis, but it does not always show good clinical correlation. It has been suggested that IgG may be partly involved in allergen-induced reaction. This study was designed to investigate the clinical significance of specific IgG antibody to egg white (EW) in atopic dermatitis patients who showed improvement with egg elimination diet. METHOD: Eleven atopic dermatitis patients who responded to egg elimination diet were selected. They were classified into two groups; group I (n=5) with positive specific IgE to EW and group II (n=6) with normal levels of serum specific IgE. Two volunteers with no allergic diseases were enrolled in the control group. EW-specific IgG western blotting was performed with patient's serum and purified protein extracted from EW. RESULTS: IgG western blotting to EW in group I showed bands at 51.8 kD in two patients and bands at 35 kD in the others. In group II, two showed bands at 51.8 kD, and diffuse bands at 35 kD~51.8 kD were found in four patients. There were no bands in the control group. CONCLUSION: Regardless of the presence of specific IgE, specific IgG to EW was detected by western blotting in patients with egg-associated atopic dermatitis, suggesting that specific IgG may play a role in the pathogenesis of atopic dermatitis.

A Case of Ramsay-Hunt Syndrome

Ramsay Hunt Syndrome was initially described by Ramsay Hunt in 1907 as herpes zoster of the geniculate ganglion. The frequency of RHS in patients with herpes zoster has been calculated to be about 1%. RHS affects mostly adults; however a small number of children with herpes zoster oticus have been reported. We describe a case of RHS in a healthy 39/12-year-old boy. A complete peripheral facial palsy on the left side was noted as well as vesicles of herpes zoster type on the pinna of the same side. The analysis of sera confirmed the clinical diagnosis of RHS with a positive IgG-ELISA antibody titier. Treatment of acyclovir was tried for 7 days, starting 2 days after facial palsy was objerved. In addition, oral prednisolon, divided into two doses per day, was administered for 5 days and then tapered to zero over the following 10 days. Complete recovery observed after 1 month.

The Transfer of Maternal IgG subclasses to Full-term Fetus / 소아알레르기및호흡기학회지

PURPOSE: All IgG subclasses such as IgG1, IgG2, IgG3 and IgG4 can be transferred from mother to fetus through the placenta, though the amount of each IgG subclass is different from one another. Maternally acquired immunity might have an important role for the protection against the infections. We studied transplacental passage of IgG subclasses. METHODS: In this study, we observed the transplacental passage of IgG-subclasses in 22 paired samples of maternal and full- term fetal cord sera. Gestational ages varied from 37 to 42 weeks. The concentrations of IgG subclasses were analyzed by radial immunodiffusion method using commercialized Human IgG Subclass Combi kit. RESULTS: The concentrations of IgG subclasses, IgG1, IgG3 and IgG4 in cord sera exceed the maternal concentration, while IgG2 did not. The ratio of serum levels of cord to maternal were 1.330+/-0.067 for IgG1, 0.859+/-0.039 for IgG2, 1.258+/-0.058 for IgG3 and 1.159+/-0.038 for IgG4. CONCLUSION: This result suggested that the placenta may play a selective barrier for passage of IgG2.

Characterization of IL-3, IL-5, GM-CSF-induced Eosinophils from Human CD34+ Cord Blood Cells / 소아알레르기및호흡기학회지

PURPOSE: Eosinophils are cells of the granulocyte lineage that participate in host defence against parasitic disease and mediate allergic inflammation. In this study by using the combination of cytokines IL-3, IL-5 and GM-CSF, we explore the characterization of cultured eosinophils from CD34+ CBCs. METHODS: Mononuclear cells were isolated heparinized umbilical cord blood by Ficoll-paque (1.077 g/ml) density gradient centrifugation method. The CD34-bearing hematopoietic progenitor cells were collected by elution after their adhesion to a magnetic cell sepatation (MACS) column. The CD34+ cells were incubated in RPMI-1640 medium supplemented with 10% fetal bovine serum (FBS) and were cultured in the presence of IL-3, IL-5, and GM-CSF in a 6-well plate bottomed tissue culture plate at 37 degrees C for 7-28 days in humidified 5% CO2 and 95% air atmosphere. For the identification of cultured eosinophils Wright's and Giemsa staining, RT-PCR, Southern blotting and FACS analysis are used. RESULTS: We analyzed the cultured eosinophils Wright's and Giemsa staining, the total cell number of cells increased 50-fold by days 28 of culture. Also, using the sensitive RT-PCR technique, we monitered the appearance of mRNA transcrips of EPO. Identity of each RT-PCR product was confirms by southern blotting with independent gene-specific oligonucleotide probes and we found increasing of hybridization signals for EPO at 7th culture days. In addtion, we identified eosinophils in cultured CD34+ CBCs by flow cytometry. As s results, we succeeded in developing of pure eosinophils efficiently from CD34+ of CBCs in the presence of IL-3, IL-5 and GM-CSF. CONCLUSION: The in vitro growth of CD34+ CBCs may provide a useful system to study growth factor and stage-dependent adhesion molecule expression, as well as function on developing eosinophils.

Beneficial Effect of Verapamil Against Ischemic Acute Renal Failure in Rabbits / 대한신장학회잡지

This study was undertaken to determine whether verapamil protects renal function in rabbits with ischemic acute renal failure. Renal ischemia was induced by clamping bilateral renal arteries for 60 min. One group received intravenously an infusion of verapamil (lmg/kg) for 30 min prior to initiation of renal artery clamping and the other group received equal volume of saline. Renal blood flow was measured with flowmeter before (basal) and 24 hr after ischemia. Serum creatinine level increased 24 hr after ischemia and remained high to 72 hr. When verapamil was pretreated, the level 48 and 72 hr after ischemia was significantly decreased compared with saline insusion. Urine flow was markedly decreased 24 hr after ischemia and remained depressed to 72 hr, but it was significantly increased 72 hr after ischemia in verapa- mil-pretreatment animals as compared with the saline-infusion animals. GFR were markedly reduced 24 hr after ischemia and remained depressed to 72 hr, which was significantly prevented by verapamil pretreatment. Ischemia caused a significant increase in FEVa and a reduction in Uosm, and TcH2O, indicating impairment in urine concentrating ability of tubules, and the impairment was significantly attenuated by verapamil. The uptake of p-aminohippurate in cortical slices was depressed by ischemia, which was significantly prevented by verapamil pretreatment. In salineinfusion animals, renal blood flow was not significantly different between the basal value and that after 24 hr of reflow. Renal blood flow was not significantly altered by verapamil pretreatment. Anoxia/reoxygenation injury in the control renal slices was not significantly prevented by Ca channel blockers. These results suggest that verapamil exerts a protective effect in ichemic acute renal failure, and the beneficial effects may be attributed to effects other than vasodilation. These data also indicate that a reduction in GFR following ischemia does not result from change in renal blood flow.

A case of ulcerative colitis

No abstract available.