Cytokine Gene Expression According to Cell Separation of Cord Blood and Bone Marrow / 대한소아혈액종양학회지

PURPOSE: Umbilical cord blood (CB) transplantation is an alternative method instead of allogeneic bone marrow (BM) transplantation. CB transplantation has used grafts with red blood cell (RBC) depletion but previous reports in CB investigated the immune reaction about mononulcear cell separated by density gradient. To study the real immune response in CB transplantation, the experimental group designed the total nuclear cell (TNC) groups by RBC depletion and the mononuclear cell (MNC) groups by Ficoll Hypaque in CB and BM. METHODS: We evaluated the various cytokine gene expression by semiquantitative RT- PCR method after immune stimulation with phytohemagglutinin in cord blood and bone marrow according to cell separation method. RESULTS: 1) All samples of CB and BM expressed IL-2 mRNA. There was no difference in amounts of IL-2 mRNA between CBTNC and CBMNC, between BMTNC and BMMNC. 2) All samples of CB and BM expressed IL-10 mRNA. There was no difference in amounts of IL-10 mRNA between the CBTNC and CBMNC but significantly different between the BMTNC and BMMNC (P<0.05). The amounts of IL-10 mRNA in the BMMNC and CBTNC group showed larger than in the BMTNC group (P<0.05). 3) The expression of IL-4, IFN-gamma was not shown in this study. These results suggest that no difference of IL-2 mRNA between CB and BM may reveal IL-2 as major cytokine gene in CB after immune stimulation. The expression of IL-10 mRNA in CBTNC showed more than in BMTNC group. Conclusion: Our results suggest that CBTNC may contain a lot of cells producing IL-10, as a cytokine of immunoregulatory function, and therefore the immune reaction in CB transplantation may be less apparent than in BM transplantation. The cell component producing IL-10 in CB may be T regulator cell. There were not showed the IFN-gamma and IL-4 mRNA due to short duration of immune stimulation. Therefore, further studies will identify the IL-4 and IFN-gamma mRNA expression after long time stimulation and various cytokine gene expression to certificate the precise immune response after flow cytometry to composed of immune cells in CB and BM.

Two Cases of Pseudohypoparathyroidism / 대한소아내분비학회지

Pseudohypoparathyroidism(PHP) is a genetic disorder characterized by target cell resistance to the effect of parathyroid hormone(PTH). The disorder is classified into type I a, I b, I c and II depending on the phenotype and biochemical findings. In type I a, urinary cyclic AMP and urinary phosphate excretion are not increased after PTH stimulation because of deficient G unit activity in target cells. Deficiency of the G unit is a generalized cellular defect and accounts for the association of other endocrine disorders with type I a PHP. Type I b PHP shows resistance to PTH but not to other hormones and normal phenotypic appearance. In type I c PHP affected children have defect in catalytic unit of adenylate cyclase and in addition to resistance to PTH, resistance to the metabolic effects of TSH, gonadotropins, and glucagon may be detected. Typical appearance of Albright's hereditary osteodystrophy is common in PHP type Ia and Ic. In type II PHP, urinary cyclic AMP response is generated but this does not lead to phosphaturia. We experienced two patients with PHP. One is a 11-year-old girl diagnosed type I a PHP and the other is a 11-year-old boy suspected type I b PHP. They visited emergency room because of tetanic movement. Both patients had no previous history of tetany and showed low serum calcium level, high phosphorus level and high PTH level. The girl had typical features of Albright's hereditary osteodystrophy such as round face, short neck, obese feature, brachydactyly and mental retardation but didn't have basal ganglia calcification on brain CT. The boy showed normal appearance and no mental retardation.

Treatment of Intravenous Immune-Globulin Resistant Kawasaki Disease with Corticosteroids

PURPOSE: To evaluate the efficacy of steroid therapy on prevention of development and progression of coronary artery aneurysm in intravenous immune globulin (IVIG)-resistant Kawasaki disease, we treated three children with high dose of intravenous methylprednisolone followed by low dose oral prednisolone. METHODS: We selected three children with Kawasaki disease who did not repond or who initially responded but soon developed recrudescent fever after retreatment of IVIG (total 4gm/kg). These three patients were treated with high dose methylprednisolone (10mg/kg) intravenously and followed by low dose prednisolone (1mg/kg) orally for 7 days. Echocardiographic evalutions were performed within 8 days of admission (before steroid therapy), at discharge and 1 month after discharge. RESULTS: All three patients showed rapid normalization of clinical symptoms and did not developed significant coronry artery abnormalities. No adverse reaction was observed. CONCLUSIONS: Steroid therapy (mini pulse methylprednisolone and prednisolone therapy) is valuable for patients with Kawasaki disease resistant to intravenous immune globulin therapy.

A Case of Inflammatory Linear Verrucous Epidermal Nevus

Inflammatory linear verrucous epidermal nevus(ILVEN) is a relatively rare skin disorder, characterized by chronic inflammatory psoriasiform pattern. This chronic skin disorder is generally believed to be resistant to various treatments, although some authors have reported success with 13-cis-retinoic acid and 585 nm pulsed laser therapy. We experienced a 15-month aged female ILVEN patient who has been suffered from linear erythematous verrucous pathes mainly located on left inguinal area, hand, and foot with persistent itching sense. This skin lesion have been developed at birth, and more spread out toward right extremity after 1 year old age . We initially treated this skin lesion with topical corticosteroids and oral anti-histamines, but this treatment was not successful. So we retreated this patient with etretinate(oral retinoid) and topical 13-cis-retinoic acids, we found that therapeutic responses to these applications were more remarkable. Now, we closely observe this patient's clinical course further more.