Bipotent progenitors for T and natural killer (NK) lymphocytes are thought to exist among early precursor thymocytes or liver lymphocytes. The identification of such a progenitor population or mature NK cells in such organs remains undefined. Here we report the identification of a novel receptor of NK cells, p58 (HLA class I-specific inhibitory receptors), in fetal thymocytes and fetal liver lymphocytes. Our finding suggests the NK cells mature in the developmental stage during feta1 ontogeny. Flow cytometric analysis revealed p58 positive cells in thymocytes or in fetal liver lymphocytes and reverse transcription PCR also showed amplification of p58 RNA. The result of single stranded conformational polymorphism (SSCP) showed it discriminates one or two base pair differences of the p58 gene. Although the question still remains as to whether the expression of p58 is due to the NK cells or natural T cells, it is clear the p58 is expressed in fetal thymocytes or liver lymphocytes. And SSCP analysis using appropriate sets of primers used in this study, is helpful to study the diversity of p58.
Base Pairing ; Killer Cells, Natural ; Liver* ; Lymphocytes* ; Polymerase Chain Reaction ; Polymorphism, Single-Stranded Conformational ; Reverse Transcription ; RNA ; T-Lymphocytes ; Thymocytes*

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.
Adenylyl Cyclases ; Basal Ganglia ; Brachydactyly ; Brain ; Calcium ; Child ; Cyclic AMP ; Emergency Service, Hospital ; Female ; Glucagon ; Gonadotropins ; Humans ; Hypophosphatemia, Familial ; Intellectual Disability ; Male ; Neck ; Phenotype ; Phosphorus ; Pseudohypoparathyroidism* ; Tetany

Reconstructions of extensive composite scalp and cranial defects are challenging due to high incidence of postoperative infection and reconstruction failure. In such cases, cranial reconstruction and vascularized soft tissue coverage are required. However, optimal reconstruction timing and material for cranioplasty are not yet determined. Herein, we present a large skull defect with a chronically infected wound that was not improved by repeated debridement and antibiotic treatment for 3 months. It was successfully treated with anterolateral thigh (ALT) free flap transfer for wound salvage and delayed cranioplasty with a patient-specific polyetheretherketone implant. To reduce infection risk, we performed the cranioplasty 1 year after the infection had resolved. In the meantime, depression of ALT flap at the skull defect site was observed, and the midline shift to the contralateral side was reported in a brain computed tomography (CT) scan, but no evidence of neurologic deterioration was found. After the surgery, sufficient cerebral expansion without noticeable dead-space was confirmed in a follow-up CT scan, and there was no complication over the 1-year follow-up period.

Paratuberculosis (PTB) is caused by Mycobacterium avium subsp. paratuberculosis (MAP) and is one of the most widespread and economically important diseases in cattle. After birth, calves are raised with natural breast feeding without separation from their mothers in most Korean native cattle (Hanwoo breed) farms. Vertical transmission of PTB has been reported, but the exact PTB infection route has not been revealed in Hanwoo farms. Calves of MAP seropositive dams were tested for MAP presence and MAP antibodies in feces and tissues. MAP was detected in calf tissues by using polymerase chain reaction. Expressions of genes reported to be prognostic biomarkers of MAP infection changed in both calves and cows (p < 0.05). Expression of two genes (HGF and SERPINE1) were significantly decreased in MAP-infected cattle and their offspring (p < 0.01). The results suggest that biomarker gene expression profiles can be useful in detecting early stage MAP infection. Based on the results, complete eradication of MAP may be possible if accurate diagnostic methods to detect infected calves are added to the current PTB eradication strategy, which, because infected individuals are likely to develop into fecal MAP shedders at any time, includes isolation of new born calves and feeding sterilized colostrum.
Agriculture* ; Animals ; Antibodies ; Asymptomatic Infections ; Biomarkers* ; Breast Feeding ; Cattle* ; Colostrum ; Feces ; Humans ; Mothers ; Mycobacterium avium subsp. paratuberculosis ; Paratuberculosis* ; Parturition ; Polymerase Chain Reaction ; Transcriptome

Congenital atrichia is an anomaly characterized by congenital absence of hair in varying degrees as well as the ypopigmentation & poor development of the abnormal residual hair on the scalp, eyebrows, eyelashes & body. Two cases of congenital atrichia in siblings were reviewed. The first case was associated with congenital megacolon and skin biopsy of scalp (esp. parietal region) was non-specific. The second case was associated with bilateral inguinal hernia. Congenital atrichia is inherited by autosomal dominant or recessive. Acording to the limited pedigree data, our cases were X-linked recessive inheritance We report these cases with a review of related literatures.
Biopsy ; Eyebrows ; Eyelashes ; Hair ; Hernia, Inguinal ; Hirschsprung Disease ; Humans ; Pedigree ; Rabeprazole ; Scalp ; Siblings* ; Skin ; Wills

Congenital atrichia is an anomaly characterized by congenital absence of hair in varying degrees as well as the ypopigmentation & poor development of the abnormal residual hair on the scalp, eyebrows, eyelashes & body. Two cases of congenital atrichia in siblings were reviewed. The first case was associated with congenital megacolon and skin biopsy of scalp (esp. parietal region) was non-specific. The second case was associated with bilateral inguinal hernia. Congenital atrichia is inherited by autosomal dominant or recessive. Acording to the limited pedigree data, our cases were X-linked recessive inheritance We report these cases with a review of related literatures.
Biopsy ; Eyebrows ; Eyelashes ; Hair ; Hernia, Inguinal ; Hirschsprung Disease ; Humans ; Pedigree ; Rabeprazole ; Scalp ; Siblings* ; Skin ; Wills

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.
Adrenal Cortex Hormones* ; Aneurysm ; Arteries ; Child ; Coronary Vessels ; Echocardiography ; Fever ; Humans ; Immunoglobulins, Intravenous ; Methylprednisolone ; Mucocutaneous Lymph Node Syndrome* ; Prednisolone ; Retreatment

The purpose of this study was to analyze and evaluate therapeutic results of stereotactic evacuation of hematoma, urokinase irrigation and external ventricular drainage(EVD) in comatose patients who had hypertensive supratentorial intracerebral hemorrhage. The authors carried out stereotactic evacuation of hematoma and external ventricular drainage in the consecutive 45 cases who were admitted to the Department of Neurosurgery, Chosun University Hospital. from Sep. 1990 to Jun. 1993. The therapeutic results were as follows : 1) The peak age incidences were 6th to 7th decades. The incidence was higher in female than male. The age was not related to the prognosis. 2) The hematoma was located at the basal ganglia in 37 cases, and the thalamic area in 8 cases. The prognosis was poorer as the area of hematoma extended more wider and deeper. 3) The volume of hematoma ranged from 8 to 155 ml. The prognosis of the patient was unfavorable in large volume of hematoma. 4) Intraventricular hemorrhage(IVH) occured in forty cases(89.9%) and mortality rate was 45%. The mortality rate was increased in cases with IVH than in case without it. 5) The mortality rates based on the Glasgow coma scale(GCS) on admission were 50% in GCS score 4 group, 50% in GCS score 5 group, 40% in GCS score 6 group, 27% in GCS score 7 group. Cases of low Glasgow coma scale on admission showed high mortality rate. 6) The overall mortality rate was 42%. These results suggest that stereotactic evacuation of hematoma and EVD decreased the mortality rate when compared with conservative treatment and conventional craniotomy in the literatures.
Basal Ganglia ; Cerebral Hemorrhage* ; Coma* ; Craniotomy ; Drainage* ; Female ; Glasgow Coma Scale ; Hematoma* ; Humans ; Incidence ; Intracranial Hemorrhage, Hypertensive ; Male ; Mortality ; Neurosurgery ; Prognosis ; Urokinase-Type Plasminogen Activator

PURPOSE: We compared the reproducibility of 201Tl and 99mTc-sestamibi (MIBI) gated SPECT measurement of myocardial function using the Germano algorithm. MATERIALS AND METHODS: Gated SPECT acquisition was repeated in the same position in 30 patients who received 201Tl and in 26 who received 99mTc-MIBI. The quantification of end-diastolic volume (EDV), end-systolic volume (ESV), and ejection fraction (EF) on 201Tl and 99mTc-MIBI gated SPECT was processed independently using Cedars quantitative gated SPECT software. The reproducibility of the assessment of myocardial function on 201Tl gated SPECT was compared with that of 99mTc-MIBI gated SPECT. RESULTS: Correlation between the two measurements for volumes and EF was excellent by the repeated gated SPECT studies of 201Tl (r=0.928 to 0.986; p<0.05) and 99mTc-MIBI (r=0.979 to 0.997; p<0.05). However, Bland Altman analysis revealed the 95% limits of agreement (2 SD) for volumes and EF were tighter by repeated 99mTc-MIBI gated SPECT (EDV: 14.1 ml, ESV: 9.4 ml and EF: 5.5%) than by repeated 201Tl gated SPECT (EDV: 24.1 ml, ESV: 18.6 ml and EF: 10.3%). The root mean square (RMS) values of the coefficient of variation (CV) for volumes and EFs were smaller by repeated 99mTc-MIBI gated SPECT (EDV: 2.1 ml, ESV: 2.7 ml and EF: 2.3%) than by repeated 201Tl gated SPECT (EDV: 3.2 ml, ESV: 3.5 ml and EF: 5.2%). CONCLUSION: 99mTc-MIBI provides more reproducible volumes and EF than 201Tl on repeated acquisition gated SPECT. 99mTc-MIBI gated SPECT is the preferable method for the clinical monitoring of myocardial function.
Humans ; Perfusion* ; Technetium Tc 99m Sestamibi ; Tomography, Emission-Computed, Single-Photon*

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.
Adrenal Cortex Hormones ; Etretinate ; Extremities ; Female ; Foot ; Hand ; Humans ; Isotretinoin ; Laser Therapy ; Nevus, Sebaceous of Jadassohn* ; Parturition ; Pruritus ; Skin