The distribution of transforming growth factor-α (TGF-α) and epidermal growth factor (EGF) in developing mouse embryos of gestational age 8 to 15 days was immunohistochemically (ABC method) studied to investigate the differential expression of these growth factors. Paraffin embedded sections were immunostained with antibodies for TGF-α and EGF. Staining of TGF-α was observed in several organs derived from endoderm, mesoderm and ectoderm in 9-day-old mouse embryos, such as in the heart, optic pit, head mesenchyme, neural tube and primitive gut, and the staining became more intense in 10 to 15-day-old mouse embryos. The staining of EGF was seen in the heart and primitive gut derived from mesoderm and ectoderm respectively, in 9-day-old mouse embryos, but it was observed in other organs as well in 10 to 15-day-old embryos although the intensity was weaker. In the development of heart, immunoreactivity for TGF-α was more intense than EGF, which suggests more active involvement of TGF-α. In the lung, TGF-α staining was observed both in the bronchus and lung bud, whereas EGF staining was seen only the bronchus. In the nervous system, TGF-α was expressed more extensively and more intensively than EGF. In the developing skeletal system, TGF-α staining was stronger and the expression was observed at earlier stage compared with EGF. These results indicate that the activity of TGF-α is more potent than EGF in the development of mouse embryo in general, especially, in the development of mouse heart, nervous system, mesenchyme and skeletal system.
Animals ; Antibodies ; Bronchi ; Ectoderm ; Embryonic Structures* ; Endoderm ; Epidermal Growth Factor* ; Gastrula ; Gestational Age ; Head ; Heart ; Intercellular Signaling Peptides and Proteins ; Lung ; Mesoderm ; Mice* ; Nervous System ; Neural Tube ; Paraffin

No abstract available.
Child* ; Humans ; Pheochromocytoma*

We report here several experiences of interphase cytogenetics, using fluorescence in situ hybridization (FISH) technique, for the detection of chromosome aberrations. FISH, using alpha satellite specific probes of 18, X, Y chromosomes, was done in interphase nuclei from peripheral blood of patients with Edwards' syndrome, Klinefelter's syndrome and Turner's syndrome with healthy male and female controls, respectively. The distributions of fluorescent signals in 100 interphase nuclei were well correlated with metaphase findings. Nowadays FISH plays an increasingly important role in a variety of research areas, including cytogenetics, prenatal diagnosis, tumor biology, gene amplification and gene mapping.
Adolescent ; Adult ; Cell Nucleus/*ultrastructure ; Child ; Chromosome Aberrations/*physiology ; Chromosome Banding ; Female ; Humans ; In Situ Hybridization, Fluorescence ; Infant, Newborn ; Interphase/*physiology ; Male

No abstract available.
Paget Disease, Extramammary

Dyskeratosis congenita is a rare genodermatosis of ectodermal dysplasia, which is characterized by the diagnostic triad consisting of reticulated hyperpigmentation, dystrophic nails, and leukoplakia. There is a predisposition to malignancy, particularly at sites of leukoplakia. Bone marrow failure can occur in about a half of the cases. A 16-year-old boy was presented with asymptomatic reticulated pigmentation of the neck and nail dystrophy. The patient also had leukoplakia on the tongue, nasolacrimal duct obstruction and cataract. The histopathological findings taken from the reticulated lesion were consistent with poikiloderma atrophicans vasculare. These clinical and histopathological findings were typical features of dyskeratosis congenita.
Adolescent ; Bone Marrow ; Cataract ; Dyskeratosis Congenita* ; Ectodermal Dysplasia ; Humans ; Hyperpigmentation ; Leukoplakia ; Male ; Nasolacrimal Duct ; Neck ; Pigmentation ; Tongue

No abstract available.
Hamartoma*

No abstract available.
Blood Transfusion, Autologous*

Mauriac syndrome consists of a triad of poorly controlled diabetes, profound growth retardation and hepatomegaly. We experienced a case of Mauriac syndrome in an 18-year-old girl who had poorly controlled diabetes mellitus, short stature, hepatomegaly and central obesity. Also at the time of examination, she had complications of diabetic cataract and nephropathy. Fourteen years prior to admission, she was diagnosed as diabetes mellitus at a hospital. Thereafter, she had been managed with irregular insulin injection. On physical examination at admission, her height was 135cm(<3 percentile) and her weight was 39kg(<3 percetile). She was short and obese. The liver was 5 cm palpable below the right subcostal margin. Her sexual maturation was Tanner stage I. On ophthalmologic examination, the cataracts were observed on both eyes and diabetic retinopathy was absent. Diabetic nephropathy could not be confirrned by kidney biopsy due to her mother's refusal. We studied the hormonal, radiographic and histological abnormalities. The hormonal study was normal and the bone age was by delayed as much as 10 years. The liver biopsy revealed glycogen accumulation in hepatocyte. She was consistent with Mauriac syndrome. She was managed by strict diabetic control with insulin therapy, diabetic diet and intensive education. She was discharged with well controlled blood glucose. Five months later, growth acceleration and sexual maturation have not been observed, but hepatomegaly subsided. (J Korean Pediatr Soc 2000;43-837-841)
Acceleration ; Adolescent ; Biopsy ; Blood Glucose ; Cataract ; Diabetes Mellitus* ; Diet, Diabetic ; Diabetic Nephropathies ; Diabetic Retinopathy ; Disulfiram ; Education ; Female ; Glycogen ; Hepatocytes ; Hepatomegaly ; Humans ; Insulin ; Kidney ; Liver ; Obesity, Abdominal ; Physical Examination ; Sexual Maturation

Erythema annulare centrifugum consists of centrifugally erythematous eruption with central clearing producing arcuate, festooned, and polycyclic patterns. Its etiology is mostly obscure. However, a few of the patients seem to be related to some underlying diseaseas, such as autoimmune diseases, internal malignancies, drugs, or infections and often its course parallels to the process of the underlying diseases. 27 year old female patient showed eruption over the trunk and both extremities, enlarging centrifugally with clearing in the center with frequent upper respiratory tract infections from childhood. On the chest P-A, tumor mass was revealed, and it was confirmed as the hamartoma later. The eruption was disappeared totally 24 hours after surgical removal of the hamartoma. Hence, the hamartoma seemed to be responsible in this patient for the flare-up of the erythema annulare centrifugum, but its pathogenesis is not clearly understood. The diagnosis of the erythema annulare centrifugum was assisted by the histopathological finding.
Adult ; Autoimmune Diseases ; Diagnosis ; Erythema* ; Extremities ; Female ; Hamartoma* ; Humans ; Lung* ; Respiratory Tract Infections ; Thorax

Herpes zoster is known to be more frequent and severe in patients during immunosuppressive treatment and various predisposing factors, such as Hodgkin's disease, advanced tumor stage, local X-ray irradiation, splenectomy and cutaneous anergy. This report presents the case of a 23-year-old woman who developed herpes zoater on two separate occasione during the treatment of systemic lupus erythematosus with immunosuppressive agents (corticosteroid and cyclophosphamide). In each occasion, it was involved by different and multiple dermatomes. Treatment with oral and intralesional corticosteroids administration proved to be beneficial without any complication, although some delayed recovery of the disease was noted.
Adrenal Cortex Hormones ; Causality ; Female ; Herpes Zoster* ; Hodgkin Disease ; Humans ; Immunosuppressive Agents ; Lupus Erythematosus, Systemic* ; Splenectomy ; Young Adult