No abstract available.
Diagnosis, Differential* ; Parkinson Disease, Secondary*

Sjogren-Larsson Syndrome is a rare disorder chracterized by congenital icthyosis, spastic diplegia or tetraplegia and mental retardation. The inheritance is autosomal recessive, due to deficiency of alcohol dehydrogenase activity of fatty acid matabolism. We have experienced a case of Sjogren-Larsson Syndrome in a 16 month-old male children who have dried thick skin, developmental delay, and spastic diplegia. He also has glistening spot on macula area with irregular high voltage slow wave (2-3Hz) on electroencephalography. From Biopsy finding, Lamella ictyosis with acanthosis and hyperkeratosis in epithelium is shown. He was much improved clinically for skin lesion and developmental status with etretinate supplement. We report the case with brief review of related literatures.
Acitretin ; Alcohol Dehydrogenase ; Biopsy ; Cerebral Palsy ; Child ; Electroencephalography ; Epithelium ; Etretinate ; Humans ; Infant ; Intellectual Disability ; Male ; Quadriplegia ; Sjogren-Larsson Syndrome* ; Skin ; Wills

The effect of dietary zinc deficiency on the enzymatic components of free radical defense system was observed in the skin of rats. We measured the concentration of serum zinc and the enzymatic activities of CuZn superoxide dismutase(CuZn SOD), glucose-6-phosphate dehydrogenase(GGPDH) and glutathione reductase (GSH-RD). The serum zinc level was sig nificantly lower in the zinc-deficient group compared to the zinc-supplemented group after 8 weeks of consuming the diet(P<0.01). CuZn SOD activity was not different between the two groups after 4 weeks. The Zn deficient group showed the significantly decreased activity of G6PDH after 4 and 8 weeks of consuming the diet(P<0.01). The activity of GSH-RD was increased in the zinc-deficient group compared to the supplemented group after 4 weeks of consuming the diet(P<0.01), but after 8 weeks the activity was not different between the two groups. From the results obtained, it could be concluded that GSH-RD may contribute to the oxygen free radical defense system in zinc deficiency in the earlier weeks of consum ing the zinc-deficient diet.
Animals ; Diet ; Glucose-6-Phosphate ; Glucosephosphate Dehydrogenase ; Glutathione Reductase ; Oxygen ; Rats* ; Skin* ; Superoxides ; Zinc*

We present herein a case of generalized pustular psoriasis in a 17-month-old female infant, who showed good response to etretinate therapy. The baby showed generalized pustular eruptions with high fever and marked leukocytosis preceded by annular scaly erythematous and annular pustular lesions. Etretinate was given in a dose of 10mg(0.75mg/kg/day) for 10 days, and then tapered to 7mg, 5mg, and 4mg in every 10 to 13 days. Clinical improvement was noted after 7 days' treatment and almost all of the skin lesions including erythroderma were cleared after 6 weeks' treatment. She is now maintained with 3mg of etretinate without recurrence.
Acitretin* ; Dermatitis, Exfoliative ; Etretinate* ; Female ; Fever ; Humans ; Infant* ; Leukocytosis ; Psoriasis* ; Recurrence ; Skin

No abstract available.
Hepatitis* ; Measles*

No abstract available.
Humans ; Liver Cirrhosis* ; Liver* ; Sepsis* ; Vibrio cholerae* ; Vibrio*

To investigate the mechanism of monocrotaline-induced pulmonary arterial hypertension, authors performed immunohistochemical study using antibody to von Willebrand factor(vWF), cell kinetic study using 5-bromodeoxyuridine and ultrastructural study after single subcutaneous injection of monocrotaline(MCT) to Wistar rats. The results of this study demonstrated that the expression of vWF by pulmonary endothelial cells was markedly increased from day 3 until 2 months after MCT injection. The labeling index of pulmonary microvessel endothelium began to increase after six days and was maximal on the third weeks, and thereafter it remained slightly increased above basal level. Electron microscopic study revealed attachment of inflammatory cells an platelets to endothelium from 6 hours and degranulation of attached platelets 24 hours after MCT injection. Evidences of endothelial injury began to appear from 12 hours after MCT injection. Evidences of endothelial injury began to appear from 12 hours and was maximal after 48 hours. From the third day, ultrastructural change of cell regeneration and hypertrophy began to appear and was continuosly observed until 2 months. In addition, we evaluated the changes in the number of pulmonary neuroendocrine cells using antibody to gastrin releasing peptide but it demonstrated no change until 2 months suggesting no role of neuroendocrine cells in the development of pulmonary hypertension of Wistar rats at early stage. In conclusion, the results indicate that pulmonary hypertension by MCT injection is due to increased vascular resistance caused by vasoconstriction and hyperplasia of endothelium with musculariz ation of the pulmonary arterioles induced by endothelial dysfunction and some biologic substances released form endothelium and platelets.
Rats ; Animals

We report a case of type V hyperlipoproteinemia and xanthoma eruptivum associated with diabetes mellitus in a 12-year-old female. She had diffuse erythematous yellowish papules on the extremities and trunk with itching. The laboratory findings showed increased serum blood glucose, cholesterol and triglyceride, increasing pre- and chylomicron bands on electrophoresis of lipoprotein. The biopsy specimen of an erythematous yellowish colored papule on the trunk showed characteristic findings of xanthoma.
Biopsy ; Blood Glucose ; Child ; Cholesterol ; Diabetes Mellitus* ; Electrophoresis ; Extremities ; Female ; Humans ; Hyperlipoproteinemia Type V* ; Lipoproteins ; Pruritus ; Triglycerides ; Xanthomatosis*

The computed tomographic (CT) and clinical findings in 9 patients with serologically confirmed Japanese encephalitis were reviewed. In 6 patients with prominent pyramidal and extrapyramidal symptoms and signs, the main CT finding was low density areas in the thalamus, internal capsule, basal ganglia, and midbrain. Low density areas were compatible with the anatomical distribution of pathologic lesions in Japanese encephalitis. Diffuse low density in the periventricular white matter was evident in 5 of 6 cases, with ventricular compression in 2 cases, between the 1st and 7th day of illness. There was enlargement of the third and lateral ventricles and cortical sulci dilatation in 3 cases on or after the 21st day of illness. In once CT scan obtained on the 10th day of illness, diffuse low density in the periventricular white matter was observed with enlargement of lateral ventricles and cortical sulci dilatation. Gyral enhancement was noted in 3 of 4 cases within 4 days after the onset of symptoms. There was a limited correlation between the clinical and CT findings.
Asian Continental Ancestry Group* ; Basal Ganglia ; Dilatation ; Encephalitis, Japanese* ; Humans ; Internal Capsule ; Lateral Ventricles ; Mesencephalon ; Thalamus ; Tomography, X-Ray Computed

We describe 7 patients with sleep related periodic leg movements(SRPLM) associated with variable neurological diseases, including focal thoracic cord lesions(4 cases), idiopathic Parkinson's diseased case), recurrent aseptic meningitis(1 case), and subacute sensory polyneuropathy(l case). Four patients with SRPLM associated with thoracic cord lesions also had paraparesis, suggesting involvement of pyramidal tract in the genesis of SRPLM. All the other three patients had dysesthesia, and at least two of them had restless legs syndrome. SRPLM of a patient with a thoracic schwannoma disappeared completely after surgical removal of tumor. One patient with intramedullary thoracic cord lesion and one asociated with Parkinson's disease showed marked improvement of SRPLM after levodopa treatment. Damage to the pyramidal tract or abnormal sensory inputs seem to activate lumbosacral generator selectively. Such neuronal systems are suspected to be modulated by. The dopaminergic neuronal system and the neuronal structures related to sleep.
Dopaminergic Neurons ; Humans ; Leg* ; Levodopa ; Neurilemmoma ; Neurons ; Paraparesis ; Paresthesia ; Parkinson Disease ; Pyramidal Tracts ; Restless Legs Syndrome