Menkes disease is an X-linked recessively inherited neurodegenerative disorder of copper metabolism leading to death in early childhood. This disease is characterized by low serum, liver and brain Cu levels, whereas the Cu content in nearly all other organs is increased. The main clinical features are irregular kinky hair, characteristic face, progresssive degeneration of the central nervous system, bone changes, arterial rupture and thrombosis, and instability of temperature. We have experienced a case of Menkes disease in a eight months old male patient, who manifested by seizure, developmental delay, hypotonia, characteristic hair and face along with low levels of serum copper and ceruloplasmin. We report the characteristic findings of cranial MRI and MR angiography in this Menke's patient. In cranial MRI, he showed progressive brain atrophy with subdural hemorrhage and effusion. MR angiography revealed tortuosity of vessels clearly obviating necessity of more invasive conventional arteriography in diagnosis of this disease. This study suggested that cranial MRI and especially MR angiography might be helpful in making early diagnosis of this disease.
Angiography ; Atrophy ; Brain ; Central Nervous System ; Ceruloplasmin ; Copper ; Diagnosis ; Early Diagnosis ; Hair ; Hematoma, Subdural ; Humans ; Liver ; Magnetic Resonance Imaging ; Male ; Menkes Kinky Hair Syndrome ; Metabolism ; Muscle Hypotonia ; Neurodegenerative Diseases ; Rupture ; Seizures ; Thrombosis

No abstract available.

Xanthogranulomatous pyelonephritis, an uncommon chronic inflammatory renal disorder of middle-aged women, is rarely seen in childhood. A 10 year-old boy with focal type of this disease in upper and lower pole was experienced. Patient had a intermittent fever and right flank pain without palpable mass. The blood analysis only revealed ESR increase but no anemia, no leukocytosis. There was no history or signs of urinary tract infection or calculi. The urine analysis and culture showed nothing abnormal. An intravenous pyelogram, ultrasonogram, abdominal CT and selective renal arteriography only demonstrated a non functioning upper pole of right kidney. During operation, a gross finding and frozen section strongly showed xanthogranulomatous pyelonephritis and diagnosis was made on histological examination, After nephrectomy, there had been no evidence of disease recurrence during 2 years follow-up period.
Anemia ; Angiography ; Calculi ; Child* ; Diagnosis ; Female ; Fever ; Flank Pain ; Follow-Up Studies ; Frozen Sections ; Humans ; Kidney ; Leukocytosis ; Male ; Nephrectomy ; Pyelonephritis, Xanthogranulomatous* ; Recurrence ; Tomography, X-Ray Computed ; Ultrasonography ; Urinary Tract Infections

To clarify the mechanism of the protective effect of hemodialysis on lipid peroxidation in RBC membrane structures, the level of malondialdehyde (MDA) which is the lipid peroxidation product, and the activities of antioxidant enzymes such as superoxide dismutase (SOD), catalase, and glutathione peroxidase (GSH-Px) were determined before and after hemodialysis in the RBCs from 20 patients with end stage renal disease (ESRD), and from 14 healthy subjects. Before dialysis, MDA levels in the RBCs from the patients with ESRD were higher than those from healthy controls. SOD and catalase activities in the RBCs were lower. After hemodialysis, MDA, SOD, and catalase in the RBCs from the patients with ESRD were normalized. These results indicate that hemodialysis treatment is helpful to protect the peroxidative darnage through normalizing the activities of antioxidant enzymes.
Catalase ; Dialysis ; Erythrocytes* ; Glutathione Peroxidase ; Humans ; Kidney Failure, Chronic* ; Lipid Peroxidation ; Malondialdehyde* ; Membranes ; Renal Dialysis* ; Superoxide Dismutase

PURPOSE: Periventricular leukomalacia(PVL) is associated with the characteristic neurologic sequale of spastic diplegia or quadriplegia, and cortical blindness. So early diagnosis of PVL in the neonatal period is of importance because of the prognostic implications of this lesion. Novotny et al reported that Positive Rolandic Sharp waves(PRS) on the EEGs was a marker of PVL because most infants who had excessive PRS on their EEGs also had PVL on their neurosonography. PRS were present in most neonates at a time when neurosonography showed periventricular echodense lesions, prior to the appearance of cystic PVL. Therefore, the appearance of excessive PRS on the EEG is very useful for the early detection of PVL. The aim of this study is to assess the relationship between the PVL and PRS on the EEGs, to observe the appearance timing of PRS on the EEGs, and to evaluate the risk factors of later neurologic sequale. METHODS: Among the neonates admitted to the Sowha Childern's Hospital between 1989 and June 1993, 25 neonates with cystic PVL on neurosonography or CTscan were the study group. Control group were the age-matched 20 neonates who were admitted at the same time of study period. We reviewed the neurosonographic findings, EEG features, and charts retrospectively. RESULTS: 1) Excessive PRS were observed in 20 cases of study group(80%), whereas 1 case in control group(5%)(p<0.001). 2) The frequency of PRS was not affected by the abnormality of EEG background activity. 3) The frequency of PRS in a 10 minutes epoch was 14.5+11.1 in the study group, whereas 1.57+1.96 in the control group(p<0.001). 4) In most neonates, PRS were present at a time when the neurosonography showed periventricular echodense lesion or cystic PVL. 5) In a follow-up neurosonography, 12 among 17 neonates with localized PVL were normalized and only 5 cases had ventriculomegaly. Whereas, all of 5 extensive PVL had the sequale of ventriculomegaly or cerebral atrophy. 6) In a follow-up neurologic examination, the poorer sequale including spastic diplegia or quadriplegia was associated with the larger, the more extensive, and the subcortical PVL. CONCLUSIONS: 1) Excessive PRS were significantly more observed in the study group than in the control group(p<0.001). 2) The frequency of PRS was not affected by the abnormality of EEG background activity. 3) The prognosis of the neonates with PVL was depended on the size, extent, and location of the PVL rather than the presence or frequency of PRS.
Atrophy ; Blindness, Cortical ; Cerebral Palsy ; Early Diagnosis ; Electroencephalography ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Leukomalacia, Periventricular* ; Neurologic Examination ; Prognosis ; Quadriplegia ; Retrospective Studies ; Risk Factors

No Abstract Available.
Methicillin Resistance* ; Methicillin* ; Methicillin-Resistant Staphylococcus aureus* ; Staphylococcus aureus* ; Staphylococcus*

No Abstract Available.
Vibrio parahaemolyticus* ; Vibrio* ; Virulence Factors* ; Virulence*

No Abstract Available.
Methicillin Resistance* ; Methicillin* ; Methicillin-Resistant Staphylococcus aureus* ; Staphylococcus aureus* ; Staphylococcus*

No Abstract Available.
Vibrio parahaemolyticus* ; Vibrio* ; Virulence Factors* ; Virulence*

Forty six cases of Legg-Calve-Perthes disease were reviewed clinically and roentgenographically in order to determine a satisfactory method of assessing the prognosis and to correlate the clinical and roentgenographical results. 1. The patients were children 3 to 12 years of age, with the peak at 6 years. The average age of visit (or diagnosis) was 6.7 years and that of onset was 6.4 years. 2. The total number of hips involved were 49, three cases being bilateral (6. 5%). Boys predominated girls by a ratio of 4.7:1 3. End results in children below 6 years of age were better than those in children above 6 years. The end results of treatment with the containment method were better than those with the noncontainment method. 4. The hips with excellent result had an average of 2.5mm. of uncovering of the femoral head at the time of diagnosis. The degree of uncovering increased as the roentgenographical result deteriorated, in the poor hips the average uncovering being 9mm. The average for the normal side was 2mm. The uncovering at onset is an accurate guide to the likely end result. 5. The Catterall assessment was of reliable prognostic value. In the excellent group, the majority of the hips belonged to Groups 1 and 2, In the poor group, there were no hips in Group 1 and increasing number of hips belonged to Groups 2,3 and 4. 6. The head at risk judged by Gage's sign, calcification of lateral epiphysis, lateral subluxation of the femoral head, and presence of a horizontal epiphyseal line indicated poor prognosis. 7. There was a close correlation between the severity of clinical features and the severity of residual roentgenographic findings.
Child ; Clinical Study ; Containment of Biohazards ; Diagnosis ; Epiphyses ; Female ; Head ; Hip ; Humans ; Legg-Calve-Perthes Disease ; Methods ; Prognosis