To know the efficacy of intravenous gammaglobulin(IVGG) treatment and the resulting outcome at 18 months for acute Guillain-Barr syndrome (GBS) in children, clinical courses of 27 GBS patients who had been treated with IVGG in a dose of 1g/kg/day oyer 2 consecutive days were investigated. One died with respiratory failure and 8 patients received assisted ventilatory care with improvement. All consecutively treated patients responded well to IVGG. The mean plateau period in 26 recovered patients was 8.5 days. At the 6th month evaluation, only one failed to reach grade 2 on GBSSG scales. At the 18th month evaluation, minor motor symptoms such as easy fatigability or mild tremor on writing were remained in 9 patients. 9 patients showed mild to moderate febrile response to IVGG infusion, but no significant side effects were noted. IVGG can be chosen as an initial treatment for acute GBS in children because of its rapid effect, relative safety even in unstable patients and convenience of administration.
Child* ; Humans ; Respiratory Insufficiency ; Tremor ; Weights and Measures ; Writing

Duplication of the urethra within a single penile shaft is a rare anomaly. These can be divided into those that are in the sagittal plane, which is most common, and those that occur side by side. In some cases, the accessory channel is complete, having a separate bladder opening and no communication with the more normal ventral urethra. In other cases, the accessory urethra is incomplete, either ending blindly or communicating with the urethra distal to the bladder neck. We report on a 20-year-old male with incomplete double urethra.
Humans ; Male ; Neck ; Urethra* ; Urinary Bladder ; Young Adult

PURPOSE: Cerebral cortical dysplasias are one of the important causes for epileptic seizures and developmental disabilities in children, particularly in diffuse or bilateral cases. These developmental malformations are generally regarded as a group of neuronal migration disorders, however, the classification system and pathogenetic mechanisms of cortical dysplasias are not yet entirely clear. Even a novel entity, congenital bilateral perisylvian syndrome, characterized by speech delay, pseudobulbar Palsy, intractable seizures, and bilateral perisylvian abnormalities on imaging studies, have rather diverse figures on morphology and symptomatology than initially considered. We have studied the clinical features and correlations of clinical outcomes 3nd magnetic resonance imaging(MRI) findings of bilaterally involved cortical dysplasias. METHODS: 20 cases of bilateral cortical dyspalsias were grouped into three categories on the basis of MRI findings; centroparietal dysplasias(6 cases), diffuse dysplasias with (5) or without white matter lesions(4), and schizencephaly(5). EEGs, motor and language development, epilepsy, and outcomes were reviewed in each groups. RESULTS: Language delay(100%), motor developmental delay(94.7%), motor deficit(65%), epilepsy(40%) were the main reasons for their initial hospital visits. A meaningful word expression was possible at the mean age of 2 years and 2 months(1 to 4 years of age). Hypotonia and spastic motor paralysis were evident in all the cases of diffuse dysplasia with white matter lesions and the schizencephaly groups. All but one case of centroparietal dysplasia showed motor developmental delay. Epilepsies were developed in 8 cases at the mean age of 5 years and 5 months(2 months to 12 years of age) and the seizures were relatively well controlled with anticonvulsants. EEG findings were variable; normal, focal or diffuse abnormalities. High amplitude diffuse fast activities were only noted in the diffuse dysplasia group. CONCLUSION: The severity of neurological deficits and developmental delay had correlated to the size of cortical abnormalities. The epilepsies were relatively well controlled during childhood and the EEG finding of high amplitude diffuse fast activities was specific for the diffuse cortical dysplasias. Bilateral centroparietal dysplasias should be included on consideration of the causes for developmental aphasia.
Anticonvulsants ; Aphasia ; Child ; Classification ; Developmental Disabilities ; Electroencephalography ; Epilepsy ; Humans ; Language Development ; Language Development Disorders ; Magnetic Resonance Imaging* ; Malformations of Cortical Development* ; Muscle Hypotonia ; Muscle Spasticity ; Neuronal Migration Disorders ; Paralysis ; Pseudobulbar Palsy ; Seizures

Histiocytic necrotizing lymphadenitis that causes unusual lymphadenopathy is a newly recognized disease of unknown etiology and may be mistaken for other conditions such as malignant lymphoma and tuberculosis, in which specific treatment will be required. Local lymph node enlargement especially in neck is the only characteristic feature, sometimes accompanied by pain, tenderness, fever and chill like a cold, nausea, vomiting. Laboratory investigations are usually noncontributory with an occasional case showing leukopenia or an elevated erythrocyte sedimentation rate. For further understanding of this disease and helping make diagnosis, clinical features were reviewed in 15 patients (29 lymph node enlargement), who were diagnosed as histiocytic necrotizing lymphadenitis on clinical evaluation (9 patients) or excisional biopsy (6 patients). The results are as follows; Disease was more common in female(1:2) and young patient. It involved left sided neck nodes frequently (67%) and the most common site was suboccipit-al triangle (62%). 47% of patient showed multiple enlargement of lymph nodes and the size of nodes were varied from 8 to 23 mm (mean: 12.4 mm). Most nodes were movable and showed oval in shape. Fever (47%), pain (33%), tenderness (53%), recent history of upper respiratory infection (33%), leukopenia (47%) and increased erythrocyte sedimentation rate (67%) were showed in many cases. Ultrasonogrphic and CT scan showed well defined oval to round lymph nodes enlargement with inhomogenous enhancement. All affected nodes are resolved spontaneously within 3-10 weeks (mean 5.1 weeks) without specific treatment. After reviewing all of above results, it is concluded that histiocytic necrotizing lymphadenitis is self-limiting disease without characteristic clinical features, but some factors like fever, pain, tendrness, leukopenia and increased erythrocyte sedimentation rate with cervical lymphadenopathy in young female patient are thought to be very helpful in diagnosis and in these circumstances, lymph node biopsy must be delayed for 2-3 months.
Biopsy ; Blood Sedimentation ; Diagnosis ; Female ; Fever ; Histiocytic Necrotizing Lymphadenitis* ; Humans ; Leukopenia ; Lymph Nodes ; Lymphatic Diseases ; Lymphoma ; Nausea ; Neck ; Tomography, X-Ray Computed ; Tuberculosis ; Vomiting

No abstract available.
Hepatorenal Syndrome*

Polyamines are closely related to cell growth and differentiation and increased levels of urine polyamines (UPs) has been reported in various human cancers. However, there were few reports on changes of UPs in patients with cervical carcinoma. We investigated the clinical utility of UPs as well as serum squamous cell carcinoma (SCC) antigen in cervical carcinoma. The association of pretreatment SCC antigen and UPs with clinicopathologic parameters was assessed in 478 patients with cervical carcinoma. SCC antigen was measured by radioimmunoassay and UPs by enzymatic assay method. The prognostic significance of pretreatment SCC antigen and UPs, and the relationship between pretreatment and posttreatment SCC antigen and UPs according to treatment modalities were analyzed. There was a trend of increased level of UPs with cancer progression, whereas significant difference of SCC antigen value was found with cancer progression. Among various clinicopathologic parameters, tumor size and macroscopic lymph node metastasis were associated with pretreatment SCC antigen and UPs level as well. Increased pretreatment SCC antigen level (>2.0ng/ml) and UPs level (>45 micromol/g creatinine) had significant impact on survival. Multivariate analysis revealed that pretreatment SCC antigen, lymph node metastasis and tumor size were independent prognostic factors on survival in the same stage patients. SCC antigen levels decreased after radiotherapy and neoadjuvant chemotherapy. In patients treated by radiation, response status was associated with postradiation SCC antigen, which showed a good correlation with survivals. UPs positivity and SCC antigen positivity in 42 recurrent cervical cancers were 64.7% and 57.9%, respectively. Pretreatment SCC antigen, combination of SCC antigen and UPs, tumor size, macroscopic lymph node metastasis and invasion depth were correlated with recurrent or residual cervical carcinoma. In conclusion, UPs together with SCC antigen may be used to assess the extent of disease status and to define the prognosis in cervical carcinoma.
Carcinoma, Squamous Cell* ; Drug Therapy ; Enzyme Assays ; Humans ; Lymph Nodes ; Multivariate Analysis ; Neoplasm Metastasis ; Polyamines* ; Prognosis ; Radioimmunoassay ; Radiotherapy

No abstract available.
Humans ; Infant, Newborn* ; Leukocytes*

Keratosis punctata of the palmar creases (KPPC) is rare skin condition characterized by punctiform hyperkeratotic pits confined to the palmar and digital creases. Although this condition has been regarded as a variant of classical punctate keratoses, there are some differences between classical punctate keratosis and KPPC. We herein report a case of KPPC in a 22-year-old man who had numerous, tiny, hyperkeratotic pits limited to the palmar creases of both hands with typical histologic findings.
Hand ; Humans ; Keratosis* ; Skin ; Young Adult

No abstract available.
Hepatitis B virus* ; Hepatitis B* ; Hepatitis* ; Humans

Hypertensive encephalopathy is characterized by an acute, severe rise in blood pressure associated with headache, nausea, vomiting, altered mental status, and focal neurologic deficits, and rapid improvement after control of blood pressure. An eight-year old boy had been admitted with symptoms of red urine, fever, headache, convulsion, and visual blurring of vision. Blood pressure was 140/90mmHg, and CSF exam revealed WBC 8/mm3, glucose 83mg/dL, protein 106mg/dL. Serum C3 was 8mg/dL, C4 17mg/dL, ASO 1,024 Todd units. Brain CT showed non specific findings, however, MRI revealed symmetric high signal intensity lesions on T2WI over the parieto-occipital areas bilaterally. These lesions had been completely resolved on the following MRI 4 weeks later.
Blood Pressure ; Brain* ; Fever ; Glomerulonephritis* ; Glucose ; Headache ; Humans ; Hypertensive Encephalopathy* ; Magnetic Resonance Imaging* ; Male ; Nausea ; Neurologic Manifestations ; Seizures ; Vomiting