We reported a case of Wilson's disease, which was histologically confirmed by liver biopsy in a 15 year-old boy and made a comprehensive analysis on the sum of 22 cases reported in Korean literature. Although Wilson's disease is still rare, it should be included in the differential diagnosis of chronic liver diseases particularly in children of 5 to 15 years old. For the clinical diagnosis of Wilson's disease, changes in serum ceruloplasmin, plasma copper and urine copper values are most important as well as Kayser-Fleischer ring and family hestory. The serum level of GOT, when elevated, were always higher than GPT. This atypical transaminase profile may be a clue for diagnosis of hepatic diseases by a metabolic derangement. Among various histologic changes of the liver in Wilson's disease, what have diagnostic importance are anisonucleosis, nuclear glycogenosis and Mallory body, all of which may appear in any stages of the disease. In 3 cases only neurologic symptoms such as dysarthria and athetosis were found. Hemolytic anemia was another rare complication.
Child ; Male ; Female ; Humans ; Diagnosis, Differential ; Biopsy

Adreno-hepatic fusion is rare condition defined as adhesion of the liver and right adrenal cortex with close intermingling of the respective parenchyme. It is suggested to be an aging phenomenon, because its incidence is much higher in older age group. Clinically it may pose a problem of operability of the organ involved. We report a case of incidentally found adreno-hepatic fusion in a 49 year old female patient with adenocarcinoma of the sigmoid colon. The segementectomy of VIII segement of the liver was done due to a 6 4 cm sized metastatic nodule of adenocarcioma. Pathological examination of the liver revealed an ovoid shaped, 1 0.5 cm sized adrenal cortical tissue. It was subcapsularly located and about 1cm apart from the metastatic adenocarcinoma with an intervening normal hepatic tissue. The adrenal tissue was mainly composed of zona fasciculata without medullary tissue. In the interphase, the adrenal tissue and liver tissue were admixed closely and partially septated by thin fibrous tissue. There was no inflammatory response to the heterotropically located adrenal tissue and there was no symptom related to the adrenal gland.
Adenocarcinoma ; Adrenal Cortex ; Adrenal Glands ; Aging ; Colon, Sigmoid ; Female ; Humans ; Incidence ; Interphase ; Liver ; Middle Aged ; Zona Fasciculata

Forty-nine lobectomized hepatocellular carcinomas(HCC) were classified according to the gross anatomical features. Because the presence of cirrhosis in the remaining liver has a good clinico-pathological implication, cases of HCC were divided into non-cirrhotic(non-LC) and cirrhotic(LC) groups. In both groups, the tumors themselves belonged to either expanding, focal spreading, spreading or mixed type. Another special type, which has been called a "diffuse type" is added in the LC group with the name of "cirrhotomimetic type" Among 49 cases, 21 belonged to the non-LC group and 28 to the LC group. Most common was expanding type(20 cases, 40.8%), which was followed by spreading(32.7%), focal spreading(16.3%), mixed(6.1%) and cirrhotomimetic(4.1%) types. Expanding type of the LC group was the single most common type(13 cases, 26.5%). The accordance rate of gross typing was 0.94. Tumor masses of the LC group showed a greater tendency of having a fibrous capsule(60.7%) and a lobulated cut surface(82.1%), in contrast to those of the non-LC group (28.6% and 42.9% respectively). The patient's age and the HBsAg seropositivity were not different between the groups and between the types. Increased serum level of AFP was particularly frequent in the spreading type(81.3%) of both groups and in the cirrhotomimetic type(100%).
Carcinoma, Hepatocellular

No abstract available.
Fever* ; Microwaves* ; Perfusion*

Generalized perforating granuloma annulare is a rare variant of granuloma. annulare, which was first described by Duncan et al 1) in 1973. A 21-year-old female had hundreds of 3 to 5mm papules over the whole body, which were mainly distributed in the extremities and trunk since three months ago. Individual papules developed a central umbilication with a peripheral collarette of scale. Histopathologic examination showed an epidermal perforation and transepidermal elimination of necrobiotic material. The epidermal perforation communicated with areas of necrobiotic collagen surrounded by palisading granulorna in the dermis. We treated her with oral prednisolone and dapsone, but there was no clinical improvement.
Collagen ; Dapsone ; Dermis ; Extremities ; Female ; Granuloma Annulare* ; Granuloma* ; Humans ; Prednisolone ; Young Adult

No abstract available.
Animals ; Embryonic Structures* ; Fertilization in Vitro* ; Humans* ; Mice* ; Quality Control*

No abstract available.
Basement Membrane* ; Epithelium*

Treatment of chronic localized osteomyelitis is the same as the other osteomyelitis, that is, curettage and debridement of all dead tissue which is called saucerization. After saucerization there leaves empty cavity and autogenous bone graft has been used for filling the cavity in lower extremity hecause of weight bearing and avoiding fracture. We treated seven cases of chronic localized osteomyelitis with one-staged saucerization and cancellous chip allograft and retrospective analysis was done. All healed without complication and no recurrence was ohserved. Most orthopaedic surgeons are not willing to use allograft on the site of osteomyelitis because of the fear of recurrence. But. we think that as far as complete removal of infected and dead tissue, allograft could he a good solution in terms of rapid remodelling and early weight hearing. Another advantages of allograft are to get a large amount of bone and no damage of iliac apophysis. In summary, one-staged saucerization and cancellous chip allograft would he very useful treatment for chronic localized osteomyelitis.
Allografts* ; Curettage ; Debridement ; Hearing ; Lower Extremity ; Osteomyelitis* ; Recurrence ; Retrospective Studies ; Transplants ; Weight-Bearing

A study has been made on the skin response of mouse hind limb to radiation to evaluate the difference of skin response to superfractionation and conventional fractionation schedules, and to optimize the time interval betweenfractions and the dose per fraction in the superfractionated irradiation. 96 mouse hind limbs were dvided into 12groups and were irradiated with 10 consecutive fractions by intervals of 6, 12 or 24 hours and dose per fractionsof 400, 500, 600 or 700 rads. The skin changes of the irradiated hind limb were observed for 30 days and the skin response were analyzed. The results are as follows; 1. There was no significant difference of early skin response along the time interval, from 6 to 24 hours, up to 600 rads per fraction. 2. Mean duration to maximum skin reaction in superfractionation (15.48±2.80 days) is shorter than in conventional fractionation (18.05±3.20 days)by 2.57 days. (p<0.05). 3. Optimum time interval betwen fractions in superfractionation may be 6 hours or less. 4. Optimum dose per fraction in superfractionation may be 500 rads or less.
Animals ; Appointments and Schedules ; Extremities ; Mice ; Skin

Benign epiepsy of childhood with centrotemporal spikes (BECCT) is an electroclinical syndrome characterized by noctural seizure that remit spontaneoully before adulthood, and belong to idiopathic age and location related epilepsies. We reviewed the medical records to analyse the seizure the seizure pattern, and also inspect the EEG recording to identify topography of the epileptiform discharge of 24 patient who met the following criteria: 1) presence of nocturnal seizure or partial seizure confined to the face, 2) normal intecual and development, 3) normal background EEG feature, 4) monomormhic spikes or sharp wave that increase in the frequency during sleep if those state were achieved. The result as follow: 1) The age of seizure onset were 3 to 11 years old (7.08 1.89) and male to female ratio was 1, 4:1. 2) Past history of birth trauma, CNS infection and other diseases involving CNS, were not found. There was past history of febrile convulsoin 33.3% of patient and epilepsy in 8.3%. One of paient's sister had childhood abscence epilepsy and never a nocturnal partial seizure, also and had centrotemporal spikes in EEG. 3) In 91.7% of Seizures occurred during sleep, especially short after sleep onset and the symptomatology of seizure was shown 66.7% of oropharyngeal sign, such as hypersalivation and gargle sound, and 54.2% of hemifacial involvement, 33.3% of speech arrest, 25.5% of upper limb involement, 18.3% of lower limb involvement, and 45.8% of general seizure. 4) In 91.5% of patients had on location of epileptiform discharge that located central (37.5%) midtemporal (33.3%), parietal and frontal. We could observe tangental dipole in 16.7% of patients.
Child ; Electroencephalography ; Epilepsy ; Epilepsy, Rolandic* ; Female ; Humans ; Lower Extremity ; Male ; Medical Records ; Parturition ; Rabeprazole ; Seizures ; Sialorrhea ; Siblings ; Upper Extremity