Incontinentia pigmenti acb.romians(Ito) is characterized by progressive bizarre or whorl-like hypopigmentation on trunk and extrementies during childhood. It is similar to a negative picture of incontinentia pigmenti(Blocb-Sulzberger) and not infrequently associated with mental, bony and ocular defects. The incidence of this disease is predominent in femaIe without hereditary background. Case 1. Two years old female was visited to our clinic because of mottled depigmented patches on right thigh for about 8 months. Case 2. 14 months oId female was visited to our clinic because of linear and. mottled depigmented natches on their limbs for about 7 months. Histopathological findings of both cases shows the focal depigmentation on basal layer. They are treated with steroid ointment with moderate to good effects.
Extremities ; Female ; Humans ; Hypopigmentation ; Incidence ; Incontinentia Pigmenti* ; Pigmentation Disorders* ; Thigh

The results of hepatic portojejunostomy performed in 34 patients with biliary atresia between May 1989 and December 1997 were analyzed. These patients were divided into three groups based on age of surgery; group 1 (< or =60 days, n = 11); group 2 (61~90 days, n = 14), and group 3 (>90 days, n = 9). Jaundice was cleared in 22 cases (64.7%). There was no significant difference of jaundice-free rate according to the age at operation between group 1, 2, 3. After a mean follow-up period of 33.9 month (range 18days-105.7 month), there were eleven deaths from hepatic failure(6), esophageal varix bleeding even though anicteric(2), sepsis by cholangitis(1), heart failure(1) and renal failure(1). Two of group 1 and 3 had received liver transplantation. With follow-up of average 33.9 months (18days-106months, one follow-up loss), 5-year cumulative survival rate by Kaplan-Meier method was 65.9%. The 5-year survivals of group 1, 2, 3 were 63.6%, 77.9%, 41.4%. There was no significant difference of survival rates according to the age at operation between group 1, 2, 3. Moreover, two cases of group 3 survived more than 5 years. Therefore, hepatic portojejunostomy can be considered as a primary surgical modality for biliary atresia in a patient of 90 days or more. Early detection of esophageal varix and possible sclerotherapy is necessary for long term survival.
Biliary Atresia* ; Chungcheongnam-do* ; Esophageal and Gastric Varices ; Follow-Up Studies ; Heart ; Hemorrhage ; Humans ; Jaundice ; Liver Transplantation ; Sclerotherapy ; Sepsis ; Survival Rate

One case of subungual glomus tumor was reported which had characteristic clinical manifestations of paroxysmal pain and tenderness for about 20 years. Histopathologically it was confirmed as subungual glomus tumor. This case was treated completely with simple surgics,l excision of the mass.
Glomus Tumor*

PURPOSE: Septic arthritis of the knee is a relative rare complication of the arthroscopic anterior cruciate ligament reconstruction, but inadequate treatment of this complication may lead to a devastating conse- quence of a chronic infection. Moreover, no guidelines have been proposed for the treatment of patients with knee infections after the anterior cruciate ligament reconstruction. To determine the optimal diagno- sis and treatment recommendation, we performed this study. MATERIALS AND METHODS: We performed retrospective study of the knee joint infections after the arthroscopic anterior cruciate ligament reconstruction between August 1993 and August 1998, Of the 420 patients who reviewed arthroscopic ACL reconstruction during this period from one surgeon, we experi- enced 4 cases(0.95%) of postoperative deep infections of the knee. RESULTS: All 4 patients were male, and the average age was 32 years(range from 19 to 43). Two patients had acute(< 2 weeks), two patients had subacute infections(2 weeks to 2 months). The acute cases had more severe symptoms, and positive cultures from knee joint aspirates. All patients underwent immediate open(1 case), or arthroscopic irrigation and debridement. One patient underwent repeat irriga- tion and debridement. The graft was removed from all patients in acute cases. CONCLUSION: The clinical symptoms, and the infected organism were more helpful than the peripheral blood counts in the diagnosis of a deep infection of the knee. Arthroscopic lavage, and debridement com- bined with intravenous antibiotics were effective treatment options. Clinical symptoms were more severe in acute cases, and early graft removal is an adequate method of surgical treatment.
Anterior Cruciate Ligament Reconstruction* ; Anterior Cruciate Ligament* ; Anti-Bacterial Agents ; Arthritis, Infectious* ; Debridement ; Diagnosis ; Humans ; Knee Joint ; Knee* ; Male ; Retrospective Studies ; Therapeutic Irrigation ; Transplants

Spinal stenosis is a localized narrowing of spinal canal due to strurctural abnormalities. Its symptoms are obscure, and characterized by chronic, poorly localized, bilateral nerve root compression signs. Computerized tomography(CT) has been accepted as almost an absolute diagnostic method for spinal stenosis after its application in the orthopedic field. During the period of 2 years, from March 1982 to March 1984, 26 cases of spinal stenosis were treated surgically after botlt CT scanning and myelography. We obtained following results about advantage of CT scanning in spinal stenosis. 1. Myelography showed following findings: Hourglass defect 9, Complete block 8, Unilateral focal defect 4, Uniform narrowing 1, Mixed 2, Negative finding 2. Myelographic finding was not specific for spinal stenosis and diagnostic accuracy was inferior to CT. 2. The type of spinal stenosis was identified by CT scan finding. Of 26 cases, 21 cases were degenerative spinal stenosis, of which central stenosis was present in 7 cases, and lateral stenosis was present in all cases. The remaining 5 cases were combined type, and both central and lateral stenosis were present in all cases. 3. The diagnostic accuracy,in localizing the level of stenosis was 61.5% by myelography, and 96.2% by CT scan. CT was especially superior to myelography in the localization of the multi-segmental stenosis. 4. CT gives valuable information about extent and degree of stenosis, especially lateral recess stenosis, thus helping the surgeon to decide on the extent of decompressive lamininectomy preoperatively.
Clothing ; Constriction, Pathologic ; Methods ; Myelography ; Orthopedics ; Radiculopathy ; Spinal Canal ; Spinal Stenosis ; Tomography, X-Ray Computed

Congenital afibrinogenemia is a rare disorder that refers to a congenital lack of production of fibrinogen, a key component of the hemostatic system. Bleeding manifestations of congenital afibrinogenemia vary in severity from mild to catastrophic. This is a case report of splenic rupture occurred in an eight-year-old boy with congenital afibrinogenemia. A conservative treatment was carried out with perfusion of cryoprecipitate and purified virally inactivated fibrinogen concentrates and splenectomy was avoided.
Afibrinogenemia* ; Fibrinogen ; Hemorrhage ; Humans ; Male ; Perfusion ; Rupture* ; Spleen* ; Splenectomy ; Splenic Rupture

Infantile fibrosarcoma is a rare malignant soft tissue tumor occurring especially in newborn and young children under 2 years. We experienced three cases of infantile fibrosarcoma presenting in the neonatal period. Case 1 presented with a multiseptated cystic mass on his left thigh at birth that was diagnosed as lymphangioma. After picibanil injection, we noted the size of the mass doubled and a solid lesion was prominent in the magnetic resonance image. Case 2 was found to have a reddish mass on his lower back mimicking hemangioma. Over 2 weeks, the mass grew rapidly with internal hemorrhaging. Case 3 was noted to have an encircling mass around the splenic flexure, which developed into congenital bowel obstruction. All of the tumors were resected completely, but microscopic resection margin was not clear in two patients. The two patients received adjuvant chemotherapy and all patients are well without evidence of recurrence.
Chemotherapy, Adjuvant ; Child ; Colon, Transverse ; Fibrosarcoma ; Hemangioma ; Humans ; Infant, Newborn ; Lymphangioma ; Magnetic Resonance Spectroscopy ; Parturition ; Picibanil ; Recurrence ; Thigh

The distal ulnar resection, Darrach operation, has been used for several conditions such as fracture about wrist joint, rheumatoid arthritis or ulno-carpal impingement syndrome. But, this operation could be led to have excessive unphysiologic increase radial loading on the radio-carpal joint and early osteoarthritis is thought to be inevitable in long-term follow-up. So, we performed various kind of reconstruction for 19 cases with dislocated distal radioulnar joint, and compared their radiologic and clinical results with that of the group for total 13 cases with distal ulnar resection. Through this study, we could find out that the preservation of normal relationship of dislocated distal radioulnar joint is considered as more desirable than that of removal of ulnar head.
Arthritis, Rheumatoid ; Dislocations ; Follow-Up Studies ; Head ; Joints ; Osteoarthritis ; Wrist Joint

OBJECTIVES: Recent studies have reported a correlation between obstructive sleep apnea syndrome (OSA) and depression. In attempt to verify the suggestion that eveningness is related to depression, we examined the effect of morningness-eveningness on their depressive mood in patinets with OSA. METHODS: The examination was based on the medical records and polysomnography reports of 211 OSA patients. Information was gathered from the patients who filled out the Horne and Ostberg questionnaire (HOQ), profile of mood states-Korean version (K-POMS), and Epworth sleepiness scale (ESS). We compared mean values of K-POMS total, subscales of K-POMS, ESS, and OSA severity variables among the 3 morningness-eveningness groups (morningness, eveningness, and neither groups). Partial correlation analysis was performed between variables and ANCOVA was performed among the 3 groups after adjustment with age and weight. RESULTS: There were significant negative correlations between HOQ and the followings : K-POMS total, POMS-T (tension-anxiety), POMS-D (depression-dejection), POMS-A (anger-hostility), POMS-F (fatigue-inertia), POMS-C (confusion-bewilderment), spontaneous arousal index, average O2 saturation. There were significant positive correlations between HOQ and the followings : POMS-V (vigor-activity), apnea-hypopnea index, respiratory arousal index, snore time. There were significant negative correlations between POMS-D and the followings : HOQ, POMS-V, stage 1 sleep (%), AHI, TAI (total arousal index), oxygen desaturation index, respiratory arousal index, neck circumference, average O2 desaturation, snore time (%). There were significant positive correlations between POMS-D and K-POMS total, POMS-T, POMS-A, POMS-F, POMS-C, sleep latency, stage 2 sleep (%), heart rate, spontaneous arousal index. There were significant differences in K-POMS total, POMS-T, POMS-D, POMS-F, POMS-C, spontaneous arousal index among the three HOQ groups in ANCOVA. CONCLUSION: The depressive correlates of OSA patients might be affected, not by excessive daytime sleepiness or OSA severity indexes, but by eveningness circadian characteristics. It would be important to take into account the morningness-eveningness tendency when we manage the depressive mood of OSA patients.
Animals ; Arousal ; Circadian Rhythm ; Depression ; Disorders of Excessive Somnolence ; Heart Rate ; Horns ; Humans ; Medical Records ; Neck ; Oxygen ; Polysomnography ; Surveys and Questionnaires ; Sleep Apnea, Obstructive

Infantile hypertrophic pyloric stenosis (IHPS) is known to be prevalent in full-term babies, and relatively rare in prematures. The diagnosis of IHPS in premature infants may be obscured because of the lack of classical symptoms and signs and the absence of the standard criteria for ultrasonic diagnosis. The purpose of this study is to discover the clinical differences of IHPS between premature and full-term infants with pyloric stenosis, and to determine the appropriate diagnostic methods for early diagnosis in prematures. The clinical records of 52 IHPS patients who had been operated from October, 1994 to April, 1997 were reviewed. IHPS in premature infants was 25%. The onset of symptom was 4.7 weeks of age in premature, and 2.9 weeks in full-term babies. Diagnosis was established by typical symptoms, signs, and diagnostic imaging studies. In two premature infants, diagnosis was confirmed by upper gastrointestinal (GI) series, because ultrasonography did not meet the diagnostic criteria. Two premature infants diagnosed as gastroesophageal reflux by esophagography initially, were confirmed to have IHPS by upper GI series. For the diagnosis of IHPS, a new set of criteria for premature babies has to be developed.
Diagnosis ; Diagnostic Imaging ; Early Diagnosis ; Gastroesophageal Reflux ; Humans ; Infant* ; Infant, Newborn ; Infant, Premature ; Pyloric Stenosis ; Pyloric Stenosis, Hypertrophic* ; Ultrasonography