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

In complication of infective endocaditis splenomegaly and splenic infarction are not uncommon but splenic rupture is very rare. We report a case of splenic rupture complicated by infective endocarditis in 1 71-yr-old man who had been suffered from rheumatic heart disease (aortic regurgitation and stenosis and mitral regurgitation). The patient was admitted to mild fever and generalized weakness for 20 days. Diagnosis of infective endocarditis due to Staphylococcus epidermidis was made by clinical manifestaions and blood culture study.On 34th day of admissionthe patient suddenly displayed the symptoms and signs of massive intraperitoneal hemorrhage. Splenic rupture was revealed by paracentesis and radiologic studies. Rupture of spleen is an uncommon and usually fatal complication of infective endocarditis. Therefore early diagnosis and prompt treatment must be performed.
Constriction, Pathologic ; Diagnosis ; Early Diagnosis ; Endocarditis* ; Fever ; Hemorrhage ; Humans ; Paracentesis ; Rheumatic Heart Disease ; Rupture ; Spleen ; Splenic Infarction ; Splenic Rupture* ; Splenomegaly ; Staphylococcus epidermidis

The authors have experienced a case of bilateral blindness which occurred during general anesthesia for neurosurgical intervention. A 49-year-old female patient was received aneurysmal neck clipping of anterior cerebral communicating artery under N O general anesthesia in supine position on July, the 21th, 1986, and at regaining consciousness, she complained of loss of vision. At inital examination, visual acuity of right eye was light perception and left eye was hand movement. Pupils were mid-dilated and light reflexes were lost on both eyes. Anterior segments were normal with no limitation of ocular movements. The retinas were milky edematous with cherry red spot at the macula and VEPs showed flat curves on both eyes.
Anesthesia, General* ; Aneurysm* ; Arteries* ; Blindness* ; Consciousness ; Female ; Hand ; Humans ; Middle Aged ; Neck ; Prunus ; Pupil ; Reflex ; Retina ; Supine Position ; Visual Acuity

Chemonucleolysis with chymopapain has been shown to be an effective alternative to surgery for the treatment of Sciatica secondary to lumbar disc herniation after failure of conservative treatment. 92 lumbar herniated disc patients had been treated by chemonucleolysis in Eul Ji General Hospital during 3 years from March 1988 to 1991 and were evaluated clinically. 1) Of 92 patients, 60 patients(65%) were between 20 and 30 years old age group and male was predominent. 2) The lesion was the most common in L4-5space(52 patients, 56.6%). 3) The results were better in the younger group and the more limited straight leg raising group. 4) The excellent and good results were obtained in 82.6%. 5) In noncompensation cases, 69 patients(88.0%) had marked improvement, but in compensation cases, 7 patients(50%) had marked improvement. 6) In conclusion, above finding suggest that chemonucleolysis is a safe and effective nonsurgical method for treatment of herniated lumbar disc before surgical intervention.
Adult ; Chymopapain ; Compensation and Redress ; Hospitals, General ; Humans ; Intervertebral Disc Chemolysis ; Intervertebral Disc Displacement ; Leg ; Male ; Sciatica ; Spine*

No abstract available.

No abstract available.
Aortic Stenosis, Supravalvular*

Surgical reconstruction of the anterior cruciate ligament is undergoing constant evolution. The variable success of these procedures can be attributed to many factors including graft selection, graft placement, graft tensioning, graft remodelling and rehabilitation program. Rigid fixation is probably the most important factor in the success of these procedures during the initial stages of healing. Arthroscopically assisted reconstruction of the ACL-deficient knee using a bone-patellar tendon-bone graft(two-incision technique) is a reliable and evolving technique. Rosenberg et al, described a one-incision technique that allows endosteal fixation of bone plugs within their femoral tunnels intraarticularly via the intercondylar notch. These two techniques are different from their femoral tunnel direction and bone plug placement. The present study was designed to develop a swine model for in vitro biomechanical testing of the bone-patellar tendon-bone ACL autograft to compare the pull-out strength of Outside-In technique and Inside-Out technique. The result were as follows: 1. Normal ACL(N=10 knees) pull-out tensile strength tests showed a maximum tensile strength of 1295.34(N), a stiffness of 120.95(N/mm), and a linear load of 1104.26(N). The failure sites were all tibial attachments. 2. Femoral and Tibial interference fit pull-out tensile strength tests(N=10 knees) showed a maximum tensile strength of 463.23±42.27(N), a stiffness of 33.62±3.18(N/mm), and a linear load of 392.51±95.42(N). The failure sites were all in the tibial tunnel area(N=9 knees), except for one which femoral tunnel area. 3. Inside-Out technique(N=15 knees) showed a maximum tensile strength of 1250.64±75.67(N), a stiffness of 94.31±26.44(N/mm), and a linear load of 1046.92±367.55(N), but the Outside-In tech-nique(N=15 knees) showed a maximum tensile strength of 805.35±54.61(N), a stiffness of 83.33±10.98(N/mm), and a linear load of 685.99±86.32(N)(p < 0.0001). The failure sites were all in the femoral tunnel area. 4. Tensile testing demonstrated that the Inside-Out technique was significantlly stronger than Outside-In technique for maximum tensile strength, stiffness and a linear load.
Anterior Cruciate Ligament Reconstruction ; Anterior Cruciate Ligament ; Autografts ; In Vitro Techniques ; Knee ; Rehabilitation ; Swine ; Tensile Strength ; Transplants

No abstract available.
Child* ; Humans ; Neurilemmoma* ; Neurofibromatosis 1

No abstract available.
Cisplatin* ; Humans ; Nausea* ; Ondansetron* ; Vomiting*

Intracranial hemorrhage is the most common neuropathologic finding in premature infants. But in full-term infants, it is less common and rarely causes death. We found out intracranial hemorrhages in 21 full-term neonates by real-time neurosonography and concluded as followings. 1) Among 21 neonates, 17 infants were male and 4 infants were female. 2) In 11 (52.3%) infants the hemorrhage was detected within 7 days after birth. 3) The intracranial hemorrhage was not related with delivery type nor Apgar score. 4) In 13 cases (61.9%) the hemorrhage was in the subependymal germinal matrix and the degree was Grade I. 5) Precipitating or associated factors were asphyxia, pneumonia, ventilator care, RDS and congenital heart disease. 6) Symptoms and signs were seizure, apnea, lethargy, cyanosis, jaundice, anemia or bulging fontanel.
Anemia ; Apgar Score ; Apnea ; Asphyxia ; Cyanosis ; Female ; Heart Defects, Congenital ; Hemorrhage ; Humans ; Infant ; Infant, Newborn* ; Infant, Premature ; Intracranial Hemorrhages* ; Jaundice ; Lethargy ; Male ; Parturition ; Pneumonia ; Seizures ; Ultrasonography* ; Ventilators, Mechanical