No abstract available.
DiGeorge Syndrome*

No abstract available.
Child* ; Humans

A 7-years-old female patient was first seen in our out-patient clinic on June 21, 1975 with five day history of progressive proptosis of the left eye. Biopsy was taken from the orbit and the submandibular lymphnodes. Exploratory laparotomy revealed ovarian malignancy. Pathologic examination confirmed generalization of ovarian granulosa-theca cell tumor.
Biopsy ; Exophthalmos ; Female ; Generalization (Psychology) ; Granulosa Cell Tumor* ; Granulosa Cells* ; Humans ; Laparotomy ; Neoplasm Metastasis* ; Orbit* ; Outpatients

A synophthalmia, another form of cyclopia, in which the element of the two eyes are partially fused to form an apparently single eye in the middle of the forehead. The synophthalmia is a result of complex, neural plate misdevelopment syndrome involving the eye, brain, skull and face. It is well known that synophthalmia is due to heterogenous causes, most of which chromosomal imbalances. We experienced a case of synophthalmia associated with proboscis, alobar holoprosencephaly and chromosomal anomaly 46, XX, -15,t (15 q, 21 q). Diagnosis was confirmed by brain MRI and autopsy, The patient died about 20 hours of age and autopsy was done. A brief review of the literatures was also presented.
Autopsy ; Brain ; Diagnosis ; Forehead ; Holoprosencephaly ; Humans ; Magnetic Resonance Imaging ; Neural Plate ; Skull

The Ankle joint is a modified complex hinge joint which plays an importnat role in weight bearing, walking and standing. Injuries about the ankle joint cause destruction of not only bony architecture but also the ligament and soft tissue components. We performed arthrography in 52 cases of ankle fracture. The Result obtained from this study was as follow; The ligament & interosseous membrane injuries were more severe, when fracture of lateral malleolus was occured higher level than ankle joint.
Ankle Fractures ; Ankle Joint ; Ankle ; Arthrography ; Clinical Study ; Joints ; Ligaments ; Membranes ; Walking ; Weight-Bearing

Congenital hand anomalies encompass a very broad spectrum of deformity, and precise classification of these deformities has always posed a major problem. We reviewed 50 cases of congenital hand deformities in 47 patients, managed at Department of,Orthopedic Surgery, Severance Hospital from Jan. 1980. to Jun. 1984 and the following results were obtained. 1. There were 26 males and 21 females in 47 patients and the ratio between male and female was 1.2: 1. Right hands were involved in 16 patients and both hands were in 14 patients. 2. The most commomn type of anomalies were polydactylysm(48%), next were syndactylysm (24%) and the follwing anomalies were found: camptodactyly, congenital constriction band syndrome, congenital ulnar deficiency, congenital clasped thumb, clinodactyly, congenital finger deficiency, symphalangism. 3. 9 associated congenital anomalias were found in 5 patients, in which, anomalies of the foot were most common. 4. Prenatal history such as drug ingestion, preeclampsia, breech delivery, prematurity, low birth weight were found and 2 cases of family history were found. 5. Treatment was stressed upon the function of hand and the improvement of the deformity.
Classification ; Clinical Study ; Congenital Abnormalities ; Constriction ; Eating ; Female ; Fingers ; Foot ; Hand Deformities, Congenital ; Hand ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Male ; Pre-Eclampsia ; Thumb

Since the first attemt of internal fixation not much more than a century ago, implants have come to play an important role in orthopaedic surgery. Yet little has been reported about complications which may result from implant surgery. During the period of May 1980 to April 1984, 32 cases of fixation failures were treated in the Department of Orthopaedic Surgery, Hallym College, Kang Nam Sacred Heart Hospital and results were obtained as follows: 1. The most common area involved femoral shaft, but the highest rate was distal femur. 2. The most common period of failure was between 2 to 4 months postoperatively. 3. The leading cause of failure was remaining defects at fracture site. 4. The authors recommend accurate reduction of fracture and bone graft, if necessary, adequate immobilization postoperatively and co-coperation of patient to avoid failure of implant.
Femur ; Heart ; Humans ; Immobilization ; Transplants

PURPOSE: This retrospective study was to determine the functional results of patients who were amputated of their fingertip between patients who were treated with replantation and patients who were treated with thenar flap. MATERIALS AND METHODS: From 2004 to 2007, we identified and operated 159 patients who were diagnosed with fingertip amputations. Of 159 patients, Eighty-two patients were treated by replantation (67 in men and 14 in women) and the mean age at the operation was 41 years (range, 15-68 years). Seventy-nine patients was treated with thenar flap(54 in men and 25 in women) and the mean age at the operation was 43 years(range, 21-70 years). We compared variables between two groups including, age, gender, diagnosis, duration of hospital admission, grip strength, two-point discrimination, Semmes Weinstein monofilament test, active range of motion (ROM) of the proximal and distal interphalangeal (PIP and DIP) joint, pain (or tenderness), paresthesia, cold intolerance, the Disabilities of the Arm, Shoulder, and Hand (DASH) questionnaire, and finger for activities of daily living (ADLs). RESULTS: The duration of admission was longer in Replantation group than in Thenar flap group(p=0.001). However, the grip strength (p=0.003) and Semmes Weinstein monofilament test (p=0.029) in the Replanation group were statistically superior to the Thenar flap group. The average DASH disability (p=0.003)/symptom score (p=0.007) and ADLs (p<0.001) in the Replantation group was statistically better. In addition, cold intoleranace test of Thenar flap group is worse than the Replantation group. CONCLUSION: This study demonstrate that fingertip replantation have demonstrated not only to obtain the best appearance but also to gain better functional outcome. However, it is impossible to perform replatation, the thenar flap can be limited alternative method for fingertip amputation in aspect of preservation of range of motion and hospitalization time.
Activities of Daily Living ; Amputation ; Arm ; Cold Temperature ; Discrimination (Psychology) ; Fingers ; Hand ; Hand Strength ; Hospitalization ; Humans ; Joints ; Male ; Paresthesia ; Surveys and Questionnaires ; Range of Motion, Articular ; Replantation ; Retrospective Studies ; Shoulder

Acute lymphoblastic leukemia (ALL), currently the most common pediatric leukemia, has a high curability rate of up to 90%. Endocrine disorders are highly prevalent in children with ALL, and skeletal morbidity is a major issue induced by multiple factors associated with ALL. Leukemia itself is a predominant risk factor for decreased bone formation, and major bone destruction occurs secondary to chemotherapeutic agents. Glucocorticoids are cornerstone drugs used throughout the course of ALL treatment that exert significant effects on demineralization and osteoclastogenesis. After completion of treatment, ALL survivors are prone to multiple hormone deficiencies that eventually affect bone mineral accrual. Dual-energy X-ray absorptiometry, the most widely used method of measuring bone mineral density, is used to determine the presence of childhood osteoporosis and vertebral fracture. Supplementation with calcium and vitamin D, administration of pyrophosphate analogues, and promotion of mobility and exercise are effective options to prevent further bone resorption and fracture incidence. This review focuses on addressing bone morbidity after pediatric ALL treatment and provides an overview of bone pathology based on skeletal outcomes to increase awareness among pediatric hemato-oncologists and endocrinologists.

Hydroa vacciniforme is a very rare photosensitivity disorder. The primary skin lesion is a vesicle or bulla which then heals with vacciniform scarring. We report a case of hydroa vacciniforme recurred after 3 years period of quiescence in a 20 year-old man who had had history of the disease from the age of two. The duplication of the natural lesion, clinically and histologically, was successfully made by artificial UV-A irradiation on the patient's back.
Cicatrix ; Hydroa Vacciniforme* ; Photosensitivity Disorders ; Skin