The “whistling face” syndrome is a dominantly inherited disorder involving the face and musculoskeletal system. Sjnce its first description in 1938 by Freeman and Sheldon, sporadic case reports have appeared in the literature under various syndromes, cranio-carpo-tarsal dystrophy, cranio-facio-corporal syndrome, cranio-carpo-tarsal dysplasia, whistling face-Windmill Vane Hand syndrome and the chirocheilo-podalic syndrome. This report reviews the clinical manifestation of 5 cases of whistling face syndrome in 3 generation in one family.
Hand ; Humans ; Musculoskeletal System ; Singing

Combined procedue of open reduction and shortening of femur for treatment of congenital hip dislocation in the older child is known to minmize the incidence of avascular necrosis, redislocation and stiffness. Thirty six hips in thirty three children were operated upon in the past six years with combined procedue of open reduction and shortening of femur. And twenty eight hips in twenty five children who were followed up more than one year were analysed. By the results, we obtained excellent in twenty nine percent, good in fifty three percent, fair in fourteen percent and poor in four percent.
Child ; Dislocations ; Femur ; Hip Dislocation, Congenital ; Hip ; Humans ; Incidence ; Necrosis

Two hundred and thirty-seven patients who had paresis or paralysis of the quadriceps muscles underwent a transfer of the biceps femoris by Caldwell's method or medial hamstring to the patella. Seventy-six legs of seventy-two patients who had followed-up more than one year were reviewed and analysed. The results showed there was an overall improvement measured by decreased extension lag in all age groups and relatively uniform good results in young age group. Again we reviewed the forty-six patients chronologically to eliminate the outer factors who were transfered normal biceps femoris or medial hamstring power to absent quadriceps. By that we found the extension power of the knee joint after muscle transfer had been decreased by age.
Clothing ; Humans ; Knee Joint ; Leg ; Methods ; Paralysis ; Paresis ; Patella ; Poliomyelitis ; Quadriceps Muscle

No abstract available.
Mycobacterium tuberculosis* ; Mycobacterium* ; Polymerase Chain Reaction*

No abstract available.
Extremities* ; Osteogenesis, Distraction*

No abstract available.
Contracture* ; Knee* ; Osteotomy* ; Poliomyelitis*

Limb lengthening with any kind of lengthening apparatus is accepted as a standard method to correct leg length discrepancy. And furthermore, the deformity accompanying shortening is corrected by multifocal lengthener. twenty two patients have undergone lower limb reconstruction by the technique of unilateral bone transport for diaphyseal bone defect, nonunion or deformity in the presence of shortening from May 1990 to August 1993 in Wilson Leprosy Center & Rehabilitation Hospital. All Patients had bifocal procedure using bifocal monofixator(Orthofix) and bone defects were graduaily filled by bone transport. Average healing index(days/cm)was 61. Average bone defect was 7,4cm, and average transported length was 5.4cm. In conclusion, the unilateral bone transport system using bifocal monofixator has satisfactory outcome, and salvaged limbs where amputation has been previously the only option.
Amputation ; Congenital Abnormalities ; Extremities ; Humans ; Leg ; Leprosy ; Lower Extremity ; Methods ; Rehabilitation

The hip joint subluxation and dislocation due to poliomyelitis worsen the limping and caused the painful hip. Shelf procedure has been described as a safe, conservative and reliable approach to the of a dysplastic acetabulum. We performed shelf procedure for the dysplastic hip in poliomyelitis in thirteen patients since May, 1990. The age at operation was 21 to 38 years old and three of them were male and ten were female. All the patients were followed up for average 2.5 years(1-4. 5yrs). The hip joints showed better stability after shel'f procedure and less limping. There was no complication except one redislocation.
Acetabulum ; Dislocations ; Female ; Hip Joint ; Hip ; Humans ; Male ; Poliomyelitis

Calcific myonecrosis is a rare late post-traumatic condition in which an entire single muscle is replaced by a fusiform mass with central liquefaction and peripheral calcification. The compartmental syndrome is suggested to be the underlying cause. We report a case of 70 year-old man whose right tibialis anterior and extensor hallucis longus muscle were replaced by calcific myonecrosis and treated with en-bloc resection & tendon reconstruction.
Tendons

We examined the expressions of CD44 splice variants (v4/5, v6), alpha-smooth muscle actin, nm23 to evaluate their roles as prognostic factors in 70 cases of uterine cervical carcinoma (stage IB to IIB) who were surgically treated from January 1989 to June 1990 with a clinical follow-up of a minimum of 5 years. The expression was examined by an immunohistochemical method using archival formalin fixed paraffin embedded tissue. In the 70 cases, 61 cases were squamous cell carcinoma and 9 cases were adenocarcinoma. CD44v4/5, CD44v6, alpha-smooth muscle actin, and nm23 were detected in 41.4%, 70%, 100%, and 74.3% of tumor samples, respectively. CD44 splice variants and nm23 showed membrane and cytoplasmic staining of tumor cells, respectively. The expression of alpha-smooth muscle actin showed cytoplasmic staining confined to stromal cells and was classified into three grades by the extent in stromal cells: with less than 10% of stromal cells; 32.9%, 10-50% of stromal cells; 40.0%, more than 50%; 27.1%. These expressions were not correlated with histologic types, lymph node involvement, recurrence, and grades of tumor infiltrating lymphocyte (TIL). But CD44v4/5 had significantly inverse correlation with TIL (p=0.049). The expression of CD44v4/5 was significantly correlated with that of CD44v6 (p=0.05), and that of alpha-smooth muscle actin was inversely correlated with that of nm23 (p=0.049). In conclusion, in FIGO IB-IIB uterine cervical carcinoma CD44 variants, nm23, and SMA show high prevalence, however, with little prognostic significance assessed by recurrence and lymph node metastasis.
Actins* ; Adenocarcinoma ; Carcinoma, Squamous Cell ; Cytoplasm ; Follow-Up Studies ; Formaldehyde ; Lymph Nodes ; Lymphocytes ; Membranes ; Neoplasm Metastasis ; Paraffin ; Prevalence ; Recurrence ; Stromal Cells ; Uterine Cervical Neoplasms*