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.
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

The utility of computed axial tomography(CAT) in the diagnosis of spinal disorders has been the subject of considerable interest since the introduction of CAT scanning. Computed tomographic guided needle biopsy of 12 consecutive patients with a variety of undermined spinal disorders resulted in a 100% yield of positive tissue diagnoses, without signlficant complications. The principal advantage of cat guidance is the continuous direct observation of the needle tip position in relation to the target volume. We think that the application of computed tomographic guided needle biopsy in the lesions of the spine is of great value, because it is the single most accurate method of obtaining diagnosis other than by open surgical biopsy.
Animals ; Biopsy ; Biopsy, Needle ; Cats ; Diagnosis ; Humans ; Methods ; Needles ; Spine

OBJECTIVES: Echocardiographically determined left ventricular hypertrophy is associated with increased risk for sudden cardiae death and for complex ventricular arrhythmias in 24-hour ambulatory electrocardiographic monitoring. In subjects with left ventricular hypertrophy, the presence of asymptomatic complex ventricular arrhythmias is associated with higher incidence of sudden cardiac death and higher cardiovascular mortality. However, their accurate relationship and prognostic significances have been remained to be established. The purpose of this study was to evaluate the relationship between complex ventricular arrhythmias, left ventricular hypertrophy, and sudden cardiac death in Korean patients. METHODS: Twenty four hour ambulatory electrocardiographic monitoring, echocardiographic data and medical records were reviewed in 360 subjects from 1991 to 1994. We evaluated the relationship between complex ventricular arrhythmias and left ventricular mass index, and the prognostic values of them. Of the 360 subjects, 187 could be followed up for one to four years. The mean follow-up period was 2.8 years. RESULTS: The incidence of complex ventricular arrhythmias was significantly correlated with left ventricular mass index and ejection fraction in all subjects. During the follow-up periods, seven of 187 subjects died from sudden cardiac death. Six of them had complex ventricular arrhythmias with left ventricular hypertrophy. CONCLUSION: The incidence of complex ventricular arrhythmias was significantly correlated with echocardiographically determined left ventricular hypertrophy and it is suggested that subjects with complex ventricular arrhythmias combined with left ventricular hypertrophy have higher risk for sudden cardiac death.
Arrhythmias, Cardiac* ; Cardia ; Death, Sudden, Cardiac ; Echocardiography ; Electrocardiography, Ambulatory ; Follow-Up Studies ; Humans ; Hypertrophy, Left Ventricular* ; Incidence ; Medical Records ; Mortality ; Prognosis

BACKGROUND: Protamine sulfate (PS), used to neutralize the effect of heparin, is often associated with systemic hypotension. The present study was aimed to investigate the protective effects of CaCl2 on adverse hemodynamic reaction to PS. METHODS: Forty-six dogs of three groups were studied. Animals were randomly allocated to receive either; saline 10 ml (controls, n = 26), CaCl2 5 mg/kg (n = 10) or 10 mg/kg (n = 10), added to PS 3 mg/kg given intravenously 5 min after heparin (300 IU/kg, iv). Mean aortic pressure (MAP), mean pulmonary arterial pressure (MPAP), left ventricular end-diastolic pressure (LVEDP), LVdP/dtmax, and -LVdP/dtmin, and pulmonary artery (cardiac output) and left circumflex blood flow (LCX flow) were recorded up to 15 min after PS. RESULTS: PS caused significant reductions in MAP (-49%), cardiac index (CI, -66%), systemic vascular resistance index (SVRI, -22%), LVEDP (-67%), LVdP/dtmax (-36%), and LVdP/dtmin (-55%), but increased MPAP (39%) and PVRI (3.8 fold), which all increases and reductions peaked 1-3 min after PS injection in the control group. The addition of CaCl2 to PS significantly attenuated reductions of MAP (-35 and -26% in CaCl2 5 and 10 mg/kg treated dogs, respectively), CI (-34 and -37%), LVdP/dtmax (-14, -11% ), and -LVdP/dtmin (-34, -21%), and increases in PVRI (1.8 and 2.4 fold). However, both doses of CaCl2 increased MPAP (48, 82%), but not SVRI (-28, -21%) or LVEDP (-73, -75%). LCX flow increased similarly in all groups (75-137%). CaCl2 5 and 10 mg/kg iv increased plasma Ca2+ by 0.23 and 0.36 mM, respectively. CONCLUSIONS: These results suggest that the simultaneous administration of CaCl2 attenuates the adverse hemodynamic effects of PS, used to reverse heparin anticoagulation in the dog.
Animals ; Arterial Pressure ; Calcium Chloride* ; Calcium* ; Dogs* ; Hemodynamics* ; Heparin ; Hypotension ; Plasma ; Protamines* ; Pulmonary Artery ; Vascular Resistance