Incontinentia pigmenti is an uncommon genodermatosis. It affects female infants predominantly, described first by Bardach in l925, with the diagnosis of systematized nevus and Bloch in 1926, and Sulzberger in 1928 rnore compIetely. Skin lesions are characterized by 3 stages such as vesicobullous, verucous and finally pigmentary lesions and leave brownish pigmentation on the extremities and trunk. Hesides skin lesions some ectodermaI and mesodermal organs are affected and show developmental abnormalities. We experienced a case of incontinentia pigmenti in a 45-day-old female infant and present it with the review of literature. She showed extensive vesicobullopustular eruption with linear and reticular pigmentation on the extremities and trunk. Clinical and histopathologic findings of these lesions are compatible with Bloch-Sulzberger type of incontinentia pigmenti.
Diagnosis ; Extremities ; Female ; Humans ; Incontinentia Pigmenti* ; Infant ; Mesoderm ; Nevus ; Pigmentation ; Skin

No abstract available.
Craniosynostoses*

After dual plating with a locking compression plate for comminuted intraarticular fractures of the distal humerus, the incidence of ulnar nerve injury after surgery has been reported to be up to 38%. This can be reduced by an anterior transposition of the ulnar nerve but some surgeons believe that extensive handling of the nerve with transposition can increase the risk of an ulnar nerve dysfunction. This paper reports ulnar nerve injuries caused by the incomplete insertion of a screw head in dual plating without an anterior ulnar nerve transposition for AO/OTA type C2 distal humerus fractures. When an anatomical locking plate is applied to a distal humeral fracture, locking screws around the ulnar nerve should be inserted fully without protrusion of the screw because an incompletely inserted screw can cause irritation or injury to the ulnar nerve because the screw head in the locking system usually has a slightly sharp edge because screw head has threads. If the change in insertion angle and resulting protruded head of the screw are unavoidable for firm fixation of fracture, the anterior transposition of the ulnar nerve is recommended over a soft tissue shield.
Head* ; Humeral Fractures ; Humerus* ; Incidence ; Intra-Articular Fractures ; Surgeons ; Ulnar Nerve*

Pseudohypoparathyroidism is a medical disorder characterized by a complex disorder of renal resistance to parathyroid hormone and the mechanism underlying the disease is still unclear. The authors described two cases of pseudohypoparathyroidism in sibling,who had metabolic anomalies(hypocalcemia and hyperphosphatemia, high circulatin immunoreactive PTH)and basal ganglia calcification. Bilateral basal ganglia calcifications, which was not visible on plain skull film, was detected by CT scan of brain MRI. We report these cases with a review of related literatures.
Basal Ganglia ; Brain ; Humans ; Hyperphosphatemia ; Magnetic Resonance Imaging ; Parathyroid Hormone ; Pseudohypoparathyroidism* ; Siblings* ; Skull ; Tomography, X-Ray Computed

A persistent left superior vena cava is the most common anomaly of the superior caval system. Usually the persistent left superior vena cava is connected with the right atrium via the coronary sinus, resulting in no physiologic derangement : however in 7 to 8 percents of the patietns with a persistent left superior vena cava, the anomalous vessel communicates with the left atrim. In the absence of obstruction to the flow from the left atrium to the left ventricle. this anatomic situation usually results in right to left shunting of varying degress. We recently experienced a case of persistent left superior vena cava in a 52-year-old female who complained of chest discomfort, epigastric pain and dyspnea(NYHA functional class II). Cine-angiography showed that the contrast passed from the left SVC through the dilated coronary sinus into right atrium. And right sided SVC was not seen. The patient was treated with conservative measures and discharged with improved condition.
Coronary Sinus ; Female ; Heart Atria ; Heart Ventricles ; Humans ; Middle Aged ; Thorax ; Tricuspid Valve Insufficiency* ; Vena Cava, Superior

PURPOSE: To assess CT findings of pulmonary sarcoidosis and correlate these with sACE level and PFT. MATERIALS AND METHODS: Between 1989 and 1995, 14 patients (4 men and 10 women, aged between 28 and 55 years) with histologically confirmed pulmonary sarcoidosis were consecutively selected. HRCT scans were performed in 12 patients and conventional CT scans in two. CT findings were reviewed by three radiologists, and were correlated with the index of disease activity based on sACE level and pulmonary function test. RESULTS: Pulmonary parenchymal abnormalities were seen in all patients ; small nodules of less than 3 mm in diameter were seen in eight. Other abnormalities were nodules of more than 3 mm in diameter (n=7), confluent nodules (n=5), ground glass opacity (n=5), patchy areas of consolidation with air bronchogram (n=5), and architectural distortion (n=3). The upper lung zone was more frequently involved than the middle or lower zone. In ten patients, the peripheral interstitum was predominantly involved, while only three patients showed predominant peribronchovascular involvement. Lymphadenopathy was noted in 13. There was no correlation between sACE level, the results of a pulmonary function test and the extent of parenchymal involvement. CONCLUSION: HRCT is valuable for the identification, characterization, and determination of the extent to which parenchymal lung is involved in sarcoidosis. The extent of this involvement does not correlate with sACE level and pulmonary function test results.
Female ; Glass ; Humans ; Lung ; Lymphatic Diseases ; Male ; Respiratory Function Tests ; Sarcoidosis ; Sarcoidosis, Pulmonary* ; Tomography, X-Ray Computed

PURPOSE: To determine the upper limit of peripheral nerve lengthening without loss of function, the recovery time of peripheral nerve palsy due to nerve lengthening and their histological changes. MATERIALS AND METHODS: Twelve adult New Zealand rabbits weighing about 2.5 kg were assigned to the following groups. Group 1 (n=6) was subjected to slow tissue expansion with 30% of nerve lengthening while group 2 (n=6) was rapid expansion with 40% lengthening of nerve. The expanders were refilled every 2 times during the 2nd and 4th weeks making a total four times of expansion. The rabbits were assessed in terms of affected leg paralysis, neural length gain, EMG with nerve conduction velocity and histological changes. RESULTS: In group 1 (n=6), paralysis on affected leg was found in one rabbit and was recovered spontaneously on the 4th weeks after expander removal. In Group 2 (n=6), paralysis was found in four rabbits, and three of them were recovered on 4, 5 and 9 weeks after removal of the expander. EMG study showed increase in distal latency of 2.50+/- 0.20 m/sec, and decrease in nerve conduction velocity of 62.49+/- 5.30 m/sec compared to normal side with 1.89+/- 0.14 m/sec and 75.39+/- 7.82 m/sec. The mean neural length gain was 6 mm (30% of 20 mm of initial pre-experimental nerve length) in group 1 and 8 mm (40% of 20 mm) in group 2. Light microscopic examination revealed the loss of segmental myelination, decrease of myelination, and vacuolation. Electron microscopic examination showed that the normal ring shaped contour of axon was changed to convoluted shape.
Adult ; Axons ; Humans ; Leg ; Myelin Sheath ; Nerve Expansion ; Neural Conduction ; Paralysis ; Peripheral Nerves ; Rabbits ; Sciatic Nerve* ; Tissue Expansion*

PURPOSE: To investigate the expression of Vascular Endothelial Growth Factor (VEGF) in diabetic frozen shoulders. MATERIALS AND METHODS: We preformed arthroscopic adhesiolysis on 9 diabetic frozen shoulder patients, and observed the arthroscopic findings. Also, we examined the potential role of VEGF by using samples of synovial tissues from 5 patients, and 2 normal synovial tissues. Immunohistochemical staining and Western blotting were performed using polyclonal antibodies against VEGF. RESULTS: There was hyperemic synovitis in the 9 diabetic frozen shoulder patients. In the 5 patients' tissue samples, there was strong immunostaining and expression to VEGF, but there was little staining and expression in the control group. CONCLUSION: We postulate that VEGF is synthesized and secreted in the synovium of diabetic frozen shoulders and that secreted VEGF binds specific receptors on the endothelial cells of nearby small blood vessels, and leads to the subsequent development of frozen shoulders in diabetic patients.
Antibodies ; Blood Vessels ; Blotting, Western ; Bursitis* ; Endothelial Cells ; Humans ; Shoulder ; Synovial Membrane ; Synovitis ; Vascular Endothelial Growth Factor A*

PURPOSE: This study was designed to clarify the osseointegration of the titanium screw coated with CMP, in regard to the time schedule, through the characteristic of early osseointegration. MATERIALS AND METHODS: Mechanical, radiological and histomophometric measurements were performed in 28 rabbit tibial proximal metaphyseal cortical bone screws 6, 12, 26 and 52 weeks after surgery for the in vivo comparison of the osseointegration of titanium screws (3.75 mm diameter, 5 mm length) with different surface treatments: CMP coating group, with the sol-gel method (experimental group) and uncoated group (control group). RESULTS: 1. Radiology: There were no differences between the two groups without a radiolucent line or in regard to the time schedule. 2. Histology: There were no differences between the two groups without a fibrous tissue intervening surface or in regard to the time schedule. 3. Torque test: The test results for the CMP coated group were 1.5 times higher than those for the uncoated group, which was statistically meaningful, but there was no difference in regard to the time schedule. CONCLUSION: CMP coating is an option to increase the osseointegration of the titanium screw.
Appointments and Schedules ; Bone Screws ; Ceramics* ; Osseointegration* ; Tibia ; Titanium ; Torque

A 23 years old single nulligravida woman underwent laparoscopic removal of a huge cystic adnexal mass that occupied her entire abdomen, giving the appearance of a full term pregnancy. After anesthesia, a vertical infra-umbilical incision, 1 cm long, was made and a telescope was introduced through the port to determine the status of the intra-abdomen and the surface contour of the mass. A needle tipped with a laparoscopic suction apparatus was inserted into the cyst through the infra-umbilical port, directly under the mass. Subsequently, 3, 200 ml of cystic fluid was aspirated without spillage. A huge cyst, reaching to the level of the xyphoid process was effectively excised through the operative laparoscopy after prelaparoscopic drainage. Operation time was 140 minutes and hospital stay was 2 days. There were no complications during hospital stay and after discharge. It seems the size of the cyst is not a criteria for the contraindication of laparoscopic surgery.
Abdomen ; Anesthesia ; Drainage ; Female ; Humans ; Laparoscopy ; Length of Stay ; Needles ; Ovarian Cysts* ; Pregnancy ; Suction ; Telescopes ; Young Adult