Periumbilical perforating pseudoxanthoma elasticum (PPPXE) is a localized acquired disorder found most frequently in obese, multiparous, middle-aged women. It is characterized clinically by yellowish, lax, well-circumscribed, reticulated or cobblestoned patches or plaques in the periumbilical region. Multiparity, obesity, massive ascites, and abdominal surgery are thought to the initiating factors. There is controversy about the etiology of PPPXE. Some authors have classified it as a separate entity from hereditary pseudoxanthoma elasticum (PXE), while others speculate that this condition merely represents a variable expression of PXE in which systemic associations are likely. We report a case of periumbilical perforating pseudoxanthoma elasticum associated with a clinical PXE lesion on the anterior neck.
Ascites ; Female ; Humans ; Neck ; Obesity ; Parity ; Pseudoxanthoma Elasticum*

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

BACKGROUND: We have already shown that a new vitamin D3 analogue, calcipotriol is a potent growth inhibitor for human keratinocytes, indicating the close relationship between the in vitro inhibitory effect of calciipotriol and its therapeutic effectiveness or psoriasis. OBJECTIVE: Our purpose was to evaluste the clinical efficscy, safety and tolerability of calcipotriol (50ug/g) ointment twice daily in the treatment of psoriasis. MEHTODS: We treated 31 patients with calcipotriol for 6 weeks. Patients were provided with a maximum of 100g of ointment per week. Efficacy, as measured by the Psoriasis Area and Severity Index(PASI), and safety were assessed at 2,4, and 6 weeks. RESULTS: Reduction of PASI was stastistically significant at all time points for treatments (p<0.01). At the completian of 6 weeks of treatment, the mean PASI reduction was 3.61. An analysis of patient overall self-assessment, at 6 weeks showed clearance or marked improvement in 61%. Some patients developed minimal irriltation of lesional or perilesional skin(6.5%). Laboratory tests did not show any significant changes, in particular there was no change in serum calcium levels. CONCLUSIONS: Calcipotriol ointment was effective as measured by the PASI and the self-assessment in patients with psoriaeis. This treatrnent was well tolerated.
Calcium ; Cholecalciferol ; Humans ; Keratinocytes ; Psoriasis* ; Self-Assessment

Heat-treated autogenous bone graft has been utilized in the cases with large hone defects associated with tumors, chronic osteomyelitis, and trauma. Conventional autoclaved autogenous bone grafting, however, has inherent disadvantages that included decreased biomechanical strength, and loss of osteogenesity. In contrast, despite devitalizing the tumor cells, low heat-treatment can render autogenous hone grafts to retain better biomechanical strength as well as to preserve osteogenesity by avoiding destruction of hone morphogenetic protein. To investigate biomechanical strength and osteogenesity of heat-treated autogenous bone grafts, rahbits were classified into three groups: groupl, in-situ implantation of autogenous graft in the diaphyseal defect of the tibia without any heat treatment; group 2, reimplantation of graft after low heattreatment; group 3, reimplantation of graft after autoclaving. Radiological, histological, and scintigraphic examination were performed postoperativeIy at 2nd, 4th, 6th, 9th, 12th weeks. And biomechanical test was performed postoperatively at 6th, 9th, 12th weeks. Biomechanical test revealed that there were no statistical differences among three groups at 6th week and 9th week. However, at 12th week postoperatively, there were significant differences between group 2 and group 3, and between group 1 and group 3. Radiological and histological examinations showed that new bone formation started earlier in groups l and 2, compared to group 3. Also bone remodeling was advanced in group I and 2, com- pored to group 3. Scintigraphically, the increase of 99mTc-MDP uptake was delayed in group3, compared to group l and 2. Based upon above findings, it was concluded that low heat-treated autogenous bone grafting was hetter than autoclaved autogenous bone grafting in promoting bone healing with retained biomechanical strength.
Bone Remodeling ; Bone Transplantation* ; Hot Temperature* ; Osteogenesis ; Osteomyelitis ; Replantation ; Technetium Tc 99m Medronate ; Tibia ; Transplants

The syndrome of inappropriate ADH secretion is a disorder characterized by hyponatremia which results from water retention attributable to ADH release. The hallmark of SIADH is hyponatremia due to water retention, in the presence of urinary osmolality above plasma osmolality. The SIADH was initially described by Schwartz et al (1957). This syndrome, first recognized in patients with bronchogenic carcinoma, has now been observed in a variety of other illnesses. Recently, we encountered a 59 year-old female with small cell lung cancer, also she had SIADH. Thus, we present a case and review the literature on the subject.
Carcinoma, Bronchogenic ; Female ; Humans ; Hyponatremia ; Inappropriate ADH Syndrome* ; Osmolar Concentration ; Plasma ; Small Cell Lung Carcinoma* ; Water

The recent progress in the basic knowledge of basal ganglia pathways and advances in the techniques of the neuroimaging studies enabled subthalamic deep brain stimulation (STN DBS). In Korea, more than three hundreds and fifty patients with PD have been treated with STN DBS since the first trial at March 2000. STN DBS effectively improves all parkinsonian deficits occurring especially during levodopa 'off' period and decreases the daily 'off' time. The daily requirement of levodopa dosage can be reduced to about half of the preoperative one. The favorable responses to the STN DBS can be maintained even after five years. However, parkinsonian deficits during levodopa 'on' period can not be controlled as effectively as those during the levodopa 'off' period. The axial symptoms including gait disturbance and postural instability during the levodopa 'on' period cannot be improved or even are worsen by STN DBS. Patients aged over 70 frequently show less remarkable improvement of parkinsonian deficits than the younger ones. Therefore, selection of appropriate candidate for STN DBS is the most important factor deciding the outcome of the STN DBS.
Basal Ganglia ; Deep Brain Stimulation* ; Gait ; Humans ; Korea ; Levodopa ; Neuroimaging ; Parkinson Disease* ; Subthalamic Nucleus

The author has studied 33 cases of Korean embryos of Carnegie stage 11 ~23 and 18 cases of fetuses to demonstrate the development of the hip joint. The external feature of the lower extremity was observed by stereoscope and digital camera, and the internal structures were studied by light microscopic observation. The results obtained were as follows: In stage 13 lowerlimb buds were appeared. In stage 17 mesenchymal condensation for femur and hip bone, and one -layered interzone were observed. In stage 18 cartilage models for ilium and ischium were visible. In stage 22 three -layered interzone between the head of femur and hip bone was formed. In stage 23 acetabular labrum and distinct three -layered interzone was visible. In the 9th weeks mesenchymal ligamentum capitis femoris and transverse acetabular ligament are appeared, and acetabular labrum was reacted tracely to trichrome stain. In the 10th week the joint space was formed between the femoral head and hip bone, and shallow depression in acetabulum is found. In the 12th week the articular capsule was directed lateral to acetabular labrum, and numerous blood vessels were found in acetabular fossa and ligamentum capitis femoris, and cartilage canal were developed in femoral neck. In the 14th week cartilage canal was found in middle of femoral head, and synovial fold were developed, and ligamentum capitis femoris was shown strongly positive reaction. In the 16th week cartilage canals were more found, and numerous blood vessels were observed in fovea capitis. In the 18nd week the neck of femur was narrow, and femoral head was lied deeply in acetabulum with acetabular labrum. Consequently the lower extremity of Korean embryos and fetuses was first appeared in stage 13, and hip joint development was started at stage 17. The articular cavity was first formed at the 9th week of development, the acetabulum labrum was developed at stage 23. The mesenchymal ligamentum capitis femoris was appeared at the 9th week. At the same time the transverse acetabular ligament fully encircled the femoral head. The articular capsule has lined the articular cavity at the 12th week, and synovium was formed at the 14th week of development. At the 18th week the hip joint has attained its final shape.
Acetabulum ; Blood Vessels ; Cartilage ; Depression ; Embryonic Structures* ; Femur ; Femur Neck ; Fetus* ; Head ; Hip Joint* ; Hip* ; Humans* ; Ilium ; Ischium ; Joint Capsule ; Joints ; Ligaments ; Lower Extremity ; Neck ; Synovial Membrane

No abstract available.
Hematoma, Epidural, Spinal* ; Hemorrhage*

The unilateral megalencephaly is a rare brain malformation characterized by cerebral asymmetry and cortical dysplasia caused by faulty migration of the subependymal neuroblasts. We experienced a case of unilateral megalencephaly in a two day-old male with the chief complaint of asymmetric head appearance. Large left hemisphere with agyria, pachygyria, dilatation of lateral ventricle, and the thick cortex of the ipsilateral hemisphere were showed in brain MRL, Clinical findings in this case were intractable seizure, hemiparesis, and psychomotor retardation. A review of literatures was also presented briefly.
Brain ; Dilatation ; Head ; Humans ; Lateral Ventricles ; Lissencephaly ; Male ; Malformations of Cortical Development ; Paresis ; Seizures

To observe the antihypertensive effects of the angiotensin-converting enzyme inhibitor, enalapril, a daily average dosage of 20mg was administred to 38 patients with essential hypertension for 4 weeks. Changes in blood pressure and heart rate, and the frequencies of adverse reactions during the drug administration were constantly observed. At the end of the 4th week of administration the mean diastolic blood pressure decreased from 104+/-11.3 to 91+/-7.9mmHg(<0.001), and the mean systolic blood pressure from 165+/-16.8 to 142+/-10.6mmHg(P<0.001), An effective antihypertensive action of enalapril was observed in 31 out of 38 cases(82%), and normalization of diastolic blood pressure below 85mmHg was shown in 27 cases(71%). The most remarkable antihypertensive effects were shown at the end of first week, and the effect increased progressively until the end of 4th week of therapy. There were no significant changes in heart rate during the observation period. Adverse reactions occured in only 7 cases(18%). These reactions included headache, dizziness, fatigue, skin rash and facial flushing in the order of frequency. Almost all of these reaction were mild and transient, disappearing spontanously without discontinuation of the medication. In conclusion, enalapril seems to be a safe and effective primary antihypertensive drug for the treatment of essential hypertension.
Blood Pressure ; Dizziness ; Enalapril* ; Exanthema ; Fatigue ; Flushing ; Headache ; Heart Rate ; Humans ; Hypertension