Bart's syndrome was initially described as a genodermatosis characterized by congenital localized absence of the skin with blistering and nail deformities 1-3. However, it is considered as any type of epidermolysis bullosa(EB) with localized congenital absence of the skin on the extremities. A 33-day-old fbmale baby was presented with congenital absence of the skin over the left shin and dorsa of both feet which were covered with the thin, translucent, and brown-red glistening membranes. Blistering of the right calf and left great toe nail deformity were also noted. She was diagnosed as a recessive dystrophic EB by the histopathological, ultra- structural and immunomapping studies.
Blister ; Congenital Abnormalities ; Extremities ; Foot ; Membranes ; Skin ; Toes

Platelet-derived growth factor(PDGF) is known to accelerate soft tissue fracture and periodontal bone healing, but little information is available for characterizing the healing of articualr cartilage. This study was designed to demonstrate the regeneration potential of pasteurized autogeneous osteochondral graft when PDFG was applied locally in vivo. Eighteen rabbits in two groups were used in the experiment. The free osteochondral fragment(1x1x1 cm) were taken from distal femur. The fragment were pasteurized in 65degrees C for 5 minutes. In experimental group(group II), the fragment were soaked in 0.25 mg/ml of recombinant human PDGF mixed 5 ml normal saline and reimplanted to the resected site, and followed with local administration of 0.25 mg/ml PDGF to knee joint. In control group(group I), the pasteurized fragment were reimplanted in the resected site without any treatments. The histologic characteristics of the transplanted autografts for three rabbits in each group were observed at 3, 5 and 7 weeks postoperatively. The results were as follows; Group II revealed the more favorable regeneration of articular cartilage, less arthritic changes and more mature arrangement of chondrocytes than group I. Even thought the cartilage is avascular and the regeneration capacity of pasteurized cartilage is severely limited, the regeneration of grafted articular cartilage is thought to be accellerated by increased newly formed blood vessels and soft tissues due to the diffusion of PDGF on grafted cartilage, and probably also by differentiation of mesenchymal cells in the initial stage of experiment. The results of this study suggest that local administration of PDGF could be used for the treatment of posttraumatic or degenerative arthritis and various cartilage damages.
Autografts ; Blood Vessels ; Cartilage ; Cartilage, Articular ; Chondrocytes ; Diffusion ; Femur ; Humans ; Knee Joint ; Osteoarthritis ; Platelet-Derived Growth Factor* ; Rabbits* ; Regeneration* ; Transplants*

Requirement of blood transfusion has been progressively increased and supplements by volunteer donor are not sufficient to their requirements. Moreover homologous blood transfusion are not supplied without risks, espcially the transmission of the serum hepatitis. Twenty procedures, including two total hip replacement arthroplasty, were carried out with acute hemodilutional autotransfusion. The results of this study show that autotransfusion is a safe method without difficulty of blood replacement in elective orthopedic procedures.
Arthroplasty ; Arthroplasty, Replacement, Hip ; Blood Transfusion ; Blood Transfusion, Autologous ; Clinical Study ; Hepatitis ; Humans ; Methods ; Orthopedic Procedures ; Orthopedics ; Tissue Donors ; Volunteers

Fractures involving joints and about the joints provide complex problems with small comminuted fractures, surrounding soft tissue injuries and articular cartilage damage. Various problems are also encountered on the weight bearing surface, especially on the convex side of the distal Femur. Even though many auther had recommended kinds of management upto now, operative methods are more suggested in order to get good results regarding with the accurate anatomical reduction, rigid internal fixation and early joint motion. The clinical analysis of rnanagements of 20 cases of distai femoral fracture demonstrate that seperate dual incision-bilateral or right angle plane-to originate fracture site, and anatomical reduction & rigid internal fixation, early non-weight bearing ROM exercise of joint were sometimes mandatory to treat the displaced, comminuted fracture.
Cartilage, Articular ; Femoral Fractures ; Femur ; Fractures, Comminuted ; Joints ; Soft Tissue Injuries ; Weight-Bearing

Interventions for targeted symptoms are important when setting treatment strategies for individuals with autism spectrum disorder (ASD) and developmental disabilities. Especially, the goal should be to achieve individual “niche construction” by allowing them to select and adjust an environment where they can demonstrate their special characteristics and strengths. In addition, these choices should vary depending on the stage of development of each person with ASD and developmental disabilities. It is necessary to establish a detailed and systematic plan for diagnosis and treatment necessary for infants and toddlers, school placement in school age, and employment or self-reliance in adult transition period to establish customized treatment strategies that fit the individual level of people with ASD and developmental disabilities.

STUDY DESIGN: Retrospective case series. OBJECTIVES: We retrospectively evaluated the clinical efficacy of postganglionic nerve block in symptomatic Schmorl nodules (SNs). SUMMARY OF LITERATURE REVIEW: SNs are common lesions that are often asymptomatic. In certain cases, SNs have been reported to cause severe axial back pain, thereby considerably impacting patients' quality of life. No consensus currently exists on the treatment of symptomatic SNs. MATERIALS AND METHODS: From October 2015 to October 2017, a total of 21 patients with symptomatic SNs diagnosed by magnetic resonance imaging (MRI) that did not respond to conservative treatment after 4 weeks were included in the study. All patients received postganglionic nerve block. We evaluated effective pain relief (improvement of back pain of more than 50% compared with before the intervention) and functional improvements, assessed by visual analogue scale (VAS) and Oswestry Disability Index scores obtained at 4 hours, 4 weeks, 8 weeks, 3 months, and 6 months after the procedure. RESULTS: Symptomatic SNs were more common at the L2-3 level, and the lower end plate was more commonly involved than the upper end plate. Eighteen of the 21 patients (85.7%) showed effective pain relief, and no deterioration was observed within the follow-up period. Throughout the follow-up period, the VAS remained significantly improved compared to before the procedure (p < 0.05). Complications were not reported in any cases. CONCLUSIONS: Postganglionic nerve block for symptomatic SNs that do not respond to conservative treatment is a non-invasive modality for pain relief.
Back Pain ; Consensus ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Nerve Block ; Quality of Life ; Retrospective Studies ; Treatment Outcome

OBJECTIVES: We retrospectively evaluated the clinical efficacy of postganglionic nerve block in symptomatic Schmorl nodules (SNs).SUMMARY OF LITERATURE REVIEW: SNs are common lesions that are often asymptomatic. In certain cases, SNs have been reported to cause severe axial back pain, thereby considerably impacting patients' quality of life. No consensus currently exists on the treatment of symptomatic SNs. MATERIALS AND METHODS: From October 2015 to October 2017, a total of 21 patients with symptomatic SNs diagnosed by magnetic resonance imaging (MRI) that did not respond to conservative treatment after 4 weeks were included in the study. All patients received postganglionic nerve block. We evaluated effective pain relief (improvement of back pain of more than 50% compared with before the intervention) and functional improvements, assessed by visual analogue scale (VAS) and Oswestry Disability Index scores obtained at 4 hours, 4 weeks, 8 weeks, 3 months, and 6 months after the procedure. RESULTS: Symptomatic SNs were more common at the L2-3 level, and the lower end plate was more commonly involved than the upper end plate. Eighteen of the 21 patients (85.7%) showed effective pain relief, and no deterioration was observed within the follow-up period. Throughout the follow-up period, the VAS remained significantly improved compared to before the procedure (p < 0.05). Complications were not reported in any cases. CONCLUSIONS Postganglionic nerve block for symptomatic SNs that do not respond to conservative treatment is a non-invasive modality for pain relief.

OBJECTIVE: The aim of this study was to determine whether living with spouse contributes to cognitive decline and depressive symptom among elderly people. METHODS: 939 participants were recruited through Keum-Cheon center for dementia in Seoul. All subjects were assessed using the Korean version of Mini-Mental Status Examination (MMSE-K), Korean version of Consortium to Establish a Registry for Alzheimer's disease (CERAD-K) and Geriatric Depression Scale (GDS). Furthermore, epidemiological factors (age, sex, education year, and characteristics of family) assessed to find correlation with cognitive decline. RESULTS: Controlling for gender, age, and education year, living with spouse was significantly associated with cognitive decline (B=-0.486, S.E=0.203, Wald=0.331, Exp (B)=1.107, p=0.01). Living with spouse group has lower depressive symptom, compared to living without spouse group (F=14.6, p<0.01). CONCLUSION: Correctly, our results suggest that spouse absent state may accelerate cognitive decline and depression. Further, elderly people living alone should be closely monitored for both depression and cognitive impairment.
Aged ; Alzheimer Disease ; Dementia ; Depression ; Humans ; Spouses

PURPOSE: To determine the usefulness of fluid attenuated inversion recovery(FLAIR) imaging for the in detection of high signal intensity of hippocampus or amygdala in mesial temporal sclerosis (MTS), compared with that of turbo spin-echo T2-weighted imaging. MATERIALS AND METHODS: Two neuroradiologists independently analyzed randomly mixed MR images of 20 lesions of 17 patients in whom MTS had been diagnosed, and ten normal controls. All subjects underwent both who performed both FLAIR and turbo spin-echo T2-weighted imaging, in a blind fashion. In order to determine hippocampal morphology, oblique coronal images perpendicular to the long axis of the hippocampus were obtained. The detection rate of high signal intensity in hippocampus or amygdala, the radiologists 'preferred imaging sequence, and intersubject consistency of detection were evaluated. Signal intensity in hippocampus or amygdala was considered high if substantially higher than signal intensity in the cortex of adjacent temporo-parietal lobe. RESULTS: In all normal controls, FLAIR and spin-echo T2-weighted images showed normal signal intensity in hippocampus or amygdala. In MTS, the mean detection rate of high signal intensity in hippocampus or amyg-dala, as seen on FLAIR images was 93%, compared with 43% on spin-echo T2-weighted images. In all cases in which signal intensity on FLAIR images was normal, signal intensity on spin-echo T2-weighted images was also normal. The radiologists preferred the contrast properties of FLAIR to those of spin-echo T2-weighted images. CONCLUSION: In the diagnosis of MTS using MRI, FLAIR images are more useful for the detection of high signal intensity of hippocampus or amygdala than are spin-echo T2-weighted images. In the diagnosis of MTS, FLAIR imaging is therefore a suitable alternative to spin-echo T2-weighted imaging.
Amygdala ; Axis, Cervical Vertebra ; Diagnosis ; Hippocampus ; Humans ; Magnetic Resonance Imaging ; Sclerosis

No abstract available.
Humans