Metaphyseal Chondrodysplasia is rare, hereditary disease characterized by defective enchondral bone formation with major manifestation at the metaphysis. Jansen originally used the term metaphyseal dysostosis in 1934 to describe a patient who has a short stature with irregular metaphysis of the lower extremity and hands. Schmid reported a milder form of Metaphyseal dysostosis in 1949, which is more common and is transmitted in autosomal dominant trait. Mukusick reported another form of Metaphyseal Chondrodysplasia which is associated with ectodermal abnormalities in 1964. The other different types were reported alos, but they are extremly rare. The basic defect in the disease may be the failure of hypertrophic cells to mature and degenerate, caused by a block in or deficiency of enzymes of glycolytic cycle. The skull and spine are spared. Serum chemistry and kidney function are normal. The only treatment necessary, once adequate diagnosis has been estabilished, is careful observation and properly timed corrective orthopaedic surgery. We experienced one case of Schmid Type Metaphyseal Chondrodysplasia. Corrective osteotomy was performed and satisfactory result was obtained.
Chemistry ; Diagnosis ; Dysostoses ; Ectoderm ; Genetic Diseases, Inborn ; Hand ; Humans ; Kidney ; Lower Extremity ; Osteogenesis ; Osteotomy ; Skull ; Spine

Twenty-four cases of complete acromioclavicular dislocation had been treated surgically by Weaver and Dunn method at Hanil Hospital during the period from March 1976 to March 1988. Among twenty-four cases, we had average 84.4 months follow-up data on fifteen cases with acute complete dislocation and two cases with chronic complete dislocation. The following results were obtained. 1. The transferred coracoacromial ligament had been secured to the resected clavicular end by wire or silk(silk in eleven cases, wire in six). All patients in wire sutured group had to be reoperated on because of breakage of wire. But no significat differences in final clinical function of the shoulder was seen. 2. Calcification was found in the region of the transferred coracoacromial ligament in twelve cases(70.6%). 3. All patients had normal shoulder motion, sixateen(94.1%) were asymptomatic, and only four(23.5%) felt mild weakness of shoulder. 4. On the clinical assessment by the criteria of Imatani et. al., 12 patients(70.6%) were graded excellent, 4 patients(23.5%) were good, and one patient(5.9%) was fair.
Acromioclavicular Joint ; Dislocations ; Follow-Up Studies ; Humans ; Ligaments ; Methods ; Shoulder

OBJECTIVES: Inverted papilloma is characterized by a high rate of recurrence, local aggressiveness and association with malignancy. We reviewed the 16-year experience of our institution in clinical presentation, history and the recurrence rate according to the tumor stage and surgical modalities. MATERIALS AND METHOD: A retrospective study was conducted on 102 patients diagnosed as inverted papilloma between January, 1986 through August, 2002. Demographic data, clinical presentations, and association with malignancy were analyzed. All patients with inverted papilloma were staged according to the Krouse's staging system. Sixty eight patients followed up at least 6 months were divided into endoscopic and conventional surgery groups. We compared the recurrence rate between the two groups according to the tumor stage. RESULT AND CONCLUSION: Patients were aged 19 to 81 years, and 73.5% were male. The most frequent presenting complaint was nasal obstruction. The most common site of involvement was the lateral nasal wall in the region of the middle meatus. The overall malignancy rate was 8.8% (9 cases), with synchronous tumors accounting for 7 cases and metachronous tumors accounting for 2 cases. When patients were first diagnosed, the most common stage was T2 (52.7%). The recurrence rates of T1, T2 and T3 were 10%, 11.1% and 4.8%, respectively, with no significant difference. Also the recurrence rates in endoscopic and conventional surgery group according to the stage had no significant difference.

Various bone conduction hearing aids have been widely used to improve hearing fuction for the last 30 years. Sophono(R), a newly introduced device without percutaneous abutment, improved disadvantages of previously used bone conduction hearing aids. Recently, we experienced a case of Sophono(R) implantation in a patient presenting with mixed hearing loss followed by tumor surgery. The patient gained air conduction threshold of 60 dB HL compared to preoperative pure tone audiogram and a speech discrimination test in noisy environment showed improved results. Furthermore, the patient has had no cutaneous complications since implantation. The results of this case demonstrate the feasibility of implanting Sophono(R) in patients similar to this case. Considering its advantages of MRI compatibility and non-invasive surgery, as well as esthetical benefit, Sophono(R) implantation could be a preferable option for hearing rehabilitation.
Bone Conduction ; Hearing Loss, Mixed Conductive-Sensorineural ; Hearing Loss, Unilateral* ; Hearing* ; Humans ; Magnetic Resonance Imaging ; Meningioma* ; Rehabilitation* ; Speech Discrimination Tests

In this study, the effects of verapamil on growth rate, apoptosis, production of transforming growth factor (TGF-beta) and fibronectin were evaluated in keloid and normal human dermal fibroblasts. Both fibroblasts were primarily cultured from earlobe keloids of three female patients and treated with various concentrations of verapamil. Cell toxicity was assessed by MTT assay, growth rate and apoptosis by FACS, and the production of TGF-beta and fibronectin by ELISA and Western blot, respectively. In the MTT50, the cell growth was more suppressed in keloid fibroblasts. In the MTT90, cell growth was more stimulated in normal fibroblasts. No significant effect appeared on TGF-beta expression but an increase in extracellular fibronectin secretion was found in keloid fibroblasts. Keloid fibroblasts responded to verapamil more sensitively, and the percentage of apoptosis was higher at the MTT50l. In brief, verapamil had growth-inhibitory effect with inducing apoptosis at the MTT50, but rather growth-stimulatory effect at the MTT90. The biphasic effect of verapamil depending on the dose might explain one of the reasons of relapse after keloid treatment with verapamil. Clinical application with high concentration (2.5mg/ml) is advised unless excessive dosage is used.
Apoptosis* ; Blotting, Western ; Enzyme-Linked Immunosorbent Assay ; Female ; Fibroblasts* ; Fibronectins ; Humans ; Keloid* ; Recurrence ; Transforming Growth Factor beta ; Transforming Growth Factors ; Verapamil*

BACKGROUND AND OBJECTIVES: It has been reported that ozone exposure exacerbate allergic rhinitis symptoms and may contribute to increase allergic rhinitis prevalence. However, a causal relationship still remained unsolved. The purpose of this study is to investigate whether prolonged exposure to ozone induce Th2 immune response without allergen. MATERIALS AND METHODS: Fourteen BALB/c mice were divided into two groups: control group and ozone exposure group. Mice were exposed to 0.3 ppm of ozone for 4 hours a day, 3 times per week, for 4 weeks. At 24 hour after the last ozone exposure, nasal lavage fluid (NLF) was obtained to measure the levels of cytokine IL-4, IL-5, and IFN-gamma. After lavage fluid was obtained, blood was obtained via inferior vena cava to measure the amount of total IgE and IgG1. The concentration of cytokines and immunoglobulins was measured using the ELISA method. In addition, Luna staining was performed to identify eosinophils infiltrated in nasal mucosa. RESULTS: The levels of IL-4 and IL-5 in NLF were significantly increased in ozone exposure group compared with control group. But the level of IFN-gamma in NLF shows no significant difference between two groups. Serum total IgE and IgG1 were significantly increased in ozone exposure group compared with control group. On histologic examination, number of eosinophils infiltrating nasal mucosa was significantly increased in ozone exposure group. CONCLUSION: These results suggest that repeated ozone exposure induces Th2 response in the nasal mucosa of mice.
Animals ; Cytokines ; Enzyme-Linked Immunosorbent Assay ; Eosinophils ; Immunoglobulin E ; Immunoglobulin G ; Immunoglobulins ; Interleukin-4 ; Interleukin-5 ; Mice* ; Nasal Lavage Fluid ; Nasal Mucosa ; Ozone* ; Prevalence ; Rhinitis ; Therapeutic Irrigation ; Vena Cava, Inferior

Cholesteatoma is a benign disease but it has an aggressive feature that may lead to serious conditions. Many strategies have been introduced for the treatment cholesteatoma, yet no definite single method has been established: it should be treated respectively regarding the completion of cholesteatoma, conservation of tympanic mucosa, proper ventilation of middle ear and preservation of hearing. A 25-year old patient visited our clinic for right-sided facial paralysis of 5 days. The patient underwent canal wall down mastoidectomy 10 years ago due to cholesteatoma and a few revision surgeries under local anesthesia for recurrence. Pre-operative temporal bone CT showed suspected recurred cholesteatoma in internal auditory canal and labyrinthine segment of facial nerve nearby. The patient underwent a removal of cholesteatoma via middle cranial fossa approach. This case report is a successful completion of recurred cholesteatoma, for which no recurrence is shown. Facial nerve function is improved at postoperative 1 year.
Anesthesia, Local ; Cholesteatoma* ; Cranial Fossa, Middle* ; Ear, Middle ; Facial Nerve ; Facial Paralysis ; Hearing ; Humans ; Mucous Membrane ; Recurrence ; Temporal Bone ; Ventilation

BACKGROUND AND OBJECTIVES: The imbalance between a Th1 response and a Th2 response may be one feature of allergic diseases' pathophysiology. IL-18, originally called the IFN-gamma inducing factor, stimulates both Th1 and Th2 responses depending on cytokine milieu. The aim of this study was to investigate and compare the effect of intranasal and intraperitoneal administration of IL-18 in mice with experimentally induced airway allergy. MATERIALS AND METHOD: Female BALB/c mice were systemically sensitized with ovalbumin mixed with aluminum hydroxide and were challenged with the intranasal instillation of ovalbumin. IL-18 was instilled to nasal cavities or injected into peritoneum according to the experimental group. After provocation test, bronchoalveolar lavage fluid (BALF) was collected and IL-4, IL-5, IL-18 and IFN-gamma were measured. The degree of eosinophilic infiltration were evaluated at the peribronchial region of the lung specimen. RESULTS: The concentration of IL-4 and IL-5 within BALF and peribronchial infiltration of eosinophils were significantly suppressed in the groups treated with IL-18 than in the control group. The intranasal instillation of IL-18 suppressed Th2 cytokines more effectively than the intraperitoneal injection. CONCLUSION: It is suggested that allergic inflammation can be suppressed with the intranasal instillation of IL-18.
Aluminum Hydroxide ; Animals ; Bronchoalveolar Lavage Fluid ; Cytokines ; Eosinophils ; Female ; Humans ; Hypersensitivity ; Inflammation ; Injections, Intraperitoneal ; Interleukin-18* ; Interleukin-4 ; Interleukin-5 ; Lung ; Mice* ; Nasal Cavity ; Ovalbumin ; Peritoneum