Osteogenesis imperfecta is a rare affection characterized by fragility of the bones, blue sclerae, and deafness, less freqently by hypermobility of the joints. The etiology is unknown, but it appears to be a mesenchymal defect. A case of osteogenesis imperfecta(tarda form). in a 8 ycar old girl, is presented with a review of the literatures. The chief complaints were bowing deformities of the right upper arm and both lower extremities, stunted growth, blue sclerae, dental defects, weakness of the muscles and pigeon breast. X-Ray showed multiple malunited fractures of the right humerus, both femurs, and bilaterally of tibiae and fibulae.
Arm ; Breast ; Columbidae ; Congenital Abnormalities ; Deafness ; Female ; Femur ; Fibula ; Fractures, Malunited ; Growth Disorders ; Humans ; Humerus ; Joints ; Lower Extremity ; Muscles ; Osteogenesis Imperfecta ; Osteogenesis ; Sclera ; Tibia

BACKGROUND: Chronic exposure to ultraviolet radiation(UVE) nduces photoaging characterized by dry, deeply wrinkled, inelastic, leathery, and irregulaity pigmented skin. UVR also induces solar keratosis and carcinoma, and is a contributing factor in melanoma. Sunscreens are used to prevent solar damage to skin and, if used on a daily lass should significantly reduce the incidence of the chronie photodamaging events. OBJECTIVE: We tried to evaulate the photoaging effects of UVR in the skin and the photoprotective effect of sunscreens. METHOD: We examined the gross and microscopic changes skin of albino hairless Skh : HR-1 mice exposed chronically to ultraviolet B(UVB) and suncreen-treated mice. RESULTS: The skin of the UVB-irradiated mouse shows chear, cteristic signs of photoaging, such as deep wrinkles across the back, and thickened and a hery skin. Histologically, the photoaged skin shows increased epidermal thickness, numeross fibroblasts and inflammatory cell infiltration in the upper dermis, and many enlarged keratering cysts in the lower dermis. By the 20th week, seven of the total of 9 mice(78%) in UVB irradiated mice developed at least one tumor. Histologically, the tumor is a papilloma, but the he are many dyskeratotic cells and loss of polarity in epidermis. Octyl methoxycinnamate or TiO ZnO Talc-treated mic show a significantly decreased wrinkling score, mimmal epidermal hyperplasia, slightly increased dermal cellularity, and lack of proliferation of cysts. The octyl dimethyl PABA-treatd mice shows significantly increased wrinkling score and marked inflammatory infiltration dermis. By the 20th week, only one mouse had developed a tumor in the octyl methoxy irmamate-treated group and no evidence of tumor was seen in the TiO ZnO Talc-treated group. In the octyl dimethyl PABA-treated group, five of 7 mice(71%) developed at least one tumor. CONCLUSION: The skin which is chronically exposed to UVB is subject to photoaging and photocarcinogenesis and regular use of an adequate sunscreen would prevent these photodamaging effects of UVB.
Animals ; Dermis ; Epidermis ; Fibroblasts ; Hyperplasia ; Incidence ; Keratosis ; Melanoma ; Mice ; Mice, Hairless ; Papilloma ; Skin* ; Sunscreening Agents*

The term pseudolymphoma is not specific, and a variety of infl; m atory reactions may simulate clinically and histopat,hologically a cutaneous malignant lymphomas udolymphoma induced by drug therapies, especially anticonvulsants, have been reportecl. These pseidayrnphornas most commonly appear as single lesions. Characteristically, few localized lesions disappear after discontinuing therapy with the offending drug. Multiple and generalized tumors are very rate. We report a case of generalized cutaneous pseudolymphoma assocated with phenytoin therapy in a 52-year old male patient.
Anticonvulsants ; Drug Therapy ; Humans ; Lymphoma ; Male ; Middle Aged ; Phenytoin* ; Pseudolymphoma*

OBJECTIVES: Daytime drowsiness or sedation and changes in night sleep are commonly seen in patients treated with clozapine. There is, however, very limited informatio on their degree and nature during the course of treatment. The purpose of this study was to understand the sleep patterns in chronic schizophrenic patients with clozapine treatment over a period of 24 weeks. METHOD: The sleep pattern was evaluated using a set of 5-point scale questionnaire, to record subjective impressions of the night sleep induction, maintenance and quality, and daytime drowsiness and fatigue. In addition, unusual experiences associated with night sleep were recorded. The sleep questionnaire was repeatedly administered at baseline and at 1, 2, 4, 8, 12 and 24 weeks of drug treatment. At present, data on 12 patients has been collected. RESULTS: All the components of night sleep were significantly improved in th 1st through the 12 th week after treatment with clozapine. Daytime drowsiness was significantly higher in the 1st to the 2nd week after the treatment and fatigue was also significantly higher in the 1st to the 4th week after the treatment. Eight patients experienced noticeable increases in salivation during night sleep, and of these, one also reported frequent nocturnal urination and even enuresis. However, all these adverse factors did not affect the major sleep patterns. CONCLUSIONS: These finding suggest that the beneficial effects of clozapine on night sleep might last much longer than the undesirable effect of daytime drowsiness and fatigue. In other words, tolerance of the hypnotic action of clozapine might develop late and tolerance of the daytime drowsiness and fatigue might be evident earlier.
Clozapine* ; Enuresis ; Fatigue ; Humans ; Surveys and Questionnaires ; Salivation ; Sleep Stages ; Urination

No abstract available.
Child* ; Humans

One adverse effect of PUVA therapy is the development of severe dermatitis. Only a few cases of photoallergy to psoralens during PUVA therapy have been reported. We describe herein a patient with photoallergic dermatitis induced by PUVA with 8-methoxypsoralen(8-MOP). A 38-year old woman with generalized vitiligo had acute pruritic exanthematous maculopapular lesions in the treated areas after PUVA therapy with 8-MOP. A patch test and photopatch test were performed with 8-MOP, 5-MOP, and 4,5,8-trimethylpsoralen(TMP). The patch test carried out with these psoralen derivatives were all negative, but the photopatch test showed a positive reaction to 8-MOP. The patient consequently had PUVA therapy with 5-MOP and she had no further experience of a photoallergic reaction.
Dermatitis ; Dermatitis, Photoallergic* ; Female ; Ficusin ; Furocoumarins ; Humans ; Methoxsalen* ; Patch Tests ; PUVA Therapy ; Vitiligo

The gene encoding the 66 kilodalton (kDa) protein of Borrelia hermsii HS1 was cloned and sequenced. Chromosomal DNA was prepared from purified B. hermsii and used in construction of genomic library. The library was screened for positive clones by 314 bp DIG-labeled probe synthesized on the basis of the part of the sequence of B. hermsii. Positive clone was subcloned into p2ErO vector and was designated as pBH11. pBH11 were subcloned into pBluscript vector and were designated as pBH11-1 (500 bp), pBH11-2 (800 bp), pBH11-3 (600 bp) and pBH11-4 (800 bp). The plasmids were sequenced and determined the nucleotide sequence of p66. The open reading frame of the p66 consisted of 1803 base pairs coding for 600 amino acid protein. The basic information on the p66 gene of B. hermsii HS1 obtained from this study will be useful for further analysis and experiment of pathogenesis of the borrelia.
Base Pairing ; Base Sequence ; Borrelia* ; Clinical Coding ; Clone Cells* ; Cloning, Organism* ; DNA ; Genomic Library ; Open Reading Frames ; Plasmids

Hydroa vacciniforme is a very rare photosensitivity disorder. The primary skin lesion is a vesicle or bulla which then heals with vacciniform scarring. We report a case of hydroa vacciniforme recurred after 3 years period of quiescence in a 20 year-old man who had had history of the disease from the age of two. The duplication of the natural lesion, clinically and histologically, was successfully made by artificial UV-A irradiation on the patient's back.
Cicatrix ; Hydroa Vacciniforme* ; Photosensitivity Disorders ; Skin

BACKGROUND: The execution of fibular allograft augmentation in unstable proximal humerus fractures (PHFs) was technically demanding. In this study, the authors evaluated the clinical and radiographic outcomes after tricortical iliac allograft (TIA) augmentation in PHFs. METHODS: We retrospectively assessed 38 PHF patients treated with locking-plate fixation and TIA augmentation. Insertion of a TIA was indicated when an unstable PHF showed a large cavitary defect and poor medial column support after open reduction, regardless of the presence of medial cortical comminution in preoperative images. Radiographic imaging parameters (humeral head height, HHH; humeral neck-shaft angle, HNSA; head mediolateral offset, HMLO; and status of the union), Constant score, and range of motion were evaluated. Patients were grouped according to whether the medial column support after open reduction was poor or not (groups A and B, respectively); clinical outcomes were compared for all parameters. RESULTS: All fractures healed radiologically (average duration to complete union, 5.8 months). At final evaluation, the average Constant score was 73 points and the mean active forward flexion was 148°. Based on the Paavolainen assessment method, 33 patients had good results and 5 patients showed fair results. The mean loss of reduction was 1.32 mm in HHH and 5.02% in HMLO. None of the parameters evaluated showed a statistically significant difference between the two groups (poor and not poor medial column support). CONCLUSIONS: In unstable PHFs, TIA augmentation can provide good clinical and radiological results when there are poor medial column support and a large cavitary defect after open reduction.
Allografts ; Head ; Humans ; Humerus ; Methods ; Range of Motion, Articular ; Retrospective Studies

PURPOSE: To report two cases with recurrent involvement of the optic nerve as the initial sign of acute leukemic relapse.CASE SUMMARY: An 8-year-old male with acute lymphoblastic leukemia on the maintenance chemotherapy was referred for a decrease in visual acuity in the right eye. The visual acuity and optic disc swelling were completely resolved with high-dose steroid therapy. Two months after the initial presentation, the symptoms recurred and brain/orbit magnetic resonance imaging (MRI) showed high intensity along the right optic nerve from the retrobulbar area to the optic chiasm. The visual acuity was restored after high-dose steroid therapy. One month after the second attack, the symptoms recurred and the cerebrospinal fluid cytology was positive for lymphoblasts. Three weeks after the intrathecal chemotherapy, the visual acuity improved fully, but optic disc atrophy developed. A 45-year-old male, who received allogenic peripheral blood stem cell transplantation for acute myeloid leukemia, presented with a decrease in visual acuity in the left eye. The left optic disc swelling improved with high-dose steroid therapy, but the medication was restarted due to the recurrence of symptoms 3 weeks later. Brain MRI showed a mass lesion compressing the left optic nerve, presumed to be a myeloid sarcoma. One month after local irradiation, the visual acuity was no light perception in the left eye.CONCLUSIONS: In patients with a prior history of acute leukemia, the recurrent involvement of the optic nerve should be considered as a central nerve system relapse, regardless of improvement with steroid treatment.