Maffuccis syndrome was first reported by Maffucci, in 1881. This syndrome is manifested by multiple enchondromas and hemangiomas, characterized by non-hereditary mesenchymal neoplasia that has a propensity for malignant transformation. Involvement of long bones, particularly the bones of the hands and feet, is most common. The most important complication of this syndrome is the high frequency of chondr osarcomatous metaplasia. We report a case of Maffuccis syndrome in a 56-year-old woman with multiple hemangioma on both the hands and forearms, left foot and enchondromas of multiple bones. The disease started at the age of 8 and was associated with deformity, but malignant transformation was not found.
Chondroma ; Congenital Abnormalities ; Enchondromatosis ; Female ; Foot ; Forearm ; Hand ; Hemangioma ; Humans ; Metaplasia ; Middle Aged

No abstract available.
Pregnancy*

BACKGROUND: This study investigated the efficacy and safety of combined subacromial and intravenous patient-controlled analgesia for control of postoperative pain after arthroscopic rotator cuff repair. METHODS: Between May 2012 and August 2014, 60 patients who underwent arthroscopic rotator cuff repair with acromioplasty and received patient-controlled analgesia were studied prospectively. Cases were divided into 2 groups: combined subacromial and intravenous infusion group (group A, 30 cases) and solitary intravenous infusion group (group B, 30 cases). The visual analogue scale was used to record the patient's level of pain every 12 hours during postoperative 72 hours and the following 48 hours after the suspension of patient-controlled analgesia. RESULTS: The mean preoperative visual analogue scale score was 7.8 in group A and 7.6 in group B, and the immediate postoperative visual analogue scale score was 7.9 and 8.1 for each group. At postoperative time (From 12 hours to 72 hours after operation), the scores of combined subacromial and intravenous infusion were significantly lower than those of solitary intravenous infusion. Significant difference in the frequency of supplemental analgesic injections was observed between group A and group B (p=0.008). However, no significant difference in complication rate was observed between the two groups (p=0.562). CONCLUSIONS: Combined subacromial and intravenous patient-controlled analgesia after arthroscopic rotator cuff repair is more effective than solitary intravenous infusion without significantly increasing complications. Therefore, combined subacromial and intravenous patient-controlled analgesia could be a effective pain control method.
Analgesia, Patient-Controlled* ; Humans ; Infusions, Intravenous ; Methods ; Pain, Postoperative ; Prospective Studies ; Rotator Cuff*

We report a case of epidermolysis bullosa simplex occurred in a 12-year-old girl, who presented with intractable bullous lesions of the hands and feet after minor traumas ever since her early neonatal period. The bullous lesions were noted on the 2nd and 4th webs of the fingers and dorsum of the hands as well as on the skin of the ankle. The lesions were healed without scar formation. Family history was not contributary and seasonal pattern was not noted. Histologic sections revealed intraepidermal bullae just above the well preserved basement membrane. Electron microscopic findings revealed cytolysis of basal cells with preservation of the basement membrane, indicating the epidermal type of epidermolysis bullosa simplex.
Infant, Newborn ; Humans

BACKGROUND: The aim of the present study was to determine the correlation between the amount of pain reduction after local anesthetic injection into the subacromial space preoperatively and clinical outcome after arthroscopic rotator cuff repair. METHODS: A total of 127 patients who underwent arthroscopic rotator cuff repair and followed up at least 1 year were analyzed retrospectively. Preoperatively, a visual analogue scale (VAS) for pain was measured in all patients before and after the ultrasound guided impingement test. The participants were divied into four groups according to pain reduntion ater impingement test (Group A: >75%, Group B: 50%–75%, Group C: 25%–50%, Group D: <25%). VAS for pain, shoulder range of motion, shoulder isometric strength, ASES score were evaluated preoperatively and at 3, 6, 9, and 12 months postoperatively. RESULTS: After surgery, the amount of pain reduction shows significantly at 3, 6 months in Groups A, B as compared to Groups C, D (p<0.05). Among the range of motion of shoulder joint, forward flexion was significantly improved in Group A at 3 months (p<0.05). The ASES score significantly improved at 3, 6 months in Groups A, B as compared to Group C, D (p<0.05). CONCLUSIONS: Preoperative degree of pain reduction after impingement test correlates with the improvement of pain after arthroscopic rotator cuff repair, especially in the early phase. Therefore, the impingement test could be effectively used.
Humans ; Range of Motion, Articular ; Retrospective Studies ; Rotator Cuff* ; Shoulder ; Shoulder Joint ; Shoulder Pain ; Ultrasonography

CHAP syndorme consists of choreoathetosis, orofacial dyskinesia, affective change, hypotonia, and pseudobulbar signs. CHAP syndrome was firstly des- cribed with onset 1 to 7 days after induced profound hypothermia and complete circulatory arrest for con- genital heart lesion repair. Since then, only a few cases have been reported until now. Case We report a patient who demonstrated all of the elements of the CHAP syndrome in severe cachexic state. Brain SPECT(99mTC-ECD) shows decreased perfusion in left fronto-parieto-temporal lobe. Comments The pathogenesis of CHAP syndrome is obscure. However our case led us to suggest that more essential component pre- disposing to the CHAP syndrome is hypoxia rather than hypothermia. Furthermore, it seems to be related with circulatory insufficiency in extrapyramidal nervous systems.
Anoxia ; Brain ; Heart ; Humans ; Hypothermia ; Movement Disorders ; Muscle Hypotonia ; Nervous System ; Perfusion

BACKGROUND: As co-factors of metalloenzymes, zinc(Zn) and copper(Cu) have a considerable effect on nearly all the metabolism that takes place in organs of the body, including the skin. OBJECTIVE: The purpose of this study was to evaluate zinc and copper status in alopecia areata patients. METHODS: We measured serum levels of zinc and copper in 30 cases of alopecia areata patients and 10 normal individuals by atomic absorption spectrometry. Furthermore, we studied the significance of the ratio between serum levels of the two metals. RESULTS: The serum levels of zinc were significantly lower in alopecia areata patients than in healthy controls. The serum levels of copper were slightly lower in alopecia areata patients than in healthy controls. Ratios of Cu/Zn were slightly higher in alopecia areata patients than in healthy controls. But their differences were not statistically significant. CONCLUSION: We suggest that a serum Zn assay should be included in the chemical assessment of patients with alopecia areata.
Absorption ; Alopecia Areata* ; Alopecia* ; Copper* ; Humans ; Metabolism ; Metals ; Skin ; Spectrum Analysis ; Zinc*

A 29 year old woman with unresectable leiomyosarcoma of the inferior vena cave extending into the right atrium of the heart was presented. The upper and middle inferior vena cava involved and there were both intrinsic and extrinsic tumor growth components. The diagnosis was made by a combination of radiological studies including ultrasound, CT, inferior vena cavography and arteriography and the diagnosis was confirmed by ultrasound guided rumor biopsy. The clinical and radiological presenting features were reviewed.
Angiography ; Biopsy ; Diagnosis ; Female ; Heart ; Heart Atria ; Humans ; Leiomyosarcoma* ; Ultrasonography ; Vena Cava, Inferior*

A 29 year old woman with unresectable leiomyosarcoma of the inferior vena cave extending into the right atrium of the heart was presented. The upper and middle inferior vena cava involved and there were both intrinsic and extrinsic tumor growth components. The diagnosis was made by a combination of radiological studies including ultrasound, CT, inferior vena cavography and arteriography and the diagnosis was confirmed by ultrasound guided rumor biopsy. The clinical and radiological presenting features were reviewed.
Angiography ; Biopsy ; Diagnosis ; Female ; Heart ; Heart Atria ; Humans ; Leiomyosarcoma* ; Ultrasonography ; Vena Cava, Inferior*

No abstract available.
Hernia, Umbilical* ; Ultrasonography*