No abstract available.
Osteopetrosis*

No abstract available.
Hyperostosis, Cortical, Congenital*

No abstract available.
Pyloric Stenosis, Hypertrophic*

The clinical entity of Dysplasia Epiphyseal Multiplex was first descrihed by Fairbank in 1935, characterized by the disturbance of endochondral ossification in hoth epiphyseal centers and regions of physeal growth. It manifests itself radiologically as late appearance and mottling of the ossification centers and clinically as short stature, stubby digits and painful stiffness of multiple joints. It is typically transmitted as an autosomal dominant trait though recessive forms have been described. The spine is normal apart from a mild increased lumbar lordosis. Many patients are referred to an orthopaedic surgeon for bilateral Perthes disease, as was one of the authors cases. This Paper reports four cases of multiple epiphyseal dysplasia which affected one family.
Animals ; Hip ; Humans ; Joints ; Knee ; Legg-Calve-Perthes Disease ; Lordosis ; Osteochondrodysplasias* ; Spine

Patients with pixie ear have an attached, tapering, and low-set earlobe. Traditional methods usually describe excision of the caudal portion of the lobule and reattachment in a more superior position. The present report suggests a simplified skin redraping method for correction of pixie ear. The procedure provides easy method to design and perform, which only requires elevation and trimming of the skin. Other ancillary procedures, such as flap design, anchoring, plication, and subdermal fixation, are not required. This method produces satisfactory results. Postoperative scar is invisible because the incision is on the retroauricular region, and the corrected earlobe has a more natural appearance than the repositioned earlobe. Moreover, skin redraping avoids tension, which contributes to minimization of the postoperative scar.

Background: Spine-hip discordance (SHD) increases fracture risk. However, its prevalence and clinical implications have not been investigated in patients with hip fractures. This study determined the prevalence and association of SHD with mortality and investigated the cause of SHD in patients with hip fractures. Methods: This study included patients admitted for fragility hip fractures between 2011 and 2020. All patients underwent dual energy X-ray absorptiometry and anteroposterior and lateral views of the lumbosacral spine during admission. Data on demographics, diagnosis, American Society of Anesthesiologists score, and mortality were collected. A T-score difference of more than 1.5 between L1-4 and the femur neck was considered discordant, and 3 groups (lumbar low [LL] discordance, no discordance [ND], and femur neck low [FL] discordance) were compared. In the discordance group, lumbar radiographs were reviewed to determine the cause of discordance. Results: Among 1,220 eligible patients, 130 were excluded due to patient refusal or bilateral hip implantation; therefore, this study included 1,090 patients (271 male and 819 female). The prevalence of LL, ND, and FL was 4.4%, 66.4% and 29.2% in men and 3.9%, 76.1%, and 20.0% women. Mortality was not associated with discordance. The most common causes of discordance were physiological in the LL group and pathological in the FL group for both sexes. Conclusions Patients with hip fractures showed lower rates of ND and higher rates of FL compared to the general population. True discordance should be carefully judged for pathological and artifact reasons. The clinical implications of SHD require further investigation.

No abstract available.
Shock, Septic*

No abstract available.
Niemann-Pick Disease, Type A*

BACKGROUND: Hypothermia protects the brain by suppressing the cerebral metabolism and it is performed well enough before the total circulatory arrest(TCA) in the operation of aortic disease. Generally, TCA has been performed depending on the rectal or nasopharyngeal temperatures; however, there is no definite range of optimal temperature for TCA or an objective indicator determining the temperature for safe TCA. In this study, we tried to determine the optimal range of temperature for safe hypothermic circulatory arrest by using the intraoperative electroencephalogram(EEG), and studied the role of EEG as an indicator of optimal hypothermia. MATERIAL AND METHOD: Between March, 1999 and August 31, 2000, 27 patients underwent graft replacement of the part of thoracic aorta using hypothermia and TCA with intraoperative EEG. The rectal and nasopharyngeal temperatures were monitored continuously from the time of anesthetic induction and the EEG was recorded with a ten-channel portable electroencephalography from the time of anesthetic induction to electrocerebral silence(ECS). RESULT: On ECS, the rectal and nasopharyngeal temperatures were not consistent but variable(rectal 11degree C -25degree C, nasopharynx 7.7degree C -23degree C). The correlation between two temperatures was not significant(p=0.171). The cooling time from the start of cardiopulmonary bypass to ECS was also variable(25-127min), but correlated with the body surface area(p=0.027). CONCLUSION: We have found that ECS appeared at various body temperatures, and thus, the use of rectal or nasopharyngeal temperature were not useful in identifying ECS. Conclusively, we can not fully assure cerebral protection during hypothermic circulatory arrest in regards to the body temperatures, and therefore, the intraoperative EEG is one of the necessary methods for determining the range of optimal hypothermia for safe circulatory arrest.
Aorta, Thoracic ; Aortic Diseases ; Body Temperature ; Brain ; Cardiopulmonary Bypass ; Electroencephalography* ; Humans* ; Hypothermia ; Metabolism ; Nasopharynx ; Transplants

Ureteral obstruction causes increase in renal blood flow (RBF) and partial impairment of the autoregulation of RBF. Although increased renal prostaglandin production is responsible for the former, it is not clear whether or not it is also responsible for the latter. Therefore, we investigated the role which prostaglandins play in the autoregulation of RBF during an ureteral pressure elevation (40 cmH2O). Since the major mechanism of RBF autoregulation is the tubuloglomerular feedback studying the interaction between ureteral pressure and RBF autoregulation may reveal the role of prostaglandin in tubuloglomerular feedback. To pursue the purpose, six anesthetized dogs were prepared for the measurements of RBF, mean systemic and renal arterial pressure (RAP) and the manipulation of ureteral pressure. The autoregulation curves were determined during both control and elevation of the ureteral pressure, before and after the pretreatment with indomethacin, a cyclooxygenase inhibitor. The desired ureteral pressure was achieved by vertically elevating the water-filled reservoir connected to the ureteral catheter to 40 cm above the kidney level. In response to the elevation of the ureteral pressure, RBF increased from 170 +/- 8 ml cntdot min -1 to 189+/-8, and the systemic arterial pressure didn't change significantly. During spontaneous urine flow, RBF autoregulation was abolished when RAP was reduced to 59+/-3 mmHg. On the other hand, during the ureteral pressure elevation, the autoregulation curves shifted upward and rightward from control, and the pressure when RBF autoregulation was abolished was 74 +/- 3 mmHg. The pretreatment of the dogs with indomethacin failed to affect the lower limit of RBF autoregulation during both control (63 +/- 5 mmHg) and the elevated ureteral pressure (77 +/- 5 mmHg). Since RBF failed to increase in response to the elevated ureteral pressure, RBF autoregulation curves obtained during the elevated ureteral pressure shifted only rightward from indomethacin control. The results indicate that the increased intrarenal level of prostaglandin or prostaglandin-induced vasodilation does not appear to bear any relation to the reduction in the autoregulatory capacity during partial ureteral obstruction. It seems that the partial impairment of the autoregulation during acute ureteral obstruction is due to the consumption of tubuloglomerular feedback mechanism at spontaneous RAP and that prostaglandin is neither mediator nor effector of tubuloglomerular feedback mechanism.
Animals ; Arterial Pressure ; Dogs* ; Hand ; Homeostasis ; Indomethacin ; Kidney ; Prostaglandin-Endoperoxide Synthases ; Prostaglandins ; Prostaglandins I ; Renal Circulation* ; Social Control, Formal* ; Ureter* ; Ureteral Obstruction* ; Urinary Catheters ; Vasodilation