Treatment of perianal and vulvar extramammary Paget disease (EMPD), rare intraepithelial malignancies, is often challenging because of its potential to spread into the anal canal. However, there is still no consensus regarding the optimal resection margin within the anal canal. Between 2004 and 2014, six patients (three with perianal EMPD and three with vulvar EMPD) in which the spread of Paget cells into the anal canal was highly suspected were referred to our department. To evaluate the disease extent within the anal canal, preoperative mapping biopsy of the anal canal was performed in five out of six patients. Two patients were positive for Paget cells within the anal canal (one at the dentate line and the other at 0.5 cm above the dentate line), whereas in three patients, Paget cell were present only in the skin of the anal verge. Using 1 cm margin within the anal canal from the positive biopsy sites, we performed anal-preserving wide local excision (WLE), and negative resection margins within the anal canal were confirmed in all five patients. The remaining one patient with perianal EMPD did not undergo mapping biopsy of the anal canal because preoperative colonoscopy revealed that the Paget cells had spread into the lower rectum. Therefore, WLE with abdominoperineal resection was performed. During the median follow-up period of 37.3 months, no local recurrence was observed in all patients. Our small case series suggest the usefulness of mapping biopsy of the anal canal for the treatment of perianal and vulvar EMPD.
Anal Canal* ; Biopsy* ; Colonoscopy ; Consensus ; Follow-Up Studies ; Humans ; Paget Disease, Extramammary* ; Rectum ; Recurrence ; Skin

Treatment of perianal and vulvar extramammary Paget disease (EMPD), rare intraepithelial malignancies, is often challenging because of its potential to spread into the anal canal. However, there is still no consensus regarding the optimal resection margin within the anal canal. Between 2004 and 2014, six patients (three with perianal EMPD and three with vulvar EMPD) in which the spread of Paget cells into the anal canal was highly suspected were referred to our department. To evaluate the disease extent within the anal canal, preoperative mapping biopsy of the anal canal was performed in five out of six patients. Two patients were positive for Paget cells within the anal canal (one at the dentate line and the other at 0.5 cm above the dentate line), whereas in three patients, Paget cell were present only in the skin of the anal verge. Using 1 cm margin within the anal canal from the positive biopsy sites, we performed anal-preserving wide local excision (WLE), and negative resection margins within the anal canal were confirmed in all five patients. The remaining one patient with perianal EMPD did not undergo mapping biopsy of the anal canal because preoperative colonoscopy revealed that the Paget cells had spread into the lower rectum. Therefore, WLE with abdominoperineal resection was performed. During the median follow-up period of 37.3 months, no local recurrence was observed in all patients. Our small case series suggest the usefulness of mapping biopsy of the anal canal for the treatment of perianal and vulvar EMPD.
Anal Canal* ; Biopsy* ; Colonoscopy ; Consensus ; Follow-Up Studies ; Humans ; Paget Disease, Extramammary* ; Rectum ; Recurrence ; Skin

To clarify changes in body temperature during endurance exercise in patients with spinal cord injury (SCI), we measured tympanic temperature (Tty) and skin temperature in the head, arm, chest, thigh, shin and calf in 5 patients with SCI (T6-T 12) and 7 normal controls during 30 minutes arm cranking exercise (20 watts) from 10 minutes before the initiation of exercise until 10 minutes after the termination of exercise in an artificial climate room at a temperature of about 25°C with a relative humidity of about 50%. The Tty in the SCI group was lower than that in the control group from 10 minutes before the initiation of exercise to 10 minutes after the termination of exercise with a significant difference only at the initiation of exercise. The difference in Tty slightly decreased with continuation of exercise. The Tty in the SCI group at rest was 36.05-37.15°C. Four patients in this group showed a decrease of 0.04-0.12°C in the early stage and an increase of 0.66°C±0.19 (mean±SD) at the end of exercise over the value at the initiation of exercise.
The skin temperature was lower in the SCI group than in the control group in all sites excluding the arm. Significant differences were observed in the head in the early stage of exercise and after exercise, in the chest from 10 minutes before the initiation of exercise to 5 minutes after the termination of exercise, in the thigh from 10 minutes before the initiation of exercise to 10 minutes after the termination of exercise, in the shin 10 minutes and 5 minutes before the initiation of exercise, and in the calf from before to 15 minutes after the initiation of exercise. In the SCI group, marked individual differences were observed in the skin temperatures in the thigh, shin, and calf, suggesting specificity of the skin temperature response in and near the paralysis area.
Results in Tty in this study suggested no heat retention in the SCI patients. Therefore, the risk for heat disorders seems to be low during moderate or mild exercise under moderate temperature environment at a temperature of about 25°C with a relative humidity of about 50% even when the skin temperature is low, and thermolysis is not marked.

Eighteen persons with both severe mental retardation and severe physical disabilities and ten normal persons participated to investigate cardiovascular response to hydrostatic pressure stress using supine and sitting positions. All of the handicapped subjects lay down on the bed during the entire day. They were classified into three groups. That is, the first group (LD) had not opportunity of sitting passively in daily living at all. Another two groups were given opportunity of sitting. In addition, the second group (ST 1) was unable to sit and stand independently in the past but the third group (ST 2) was able. The LD showed unchanged heart rate and decreased blood pressure with reduced peripheral blood flow in the sitting position. This suggested that serious lower function or dysfunction in both central and peripheral circulatory system. The cardiovascular function of the ST 1 was characterized by elevated heart rate and lowered blood pressure. This seemed to indicate lower baroreflex control to the peripheral vascular system. The ST 2 demonstrated similar function to the normal persons. The cardiovascular regulation in the severely handicapped persons may depend on opportu-unity of taking orthostatic posture in daily living in addition to motor adility in the past.

Daily physical activities for 12 severely physically and mentally handicapped persons were investigated using heart rates during a 24-hr period. The recorded heart rates were processed statistically and analyzed. The standard deviation and skewness for the severely handicapped persons with low ambulatory ability were significantly greater than those for the most severely handicapped persons without ambulatory ability. These properties confirmed that physical activities of daily living were extremely different for the two groups. The severely handicapped persons with low ambulatory ability had higher heart rates of about 10% relating to active physical activities. It was suggested that the active physical activities of daily living could contribute to the improvement of physical work capacity if these activities were more prolonged in duration. On the other hand, such higher heart rates were not observed in the case of most severely handicapped persons and extremely low function in their cardiorespiratory system was suspected. It became impor. tant to apply physical training to the most severely handicapped persons even though light work was performed passively.