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Paget's disease of the breast

Paget's disease of the breast, also known as Paget's disease of the nipple, is a condition that outwardly may have the appearance of eczema - with skin changes involving the nipple of the breast. Usually only affecting one nipple, there may be redness, oozing and crusting and a sore that does not heal. more...

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Medicines

Paget's disease is caused by breast cancer and is present in about 2% of all breast cancers. It typically results when malignant cells from an underlying carcinoma that originated in the ducts of the mammary glands spread to the epithelium.

Recommended tests are a mammogram and a biopsy to confirm the diagnosis.

Treatment usually involves some kind of mastectomy to surgically remove the tumour. Chemotherapy and/or radiotherapy may be necessary.

The condition is named after Sir James Paget, an English surgeon who first described it in 1874.

Read more at Wikipedia.org


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Breast cancer in the nineties
From AORN Journal, 7/1/96 by Jo Buyske

Carcinoma of the breast is the most common cancer in women, and it is the second leading cause (after lung cancer) of cancer deaths in women, causing 46,000 deaths in 1993 (Figure 1).(1) In 1993, there were 1.7 million new cancer cases in the United States, and of that number, 182,000 were carcinomas of the female breast (Figure 2). Although carcinoma of the breast also develops in men, the incidence is greatly reduced--only 300 male deaths from breast cancer were reported in 1993.(2)

[Figures 1 & 2 ILLUSTRATION OMITTED]

The incidence of breast cancer has increased during the past two decades, but this rate has slowed since 1993.(3) Knowledge of breast cancer and its epidemiology, natural course, and response to treatment continue to evolve. Screening, diagnosis, and treatment are essential to survival, and the importance of early detection cannot be overemphasized. Patients must be well informed and should decide, in partnership with expert physicians and nurses, what their options are for treatment. This article discusses current understanding of these issues and provides an overview of screening, diagnosis, and treatment of breast cancer.

SCREENING

Thirty years ago, the five-year survival rate for patients with breast cancer was 60%. Currently, the survival rate for all stages of breast cancer is as high as 78%.(4) Most of this improvement is the result of earlier detection from improved screening and awareness, as well as from new chemotherapy, radiation therapy, and surgical treatment modalities.

The goal of screening is to identify breast cancer at the earliest possible stage, which allows the highest possibility of cure. Breast cancer begins as precancerous masses that progress to preclinical tumors (de, tumors too small to be detected by mammography or physical examination), then to tumors that can be detected only by mammography, and finally to tumors that are large enough to be detected by physical examination.

Mammography. Until the 1980s, the only way to identify breast cancer was by detecting palpable breast tumors, and these tumors often were far advanced by the time they were found. With mammography, breast tumors sometimes can be identified as many as two years before they become palpable, allowing a greater likelihood of cure. Mammography can identify tumors less than 1 mm in size that consist of only a few thousand cells.

Mammography is associated with a false-negative rate of 10% to 15%, however, which means that 10% to 15% of proven breast cancers cannot be seen on mammograms.(5) This may be because of the density or location of the tumors, the density and heterogeneity of the breasts, or the quality of the mammograms. It is, therefore, essential that women undergo physical examinations in addition to mammograms.

Although the National Cancer Institute has wavered in its recommendations on mammograms for women less than 50 years of age, the American Cancer Society continues to recommend that women have mammograms every one to two years beginning between 40 and 50 years of age.(6) At the Lahey Clinic Medical Center, Burlington, Mass, we recommend that patients less than 50 years of age have mammograms at least every two years and more often when any risk factors are present. We also recommend that after women reach 20 years of age, they perform monthly breast self-examinations and have annual examinations by their physicians. Women with higher than average risk factors, however, may require more intensive surveillance.

Risk factors. American women in the 1990s are aware of some of the risk factors for breast cancer, especially that of having family histories of breast cancer. What they are less aware of is that 80% of breast cancers are found in women who have no major risk factors.(7)

Variables that can increase a woman's risk of developing breast cancer include early menarche, late menopause, first full-term pregnancy after 30 years of age or no pregnancies at all, a family history that includes first-degree relatives (de, mother, sister, daughter) with breast cancer, a previous history of breast cancer, and a biopsy showing hyperplasia with atypia (de, cellular variations from normal that are not yet cancerous).(8) Simple hyperplasia and fibrocystic breast disease are not risk factors for breast cancer.

EVALUATION OF A BREAST TUMOR

When a woman's mammogram shows abnormal results or the woman has a palpable lump in her breast, several factors must be considered in deciding whether to proceed with a biopsy. The initial step in the evaluation is a thorough patient history, which includes

* information about the breast lump's presentation, its duration, and its growth pattern;

* the presence or absence of pain;

* fluctuation of the lump with menstrual cycles;

* the patient's menopausal status;

* the patient's use of estrogen replacement therapy or oral contraceptives; and

* the presence of significant risk factors.

On physical examination of a breast lump, the physician assesses such features as fixation of the lump to overlying skin or to muscle, the presence of enlarged axillary or supraclavicular lymph nodes, and hepatomegaly. The physician may choose to evaluate a breast mass by using bilateral mammograms to assess the remainder of the involved breast and the opposite breast or by using ultrasonography to establish whether the mass is cystic or solid. Although a patient's mammogram may not suggest a cancerous tumor, the physician will still recommend obtaining a tissue sample of a clinically suspicious mass. Tissue sampling of a breast mass can be accomplished by one or a combination of several methods: fine-needle aspiration or core-cutting needle biopsy, incisional biopsy, or excisional biopsy.

Fine-needle aspiration or core-cuffing needle biopsy. Fine-needle aspiration (FNA) can be performed in a physician's office. During this procedure, the physician passes a 22-gauge needle attached to a syringe through the breast mass several times while applying suction, which causes cells in the mass to break loose. The physician sends the aspirate to a laboratory for cytologic examination. This technique can provide a result in only a few hours. Although FNA can confirm the presence of cancer, it cannot definitively rule it out. When a mass is suspicious, it must be removed regardless of the FNA results. A core-cutting needle biopsy also provides a rapid, relatively painless method of evaluating a breast mass. The physician obtains and sends a core of breast tissue for cytologic examination.

Incisional and excisional biopsies. A breast biopsy can be obtained on a palpable breast mass or with radiographic assistance, on a breast mass identified only by mammography. Most breast biopsies are performed with local anesthesia, and IV sedation is used if necessary.

For a breast mass seen on a mammogram, the physician performs a needle localization procedure. Under mammographic guidance, the surgeon passes a hooked wire into the breast and into the tumor (Figure 3). The hook holds the wire in the breast tissue. The patient is brought to the OR with the wire in place (Figure 4), and the surgeon makes a separate incision and removes the core of tissue surrounding the wire (de, incisional biopsy). The surgeon sends the tissue specimen to the radiology department, where a repeat mammogram of the specimen confirms that the specimen contains abnormal tissue. The tissue specimen is then sent to the laboratory for cytologic examination.

[Figures 3 to 4 ILLUSTRATION OMITTED]

For a biopsy of a palpable breast mass, the patient's entire breast is prepared and draped into the field to permit appropriate orientation of the incision according to landmarks. The surgeon makes a circumferential incision in the upper half of the patient's breast and a radial incision in the lower half of the breast and then excises the mass with a surrounding margin of normal breast tissue (de, excisional biopsy).

Proper handling of the tissue specimen is essential. The specimen should not be placed in formalin. The circulating nurse should send the specimen promptly to the cytology department to allow the pathologist to evaluate the margins and obtain tissue samples for hormone receptors (de, estrogen receptors [ERs], progesterone receptors [PRs]. In the past, most large, palpable tumors could be diagnosed immediately by frozen section; today, the smaller tumors seen with mammography often cannot be subjected to frozen section analysis. This means patients often have to wait an additional one to three days for biopsy results.

When cancer is detected, the breast tissue specimen's ERs and PRs need to be assessed. Until recently, this required a fresh tissue sample frozen by the pathologist. Now, however, immunohistochemical techniques using monoclonal antibodies can assess ERs and Pits, even on fixed tissue. Other tumor markers, such as aneuploidy (de, having an uneven multiple of the basic number of chromosomes), oncogene expression, and protease cathepsin D remain of unproven benefit. These tests can be performed on fixed tissue as well, but until their use is proven, they should not be performed routinely.

Histopathologic analysis of the breast biopsy specimen may reveal one of the following types of tumors:

* carcinoma in situ,

* infiltrating ductal carcinoma,

* medullary carcinoma,

* tubular carcinoma,

* mucinous carcinoma,

* infiltrating lobular carcinoma,

* Paget's disease, or

* inflammatory breast carcinoma.(9) The type of breast carcinoma and the extent of disease present determine the patient's treatment options.

POSITIVE BREAST BIOPSY

When the results of a patient's breast biopsy indicate cancer, a plan for further evaluation and treatment is established. The basic issues in breast cancer treatment are

* local treatment of the primary tumor,

* staging of the tumor,

* systemic treatment of metastatic disease, and

* breast reconstruction.

A metastatic workup of the patient should be performed, including liver function tests, chest radiographs, and a bone scan. The patient will be seen by a multispecialty team consisting of medical oncologists, radiation therapists, plastic and reconstructive surgeons, perioperative nurses, and social workers.

Staging of the tumor. The term staging refers to the grouping of patients according to the extent of their disease. Staging helps physicians and patients select appropriate treatment, estimate prognoses, and compare the results of available treatment programs. There are two staging systems used by physicians who treat women with breast cancer. One is based on tumor, nodes, and metastases (de, TNM classification system), and the other system groups patients according to the extent of the disease in the primary tumor sites, nodal areas, and distant metastases (de, Columbia Clinical Classification). Staging requires surgery to examine the extent of the patient's disease.(10)

Local treatment of the primary tumor is accomplished by surgery. The two major surgical options for treatment of breast cancer are modified radical mastectomy (MRM) and breast-conserving treatment (de, lumpectomy and axillary dissection). With both of these procedures, the breast tumor is removed, as well as lymph nodes in the axilla.

Modified radical mastectomy. In a MRM, the surgeon makes a single, long transverse incision on the patient's chest. He or she removes the patient's nipple and areola complex, axillary lymph nodes, and breast tissue. The pectoral muscles are left intact. The surgeon places drains in the axilla and under the muscle flaps on the chest wall.

Lumpectomy and axillary dissection. Lumpectomy and axillary dissection are performed together regardless of the patient's lymph node status. In a lumpectomy and axillary dissection, the surgeon excises the previous biopsy site and removes the biopsy cavity and any residual tumor. He or she may use clips to mark the new cavity and guide the radiation oncologist. The surgeon makes a separate incision below the hair-bearing portion of the axilla to remove the lymph nodes and identifies the pectoralis major and minor muscles and the medial pectoral nerve. The surgeon identifies and preserves the long thoracic nerve, which supplies the serratus antenor. and the thoracodorsal nerve, which supplies the latissimus dorsi muscle. He or she removes all nodal tissue between these two nerves and below the axillary vein (Figure 5). The surgeon places a drain in the axilla for lymphatic drainage.

[Figure 5 ILLUSTRATION OMITTED]

Modified radical mastectomy removes all breast tissue on the affected side, and no further local treatment is needed. Lumpectomy and axillary dissection. however, have a local tumor recurrence rate of 40% when no further treatment is given; therefore, lumpectomy must be followed by adjuvant radiation therapy to treat the remaining breast tissue. This decreases the local tumor recurrence rate to 10%.(11) Lumpectomy and axillary dissection with radiation therapy is associated with the same survival rate as mastectomy, but the local recurrence rate is higher with lumpectomy and axillary dissection, even when postoperative radiation therapy is given.(12)

Removal of the axillary lymph nodes during axillary dissection or MRM allows the tumor to be staged and aids in determining which adjuvant therapy should be used. Adjuvant therapy is chemotherapy or hormonal therapy (de, tamoxifen) that is given after surgery to decrease the potential for tumor recurrence. The decision on which therapy to use is based on factors such as tumor size, the presence or absence of axillary lymph nodes, tumor histology, the patient's menopausal status, and whether the tumor is hormone-receptor positive or negative.(13)

The patient's options regarding adjuvant therapy include not using adjuvant therapy, using either chemotherapy or hormonal therapy, or using chemotherapy and hormonal therapy.(14) Research on this subject is ongoing, but at this time, the National Institutes of Health recommend that women with tumors less than I cm and negative axillary lymph nodes not receive chemotherapy or hormonal therapy.(15) Patients with tumors larger than 1 ,cm usually are treated with hormonal therapy or chemotherapy or both.(16) Patients with positive axillary lymph nodes receive chemotherapy:(17) Many variables must be taken into account, including the patient's menopausal status and comorbid disease; therefore, treatment is individualized for each patient.

BREAST RECONSTRUCTION

Breast reconstruction after a mastectomy is an emotionally charged issue. The patient's options regarding breast reconstruction are to have no reconstruction, immediate reconstruction at the time of the mastectomy, or delayed reconstruction. Each option must be reviewed carefully with the patient and the plastic surgeon, taking into account the patient's wishes, body habitue, and overall health.

There are three types of basic reconstruction. One is the transverse rectus abdominus myocutaneous (TRAM) flap, in which the rectus abdominus muscle is dissected free along with an overlying pad of fat and skin. The blood supply to the muscle is left intact. The flap is brought underneath the patient's abdominal tissue and used to reconstruct the breast (Figure 6). This procedure can be performed immediately or at a later date, although the best results are obtained with immediate reconstruction. The nipple and areola can be reconstructed in a second procedure. The advantages of the TRAM flap procedure are that it provides excellent bulk, and the patient rarely requires an implant. The disadvantages are the lengthy procedure and the potential for hernias and problems with vascularity and wound healing.

[Figure 6 ILLUSTRATION OMITTED]

Another type of reconstruction procedure is the use of a tissue expander, in which an empty tissue expander is placed underneath the patient's pectoralis major muscle after a mastectomy. During the next several months, the tissue expander is inflated with normal saline infusions until the patient's skin has expanded to the desired size. The expander is then replaced with a permanent breast implant. The advantages of this approach include gradual stretching of the skin and improved projection of the reconstructed breast. Disadvantages include many postoperative visits, time requirements, and the need for second-stage surgery to insert the implant.

The third method of breast reconstruction is the myocutaneous latissimus dorsi flap, which uses the latissimus dorsi muscle and overlying skin from the patient's back to reconstruct the breast. This surgical procedure may require skin grafts to the donor site on the patient's back and implant placement under the patient's muscle at the site of the mastectomy to supply adequate bulk. This procedure does, however, produce an appealing contour axillary fold.

PERIOPERATIVE NURSING CARE

The diagnosis of breast cancer carries lifelong implications. Optimal treatment requires a team approach, including surgeons, internists, radiation therapists, physical therapists, perioperative nurses, and social workers. Immediately after receiving the diagnosis of breast cancer, the patient is in need of emotional support and information. After the decision has been made regarding which surgical approach will be taken, the patient needs preoperative education about what to expect.

The patient will spend one or two nights in the hospital after surgery. During this time, nurses will teach the patient how to care for the surgical drains and incisions as well as how to perform shoulder exercises after the drains are removed. Nurses provide encouragement and support throughout the patient's treatment. All these issues can be addressed by the perioperative nurse in a way that can minimize some of the shock and pain that the patient experiences with the diagnosis and treatment of breast cancer.

Possible nursing diagnoses related to patients undergoing surgery for breast cancer include

* anxiety related to fear of cancer or surgical intervention,

* knowledge deficit related to surgery, body image disturbance related to loss of body part,

* anticipatory grieving related to loss of body part,

* potential for injury related to the surgical environment and the administration of medication, and

* alteration in skin integrity related to the surgical procedure.(18)

After these nursing diagnoses have been formulated, the perioperative nurse develops a plan of care that guides and evaluates perioperative nursing actions.(19) The nurse intervenes to help the patient decrease her anxiety, verbalize her feelings about the cancer and the surgery, and understand perioperative routines.

The nurse also positions and pads the patient carefully to avoid brachial plexus injury and applies the electrosurgical unit (ESU) dispersive pad to a dry, well-muscled site on the patient to prevent injury related to the use of the ESU. The nurse handles the biopsy specimen appropriately to facilitate acquisition of essential histopathologic information. He or she communicates essential information to the postanesthesia care unit (PACU) nurse to ensure continuity of care. The nurse also may refer the patient to a wellness and exercise program to help her regain strength and mobility in the affected muscles.

CASE STUDY

Ms B was a 36-year-old female who was diagnosed as having a breast tumor discovered during a routine mammogram (Figure 7). Ms B became anxious and upset, and her anxiety increased during the three-day delay before she could see her surgeon. As an OR nurse, Ms B knew that not all tumors are cancerous, and she was surprised that she felt so anxious and scared.

[Figure 7 ILLUSTRATION OMITTED]

Ms B and her surgeon discussed treatment options. They decided to excise the tumor for biopsy because Ms B did not want to wait six months to see whether the tumor changed. Ms B was worried that she would be like her friend, who had undergone a radical mastectomy and chemotherapy and subsequently lost her hair.

Ms B decided to be an outpatient in the hospital where she worked. She chose her surgeon, anesthesia care provider, and perioperative nurse, and being able to make these choices relieved some of her anxiety. Ms B had instructed her surgeon to not prepare a frozen section biopsy because she did not want to risk a false-negative result. The surgeon completed the excisional biopsy and segmental resection without incident. Ms B awoke in the PACU and cried because she was glad the procedure was finished and because she was in pain.

When Ms B arrived home, she had three reminders of her surgery. The first reminder was the scar on her breast. The second was a bruise on her right anterior thigh, which suggested that someone's elbow had pressed on her leg during the surgical procedure. The third reminder was a reddened area on her left thigh in the exact shape of an ESU dispersive pad.

One week after the biopsy, Ms B received the pathology report, which revealed that the tumor was not malignant. Ms B was relieved and overjoyed. The bruise and reddened area eventually disappeared. Ms B was left with an asymmetrical breast, but she did not consider this a noticeable problem.

CONCLUSION

Health care providers have better knowledge about the screening, diagnosis, and treatment of breast cancer than they did 20 years ago. This has helped decrease the incidence of new breast cancer cases and has helped increase patients' survival rates. Technology, early detection, and understanding must continue to evolve before breast cancer is no longer the most common type of cancer in women.

NOTES

(1.) American Cancer Society, Inc, Cancer Facts and Figures-1993 (Atlanta: American Cancer Society, Inc, 1993).

(2.) Ibid.

(3.) C C Boring, T S Squires, T Tong. Cancer statistics, 1992," CAA Cancer Journal for Clinicians 42 (January/February 1992) 19-38.

(4.) Ibid.

(5.) L H Baker, "Breast cancer detection demonstration project: Five-year summary report," CA-A Cancer Journal for Clinicians 32 (July/August 1982) 194-225.

(6.) R S Hayward et al, "Preventive care guidelines: 1991," Annals of Internal Medicine 114 (May 1, 1991) 758-783; American Medical Association. Mammography screening in asymptomatic women 40 years and older (Resolution 93. 1-87) (Chicago: American Medical Association, 1988); American Cancer Society, Summary of current guidelines for the cancer-related checkup: Recommendations (New York: American Cancer Society, 1988).

(7.) M H Gail et al, "Projecting individualized probabilities of developing breast cancer for white females who are being examined annually," Journal of the National Cancer Institute 81 (Dec 20, 1989) 1879-1886.

(8.) J B Kampert, A S Whittemore, R S Paffenbarger, Jr, "Combined effect of childbearing, menstrual events, and body size on age-specific breast cancer risk," American Journal of Epidemiology 128 (November 1988) 962-979; D Trichopoulos, B MacMahon, P Cole, "Menopause and breast cancer risk," Journal of the National Cancer Institute 48 (March 1972) 605-613; E White, "Projected changes in breast cancer incidence due to the trend toward delayed childbearing," American Journal of Public Health 77 (April 1987) 495-497; W D Dupont, D L Page, "Risk factors for breast cancer in women with proliferative breast disease," The New England Journal of Medicine 312 (Jan 17, 1985) 146-151.

(9.) J R Harris, M Morrow, G Bonadonna, "Cancer of the breast," in Cancer: Principles & Practice of Oncology, fourth ed, V T DeVita, Jr, S Hellman, S A Rosenberg, eds (Philadelphia: J B Lippincott Co, 1993) 1272-1273.

(10.) Ibid, 1278-1279.

(11.) B Fisher et al, "Eight-year results of a randomized clinical trial comparing total mastectomy and lumpectomy with or without irradiation in the treatment of breast cancer," The New England Journal of Medicine 320 (March 30, 1989) 822-828; J A Jacobson et al, "Ten-year results of a comparison of conservation with mastectomy in the treatment of stage I and II breast cancer," The New England Journal of Medicine 332 (April 6. 1995) 907-911.

(12.) Ibid.

(13.) E R Fisher et al, "Prognostic factors in NSABP studies of women with node-negative breast cancer," Journal of the National Cancer Institute Monographs 11 (1992) 151-158.

(14.) Consensus Development Panel, Treatment of Early-Stage Breast Cancer (Bethesda, Md: NIH Consensus Statement Online 8, June 18-21,1990) 1-19.

(15.) Ibid.

(16.) E G Mansour et al, "Efficacy of adjuvant chemotherapy in highrisk node-negative breast cancer: An intergroup study," The New England Journal of Medicine 320 (Fete 23, 1989) 485-490; B Fisher et al, "A randomized clinical trial evaluating tamoxifen in the treatment of patients with node-negative breast cancer who have estrogen-receptor-positive tumors," The New England Journal of Medicine 320 (Fete 23, 1989) 479-484; B Fisher et al, "A randomized clinical trial evaluating sequential methotrexate and fluorouracil in the treatment of patients with node-negative breast cancer who have estrogenreceptor-negative tumors," The New England Journal of Medicine 320 (Fete 23, 1989) 473-478.

(17.) Anonymous, "Consensus conference: Adjuvant chemotherapy for breast cancer," Journal of the American Medical Association 254 (Dec 27, 1985) 3461-3463.

(18.) L J Carpenito, Nursing Diagnosis: Application to Clinical Practice, fifth ed (Philadelphia: J B Lippincott Co, 1993).

(19.) A L M Wynne, "Concepts basic to perioperative nursing," in Alexander's Care of the Patient in Surgery, ninth ed, M H Meeker, J C Rothrock, eds (St Louis: Mosby-Year Book, Inc, 1991) 2-11.

Jo Buyske, MD, is a general surgeon at the Lahey Hitchcock Medical Center, Burlington, Mass.

Gasan Mackarem, MD, is a breast cancer research fellow at the Lahey Hitchcock Medical Center, Burlington, Mass.

Brenda C. Ulmer, RN, BS, CNOR, is a senior clinical educator, Valleylab, Inc, Boulder, Colo, and a PRN staff nurse in the OR, Eastside Medical Center, Snellville, Ga.

Kevin S. Hughes, MD, is codirector of the Breast Cancer Treatment Center at Lahey Hitchcock Medical Center, Burlington, Mass.

RELATED ARTICLE: Co-directors of AORN Perioperative Nursing Research Appointed

Suzanne C. Beyea, RN, PhD, and Leslie H. Nicoll, RN, PhD, have been appointed AORN co-directors/perioperative nursing research, effective June 1, 1996.

Drs Beyea and Nicoll are responsible for promoting and coordinating perioperative nursing research within AORN, among the AORN membership, and in cooperation with other professional and technical health care disciplines, corporations, consumer organizations, and payer groups. They will provide theoretical and methodological consultations in research for the AORN Board of Directors, national committees, specialty assemblies, and Head quarters staff members. Drs Beyea and Nicoll also will function as principal investigators for major AORN research projects.

Dr Beyea earned her bachelor of science degree in nursing from the University of Vermont, Burlington, and her master of science and doctor of philosophy degrees from Boston College. She has extensive medical/surgical nursing and teaching experience, in-depth knowledge of critical pathways, and a background as a research consultant.

Dr Nicoll earned her bachelor of science degree in nursing from Russell Sage College, Troy, NY; her master of science degree in nursing from the University of Illinois at the Medical Center, Chicago; her master's degree in business administration from the University of New Hampshire, Durham; and her doctor of philosophy degree in nursing from the Frances Payne Bolton School of Nursing, Case Western Reserve University, Cleveland. Dr Nicoll has in-depth knowledge of computer systems and is editor of Computers in Nursing. She also has extensive experience in grant writing.

Dr Beyea is located in Gilford, NH, and Dr Nicoll in Portland, Maine. They are sharing this position and working through a telecommuting arrangement. AORN members may leave messages for Drs Beyea and Nicoll by calling (800) 755-2676 x 277. Members may call Dr Beyea directly at (603) 528-1841 and Dr Nicoll at (207) 780-4568.

COPYRIGHT 1996 Association of Operating Room Nurses, Inc.
COPYRIGHT 2004 Gale Group

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