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D R Farley

Publications and source records attributed to D R Farley.

At least 19 recordsLinked to original sources
PubMed2001

Paget's disease of the breast: a clinical perspective.

Paget's disease of the breast is a rare disorder of the nipple-areola complex often associated with an underlying in situ or invasive carcinoma. Eczematoid changes of the nipple-areola complex and persistent soreness or itching should raise suspicion of this disease. The histogenesis of Paget's disease of the breast continues to be debated and is important when considering treatment options. The epidermotropic theory suggests that Paget's cells are ductal carcinoma cells that have migrated from an underlying carcinoma of the breast parenchyma to the epidermis of the nipple. The in situ transformation theory has been proposed to explain the development of this disorder in patients in whom an underlying mammary carcinoma is not found or when there is an underlying carcinoma anatomically remote from the nipple-areola complex. Paget's cells are believed to arise as malignant cells in the epidermis of the nipple independent from any other pathologic process within the breast parenchyma. The current standard treatment of biopsy-proven Paget's disease involves mastectomy, although some studies have proposed the use of breast conservation therapy for patients in whom an underlying breast cancer cannot be located. We propose a treatment algorithm for patients presenting with Paget's disease of the breast.

Algorithms↗
PubMed2001

Primary aldosteronism: factors associated with normalization of blood pressure after surgery.

BACKGROUND: Hypertension often persists after adrenalectomy for primary aldosteronism. OBJECTIVE: To determine factors associated with resolution of hypertension after adrenalectomy for primary aldosteronism. DESIGN: Retrospective cohort study. SETTING: Tertiary care referral center in Rochester, Minnesota. PATIENTS: All patients who underwent adrenalectomy for primary aldosteronism between 1 January 1993 and 31 December 1999. MEASUREMENTS: Preoperative plasma renin activity, plasma and urinary aldosterone concentrations, and adrenal imaging. Follow-up blood pressure, measured at a clinic visit or at home, was reviewed. RESULTS: 97 adrenalectomies were performed, and follow-up was available in 93 patients. Hypertension was resolved at follow-up (blood pressure < 140/90 mm Hg) without use of antihypertensive agents in 31 of 93 patients (33%). According to a stepwise multivariable logistic regression analysis adjusted for duration of follow-up, resolution of hypertension was independently associated with family history of hypertension in no more than 1 first-degree relative (odds ratio [OR], 10.9; P < 0.001) and preoperative use of two or fewer antihypertensive agents (OR, 4.7; P = 0.005). Additional factors associated with resolution of hypertension based on univariate analysis included younger age, shorter duration of hypertension, higher preoperative ratio of plasma aldosterone concentration to plasma renin activity, and higher urine aldosterone level (P < 0.05). CONCLUSIONS: Resolution of hypertension after adrenalectomy for primary aldosteronism is independently associated with a lack of family history of hypertension and preoperative use of two or fewer antihypertensive agents.

Adenoma↗
PubMed2001

Adrenocortical carcinoma: surgical progress or status quo?

HYPOTHESIS: Outcome of patients with adrenocortical carcinoma (ACC) has improved with the advent of more widely available and higher quality imaging. Operative management strategies and use of adjuvant therapy have not changed. DESIGN: Retrospective review of patient histories, imaging studies, operative data, adjuvant therapy, and outcomes at a single institution. Follow-up was complete for a mean of 53 months. Data was compared with prior institutional experience. SETTING: Tertiary care referral center. PATIENTS: All patients undergoing operative management for ACC during the period from 1980 to 1996. MAIN OUTCOME MEASURES: Determinants of recurrence, survival, and the effect of adjuvant therapy on overall outcome. RESULTS: Fifty-eight patients (30 men, 28 women) with a mean age of 53 years underwent primary operative management for ACC. Functional tumors were identified in 27 patients (47%). Mean tumor size was 12.5 cm. Stage according to the TNM staging system (AJCC Cancer Staging Manual) at presentation was I (n = 0), II (n = 30), III (n = 7), and IV (n = 21). Surgical management included curative resection in 41 (71%), noncurative resection in 14 (24%), and open biopsy in 3 (5%). Perioperative mortality was 5%. Recurrence occurred in 30 patients (73%) with a median time to recurrence of 17 months. Five-year survival by the Kaplan-Meier method was 37%. Prognostic factors (P<.05) included functional status, stage, and chemotherapy in stage III/IV patients. When compared with our prior institutional experience (1960-1980), current patients were more likely to present with stages I to II (52% vs. 34%), have curative resections (71% vs. 50%), and have improved 5-year survival (37% vs. 16%). CONCLUSIONS: (1) Surgical resection remains the principal treatment for stage I to III disease. (2) Adjuvant therapy may improve survival in patients with stage III or IV disease. (3) Current patients were more likely to present at an earlier stage, undergo curative resections, and have improved 5-year survival than institutional historical comparisons.

Adolescent↗
PubMed2001

Surgical management of duodenal leiomyomas.

Duodenal leiomyomas are rare neoplasms and when present are usually asymptomatic. There are, however, isolated case reports of such leiomyomas giving rise to complications including gastrointestinal hemorrhage and obstruction. The purpose of this study was to review the surgical experience with duodenal leiomyomas at a large tertiary referral center. A retrospective review was performed of all patients with histologically proven duodenal leiomyomas encountered from 1975 to 1995. Twelve patients with surgically treated duodenal leiomyomas were identified. There were eight men and four women with a mean age of 57 years (range 20-73 years) at diagnosis. Anemia was the presenting sign in 10 patients (83%). The mean hemoglobin level at presentation was 10.8 +/- 2.67 g/dl. Other presenting signs included melena (n = 5) and epigastric tenderness (n = 4). No patient had obstructive symptoms. Upper endoscopy visualized the lesion in 9 of 10 patients, but endoscopic biopsy provided an accurate histologic diagnosis in only two of six patients biopsied. All resected specimens underwent frozen section and permanent histopathologic analysis. Local excision was performed in eight patients, as benignancy was anticipated at surgical exploration and frozen section findings. Two patients underwent segmental duodenal resection with primary end-to-end anastomosis, and two patients underwent a Whipple procedure. There was no perioperative mortality. Morbidity was minimal. There was no tumor recurrence during a median follow-up of 8.4 years. Gastrointestinal blood loss is the most common complication of duodenal leiomyomas requiring surgical intervention. Small symptomatic leiomyomas with benign features may be safely treated with local excision.

Adult↗
PubMed2001

Cystic neoplasms of the pancreas: benign to malignant epithelial neoplasms.

Clinical presentation and state-of-the-art imaging permit the differentiation of most cystic pancreatic neoplasms not only from other cystic pancreatic disorders but also from one another. The differentiation of serous cystic lesions from the mucinous neoplasms (cystadenoma or carcinoma and IPMT) is crucial because of the radically different biological characteristics of these two neoplasms. Although mucinous cystic neoplasms should be resected because of their premalignant or overtly malignant tendency, most patients with serous neoplasms require no operative intervention unless they are symptomatic. IPMT is best treated by a total pancreatectomy, although lesser subtotal resections should be strongly considered depending on patient age, medical comorbidity, and psychosocial situations.

Adenocarcinoma, Mucinous↗
PubMed2001

Paget's disease of the breast.

Paget's disease of the breast is a rare disorder of the nipple-areola complex that is often associated with an underlying in situ or invasive carcinoma. A change in sensation of the nipple-areola, such as itching and burning, is a common presenting symptom. Objectively, eczematoid changes of the nipple-areola complex are common. The later stages of Paget's disease of the breast are characterized by ulceration and destruction of the nipple-areola complex. Eczematoid changes of the nipple-areola complex and persisting soreness or itching, without obvious reason, is a suspicious symptom for Paget's disease of the breast and calls for thorough evaluation, including mammography. Exfoliative cytology with demonstration of Paget's cells may be useful, but a negative finding does not exclude Paget's disease of the breast. Surgical biopsy is the diagnostic standard and therefore the diagnosis should always be confirmed by open (surgical) biopsy. The histogenesis of Paget's disease of the breast continues to be debated. The epidermotropic theory holds that Paget's cells are ductal carcinoma cells that have migrated from the underlying breast parenchyma to the nipple epidermis. According to the in situ transformation theory, the Paget's cells arise as malignant cells in the nipple epidermis independent from any other pathologic process within the breast parenchyma. This theory has been proposed to explain those cases in which there is no underlying mammary carcinoma or when there is a carcinoma remote from the nipple-areola complex. Each of these theories is plausible; however, treatment approaches differ markedly depending on the theory of histogenesis. Mastectomy has been considered the standard of care in the management of patients with Paget's disease of the breast. Nowadays, however, some patients with Paget's disease of the breast are candidates for breast-conserving therapy. Patients must be selected carefully on an individual basis. Until there is a better understanding of the relationship of Paget's disease of the breast to the underlying cancer the surgeon should understand the natural history and behaviour of this lesion and be aware of both the risks of under- and over-treating patients with Paget's disease of the breast.

Breast Neoplasms↗
PubMed2001

Calciphylaxis causing necrotizing mastitis: a case report.

Calciphylaxis is a rare condition most commonly related to ESRD and hyperparathyroidism. We report a case of necrotizing mastitis caused by calciphylaxis following routine breast biopsy for microcalcifications. Early recognition of the potential for this condition should help in clinical management of patients with calciphylaxis.

Journal Article↗
PubMed2001

Reexploration for symptomatic hematomas after cervical exploration.

BACKGROUND: Hematomas requiring reoperation affect 1% of patients undergoing cervical exploration. This complication has implications for the trend toward outpatient procedures. METHODS: Retrospective review of 13,817 patients undergoing thyroidectomy and parathyroidectomy at this institution (1976 to 2000) identified 42 patients with hematomas requiring reoperation. Case controls (n = 42) were matched for age, gender, and type and year of operation. Perioperative risk factors and outcome were compared. RESULTS: Study and control groups were composed of 18 men and 24 women (mean age, 62 years) undergoing thyroidectomy (n = 21) or parathyroidectomy (n = 21). Comparison of perioperative risk factors yielded no significant difference between groups. Symptoms included respiratory distress in 21 patients, pain/pressure in 11, dysphagia in 8, and drainage in 6. Mean time to symptom onset was 17 hours (range, 10 minutes to 5 days). Eighteen hematomas presented within 6 hours postoperatively, 16 between 7 and 24 hours, and eight beyond 24 hours. The bleeding source was arterial in 11 patients, venous in 8, thyroid/soft tissue in 13, and indeterminate in 10. Mean hospital stay was longer in the study group (7.2 vs 3.6 days, P = .004). The number of patients with complications was higher in the study group (17 vs 7, P = .03). No single complication reached significance: hypocalcemia (8 vs 3, P = .12), wound infection (3 vs 0, P = .87), and others (10 vs 5, P = .21). CONCLUSIONS: (1) No factor foreshadowed this complication. (2) The definition of a high-risk population remains obscure. (3) Sixty percent presented beyond 6 hours postoperatively. (4) Reexploration increased morbidity and lengthened hospital stay.

Adult↗
PubMed2001

Laparoscopic adrenalectomy for adrenocorticotropin-dependent Cushing's syndrome.

Bilateral adrenalectomy is indicated for the treatment of ACTH-dependent Cushing's syndrome when the tumorous source of ACTH hypersecretion cannot be identified or removed. Potential advantages of laparoscopic over open adrenalectomy include shorter hospitalization, decreased requirement for postoperative analgesia, and decreased postoperative morbidity due to incisional complications. Bilateral laparoscopic adrenalectomy performed for the treatment of ACTH-dependent Cushing's syndrome was attempted in 19 patients at our institution between 1995 and 1998. Conversion to an open procedure was required in three patients. All patients who underwent bilateral laparoscopic adrenalectomy were subsequently followed to assess the outcome of this intervention. Twelve patients with pituitary-dependent Cushing's syndrome and four with ectopic ACTH syndrome underwent successful bilateral laparoscopic adrenalectomy. All patients experienced resolution of the signs and symptoms (e.g. proximal myopathy, hirsutism, and emotional lability) of Cushing's syndrome as well as weight loss, improved glucose tolerance, and improved control of blood pressure. No residual cortisol secretion was detected in the patients. Bilateral laparoscopic adrenalectomy is a safe and effective treatment for Cushing's syndrome when the ACTH-secreting neoplasm cannot be removed.

ACTH Syndrome, Ectopic↗
PubMed2001

Risk of parathyromatosis after fine-needle aspiration.

Reoperative surgery for hyperparathyroidism (HPT) is fraught with hazard. When preoperative imaging studies are inconclusive or patient comorbidities are extensive fine needle-aspiration (FNA) is helpful to confirm the presence of suspected parathyroid tissue in the neck. Some surgeons refrain from using FNA because of the concern of tissue implantation (parathyromatosis). A retrospective review (1984-1996) of all patients diagnosed with HPT undergoing FNA of suspected parathyroid tissue was performed to document whether a correlation exists between FNA of suspected parathyroid tissue and subsequent development of parathyromatosis. Parathyromatosis was considered to have occurred when proven by histology or suspected on the basis of clinical studies. Of 81 patients with HPT undergoing ultrasound-guided FNA to assess abnormalities in the neck 41 patients with confirmed parathyroid tissue were identified. The indications for FNA in these 41 patients were: prior failed cervical exploration (n = 33), prior neck surgery and/or radiation (n = 2), inconclusive noninvasive imaging studies (n = 15), and severe comorbidities (n = 8). Mean follow-up was 5.8 years. No case of FNA-induced parathyromatosis was identified. FNA is useful to confirm the presence of parathyroid tissue in very select patients with hyperparathyroidism. FNA often eliminates the need for other imaging studies, may prevent a needless or likely fruitless re-exploration, and does not cause parathyromatosis.

Adult↗
PubMed2000

Accuracy of sentinel lymph node biopsy in patients with large primary breast tumors.

BACKGROUND: Patients with large breast tumors are increasingly undergoing neoadjuvant treatment to downstage local disease; however, accurate staging of the axilla before the initiation of chemotherapy remains problematic. In the current study, the authors report on the accuracy of sentinel lymph node (SLN) biopsy in such patients to determine the feasibility of applying this technique before induction chemotherapy. METHODS: One hundred three patients with 104 tumors classified as American Joint Committee on Cancer (AJCC) T2 (tumor >/= 2 cm but </= 5 cm) or larger were recruited at the University of Pennsylvania and the Mayo Clinic. In the majority of cases, combined blue dye and radiotracer was used for SLN identification. After SLN identification, a completion axillary lymph node dissection was performed in 87 cases. The SLN was evaluated with hematoxylin and eosin and immunohistochemistry. RESULTS: The SLN was identified in 99% of cases. The overall rate of lymph node metastasis was 59% (95% exact confidence interval [95% CI], 49-68%) (61 of 104 cases). The SLN false-negative rate was 2% (95% exact CI, < 1-11.5%) (2 patients). In 56 tumors >/= 3 cm, 1 false-negative result (2% [95% exact CI, < 1-15%]) was identified, and the rate of lymph node metastasis was 62.5% (95% exact CI, 48. 5-75%) (35 of 56 tumors). Within 30 SLN positive patients with tumors >/= 3 cm and complete axillary lymph node dissection, 3 of 8 patients (37.5% [95% exact CI, 8.5-75.5%]) with micrometastasis (</= 2 mm) to the SLN had positive non-SLN compared with 21 of 22 patients (95.5% [95% exact CI, 77-100%]) with macrometastasis (> 2 mm) to the SLN (P = 0.002). CONCLUSIONS: SLN biopsy for patients with large breast tumors is technically feasible and highly accurate. SLN biopsy should be considered for the staging of clinically negative axilla in patients scheduled to receive neoadjuvant chemotherapy.

Adult↗
PubMed2000

What prognostic factors are important in duodenal adenocarcinoma?

HYPOTHESIS: Survival of patients with adenocarcinoma of the duodenum depends on the ability to perform a complete resection and the tumor stage DESIGN: Retrospective case series. SETTING: Tertiary care referral center. PATIENTS: A cohort of 101 consecutive patients (mean age, 62 years), undergoing surgery for duodenal adenocarcinoma from January 1, 1976, through December 31, 1996. Patients with ampullary carcinoma were specifically excluded. Mean duration of follow-up was 4 years. INTERVENTIONS: Surgery was curative in 68 patients (67%) and palliative in 33 patients (33%). Of the curative group, 50 patients (74%) underwent radical surgery, ie, 30 (60%), pancreaticoduodenectomy; 15 (30%), pylorus-preserving pancreaticoduodenectomy; and 5 (10%), total pancreatectomy. A more limited resection procedure was used in 18 patients (26%) involving a segmental duodenal resection in 15 (83%) and a transduodenal excision in 3 (17%). patient survival, and correlation with patient and tumor variables using univariate and multivariate analysis. RESULTS: Actuarial 5-year survival for the curative group was 54%. Only 1 patient in the unresected group survived beyond 3 years. Nodal metastasis (P = .002), advanced tumor stage (P<.001), positive resection margin (P = .02), and weight loss (P<.001) had a significant negative impact on survival in multivariate analysis. Tumor grade, size, and location within the duodenum had no impact on survival. Patient age and tumor depth of invasion influenced survival in univariate analysis, but lost their prognostic significance in multivariate analysis. CONCLUSIONS: Metastasis to lymph nodes, advanced tumor stage, and positive resection margins are associated with decreased survival in patients with duodenal adenocarcinoma. An aggressive surgical approach that achieves complete tumor resection with negative margins should be pursued. Pancreaticoduodenectomy is usually required for cancers of the first and second portion of the duodenum. Segmental resection may be appropriate for selected patients, especially for tumors of the distal duodenum.

Adenocarcinoma↗
PubMed2000

Are patients with multiple endocrine neoplasia type I prone to premature death?

Multiple endocrine neoplasia type I (MEN-I) is an autosomal dominant disorder characterized by endocrinopathies involving the anterior pituitary gland, parathyroid glands, and pancreas. The long-term prognosis for patients affected with this disorder is uncertain. To better characterize this prognosis, we performed a retrospective review of all patients with MEN-I treated at a single institution during the period 1951-1997. A group of 233 patients served as the study population. Their records were analyzed for confirmation of diagnosis, treatments received, long-term survival, and cause of death. Altogether, 108 eight male patients (46%) and 125 female patients (54%) were identified. At the conclusion of the study, 164 (70%) were alive and 69 (30%) were deceased, with a median follow-up for patients alive at last contact of 13.4 years (range < 1 month to 54.3 years). The cause of death was reliably obtained in 60 patients. Of these patients, 17 (28%) died of causes related to MEN-I, most commonly metastatic islet cell tumors (10 patients). The remaining patients died of causes unrelated to MEN-I, most commonly coronary artery disease and nonendocrine malignancies (14% each). The overall 20-year survival of MEN-I patients was 64% (95% CI was 56-72%), and that of an age- and gender-matched upper Midwest population was 81% (p < 0.001). Patients with MEN-I appear to be at increased risk of premature death. Earlier diagnosis and treatment of potentially malignant pancreatic islet cell neoplasms may result in a decrease of this premature mortality.

Adolescent↗
PubMed2000

Hemosuccus pancreaticus complicating chronic pancreatitis: an obscure cause of upper gastrointestinal bleeding.

BACKGROUND: Hemosuccus pancreaticus, a rare form of upper gastrointestinal bleeding, may complicate chronic pancreatitis and pose a significant diagnostic and therapeutic dilemma. AIM: To present our experience with this potentially life-threatening complication of chronic pancreatitis. METHODS: We reviewed our experience with management (both operative as well as angiographic embolization) of patients with hemosuccus pancreaticus complicating histologically documented chronic pancreatitis between 1976 and 1997. Diagnosis of hemosuccus pancreaticus was based on clinical presentation, preoperative endoscopic and radiographic imaging, operative findings, and pathologic evaluation. RESULTS: During the period, we managed eight patients with hemosuccus pancreaticus (1.5% of all patients with chronic pancreatitis treated surgically). Gastrointestinal bleeding presented as hematemesis in three and hematochezia in three, but all had recent melena and were anemic; three of these patients were hemodynamically unstable. Abdominal pain was present in six. When performed, angiography (n=6) was diagnostic of a pseudoaneurysm; computed tomography (n=7) showed a pseudoaneurysm in two and a pseudocyst in five. Endoscopy (n=8) revealed blood issuing from the ampullary papilla in two patients. Operative management (n=6) involved distal pancreatectomy, pancreatoduodenectomy, or total pancreatectomy in two patients each. Angiographic embolization was successful in one patient, but the other died from uncontrollable hemorrhage. CONCLUSIONS: Hemosuccus pancreaticus is rare, but should be considered in patients with chronic pancreatitis and gastrointestinal bleeding. In the absence of pancreatitis-related indications for surgery, angiographic embolization can be definitive treatment. If there are pancreatitis-related indications for operation, angiographic embolization may allow an elective operative procedure based on structural changes of the pancreas. If embolization fails, pancreatic resection is usually required, often on an emergent basis.

Adult↗
PubMed2000

The significance of sinistral portal hypertension complicating chronic pancreatitis.

BACKGROUND: Sinistral portal hypertension, a localized (left-sided) form of portal hypertension may complicate chronic pancreatitis as a result of splenic vein thrombosis/obstruction. AIM: To determine appropriate surgical strategy for patients with splenic vein thrombosis/obstruction secondary to chronic pancreatitis. METHODS: We reviewed our experience with operative management of 484 consecutive patients with histologically documented chronic pancreatitis treated between 1976 and 1997. The diagnosis of sinistral portal hypertension was based on clinical presentation, preoperative endoscopic and radiographic imaging, and operative findings. "Symptomatic," herein defined, denotes those patients with sinistral hypertension and either gastrointestinal bleeding or hypersplenism. "Asymptomatic" patients were those with sinistral hypertension alone. RESULTS: Sinistral portal hypertension was present in 34 of the 484 patients (7%). Gastric or gastroesophageal varices were confirmed in 12 patients (35%), of whom 6 had variceal bleeding and 4 had hypersplenism (25%). All symptomatic patients were treated by splenectomy alone or in conjunction with distal pancreatectomy. Splenectomy at the time of pancreatectomy for primary pancreatic symptoms was also performed in 15 patients with (asymptomatic) sinistral portal hypertension. None of the 23 patients who had splenectomy rebled in mean follow-up of 4.8 years. In contrast, 1 of the 11 patients with asymptomatic sinistral portal hypertension who underwent pancreatic surgery without splenectomy died of later variceal bleeding 3 years after lateral pancreatojejunostomy. CONCLUSIONS: Symptomatic sinistral portal hypertension is best treated by splenectomy. Concomitant splenectomy should be strongly considered in patients undergoing operative treatment of symptomatic chronic pancreatitis if sinistral portal hypertension and gastroesophageal varices are also present.

Adult↗
PubMed2000

Papillary thyroid cancer with pulmonary metastases in children: long-term prognosis.

BACKGROUND: Papillary thyroid cancer (PTC) in young patients may rarely be encountered with pulmonary metastases. Previous studies have suggested that, in the pediatric population, this may not portend a lethal outcome. Our present study, children with pulmonary metastases, was designed to clarify this issue. METHODS: Fourteen children and young adolescents (mean age, 13.5 years; range, 9.8-17 years) with PTC and pulmonary metastases were treated at our institution between 1937 and 1998. Surgical treatment consisted of total thyroidectomy (n = 10 patients), subtotal thyroidectomy (n = 3 patients), and a biopsy only procedure (n = 1 patient). All patients who underwent thyroidectomy also underwent a variety of cervical lymph node dissections, and all patients proved to have regional nodal disease. After the operation, 12 patients were treated with ablative doses of (131)I, 1 patient was treated with external beam irradiation, and all patients were placed on suppressive thyroid hormone therapy. The mean length of follow-up was 19.3 years (range, 1-45 years). RESULTS: Regional recurrent disease developed in 2 patients (15%). No patient experienced the development of worsening pulmonary disease or extra-pulmonary metastases. All patients with recurrent disease underwent selective nodal resections. No patient died of metastatic PTC. Seven patients (50%) remain completely free of disease and are probably cured; 7 patients (50%) are asymptomatic with residual pulmonary disease. CONCLUSIONS: A stepwise treatment approach allows long-term survival and frequent cure for young patients with PTC and concomitant pulmonary metastases.

Adolescent↗
PubMed2000

Is calciphylaxis best treated surgically or medically?

BACKGROUND: Calciphylaxis is a rare, painful, life-threatening problem of cutaneous necrosis and refractory healing in patients with uremia and secondary hyperparathyroidism. The pathogenesis involves abnormalities in calcium and phosphorus metabolism and acute deposition of calcium in tissues. METHOD: The clinical course of 16 patients who were diagnosed with calciphylaxis at our institution from 1994 through 1998 was reviewed. RESULTS: Fourteen female patients and 2 male patients had chronic renal disease, secondary hyperparathyroidism, and characteristic skin necrosis (mean age, 56 years; range, 39-70 years). All patients underwent intensive medical therapy, including ongoing hemodialysis (n = 16 patients), parathyroidectomy (n = 7 patients), and debridement of cutaneous lesions (n = 8 patients). Mean serum values in surgical and nonsurgical patients were significantly different for phosphorus, calcium-phosphorus product, and parathormone levels. Median survival was 9.4 months; 15 patients (93%) have died. The median survival time for parathyroidectomy versus nonparathyroidectomy was 14.8 and 6.3 months (P =.22), for skin debridement versus nondebridement was 14.1 and 6.1 months (P =.08), and for diabetic versus nondiabetic patients was 6.5 and 13.9 months (P =.11). CONCLUSIONS: Calciphylaxis has a female preponderance, with a dismal prognosis. A multidisciplinary approach that uses frequent hemodialysis to normalize calcium and phosphorus levels and local debridement of skin lesions seems prudent. Parathyroidectomy cannot be recommended routinely in all patients, unless severe hyperparathyroidism mandates intervention.

Adult↗