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Werner's syndrome with associated malignant neoplasms
CURRENT LlTERATURE
Werner’s syndrome with associated malignant neoplasms. Hrabka RP, Milgrom H, Schwartz RA. Arch
Anatomical Dead Space and Airway Resistance after Glycopyrrolate or Atropine Premeditation. Gotta AW,
Dermatol
Ray C, Sullivan CA, Goldiner 363, 1981
118: 106, 1982
Werner’s syndrome is an autosomal recessive disease of connective tissue characterized by features of accelerated aging, bilateral juvenile cataracts, scleroderma-like skin changes, and a high incidence of neoplasms. This report presents a 57-year-old man who was originally referred for the excision of two basal cell carcinomas on the dorsum of his nose. His surgical history consisted of removal of bilateral juvenile cataracts at age 8, correction of undescended testes and diverticulosis at age 39, and thoracotomy at age 46 for removal of a significant mediastinal mass, which was later histologically diagnosed as a weli-differentiated fibrosarcoma. Physical examination disclosed a well-developed man with the characteristic facies of Werner’s syndrome. Pseudoexophthalmos and a bound down beaklike nose were prominent findings. Scleroderma-like skin changes were noted, such as atrophic facial skin with numerous telangiectases, and tightly drawn circumoral skin with numerous radial furrows. The lenses were absent, and the remaining testis was atrophic. Two crusted, translucent plaques were present on the nose, and there was a similar lesion on the upper lip. A glucose tolerance test showed latent diabetes mellitus, with other laboratory values being relatively normal. This case is significant in that multiple basal cell epitheliomas have been rarely reported with this syndrome. The authors suggest that early recognition of Werner’s syndrome allows early detection of associated malignant neoplasms, which are a common cause of death in these patients.-BRAD A. THERIOT
25:
Anatomic dead space (VD anal), I- and 3-second forced expiratory volumes (FEV, and FEVA, total forced expiratory volume (FEV). and maximal expiratory flow variables were determined before and four hours after intramuscular injection of saline, 0.0085 mg/kg atropine. or 0.005 mg/kg of glycopyrrolate. Both atropine and glycopyrrolate increased dead space in all subjects. Maximal effect of atropine occurred at one hour and was diminished by four hours. Glycopyrrolate was maximal at two hours and was stable at four hours. Both drugs increased FEV,, but the increase was significant one-half hour after injection. Both FEV and FEV:, were unchanged for both medications. Atropine and glycopyrrolate have a known bronchodilation capacity. However, this study demonstrates that both drugs dilate smaller conducting airways without affecting airway resistance.-TIMOTHY KABOT A Case-control Study of the Possible Association Between Oral Contraceptives and Malignant Melanoma.
Adam SA, Sheaves JK, Wright NH, Mosser G, Harris RW, Vessey MP. Br J Cancer 44:45. 1981 The incidence and mortality of malignant melanoma have increased more in recent years than most other cancers. In 1977 Beral et al reported an association between oral contraceptives and malignant melanoma in three separate sets of data collected in California. The purpose of this study was to reexamine the relationship between oral contraceptives and malignant melanoma. This casecontrol study consisted of 169 women with malignant melanoma, aged 15 to 49 years, and 507 matched controls. Data about medical, reproductive. drug. and smoking histories were obtained by a review of general practitioners’ records as well as by direct answers from the subjects on a mailed questionnaire. The authors state that there was no significant evidence of any overall increase in the risk of melanoma in the users of oral contraceptives. However, the risk estimated from the data in the mailed questionnaire was higher in women who had used oral contraceptives for five years or more. Previously demonstrated risk factors for melanoma, such as fair skin, blonde or red hair, and Celtic origin were found to be more common in the cases than in the COnWOk-JOHN A. GASSER
Postmenopausal osteoporosis: A heterogenous disorder as assessed by histomorphometric analysis of iliac crest bone from untreated patients. Whyte MP,
Bergtield MA, Murphy WA, Avioli LV, Teitelbaum Am J Med 72:193, 1982
PL. Surv Anesthesiol
SL.
Osteoporosis is a disorder generally characterized by a decreased skeletal mass without an abnormality in either composition or proportion of the mineral and organic phases of bone. In this study, 26 women with untreated postmenopausal osteoporosis underwent tetracycline bone labeling and mineral studies followed by iliac crest bone biopsy. Histomorphometric assessment of their bone remodeling revealed a histologically heterogenous disorder with morphologic expression in bone that could not be predicted by clinical and biochemical studies. Eight women had no evidence of active bone formation (inactive remodeling osteoporosis), whereas the others showed a spectrum of bone formation (active remodeling osteoporosis). These findings suggest that the rate of bone turnover is heterogeneous and varies considerably from patient to patient. Bone biopsy, necessary to identify the histologic lesion and assess skeletal dynamics, may prove useful in determining optimal therapy for osteoporosis, as a variety of agents with different effects on bone remodeling are available.-PHYLLIS CLARK
Pulmonary Function and Its Correlation to the Degree of Obesity in 294 Patients. Lee JJ, Larsen RH, Buckley
JJ, Roberts RR. Surv Anesthesiol
25:356, 1951
A retrospective study was made of pulmonary function test results of 294 morbidly obese patients who underwent jejunoileal bypass surgery. The objective was to correlate the pulmonary function in these patients with the degree of obesity, expressed as the ratio of actual weight to ideal weight, and with age. The weight distribution of the patients was from 85 kg to 260 kg with a mean of 130 kg. The ratio of actual to 609
Werner’s syndrome with associated malignant neoplasms. Hrabka RP, Milgrom H, Schwartz RA. Arch
Anatomical Dead Space and Airway Resistance after Glycopyrrolate or Atropine Premeditation. Gotta AW,
Dermatol
Ray C, Sullivan CA, Goldiner 363, 1981
118: 106, 1982
Werner’s syndrome is an autosomal recessive disease of connective tissue characterized by features of accelerated aging, bilateral juvenile cataracts, scleroderma-like skin changes, and a high incidence of neoplasms. This report presents a 57-year-old man who was originally referred for the excision of two basal cell carcinomas on the dorsum of his nose. His surgical history consisted of removal of bilateral juvenile cataracts at age 8, correction of undescended testes and diverticulosis at age 39, and thoracotomy at age 46 for removal of a significant mediastinal mass, which was later histologically diagnosed as a weli-differentiated fibrosarcoma. Physical examination disclosed a well-developed man with the characteristic facies of Werner’s syndrome. Pseudoexophthalmos and a bound down beaklike nose were prominent findings. Scleroderma-like skin changes were noted, such as atrophic facial skin with numerous telangiectases, and tightly drawn circumoral skin with numerous radial furrows. The lenses were absent, and the remaining testis was atrophic. Two crusted, translucent plaques were present on the nose, and there was a similar lesion on the upper lip. A glucose tolerance test showed latent diabetes mellitus, with other laboratory values being relatively normal. This case is significant in that multiple basal cell epitheliomas have been rarely reported with this syndrome. The authors suggest that early recognition of Werner’s syndrome allows early detection of associated malignant neoplasms, which are a common cause of death in these patients.-BRAD A. THERIOT
25:
Anatomic dead space (VD anal), I- and 3-second forced expiratory volumes (FEV, and FEVA, total forced expiratory volume (FEV). and maximal expiratory flow variables were determined before and four hours after intramuscular injection of saline, 0.0085 mg/kg atropine. or 0.005 mg/kg of glycopyrrolate. Both atropine and glycopyrrolate increased dead space in all subjects. Maximal effect of atropine occurred at one hour and was diminished by four hours. Glycopyrrolate was maximal at two hours and was stable at four hours. Both drugs increased FEV,, but the increase was significant one-half hour after injection. Both FEV and FEV:, were unchanged for both medications. Atropine and glycopyrrolate have a known bronchodilation capacity. However, this study demonstrates that both drugs dilate smaller conducting airways without affecting airway resistance.-TIMOTHY KABOT A Case-control Study of the Possible Association Between Oral Contraceptives and Malignant Melanoma.
Adam SA, Sheaves JK, Wright NH, Mosser G, Harris RW, Vessey MP. Br J Cancer 44:45. 1981 The incidence and mortality of malignant melanoma have increased more in recent years than most other cancers. In 1977 Beral et al reported an association between oral contraceptives and malignant melanoma in three separate sets of data collected in California. The purpose of this study was to reexamine the relationship between oral contraceptives and malignant melanoma. This casecontrol study consisted of 169 women with malignant melanoma, aged 15 to 49 years, and 507 matched controls. Data about medical, reproductive. drug. and smoking histories were obtained by a review of general practitioners’ records as well as by direct answers from the subjects on a mailed questionnaire. The authors state that there was no significant evidence of any overall increase in the risk of melanoma in the users of oral contraceptives. However, the risk estimated from the data in the mailed questionnaire was higher in women who had used oral contraceptives for five years or more. Previously demonstrated risk factors for melanoma, such as fair skin, blonde or red hair, and Celtic origin were found to be more common in the cases than in the COnWOk-JOHN A. GASSER
Postmenopausal osteoporosis: A heterogenous disorder as assessed by histomorphometric analysis of iliac crest bone from untreated patients. Whyte MP,
Bergtield MA, Murphy WA, Avioli LV, Teitelbaum Am J Med 72:193, 1982
PL. Surv Anesthesiol
SL.
Osteoporosis is a disorder generally characterized by a decreased skeletal mass without an abnormality in either composition or proportion of the mineral and organic phases of bone. In this study, 26 women with untreated postmenopausal osteoporosis underwent tetracycline bone labeling and mineral studies followed by iliac crest bone biopsy. Histomorphometric assessment of their bone remodeling revealed a histologically heterogenous disorder with morphologic expression in bone that could not be predicted by clinical and biochemical studies. Eight women had no evidence of active bone formation (inactive remodeling osteoporosis), whereas the others showed a spectrum of bone formation (active remodeling osteoporosis). These findings suggest that the rate of bone turnover is heterogeneous and varies considerably from patient to patient. Bone biopsy, necessary to identify the histologic lesion and assess skeletal dynamics, may prove useful in determining optimal therapy for osteoporosis, as a variety of agents with different effects on bone remodeling are available.-PHYLLIS CLARK
Pulmonary Function and Its Correlation to the Degree of Obesity in 294 Patients. Lee JJ, Larsen RH, Buckley
JJ, Roberts RR. Surv Anesthesiol
25:356, 1951
A retrospective study was made of pulmonary function test results of 294 morbidly obese patients who underwent jejunoileal bypass surgery. The objective was to correlate the pulmonary function in these patients with the degree of obesity, expressed as the ratio of actual weight to ideal weight, and with age. The weight distribution of the patients was from 85 kg to 260 kg with a mean of 130 kg. The ratio of actual to 609