- Email: [email protected]
Maternal and fetal complications of pregnancy in the Marfan syndrome
L
WBC
Ethnicity
Nativity
abortion
Pregnancy
of pregnancy
in the Marfan syndrome.
Aortic dissection disease
lupus erythematosus Interstitial
Lung pathology pneumonitls
Analy-
The nature and frequency of pulmonary involvement in systemic lupus erythematosus (SLE) is controversial. We reviewed autopsy files at The Johns Hopkins Hospital of 120 patients with SLE to determine pulmonary changes that were attributable to SLE rather than possible afternative explanations. Moderate or severe pulmonary parenchyrnal alterations attributable to SLB were found in 22 patients (18 percent) including: five patients with interstitial fibrosis, two with pulmonary vasculitis, one with pulmonary hematoxylin bodies and 11 of 15 (73 percent) with interstitial pneumonitis. Alveolar hemorrhage was unexplained in only two of 29 (7 percent) patients Pleuritis and pleural effusions were attributed to SLE in 22 of 36 (81 percent) and three of 28 (11 percent) patients, respectively. The findings suggest that many nonspecific pulmonary lesions previously attributed to SLE, such as alveolar hemorrhage. alveolar wall necrosis, edema and hyaline membranes, are probably secondary to intercurrent infection, congestive heart failure, renal failure or oxygen toxicity.
Haupt HM. Moore GW, Hutchins GM: The lung in systemic lupus erythematosus sis of the pathologic changes in 120 patients. Am J Med 1981; 71: 791-798.
Pulmonary
Continued on page A40
The medical literature reports 20 episodes of aortic dissection assocrated with pregnancy in 32 women affected by the Marfan syndrome who had at least one pregnancy. Most women had pre-existing aortic disease. Because women with the Marfan syndrome usually desire children, our clinic records were reviewed and patients were contacted to determine a more representative estimate of maternal risks. One of 26 women with the Marfan syndrome died shortly after pregnancy of endocarditis; she was the only woman to have a severe, preexisting cardiovascular condition. The prevalences of milder, pregnancy-associated cardiovascular and general complications did not differ from those in control subjects. The rate of early spontaneous abortion was higher in women with the Marfan syndrome than in control subjects. These results suggest the risk of maternal death is low in women with the Marfan syndrome who have minimal cardiovascutar disease. Women with the Marfan syndrome shoufd be counseled regarding pregnancy risks only after review of their cardiovascular status, including echocardiography.
Pyeritz RE: Maternal and fetal complications Am J Med 1981; 71: 784790.
Spontaneous
Systemic
occurrence
Martan syndrome
Famlllal
Kaposi’s sarcoma
Identification of familial cases of Kaposi’s sarcoma may help elucidate the role of genetic factors in this disease. To assess the prevalence of familial occurrences, the ethnic distribution of Kaposi’s sarcoma and the prevalence of other diseases in our patient population, we have reviewed all cases of Kaposi’s sarcoma seen at Memorial Sloan-Kettering Cancer Center between 1954 and 1975. Each patient was categorized on the basis of sex, age of onset of Kaposi’s sarcoma, ethnic background, nativity, family history of Kaposi’s sarcoma and prevalence of other diseases. Only one documented occurrence of familial Kaposi’s sarcoma was found out of 90 cases reviewed. The frequency of familial Kaposi’s sarcoma in our patient population supports the findings of other investigators. An ethnic predominance of Kaposi’s sarcoma was substantiated, with most patients being immigrants from high-incidence areas (54 of 77) and predominantly of Jewish and Italian heritage (52 Jewish and 17 Italian of 87).
count
The long-term pattern of prednisone-induced leukocytosis was examined in 80 patients. A variable leukocytic response was found in which the white blood cell count surpassed 20,000 mm3 as early as the first day of treatment and persisted for the duration of therapy. Although the degree of leukocytosis was unrelated to the dosage administered, it did appear sooner with hi@er doses. Leukocytosis reached maximal values within two weeks in most cases, after which the white blood cell count decreased, albeit not to pretreatment levels. Even small doses of prednisone, administered over a prolonged period of time, can induce extreme and persistent leukocytosis. This observation is of consequence especially when infection is suspected, particularly in an immunocompromised host. A shift to the left in the peripheral white blood cells and the appearance of toxic granulation may assist in the differential diagnosis between infection, in which the latter are observed, and corticosteroid-induced leukocytosis, in which they are rare.
Corticosteriods
DiGiovanna JJ, Safei B: Kaposi’s sarcoma. Retrospective study of 90 cases with particular emphasis on the familial occurrence, ethnic background and prevalence of other diseases. Am J Med 1981: 71: 779-783.
host
Leukocytosis
Shoenfeld Y, Gurewich Y, Gallant LA, Pinkhas J: Prednisone-induced leukocytosis. fnfluence of dosage, method and duration of administration on the degree fo leukocytosis. Am J Med 1981; 71: 773-778.
lmmunocompromised
Prednisone
WBC
Ethnicity
Nativity
abortion
Pregnancy
of pregnancy
in the Marfan syndrome.
Aortic dissection disease
lupus erythematosus Interstitial
Lung pathology pneumonitls
Analy-
The nature and frequency of pulmonary involvement in systemic lupus erythematosus (SLE) is controversial. We reviewed autopsy files at The Johns Hopkins Hospital of 120 patients with SLE to determine pulmonary changes that were attributable to SLE rather than possible afternative explanations. Moderate or severe pulmonary parenchyrnal alterations attributable to SLB were found in 22 patients (18 percent) including: five patients with interstitial fibrosis, two with pulmonary vasculitis, one with pulmonary hematoxylin bodies and 11 of 15 (73 percent) with interstitial pneumonitis. Alveolar hemorrhage was unexplained in only two of 29 (7 percent) patients Pleuritis and pleural effusions were attributed to SLE in 22 of 36 (81 percent) and three of 28 (11 percent) patients, respectively. The findings suggest that many nonspecific pulmonary lesions previously attributed to SLE, such as alveolar hemorrhage. alveolar wall necrosis, edema and hyaline membranes, are probably secondary to intercurrent infection, congestive heart failure, renal failure or oxygen toxicity.
Haupt HM. Moore GW, Hutchins GM: The lung in systemic lupus erythematosus sis of the pathologic changes in 120 patients. Am J Med 1981; 71: 791-798.
Pulmonary
Continued on page A40
The medical literature reports 20 episodes of aortic dissection assocrated with pregnancy in 32 women affected by the Marfan syndrome who had at least one pregnancy. Most women had pre-existing aortic disease. Because women with the Marfan syndrome usually desire children, our clinic records were reviewed and patients were contacted to determine a more representative estimate of maternal risks. One of 26 women with the Marfan syndrome died shortly after pregnancy of endocarditis; she was the only woman to have a severe, preexisting cardiovascular condition. The prevalences of milder, pregnancy-associated cardiovascular and general complications did not differ from those in control subjects. The rate of early spontaneous abortion was higher in women with the Marfan syndrome than in control subjects. These results suggest the risk of maternal death is low in women with the Marfan syndrome who have minimal cardiovascutar disease. Women with the Marfan syndrome shoufd be counseled regarding pregnancy risks only after review of their cardiovascular status, including echocardiography.
Pyeritz RE: Maternal and fetal complications Am J Med 1981; 71: 784790.
Spontaneous
Systemic
occurrence
Martan syndrome
Famlllal
Kaposi’s sarcoma
Identification of familial cases of Kaposi’s sarcoma may help elucidate the role of genetic factors in this disease. To assess the prevalence of familial occurrences, the ethnic distribution of Kaposi’s sarcoma and the prevalence of other diseases in our patient population, we have reviewed all cases of Kaposi’s sarcoma seen at Memorial Sloan-Kettering Cancer Center between 1954 and 1975. Each patient was categorized on the basis of sex, age of onset of Kaposi’s sarcoma, ethnic background, nativity, family history of Kaposi’s sarcoma and prevalence of other diseases. Only one documented occurrence of familial Kaposi’s sarcoma was found out of 90 cases reviewed. The frequency of familial Kaposi’s sarcoma in our patient population supports the findings of other investigators. An ethnic predominance of Kaposi’s sarcoma was substantiated, with most patients being immigrants from high-incidence areas (54 of 77) and predominantly of Jewish and Italian heritage (52 Jewish and 17 Italian of 87).
count
The long-term pattern of prednisone-induced leukocytosis was examined in 80 patients. A variable leukocytic response was found in which the white blood cell count surpassed 20,000 mm3 as early as the first day of treatment and persisted for the duration of therapy. Although the degree of leukocytosis was unrelated to the dosage administered, it did appear sooner with hi@er doses. Leukocytosis reached maximal values within two weeks in most cases, after which the white blood cell count decreased, albeit not to pretreatment levels. Even small doses of prednisone, administered over a prolonged period of time, can induce extreme and persistent leukocytosis. This observation is of consequence especially when infection is suspected, particularly in an immunocompromised host. A shift to the left in the peripheral white blood cells and the appearance of toxic granulation may assist in the differential diagnosis between infection, in which the latter are observed, and corticosteroid-induced leukocytosis, in which they are rare.
Corticosteriods
DiGiovanna JJ, Safei B: Kaposi’s sarcoma. Retrospective study of 90 cases with particular emphasis on the familial occurrence, ethnic background and prevalence of other diseases. Am J Med 1981: 71: 779-783.
host
Leukocytosis
Shoenfeld Y, Gurewich Y, Gallant LA, Pinkhas J: Prednisone-induced leukocytosis. fnfluence of dosage, method and duration of administration on the degree fo leukocytosis. Am J Med 1981; 71: 773-778.
lmmunocompromised
Prednisone