Biomedical subjects

J Bar

Publications and source records attributed to J Bar.

At least 19 recordsLinked to original sources

PubMed2006

Prostaglandin E2 induction of labor for isolated oligohydramnios in women with unfavorable cervix at term.

OBJECTIVE: To evaluate the maternal and neonatal outcomes of pregnancies complicated with isolated oligohydramnios at term, managed by induction of labor. METHODS: We conducted a retrospective case-control study. 138 women with uncomplicated oligohydramnios at term [amniotic fluid index (AFI) < or =5 cm] and a low Bishop score (< or =6) underwent induction of labor with prostaglandin E2. These women were compared to 67 women who underwent induction of labor at 42 weeks' gestation and 276 women at low-risk pregnancy and spontaneous onset of labor, matched for parity and race. RESULTS: Cesarean section (CS) rate was similar in the study and the post-date group (17.4 and 17.9%, respectively), but significantly higher than the spontaneous labor group (5.8%, OR 3.42, 95% CI 1.75-6.68). No differences were found with other outcomes. CONCLUSION: Pregnancies with isolated oligohydramnios at term apparently are not at higher risk of perinatal complications, but induction of labor is associated with increased rate of CS.

Case-Control Studies↗

PubMed2006

Repression of the MSP/MST-1 gene contributes to the antiapoptotic gain of function of mutant p53.

Tumor-associated mutant forms of p53 can exert an antiapoptotic gain of function activity, which confers a selective advantage upon tumor cells harboring such mutations. We report that mutant p53 suppresses the expression of the MSP (MST-1/HGFL) gene, encoding the ligand of the receptor tyrosine kinase RON, implicated in a variety of cellular responses. Mutant p53 associates with the MSP gene promoter and represses its transcriptional activity, leading to a decrease in mRNA levels and a subsequent decrease in the levels of secreted MSP protein. Forced downregulation of MSP expression in H1299 cells, derived from a large-cell lung carcinoma, confers increased resistance against etoposide-induced cell death. These antiapoptotic consequences of MSP downregulation seemingly conflict with the well-documented ability of the RON receptor to promote cell survival and tumor progression when aberrantly hyperactive. Yet, they are consistent with the fact that reduced MSP expression was observed in many types of human cancer, including large-cell lung carcinoma. Thus, repression of MSP gene expression by mutant p53 may contribute to oncogenesis in a cell type-specific manner.

Apoptosis↗

PubMed2005

The PI3K inhibitor LY294002 prevents p53 induction by DNA damage and attenuates chemotherapy-induced apoptosis.

The p53 tumor suppressor plays a key role in the natural protection against cancer. Activation of p53 by DNA-damaging agents can contribute to successful elimination of cancer cells via chemotherapy-induced apoptosis. The phosphatidylinositol-3 kinase (PI3K) pathway, triggered in normal cells upon exposure to growth factors, regulates a cascade of proliferation and survival signals. The PI3K pathway is abnormally active in many cancers, thus making it an attractive target for inactivation in an attempt to achieve better cancer therapy. We report here that exposure to LY294002, a potent PI3K inhibitor, aborts the activation of p53 by several drugs commonly used in cancer chemotherapy. Concomitantly, LY294002 attenuates p53-dependent, chemotherapy-induced apoptosis of cancer cells. These findings invoke an unexpected positive role for PI3K in p53 activation by anticancer agents, and suggest that the efficacy of PI3K inhibitors in cancer therapy may be greatly affected by the tumor p53 status.

Animals↗

PubMed2005

Maternal and fetal outcome of lupus pregnancy: a prospective study of 29 pregnancies.

The aim of this study was to analyse pregestational and pregnancy risk factors for adverse fetal and maternal outcome in lupus pregnancy. Twenty women with systemic lupus erythematosus (SLE) (29 pregnancies) were prospectively evaluated. Mean patient age was 29.5+/-4.7 years, and mean disease duration, 6.3+/-6.5 years. Twenty-two pregnancies (75.9%) ended in live births; preterm delivery occurred in 17.4%, intrauterine growth restriction in 50%, preeclampsia in 3.7%, and gestational hypertension in 8%. Six pregnancies (20.7%) ended in spontaneous abortions. Adverse live-birth outcome was significantly associated with low pregestational serum albumin level, elevated gestational anti-dsDNA antibody, and diabetes mellitus. Spontaneous abortion was directly associated with low levels of pregestational serum albumin, positive anticardiolipin IgA, anti-beta2-glycoprotein I IgM, and anti-La antibodies, and inversely associated with number of patients' children. Postgestational lupus flare-up was noted in six pregnancies. Risk factors included high pregestational SLE Disease Activity Index (SLEDAI), lower serum albumin, elevated serum antibody to dsDNA, proteinuria, and use of prednisone and hydroxychloroquine. We conclude that despite high rate of obstetrical complications and postpartum lupus flare-up, pregnancy poses low risk for the majority of women with SLE.

Adult↗

PubMed2005

The pharmacologic approach to the prevention of preeclampsia: from antiplatelet, antithrombosis and antioxidant therapy to anticonvulsants.

Preeclampsia has been suggested to be a two-stage disorder of an alteration in placental perfusion (stage 1) leading to generalized vascular endothelial damage (stage 2). Because the mechanism linking the two stages remains unclear, effective primary prevention is still impossible. However, advances made in our understanding of the pathophysiology of preeclampsia have paved the way for secondary and tertiary prevention approaches. Platelets are known to be activated in early pregnancy. They also play a pivotal role in the process of inflammation, as demonstrated by the finding that CD40 ligand is shed from activated platelets to directly initiate inflammation of the vessel wall. According to the Cochrane Library Update summarizing data from over 30,000 women, secondary prevention with antiplatelet drugs is associated with a 19% decrease in the risk of preeclampsia. Additional randomized controlled trials are needed to establish the association between preeclampsia and thrombophilia. The effect of the antithrombotic agent heparin on pregnancy outcome in preeclampsia and its potential preventive action in high-risk patients need to be elucidated. One of the several hypotheses of the pathogenesis of preeclampsia focuses on the oxidative stress caused by the imbalance in prooxidant and antioxidant forces. Preliminary findings on vitamin E and vitamin C supplementation in preeclamptic women are encouraging, and suggest a rationale for larger clinical trials. Although there is currently no explanation for the positive effect of magnesium sulfate on eclamptic seizures, studies have provided enough evidence to encourage its worldwide use as the primary anticonvulsant of choice in the tertiary prevention of maternal and perinatal death in severe preeclampsia/eclampsia. In conclusion, secondary and tertiary prevention of preeclampsia is possible when targeted at reducing maternal and neonatal morbidity and mortality.

Anticonvulsants↗

PubMed2004

TP53 status determines clinical significance of ERBB2 expression in ovarian cancer.

ERBB2 expression has been found in 19 to 44% of ovarian carcinomas; however, its predictive value has not been demonstrated, and trastuzumab has not found clinical application in ovarian cancer patients. We evaluated clinical significance of ERBB2 expression in relation to TP53 accumulation in ovarian carcinoma patients treated with platinum-based regimens. Immunohistochemical analysis with CB11 and a novel NCL-CBE356 antibody (against the internal and external domains of ERBB2, respectively) was performed on 233 tumours (FIGO stage IIB-IV); the US Food and Drug Administration-approved grading system with 0 to 3+ scale was used for evaluation, and the results were analysed by the Cox and logistic regression models. In all, 42% of the tumours expressed (category 1+, 2+ or 3+) either CB11 or CBE356 or both (CB11/CBE356 parameter). Associations between ERBB2 expression and clinical factors were observed only if tumours with staining category 1+ were grouped together with tumours showing staining categories 2+ and 3+. CB11/CBE356 parameter had a better predictive value than CB11 alone. CB11/CBE356 expression was negatively associated with platinum sensitivity (PS) in the TP53(-) group (P=0.022) and with disease-free survival (DFS) in the TP53(+) group (P=0.009). Our results may suggest that trastuzumab should be given postoperatively to patients with TP53(-)/ERBB2(+) ovarian carcinomas to enhance PS, and after completion of chemotherapy to patients with complete remission and TP53(+)/ERBB2(+) carcinomas to extend DFS time (in total to 30.4% of all patients analysed). Thus, novel criteria for ovarian cancer patient inclusion for clinical trials with trastuzumab should be considered and tested.

Adult↗

PubMed2004

Accuracy of sonographically estimated fetal weight in 840 women with different pregnancy complications prior to induction of labor.

OBJECTIVES: To evaluate the accuracy of sonographically estimated fetal weight (EFW) shortly before induction of labor in the presence of different pregnancy complications, and to define possible variables affecting it. METHODS: The study sample consisted of 840 women with singleton pregnancies and cephalic presentation who were admitted to our unit for induction of labor between January 1999 and December 2000. All underwent detailed ultrasound assessment for EFW, amniotic fluid index, biophysical profile and placental location. Indications included previous Cesarean section, postdate pregnancy, pregnancy-induced hypertension, diabetic pregnancy, suspected large-for-gestational age (LGA) infants, suspected fetal growth restriction (FGR), oligohydramnios, decreased fetal movements, premature rupture of membranes at or before term. EFW was calculated after measuring fetal abdominal circumference and femur length. The EFW was compared with the weight at delivery, 1-3 days later. RESULTS: There was a high correlation between EFW and birth weight (R(2) = 0.775, P < 0.001). The mean birth weight was 3207 +/- 561 g, and mean absolute weight difference was 227 +/- 197 g; (absolute range, 0-1700 g; actual range, - 986 to + 1700 g). The mean weight difference was significantly different between the patients with LGA infants, FGR infants and preterm premature rupture of membranes (PPROM) (- 110 +/- 281 g, + 113 +/- 195 g and + 115 +/- 307 g, respectively, P < 0.01). Stepwise linear regression analysis of the effects of maternal and pregnancy characteristics on the weight difference yielded lower gestational age, higher birth weight, anterior placenta, higher gravidity, and younger maternal age as independent and significant variables associated with greater actual weight difference inaccuracy (R(2) = 0.099, P < 0.001), and higher birth weight as the only independent and significant variable associated with greater absolute weight difference (R(2) = 0.09, P = 0.018). CONCLUSIONS: The sonographic EFW is highly correlated with birth weight. However, clinicians should be aware of the risk of overestimation in pregnancies with suspected LGA and underestimation in pregnancies with PPROM and suspected FGR.

Adult↗

PubMed2004

Results of in vitro fertilization cycles in women aged 43-45 years.

Our objective was to evaluate the results of in vitro fertilization (IVF) cycles in the elderly (43-45 years old) female population. All consecutive women aged 43-45 years admitted to our IVF unit from January 1996 to December 2001 were enrolled in the study. Ovarian stimulation characteristics, number of oocytes retrieved, number of embryos transferred and pregnancy rate were assessed. Seven hundred and eight consecutive IVF cycles in 276 patients were evaluated. Two hundred and seven cycles were cancelled (cancellation rate 29.2%). Forty-seven patients achieved a clinical pregnancy (pregnancy rate 6.6% per cycle and 9.4% embryo transfer) with a 30% live birth rate. In patients who underwent embryo transfer, there were no differences between conception and non-conception cycles in patient's age, number of gonadotropin ampules used, length of ovarian stimulation, number of oocytes retrieved, fertilization rate or cleavage rate. However, the conception cycles were associated with a significantly lower peak estradiol level (p < 0.04) and higher number of total (p < 0.03) and good-quality (p < 0.005) embryos transferred, in addition to a lower ratio of estradiol level/number of follicles > 14 mm on day of human chorionic gonadotropin administration and of estradiol level/number of oocytes retrieved. We conclude that, although older female age is a major contributor to IVF failure, successful IVF cycles can be expected in patients aged 43-45 years in the presence of low ratios of peak estradiol to either number of follicles > 14 mm on day of human chorionic gonadotropin administration or number of oocytes retrieved that reach the stage of embryo transfer with at least two good-quality embryos.

Adult↗

PubMed2004

Is assisted hatching beneficial in patients with recurrent implantation failures?

PURPOSE: To assess the possible role of assisted hatching in patients with recurrent implantation failure during IVF cycles. DESIGN: Prospective randomized study. SETTING: IVF unit of an academic medical center. PATIENTS: Women who underwent IVF after at least three failed IVF-ET attempts. INTERVENTIONS: Patients were prospectively randomized to undergo assisted hatching of their embryos prior to their replacement by mechanical partial zona dissection. RESULTS: The study (assisted hatching) and control groups included 104 and 103 patients, respectively. There were no significant between-group differences in patient age, cause of infertility, mean number of previous IVF trials, number of oocytes retrieved, fertilization rate, or number of embryos transferred. No difference in pregnancy rate was noted on comparison of the whole study group, to the whole control group (21% and 27%, respectively). However, when the results were re-analyzed by age groups, assisted hatching was found to be harmful in the youngest group (< 34 years), significantly decreasing pregnancy rates (15% vs 35%, p < 0.05). CONCLUSION: Repeated implantation failure alone is not an indication for assisted hatching. Although assisted hatching appears to be effective in a selected group of older patients, in younger patients it may further hamper implantation and should be avoided.

Adult↗

PubMed2003

Strict glycemic control in diabetic pregnancy-implications for second-trimester screening for Down syndrome.

OBJECTIVES: Studies in the early 1990s showed that the normal levels of the biochemical markers used to screen for Down syndrome in the second trimester of pregnancy differ between healthy women and women with insulin-dependent diabetes mellitus (IDDM). Thereafter, most laboratories adopted correcting factors to adjust for these differences. However, the current validity of these factors in light of the recent improvements in glycemic control in diabetic pregnancy has not been investigated. METHODS: The sample consisted of 35 pregnant women with strictly controlled IDDM and 40 healthy controls matched for age and gestational week. All women had singleton pregnancies and were followed till delivery. RESULTS: Comparison of the triple test results between the two groups after adjustment with the traditional corrective factors yielded no significant differences in serum levels of any of the markers (unconjugated estriol, human chorionic gonadotrophin, alpha-fetoprotein). CONCLUSIONS: These results suggest that the recent improvement in glycemic control of pregnant women with IDDM changes the metabolic milieu that might cause the biochemical differences with healthy pregnant patients.

Adult↗

PubMed2003

The 'virtual' cervical internal os: diagnosis during the first trimester of pregnancy.

OBJECTIVE: To determine the appropriate time during gestation for assessing the cervix for possible incompetence by ascertaining the gestational week at which the sac reaches the level of the internal os. METHODS: Three hundred and eighteen women with an intact singleton pregnancy at 5 to 15 weeks' gestation underwent endovaginal sonographic examination to measure the distance between the gestational sac and the cervical internal os. The change in location of the gestational sac in the endometrial cavity over time, and the earliest gestational week at which the gestational sac reached the level of the internal os, were calculated and analyzed with one-way analysis of variance. RESULTS: The distance between the gestational sac and the 'virtual' cervical internal os decreased from a mean of 4.72 cm at 5 weeks to 1.71 cm at 13 weeks. This distance decreased significantly during the early first trimester (weeks 5 to 7) (P = 0.004), but remained almost unchanged from 12 to 15 weeks. Only at 12 weeks' gestation could the entire cervical length be determined and the configuration of the cervical internal os assessed for incompetence. CONCLUSIONS: Transvaginal sonography is a good method for evaluating the cervix during pregnancy. The location of the 'virtual' internal os can be determined in the first trimester using the urinary bladder as a reference point.

Adult↗

PubMed2002

Sonographic evaluation of the puerperal uterus: correlation with manual examination.

OBJECTIVES: To document uterine involution after vaginal delivery and cesarean section by abdominal sonography and to compare the efficacy of manual examination and ultrasonography. STUDY DESIGN: Postpartum manual and sonographic assessment of uterine involution was performed in 120 patients following vaginal and cesarean delivery with an attempt to build a database of changes in uterine dimensions. The patients' reports on the intensity of uterine contractions and vaginal bleeding were compared to the results of sonographic imaging. RESULTS: Palpation revealed proper uterine involution in 80 and 25% of patients after vaginal delivery and cesarean section, respectively. It could not be performed in 2.5% after vaginal delivery compared to 50% after cesarean section. Uterine length was found to be significantly greater after cesarean section than after vaginal delivery (p = 0.0001), and the anterior uterine wall was significantly thinner than the posterior wall (p = 0.0001). Uterine length was significantly greater in the presence of blood accumulation in the uterine cavity (20.7 cm), than when the uterus was empty (18.8 cm) (p = 0.001). In correlating between the patient's report of intense bleeding and the sonographic picture of blood in the uterine cavity sonography had a sensitivity of 0.56 and a specificity of 0.83, whereas the patients' reports had a positive predictive value of 0.22. The difference in information provided by the patients versus that provided by sonography was highly significant (p = 0.001, chi(2) test). CONCLUSION: Within 3 days after delivery, patients particularly those having had a cesarean section, should undergo uterine sonographic scanning and manual palpation to evaluate involution and presence of blood in the uterine cavity.

Adult↗

PubMed2002

Beta2-microglobulin and hypertensive complications in pregnant women at risk.

BACKGROUND AND AIM: Beta2-microglobulin (beta2-m) is a polypeptide, which is freely filtered through the glomerular basement membrane and absorbed almost entirely by the proximal tubular cells. Preeclampsia, a common complication of pregnancy, is characterized by pathological renal changes, mainly glomerular lesions. The aim of the present study was to investigate whether serum beta2-m measured in the early stages of pregnancy could be used as a marker to predict hypertensive complications in women at increased risk. PATIENTS AND METHODS: Serum beta2-m concentrations were prospectively measured in 75 pregnant women with history of chronic hypertension, chronic renal disease, chronic vascular disease or preeclampsia and compared with those in 16 healthy pregnant women. RESULTS: Of the 75 women in the study group, 10 (13%) developed preeclampsia and 20 (26%) had other complications, such as intrauterine growth restriction (n = 8), fetal or neonatal loss (n = 9) and delivery before 30 weeks of gestation (n = 8). Gestational age at delivery, birth weight and cesarean section rate were significantly worse in the patients with complications than in those without and in the healthy controls. No significant difference was detected in early serum beta2-m concentrations between the women who later developed preeclampsia or other complications and those who did not. There was a significant positive correlation of beta2-m concentrations with serum creatinine level (R2 = 0.394, p < 0.001), but not with gestational week at blood collection. CONCLUSION: Serum beta2-m concentrations are not predictive of the development of preeclampsia or other complications in woman at risk.

Adult↗

PubMed2002

A survey of physicians' attitude and approach to hormone replacement therapy during menopuase.

OBJECTIVE: A major problem with postmenopausal hormone replacement therapy (HRT) is its relatively low long-term continuation rate. The aim of the present study was to assess the contribution of physicians to the low long-term continuation rate by surveying their attitude and approach to the use of HRT in postmenopausal women. PATIENTS AND METHODS: A questionnaire was sent to registered members of the North American Menopause Society. Data were collected on demographics, medical education and affiliation, attitude to the use of HRT and its contraindications, and follow-up, strategies. RESULTS: The response rate was 21% (n = 218). Sixty-six percent of the physicians recommended HRT for every postmenopausal woman with no contraindications. and 11% also took age and/or time since menopause into consideration. Eighty-six percent claimed they would try to persuade symptom-free women not interested in HRT into changing their minds. There was no correlation between the time since completion of residency or affiliation with a medical school and physicians' attitude to prescribing HRT or contraindication to HRT, or management strategy. However, type of specialty was significantly correlated with physicians' tendency to recommend HRT. Specialists in menopause showed a lower tendency to unconditionally recommend HRT (in the absence of contraindications) (67%) than specialists in reproductive endocrinology (90%), infertility (90%). gynecology (83%), and perinatology (84%) (p < 0.006, C-measure = 0.25). CONCLUSIONS: The attitude toward HRT and the management strategies of members of the North American Menopause Society correlate with contemporary recommendations in the literature, indicating good training of young physicians and adequate updating of older ones. Thus, to increase the continuation rate of HRT, educational efforts should be directed primarily to the public rather than to medical professionals.

Adult↗

PubMed2001

Human acid ceramidase: processing, glycosylation, and lysosomal targeting.

The biosynthesis of human acid ceramidase (hAC) starts with the expression of a single precursor polypeptide of approximately 53-55 kDa, which is subsequently processed to the mature, heterodimeric enzyme (40 + 13 kDa) in the endosomes/lysosomes. Secretion of hAC by either fibroblasts or acid ceramidase cDNA-transfected COS cells is extraordinarily low. Both lysosomal targeting and endocytosis critically depend on a functional mannose 6-phosphate receptor as judged by the following criteria: (i) hAC-precursor secretion by NH(4)Cl-treated fibroblasts and I-cell disease fibroblasts, (ii) inhibition of the formation of mature heterodimeric hAC in NH(4)Cl-treated fibroblasts or in I-cell disease fibroblasts, and (iii) blocked endocytosis of hAC precursor by mannose 6-phosphate receptor-deficient fibroblasts or the addition of mannose 6-phosphate. The influence of the six individual potential N-glycosylation sites of human acid ceramidase on targeting, processing, and catalytic activity was determined by site-directed mutagenesis. Five glycosylation sites (sites 1-5 from the N terminus) are used. The elimination of sites 2, 4, and 6 has no influence on lysosomal processing or enzymatic activity of recombinant ceramidase. The removal of sites 1, 3, and 5 inhibits the formation of the heterodimeric enzyme form. None of the mutant ceramidases gave rise to an increased rate of secretion, suggesting that lysosomal targeting does not depend on one single carbohydrate chain.

Ammonium Chloride↗

PubMed2001

Effect of thrombophylaxis on uterine and fetal circulation in pregnant women with a history of pregnancy complications.

PURPOSE: The aim of this study was to investigate the effect of thromboprophylactic therapy on fetal and maternal Doppler flow parameters in pregnant women with severe complications in previous pregnancies and evidence of acquired or congenital thrombophilia in the current pregnancy. METHODS: Sixty-five patients with a history of recurrent abortions, intrauterine fetal death, intrauterine growth restriction (IUGR), and severe early-onset preeclampsia were tested for the presence of acquired or congenital thrombophilia. Those with positive findings were prescribed low-dose aspirin plus low-molecular-weight heparin (LMWH) (enoxaparin); the remainder received low-dose aspirin only. A Doppler flow study was performed before and after treatment and in the third trimester of pregnancy. RESULTS: Of the 65 pregnancies, four ended in spontaneous abortion and were excluded from the analysis. Of the 61 women with completed pregnancies, 37 (61%) had evidence of acquired or congenital thrombophilia: 22 (36%) protein S deficiency; 1 (2%) protein C deficiency; 2 (3%) activated protein C resistance (APC-R); 2 (3%) IgG for antiphospholipid antibodies; 1 (2%) circulating anticoagulant; and 9 (15%) a combined defect. This group showed a significant decrease in mean uterine artery pulsatility index (PI) before and after treatment (1.32+/-0.36 vs. 1.04+/-0.23, P=.006), whereas the remaining 24 patients treated with low-dose aspirin only had nonsignificant changes. Pearson's correlation test yielded no correlations of the pregnancy outcome parameters with Doppler flow values in the umbilical or uterine arteries. CONCLUSIONS: Thromboprophylactic therapy transiently improves maternal circulation parameters in patients with thrombophilia at risk of fetal loss and other severe complications of pregnancy, but not in correlation with their pregnancy outcome. Therefore, Doppler examination of maternofetal circulation in the second trimester is not predictive of pregnancy outcome.

Abortion, Habitual↗

PubMed2001

Brachial plexus injury and obstetrical risk factors.

OBJECTIVE: To determine whether known historical risk factors of brachial plexus injury differ between affected neonates and healthy controls. METHODS: The files of all 62 children with Erb's palsy who were diagnosed after birth were reviewed. The control group consisted of 124 randomly selected uninjured infants born within the same period. RESULTS: Compared with the control group, the mothers of the neonates with brachial plexus injury were found to be significantly older (32.1+/-5.2 years vs. 28.9+/-5.8 years, P = 0.01), and had a significantly higher incidence of diabetic pregnancy (69% vs. 14.5%, P = 0.001); the infants had a significantly higher mean birth weight (3846+/-576 g vs. 3220+/-582 g, P = 0.0001) and higher incidence of birth weight > or = 4000 g (27% vs. 4.8%, P = 0.0001). Two of the infants in the study group (3.2%) were born by elective cesarean section. CONCLUSIONS: Brachial plexus injury is associated with several non-predictable or preventable risk factors.

Adult↗

PubMed2001

Pulmonary-renal syndrome in systemic sclerosis.

BACKGROUND AND OBJECTIVE: Renal failure, pulmonary hypertension, and interstitial lung disease are major causes of morbidity and mortality in systemic sclerosis (SSc). However, the concomitant occurrence of pulmonary hemorrhage associated with acute renal failure in SSc has been rarely described. The present study is the first analysis of pulmonary-renal syndrome in SSc. PATIENT AND METHODS: We present a 44-year-old woman with SSc who died of a fulminant course of acute renal failure associated with diffuse alveolar hemorrhage. We termed this uncommon and fatal complication of SSc scleroderma-pulmonary-renal syndrome (SPRS). A search of the English-written literature yielded reports of 10 additional similar cases. These patients, together with our present case, form the basis of the present analysis. RESULTS: The average age of the patients with SPRS was 46 years. The majority of the patients (80%) were women, and most had diffuse SSc. SPRS occurred an average of 6.4 years after disease onset and was associated with prior fibrosing alveolitis and/or D-penicillamine treatment. Interestingly, normotensive renal failure seems to characterize the scleroderma patients, because 9 of 11 (82%) had normal blood pressure. SPRS bears a poor prognosis: all of the 11 patients (100%) died within 12 months of admission. However, only 60% of the 5 patients for whom we have treatment data received corticosteroids. CONCLUSIONS: Pulmonary-renal syndrome is a rare but fatal complication of SSc. Because the treatment data are scarce and the prognosis is poor, aggressive treatment with pulse corticosteroids, cyclophosphamide, and possibly plasmapheresis is suggested.

Acute Kidney Injury↗