Chronic intestinal pseudo-obstruction in systemic lupus erythematosus

Intestinal pseudo-obstruction (IPO) is an uncommon and severe complication of systemic lupus erythematosus (SLE). We report a 24-year-old female with a 2 year SLE duration who presented with abdominal pain, vomiting, constipation and abdominal distention. Plain abdominal radiograph showed multiple air-fluid levels of the small bowel. Computed tomographic scan of the abdomen revealed dilated small bowel loops without mechanical obstruction. Urinary tract involvement was also demonstrated. IPO was diagnosed and the patient responded well to immunosuppressive treatment. IPO is a recently recognized manifestation of SLE that may be the presenting manifestation of the systemic disease or occur more commonly during disease course. Early recognition of IPO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.     

The relationship between angiotensin converting enzyme gene polymorphism, coronary artery disease, and stent restenosis: the role of angiotensin converting enzyme inhibitors in stent restenosis in patients with diabetes mellitus

Patients with diabetes mellitus (DM) have advanced atherosclerosis compared with nondiabetics. Restenosis after intracoronary stent implantation occurs frequently in diabetic patients. Angiotensin II is an important growth factor for the development of neointimal hyperplasia after vascular injury. The aim of our study was to evaluate the relationships between angiotensin converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism and coronary artery disease (CAD) and stent restonosis in diabetic patients. One hundred and thirty consecutive patients with CAD and 47 consecutive patients (14 males, mean age, 58.0 +/- 10.0) without CAD were enrolled in the study. All patients had type 2 (noninsulin dependent) DM. The patients with CAD underwent percutaneous transluminal coronary angioplasty (PTCA) and stenting. Ninety-four (59 males, mean age, 60.3 +/- 9.8) underwent control coronary angiography at the end of the follow-up period (mean duration, 9.1 +/- 2.9 months). ACE gene I/D genotyping was identified in all patients. No significant difference was found among patients with and without CAD with respect to ACE gene I/D polymorphism (P = 0.460). In the control coronary angiography, stent restenosis and new lesion development were comparable in each genotyping subgroup. However, a significant relationship was observed between restenosis and the use of ACE inhibitors (ACEI) in patients with D allele (ACEI ratio, 43.5% in the restenosis group and 56.5% in non-restenotic group, P < 0.05). We did not find any relationship between ACE gene I/D polymorphism and CAD and stent restenosis and new lesion development in diabetic patients. On the other hand, ACEI treatment may reduce stent restenosis in type 2 diabetic patients with D allele (DD or ID).     

The role of maternal obesity in infant outcomes in polycystic ovary syndrome—A systematic review,meta‐analysis,and meta‐regression

Polycystic ovary syndrome (PCOS) is associated with worsened pregnancy and infant outcomes, higher body mass index (BMI), and longitudinal weight gain. Despite most of the clinical features of PCOS being risk factors for worsened infant outcomes in the general population, their impact on infant outcomes in PCOS is unknown. We aimed to investigate the association of PCOS with infant outcomes considering maternal adiposity, other known risk factors, and potential confounders. The meta‐analyses included 42 studies in 7041 women with PCOS and 63 722 women without PCOS. PCOS was associated with higher gestational weight gain (GWG) and with higher preterm birth and large for gestational age and with lower birth weight with this association varying by geographic continent, PCOS phenotypes, and study quality. However, PCOS was associated with none of these outcomes on BMI‐matched studies. Gestational diabetes was significantly associated with an increased preterm birth on meta‐regression. We report for the first time that GWG is higher in PCOS. Infant outcomes vary by geographic continent and study quality but are similar in BMI‐matched women with and without PCOS. This suggests that infant outcomes in PCOS may be related to maternal obesity. These novel findings warrant future studies in PCOS investigating screening and management of infant outcomes with consideration of maternal obesity.     

Mediastinal fibrosis combined with Behçet's disease. Three case reports

PURPOSE: Intrathoracic manifestations other then vascular aneurysm and thrombosis are rare in the course of Beh?et's disease. Mediastinal fibrosis is also unusual. METHODS: Three cases of Beh?et's disease with initial mediastinal fibrosis are reported. Diagnosis means, pathogenic mechanisms and therapeutic modalities are also more precisely defined. RESULTS: Three male patients (21, 25 and 27 years of age, respectively) were hospitalized for progressive vena cava superior syndrome accompanied by mediastinal fibrosis demonstrated by MRI and/or CT scan which both showed infiltration of the mediastinal fat. Multiple calcifications were also present in one patient. Histology was performed in one case. Thrombosis of the vena cava superior and innominata veins was associated with fibrosis in all three cases. Thrombosis of venae subclavia and left jugularis was also present in one case. Other manifestations leading to the diagnosis of Beh?et's disease were demonstrated by the patient's history and, in one case, occurred on the fourth day of hospitalization. After initiation of a treatment including corticoids, anticoagulants and colchicine, the outcome was favorable in two cases. The third patient died. CONCLUSION: Mediastinal fibrosis combined with Beh?et's disease has rarely been described. However, it should be included in manifestations of the disease. The causative process which might be vasculitis has to be demonstrated.     

Bullous pemphigoid and cholestatic hepatitis associated with Castleman's disease.

The case of a 21-yr-old woman admitted with a two-week history of icterus, fever, multiple peripheral lymphadenopathy and pruritic eruption is presented. A full evaluation including computed tomography, endoscopic retrograde cholangiography, liver, skin and lymph node biopsies and biochemical tests confirmed the diagnosis of multicentric Castleman's disease (angiofollicular lymph node hyperplasia). All symptoms improved within four weeks of commencing prednisone therapy. Castleman's disease should be considered in the differental daignosis of cholestatic hepatitis and bullous pemphigoid.     

USE OF TRANSJUGULAR INTRAHEPATIC PORTOSYSTEMIC SHUNT AS A BRIDGE TO TRANSPLANTATION IN FULMINANT HEPATIC FAILURE DUE TO BUDD CHURI SYNDROME

We report a case of fulminant hepatic failure in a 55-yr-old man due to Budd-Chiari syndrome in the setting of polycythemia rubra vera. The patient presented with acute hepatic failure, which rapidly progressed to grade IV hepatic encephalopathy. Placement of a transjugular intrahepatic portosystemic shunt resulted in marked improvement of the encephalopathy and stabilized the liver failure. Suhsequently, he underwent successful nonemergent orthotopic liver transplantation. Transjugular intrahepatic portosystemic shunt placement is a safe, effective, therapeutic option to bridge patients with fulminant Budd-Chiari to liver transplantation.     

Pseudo-obstruction intestinale au cours du lupus érythémateux systémique

Intestinal pseudo-obstruction (IPO) is an uncommon and severe complication of systemic lupus erythematosus (SLE). We report a 24-year-old female with a 2 year SLE duration who presented with abdominal pain, vomiting, constipation and abdominal distention. Plain abdominal radiograph showed multiple air-fluid levels of the small bowel. Computed tomographic scan of the abdomen revealed dilated small bowel loops without mechanical obstruction. Urinary tract involvement was also demonstrated. IPO was diagnosed and the patient responded well to immunosuppressive treatment. IPO is a recently recognized manifestation of SLE that may be the presenting manifestation of the systemic disease or occur more commonly during disease course. Early recognition of IPO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.     

Effect of smoking on neonatal and maternal serum and breast milk leptin levels

Maternal smoking is considered to be a risk factor for low birth weight. It is hypothesized that alteration in leptin concentration may be associated with reduced fetal growth. In this study, we assess the effect of smoking during pregnancy on maternal and neonatal serum leptin concentrations, and also on breast milk leptin levels. When the infants were brought to routine physical examination at 7 days old, blood samples and breast milk specimens were taken for leptin measurement from mothers who smoked during pregnancy and their newborns. Nonsmoking mothers and their infants were recruited randomly over the same period as a control group. Maternal age, number of pregnancy, weight of the mothers, birth weight, and gestational age of the infants were similar in both groups (p > 0.05). There was no significant difference between groups in maternal serum and breast milk leptin levels (p = 0.14 and p = 0.96, respectively). However, serum leptin levels were found significantly lower in neonates born to smoking mothers compared with infants born to nonsmoking mothers (p = 0.02). Our findings suggest that maternal smoking dose not have an effect on maternal serum and breast milk leptin levels but decreases neonatal serum leptin concentration independent of birth weight.     

Antiproliferative and antitumor effects of alpha-interferon in renal cell carcinomas: correlation with the expression of a kidney-associated differentiation glycoprotein

Human leukocyte alpha-interferon (IFN-alpha) has significant antitumor activity in advanced renal cell carcinoma (RC), with approximately 15% (range, 5 to 29%) of patients subjected to IFN-alpha therapy exhibiting a major objective response. We assayed 16 RC cell lines for intrinsic sensitivity to the growth-inhibitory effects of recombinant IFN-alpha. Similar to results observed in patients, cultured RCs could be divided into those that are inhibited by IFN-alpha and those that are not. In addition, the IFN-alpha-sensitive or -resistant phenotype of cultured RCs was correlated with surface expression of six unrelated kidney-associated differentiation antigens. The expression of one antigen, a Mr 160,000 glycoprotein (gp160), was found to correlate with resistance to IFN-alpha. Proliferation of seven RC cell lines expressing gp160 (gp160+) was not significantly inhibited by IFN-alpha at concentrations as high as 3000 units/ml. In contrast, proliferation of eight of nine RC cell lines lacking expression of gp160 (gp160-) was markedly inhibited by IFN-alpha. The effect of IFN-alpha on gp160+ and gp160- RC xenografts in nu/nu mice was examined. In separate experiments, two gp160+ RC cell lines and five gp160- RC cell lines were injected s.c. into nu/nu mice; one half of the mice were subsequently treated with 10(6) units of IFN-alpha i.p. 3 times a wk, and one half received no IFN-alpha. Tumors appeared at the sites of inoculation in all mice given injections of gp160+ RC cell lines within 10 to 25 days regardless of INF-alpha therapy. Mice given injections of gp160- RC cell lines, but not receiving IFN-alpha, also formed tumors. In contrast, gp160- RC cell lines injected into mice that were treated with IFN-alpha exhibited a marked sensitivity, as demonstrated by either no tumor formation or delayed tumor formation. We conclude that the absence of gp160 expression by RCs may be predictive of sensitivity to the antitumor effects of IFN-alpha and, thus, provide a basis for identifying IFN-responsive patients.