Clinicobiochemical Parameters and Predictors of Liver Disease in Hospitalized Asian Indian Pregnant Women in a Tertiary Care Center in Northern India.

Introduction  During pregnancy, liver dysfunction is more frequent than expected and may require specialized care. For the early diagnosis, it is important to determine if changes in liver physiology may develop into liver disease. Liver disease during pregnancy may require intervention from a hepatologist for adequate monitoring of mother-fetus health outcomes. This study was aimed to evaluate the clinical profile and predictors of maternal mortality in patients with liver diseases among Asian-Indian-females. Methods We conducted a prospective, open-label, consecutive all-comers study of 2,663 pregnant Asian Indian women admitted in the hospital, which included 92 with liver dysfunction. The medical aspects of the pregnancy were then followed prospectively with laboratory and clinical data during the hospital stay and analyzed. The current study was approved by the Institutional Ethical Committee. Results We found that 92 out of 2,663 patients had liver dysfunction with a prevalence of 3.45%. Fifty-four (58.7%) patients had icterus followed by fever in 23 (25.0%), hypertension in 22 (23.9%), central nervous system manifestations in 21 (22.8%), abdominal pain in 19 (20.6%), vomiting in 19 (20.6%), and pruritus in six (6.5%). Predictors of maternal mortality were icterus (p = 0.04), hepatomegaly (p = 0.04), presenting serum-bilirubin greater than 10 milligram% (mg%) (p = 0.008). The most common etiology was acute viral hepatitis (45.6%), followed by a hypertensive disorder of pregnancy (29.3%), acute fatty liver of pregnancy (1.1%), cholestatic jaundice (9.8%), hyperemesis gravidarum (2.2%), septicemic hepatitis (3.3%), dengue immunoglobulin M (IgM), and plasmodium vivax malaria antigen positive in (2.2%) each. Four patients (4.3%) were leptospira IgM reactive and had co-infection with hepatitis E virus. There was one patient (1.1%) with underlying chronic liver disease. Idiopathic liver disease was present in 5.4% of patients. Conclusion Liver disease is relatively common in Indian pregnant women. It is associated with high maternal and perinatal mortality, even in a tertiary referral center. When managing pregnancy in a tertiary care center, for adequate follow-up of the disease and to prevent adverse consequences for mother and child, it is important to discard liver alterations early. For this purpose, liver disease during pregnancy needs early diagnosis for proper management. Furthermore, it is difficult to manage patients with preexisting liver disease, and it may require specialized intervention from a hepatologist and a gastroenterologist.

Pathogenic FBN1 variants in familial thoracic aortic aneurysms and dissections.

Marfan syndrome (MFS) due to mutations in FBN1 is a known cause of thoracic aortic aneurysms and acute aortic dissections (TAAD) associated with pleiotropic manifestations. Genetic predisposition to TAAD can also be inherited in families in the absence of syndromic features, termed familial TAAD (FTAAD), and several causative genes have been identified to date. FBN1 mutations can also be identified in FTAAD families, but the frequency of these mutations has not been established. We performed exome sequencing of 183 FTAAD families and identified pathogenic FBN1 variants in five (2.7%) of these families. We also identified eight additional FBN1 rare variants that could not be unequivocally classified as disease-causing in six families. FBN1 sequencing should be considered in individuals with FTAAD even without significant systemic features of MFS.

Straddling patent foramen ovale thrombus associated with pulmonary embolism managed with thrombolysis.

A straddling patent foramen ovale thrombus protruding into both atria and ventricles is rare. We hereby describe a case of an acutely ill young male with deep Venous Thrombosis and sub massive pulmonary embolism with a large straddling patent foramen ovale thrombus detected on echocardiography and effectively dissolved with chemical lysis.

Analysis of multigenerational families with thoracic aortic aneurysms and dissections due to TGFBR1 or TGFBR2 mutations.

BACKGROUND: Mutations in the transforming growth factor beta receptor type I and II genes (TGFBR1 and TGFBR2) cause Loeys-Dietz syndrome (LDS), characterised by thoracic aortic aneurysms and dissections (TAAD), aneurysms and dissections of other arteries, craniosynostosis, cleft palate/bifid uvula, hypertelorism, congenital heart defects, arterial tortuosity, and mental retardation. TGFBR2 mutations can also cause TAAD in the absence of features of LDS in large multigenerational families, yet only sporadic LDS cases or parent-child pairs with TGFBR1 mutations have been reported to date. METHODS: The authors identified TGFBR1 missense mutations in multigenerational families with TAAD by DNA sequencing. Clinical features of affected individuals were assessed and compared with clinical features of previously described TGFBR2 families. RESULTS: Statistical analyses of the clinical features of the TGFBR1 cohort (n = 30) were compared with clinical features of TGFBR2 cohort (n = 77). Significant differences were identified in clinical presentation and survival based on gender in TGFBR1 families but not in TGFBR2 families. In families with TGFBR1 mutations, men died younger than women based on Kaplan-Meier survival curves. In addition, men presented with TAAD and women often presented with dissections and aneurysms of arteries other than the ascending thoracic aorta. The data also suggest that individuals with TGFBR2 mutations are more likely to dissect at aortic diameters <5.0 cm than individuals with TGFBR1 mutations. CONCLUSION: This study is the first to demonstrate clinical differences between patients with TGFBR1 and TGFBR2 mutations. These differences are important for the clinical management and outcome of vascular diseases in these patients.

Serum IL-6 levels are associated with significant coronary stenosis in cardiovascularly asymptomatic inner-city black adults in the US.

OBJECTIVES AND DESIGN: The objective of this study was to explore whether increased levels of inflammatory cytokines are associated with the risk of clinically silent coronary artery disease. SUBJECTS: Three-hundred-fifty-six black adults aged 25-54 residing in inner city of Baltimore, Maryland, United States were included in this study. METHODS: Sociodemographics were assessed as were lipid profiles, IL-6, tumor necrosis factor-alpha (TNF-alpha), soluble intercellular adhesion molecule-1 (sICAM-1), and high-sensitivity C-reactive protein (hs-CRP) levels. Computed tomography (CT) coronary angiography was performed. RESULTS: Coronary calcification was identified in 22.5 % participants and 14 % had significant (>or=50 %) coronary stenosis. Multiple logistic regression analyses suggested that IL-6 levels were independently associated with the presence of coronary calcification and significant coronary stenosis, while TNF-alpha, sICAM-1 and hs-CRP levels were not. CONCLUSIONS: This study underscores a critical role for IL-6 in atherosclerosis and suggests that IL-6 may be a marker for significant coronary stenosis in cardiovascularly asymptomatic individuals.

Use of seclusion in an English high security hospital.

There is a paucity of information about the use of seclusion in secure settings. The aim of this paper was to establish if any significant trends could be found for its use in a high secure setting. A retrospective descriptive survey of seclusions occurring over a one-year period at Rampton Hospital was utilised. Of the 443 patients within the study sample, 131 (29.6%) were involved in episodes of seclusion. Forty-five per cent of women experienced episodes of seclusion as opposed to 27% of men (p = 0.004). Women were secluded at an average of 11.4 times, mostly following threatening behaviour, whereas males were secluded at an average of 4.3 times (p = 0.03). There were two peak times for initiation of seclusion--10.00 hrs and 17.00 hrs. The survey concluded that women were nearly twice as likely to be secluded as men and averaged three times as many episodes of seclusion. Younger patients were more likely to experience seclusion and spent longer periods of time in seclusion. No statistically significant differences were found between the use of seclusion and ethnicity. The observations related to female patients were particularly interesting and further studies on seclusion are required to inform upon strategies to minimise its use, with specific attention to female patients.

Acanthamoeba encephalitis.

Central nervous system infection with free-living amoebae is rare. We present a fatal case of Acanthamoeba encephalitis in a 63-year-old female from India where acanthamoebae were demonstrated and cultured from CSF. In spite of treatment with amphotericin B, fluconazole and rifampicin the patient did not survive. Amoebic infection should be suspected in a patient of encephalitis of unexplained aetiology as timely diagnosis can lead to a favourable outcome.

Efficacy of cell saver in reducing homologous blood transfusions during OPCAB surgery: a prospective randomized trial.

Despite the refinements in surgical technique, rates of homologous blood transfusion (HBT) in cardiac surgery remain high. The adverse effects of blood transfusion are well documented. Retransfusion of shed mediastinal blood reduces the requirement for HBTs during conventional coronary artery bypass grafting. However, some studies have found that autotransfusion leads to bleeding diathesis and paradoxical increase in blood transfusions. Through this prospective randomized trial, we have studied the safety and efficacy of this modality in patients undergoing off-pump coronary artery bypass grafting (OPCAB). Fifty patients enrolled in the study and 49 fulfilled the study criteria. They were randomly divided into group C (cell saver) and group N (non-cell saver). Whereas the cell saver group received processed shed autologous blood and homologous blood if necessary, the non-saver group was transfused homologous blood only. The threshold for transfusion was haemoglobin of 9 g dL(-1) in both the groups. The cell saver group required significantly less number of HBTs (1.6 +/- 1.2 vs. 2.4 +/- 1.3 units). The incidence of re-exploration was zero in both the groups. The mean mediastinal drainage in both the groups was not significantly different (355 +/- 196 vs. 316 +/- 119.8 mL). The number of patients requiring any blood transfusion however was very high. All the patients in the non-saver group and 20 (83%) of the patients in the saver group received homologous blood. During OPCAB surgery, the use of cell saver reduced the requirement for HBT. Its use is not associated with any clinically significant bleeding diathesis.

PET-CT detection of abdominal recurrence of ovarian cancer: radiologic-surgical correlation.

The diagnosis of recurrent ovarian cancer can be difficult on cross-sectional imaging, and variable sensitivities and specificities have been reported for positron emission tomography (PET). Combined functional and anatomic imaging with PET plus computed tomography (CT) potentially allows for improved detection of tumor masses. We investigated the sensitivity, specificity, and accuracy of PET-CT for the diagnosis of recurrent ovarian cancer. Sixteen women with previously treated ovarian cancer underwent imaging on a combined PET-CT scanner followed by surgery to assess for possible recurrent disease. The fused PET-CT images were retrospectively reviewed for recurrent disease, and the results of PET-CT were compared with the operative notes. Eleven of the 16 patients had recurrent disease at surgery. The sensitivity, specificity, and accuracy of PET-CT for disease detection on a per-patient basis were 72.7%, 40%, and 62.5%, respectively. For cases of malignant adenopathy (n = 7), 100% were detected on PET-CT. For peritoneal lesions no larger than 1 cm (n = 23), 13% were detected on PET-CT. For peritoneal lesions larger than 1 cm (n = 8), 50% were detected on PET-CT. The sensitivity of PET-CT for recurrent ovarian cancer is moderate in patients with low volume disease. A trial involving a larger number of patients with a spectrum of disease volumes is necessary to determine the impact of PET-CT in clinical practice.

Syncope in a middle aged male due to acute rheumatic fever.

Rheumatic fever is a multi system disease which occurs following infection with group A beta hemolytic streptococcus. It is commonest in the age group of 5-15 years but can occur in adults also. First degree atrioventricular block is a common manifestation of acute rheumatic fever and is included in the Jones criteria but Wenckebacks phenomena and complete heart block are relatively rare manifestations of rheumatic fever. Syncope occurring in acute rheumatic fever is also infrequently reported. We report the case of a 38-year-old male with rheumatic carditis who had advanced atrioventricular block which resulted in syncope and required a temporary pacemaker insertion.

Focal levator ani eventrations: detection and characterization by magnetic resonance in patients with pelvic floor dysfunction.

The aim of this study was to assess the frequency and imaging characteristics of focal levator eventrations in patients with pelvic floor dysfunction on magnetic resonance (MRI). A review of 81 dynamic MR pelvic examinations in patients with pelvic floor dysfunction was carried out to detect and characterize focal eventrations in the levator ani muscle. These were defined as muscle outpouchings which made an angle of >180 degrees with the remainder of the muscle and had a depth of >or=1 cm. Of 81 patients 11(13.5%) had focal eventrations in the levator muscle on MRI: bilateral in 2 cases, right in 5 and left in 4. There was protrusion of pelvic viscera into the eventration in 5 cases, fat in 7 and fluid in 1. Focal levator ani muscle abnormalities are not uncommon on MRI in patients with pelvic floor dysfunction. Characterization of levator muscle morphology can be useful as a research tool in this population.

Lipid profile before and after renal transplantation--a longitudinal study.

BACKGROUND: The data on lipid profile in renal transplant recipients from the Indian subcontinent is scant. METHODS: Lipid profile was studied in 30 consecutive patients of end stage renal disease before renal transplantation (0 month) and prospectively posttransplantation at 1, 3, and 6 months. The results were compared with 30, age and sex matched, healthy controls. All the patients received triple immunosuppression (prednisolone, azathioprine and cyclosporine). RESULTS: Pretransplantation, the hypertriglyceridemia and hypercholesterolemia was present in 20% and 7% of the patients and the difference (elevation) in the mean values of various lipid fractions was not significant compared to healthy controls except a fall in HDL (p < .01). After renal transplantation, there was a significant elevation in the mean values of total cholesterol, triglycerides, VLDL, and LDL cholesterol at 1, 3, and 6 months. HDL cholesterol levels remained significantly lower as compared to healthy controls. Although, the mean values of serum triglycerides and cholesterol were significantly higher in diabetic end stage renal disease compared to nondiabetic ESRD, however there was insignificant difference in the lipid profile amongst diabetic and nondiabetic renal allograft recipients. CONCLUSION: Our data shows distinct elevation in the lipids and lipoproteins after renal transplantation and immunosuppressive drugs seem to be the culprit.

Multidetector computed tomographic evaluation of the renal artery.

Successful renal computed tomographic (CT) arteriography requires thin collimation and adequate z-axis coverage. With multidetector CT, 1-mm slices covering the necessary volume are feasible for optimal arterial visualization. In this pictorial essay, we illustrate the usefulness of multidetector CT in evaluating normal arterial anatomy, stenosis, and the renal arteries in aortic disease. The techniques for performing the study and three-dimensional rendering are described.

Magnetic resonance imaging of pelvic organ prolapse.

Magnetic resonance imaging (MRI) of pelvic organ prolapse is technically feasible and has several advantages when compared with fluoroscopic cystoproctography. Organ descent and the supportive structures of the pelvic floor can be assessed with MRI. The role of MRI in evaluating patients with pelvic floor dysfunction is evolving, and there have been many developments in the past few years. The current status of MRI of pelvic organ prolapse is reviewed.

Controversies in female urethral anatomy and their significance for understanding urinary continence: observations and literature review.

To re-examine the anatomy of the female urethra and related structures, three female pelves serially sectioned in sagittal, coronal or transverse planes, and four sets of transverse histological slides of female urethras, were studied. The observations were assembled, rendered as illustrations, and correlated with published works to present an overall explanation of the gross and histological anatomy of the female pelvis and perineum as related to continence. The figures accompanying the text present the anatomy in a series of views in the three anatomical planes. The anatomical relationships of the paraurethral and paravaginal tissues are examined in relation to the conflicting nomenclature applied to these structures. The figures show the spatial relationships within the pelves and perineum that explain their effective function in urinary continence.

Evaluation of the bony pelvis in classic bladder exstrophy by using 3D-CT: further insights.

OBJECTIVES: To provide a complete look at the bony pelvis in children with classic bladder exstrophy: dimensions, orientation, and relationships. METHODS: Three-dimensional computed tomography was used in 6 boys and 1 girl, 5 of whom underwent primary closure and 2 who underwent reclosure at 4 and 8 months. These exstrophy pelves (intrapelvic angles and osseous dimensions) were compared with 26 age and sex-matched controls. RESULTS: The iliac wing angle was 11.4 degrees larger in the classic bladder exstrophy cases. The sacroiliac joint angle was 9.9 degrees more externally rotated in the exstrophy cases. The pelvis was rotated 14.7 degrees in the superoinferior plane in the exstrophy cases. The mean pubic diastasis was 4.2 cm (0.6 cm in controls). The inter-triradiate distance in the patients with classic bladder exstrophy averaged 6.0 cm (4.2 cm in controls). CONCLUSIONS: These new findings provide a better understanding of the bony pelvis, especially its posterior portion, in patients with classic bladder exstrophy. The results of this study revealed the orientation of the sacroiliac joints to be more externally oriented than previously thought and the pelvis to be rotated inferiorly, a previously unknown observation. Both of these factors will be important in the planning of newer osteotomies and pelvic reconstruction.

Complications of endoscopic retrograde cholangiopancreatography: spectrum of abnormalities demonstrated with CT.

Endoscopic retrograde cholangiopancreatography (ERCP) is an invasive procedure that is performed to diagnose and treat pancreatic and biliary disease. In approximately 5%-10% of cases, the procedure itself causes adverse events. Diagnosis and management of ERCP-induced complications are performed with clinical, laboratory, and radiologic procedures. Evaluation of the type and severity of the complication is necessary and is successfully performed with computed tomography (CT). The most common causes of post-ERCP pain are acute pancreatitis and duodenal perforation. In severe pancreatitis, the pancreas is enlarged and enhances heterogeneously at CT. Pancreatic enhancement is diminished in areas of glandular necrosis. In duodenal perforation, CT may reveal extraluminal air or fluid. CT findings of acute duodenal hemorrhage are duodenal wall thickening and a high-attenuation mass in the duodenal wall. In infection, the bile ducts can be dilated and the attenuation of the bile can be increased at CT. Abscesses appear as hypoattenuating masses with enhancing capsules. CT findings of stent migration are an atypical location of the stent and bowel impaction. Other complications of ERCP are those related to endoscopy and include esophageal, liver, and splenic injury.

Dynamic pelvic magnetic resonance imaging and cystocolpoproctography alter surgical management of pelvic floor disorders.

PURPOSE: Pelvic organ prolapse results in a spectrum of progressively disabling disorders. Despite attempts to standardize the clinical examination, a variety of imaging techniques are used. The purpose of this study was to evaluate dynamic pelvic magnetic resonance imaging and dynamic cystocolpoproctography in the surgical management of females with complex pelvic floor disorders. METHODS: Twenty-two patients were identified from The Johns Hopkins Pelvic Floor Disorders Center database who had symptoms of complex pelvic organ prolapse and underwent dynamic magnetic resonance, dynamic cystocolpoproctography, and subsequent multidisciplinary review and operative repair. RESULTS: The mean age of the study group was 58 +/- 13 years, and all patients were Caucasian. Constipation (95.5 percent), urinary incontinence (77.3 percent), complaints of incomplete fecal evacuation (59.1 percent), and bulging vaginal tissues (54.4 percent) were the most common complaints on presentation. All patients had multiple complaints with a median number of 4 symptoms (range, 2-8). Physical examination, dynamic magnetic resonance imaging, and dynamic cystocolpoproctography were concordant for rectocele, enterocele, cystocele, and perineal descent in only 41 percent of patients. Dynamic imaging lead to changes in the initial operative plan in 41 percent of patients. Dynamic magnetic resonance was the only modality that identified levator ani hernias. Dynamic cystocolpoproctography identified sigmoidoceles and internal rectal prolapse more often than physical examination or dynamic magnetic resonance. CONCLUSIONS: Levator ani hernias are often missed by physical examination and traditional fluoroscopic imaging. Dynamic magnetic resonance and cystocolpoproctography are complementary studies to the physical examination that may alter the surgical management of females with complex pelvic floor disorders.

Liver transplantation: preoperative CT evaluation.

Liver transplantation is a successful therapeutic option for patients with chronic liver disease and liver failure in that 1-year survival is greater than 80%. Orthotopic transplantation is usually performed from a cadaveric or living adult donor. The necessary evaluation of recipients and donors prior to transplantation can be successfully performed with computed tomography (CT). CT is useful in determining clinically relevant information for recipients such as size of the caudate lobe, exclusion of advanced hepatocellular carcinoma and other malignancy, patency of the venous system, presence of perihepatic varices, patency of the celiac artery, exclusion of splenic artery aneurysm, and position of iatrogenic venous shunts. CT in living donors may help to determine clinically relevant information about variant hepatic arterial anatomy, source of the artery to segment IV, intraparenchymal anatomy of the hepatic veins and accessory hepatic veins, trifurcation of the portal vein or hepatic duct, liver volume, and fatty change of the parenchyma. Surgical approaches and the imaging findings that influence management are reviewed.

Pelvic floor anatomy in classic bladder exstrophy using 3-dimensional computerized tomography: initial insights.

PURPOSE: We present the pelvic floor anatomy of the major pelvic floor musculature in classic bladder exstrophy, including the levator ani, obturator internus and obturator externus. By improving our knowledge of pelvic floor anatomy we hope to understand better the relationship of the pelvic floor to the bony anatomy as well as the role of osteotomy in changing pelvic floor anatomy to enhance urinary control after surgery. MATERIALS AND METHODS: 3-Dimensional computerized tomography was done in 6 boys and 1 girl, including 5 patients 2 days to 5 months old (mean age 7 months) undergoing primary closure and 2 who were 4 and 8 years old undergoing repeat closure. The pelvic floor musculature, including the levator ani, obturator internus and obturator externus, in these cases was compared to that in 26 age and sex matched controls. RESULTS: The levator ani musculature encompasses a significantly wider area of 9.5 cm.2 in patients with classic bladder exstrophy than in controls. The anterior segment of the levator ani was shorter (1.2 cm.) and the posterior segment of the levator ani was longer (2.5 cm.) than in controls. The degree of divergence of the levator ani in classic exstrophy was significantly more outwardly rotated (38.8 degrees) than controls. In addition, the transverse diameter of the levator hiatus was 2-fold that in our control group and in that of published controls, while the length of the hiatus was 1.3-fold that in normal controls. There was also significant flattening, involving a 31.7 degree decrease in steepness between the right and left halves of the levator ani, of the puborectal sling in classic bladder exstrophy versus controls. Because of these findings, there is more anterior superior rotation in the pelvic floor in exstrophy cases. The obturator internus was more outwardly rotated (15.1 degrees) in exstrophy and the obturator externus also showed more outward rotation (16.9 degrees) than in controls. CONCLUSIONS: This study provides better understanding of the pelvic floor anatomy in classic bladder exstrophy. Significant differences have been documented in the pelvic floor in classic bladder exstrophy cases and controls. Hopefully these differences may have a pivotal role in providing new insight into long-term issues, such as urinary and fecal incontinence, and pelvic organ prolapse, in classic bladder exstrophy.