Intractable ascites as a manifestation of Wolman’s disease: report of two sibs

Wolman disease (WD) is a rare, inherited, rapidly fatal condition presenting in early infancy. The disease manifests in the first month of life with failure to thrive, vomiting, diarrhea, abdominal distension, hepatosplenomegaly and bilateral adrenal calcification and is nearly always fatal before the age of 1?year. Barring a case report of isolated fetal ascites, there is no report of intractable ascites as the presentation of WD till date. We report two siblings with WD who both had intractable ascites and required therapeutic paracentesis, albumin infusion, and diuretics to control tense ascites. Although rare, WD should be considered in the differential diagnosis of infantile ascites.     

Aqueous colloidal systems of bovine serum albumin and functionalized surface active ionic liquids for material transport

Detailed physicochemical and computational investigation are made to explore different aspects of complexation between bovine serum albumin (BSA) and three structurally different surface active ionic liquids (SAILs), 1-dodecyl-3-methylimidazolium chloride, [C₁₂mim][Cl]; 3-(2-(dodecylamino)-2-oxoethyl)-1-methyl-1H-imidazol-3-ium chloride, [C₁₂Amim][Cl] and 3-methyl-1-dodecyloxy carbonyl methylimidazolium chloride, [C₁₂Emim][Cl]. The interfacial and bulk complexation behavior has been monitored using tensiometry, conductivity, steady-state fluorescence and turbidity measurements. Thermodynamic insights about complexation have been obtained using isothermal titration calorimetry (ITC) measurements whereas molecular docking studies were used to predict the possible binding sites of SAILs on BSA. The information obtained from these studies helped in establishing the formed BSA–SAIL complex as a pH dependent colloidal transport system for controlled transport of a lipophilic dye, Rhodamine 6G (R6G), in aqueous phase, which is supported by confocal laser scanning microscopy (CLSM). In the present work, the effect of functionalization over the alkyl chain of SAILs, modulating the colloidal properties of SAIL–BSA systems, has been explored along with the utilization of these complexes as a pH dependent reversible carrier of lipophilic molecules. It is expected that besides providing basic understanding of colloidal complexes of BSA with SAILs, the present work is expected to be helpful in extending the applications of such colloidal systems for material transport.

Physicochemical and computational investigation of complexation between BSA and SAILs with application in material transport.     

Pulmonary embolectomy with use of pediatric bronchoscope for the detection of residual emboli: an innovative surgical technique

Pulmonary embolectomy in the treatment of massive pulmonary embolus has been in doubt since the introduction of thrombolytic therapy. Recent indications for surgical intervention are- contraindication to thrombolysis, failed medical treatment and severe Right Ventricular (RV) dysfunction. A 54-year male came to us with complaints of palpitation, tachypnea and repeated syncope . Transthoracic echocardiography and Computed Tomography pulmonary angiogram demonstrated- Biatrial mass with right atrial mass extending into right ventricle and main pulmonary artery. Pulmonary embolectomy was performed. Residual emboli of bilateral pulmonary arteries were detected with a fiberoptic pediatric bronchoscope and removed. Bronchoscopic evaluation appears to be safe and useful for direct visual detection of emboli.     

Impact of comorbidity on outcomes and overall survival after open and minimally invasive esophagectomy for locally advanced esophageal cancer

Background

The aim of this study was to examine the impact of the Charlson Comorbidity Index-Grade (CCI-G) on predicting outcomes and overall survival after open and minimally invasive esophagectomy (MIE).

Methods

One hundred and forty-six patients who underwent esophagectomy between 1995 and 2011 for stage II and III cancer were selected and separated into open esophagectomy (Open) and MIE groups. Risk adjustment was performed using the CCI-G. The outcomes of interest were operative time, estimated blood loss (EBL), lymph node harvest, length of hospital stay (LOS), major complications, 30-day mortality, and overall survival.

Results

Sixty-four patients (44 %) underwent Open while 71 (49 %) had MIE. An additional (7 %) were converted and classified with MIE. There was no significant difference between MIE and Open in terms of operative time. MIE had less EBL (mean difference = 234 mL, p < 0.001), higher lymph node harvest (mean = 7.4 nodes, p < 0.001), and shorter LOS (median = 1.5 days, p = 0.02). Atrial arrhythmias were the most frequent complication, occurring in 33 % of patients in both the MIE and the Open group (p = 0.988). Thirty-day mortality was 2 % for MIE and 5 % for Open (p = 0.459). Five-year survival was 41 % for MIE and 33 % for Open (p = 0.513). Operative approach, age, gender, BMI, clinical stage, and neoadjuvant therapy did not have any significant effect on the outcomes or overall survival. CCI-G influenced outcomes with operative time, LOS, cardiovascular complication, and anastomotic leak rate, favoring CCI-G 0 compared to CCI-G 3. Overall survival was worse for CCI-G 1 in comparison with CCI-G 0 [hazard ratio (HR) 1.99, p = 0.027].

Conclusions

MIE is a safe alternative to open esophagectomy for the treatment of locally advanced esophageal cancer. The presence of comorbidities increased operative time, length of hospital stay, and postoperative complications while worsening overall survival.     

Reduction in augmentation index after successful renal transplantation

Background

Patients of end stage renal disease (ESRD) have an increased risk of cardiovascular events. Arterial stiffness is an established independent predictor of cardiovascular morbidity and mortality in ESRD patients. Carotid femoral pulse wave velocity (c-f PWV) and augmentation index (AI) are the indices which are used for the noninvasive assessment of arterial stiffness. Renal transplantation (RT) as a treatment modality in ESRD patients is associated with improvement in cardiovascular survival. Whether this improvement is due to attenuation of arterial stiffness has been inadequately investigated. The present study was conducted in ESRD patients before and 3 months after RT to assess the reversibility of the abnormalities of vascular compliance that are known to be associated with adverse outcome.

Methods

Arterial stiffness indices (c-f PWV and AI) were measured using the principle of applanation tonometry with a SphygmoCor^® CvMS system (Atcor Medicals, Australia) in 23 ESRD patients (age: 35.9 ± 9.3 years) before and 3 months after successful RT.

Results

After transplantation, augmentation index values reduced significantly as compared to their pre-transplant values (27.7 ± 11.3 % vs. 17.1 ± 9.0 %; P < 0.0001), while the carotid femoral pulse wave velocity values did not differ significantly (8.7 ± 2.0 vs. 8.6 ± 3.2 m/s). The augmentation index was correlated with the biochemical parameters of serum creatinine (Pearson r = 0.3628; P = 0.0128) and calcium phosphate product (Pearson r = 0.3868; P = 0.0079).

Conclusions

Restoration of renal function following successful RT is associated with differential effects on the two indices of arterial stiffness. The salient finding of our study is that 3 months after transplantation, functional changes in vasculature lead to a significant reduction in the augmentation index, while the pulse wave velocity may take longer to show an improvement.