Leucodystrophies are a group of white matter diseases caused by an abnormality in the formation or maintenance of one or more components of the myelin sheath. The devastating course of the disease coupled with the lack of any defi nitive treatment render them potentially fatal and incurable. Van der Knaap Syndrome, also known as megaloencephalic leucoencephalopathy with subcortical cyst, is a relatively new and rare entity. Although reported sporadically from Europe, Asia and the Agarwal community in India, there is practically no case report from the eastern part of India. We present a case of van der Knaap syndrome in an adult female with some atypical features.

Serous ovarian cancer is the most common malignant ovarian tumour. Traditional management consists of surgical resection with postoperative chemotherapy. Currently neoadjuvant chemotherapy is offered to patients with advanced stage disease. The present study aims to analyse the histomorphological alterations in serous ovarian cancer following neoadjuvant chemotherapy. Correlation of these morphological alterations with survival is also presented here. Serous ovarian cancers from 100 advanced stage cases were included; 50 were treated with pre-surgery chemotherapy. Semi-quantitative scoring was used to grade the alterations in tumour morphology. Survival data was correlated with the final morphological score. Tumour morphology was significantly different in cases treated with neoadjuvant chemotherapy (CT group) as compared to cases with upfront surgery. The CT group cases showed more fibrosis, calcification, and infiltration by lymphocytes, plasma cells, foamy and hemosiderin-laden macrophages. The residual tumour cells had degenerative cytoplasmic changes with nuclear atypia. Patients with significant morphological response had a longer median survival, although it did not attain statistical significance in the current study. With the increasing use of neoadjuvant chemotherapy in management, the pathologist needs to be aware of the altered morphological appearance of tumour. Further studies are required to establish a grading system to assess the tissue response which can be helpful in predicting the overall therapeutic outcome and the prognosis of patients.

BACKGROUND/AIMS: Aberrant crypt foci (ACF) are early microscopic lesions of the colonic mucosa, which can be detected by magnified chromoendoscopy. Herein, we have investigated whether ACF identified in different clinical groups can be differentiated based on their characteristics. METHODS: Macroscopically unremarkable mucosal flaps were collected from 270 fresh colectomies and divided into 3 clinical groups: colorectal carcinoma (group A), disease controls having known pre-neoplastic potential (group Bc), and disease controls without risk of carcinoma development (group Bn). Topographic and histologic analysis, immunohistochemistry, and molecular studies (high-resolution melt curve analysis, real-time polymerase chain reaction, and Sanger sequencing) were conducted for certain neoplasia-associated markers. RESULTS: ACF were seen in 107 cases, out of which 72 were left colonic ACF and 35 right colonic ACF (67.2% vs. 32.7%, P=0.02). The overall density of left colonic ACF was 0.97/cm, which was greater than the right colonic ACF density of 0.81/cm. Hypercrinia was present in 41 out of 72 left colonic ACF and in 14 out of 35 right colonic ACF (P=0.01). Immunohistochemical expression of p53 was also greater in left colonic ACF than in right colonic ACF (60.5% vs. 38.2%, P=0.03). However, ACF identified among the 3 clinical groups did not show any distinguishing topographic, histological, or genetic changes. CONCLUSIONS: Left colonic ACF appear to be high-risk based on their morphological and prototypic tumor marker signature. ACF identified in different clinical groups do not show significant genotypic or topographic differences. Further detailed genetic studies are required to elucidate them further.
Aberrant Crypt Foci ; Colectomy ; Colon ; Colorectal Neoplasms ; Humans ; Immunohistochemistry ; Mucous Membrane ; Real-Time Polymerase Chain Reaction

The extensive regenerative capacity of hepatocytes and the key roles of the liver in metabolic processes have generated interest in the liver as an appropriate target for cell and gene therapy. If cells were considered as natural biomaterials, then liver cell transplantation would fall within the general field of bioengineering. While unmodified hepatocytes engraft in the liver and ectopic sites, biological modifications and optimization of bioengineered systems would facilitate engraftment and survival of transplanted cells, especially in ectopic locations. Acute liver failure, chronic liver disease and metabolic deficiency states are among the conditions that can potentially be treated by cell transplantation. In acute liver failure, cell transplantation into the liver, along with the creation of an extrahepatic reservoir of cells might be required because engraftment and proliferation of transplanted cells in the liver needs time. In other situations, gradual liver repopulation alone might well be effective without additional manipulations.
Animal ; Biocompatible Materials* ; Biomedical Engineering* ; Hepatocytes/transplantation* ; Human ; Liver*

AIMTo evaluate the possible antifertility activity of Sarcostemma acidum (Roxb) Voigt. stem extract in male rats.

METHODMale rats were given 70% methanol extract of S. acidum stem orally at dose levels of 50 and 100 mg/kg/day for 60 days. Fertility was evaluated with mating test. Sperm motility and sperm density in cauda epididymides were also assessed. Biochemical and histological analyses were performed on blood samples and on the reproductive organs.

RESULTSS. acidum stem extract resulted in an arrest of spermatogenesis without any systemic side effect. Sperm motility as well as sperm density was reduced significantly. Treatment caused a 80% reduction in fertility at the 50 mg dose and complete suppression of fertility at the 100 mg dose. There was no significant change in RBC and WBC count, hemoglobin, haematocrit, sugar and urea in the whole blood and cholesterol, protein and phospholipid in the serum. The protein and glycogen content of the testes, fructose in the seminal vesicle and protein in epididymides were significantly decreased. Cholesterol in the testes was elevated. Treatment at both of the doses caused a marked reduction in the number of primary spermatocytes (preleptotene and pachytene), secondary spermatocytes and spermatids. The number of mature Leydig cells was decreased, and degenerating Leydig cells was increased proportionately.

CONCLUSIONS. acidum stem extract arrests spermatogenesis in male rats without noticable side effects.

Animals ; Blood Cell Count ; Body Weight ; drug effects ; Cholesterol ; metabolism ; Contraceptive Agents, Male ; pharmacology ; Glycogen ; metabolism ; Leydig Cells ; drug effects ; metabolism ; Male ; Phospholipids ; blood ; Plant Extracts ; pharmacology ; Plants, Medicinal ; Rats ; Rats, Sprague-Dawley ; Spermatids ; drug effects ; Spermatocytes ; drug effects ; Spermatogenesis ; drug effects

Isolated trochlea fracture in adults is a rare surgical entity as compared to its capitellar counterpart. It has been only mentioned sporadically in the literature as case reports. Fracture of the trochlea is accompanied by other elbow injuries like elbow dislocation, capitellum fracture, ulnar fracture and extraarticular condylar fracture. Here we report a unique case of isolated displaced trochlea fracture associated with fractures of the lateral end clavicle and the distal end radius. We propose a unique mechanism for this rare combination of injuries: typical triad of injury, i.e. fracture of the distal end radius with trochlea and fracture of the lateral end of the clavicle. Nonoperative treatment is recommended for undisplaced humeral trochlea fractures; but for displaced ones, anatomical reduction and internal fixation are essential to maintain the congruous trochlea-coronoid articulation and hence to maintain the intrinsic stability of the elbow.
Accidents, Traffic ; Adolescent ; Clavicle ; diagnostic imaging ; injuries ; Fracture Fixation, Internal ; methods ; Humans ; Humeral Fractures ; diagnostic imaging ; surgery ; Male ; Radiography ; Radius Fractures ; diagnostic imaging ; surgery

OBJECTIVETo investigate the effect of Lead (Pb) acetate exposure on Semliki forest virus (SFV) pathogenesis in mice.

METHODSDifferent doses (62.5, 125, 250 and 500 mg/Kg body weight) of Pb dissolved in normal saline were given to mice by oral intubation in a sub-acute (28 days) and sub-chronic (90 days) regimen followed by SFV infection. Morbidity, mortality, clinical symptoms, mean survival time (MST), changes in body and organ weight, accumulation of lead in soft tissues, virus titre in brain and histopathological alterations were compared between lead exposed and infected groups.

RESULTSEarly appearance of virus symptoms, increased mortality, decreased MST, enhanced SFV titre and greater tissue damage were observed in lead exposed-SFV-infected mice.

CONCLUSIONPre-exposure to lead increases the susceptibility of mice towards SFV infection. Further studies are suggested in view of the persistence of lead in the environment and the possibility of infection by microbial pathogens.

Alphavirus Infections ; etiology ; veterinary ; Animals ; Brain ; pathology ; Dose-Response Relationship, Drug ; Kidney ; pathology ; Lead ; administration & dosage ; toxicity ; Liver ; pathology ; Mice ; Semliki forest virus ; pathogenicity

PURPOSEBoth cannulated cancellous screw (CCS) and sliding hip screw (SHS) are used in femoral neck fracture fixations, but which is superior is yet to be determined. This study was aimed to compare the clinicoradiological outcome of femoral neck fracture treated with SHS or CCS in young adults.

METHODSAdults (16e60 years) with femoral neck fracture were divided into Group 1 fixed with SHS and Group 2 fixed with three CCS after closed reduction. Pain relief, functional recovery and postoperative radiographs at 6 weeks, 3 months, 6 months and then yearly for upto 4 years were analyzed.

RESULTSGroup 1 (n=40) achieved radiological union at mean of 7.6 months, with the union rate of 87.5% (n=35), avascular necrosis (AVN) rate of 7.5% (n=3) and mean Harris Hip Score (HHS) of 86.15 at the end of 4 years. In Group 2 (n=45) these parameters were union at 7.1 months, union rate of 82.22% (n=37), AVN rate of 6.67% (n=3) and HHS of 88.65. Comparative results were statistically insignificant.

CONCLUSIONThere is no significant difference in clinicoradiological outcome between the two implants.

Adolescent ; Adult ; Bone Screws ; Female ; Femoral Neck Fractures ; diagnostic imaging ; physiopathology ; surgery ; Humans ; Male ; Middle Aged ; Osteonecrosis ; epidemiology ; Postoperative Complications ; epidemiology ; Prospective Studies ; Recovery of Function ; Young Adult