A closer look at ARSA activity in a patient with metachromatic leukodystrophy.

Metachromatic leukodystrophy (MLD) is an autosomal recessive lysosomal storage disease mainly caused by a deficiency of arylsulfatase A activity. The typical clinical course of patients with the late infantile form includes a regression in motor skills with progression to dysphagia, seizures, hypotonia and death. We present a case of a 4-year-old female with rapidly progressive developmental regression with loss of motor milestones, spasticity and dysphagia. MRI showed volume loss and markedly abnormal deep white matter. Enzymatic testing in one laboratory showed arylsulfatase A activity in their normal range. However, extraction of urine showed a large increase in sulfatide excretion in a second laboratory. Measurement of arylsulfatase A in that laboratory showed a partial decrease in arylsulfatase A activity measured under typical conditions (about 37% of the normal mean). When the concentration of substrate in the assay was lowered to one quarter of that normally used, this individual had activity \u3c10% of controls. The patient was found to be homozygous for an unusual missense mutation in the arylsulfatase A gene confirming the diagnosis of MLD. This case illustrates the importance of careful biochemical and molecular testing for MLD if there is suspicion of this diagnosis

Humeral Metastasis in a case of Squamous Cell Carcinoma - a Case Report

A rare case of squamous cell carcinoma with metastasis to distal acralskeleton – humerus within two months of diagnosis of the primary is being reported. The metastasis to the bones from carcinoma cervix is uncommon especially in the distal appendicular skeleton. A 47 years female came with spontaneous fracture of right humerus shaft. The tissue from fracture site revealed metastasis of squamous cell carcinoma. X-Ray lung and USG abdomen did not show any organ involvement. She was diagnosed assquamous cell carcinoma cervix stage III B two months before and put on radiotherapy and chemotherapy. This is one of the few documented cases of metastasis to humerus without involvement of lung liver or brain within two months of diagnosis of the primary

Microbial infestation in black pepper and its control measures

The information available on nature and extent of microbial contamination in black pepper and various processes developed for their elimination are reviewed. &nbsp

Post-harvest technology of spices: pre-treatments, ouring, cleaning, grading and packing

The post harvest technology of spices comprises of a whole gamut of operations such as pretreatments, chemical treatments, curing and similar operations, drying, cleaning, sorting, grading and packing. These aspects are briefly covered in this review. Wherever applicable the problems involved and the relative advantages and disadvantages of the various processes are indicated. &nbsp

Microbial infestation in black pepper and its control measures

The information available on nature and extent of microbial contamination in black pepper and various processes developed for their elimination are reviewed. &nbsp

Incisional Hernia in a 12-mm Nonbladed Trocar Site Following Laparoscopic Nephrectomy

Non-bladed trocars and radially dilating systems are considered less traumatic to the abdominal wall because they do not incise the fascia itself. Since the fascia is not cut, it has believed that the fascia closes by itself. Consequently, several authors have suggested that closure of the abdominal fascia may be unnecessary when such non-bladed laparoscopic trocars are used. We report of a case in which a port site hernia was diagnosed at the site of a 12 mm non-bladed trocar 11 days after laparoscopic nephrectomy. Although it may be true that in many cases port site closure is unnecessary and does not result in bowel herniation, this case along with a prior report serve as important reminders that port site hernias are possible even in the use of non-bladed or radial dilating systems, and that there exists a number of potential variables that may predispose to herniation and consequently the ability to predict such events in individual patients remains uncertain. As such, we recommend closing 10 mm or larger port sites irrespective of trocar design

Resource bricolage and growth of product and market scope in social enterprises

This research aims to understand how resource bricolage strategy plays a role in the growth of social enterprises in terms of their product and market. Based on interviews with nine social enterprises, our exploratory finding suggests that social enterprises often employ both internal and network resources in the process of making do. We further explore the relationship between the form of resource utilisation and the nature and scope of activities that the social enterprises embark upon, and find that only those relying on both internal and network bricolage are able to expand into new markets utilising newly developed products. We also find that social enterprises relying on only internal resources can reach the same point through incremental improvisation, by first moving towards either product extension or market expansion, before then embarking on the other. This research contributes to the social entrepreneurship literature by enhancing our understanding of the relationship between resource bricolage strategy and growth of social enterprises through product/ market scope in a penurious environment. The findings of this research also have implications for social enterprise managers and policy makers in utilising their resources and responding to environmental opportunities and challenges

Circadian and circannual changes in the testicular function of adult rhesus monkeys

The endocrine and gametogenic status of the testes were studied in 9 healthy adult rhesus monkeys of proven fertility throughout a one-year period. Testosterone levels were estimated by radioimmunoassay in blood samples collected at 4 h intervals over a 24 h period once a month. Semen samples and testicular biopsies were also examined once a month. A well-defined circandian rhythm was evident in serum levels of testosterone. The rhythmicity was less pronounced in February and September. The 24 h mean levels of serum testosterone were high between the months of August to March and low in the months of May to July. All animals did not uniformly respond to electro-ejaculation in April and May. Semen volume and total number of spermatozoa were maximal between September and March and least from April to August. Testicular biopsies indicated that all stages of spermatogenesis were evident between September and March and the spermatogenic activity was less evident between April and August. The contents of Sertoli cells showed a seasonal cyclicity; they were laden with lipid droplets during April to August when spermatogenesis was quiescent and vacuolated during September to March when spermatogenesis was active. These studies indicate that the testing of contraceptive drugs needs to be restricted to months of September to March in male rhesus monkeys otherwise, it is possible that the naturally occurring reproductive quiscence may be attributed to the effect of the drug being tested. The data accrued from the present studies also provide quantitative information on circulating levels of testosterone which could be used as a reference background while evaluating the contraceptive drug-effects in male rhesus monkeys