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

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

Fluctuation and flow probes of early-time correlations in relativistic heavy ion collisions

Fluctuation and correlation observables are often measured using multi-particle correlation methods and therefore mutually probe the origins of genuine correlations present in multi-particle distribution functions. We investigate the common influence of correlations arising from the spatially inhomogeneous initial state on multiplicity and momentum fluctuations as well as flow fluctuations. Although these observables reflect different aspects of the initial state, taken together, they can constrain a correlation scale set at the earliest moments of the collision. We calculate both the correlation scale in an initial stage Glasma flux tube picture and the modification to these correlations from later stage hydrodynamic flow and find quantitative agreement with experimental measurements over a range of collision systems and energies.Comment: Proceedings of the 28th Winter Workshop on Nuclear Dynamics, Dorado del Mar, Puerto Rico, April 7-14, 201

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

oai:ojs.updatepublishing.com:article/4278The 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

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

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