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Hurler syndrome (MPS-IH) is an autosomal recessive mucopolysaccharide storage disorder caused by deficiency of lysosomal alpha-L-iduronidase (IDU) enzyme activity. This results in accumulation of heparan sulfate and dermatan sulfate substances. Untreated children develop progressive developmental
OBJECTIVE
To describe the pathology of crypt abscesses in the caeca of feral rabbits in the Manawatu region of New Zealand, and to examine the possible relationship between their prevalence and rabbit haemorrhagic disease (RHD) virus (RHDV) infection.
METHODS
During the course of a 3-year study of
A 17-year-old male patient with history of intraventricular haemorrhage in 2007 underwent a brain MRI scan in 2013 owing to headache. Brain MRI scan showed an expansive lesion adjacent to the left lateral ventricle infiltrating the anterior portion of the corpus callosum. After surgery, pathology
Periodic structures with equally spaced radial striations, identified as Liesegang-like rings, were encountered in fine needle aspirates of two patients' hemorrhagic renal/perirenal cysts. The patients, one 60 and the other 39 years old, had acute right-flank pain; both underwent nephrectomy. The
Periodic structures with equally spaced radial striations identified as Liesegang-like rings were seen in two male patients' hemorrhagic perirenal cysts. The patients, one 48 and the other 60 years old, had acute right-flank pain and anemia; both had nephrectomy. The rings, initially believed to
An adult patient with macular cherry-red spots, a gargoyle-like physical appearance, cerebellar ataxia, myoclonus, convulsive seizures, and pyramidal tract signs showed a profound deficiency of beta-galactosidase in liver and brain. Thrombocytopathy of undetermined etiology was evident since
Neurosecretion, PAS-positive mucopolysaccharides and the Nissl substance were studied in the neurons of the rostral, medial and caudal hypothalamus of continuously irradiated ewes. The study was performed on 21 ewes of the Slovak Merino breed at the live weight of 34 kg. The animals were in the
The teratogenic effects of 5-fluoro-2-desoxyuridine on Wistar rat fetuses were studied macroscopically, microscopically and ultrastructurally. In no case did palatal shelf elevation occur, and a palatal shelf elevating force could not be demonstrated in freshly-removed fetuses. The shelves, like the
In the first of a two-part study, the authors review the known biochemical, pharmacological, toxicological, and experimental data concerning chymopapain and the intervertebral disc. They describe the action of this proteolytic enzyme, which apparently disrupts the protein mucopolysaccharide
A pathomorphological examination of a congenital disease - incomplete desmogenesis (ID) - was made in 5 women aged 19-34 years. They had spontaneous ruptures of the major arteries (subclavial, brachial, splenic, gastric). Microscopically, the wall of the ruptured artery contained no or minimal
A 59-year-old female who had been treated for hypothyroidism was admitted with angina pectoris and underwent aorto-coronary bypass grafting. On closing of the sternum, there was unexpected bleeding from the suture line between the vein graft and the ascending aorta. Histological examination of the
Non-traumatic intracranial dissecting aneurysm (IDA) has been recently reported with increasing frequency and is recognized as a possible cause of subarachnoid hemorrhage. However, the pathogenesis of this disease is still unclear. Cystic medial necrosis (CMN) is known to be a cause of aortic
A 56-year-old man presented with a left ciliary body mass, anterior vitreous hemorrhage and a subluxed, cataractous lens. The clinical course included rapid enlargement of the mass, anterior uveitis, cataract progression and secondary glaucoma. Investigation included fluorescein angiography,