Diverticulum hypopharyngis lateralis with a record of one case.

A case of lateral hypopharyngeal diverticula is reported. This type of diverticula is comparatively rare. The diagnosis can be made clinically and confirmed by x-ray examination and hypopharyngoscopy. A pharyngocoele is of differential importance as this may be present in many patients during Valsalva, but does not indicate treatment. The reported case is most probably caused by a development defect in the third branchial cleft.     

The susceptibility of muskrats and snowshoe hares to experimental infection with a chlamydial agent

Muskrats (Ondatra zibethicus) and snowshoe hares (Lepus americanus) were exposed experimentally by various routes to a chlamydial agent (designated strain M56) originally isolated during a die-off of muskrats and snowshoe hares which occurred in Saskatchewan during 1961. Both species were susceptible to experimental infection. Whereas M56 was highly lethal for snowshoe hares (18 deaths/19 exposed), it was less virulent for muskrats (6 deaths/20 exposed).The degree of susceptibility of muskrats to induced infections with M56 was influenced by the presence or absence of specific antibodies at the time of exposure. A febrile illness was observed in 11 of 20 muskrats. In the six that died, widespread focal necrosis was found in the liver. Following intraperitoneal or oral exposures, chronic infections were established and the agent was recovered from the brain and the small intestine up to 96 days post-infection. Specific antibodies were found in 11.8% of 127 sera of muskrats trapped form the wild in Saskatchewan, the Canadian Arctic, and Wisconsin.In snowshoe hares, M56 induced an acute, febrile, emaciating illness, and the almost invariable fatal course was short with terminal signs of opisthotonos, convulsions, and hypoglycemia. Snowshoe hares succumbed with intravenous doses of less than ten mouse intracerebral LD(50) of M56. The same syndrome was produced by intravenous, subcutaneous, and oral infections. M56 was found in high titers in all tissues examined. The highest titers were found in the liver and spleen which correlated with the pathology observed. M56 was recovered from female rabbit ticks (Haemaphysalis leporispalustris) engorging on experimentally infected snowshoe hares.     

Focal and segmental glomerular sclerosis (FSGS) in a man and a woman with Fabry's disease

We describe a man and a woman with Fabry's disease. Renal biopsies showed late and early stages respectively of focal and segmental glomerulosclerosis (FSGS) and vascular changes. Clinically the hemizygous patient had advanced renal disease with nephrotic range proteinuria and serum creatinine 122 micromol/l. The female carrier had minimal albuminuria, borderline GFR with a normal serum creatinine, acroparesthesias, moderate fatigue, tinnitus and headache accompanied by ischemic cerebral lesions. Enzyme replacement therapy (ERT) was initiated according to our Fabry protocol, partly due to the renal morphologic findings. We conclude that FSGS and vascular changes may be an early morphologic finding in Fabry's disease, even in patients with subtle albuminuria. The potential role of FSGS as a marker of progressive renal disease in some Fabry patients is discussed. As FSGS and vascular changes obviously may exist across a wide range of clinical presentations and have potential prognostic implications, we suggest that a renal biopsy should be performed prior to enzyme replacement therapy in all adult Fabry patients with proteinuria of various levels. Efforts should be made to develop a scoring system to evaluate potential histologic markers. Protocol biopsies may have therapeutic implications and may provide valuable information in the evaluation of start and dosing of ERT.