A severe autosomal recessive acromesomelic dysplasia,the Hunter-Thompson type,and comparison with the Grebe type

Summary Two siblings with a short-limb dwarfing condition which we call acromesomelic dysplasia, Hunter-Thompson type are reported. Abnormalities are limited to the limbs and limb joints in this severe form of dwarfism. The middle and distal segments of the limbs are most affected. The lower limbs are more affected than the upper. We are aware of one previously published case of this entity reported by A. G. W. Hunter and M. W. Thompson in 1976. Dislocations of the elbows and ankles were present in all three patients and dislocations of the hips and knees in two. One of the siblings who did not have hip and knee dislocations clinically resembled Grebe chondrodysplasia, another severe acromesomelic dwarfing condition. However, radiological analysis suggests that while acromesomelic dysplasia, Hunter-Thompson type and Grebe chondrodysplasia are related, they are not identical. Grebe chondrodysplasia has been established as an autosomal recessive trait. It appears probable that the entity we describe has the same mode of genetic transmission.     

Penetration and proliferation of BHK and HeLa cells into a three-dimensional collagen matrix of bone stroma type. Preliminary results

Some preliminary data regarding the test of penetration and proliferation of BHK and HeLa cells biocompatibility to a three dimensional stroma saturated with phosphorous and calcium salts (a biomaterial replacing bone) are presented. These data suggest that the analysed biomaterial might be used in surgical practices of prosthesis appliance on interfaces of fractured bone.     

Effect of pentobarbital on neuronal inhibitory response evoked in an isolated slab of cat auditory cortex by intracortical stimulation

Neuronal responses of an acutely isolated slab of auditory cortex (area AI) to intracortical electrical stimulation were studied intracellularly in cats anesthetized with pentobarbital. It was found that 77% of responses were primary IPSPs, and allowing for secondary inhibitory responses, an inhibitory response was observed in 92% of neurons. All types of neuronal responses in the slab were short-latency. The maximal response latency did not exceed 5 msec. Neurons responding to stimulation by IPSPs were found at all depths in the slab, with a maximum in layers II–III. Nearly all primary IPSPswere mono- and disynaptic. Pentobarbital increased the duration of individual neuronal inhibitory responses in the isolated slab of auditory cortex without affecting maximal duration of the IPSP. The mechanisms of the effect of pentobarbital on the amplitude and duration of IPSPs are discussed.I. I. Mechnikov Odessa State University. Translated from Neirofiziologiya, Vol. 16, No. 2, pp. 147–152, March–April, 1984.