Treatment modalities for inherited factor IX deficiency and the implications of inherited bleeding disorders.

Two cases of factor IX deficiency are presented. Therapeutic methods for anticipated abnormal bleeding during dental surgical procedures on these patients are discussed, and two are illustrated. One treatment regimen, plasmapheresis, has not been discussed previously in the dental literature.

Psoriasis. Palatal manifestations and physiologic considerations.

The histologic and clinical criteria for psoriasis involving the palate are discussed. A review of the literature shows the present case to be the fifth well-documented one in the literature. A possible explanation for the rarity of clinical manifestations in the oral cavity is presented.

Inherited factor X deficiency: presentation of a case with etiologic and treatment considerations.

The first documented case of inherited factor X deficiency in the dental literature is presented. Its ascertainment as a result of postoperative surgical complications illustrates the clinician's need to be familiar with the hereditary bleeding diatheses, as treatment is dependent on the underlying etiology of the specific disorder. In the present case treatment included administration of the antifibrinolytic agent epsilon-aminocaproic acid (EACA) and fresh frozen plasma.

DDAVP: review of indications for its use in the treatment of factor VIII deficiency and report of a case.

Classic hemophilia or factor VIII deficiency is a recessive, sex-linked bleeding diathesis. The primary clinical problem is hemorrhage, which can be severe and often life threatening, even in the presence of only minor trauma. In the past this inadequate hemostasis has been treated with transfusions of cryoprecipitate, fresh frozen plasma, or commercially prepared factor VIII concentrate.

Clinical, laboratory, and genetic investigations of hypophosphatasia: support for autosomal dominant inheritance with homozygous lethality.

This article presents detailed clinical and laboratory investigations of six hypophosphatasia kindreds. Serum alkaline phosphatase and urinary phosphoethanolamine comparisons between the affected population and a normal control population demonstrate these parameters routinely identify the heterozygous individual when age and sex variations are accounted for. Using clinical data from the kindred population and a detailed review of the literature, the type and frequency of clinical findings for both the homozygous and heterozygous genotype are enumerated.

Preparation of enriched populations of corticotrophs from goldfish rostral pars distalis.

A mammalian isolated adrenal cell system was validated as a bioassay for goldfish ACTH; the log dose-response curve for the goldfish hormone is parallel to that for synthetic mammalian ACTH1-24, and the two ACTHs induce the same maximum rate of corticosterone production. Using this assay, it was observed that (1) there is a marked and consistent biphasic change in pituitary ACTH content as related to the length of time the fish are held in laboratory aquaria, and (2) the absolute and relative (to tissue wt) ACTH content of the rostral pars distalis is considerably greater than that of the proximal portion of the gland. Using a trypsin technique, isolated pituitary cells were prepared from both (rostral and proximal) portions of the gland; bioassay data indicate that cell suspensions prepared from the rostral pars distalis are enriched with respect to corticotrophs.

Optical engineering and the Optical Society of America.

The role of the Optical Society of America in the changing field of optical engineering is discussed by the vice-chairman of the Technical Council of OSA in an introduction to a group of papers that constitutes a representative sampling of optical engineering in the early Eighties.

Lethal and mild hypophosphatasia in half-sibs.

This report concerns a family showing both the lethal and mild form of hypophosphatasia in half-sibs. In addition, several other paternal family members with the mild form are documented. The lethal form is characterized by extremely low to absent alkaline phosphatase activity in serum with hypomineralization of the skeleton, whereas mildly affected individuals have enzyme levels intermediate between normal and lethal states.

Ultramicro determination of serum triglycerides by bioluminescent assay.

The ultramicro (1-microL samples) assay of serum or plasma triglycerides that we describe here is potentially applicable to 1-nL samples and to isolated cells. This technically simple method involves only three reactions: (a) enzymic hydrolysis with lipase and alpha-chymotrypsin; (b) conversion by glycerol kinase of the liberated glycerol and of adenosine triphosphate added in excess to glycerol-1-phosphate and to adenosine diphosphate; and (c) assay of the residual adenosine triphosphate by the luciferin-luciferase reaction. The assay was optimized with respect to glycerol kinase, buffer, pH, temperature, and adenosine triphosphate.

Buoyancy adaptations in a swim-bladderless Antarctic fish.

The endemic Antarctic teleosts of the suborder Notothenioidei are bottom dwellers. They lack swim bladders, are heavier than seawater, and feed on or near the bottom. The midwaters surrounding the Antarctic continent are productive and underutilized by fishes.

Hepatic ultrastructural specialization in Antarctic fishes.

Compared with those of other verebrate animals, the livers of Antarctic fishes have a unique type of perisinusoidal (Ito) cell. These cells were studied in 9 species with emphasis on Dissostichus mawsoni. Perisinusoidal cells are found in large numbers throughout the liver, have long cytoplasmic arms and, in Dissostichus, contain numerous lipid droplets.