Frontometaphyseal dysplasia--evidence for X-linked inheritance.

Fewer than two dozen cases of frontometaphyseal dysplasia have been reported, some doubly or erroneously. In most reports, no information is available on possible variable manifestations in female relatives. Statements that the disorder is inherited as a dominant trait, and as an X-linked recessive have caused us to consider genetic heterogeneity.

Rebound hemodynamic events after the abrupt withdrawal of nitroprusside in patients with severe chronic heart failure.

We studied the hemodynamic events that followed abrupt withdrawal of nitroprusside in 20 patients with severe chronic heart failure. With nitroprusside, cardiac index increased from 1.96 to 2.

Differences in hemodynamic effects of nitroprusside and prazosin in severe chronic congestive heart failure: evidence for a direct negative chronotropic effect of prazosin.

To compare the hemodynamic effects of prazosin and nitroprusside in patients with severe congestive heart failure, nine patients with heart failure refractory to conventional therapy received oral prazosin and intravenous nitroprusside administered so as to produce a similar decrease in left ventricular filling pressure in each patient. By this comparison, both drugs produced similar decreases in mean right atrial pressure, mean pulmonary arterial pressure and systemic and pulmonary vascular resistance. However, with nitroprusside, cardiac index increased more (+0.

Congenital monostotic fibrous dysplasia--a new possibly autosomal recessive disorder.

Two siblings, a 3-month-old white male infant and a 12-day-old female infant, had an anterior mandibular bony lesion that, in both cases, had been present at birth. After evaluation of clinical, physical, radiographic, laboratory, and histologic findings, a diagnosis of congenital monostotic fibrous dysplasia was made. Thorough review of the literature on fibrous dysplasia yielded no similar cases.

Usher's syndrome type III.

We describe a rare example of Usher's syndrome type III in a 9-year-old boy. This type is characterized by retinitis pigmentosa and progressive sensorineural deafness.

Sustained effect of orally administered isosorbide dinitrate on exercise performance of patients with angina pectoris.

The efficacy of oral isosorbide dinitrate was evaluated in nine hospitalized patients with chronic angina pectoris and positive maximal bicycle exercise tests. Patients were randomized double-blind to receive either 20 mg of isosorbide dinitrate or placebo on successive days after a control maximal upright bicycle exercise test. On each day hourly exercise tests were performed for 4 hours after drug administration to an end point of fatigue or angina pectoris.

Melnick-Needles syndrome: radiographic alterations in the mandible.

After the adventitious finding of bilateral loculations in the mandibular rami and erosion of the coronoid processes of the mandible of a patient with Melnick-Needles syndrome (osteodysplasty), the jaws of four other individuals with the same disorder were examined for similar changes. The coronoid process of the mandible was grossly hypoplastic in all patients, and the rami were markedly abbreviated. In four of the five patients the angle was rounded.

Dyssegmental dwarfism. A lethal anisospondylic camptomicromelic dwarfism.

Dyssegment dwarfism is a lethal anisospondylic camptomicromelic form of growth retardation that appears to have autosomal recessive inheritance. It is characterized by short neck, cleft palate, narrow chest, severe shortening of long bones and trunk, reduced joint mobility, inguinal hernia, and probably hirsutism and hydroureter/hydronephrosis. Some cases are seen with occipital exencephalocele.