Dominantly inherited unilateral terminal transverse defects of the hand (adactylia) in twin sisters and one daughter.

Most previous cases of unilateral terminal transverse defects of the hand have not been familial. Several previously reported cases of apparent autosomal dominant inheritance of such defects have subsequently been reclassified as type B brachydactyly. We report a pair of adult twin women with unilateral terminal transverse defects affecting the left hand in one woman and the right hand in the other woman. The latter woman has one daughter with a unilateral terminal transverse defect affecting the left hand. The hand anomaly is characterized by absence of the terminal portions of digits 2 to 5 with a mildly hypoplastic thumb (adactylia). Tiny nail remnants are evident on the remaining digital stumps, and no soft tissue syndactyly is apparent. At 2 years of age, the daughter has hypoplastic first, fourth, and fifth metacarpals with no ossification of the second or third metacarpals or any of the phalanges. The affected mother has hypoplastic metacarpals for digits 2 to 4 and a vestigial fifth proximal phalanx on the affected hand, with no other phalanges evident by roentgenogram other than those of the thumb. The mother's twin sister has similar findings, except the ossified phalangeal remnant is on her second and third fingers rather than her fifth finger. Doppler flow arterial patterns appeared normal in each hand of affected family members. The other hand and both feet are clinically and radiologically normal in each case, and the family history is negative for any other individuals with limb anomalies. A review of the literature suggests that this family may very well be unique.

Epidemiology of Haemophilus influenzae type B disease among Navajo Indians.

During a 7-year period ending June 30, 1980, the annual incidence of all Haemophilus influenzae type b disease among Navajo children less than 5 years old was 214 per 100,000, and that of H. influenzae meningitis was 152 per 100,000. Eighty-one percent of H. influenzae meningitis occurred in children 12 months of age or younger, and 64 percent clustered in children ages 4 through 8 months. Meningitis accounted for 70 percent of all invasive disease. No epiglottitis was observed. The epidemiology is similar to that in Yupik Eskimos, who have an even higher rate of H. influenzae type b disease than Navajos but are a much smaller population. Mortality from H. influenzae meningitis was low (4 percent) among Navajo children, but neurological sequelae were observed in at least 16 percent of the survivors. This high rate of sequelae may be due in part to clustering of cases in infancy. Among normal Navajo neonates, 79 percent had maternal capsular type b antibody titers greater than or equal to 0.15 micrograms per deciliter (microgram per dl), and the whole group had a geometric mean titer of 0.51 micrograms per dl. By age 4 months, when meningitis cases became frequent, only 14 percent of Navajo infants had antibody titers greater than or equal to 0.15 micrograms per dl. Twelve of 67 asymptomatic infants (18 percent), each monitored every 2 months, had H. influenzae type b or a cross-reacting organism isolated from the pharynx on at least one occasion before they were 9 months old. Active immunization would be theoretically indicated in this population with high H.influenzae type b exposure and disease, but a vaccine would have to confer substantial immunity in very young infants.

Immunogenicity of a Haemophilus influenzae type b vaccine in combination with diphtheria-pertussis-tetanus vaccine in infants.

Seventy-eight Navajo infants (one to two months of age) were randomly assigned to one of two vaccination groups: one group (40 infants) was scheduled to receive three doses of diphtheria-pertussis-tetanus (DPT) vaccine and the other (38 infants) to receive DPT combined with Haemophilus influenzae type b polyribosyl-ribitol phosphate (DPT + PRP vaccine). In the latter vaccine, pertussis antigen served as an adjuvant for PRP. Sixty-seven infants (37 who received DPT vaccine and 30 who received DPT + PRP vaccine) completed the protocol. Local and systemic reactions were equally frequent in the two groups. Fifty percent of the infants who received DPT + PRP vaccine had definite antibody responses to PRP after three doses, and 13% had possible responses. Of the infants who received DPT vaccine, 14% and 8% had definite and possible responses, respectively; three of five infants with definite responses were infected with H influenzae type b or cross-reacting organisms, as determined by pharyngeal cultures. The immune response did not appear to be suppressed by the presence of maternal antibody.

Rhizopus osteomyelitis. A case report and review.

Mucormycosis osteomyelitis has previously been described exclusively in association with contiguous infections of rhinocerebral mucormycosis. In a patient with corticosteroid-dependent neutropenia and anemia osteomyelitis of the femur developed caused by the Mucoraceae Rhizopus. Although a primary focus was not identified, we believe this infection was hematogenous in origin. Mitogen stimulation to phytohemagglutinin (PHA) of the patient's lymphocytes revealed depressed cellullar immunity; however, there was specific response to Rhizopus extract. Treatment with systemic amphotericin B prevented further progression of the infection. A review of mucormycosis osteomyelitis is presented.