Progressive immune failure in dyskeratosis congenita. Report of an adult in whom Pneumocystis carinii and fatal disseminated candidiasis developed.

Community-acquired Pneumocystis carinii pneumonia developed in a young adult patient with dyskeratosis congenita. His hospitalization ended fatally with disseminated candidiasis. Evaluation during the admission showed evidence of cellular immune dysfunction as indicated by skin test anergy and absent lymphocyte proliferation in an in vitro mixed lymphocyte culture. Treatment with transfer factor failed to reverse the cutaneous anergy or affect the clinical course. Dyskeratosis congenita is a rare multisystem disorder with prominent dermatologic manifestations; bone marrow failure or malignant neoplasm are common fatal outcomes. Immune system abnormalities are not classically considered a part of the disease complex. Serial evaluation of our patient's condition over several years suggests that depressed immune function, especially of the cellular limb, may evolve as a feature of clinical importance in these patients.

An analysis of 110 serious enterococcal infections. Epidemiology, antibiotic susceptibility, and outcome.

A prospective, observational study of 110 patients with serious infections due to Enterococcus spp. in 6 university and community teaching hospitals in Connecticut was conducted to define the epidemiology of community and nosocomial serious enterococcal infections and to determine risk factors, including antibiotic resistances, that contribute to outcome. Serious community and nosocomial enterococcal infections involved a variety of sites, and antibiotic resistance was common. Types of infection by major organ system were cardiovascular, 54% (catheter-related bacteremia 28%, primary bacteremia 18%, endocarditis 6%, septic thrombophlebitis 1%); intra-abdominal, 13% (including cholangitis, 6%); renal, 13%; skin and soft tissue, 5%; bone and joint, 4%; pleuropulmonary, 4%; central nervous system, 3%; deep surgical wound, 3%; and endometritis, 2%. Sixty-one percent of infections were nosocomial; 48% of these occurred in the intensive care unit. Enterococcus faecium was responsible for 20% of all infections. Antibiotic resistances among the infections included high-level gentamicin resistance (26%), ampicillin resistance (10%), and vancomycin resistance (8%). Clinical cure was achieved in 64% of patients; 6.8% of patients relapsed, 6.8% had recurrence of the infection with a different pathogen, and overall mortality was 23%. Ampicillin resistance and a high acute physiology and chronic health evaluation (APACHE) II score were highly predictive of lack of cure.

Infantile myofibromatosis. Evidence for an autosomal-dominant disorder.

Infantile myofibromatosis is a distinct clinicalpathologic entity occurring primarily in neonates and infants either as a single nodular lesion or as a multicentric form. We have recently studied two cases of this disorder in neonates, and a third involving the father of one of these infants, all documented by biopsy. Evidence in support of an autosomal-dominant mode of inheritance for infantile myofibromatosis and of its potential to recur after a long period of quiescence is presented.

Pelvic cellulitis: a life-threatening complication of hemorrhoidal banding.

Rubber band ligation is a commonly employed office procedure for the eradication of symptomatic internal hemorrhoids. Since 1980 increasingly frequent reports of an often fatal complication--"pelvic cellulitis"--have appeared. Death has been avoided for some of these patients by early recognition and treatment. One such survivor is reported here, in a case report that illustrates the value of early recognition of symptoms and appropriate diagnostic and therapeutic intervention.

The incidence of bacteremia during skin surgery.

The development of transient bacteremia during simple surgical excision of cutaneous neoplasms was studied. Of 35 patients undergoing surgery on eroded, but not clinically infected, cutaneous tumors, one developed a transient bacteremia, whereas none of 15 patients developed bacteremia during surgery on cutaneous neoplasms with intact skin surfaces. The low incidence of bacteremia associated with surgery on eroded skin neoplasms suggests that, for this commonly performed surgery, prophylactic antibiotics be administered perioperatively only to patients with prosthetic heart valves and not to other patients at risk for endocarditis. This recommendation would be in keeping with the American Heart Association guidelines for prophylaxis for other surgical procedures associated with low incidences of transient bacteremia.

Cryptococcal infection of the central nervous system.

The pathogenesis, clinical signs and symptoms, laboratory manifestations, and laboratory diagnosis of cryptococcal infection of the central nervous system are reviewed, as well as the interaction between the organism and the immune system of the host. In addition, based on our own experience and that of others, the therapy and prognosis of cryptococcal meningitis are discussed.

The diagnosis of invasive aspergillosis by an enzyme-linked immunosorbent assay for circulating antigen.

An inhibition enzyme-linked immunosorbent assay (ELISA) capable of detecting 10 ng of aspergillus carbohydrate antigen/ml of serum was developed. When retrospectively applied to the sera of 19 patients with invasive aspergillosis, the ELISA detected antigen in 11 patients. None of 14 healthy controls or 28 patients with a variety of other infections were positive for circulating antigen. A rabbit model of invasive aspergillosis was also developed. Daily fungal cultures of blood were negative in the rabbits, as in human disease, but antigen was detected in increasing amounts up to the time of death. This ELISA is a sensitive, specific, and easily performed assay for circulating aspergillus antigen that should facilitate early diagnosis of invasive aspergillosis, heretofore, seldom made without invasive tests.

Hypocalcemia-induced pustular psoriasis of von Zumbusch. New experience with an old syndrome.

Hypocalcemia occurs in patients with psoriasis vulgaris, pustular psoriasis of von Zumbusch, and impetigo herpetiformis. In most cases hypocalcemia is caused by accompanying hypoalbuminemia, yet reductions in ionized serum calcium concentrations due to hypoparathyroidism or malabsorption have been reported. We report the case of a patient with surgical hypoparathyroidism in whom hypocalcemia precipitated typical pustular psoriasis of von Zumbusch. The psoriasis rapidly cleared on two occasions when the patient's serum calcium was corrected by therapy with oral calcium and vitamin D or its analogues, and reappeared when treatment was discontinued. The patient's psoriasis cleared on a third occasion when her serum calcium level returned to normal with a calcium infusion. Hypocalcemia can precipitate pustular psoriasis of von Zumbusch in susceptible persons. These psoriatic flares are due not to abnormal circulating levels of parathyroid hormone or vitamin D metabolites but to hypocalcemia.

A vaccine consisting of recombinant Borrelia burgdorferi outer-surface protein A to prevent Lyme disease. Recombinant Outer-Surface Protein A Lyme Disease Vaccine Study Consortium.

BACKGROUND: Lyme disease is a multisystem inflammatory disease caused by infection with the tick-borne spirochete Borrelia burgdorferi and is the most common vector-borne infection in the United States. We assessed the efficacy of a recombinant vaccine consisting of outer-surface protein A (OspA) without adjuvant in subjects at risk for Lyme disease. METHODS: For this double-blind trial, 10,305 subjects 18 years of age or older were recruited at 14 sites in areas of the United States where Lyme disease was endemic; the subjects were randomly assigned to receive either placebo (5149 subjects) or 30 microg of OspA vaccine (5156 subjects). The first two injections were administered 1 month apart, and 7515 subjects also received a booster dose at 12 months. The subjects were observed for two seasons during which the risk of transmission of Lyme disease was high. The primary end point was the number of new clinically and serologically confirmed cases of Lyme disease. RESULTS: The efficacy of the vaccine was 68 percent in the first year of the study in the entire population and 92 percent in the second year among the 3745 subjects who received the third injection. The vaccine was well tolerated. There was a higher incidence of mild, self-limited local and systemic reactions in the vaccine group, but only during the seven days after vaccination. There was no significant increase in the frequency of arthritis or neurologic events in vaccine recipients. CONCLUSIONS: In this study, OspA vaccine was safe and effective in the prevention of Lyme disease.