Chronic recurrent multifocal osteomyelitis associated with chronic inflammatory bowel disease in children.

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare disease of children characterized by aseptic inflammation of the long bones and clavicles. No infectious etiology has been identified, and CRMO has been associated with a number of autoimmune diseases (including Wegener's granulomatosis and psoriasis). The relationship between CRMO and inflammatory bowel disease is poorly described. Through an internet bulletin board subscribed to by 500 pediatric gastroenterologists, we identified six inflammatory bowel disease patients (two with ulcerative colitis, four with Crohn's colitis) with confirmed CRMO. In all cases, onset of the bony lesions preceded the onset of bowel symptoms by as much as five years. Immunosuppressive therapy for the bowel disease generally resulted in improvement of the bone inflammation. Chronic recurrent multifocal osteomyelitis should be considered in any inflammatory bowel disease patient with unexplained bone pain or areas of uptake on bone scan. CRMO may be a rare extraintestinal manifestation of inflammatory bowel disease; alternatively, certain individuals may be genetically predisposed to the development of both diseases.

Apparent life threatening events and infant holding practices.

Two 4 week old infants are reported who suffered apparent life threatening events (ALTE) while being cradled in adult arms. The events, which could be reproduced under controlled circumstances, were not associated with any struggling by the infants. Alteration of infant holding practices in both cases resulted in cessation of the events. A case of cardiorespiratory arrest during breast feeding in an 8 week old infant that was unnoticed by her mother is also described. These cases suggest that certain infants may not respond normally to airway occlusion while being held or nursed, and show that careful study of the events surrounding ALTE may reveal contributing environmental factors. If ALTE occur around feeding time, observation of how caretakers place the infant during and after feeding may be informative.

Atlantoaxial subluxation in children with seronegative enthesopathy and arthropathy syndrome: 2 case reports and a review of the literature.

We describe 2 HLA-B27 positive children with seronegative enthesopathy and arthropathy (SEA) syndrome who developed spontaneous (nontraumatic) atlantoaxial subluxation early in their disease course. Neither child had evidence of spinal cord compression but both had progressive atlantoaxial subluxation in spite of conservative treatment. Both underwent elective posterior cervical (C1-C2) fusion.

Covert video surveillance in Munchausen syndrome by proxy. Ethical compromise or essential technique?

It has been suggested that the use of covert video surveillance in suspected cases of Munchausen syndrome by proxy should be curtailed as it represents a breach of trust between health care workers, parents and children. We present a case of asphyxia induced by a mother, which was discovered by videotaping without consent. Two previous sudden infant deaths in the family over the preceding two years, with unexplained apnoeic episodes in the third child, were considered sufficiently suspicious to justify covert surveillance. Incontrovertible evidence of parentally induced asphyxia was obtained within 24 hours of full-time covert surveillance. Despite initial denial, the mother eventually pleaded guilty to manslaughter of the first infant and to causing grievous bodily harm to the third infant. We believe that alternative techniques, such as parent-child separation, or of videotaping only after informed consent has been obtained, could have compromised the investigation and produced unacceptable delays which would have placed the surviving infant at risk of serious morbidity or of death.

Blood pressure in schoolchildren measured under standardized conditions.

The blood pressures of 6346 children who were between the ages of seven and 17 years were measured under standardized conditions. Blood pressures were similar in prepubertal boys and girls. After puberty, the systolic blood pressures of the girls remained unchanged whereas those of the boys continued to rise. The difference between the fourth and fifth Korotkoff sounds was 2 mm at both the 50th and 95th percentiles. The systolic blood pressure was observed to fall over a 10-min period by 4-9 mmHg at the 50th percentile and 9-15 mmHg at the 95th percentile. Over the same period of time, the diastolic blood pressure was seen to fall by 2-3 mmHg and 3-6 mmHg at the 50th and 95th percentiles, respectively. There was no further fall in blood pressure after 10 min. The conditions and timing of measurement are important in blood-pressure evaluation and may explain the differences in blood pressure that have been reported for different populations.

Decline of anti-p24 antibody precedes antigenaemia as correlate of prognosis in HIV-1 infection.

A cohort of 84 homosexual men presenting with persistent generalized lymphadenopathy (PGL) was studied between March 1982 and March 1987. A progression rate to AIDS of 5% per year was seen in those who were infected with HIV. Certain clinical features and routine laboratory investigations were significantly associated with an increased risk of disease progression, but had only limited predictive value. Two hundred and fifty-two serial sera from 57 patients were analysed for the p24 antigen (Abbott), the principal core protein of HIV and antibody against p24 by direct enzyme-linked immunosorbent assay (ELISA). Patients who remained well retained a high titre of anti-p24 antibody compared with those who progressed to AIDS-related complex (ARC) or AIDS. HIV antigen was detectable in 40% of AIDS patients 2 years before diagnosis, but antigenaemia was preceded by loss of anti-p24 antibody by up to 18 months and preceded AIDS by up to 40 months. ARC patients tended to be negative when tested for both alpha-p24 antibody and antigen, suggesting a transitional state. Analysis of the humoral response against gag proteins appears to correlate closely with clinical status and may be an earlier and more consistent way of predicting disease progression than p24 antigenaemia, or clinical and routine laboratory investigations.

A case of ring 20 chromosome with cardiac and renal anomalies.

A case of ring 20 chromosome is reported. In contrast to the few children documented previously with this problem who had no significant malformations the present case had major malformations of the heart and urinary tract as well as mental retardation and seizures.