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Purpose We investigated the etiology of acute hepatitis in three children with systemic Juvenile Idiopathic Arthritis (sJIA) taking Interleukin-1 receptor antagonist (IL1RA). Methods Laboratory and clinical data for three children with sJIA diagnosed at ages 13 months to 8 years who developed acute hepatitis during treatment with IL1RA were reviewed for evidence of sJIA flare, infection, macrophage activation syndrome (MAS), malignancy, and drug reaction. Results In all patients, hepatitis persisted despite cessation of known hepatotoxic drugs and in absence of known infectious triggers, until discontinuation of IL1RA. Liver biopsies had mixed inflammatory infiltrates with associated hepatocellular injury suggestive of an exogenous trigger. At the time of hepatitis, laboratory data and liver biopsies were not characteristic of MAS. In two patients, transaminitis resolved within one week of discontinuing IL1RA, the third improved dramatically in one month. Conclusions Although sJIA symptoms improved significantly on IL1RA, it appeared that IL1RA contributed to the development of acute hepatitis. Hepatitis possibly occurred as a result of an altered immune response to a typical childhood infection while on IL1RA. Alternatively, hepatitis could have represented an atypical presentation of MAS in patients with sJIA taking IL1RA. Further investigation is warranted to determine how anti-IL1 therapies alter immune responsiveness to exogenous triggers in patients with immune dysfunction such as sJIA. Our patients suggest that close monitoring for hepatic and other toxicities is indicated when treating with IL1RA.

Pernio, or chilblains, is a localized inflam- matory lesion of the skin resulting from an abnormal response to cold. Five cases were seen among adolescent female patients who presented to our rheumatology ser- vice in a pediatric tertiary care center in the winter of 2003 to 2004. All 5 patients were thin (BMI of

Objectives: To determine if serum lactate dehydrogenase levels distinguish patients with malignant neoplasm presenting with musculoskeletal complaints from patients with juvenile rheumatoid arthritis who reported similar symptoms. Design: Retrospective case-comparison study. Setting: Tertiary care, outpatient clinics. Patients: Twelve patients with malignant neoplasms who presented with arthritis or arthralgias and normal complete blood cell counts and blood smears in whom rheumatologic diagnosis was initially made were compared with 24 children with a final diagnosis of juvenile rheumatoid arthritis. The patients with malignant neoplasms all had normal blood counts and elevated sedimentation rates at symptom onset. Interventions: None. Results: Serum lactate dehydrogenase levels were significantly higher in the cancer patients at 2.2 times the high normal values vs 0.8 times high normal for patients with juvenile rheumatoid arthritis (P=.004, MannWhitneyUtest). No significant differences were observed in white blood cell counts, hemoglobin levels, platelet counts, erythrocyte sedimentation rate, or uric acid or aspartate aminotransferase levels at initial evaluation. Conclusion: Serum lactate dehydrogenase values may distinguish patients with malignant neoplasms from those with rheumatic disease early in the course of illness when symptoms and other laboratory values are not helpful. (Arch Pediatr Adolesc Med. 1996;150:70-73)

Data reported here show that a crude cell wall fraction of Listeria monocytogenes (LCWF) and its purified derivative (PF) induce chemotaxis of human polymorphonuclear leukocytes in the presence of normal rabbit serum. In addition, both LCWF and PF can fix rabbit and guinea pig complement. The purified cell wall preparation is more active than LCWF in both chemotaxigenesis and complement fixation, indicating that these activities are due primarily to the nonprotein portion of LCWF. Complement fixation by PF was demonstrated in the presence of ethylene glycol tetraacetic acid (EGTA) and in C4-deficient serum, which is evidence that PF activates complement by the alternative pathway. Therefore, chemotaxigenesis by PF and LCWF seems to involve fixation of complement via the alternative pathway.

As the presenting complaint in 7 per cent of pediatrician visits, pain in the limbs is a common problem in childhood. It is important that the diagnosis be made expeditiously. The authors review the possible organic cause of limb pain, as well as limb pain from conversion reactions and from growing pains, giving special attention to the differential diagnosis so that appropriate treatment for the pain can be initiated.

during the acute phase of her illness, and her renal biopsy showed evidence of electron dense deposits and immune reactions by immunofluorescence. However, DNAase treatment would be expected to release any DNA antibodies that may have been hidden in complexes, and such treatment of our patient's serum did not increase the level of detectable DNA antibodies. It is possible that antibodies to nuclear components other than DNA were involved in immune complex production. Another possibility is ANA absorption by organs such as skin or kidneys? However, organ absorption is related to critical serum tilers and one would expect the ANA test to be positive sometime during the course of the disease. 1~ Finally, antibodies to non-nuclear antigens may be responsible for the described phenomenon. Antibodies to cytoplasmic proteins (e.g., Ro antigen) have been described in patients with ANA negative lupus. 11 These were not tested in our patient. In summary, although the ANA test is generally a useful screening test, ANA-negative systemic lupus erythematosus appears to be a distinct entity in the pediatric age group. In contrast to other reports, SLE associated with a negative ANA test may be clinically severe and rapidly progressive. We wish to emphasize the importance of suspecting this diagnosis on the basis of clinical criteria.

Monocytes cultured from approximately 30% of normal subjects augmented and accelerated the mitogen-induced proliferation of autologous or allogeneic lymphocytes in a co-culture system. Cells depleted of monocytes by passage over a degalan bead column did not enhance the proliferative response of co-cultured cells. Addition of monocytes to purified lymphocyte populations increased and advanced the proliferation in the co-culture. The augmentative effect of monocytes from normal subjects could not be attributed to an increased percentage of monocytes in these individuals. If mitogen was added after a pre-culture interval rather than at the initiation of a culture, monocytes also enhanced the lymphocyte proliferative response.

Large antigen-antibody complexes that are selectively removed from circulation were not selectively removed when injected into the knees of a normal rabbit. The subsequent selective removal of the complexes depended on the influx of neutrophils. Nevertheless, macrophages of the normal synovial lining were able to phagocytize some immune complexes. When antigen and antibodies were administered separately by intravenous or intraarticular routes, little or no soluble complexes were found in synovial fluid, and most of the antigen-antibody union was thought to occur in the synovial tissue.

As senior residents at the Boston Children's Hospital Medical Center, we are glad to give our views of Patient Care Rounds for the interest of Dr. Fields. Unfortunately, but true, today's busy house officer rarely has enough time to consider the environmental, social, and psychological factors attendant to the child's hospitalization. That the traumatic effects of hospitalization, the psychological aspects of hospital care, and the total welfare of the child are important is indisputable. That the organically oriented house officer needs to be reminded of the total needs of the patient is also indisputable.