Resolution of neonatal hypertrophic cardiomyopathy presumed secondary to acquired maternal ribonucleoprotein and smith autoantibodies.

Severe asymmetrical hypertrophic cardiomyopathy without heart block accompanied by neuromuscular hypotonia and feeding difficulties was evident shortly after birth in the second child of a mother with systemic lupus erythematosus who had no indication of gestational diabetes. High-level anti-ribonucleoprotein (RNP) and Smoth (Sm) antibodies arising from transplacental transfer of maternal antibodies were detected in the child's serum. The cardiac abnormalities improved with a commensurate decline in antibody titers. Previously reported cases of neonatal cardiomyopathy with endocardial fibroelastosis have been ascribed to the transplacental transfer of maternal Sjogrens Syndrome (SS) A (Ro) and Sjogrens Syndrome (SS) B (La) antibodies and have been more severe and persistent compared with our patient. We advocate close monitoring of all babies of mothers with systemic autoimmunity for changes in heart rate during pregnancy and signs of heart failure and neuromuscular weakness after delivery.

ISABEL: a web-based differential diagnostic aid for paediatrics: results from an initial performance evaluation.

AIMS: To test the clinical accuracy of a web based differential diagnostic tool (ISABEL) for a set of case histories collected during a two stage evaluation. SETTING: acute paediatric units in two teaching and two district general hospitals in the southeast of England. MATERIALS: sets of summary clinical features from both stages, and the diagnoses expected for these features from stage I (hypothetical cases provided by participating clinicians in August 2000) and final diagnoses for cases in stage II (children presenting to participating acute paediatric units between October and December 2000). MAIN OUTCOME MEASURE: presence of the expected or final diagnosis in the ISABEL output list. RESULTS: A total of 99 hypothetical cases from stage I and 100 real life cases from stage II were included in the study. Cases from stage II covered a range of paediatric specialties (n = 14) and final diagnoses (n = 55). ISABEL displayed the diagnosis expected by the clinician in 90/99 hypothetical cases (91%). In stage II evaluation, ISABEL displayed the final diagnosis in 83/87 real cases (95%). CONCLUSION: ISABEL showed acceptable clinical accuracy in producing the final diagnosis for a variety of real as well as hypothetical case scenarios.

Abdominal pain as an atypical presentation of meningococcaemia.

An atypical presentation of meningococcaemia without purpura poses diagnostic problems. The importance of the identification of shock manifest as delayed capillary refill in two children with meningococcal septicaemia presenting with fever and abdominal pain is discussed. Abdominal pain is an unusual presentation of meningococcal disease.

Depth of insertion of a lumbar puncture needle.

The depth of lumbar puncture needle insertion was recorded in 107 children. Height provided the best guide to the minimum and maximum depths of insertion. Mean depth of insertion (cm) = 0.03 x height of child (cm).

Evaluation of growth hormone (GH) responses to pulsed GH-releasing hormone administration using the MTT-ESTA bioassay.

We compared the immunoactivity of human growth hormone (hGH) with its bioactivity after stimulation of hGH release into the circulation by the administration of growth hormone-releasing hormone [GHRH(1-29)-NH2] according to a pre-determined protocol to four normal adult volunteers. We used the Hybritech immunoradiometric assay to measure the immunoactive GH concentrations. Bioactive GH concentrations were measured using the highly quantitative and precise eluted stain bioassay system (ESTA). The high sample capacity of the ESTA bioassay permitted us to monitor the bioactivities in closely timed sequential samples, and in far greater detail than has previously been possible. Two pulses of GHRH(1-29)-NH2 were administered intravenously to the four adult male volunteers (aged 24-37 years) on a weekly basis over a 4-week period. Two different doses of GHRH(1-29)-NH2 (0.1 and 1.0 micrograms/kg) were tested. These were separated by specified time intervals (60 or 120 min). Responses in the four individuals were variable. However, although the immuno- and bioactivities generally agreed well, there was a systematic and progressive increase in the bioactivity/immunoactivity (B/I) ratios as half of the response peaks were approached. After these peak concentrations, the B/I ratios subsequently returned to values that were close to unity. The enhanced bioactivity of the peak samples from the two volunteers in whom the largest magnitudes of response were observed was found to be labile after long-term storage at -20 degrees C. We suggest that the preferential rise in GH bioactivity, as opposed to immunoactivity, in response to GHRH(1-29)-NH2 was due to progressive changes in the concentrations of isoforms of GH that are not detectable in the Hybritech immunoassay.

Juvenile Krabbe's Leukodystrophy precipitated by influenza A infection.

Juvenile Krabbe's disease, a neurometabolic disorder, presented as an acute demyelinating episode characterised by an abnormal gait. The neurological presentation was preceded by influenza A infection.

Low-dose growth hormone-releasing hormone tests: a dose-response study.

We have evaluated parameters of the serum growth hormone (GH) concentration response to saline and 1-, 10- and 100-micrograms intravenous bolus doses of amide analogue of GH-releasing hormone (GHRH (1-29)NH2) given in random order to 10 adult male volunteers of median body weight 68 (60-90)kg. Compared with saline, both 10- and 100-micrograms GHRH(1-29)NH2 doses (but not 1 microgram) resulted in significant peak GH responses (means and 95% confidence intervals: 24.03 (11.22-51.29) vs 26.09 (16.40-41.50) mU/l, respectively). Although the average rate of serum GH rise was similar after both 10 micrograms (2.05 (1.13-2.97) mU.l-1.min-1) and 100 micrograms of GHRH(1-29)NH2 (1.52 (0.69-2.35) mU.l-1.min-1; ANOVA F = 0.93, p = 0.35), the average rate of serum GH decline after peak GH was slower after the higher dose (10 micrograms vs 100 micrograms: 0.65 (0.40-0.90) vs 0.37 (0.23-0.50) mU.l-1.min-1; ANOVA F = 5.14, p = 0.04), suggesting continued GH secretion. Increasing GHRH(1-29)NH2 doses delayed the time to peak GH (1 microgram: 7.00 (3.50-10.52) min; 10 micrograms: 15.80 (13.62-17.98) min; 100 micrograms: 24.80 (18.40-31.12) min) and serum GH levels were still elevated significantly 2 h after injection of 100 micrograms GHRH(1-29)NH2 compared with other doses (saline: 0.98 (0.48-2.04) mU/l; 1 microgram: 0.68 (0.48-0.93) mU/l; 10 micrograms: 1.07 (0.56-2.04) mU/l; 100 micrograms: 5.01 (2.34-10.86) mU/l; ANOVA F = 11.10, p < 0.001). In a second study we tested five adult male volunteers with lower doses (0.5-10 micrograms) of GHRH(1-29)NH2.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute endocarditis following pneumococcal meningitis.

Acute mitral valve endocarditis, presenting as a new murmur and haematuria, complicated pneumococcal meningitis in a 20-month-old child with a normal heart. Awareness of this rare complication of pneumococcaemia and its early diagnosis, using cross-sectional echocardiography, improves the clinical outcome of a condition associated with a high mortality.

Benign intracranial hypertension after ciprofloxacin administration.

Many drugs, including antibiotics, have been implicated in the aetiology of benign intracranial hypertension. We report the development of benign intracranial hypertension after the use of ciprofloxacin in a teenager with cystic fibrosis.