Cardiac manifestations in patients with HLA B27-associated juvenile arthritis.

Cardiac involvement is common in adult patients with the presence of HLA B27 with or without the HLA B27-associated spondyloarthropathy ankylosing spondylitis. Most patients with juvenile spondyloarthropathy, which begins at age 16 or younger, do not have spinal involvement and there are only few reports of cardiac involvement. This study sought to assess the prevalence of carditis in patients with HLA B27-associated juvenile arthritis (B27-JA). In a controlled study, 40 patients with B27-JA, among them only 1 with ankylosing spondylitis, were examined by electrocardiogram and echocardiography with pulsed and color-flow Doppler at rest and at the termination of a bicycle exercise and compared to an age- and sex-matched control group negative for HLA B27. Four patients with B27-JA, and none in the control group, had inflammatory aortic regurgitation. Late diastolic flow velocity was significantly increased in patients with B27-JA at the termination of exercise. HLA B27 is a risk factor for endo-/myocardial damage in patients with B27-JA, even in the presence of only short and mild articular disease. Patients with B27-JA should be screened for the presence of aortic regurgitation.

Radical surgery in a neonate with craniopharyngioma. report of a case.

Ultrasonography revealed a suprasellar tumor in a fetus at 28 weeks of gestation. The male newborn, delivered 10 weeks later, was operated at the age of 17 days, and a craniopharyngioma was completely removed. Intraoperatively, inappropriate secretion of antidiuretic hormone occurred and was followed by diabetes insipidus causing imbalance of fluid and electrolytes. The tumor recurred and was totally removed 1 year later. Further development was uneventful and, at the age of 8 years, the boy is in generally good mental and physical condition except for a left-sided hemiparesis. In contrast to the poor outcome of neonatal craniopharyngioma reviewed in the literature, this case may encourage radical surgery even in the very young.

Perioperative fluid and electrolyte management in children undergoing surgery for craniopharyngioma. A 10-year experience in a single institution.

In a retrospective study, the intra- and early postoperative data of 39 children with 46 operations for craniopharyngioma were analyzed. Diabetes insipidus (DI) occurred in 30 out of 32 cases without preoperative evidence of DI. We observed that all children who did not have a pituitary stalk preserved and 5 out of 7 patients with preserved pituitary stalk developed DI within 18 h of surgery. Short-term inappropriate secretion of antidiuretic hormone (SIADH) occurred in 2 children, but was quickly followed by DI. The time of onset of DI and SIADH did not correlate with sex, age, body weight, location of tumor, or duration or extent of surgery. Parenteral desmopressin was an effective treatment for intra- and postoperative DI. The duration of the clinical effect of desmopressin administration varied in different patients between 4 and 23 h. An approach to the immediate intra- and postoperative management of children with craniopharyngioma is presented.

Aerobic fitness and physical activity in patients with HLA-B27 positive juvenile spondyloarthropathy that is inactive or in remission.

OBJECTIVE: To assess aerobic fitness (AF) and physical activity (PA) in patients with HLA-B27 positive juvenile spondyloarthropathy (JSpA) in remission at the time of testing. METHODS: Twenty-one previous patients (12 male, 9 female), age 6-26 years (median age 17.8), and 21 healthy controls matched for age, sex, height, and weight participated. AF was determined by measuring maximal power and peak oxygen uptake (VO2 peak) during a continuous incremental cycling task. PA was assessed by Seven-Day Recall and Lipid Research Clinic questionnaire. RESULTS: There was only a tendency for a lower maximal power and O2 peak in the 21 patients compared to controls for the total group (median/range: 191/49-306 W vs 219/41-337 W; 2385/998-3962 ml x min(-1) vs 2761/10434221 ml x min(-1); both p < 0.10). However, patients who were older than 18 years had a significantly lower maximal power and VO2 peak than their controls (median/range: 199/126-306 W vs 255/194-337 W; 2785/1570-3962 ml x min(-1) vs 3308/2049-4221 ml x min(-1); both p < 0.05). Many of the older patients had also had arthritis for longer than 2 years and felt limited in their AF or PA. PA was not significantly different between patients and controls. CONCLUSION: These data suggest that some patients have an impairment of their AF long after arthritis has ceased, which is likely attributable to psychological factors. Changes in the heart and skeletal muscle might also contribute to the lower AF. Participation in physical exercise is encouraged especially in older or "anxious" patients with inactive JSpA.

[Quantification of postoperative pulmonary valve insufficiency: severity and clinical symptoms]. / Quantifizierung der postoperativen Pulmonalinsuffizienz: Schweregrad und klinische Symptomatik.

Surgical treatment of pulmonary stenosis may lead to inevitable valvular incompetence. The hemodynamic and clinical significance of post-operative pulmonary insufficiency (PI) is uncertain. In patients presenting cardiomegaly and reduced exercise tolerance reoperation may be considered. However, pulmonary valve replacement remains controversial. In order to elucidate the relevance of PI in the long-term post-operative course, quantitative data of PI were compared with chest radiographs and the physical condition. Thirty-one patients with a mean age of 18.5 years (3-36 years) were studied. Twenty-two patients had surgical correction of Tetralogy of Fallot (TOF) and nine had commissurotomy of pulmonary valve stenosis (PS). Catheterization was performed, on average, 12.9 years (2-29 years) after operation in 68% of cases with an interval of more than 10 years. PI was quantitated by digital roentgen densitometry. Regurgitant fraction (RGF) ranged between 13-61%, according to a right ventricle volume load index (VBI) of 1.15-2.6 (ratio of total to effective stroke volume). Twenty of 31 patients (12 TOF, 8 PS) had PI as the only significant lesion. VBI (1.15-2.6) correlated with the cardio thoracic ratio (0.50-0.64), r = 0.74. All patients were in good physical condition. 11 actively took part in sports: all of the eight patients studied by ergometry met normal conditions. Four patients had antiarrhythmic medication. Eleven of 31 patients had PI and additional abnormalities: tricuspid insufficiency (4), right ventricular pressure overload (5), ventricular septal defect (1), depressed left ventricular function (5), and aortic insufficiency (3).(ABSTRACT TRUNCATED AT 250 WORDS)