Hepatic manifestations in Still's disease

In children, as in adults, Still's disease usually presents with a hectic fever, a characteristic rash and arthralgia or arthritis. Visceral involvement is however classical; the hepatic manifestations were studied with respect to two cases. Biochemical changes are common, often mild: the commonest abnormality is cytolysis. Jaundice is less frequent and hepatic involvement may in exceptional cases be life threatening, usually in cases of serious polyvisceral disease often with disseminated intravascular coagulation. These manifestations may be spontaneous or secondary to salicylate therapy; the anatomical changes are the same; the salicylate would therefore seem rather to unmask and aggravate an underlying hepatic abnormality.     

The contribution of a non-invasive test to clinical care. The impact of echocardiography on diagnosis, management and patient anxiety

We prospectively studied the impact of echocardiography on a cardiologist's diagnosis and management plan and on patient anxiety for 300 consecutive referrals. There was an impact on diagnosis in 90% of patients. Most common was confirmation of diagnosis usually with the addition of information pertinent to management (81%); change of disease category or resolution of diagnostic doubt was uncommon (9%). The consultant cardiologist believed the heart to be normal in 48 patients who did not have any associated disease; none had any echocardiographic abnormality. The cardiologist reported increased diagnostic confidence in 74% of all patients but management changed in only 9%. One-third of all patients reported reduced anxiety when this was an important clinical issue but in less than half of them did the cardiologist consider that echocardiographic information was essential for reassurance. Anxiety was increased in 6%, and in 12% the anxiety response was inconsistent with the test result. The clinical contribution of the test report was most obvious for those patients (30%) in whom this data was required for (a) a decision concerning specific diagnostic or technical intervention (b) a change of management plan which implied obvious or likely health benefit (c) reassurance which was a clinically important issue. The magnitude of this contribution was related to the study indication. The value of echocardiography is obvious when assessing patients for invasive intervention or when proper treatment or adequate reassurance are impeded by diagnostic doubt. However, for many current indications, we need better definition of factors which predict a clinically useful result. In particular, when the aim is to rule-out disease, our results suggest that an expert cardiological opinion would often be more appropriate than an echocardiogram.     

Early changes in hemoglobin and hematocrit levels after packed red cell transfusion in patients with acute anemia

BACKGROUND: Equilibration of hemoglobin concentration after transfusion has been estimated to take about 24 hours, but some studies have shown that earlier measurements reflect steady-state values in persons who have not bled recently. This study was aimed at assessing the changes over time in hemoglobin concentration after transfusion in acutely anemic patients because of recent bleeding. STUDY DESIGN AND METHODS: Thirty-two normovolemic patients recovering from an acute bleeding episode who were no longer thought to be bleeding and who received a 2- unit red cell transfusion were studied. At baseline and 15, 30, 60, and 120 minutes and 24 hours after transfusion, hemoglobin concentration and hematocrit values were measured. RESULTS: The administration of 2 units of packed red cells elicited a 24-hour increase of 22.4 +/− 6.8 g per L in hemoglobin concentration. Hemoglobin values were not different at any of the defined posttransfusion times. Hematocrit levels experienced similar changes over time. Agreement between 15-minute and 24-hour values was excellent, as only 6 percent of patients exhibited a clinically significant difference (> 6 g/L) between the hemoglobin measurements. CONCLUSION: Hemoglobin and hematocrit values rapidly equilibrate after transfusion in normovolemic patients who are recovering from an acute bleeding episode. This fact would allow a rapid assessment of the effects of transfusion and of the recurrence of bleeding in patients remaining at risk.     

L-arginine reverses the antinatriuretic effect of cyclosporin in renal transplant patients

BACKGROUND: Cyclosporin has been shown to facilitate renal vasoconstriction and to have an antinatriuretic effect. The existence of an interference of cyclosporin with the vasodilating properties of endothelium mediated by nitric oxide production could mediate these effects. On the other hand, the infusion of the nitric oxide precursor L-arginine has been shown to induce renal vasodilatation and to facilitate natriuresis in normal volunteers. We have investigated the renal effects of the administration of an infusion of L-arginine in renal transplant patients chronically treated with cyclosporin. To facilitate the analysis of the data the effects of the administration of a similar dose of cyclosporin on renal function during the infusion of a vehicle were also investigated during the administration of a vehicle of L-arginine. DESIGN: Ten male renal transplant patients, chronically treated with cyclosporin and with a stable renal function were studied during 2 consecutive days after the administration of the usual morning dose of cyclosporin. The first day they received an intravenous infusion of vehicle and the second the infusion of graded doses of L-arginine (50, 100, 150 mg/kg/h) during 3 consecutive h. RESULTS: The first day, after cyclosporin administration a significant fall (P < 0.01) was observed in natriuresis and kaliuresis in the absence of changes in renal plasma flow and glomerular filtration rate. After the administration of L-arginine significant (P < 0.01) increases of renal plasma flow, glomerular filtration rate, and natriuresis were seen. The increase in blood levels of cyclosporin after its administration did not differ between days 1 and 2. CONCLUSION: These results indicate that L-arginine facilitates renal vasodilatation and natriuresis in renal transplant patients. Furthermore, the observed increase in sodium excretion could indicate that L-arginine counteracts the antinatriuretic effect of cyclosporin.      

Bone cyst in the cervical spine due to secondary amyloidosis. A case report.

A patient who had been on hemodialysis for 20 years developed an amyloid bone cyst in the cervical spine. The cyst collapsed resulting in neurologic damage to the cervical spinal cord. He was known to have high circulating levels of beta-2-microglobulin.     

The homograft department.

Perfusionists are highly skilled professionals providing essential life support, enabling patients to undergo open heart surgery. Although this is the main function at the Royal Liverpool Children's Hospital, Alder Hey, they also manage a Homograft bank. Also known as a heart valve bank, this is a completely independent branch of the perfusion department.     

Extracranial approaches to the repair of cerebrospinal fluid rhinorrhea.

This paper presents a review of the extracranial evaluation and treatment of cerebrospinal fluid (CSF) rhinorrhea. Diagnosis with attention to a careful history and physical with maneuvers which exacerbate drainage and thorough physical exam along with imaging techniques are discussed. The common etiologies of CSF rhinorrhea including trauma, spontaneous leakage, tumor, and iatrogenic injury are included. Management consists of conservative measures including the avoidance of straining maneuvers which increases intracranial pressure. Periodic drainage of CSF via lumbar puncture or continuous drainage via flow-regulated systems may also be of benefit in attempts of conservative management. Failure of conservative management, constant leakage, pneumocephalus, and recurrent meningitis are indicators for surgical repairs. Ethmoid-cribiform plate region repairs are generally approached by external ethmoidectomy and the development of mucoperiosteal flaps from various donor sites which are then rotated to the leak area to seal the defect. Frontal sinus leaks are usually repaired via an osteoplastic flap technique with direct repair of the dural defect or the use of fascial graft tucked under the bony defect, then obliterated with abdominal fat. CSF rhinorrhea presents a diagnostic and surgical challenge to the otolaryngologist. After diagnosis and localization, operative repair using extracranial approaches is accepted as the initial method of intervention in these cases.     

Diet and disadvantage: observations on infant feeding from an inner city

Background: There are few detailed nutritional studies analysing dietary intakes and weaning practices of inner city infants aged 0–12 months. Pasteurized cow's milk (PCM) is not recommended as a main drink until after 1 year of age, although early usage is still common. Premature introduction of pasteurized cow's milk is associated with increased risk of iron deficiency anaemia. Methods: We therefore prospectively examined the dietary effect of early introduction of pasteurized cow's milk on the nutritional intake of 100 infants (mean age at recruitment 7.8 months), whose mothers had already elected to introduce cow's milk before the recommended age of 12 months in a deprived inner city area. In addition, a retrospective questionnaire on feeding practices and food choices was administered and information on parental education and employment was collected, together with a 3-day dietary diary of weighed intakes at recruitment. Results: The results indicate that weaning practices are handed down from family and friends and are intuitive rather than informed. Over 80% of the infants had intakes of iron, zinc and vitamin D below the reference nutrient intake (RNI) and a further 41% had low vitamin C intakes. Prior to introduction of PCM, there was also misuse of infant formula. Twenty per cent added an extra scoop of powder to the feeds, 10% added milk powder to the bottle before addition of water and 30% used microwave ovens to heat the infant bottle. Conclusion: Feeding practices in a deprived inner city area differed substantially from guidelines and infants were at risk of developing nutrient deficiencies as well as poor feeding practices.