Decreasing reticulocyte counts associated with declining post-dose erythropoietin plasma levels in anaemia of prematurity.

UNLABELLED: A prospective sequential, multicentre trial was conducted to determine the association between erythropoietin (EPO) plasma levels and the erythropoietic response to recombinant human erythropoietin (r-HuEPO) during long-term treatment of premature infants. Twenty-nine infants, gestational ages 26-34 weeks and postnatal ages more than 14 days, received 600 IU r-HuEPO per kg per week divided into three doses subcutaneously for haemoglobin levels less than 120 g/l or haematocrit less than 36% over a period of 4 weeks. Eight additional patients were studied for a total of 10 weeks. EPO plasma concentrations and haematologic parameters were measured prior to the onset of treatment and at 2-weekly intervals thereafter. Treatment with r-HuEPO resulted in a median increase in corrected reticulocyte counts of 2.5% (range 0.2-4.6%) above patient's baseline, thereafter a decrease was observed. In the 8 patients followed for 10 weeks reticulocyte counts declined significantly during weeks 6-10 when compared with the first 4 weeks (p < 0.005). Median 72-hour post-dose EPO plasma levels increased significantly (p < 0.0001) to 57.3 mU/ml (range 5.0-160) above patient's baseline after the first injection, but declined progressively thereafter until they approached baseline values at week 10. CONCLUSION: R-HuEPO treatment after the first month was associated with a decrease in post-injection plasma levels and a decrease in erythropoietic response. This decrease in erythropoietin's efficacy and the decline observed in post-dose EPO plasma levels may be causally related.

Subcutaneous recombinant human erythropoietin in children with renal anemia on continuous ambulatory peritoneal dialysis.

Subcutaneous recombinant human erythropoietin (rHuEpo) treatment of renal anemia was performed in four boys and eight girls on CAPD, aged 0.8-12.5 (mean 7.4) years. In contrast to previous studies, our therapeutic goal was not set with a hematocrit of 30% but with full correction of anemia. Following a maximum weekly rHuEpo dosage of median 120 (range 100-240) IU/kg body weight, hematocrit increased in 10 children from 24 (14-29)% within 12 (4-17) weeks to 40.1 (33.5-48.4)%. The weekly increase in hematocrit was 1.27 (0.5-3.1)%. The corrected reticulocyte count increased from 1.3 (0.7-1.8)% to 2.3 (1.4-3.9)% within 4 (2-6) weeks. Eight children fulfilled the protocol; six with an uncomplicated course were able to maintain a hematocrit of 37.1 (35.1-42.7)% with only one sc medication per week of approximately two-thirds of their highest weekly rHuEpo dosage. No serious adverse effect of rHuEpo therapy was observed.

[Acute renal vein thrombosis in children. Early detection with duplex and color-coded Doppler ultrasound]. / Akute Nierenvenenthrombose bei Kindern. Früher Nachweis mit Duplex- und farbkodierter Dopplersonographie.

Two children with clinically suspected renal vein thrombosis were evaluated by duplex Doppler sonography and colour flow imaging. Both cases presented unspecific findings with an enlarged kidney and loss of cortico-medullary delineation on gray-scale ultrasound, but an unusual flow pattern with retrograde plateau-like frequency shifts during diastole. No venous signal could be obtained. By colour flow imaging only the main renal artery and its proximal branches could be visualised with a reverberating oscillation of blood flow. In addition, partial thrombosis of inferior vena cava in one patient and iliac vein thrombosis in the other could be demonstrated. Clinical improvement during fibrinolytic therapy in one case and nephrectomy in the other case confirmed the diagnosis.

Salivary IgA concentration is influenced by the saliva collection method.

Measurement of salivary IgA is useful for the non-invasive assessment of secretory immunity, especially in children and infants. In our study, the influence of three commonly used methods ("spitting", "suction", "Salivette") of saliva collection on the yield of salivary IgA concentration was analysed in 54 samples of salivary secretion collected from six healthy children according to a cross over protocol. Nephelometrically determined IgA concentrations were significantly lower in saliva collected by the Salivette device (mean +/- SEM: 23 +/- 7 mg/l) than in saliva collected by the suction (46 +/- 8 mg/l) or spitting method (48 +/- 8 mg/l). Salivary flow assessed by the spitting method was inversely correlated with salivary IgA concentration. We conclude that salivary IgA assessment is influenced by the saliva collection method, and that studies dealing with this topic should accurately describe the methods used for collecting saliva so that data may be properly compared.

[L-carnitine therapy and myocardial function in children treated with chronic hemodialysis]. / L-Carnitintherapie und Myokardfunktion bei chronisch hämodialysierten Kindern.

Six out of 14 chronically hemodialysed children with significantly decreased serum carnitine levels were substituted with L-carnitine (15-30 mg/kg/day-Biocarn) up to normal and above normal carnitine levels. None of these patients were digitalised. During the time of investigation plasma carnitine levels were investigated monthly and, simultaneously, three echocardiographic parameters in M-mode were quantitatively und qualitatively determined: shortening fraction (SF-%), ratio of left ventricular pre-ejection/ejection time (LVPT/LVET) and velocity of circumferential fibre shortening (Vcf). Carnitine substitution produced measurable changes in echocardiographic parameters, and a significant quantitative improvement in left ventricular function and performance: after 6 months of carnitine substitution a 24% improvement was seen, after 18 months a 44% mean improvement. No side effects of carnitine were observed; the compatibility was good. Beside these positive effects all patients reported decreased dialysis-associated spasms and polyneuropathic symptoms and increased somatic ability.

[First aid for severe burns and scalds in childhood]. / Erstversorgung schwerer Verbrennungen und Verbrühungen im Kindesalter.

A marked increase in the rate of admission of children with burns and scalding has been observed since 1977 at the Paediatric Hospital of Vienna University. The average age of 94 patients admitted between 1982 and 1986 was 2.2 +/- 2.1 years. An analysis of the age group 1 to 2 years revealed that burns with an extent of up to 10% of the body surface were seen in 18% of patients, up to a body surface of 20% in 18%, and more than 30% of the body surface in 5.3%. Among the entire group of 94 patients 32.9% had burns of up to 10% of the body surface, 41.4% of up to 20%. Written information as to primary care at the place of injury or in the nearest hospital was obtainable in only 35 out of 94 patients (38%). 21.1% of all patients had a raised temperature on the day of admission to this hospital as a consequence of a negative fluid balance. However, only 4 patients (4.2%) out of the 94 were transferred because of fever and/or septicaemia. Guidelines are proposed as to primary care at the site of trauma and during transport to hospital on the basis of a survey of the literature.

[Diffuse xanthogranulomatous pyelonephritis in childhood]. / Diffuse xanthogranulomatöse Pyelonephritis im Kindesalter.

Xanthogranulomatous pyelonephritis is a rare disease and rare cause of suppurative proliferative pseudotumor in children. An accurate diagnosis is difficult because of its clinical, radiological, sonographical and pathologic-anatomical similarities to renal tuberculosis. After removing the diseased kidney the prognosis is excellent. A 6-year old boy with xanthogranulomatous pyelonephritis is reported.

Ciclosporin monitoring in kidney-transplanted children: high-performance liquid chromatography versus radioimmunoassay.

Due to large individual differences in absorption, utilization and metabolism of the predominantly used drug ciclosporin (CsA) for selective immunosuppression in kidney graft recipients, therapeutic blood levels (immunosuppression/toxicity) can be maintained only by frequent measurements of the CsA concentrations in blood samples and dosage readjustments. The purpose of the present study was to investigate the usefulness of a high-performance liquid chromatography (HPLC) method for native CsA and a radioimmunoassay (RIA) method for CsA and metabolites measuring simultaneously using both HPLC and RIA and creatinine serum levels (Crea) in whole blood samples from 19 kidney-transplanted children over a 3-year observation period. By comparison of the results of HPLC, RIA and Crea determinations (n = 1,284) we found a highly variable metabolization rate (RIA/HPLC ratio) of 5.25 +/- 2.33 (range 1.37-12.9). No direct correlation was found between changes in RIA/HPLC ratios and kidney function, rejection and infection periods. Dosage/HPLC and dosage/RIA showed no significant correlation. A higher correlation between HPLC, Crea and nephrotoxicity was found than between RIA and Crea. Because of rapid and large variations of CsA metabolization rates in young allograft recipients, we recommend measurements of CsA blood concentrations with any HPLC method specific for unchanged drug, since CsA metabolites detected by RIA, at least those which are most abundant, have less immunosuppressive and toxic effects.

Acute renal transplant rejection in children: assessment by Duplex Doppler sonography.

Over a two year period 74 consecutive Duplex Doppler scans were performed in 23 children with renal allografts and were compared to the Doppler sonographic findings in orthotopic kidneys of 25 age matched healthy controls. The Doppler waveforms of renal arterial flow were analyzed qualitatively assessing systolic and diastolic flow amplitudes, for quantitation the Pourcelot index (PI) was used. There was no variation between the Doppler waveforms in recipients with normal allograft function and healthy controls. In 12 patients with biopsy proven acute rejection a decrease or absence of the diastolic flow amplitude was noted, resulting in increased pulsatility of the Doppler waveform. The mean PI in acute rejection differed significantly from the mean PI in normal allograft function. Duplex Doppler sonography is a useful imaging modality in the differentiation between acute rejection and normal allograft function and should therefore be integrated in the screening of children after renal transplantation.

Neopterin and interferon gamma serum levels in renal allograft recipients.

In the follow-up of children receiving renal allografts the early differential diagnosis of infections and rejection episodes is the main problem. Serum levels of neopterin (N), a pteridine released from stimulated macrophages, was determined by radioimmunoassay. Also interferon-gamma (IF) serum levels, a marker of T lymphocyte activity, were determined with an immunoradiometric assay in 19 kidney-transplanted children. Both, infections and rejection episodes, are accompanied by distinct increases in N. The IF are elevated 1-3 days earlier than N, the median values during infections being significantly (p less than or equal to 0.001) higher than those during rejection crises. The routine measurement of N and IF allow the simple, quick and reliable monitoring of the immune status, which seems to be of a high relevance for the daily monitoring of transplant recipients.

[Ocular findings in hemodialysis and following kidney transplantation in childhood and adolescence]. / Augenbefunde bei Hämodialyse und nach Nierentransplantation im Kindes- und Jugendalter.

Between August 1980 and January 1987, 23 patients undergoing treatment for chronic renal failure underwent eye examinations. Hemodialysis and subsequent kidney transplants were performed in 18 patients; in two patients a kidney transplant was performed alone, and in three others hemodialysis without transplant. The interval between dialysis and transplantation averaged 23.1 months, the mean follow-up after transplantation 20 months. Patients who underwent hemodialysis alone were followed up for periods of two, three and 85 months. The patients' ages when hemodialysis treatment was first instituted ranged from six to 17 years (average 11.8 years). The mean age at the time kidney transplants were performed was 13.6 years (ranging from one to 17 years). Seventeen patients had conjunctival and corneal infiltrations in the area of the palpebral fissure. In two cases infiltrations were confined to the conjunctiva. Four patients had no pathologic changes, in either the cornea or the conjunctiva. Slitlamp examination revealed subcapsular losses of lens transparency in eight patients; these losses were manifested by delicate punctiform and patchy configurations. In nine cases fundus ophthalmoscopy revealed constricted retinal arteries. Within the period of observation all but one of the patients had unchanged vision. The one exception (cystinosis) had reduced visual acuity due to an accumulation of crystalline inclusions in the cornea.

[Primary lymphedema in the nephrotic syndrome: case report]. / Primäres Lymphödem bei nephrotischem Syndrom: Fallbericht.

The aim of this case report is to discuss possible connections between the development of a hypoproteinaemic oedema due to the nephrotic syndrome and the occurrence of lymphoedema. Two patients (a three year-old girl and a seven year-old boy) developed lymphoedema of one leg one year after the onset of the nephrotic syndrome. The case of the six year-old girl is presented. Malignancy was excluded by clinical investigation. Direct lymphography failed to show any peripheral lymph-vessels; indirect lymphography (i.c. infusion of a newly-developed contrast medium) revealed hypoplasia of the peripheral lymph-collectors. The development of lymphoedema 12-18 months after the appearance of the nephrotic syndrome supports the hypothesis that the increase in extravascular fluid, which is caused by a reduced oncotic pressure in the plasma, may trigger off the development of lymphoedema if there is a primary defect of the lymphatic system.

[Urolithiasis in pediatrics: analysis of 34 patients]. / Urolithiasis in der Pädiatrie: Analyse von 34 Patienten.

The increasing incidence of urolithiasis makes it important to report about 34 children with urolithiasis seen between 1976 and 1986 at the Department of Pediatrics, University Medical School Vienna. At the time of the first diagnosis 59 percent of the patients were less than 7 years of age; 62 percent of our patients were males. Recurrent chronic urinary tract infection in 32 percent, metabolic disorder (secondary hyperoxaluria 5, idiopathic hypercalciuria 3, cystinuria 2, hyperuricuria 2) in 27 percent were evaluated; in 13 patients the origin of calculi was idiopathic. Most infectious stones contained magnesium ammonium phosphate, most idiopathic stones calcium oxalate. In 21 patients (62%) surgical treatment, in one patient extracorporal shock wave lithotripsie was realized. Adequate metaphylaxis (general, dietetic, medicementous) can lower the rate of occurrence of stone formation.

Quantitative EEG: investigation in children with end stage renal disease before and after haemodialysis.

Changes in brain function of 9 children (6 males and 3 females) ages 7 to 14 years (mean 12 years) with end stage renal disease (ESRD) were investigated before and after haemodialysis treatment, utilizing computer assisted spectral analysis of the scalp-recorded EEG. A control group of age-matched healthy children was studied as well. Statistical analyses demonstrated that ESRD children exhibited more Delta and Theta activity, less Beta activity, a slower dominant frequency of the Alpha activity as well as a slower centroid of the total activity before treatment than the controls. These findings suggest a deterioration of vigilance as characterized by Head. Haemodialysis decreased slow activity and increased Alpha and Beta activity, thereby inducing an improvement of brain function.

Antibiotic-associated pseudomembranous colitis as cause of intestinal obstruction in a five-day-old newborn.

The case report is presented of a newborn that developed pseudomembranous colitis and intestinal obstruction after antibiotic therapy with cefoxitin and gentamicin in the first week of life. At laparotomy on the fifth day a complete obliteration of the descending colon by pseudomembranes was encountered. After resection of the diseased segment the recovery was uneventful. We believe this case is the first to be published of antibiotic-associated pseudomembranous colitis causing intestinal obstruction.

Hepatitis B vaccination and immune response in children with malignant diseases.

Fifty children with malignant diseases were vaccinated against hepatitis B. Twenty-nine children suffered from leukaemia or non-Hodgkin's lymphoma; 14 of these were on intensive chemotherapy (group I) and 15 were without intensive therapy (group II). The other 21 children had various forms of solid tumours, 14 of them were on intensive therapy (group III) and 7 were without intensive therapy (group IV). To evaluate the immune response, we determined antibody titres over a period of more than 14 weeks after the first vaccination. As 22 out of 50 patients had received passive immunisation together with either the first or the first and second vaccination, antibody titres at the 14th and 18th week (i.e. more than 10 weeks after passive immunisation) were used to evaluate the vaccination results. An antibody titre of greater than or equal to 10 mIU/ml was considered to be a positive response. All patients of group IV, but only 4 out of 14 in group III, 4 out of 15 in group II, and 0 out of 14 in group I produced antibody titres higher than 50 mIU/ml. In contrast to the full response in group IV, two-thirds of all other patients had no immune response (less than 10 mIU/ml). Based on our experience we recommend vaccinating patients suffering from solid tumours and receiving no intensive therapy (group IV) against hepatitis B and protecting all the other children with malignant diseases by passive immunisation, if necessary.

[Associated abnormalities in the VACTERL syndrome--case report with autopsy findings]. / Assoziierte Missbildungen bei einem VACTERL-Syndrom--Fallbericht mit Obduktionsbefunden.

We report on a male newborn with a VACTERL-association. Our patient had the characteristics of the syndrome: vertebral defects, anal atresia, cardiac anomalies, tracheal stenosis and radial dysplasia. In addition to this combination of malformations we have found a left pulmonary artery sling, cholecystaplasia and biliary dysplasia-not previously reported. Etiology, embryology, coincidence and differential diagnosis are discussed.

Abdominal aortic aneurysm in a premature neonate with disseminated candidiasis: ultrasound and angiography.

When using ultrasound for detection of kidney enlargement, we found an acute abdominal aortic aneurysm secondary to aortitis following umbilical artery catheterisation in a premature neonate with systemic candidiasis. Aortography was performed to provide vascular details such as involvement of celiac, renal, iliac and femoral arteries.

[Clinical aspects and physiopathology of fetal left heart hypoplasia. 2 case reports]. / Zur Klinik und Pathophysiologie des fetalen Linksherzhypoplasiesyndroms. 2 Fallberichte.

2 cases of congenital hypoplastic left heart syndrome are presented with regard to clinical signs and pathophysiology. The hypoplastic left heart syndrome consists of a hypoplastic left ventricle and absence or atresia of the aortic valve, associated with severe hypoplasia of the ascending aorta and various degrees of mitral valve hypoplasia. Infants are well developed at birth but are unable to maintain sufficient circulation after physiologic closure of the ductus arteriosus.