The diagnostic value of anti-cyclic citrullinated peptide (CCP) antibodies in children with Juvenile Idiopathic Arthritis.

BACKGROUND: Antibodies against cyclic citrullinated peptide (anti-CCP) are considered to be specific for rheumatoid arthritis (RA). OBJECTIVE: To assess the clinical significance of anti-CCP antibodies in a cohort of patients with juvenile idiopathic arthritis (JIA) and if they can be used to identify patients with an unfavourable course of disease. METHODS: 68 serum samples were investigated. 45 patients were diagnosed with JIA (15 male and 30 female) aged 1.9-17.3 years (median 12.9 mean 11,0). 5 patients had polyarticular (RF negative), 2 polyarticular (RF positive), 25 oligoarticular JIA, 6 enthesitis related arthritis, 2 psoriatric arthritis, 3 patients had systemic disease and 2 unclassified arthritis. 23 samples were taken from patients with non-inflammatory cardiac diseases undergoing interventional cardiac therapy. Enzyme-linked immunosorbent assay (ELISA; Euroimmun, Lübeck, Germany) was used for the detection and quantification of anti-CCP antibodies in patients with JIA. RESULTS: Overall, anti-CCP antibodies were found in 2.9% (2/68) of all samples and in 4.4 % (2/45) patients with JIA. CONCLUSION: Anti-CCP antibodies are associated with RF positive polyarticular course of JIA. Anti-CCP antibodies are not relevant for other subgroups of JIA. Therefore anti-CCP Abs in patients with JIA should not be investigated routinely.

Gamma-hydroxybutyrate versus chlorprothixene/phenobarbital sedation in children undergoing MRI studies.

BACKGROUND: Few clinical studies have assessed gamma-hydroxybutyrate and chlorprothixene/phenobarbital sedation in children. This prospective trial compared the two regimes in children, in particular concerning differences in recovery time. METHODS: 28 pediatric oncology patients undergoing elective MRI studies at a university hospital were randomly assigned to either receive gamma-hydroxybutyrate or chlorprothixene/phenobarbital sedation. Time to induce deep sedation (Ramsay score of 5) and recovery time, the incidence of failure of sedation, the frequency of side effects, the need for therapeutic interventions, and the number of patients receiving additional midazolam were recorded. Analysis of hemodynamic parameters was performed at five defined time points. RESULTS: All 28 MRI studies were successfully completed. Recovery time was significantly shorter with gamma-hydroxybutyrate (p < 0.01). There were more side effects with chlorprothixene/phenobarbital, in particular tachycardia and hyperexcitation. Vomiting was the side effect most often seen in gamma-hydroxybutyrate sedation. Therapeutic interventions were not required in any patient. Additional midazolam was necessary to maintain satisfactory sedation in six children receiving gamma-hydroxybutyrate and four receiving chlorprothixene/phenobarbital. CONCLUSIONS: Due to its significantly shorter recovery time, gamma-hydroxybutyrate is a reasonable sedative drug for children undergoing non-invasive diagnostic procedures, and is superior to chlorprothixene/phenobarbital. In pediatric oncology patients gamma-hydroxybutyrate appears to be associated more often with vomiting. The long recovery time and its great variability make chlorprothixene/phenobarbital a less valuable alternative.

[Complicated malaria tropica in an infant]. / Komplizierte Malaria tropica bei einem Säugling.

We report on a five month-old infant from Cameroon, who was admitted because of febrile gastroenteritis after living in Germany for two weeks. Since the patient was somnolent, had a parasitaemia of Plasmodium falciparum of 230,000/microliter, a haemoglobin concentration of 5.3 g/dl and a thrombocytopenia, a complicated falciparum malaria was diagnosed. Treatment was started immediately with intravenous 20 mg quinine/kg bw as a loading dose, followed by 10 mg/kg bw every 12 hours, combined with intravenous clindamycin 10 mg/kg bw bd. Red blood cells were transfused once. The parasitaemia dropped to 2000 trophozoites/microliter within 48 hours. No asexual stages were detectable from the third day of treatment on. Weekly controls for the following four weeks remained negative. The mortality rate of complicated malaria is 50% in the first year of life, which can be reduced by early treatment. We present this case to draw attention to therapeutic options in infants.

Dose reduction in thorax radiography in simulated neonates with additional filtration and digital luminescence radiography.

PURPOSE: To determine the minimum acceptable radiation dose for an adequate image quality in thorax a.p. radiographs of neonates using mobile X-ray equipment. MATERIAL AND METHODS: The influence of additional filtration (1.0 mm Al + 0.1 mm Cu) on image quality and radiation dose was determined for the speed class 400 screen-film system (SFS) and digital luminescence radiography (DLR) by making radiographs of a test phantom. Conventional and digital thorax a.p. radiographs of a rabbit were produced using various tube current-time products. The quality of the rabbit radiographs was judged by eight radiologists applying image quality criteria according to the German guidelines and the recommendations of the European Community. RESULTS: The added filter resulted in a dose reduction of 39% at 66 kV. DLR gave a further dose reduction of 25% in comparison to the speed class 400 SFS while maintaining adequate image quality, i.e. the radiographs were clinically acceptable with regard to quality criteria. CONCLUSION: The radiation dose resulting from thorax a.p. radiographs of neonates can be reduced by approximately 50% with the use of additional filtration and DLR.

[Ultrasonography of hips in neonates].

For evaluation of the development of the hip joints in young infants, between Jun., 1988 and Oct., 1991, 522 hips of the infants under 4 months of age were studied by ultrasonography based on the method well described by Graf. The results revealed only 48% of the hips of neonatal group was type I (stable type). But this percentage increased with age: 90% in the group under 2 months of age; 97% in the group under 4 months of age. Compared with the findings of other studies in Europe, the development and the stability of the hips of our neonatal group seemed poorer. But this condition improved much within 2 months after birth. So we concluded that, except the high risk group, the ultrasonographic screening approach should be performed after 2 months of age, but not in neonatal period under considering the cost-benefit.

[N-acetyl-beta-glucosaminidase (beta-NAG) in urine: a parameter for early detection of diabetogenic nephropathy in childhood]. / N-Acetyl-beta-Glukoseaminidase (beta-NAG) im Urin: ein Parameter zur Früherfassung der diabetogenen Nephropathie im Kindesalter.

N-Acetyl-beta-D-glucosaminidase (NAG) can be found in kidney in high activity. By determination of this enzyme activity in urine nephropathies can be recognized reliably. We have measured this lysosomal enzyme in the urine (Colorimetric method by Noto) of 206 children suffering from diabetes mellitus to find out whether diabetic nephropathy can be detected as soon as possible. Additional beta 2-microglobulin (ELISA) was measured in urine, which is reabsorbed nearly complete in the tubulus. The several proteins in urine were identified by SDS-PAGE. A high excretion of beta-NAG was found in children and adolescents with a poor metabolic control (elevated HbA1c). There was no correlation between NAG activity, duration of the diabetes and the age of the children. An elevated excretion of beta-2-microglobuline could be found only in seven children with a duration of the diabetes about six years; their HbA1c levels were high. The SDS-PAGE showed a pattern of glomerular protein excretion according to the duration of the diabetes. However there was no significant correlation to the metabolic control. Only in four children we ascertained mixed proteinuria. The increasing levels of urinary NAG may represent the increasing severity of nephrotic damage by diabetes. The estimation of NAG levels in urine would seem to be reliable in the early detection of diabetic angiopathy also and may serve as an index of diabetic control independent of blood sugar levels.

[The interrelationship between the O2-dependent bactericidal mechanism and hyperbilirubinemia of newborn].

In order to investigate the influence of indirect bilirubin to O2-dependent bactericidal mechanism in adult and newborn phagocytes we use the NBT reduction activity of granulocytes and monocytes as index, by infiltrated granulocytes and monocytes in different concentration of indirect bilirubin with or without latex stimulator. The NBT reduction activity of granulocytes infiltrated in 20 mg/dl indirect bilirubin will increase slightly, compared to uninfiltrated; but with latex stimulator, the increment will decrease significantly, this change is reversible, when indirect bilirubin be washed, the stimulating capacity by latex stimulator in granulocyte will be stronger; there were no such change pattern in monocytes. Since there were a big difference in NBT reduction activity of granulocytes and monocytes between newborns, with or without latex stimulator, we can't get a satisfied result. Indirect bilirubin in cells will increase NBT reduction activity of monocytes, but not in granulocytes.

[Value of serum sialic acid determination in children]. / Wertigkeit der Sialinsäurebestimmung im Serum bei Kindern.

In order to find out whether sialic acid (SA) is suitable as a tumor marker this compound was determined in the sera of 48 healthy and of 168 sick children. In healthy children under six months of age lower concentrations of SA were found (x = 41 +/- 5 mg/dl) than in subjects aged six months to 18 years (x = 68 +/- 8 mg/dl). Inflammatory diseases of various etiologies lead to a significant increase in both age groups (x = 93 +/- 28 mg/dl), the same could be observed in subjects up to 14 days after surgery. In children with malignant disorders the concentration of SA was significantly higher (x = 83 +/- 27 mg/dl) than in the healthy subjects, but not higher than in patients with inflammatory diseases. We conclude that SA might play a role in the follow up protocol of patients with malignant tumors.

Retinal blood flow in diabetic children and adolescents.

A total of 209 diabetic children and adolescents aged 6-17 years (mean, 12.6 +/- 2.3 years) were examined by ophthalmoscopy, video fluorescein angiography and hemoglobin (HbA1c) test. Microaneurysms were found in 26% of the children (ophthalmoscopy, 11%; angiography, 23%). The prevalence of retinopathy was 16% in patients aged 6-10 years, 27% in those aged 11-13 years and 36% in those aged 14-17 years. Only five diabetics aged 11-17 years showed lesions other than microaneurysms (hard exudates, intraretinal hemorrhages or retinal capillar leakage). Retinal blood flow was quantified using video fluorescein angiography. The arm-retina time (ART) and the arteriovenous passage time (AVP) as parameters of the retinal microcirculation were obtained using an image analysing system. The mean value for ART was 9.7 +/- 2.5 s and that for AVP was 1.43 +/- 0.52 s. AVP was significantly shorter in diabetic children and adolescents with good glycemic control (HbA1c, less than 7%; AVP, 1.35 +/- 0.44 s) than in those with bad glycemic control (HbA1c, greater than or equal to 9%; AVP, 1.65 +/- 0.51 s).

[Clinical evaluation of a test strip for measuring creatinine in capillary blood of children]. / Klinische Erprobung eines Teststreifens zur Messung des Kreatinins im Kapillarblut von Kindern.

Basing on an enzymatic method for determining creatinine, the new reagent strip "Reflotron Creatinin" of the Reflotron system recently was presented. In this study, it will be compared with another enzymatic test (Creatinin-PAP) and with a Jaffe-based assay (Creatinin-Jaffe for Hitachi 737). The quality control (within-days and within-series) yielded a sufficient accuracy and precision (CV = 4%) for the charge of used test strips. The recovery of double measured values was very good (r greater than 0.94). As regards the method comparison, differences in both charges were seen. The values of the former charge lay 7% on an average below the Jaffe-values, those of the latter 28% below. The slope of the latter regression line was considerably steeper. Comparing both enzymatic tests, the regression analysis gave poor results (y = -0.33 + 1.35 x). Bilirubin had not remarkable influences, whereas an evelated hematocrit slightly depressed the values. It seems necessary to start further studies to clear up the difference in test strip charges and to check more capillary blood samples of newborn and children with a pathologic creatinine level.

[The effect of methotrexate pharmacokinetics and of leucovorin rescue on the prognosis of osteosarcoma]. / Einfluss der Methotrexatpharmakokinetik und des Leukovorinrescues auf die Prognose des Osteosarkoms.

A total of 129 high-dosage methotrexate therapies performed in 19 patients with osteosarcoma were retrospectively analyzed. Serum methotrexate peak concentrations were found to vary widely, both inter-individually as well as in the same patient. The measured MTX peak concentrations correlated closely with pharmacokinetic data such as area under the curve and total body clearance. No correlations were found between the serum MTX correlations and different times after methotrexate administration. Increase in leucovorin rescue or low MTX peak concentrations were associated with poor prognosis. High-dosage methotrexate therapies with leucovorin rescue need to be further optimized in accordance with biochemical knowledge of the mode of action and the individual pharmacokinetic data of methotrexate. Such optimization may be expected to improve the prognosis for osteosarcoma. Serum methotrexate concentrations should be determined not only 24, 48, and 72 hours after methotrexate administration, in order to avoid elevated toxicity of the therapy, but also at the start of methotrexate infusion, in order to influence MTX peak concentrations at an early stage if necessary. Measurement of L-leucovorin in serum will be necessary, to enable a restrictive leucovorin rescue to be performed safely.

Bare lymphocyte syndrome--combined immunodeficiency and neutrophil dysfunction.

A 4-year-old girl presented with recurrent infections. Immunoglobulin deficiency (serum and secretory IgA, serum IgG3) neutropenia and neutrophil dysfunction (defective spontaneous migration and chemotaxis) were found. T-lymphocyte counts were normal and they responded to phytohaemagglutinin but were not stimulated by Concanavalin A, pokeweed mitogen and microbial antigens in vitro. Delayed cutaneous hypersensitivity testing to purified protein derivative and candidin was negative. Despite bacille Calmette-Guérm vaccination and candidiasis, near normal beta-2-micro-globulin and human leucocyte antigen (HLA) class I concentrations were detected on mononuclear cells and phytohaemagglutinin-induced lymphoblasts. HLA class II antigens (HLA-DP, -DQ, -DR) were not expressed. These observations indicated a bare lymphocyte syndrome (BLS) type II. This is the first time neutrophil dysfunction has been noted in association with BLS.

[Intraspinal, extradural hemorrhage in a 7-year-old boy with hemophilia B]. / Intraspinale, extradurale Blutung bei einem 7jährigen Jungen mit Hämophilie B.

A 7-year-old boy with severe hemophilia B suffered an intraspinal extradural hemorrhage without preceding trauma. Since the age of 3 years the patient is HIV-1 positive. The actual hemorrhage caused a strong pain in the thoracolumbar area without signs of spinal cord compression. It was diagnosed by CT-scan. By early high dose factor-IX substitution the hemorrhage was stopped and no neurological complications occurred. The further substitution regime is described.