[Immunological aspects of a child with idiopathic pulmonary hemosiderosis and celiac disease]. / Immunologische Aspekte bei idiopathischer Lungenhämosiderose und Zöliakie.

A case report of a girl aged 3.5 years affected by the extremely rare combination of idiopathic pulmonary haemosiderosis (IPH) and coeliac disease (CD) is presented. It is the 13th such case that has been published over the last 25 years and only the 7th to be reported in a child. We believe that the concurrence of these two diseases is not coincidental, because a gluten-free diet had beneficial effects on the pulmonary symptoms not only in our case but also in other such patients. However, the pathogenetic relation between IPH and CD remains unclear. Although circulating immune complexes were detected in our patient's serum, there was no evidence of their putative damaging effect on the basement membrane of the alveolar capillaries. Furthermore, no IgA deposits could be demonstrated in alveolar basement membranes. Therefore the hypothesis that there is a reaction between IgA reticulin or endomysial antibodies and an alveolar basement membrane antigen with consecutive structural damage is unlikely.

[Predictive value of latex agglutination on Helicobacter pylori in children with recurrent abdominal pain]. / Praediktiver Wert einer Latex-Agglutination auf Helicobacter pylori bei Kindern mit rezidivierenden abdominellen Beschwerden.

In a prospective investigation, a rapid latex test for Helicobacter pylori in the serum (Pyloriset) was carried out in 39 patients with recurrent abdominal pain aged from six to 15 years. The test was positive in 19 patients. All of these children were subjected to gastroduodenoscopy. Seven cases showed a Helicobacter-associated chronic active antrum gastritis, whereas in the remaining 12 children gastritis not induced by Helicobacter or normal mucosa was found. Of the 20 Pyloriset-negative patients, only five could be biopsied. One of these showed a Helicobacter pylori-induced antrum gastritis. The latex test investigated had a positive predictive value which was too low (37%) to make it helpful in deciding for or against gastroduodenoscopy and the general anesthesia mostly associated with this.

[Congenital, generalized cytomegalovirus infection. Follow-up and therapeutic strategy with ganciclovir]. / Kongenitale, generalisierte Zytomegalie-Virus-Infektion. Verlauf und Therapiestrategie mit Ganciclovir.

We report on a severe, generalized cytomegaly-virus-infection of a neonate with typical clinical symptoms and features. An attempt to treat with the antiviral drug Ganciclovir, an acyclic nucleoside analogue of guanine is described.

[Familial lupus erythematosus with partial C4 deficiency]. / Familiärer Lupus erythematodes bei partiellem C4-Mangel.

A report is presented of a family with selective partial C4-deficiency in 3 members, two of whom suffer from systemic lupus erythematosus (SLE). C4-allotyping showed the presence of one "silent gene" for the C4B-locus (C4BQO) in these three cases. Presumably it is not the reduced C4-content per se that plays the essential role in the pathogenesis of familial SLE, but the combination of the C4BQO-allele with the HLA-DR2, which was also present in all three affected persons. However, it is of practical relevance that strikingly low C4-levels in comparison with the C3-levels in patients with early onset of SLE should initiate an investigation of the whole family. Furthermore, the C4-level in this form of familial SLE is not a suitable parameter for ganging disease activity on follow-up control investigations.

[Effectiveness and tolerance of aerosol disodium cromoglycate in children with bronchial asthma]. / Wirksamkeit und Verträglichkeit von vernebeltem Dinatrium Cromoglykat bei Kindern mit Asthma bronchiale.

Thirteen centres studied the efficacy and safety of nebulized cromolyn solution in 53 asthmatic children (36 m., 17 f.; mean age 4.8 +/- 1.7 years). During a fiveweek treatment period a significant reduction of asthmatic symptoms was observed by investigators daily symptom scores, as assessed by the parents, also showed a significant improvement during treatment period. In addition, there was also a marked decrease in the use of other anti-asthmatic medication (beta 2-sympathomimetics, theophylline and antihistamines). There were no side effects of nebulized cromolyn solution treatment.

[Regional mucoviscidosis screening using immunoreactive trypsin in umbilical cord blood]. / Regionales Mukoviszidose-Screening mittels immunreaktivem Trypsin aus dem Nabelschnurblut.

In all neonates delivered at the Steyr Landeskrankenhaus from 1987 to 1989, immunreactive trypsin (IRT) in the umbilical cord blood was determined to enable early diagnosis of mucoviscidosis (cystic fibrosis, CF). Amongst 4,507 neonates 75 were found in whom the IRT was over the cut-off value of 1,204 ng/ml. Of these babies, 69 were presented at the age of six weeks for determination of elecrolytes in their sweat. Mucoviscidosis could be diagnosed in two patients. The sensitivity of our test is likely to be 100%. There is no question that the specificity (98.38%) requires improvement. The incidence of mucoviscidosis in our catchment area is 1: 2,254. The hypertrypsinemia detected was probably transient in 73 babies. Ten of these patients showed a one-minute Apgar score of less than seven.

[Xanthogranulomatous pyelonephritis in early childhood]. / Xanthogranulomatöse Pyelonephritis im Kleinkindesalter.

We report on a two-year-old infant boy in whom a tumorous enlargement of the left kidney which was not imaged in the excretion urogram was discovered by renal sonography after the second urinary tract infection. By synopsis of the clinical data and imaging techniques, but especially by the detection of foam cells in the urinary sediment, we were already able to make the diagnosis of a xanthogranulomatous pyelonephritis before the operation. Since diffuse infiltration of the entire organ was present, total nephrectomy was carried out as the therapy of choice. Besides the case report, in particular the difficulties in the diagnosis of this form of pyelonephritis (especially in infancy) are described.

[Analysis of respiratory syncytial virus diseases in hospitalized children in the district of Steyr 1984/85]. / Analyse von Respiratory-Syncytial-Virus-Erkrankungen bei hospitalisierten Kindern im Raum Steyr 1984/85.

Respiratory syncytial virus (RSV) is the most frequent cause of serious respiratory tract infections in infancy. In the course of a one-year study the nasopharyngeal secretions of all hospitalized children with diseases of the lower respiratory tract (almost exclusively infants) were examined for viral antigens. RSV antigen could be detected in 34 of the 71 secretions. In these infants a serious clinical course, pneumonia, bronchiolitis or obstructive bronchitis was dominant, but laboratory tests showed no characteristic pattern. Pulmonary X-rays of the RSV-infected infants revealed interstitial pneumonia with or without pulmonary infiltration, in addition to symptoms of hyperinflation. We were able to examine 21 of 33 RSV-infected infants 2 years later.

[AIDS encephalopathy in childhood or the late sequela of central nervous system leukemia?]. / AIDS-Enzephalopathie im Kindesalter oder Spätfolge einer ZNS-Leukämie?

This case report is about an eleven year old boy with new developed symptoms of a cellular immundeficiency and a positive HIV-serology 33 months after a CNS-leukemia relapse. After 18 weeks a progredient neurological symptomatology is beginning with motor, cognitive and behavioral disturbances and a brain atrophy in the CT-scan. These cerebral manifestations are explainable as an encephalopathy both through HIV and after CNS-leukemia. A SSPE has been excluded. CT, EEG, Evoked Potentials do not show differential diagnostic pathognomonic findings regarding both diseases. The CSF findings hint at a persistent virus infection compatibel with the postulated slow virus pathogenesis of the AIDS-Encephalopathy. We conclude, that in this case an etiological diagnoses is only possible through histological brain examination and through demonstration of HIV or HIV-antigen in brain tissue respectively. AZT, which is reported to be effective against the cerebral AIDS-manifestations could not be applicated because of the existing pancytopenia.

[Early diagnosis and early treatment of a patient with intralobular pulmonary sequestration]. / Frühdiagnose und Frühbehandlung eines Patienten mit intralobärer Lungensequestration.

A report is given on a case of intralobar pulmonary sequestration (I. L. S), which was hospitalized with the suspect diagnosis of a cavernous tuberculous infection. The 3 6/12-year-old boy was suffering of recurrent respiratory infections. These led finally to a thoracic X-ray control. X-rays showed a cystic malformation of the left lower lobe. This localisation drew our attention especially to I. L. S. after exclusion of various other diagnostic possibilities. For this reason a retrograde aortography was carried out and showed an abnormal blood supply of the cyst from the thoracic aorta. So this procedure established the diagnosis. Because of the high pulmonary and cardiac complication rate, a surgical treatment, as early as possible, is the therapy of choice. In our case the segmental resection was sufficient.