Follow-up of adult celiac patients: which noninvasive test reflects mucosal status most reliably?1.

UNLABELLED: SPECIFIC AUTHOR CONTRIBUTIONS: Andreas Vécsei, MD, and Ulrike Graf wrote the manuscript. All authors contributed to study design, data collection and analysis, and approved the final draft for submission. The corresponding author declares that the manuscript is submitted on behalf of all authors. BACKGROUND AND STUDY AIMS: The best mode of follow-up in celiac disease has not yet been established. The intention of this study was to clarify which noninvasive follow-up investigation - serological tests or intestinal permeability test (IPT) - correlates best with histology and whether the interval between diagnosis and follow-up affects the accuracy of these tests. PATIENTS AND METHODS: Data from adult patients with celiac disease (diagnosed between December 1989 and July 2006) followed up with biopsy, IPT, and serological tests [IgG anti-gliadin antibodies (AGA-IgG), AGA-IgA, and endomysial antibodies (EMA)] were retrieved from a computerized database. Results of noninvasive tests were compared with the persistence of villous atrophy on biopsy. Patients were divided into groups A, which comprised patients followed up within 2 years after diagnosis, and B, comprising patients followed up later than 2 years. RESULTS: Forty-seven patients were evaluable. The lactulose/mannitol (L/M) ratio had a sensitivity of 85 % and a specificity of 46.2 % for mucosal atrophy, whereas saccharose excretion showed a sensitivity of 60 % and a specificity of 52.6 %. The sensitivities of AGA-IgA and AGA-IgG were 15 % and 20 %, respectively, while specificity was 100 % for both. Validity of AGA was limited due to low number of positive results. EMA assay was 50 % sensitive and 77.8 % specific. In group A (n = 23) L/M ratio performed best in terms of sensitivity (88.9 %), whereas EMA achieved a higher specificity (71.4 %). In group B, the sensitivity of the L/M ratio decreased to 85.7 %, while the specificity of EMA increased to 91.7 %. CONCLUSIONS: In this study, none of the noninvasive tests was an accurate substitute for follow-up biopsy in detecting severe mucosal damage.

Plasmapheresis-induced remission in otherwise therapy-resistant FSGS.

We report an 8-year-old Caucasian boy who presented with steroid-resistant nephrotic syndrome. Renal biopsy showed the cellular variant of focal segmental glomerulosclerosis (FSGS). Within 1 year he received a series of therapies that have induced remission in other patients with this disease, all to no avail (conventional-dose cyclosporin A, methylprednisolone pulse therapy, high-dose cyclosporin A, and therapy with mycophenolate mofetil). He achieved remission after five sessions of plasma exchange. This case argues for aggressive therapy of resistant nephrotic syndrome in the native kidney. Plasma exchange should be considered as a possible rescue therapy arm in future study protocols.

Pediatric visceral leishmaniasis in Austria: diagnostic difficulties in a non-endemic region.

Visceral leishmaniasis is usually fatal if left untreated. In Europe it is mainly caused by Leishmania infantum which is endemic in the whole Mediterranean region. While visceral leishmaniasis classically affects children, adults increasingly suffer infections in regions which are known to be endemic for HIV. Nowadays up to 70% of the patients with visceral leishmaniasis in southern Europe are HIV-infected adults. The diagnosis is known to be especially difficult to establish in this group of patients because of a frequently atypical clinical presentation, but even in non-HIV-infected patients visceral leishmaniasis often represents a diagnostic challenge particularly when the patient is living in a non-endemic region. We report on four children with visceral leishmaniasis diagnosed at St. Anna Children's Hospital, Vienna, in the last decade. Diagnostic difficulties arose (1) from inexperience with this rare disease, (2) from a long incubation period (6 to 8 months) and (3) from a travel history apparently unsuspicious for the contraction of what is considered a 'tropical' disease. In one case, specific problems resulted (4) from clinical appearance and laboratory data mimicking hemophagocytic lymphohistiocytosis. Consequently even in regions where leishmaniasis is not endemic, diagnostic efforts should be undertaken to rule out this disease especially in patients with the presumptive diagnosis of hemophagocytic lymphohistiocytosis.

[Congenital syphilis: late diagnosis in spite of screening]. / Lues connata: Späte Diagnose trotz Screening.

We report the case of an infant in whom congenital syphilis was diagnosed at the age of 5 weeks. The case is remarkable because of (a) the negative venereal disease laboratory test from the cord blood, (b) the incidental diagnosis of the disease in the fifth week of life, (c) pneumonia alba being one of the symptoms, (d) the occurrence of a mild Jarisch-Herxheimer reaction after initiation of penicillin therapy and (e) the successful treatment of infection related anaemia with recombinant human erythropoietin.