Summary:Purpose: Valproic acid (VPA) is commonly used as an antiepileptic drug (AED). Regular screening for renal side effects is uncommon. Fanconi syndrome, a generalized dysfunction of renal proximal tubular cells, occurs with some inborn errors of metabolism. In addition, it can be acquired by exposure to several toxic substances. We report a case of Fanconi syndrome after long-term therapy with VPA.Methods: An 8-year-old severely disabled and developmentally retarded boy with epilepsy was treated with VPA over a period of 7 years. He was hospitalized after a status epilepticus with laboratory findings suggesting a Fanconi syndrome. A PubMed-based worldwide review of the literature revealed that Fanconi syndrome is a rare side effect in children during longterm VPA treatment. We analyzed all 10 previously published cases by comparing age, underlying diseases, medication, and outcome.Results: Examination revealed metabolic acidosis suggestive of renal tubular malfunction. Based on typical clinical and laboratory findings, an acquired Fanconi syndrome was diagnosed. This was treated with large doses of sodium bicarbonate. After discontinuation of VPA, renal function completely normalized within 2 months.Conclusions: Fanconi syndrome appears to be a rare but severe consequence of long-term VPA therapy. Therefore patients treated with VPA should be checked regularly for the possible development of VPA-induced Fanconi syndrome.
Background: Allogeneic stem cell transplantation (alloSCT) is a treatment option for primary cutaneous T-cell lymphomas that may induce long-lasting complete remissions. Little information is available on safety and efficacy. Patients and methods: We retrospectively reviewed the data from patients with primary cutaneous T-cell lymphoma treated in the Departments of Dermatology of the Universities of Bochum, Mannheim and Cologne who received subsequent alloSCT between 2005 and 2012. Results: Nine patients with aggressive primary cutaneous T-cell-lymphoma received alloSCT. With a follow-up of 14 to 36 months after transplantation, 4 patients are alive and in complete remission. Two patients had recurrent disease post-transplantation, which was successfully treated with donor lymphocyte infusions. Non-relapse mortality was observed in three patients in advanced disease stages within six months after alloSCT. One patient showed only partial remission and died of disease after 32 months and one patient died 26 months after alloSCT with cause of death unknown. Conclusions: This report documents the possible benefit of a graft-versus-lymphoma effect in primary cutaneous T-cell lymphoma, as has been observed for other T-cell malignancies and emphasizes that alloSCT warrants further studies in this setting.
Girdle syndrome is a rare but severe adverse event associated with sickle cell disease that must be considered as differential diagnosis in patients with sickle cell disease.
Background
Early diagnosis is mandatory for the medical care of children and adolescents with pediatric-onset inflammatory bowel disease (PIBD). International guidelines (‘Porto criteria’) of the European Society for Pediatric Gastroenterology, Hepatology and Nutrition recommend medical diagnostic procedures in PIBD. Since 2004, German and Austrian pediatric gastroenterologists document diagnostic and treatment data in the patient registry CEDATA-GPGE on a voluntary basis. The aim of this retrospective study was to analyze whether the registry CEDATA-GPGE reflects the Porto criteria and to what extent diagnostic measures of PIBD according to the Porto criteria are documented.
Methods
Data of CEDATA-GPGE were analyzed for the period January 2014 to December 2018. Variables representing the Porto criteria for initial diagnostic were identified and categorized. The average of the number of measures documented in each category was calculated for the diagnoses CD, UC, and IBD-U. Differences between the diagnoses were tested by Chi-square test. Data on possible differences between data documented in the registry and diagnostic procedures that were actually performed were obtained via a sample survey.
Results
There were 547 patients included in the analysis. The median age of patients with incident CD (n = 289) was 13.6 years (IQR: 11.2–15.2), of patients with UC (n = 212) 13.1 years (IQR: 10.4–14.8) and of patients with IBD-U (n = 46) 12.2 years (IQR: 8.6–14.7).
The variables identified in the registry fully reflect the recommendations by the Porto criteria. Only the disease activity indices PUCAI and PCDAI were not directly provided by participants but calculated from obtained data. The category ‘Case history’ were documented for the largest part (78.0%), the category ‘Imaging of the small bowel’ were documented least frequently (39.1%). In patients with CD, the categories ‘Imaging of the small bowel’ (χ2 = 20.7, Cramer-V = 0.2, p < 0.001) and ‘Puberty stage’ (χ2 = 9.8, Cramer-V = 0.1, p < 0.05) were documented more often than in patients with UC and IBD-U.
Conclusion
The registry fully reproduces the guideline’s recommendations for the initial diagnosis of PIBD. The proportion of documented diagnostic examinations varied within the diagnostic categories and between the diagnoses. Despite technological innovations, time and personnel capacities at participating centers and study center are necessary to ensure reliable data entry and to enable researchers to derive important insights into guideline-based care.
Loss of the central visual field and central fixation immediately after glaucoma filtering surgery is a rare complication. Therefore, glaucoma filtering surgery can also be recommended for patients with advanced visual field defects.
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