Inflammatory bowel disease in primary immunodeficiency disorders is a heterogeneous clinical entity requiring an individualized treatment strategy: A systematic review

ObjectiveTo describe the prevalence, clinical presentation and current treatment regimens of inflammatory bowel disease (IBD) in patients with primary immunodeficiency disorders (PIDs).MethodsA systematic review was conducted. The following databases were searched: MEDLINE, Embase, Web of Science, the Cochrane Library and Google Scholar.ResultsA total of 838 articles were identified, of which 36 were included in this review. The prevalence of IBD in PIDs ranges between 3.4% and 61.2%, depending on the underlying PID. Diarrhea and abdominal pain were reported in 64.3% and 52.4% of the patients, respectively. Colon ulceration was the most frequent finding on endoscopic evaluation, while cryptitis, granulomas, ulcerations and neutrophilic/lymphocytic infiltrates were the most frequently reported histopathological abnormalities. Described treatment regimens included oral corticosteroids and other oral immunosuppressive agents, including mesalazine, azathioprine and cyclosporin, leading to clinical improvement in the majority of patients. In case of treatment failure, biological therapies including TNF- α blocking agents, are considered.ConclusionsThe overall prevalence of IBD in patients with PID is high, but varies between different PIDs. Physicians should be aware of these complications and focus on characteristic symptoms to reduce diagnostic delay and delay in initiation of treatment. Treatment of IBD in PIDs depends on severity of symptoms and may differ between various PIDs based on distinct underlying pathogenesis. An individualized diagnostic and therapeutic approach is therefore warranted.     

Correction to: Endovascular stroke treatment’s impact on malignant type of edema (ESTIMATE)

Journal of Neurology - The original keywords were not sufficiently precise in describing the article content. Therefore, the key words were amended to include hemicraniectomy and malignant edema.     

Der lange Weg von der Idee bis zur Erstattung – ein Reisebericht

Bundesgesundheitsblatt - Gesundheitsforschung - Gesundheitsschutz - Digitale Gesundheitsprodukte und -dienstleistungen beginnen sowohl die Versorgung von Kranken als auch die Prävention...     

Syndromes 22. Rieger's syndrome

Rieger syndrome is a rare autosomal-dominant disorder characterized by defects of the anterior chamber of the eye, failure of the periumbilical skin to involute, and developmental malformations of the dentition. Recognition of the dental anomalies may result in the early diagnosis of this syndrome and prevent progressive visual loss.     

Syndromes 14. Rendu-Osler-Weber disease

The Rendu-Osler-Weber disease is due to an autosomal dominant disease with multiple telangiectasia in skin and mucosa. Recurrent bleeding of the nose is due to telangiectasia of the nasal mucosa. Haemorrhage of the oral mucosa also occurs. Extensive arteriovenous malformations can be present in lungs, liver and brain. Treatment of bleedings in the oral mucosa is easily possible by means of coagulation. Patients with pulmonary arteriovenous malformations should receive prophylactic antibiotic treatment before extractions, removal of third molars and subgingival curettage.