Effect of topical calcipotriol on congenital ichthyoses

We investigated the clinical efficacy of topically applied calcipotriol in six patients with congenital ichthyosis, using a double-blind, bilaterally paired, comparative approach. Unilateral improvement, in favour of the calcipotriol-treated side, was observed in three patients with lamellar ichthyosis. A beneficial response was also observed in a patient with bullous ichthyotic erythroderma of Brocq. No clinical side-effects or laboratory anomalies were observed. This study indicates that calcipotriol constitutes a new and promising approach in alleviating disorders of keratinization characterized by hyperproliferation, other than psoriasis.     

A pilot goal-directed perfusion initiative is associated with less acute kidney injury after cardiac surgery

Background

We sought to determine whether a pilot goal-directed perfusion initiative could reduce the incidence of acute kidney injury after cardiac surgery.

Progression of liver fibrosis in HIV/HCV genotype 1 co-infected patients is related to the T allele of the rsI2979860 polymorphism of the IL28B gene

Objective

HIV/HCV co-infection is characterised by accelerated progression of liver disease. Recently, the rsl2979860 C/T polymorphism in the IL28B gene has been linked to progression towards cirrhosis in HCV mono-infected patients and to treatment response of HCV-infection in HIV/HCV co-infected patients. Our aim was to clarify by non-invasive techniques if this polymorphism affects fibrosis progression in HIV/HCV co-infection.

Methods

In a cross-sectional design, liver stiffness (transient elastography), surrogate markers of liver fibrosis (APRI and FIB-4 scores) and rsl2979860 genotypes were analysed in 84 HCV/H1V co-infected patients. IL28B genotypes were determined by real-time PCR using a light cycler. In 56 HIV/HCV co-infected patients we also studied progression of fibrosis in relation to rsl2979860 C/T genotypes over two years.

Results

82% of the patients were on HAART (74% without detectable HI viremia) and 67% were haemophiliacs, respectively. HCV genotype 1 was present in 62%. Cross-sectional median liver stiffness was 7.4 kPa and correlated with APRI and FIB-4 scores (r = 0.6 each, p < 0.001). Frequencies of IL28B genotypes were: CC 50%, CT 43% and TT 7%. In the cross-sectional analysis liver stiffness values were not different between the various IL28B-genotypes. Upon follow-up under HAART carriers of a C allele did not show further progression, while liver stiffness significantly increased in HIV/HCV co-infected patients with the T allele (p = 0.047).

Conclusion

Although progression of liver fibrosis was low under HAART in our cohort, progression was more pronounced in HIV/HCV genotype 1 co-infected patients with the T allele.     

Progressive diaphyseal dysplasia (Camurati-Engelmann): radiographic follow-up and CT findings

Sixteen patients with progressive diaphyseal dysplasia (PDD) and aged six months to 76 years were examined. Fourteen cases were hereditary, two were not. The progression of the radiologic manifestations in 13 patients who were followed up from 1 to 32 years and the computed tomography (CT) scans from five patients were obtained. The progression of PDD was slow and unpredictable, from minimal endosteal thickening of the mid-diaphyses in one pair of long bones to severe sclerosis of long bones, skull, and vertebrae. The severity of the osseous changes was not age dependent. A six-stage system was used to grade the severity of involvement and progression of PDD. CT scans demonstrated muscle mass that was preserved and showed the distribution of the osteosclerotic process, which was irregular and inhomogeneous. CT scanning was advantageous over plain radiography in this respect. Endosteal involvement was more extensive than periosteal thickening. CT scans also showed a distinct pattern of vertebral sclerosis that was confined to the posterior areas of the vertebral body and arches. In light of the paucity of characteristic clinical signs of PDD, the recognition of the radiologic features is mandatory for the diagnosis of this disease.