Long and short-term outcomes in patients requiring continuous renal replacement therapy post cardiopulmonary bypass.

OBJECTIVE: The development of acute renal failure following cardiac surgery is a rare but devastating complication with high morbidity and mortality. This study aimed to assess the incidence of acute renal failure necessitating continuous renal replacement therapy (CRRT) in patients who required cardiopulmonary bypass, to determine the factors associated with mortality and to evaluate long-term outcome. METHODS: Patients who underwent cardiac surgery between October 1997 and 2003 and treated with CRRT were included (n=98). Six patients were then excluded (already in established renal failure pre-operatively) and one patient lost to follow-up. A retrospective analysis was carried out. RESULTS: Overall CRRT was used in 2.9% (92/3172). The mean (SD) age of patients was 68 (10) years. Their mean pre-operative creatinine level and duration of cardiopulmonary bypass were 154 (87)micromol/l and 160 (84)min, respectively. Mean duration from surgery to establishment of CRRT was 50 (42)h. Mean creatinine level prior to hospital discharge was 168 (93)micromol/l. Thirty-day mortality was 42%. Significant risk factors for death were complex procedures (odds ratio=9.9), gastro-intestinal complications (OR=7.2), cross-clamp time over 88min (OR=5.9), re-exploration (OR=4.0) and patients age over 75 years (OR=3.3). Actuarial 1 and 5-year survivals (95% CI) were 53 (43, 63) % and 52 (42, 62) %, respectively. Only 2 (2.2%) patients required long term renal support. CONCLUSIONS: Acute renal failure necessitating the use of CRRT is a rare but serious complication post cardiopulmonary bypass. In the long-term, surviving patients are not likely to require further renal support.     

Molecular basis of variegate porphyria: a missense mutation in the protoporphyrinogen oxidase gene.

Variegate porphyria (VP) is an autosomal dominant disorder characterised by a partial defect in the activity of protoporphyrinogen oxidase (PPO), and has recently been genetically linked to the PPO gene on chromosome 1q22-23 (Z=6.62). In this study, we identified a mutation in the PPO gene in a patient with VP and two unaffected family members. The mutation consisted of a previously unreported T to C transition in exon 13 of the PPO gene, resulting in the substitution of a polar serine by a non-polar proline (S450P). This serine residue is evolutionarily highly conserved in man, mouse, and Bacillus subtilis, attesting to the importance of this residue. Interestingly, the gene for Gardner's syndrome (FAP) also segregates in this family, independently of the VP mutation. Gardner's syndrome or familial adenomatous polyposis (FAP) is also an autosomal dominantly inherited genodermatosis, and typically presents with colorectal cancer in early adult life secondary to extensive adenomatous polyps of the colon. The specific gene on chromosome 5 that is the site of the mutation in this disorder is known as APC (adenomatous polyposis coli), and the gene has been genetically linked to the region of 5q22.     

Does Evidence Exist to Blunt Inflammatory Response by Nutraceutical Supplementation during COVID-19 Pandemic? An Overview of Systematic Reviews of Vitamin D,Vitamin C,Melatonin, and Zinc

More than one year has passed since the first cases of coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome (SARS)-CoV-2 coronavirus were reported in Wuhan (China), rapidly evolving into a global pandemic. This infectious disease has become a major public health challenge in the world. Unfortunately, to date, no specific antivirals have been proven to be effective against COVID-19, and although a few vaccines are available, the mortality rate is not decreasing but is still increasing. One therapeutic strategy has been focused on infection prevention and control measures. In this regard, the use of nutraceutical supports may play a role against some aspect of the infection, particularly the inflammatory state and the immune system function of patients, thus representing a strategy to control the worst outcomes of this pandemic. For this reason, we performed an overview including meta-analyses and systematic reviews to assess the association among melatonin, vitamin C, vitamin D, zinc supplementation and inflammatory markers using three databases, namely, MEDLINE, PubMed Central and the Cochrane Library of Systematic Reviews. According to the evidence available, an intake of 50,000 IU/month of vitamin D showed efficacy in CRP. An amount of 1 to 2 g per day of vitamin C demonstrated efficacy both in CRP and endothelial function, and a dosage of melatonin ranging from 5 to 25 mg /day showed good evidence of efficacy in CRP, TNF and IL6. A dose of 50 mg/day of elemental zinc supplementation showed positive results in CRP. Based on the data reported in this review, the public health system could consider whether it is possible to supplement the current limited preventive measures through targeted nutraceutical large-scale administration.     

Mutations in LPAR6/P2RY5 and LIPH are associated with woolly hair and/or hypotrichosis

Background Woolly hair (WH) belongs to a family of disorders characterized by hair shaft anomalies that clinically presents with tightly curled hair, which can be divided into syndromic and non‐syndromic forms of WH. We have recently identified mutations in both LPAR6/P2RY5 and LIPH that are associated with autosomal recessive woolly hair (ARWH). Objective To study the underlying genetic causes of autosomal woolly hair in Pakistani population. Methods We studied 10 Pakistani families with ARWH for mutations in LPAR6/P2RY5 and LIPH and then performed haplotype analysis to confirm their segregation in the families. Results We identified five mutations in LPAR6/P2RY5, among which three were recurrent and two were novel in eight Pakistani families. We then showed that two of the mutations in LPAR6/P2RY5 are founder mutations in Pakistani families. Moreover, we identified two recurrent mutations in the LIPH gene in two Pakistani families. Conclusion Our study extends the spectrum of mutations in LPAR6/P2RY5 gene and underscores those mutations in LPAR6/P2RY5 and LIPH result in similar phenotypes.     

Cor triatriatum sinister, not mitral stenosis, in an adult with previous Sydenham's chorea: diagnosis and preoperative assessment by cross sectional echocardiography.

In cor triatriatum sinister, one of the rarest congenital cardiac anomalies, a membrane divides the left atrium into a pulmonary venous component above and the vestibule below. The importance of the anomaly lies in the effects of the resultant pulmonary venous obstruction that usually present in the first year of life and can mimic obstructed total anomalous venous drainage or congenital mitral stenosis. A case presented as mitral stenosis in the third decade of life, ten years after a well documented episode of Sydenham's chorea. The diagnosis was made rapidly by transthoracic echocardiography and transoesophageal echocardiography was used for complete assessment. Cardiac catheterisation added nothing to the non-invasive diagnosis or the preoperative assessment. Uncomplicated corrective surgery was undertaken.     

Development and validation of two new image receptor-holding devices for overlapping reduction of proximal surfaces in bitewing radiography: a preclinical study

Objective:To create and validate two new Image Receptor-Holding devices (IRHDs) to reduce proximal surfaces overlapping in bitewing radiography in comparison to a regular well-established and commercially available IRHD.Methods:Two IRHDs for bitewing radiographs with a wedge on the biting surface were designed and 3D-printed. These IRHDs, with a large wedge (Pr-Lw) and small wedge (Pr-Sw), were compared with a regular commercially available IRHD (Rinn XCP^®) during image acquisition of bitewing radiographs of four posterior regions (one upper region and three lower regions) in two dry skulls and mandibles. A total of 156 interproximal regions on bitewing radiographs were radiographed by 13 oral radiology graduate students and independently assessed by two oral radiologists (10 years of experience). IRHDs were compared by Cochran’s Q test regarding the number of overlapped proximal surfaces in the acquired radiographs with a significance level of 5%.Results:The observers were in perfect agreement (κ = 1.0) to classify the proximal surfaces overlapping. The Pr-Lw IRHD presented the lowest number of surfaces overlapping (19.2%) followed by the Pr-Sw IRHD (48.1%) and the XCP^®IRHD with the highest surfaces overlapping (71.2%). The Pr-Lw IRHD surfaces overlapping was statistically different from the XCP IRHD (p < 0.001), and the Pr-Sw IRHD (p = 0.014).Conclusions:The Pr-Lw IRHD demonstrated the most efficient performance in overlapping surfaces reduction, compared with the Pr-Sw IRHD and the XCP^® IRHD in adult dry skulls and mandibles.     

Early imaging signs of the use of antiresorptive medication and MRONJ: a systematic review

Objective

The main aim is to identify, by means of different imaging modalities, the early bone changes in patients “at risk” and in stage 0 MRONJ.

Materials and methods

A search of the literature was performed on PubMed, Embase, Web of Science, and Cochrane Library databases, until June 9, 2020. No language or year restrictions were applied. Screening of the articles, data collection, and qualitative analysis was done. The Newcastle-Ottawa Scale (NOS) was used for observational studies, and the Systematic Review Centre for Laboratory Animal Experimentation’s (SYRCLE) risk of bias tool for the animal studies.

Results

A total of 1188 articles were found, from which 47 were considered eligible, whereas 42 were suitable for the qualitative analysis. They correspond to 39 human studies and 8 animal studies. Radiographic findings such as bone sclerosis, osteolytic areas, thickening of lamina dura, persisting alveolar socket, periapical radiolucency, thicker mandibular cortex, widening of the periodontal ligament space, periodontal bone loss, and enhancement of the mandibular canal were identified as early bone changes due to antiresorptive therapy. All those findings were also reported later in Stage 0 patients.

Conclusion

The main limitations of these results are the lack of prospective data and comparisons groups; therefore, careful interpretation should be made. It is a fact that radiographic findings are present in antiresorptive-treated patients, but the precise timepoint of occurrence, their relation to the posology, and potential risk to develop MRONJ are not clear.

Clinical relevance

The importance of a baseline radiographic diagnosis for antiresorptive-treated patients.