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The Kidney Disease: Improving Global Outcomes (KDIGO) Clinical Practice Guideline for the Evaluation and Management of Chronic Kidney Disease (CKD) updates the KDIGO 2012 guideline and has been developed with patient partners, clinicians, and researchers around the world, using robust methodology. This update, based on a substantially broader base of evidence than has previously been available, reflects an exciting time in nephrology. New therapies and strategies have been tested in large and diverse populations that help to inform care; however, this guideline is not intended for people receiving dialysis nor those who have a kidney transplant. The document is sensitive to international considerations, CKD across the lifespan, and discusses special considerations in implementation. The scope includes chapters dedicated to the evaluation and risk assessment of people with CKD, management to delay CKD progression and its complications, medication management and drug stewardship in CKD, and optimal models of CKD care. Treatment approaches and actionable guideline recommendations are based on systematic reviews of relevant studies and appraisal of the quality of the evidence and the strength of recommendations which followed the "Grading of Recommendations Assessment, Development, and Evaluation" (GRADE) approach. The limitations of the evidence are discussed. The guideline also provides practice points, which serve to direct clinical care or activities for which a systematic review was not conducted, and it includes useful infographics and describes an important research agenda for the future. It targets a broad audience of people with CKD and their healthcare, while being mindful of implications for policy and payment., (Copyright © 2023 Kidney Disease: Improving Global Outcomes (KDIGO). Published by Elsevier Inc. All rights reserved.)

More than one-third of US adults have limited health literacy, putting them at risk of adverse clinical outcomes. We evaluated the prevalence of limited health literacy among 1578 adult kidney transplant (KT) candidates (May 2014-November 2017) and examined its association with listing for transplant and waitlist mortality in this pilot study. Limited health literacy was assessed at KT evaluation by using a standard cutoff score ≤5 on the Brief Health Literacy Screen (score range 0-12, lower scores indicate worse health literacy). We used logistic regression and adjusted Cox proportional hazards models to identify risk factors for limited health literacy and to quantify its association with listing and waitlist mortality. We found that 8.9% of candidates had limited health literacy; risk factors included less than college education (adjusted odds ratio [aOR] = 2.87, 95% confidence interval [CI]:1.86-4.43), frailty (aOR = 1.85, 95% CI:1.22-2.80), comorbidity (Charlson comorbidity index [1-point increase] aOR = 1.12, 95% CI: 1.04-1.20), and cognitive impairment (aOR = 3.45, 95% CI: 2.20-5.41) after adjusting for age, sex, race, and income. Candidates with limited health literacy had a 30% (adjusted hazard ratio = 0.70, 95% CI: 0.54-0.91) decreased likelihood of listing and a 2.42-fold (95% CI: 1.16- to 5.05-fold) increased risk of waitlist mortality. Limited health literacy may be a salient mechanism in access to KT; programs to aid candidates with limited health literacy may improve outcomes and reduce disparities., (© 2018 The American Society of Transplantation and the American Society of Transplant Surgeons.)

Objectives/introduction: Demand for nursing home (NH) care by patients with end-stage renal disease (ESRD) is likely to increase with growing numbers of older adults initiating chronic dialysis. We completed a systematic review to summarize the literature on NH residents with ESRD., Methods: MEDLINE, CINAHL, EMBASE, and relevant conference proceedings were searched to identify articles using the following MESH terms or related key words in the title or abstract: "residential facilities", "renal dialysis", "renal replacement therapy", and "chronic kidney failure". We selected case control, cohort studies, and clinical trials that included older adults with ESRD (defined as those receiving chronic dialysis or those with stage 5 chronic kidney disease) living in residential care facilities. We abstracted information on study design, quality, and results., Results: Of 198 unique citations identified by the search strategy, 14 articles met eligibility criteria. Most articles were multicenter studies that were conducted in the 1990 s. One study focused on patients with stage 5 chronic kidney disease, and the remaining 13 studies focused on patients receiving chronic dialysis, of which eight studies included only those receiving peritoneal dialysis, four studies included patients receiving both peritoneal dialysis and hemodialysis, and one study included only patients receiving hemodialysis. All studies were observational, no clinical trials were identified, and study design limitations and heterogeneity within study populations were common. Summarizing results across these studies suggests that NH residents with ESRD have limited survival, particularly early after dialysis initiation. Functional impairment is highly prevalent in this population and independently associated with poor outcomes., Conclusions: NH residents with ESRD appear to be a particularly vulnerable population, but current information on their prevalence, characteristics, and outcomes is limited. Further research is needed to provide a better understanding of modifiable predictors of survival and functional decline in this population., (Copyright © 2013 American Medical Directors Association, Inc. All rights reserved.)

This article describes a series of 21 consecutive cases, each involving a solitary median maxillary central incisor; the patients were seen in the Department of Dentistry or the Victorian Clinical Genetics Unit, Murdoch Institute, at the Royal Children's Hospital, Melbourne, from 1966 to 1997. The spectrum of anomalies and associated features present in these cases--solitary median maxillary central incisor, choanal atresia, and holoprosencephaly--is described, and the literature related to the features, including genetic studies in these conditions, is reviewed. We relate our findings in these cases to current knowledge of developmental embryology. It is hoped that the findings, together with our interpretation of them, will help to clarify understanding of solitary median maxillary central incisor syndrome. This syndrome was previously considered a simple midline defect of the dental lamina, but it is now recognized as a possible predictor of holoprosencephalies of varying degrees in the proband, in members of the proband's family, and in the family's descendants.

In this study, we analyzed the role of the TATA box in the regulation of the phosphoenolpyruvate carboxykinase (PEPCK) gene expression by dexamethasone (DEX), retinoic acid (RA), glucagon (via cAMP) and insulin (INS). The PEPCK TATA box (TATTTAAA) was absolutely required for both basal promoter activity and hormone-mediated transactivation. However, the relative induction of PEPCK gene expression by DEX, RA and cAMP, and its repression by INS, remained unaltered despite the substitution of the PEPCK TATA box with TATA elements from the herpes simplex virus-thymidine kinase gene, gene 33 or a consensus TATA box sequence, TATAAA. The results indicate that the TATA box serves a permissive, but not defining, function in the response of the PEPCK gene to hormones, and that this function can be equally facilitated by heterologous TATA box elements.

This article describes the enamel ultrastructure and clinical features in two siblings with the little known syndrome of Amelogenesis imperfecta and nephrocalcinosis. Nephrocalcinosis was diagnosed by x-ray examination of the abdomen, intravenous pyelography, ultrasonography, and computed tomography scan. Amelogenesis imperfecta was diagnosed from clinical and histologic examinations. The affected enamel was hypoplastic (approximately 0.2 mm thick), positively birefringent, generally aprismatic, porous, and consisted of loosely packed, randomly orientated, thin (approximately 10 nm wide), ribbonlike crystals. The enamel surface was rough, extensively cracked, and covered with ovoid or globular protrusions. Observations showed that in this case hypoplasia, hypocalcification, or hypomaturation defects were present in the same tooth, indicating that both secretory and maturation phases may have been affected. The study suggested the possibility of an abnormality in interstitial matrix, which could lead to dystrophic calcification in the kidney and abnormal tooth enamel formation. It also suggested the possibility of involvement of two separate but closely linked genes.

A case of a young male with the Klippel-Trénaunay-Weber syndrome is described. Typical features of hemihypertrophy, hemangiomata, macrodactyly, and macrocephaly were present. The most striking oral feature was generalized severe gingival hypertrophy confirmed histologically, ultrastructurally, and by collagen analysis. In the absence of other known systemic causes of gingival enlargement, a diagnosis of familial gingival fibromatosis in association with Klippel-Trénaunay-Weber-syndrome is concluded. The combination of gingival fibromatosis and Klippel-Trénaunay-Weber syndrome has not been reported to our knowledge, it is uncertain whether this occurrence is significant or coincidental.

Expression of the Xenopus laevis transcription factor IIIA (TFIIIA) gene is developmentally regulated. In this study we have used defined nucleotide mutations to map cis-elements involved in transcriptional regulation of the promoter for oocyte-TFIIIA (O-TFIIIA) in stage II-IV oocytes, stage VI oocytes, and tail bud embryos. During oogenesis O-TFIIIA mRNA levels decline 5- to 10-fold, and during early embryogenesis O-TFIIIA mRNA levels decline approximately 10(6)-fold per cell. In stage II-IV oocytes we find evidence for at least three distinct positive-acting cis-elements that contribute to the efficient expression of O-TFIIIA. These elements are located between -1800 to -425, -280 to -235, and -235 to -220. The most distal cis-element(s) appears to be developmentally regulated during oogenesis, since deletion of nucleotide sequences from -1800 to -425 does not reduce O-TFIIIA expression in stage VI oocytes. However, the two cis-elements located between -280 to -235 and -235 to -220 are required for the efficient expression of O-TFIIIA in stage VI oocytes. In tail bud embryos we find evidence for several developmentally regulated positive and negative cis-elements involved in O-TFIIIA expression. The positive-acting cis-elements are located between -159 to -110 and -110 to -58. The negative-acting cis-elements are found at positions -425 to -350 and -200 to -159. In addition to the developmentally regulated elements controlling O-TFIIIA gene expression in tail bud embryos, the positive-acting cis-elements active during oogenesis (located between -280 to -235 and -235 to -220) are also active during early embryogenesis. Thus, transcription from the O-TFIIIA promoter appears to be regulated by a combination of constitutive positive factors and developmentally regulated positive and negative factors during oogenesis and early embryogenesis.

Management of the adolescent with cleft lip and/or palate is ideally undertaken by a cleft palate team which has overseen care since birth, and which provides, in a paediatric hospital setting, the specialist medical, dental and ancillary care services necessary for optimal management. However, in some countries such a team is not available and individual dental practitioners and dental specialists have to undertake the necessary treatment. This is facilitated in Australia by the Federal Government's 'Cleft Lip and Palate Scheme', which subsidizes all medical and dental treatment related to the congenital defect up to 22 years of age. In such circumstances the general dental practitioner or paedodontist may need to assume an important coordinating role. The timing and integration of phases of general dental, minor oral surgical, orthodontic, periodontic and prosthetic treatment with plastic, ENT and maxillofacial surgery, speech therapy and audiology may become his responsibility. In addition, the provision of a high standard of preventive and general dental care for the cleft patient must be maintained. To carry out this coordinating role effectively, the general dentist must know his patient and family well; must understand the current concepts, objectives, treatment and investigatory techniques used in all aspects of cleft palate management; he should be aware of the genetics of the clefting conditions, medical problems which may commonly be associated with clefts and the cleft as one feature of a syndrome. Most importantly, he should be aware of the special social adjustment problems faced by many cleft adolescents in relation to their perceived 'different' facial appearance and speech.