Psychometric findings and normative values for the CLEFT-Q based on 2434 children and young adult patients with cleft lip and/or palate from 12 countries.

BACKGROUND: Patients with cleft lip and/or palate can undergo numerous procedures to improve appearance, speech, dentition and hearing. We developed a cleft-specific patient-reported outcome instrument to facilitate rigorous international measurement and benchmarking. METHODS: Data were collected from patients aged 8-29 years with cleft lip and/or palate at 30 hospitals in 12 countries between October 2014 and November 2016. Rasch measurement theory analysis was used to refine the scales and to examine reliability and validity. Normative CLEFT-Q values were computed for age, sex and cleft type. RESULTS: Analysis led to the refinement of an eating and drinking checklist and 12 scales measuring appearance (of the face, nose, nostrils, teeth, lips, jaws and cleft lip scar), health-related quality of life (psychological, social, school, speech distress) and speech function. All scales met the requirements of the Rasch model. Analysis to explore differential item functioning by age, sex and country provided evidence to support the use of a common scoring algorithm for each scale for international use. Lower (worse) scores on CLEFT-Q scales were associated with having a speech problem, being unhappy with facial appearance, and needing future cleft-related treatments, providing evidence of construct validity. Normative values for age, sex and cleft type showed poorer outcomes associated with older age, female sex and having a visible cleft. INTERPRETATION: The CLEFT-Q represents a rigorously developed instrument that can be used internationally to collect and compare evidence-based outcomes data from patients aged 8-29 years of age with cleft lip and/or palate.

Translation and cultural adaptation of the CLEFT-Q for use in Colombia, Chile, and Spain.

BACKGROUND: Cleft lip and/or palate (CL/P) is a common congenital craniofacial anomaly that may negatively affect an individual's appearance, health-related quality of life, or speech. In Spain, Colombia, and Chile the overall prevalence of CL/P ranges from 0.53 to 1.59 cases per 1000 live births. Currently, there is no patient-reported outcome (PRO) instrument that is specific for patients with CL/P. The CLEFT-Q is a new PRO instrument developed to measure outcomes of treatment in patients 8 to 29 years of age with CL/P. The aim of this study was to translate and culturally adapt the CLEFT-Q for use in Colombia, Chile, and Spain. METHODS: The CLEFT-Q was translated from English to 3 Spanish language varieties (Colombian, Chilean, and Spanish (Spain)) and Catalan. Translation and cultural adaptation guidelines set forth by the International Society for Pharmacoeconomics and Outcomes Research were followed. RESULTS: The field- test version of the CLEFT-Q consisted of 13 scales (total 154 items) measuring appearance, health-related quality of life, and facial function. Forward translations revealed 10 (7%) items that were difficult to translate into Chilean, and back translations identified 34 (22%) and 21 (13%) items whose meaning differed from the English version in at least 1 of the 3 Spanish varieties and Catalan respectively. Twenty-one participants took part in cognitive debriefing interviews. Participants were recruited from plastic surgery centres in Bogotá, Colombia (n = 4), Santiago, Chile (n = 7), and Barcelona, Spain (n = 10). Most participants were males (n = 14, 67%) and were diagnosed with CL/P (n = 17, 81%). Participants reported difficulty understanding 1 item in the Colombian, 1 item in the Spanish (Spain), and 11 items from the Catalan version. Comparison of the 3 Spanish varieties revealed 61 (40%) of the 154 items whose wording differed across the 3 Spanish versions. CONCLUSION: Translation and cultural adaptation processes provided evidence of transferability of the CLEFT-Q scales into 3 Spanish varieties and Catalan, as semantic, idiomatic, experiential, and conceptual equivalence of the items, instructions, and response options were achieved.

Recurrence of granulomatous interstitial nephritis in transplanted kidney.

Sarcoidosis is a multisystemic disease of unknown etiology. Minor renal involvement is not rare but kidney failure is uncommon and only rare cases of recurrent disease in a kidney transplant have been published. We report a patient who at age 10 yr developed ESRD secondary to renal sarcoidosis with GIN. Her disease subsequently recurred in the transplanted kidney despite standard immunosuppression with prednisone, tacrolimus, and mycophenolate mofetil. The recurrent disease appeared to respond to increased immunosuppression, which included infliximab. However, the patient died of disseminated histoplasmosis three yr post-transplant.

CLEFT-Q: Detecting Differences in Outcomes among 2434 Patients with Varying Cleft Types.

BACKGROUND: Measuring the patient perspective is important in evaluating outcomes of cleft care. Understanding how treatment outcomes vary depending on cleft type may allow for better planning of treatments, setting of expectations, and more accurate benchmarking efforts. The CLEFT-Q is a patient-reported outcome measure for patients with cleft lip and/or palate. METHODS: The 12 CLEFT-Q scales measuring appearance (i.e., face, nose, nostrils, lips, cleft lip scar, teeth, and jaws), function (i.e., speech), and health-related quality of life (i.e., psychological, school, social, and speech-related distress) were field tested in a cross-sectional study in 30 centers in 12 countries. Patients with cleft lip and/or cleft palate aged 8 to 29 years were recruited from clinical settings. Differences in CLEFT-Q scores by cleft subtypes were evaluated using one-way analysis of variance or Kruskal-Wallis H tests, with Tukey or Dunn procedure with Bonferroni corrections post hoc analyses, respectively. Scores are presented using radar charts to visualize all outcomes simultaneously. RESULTS: The field test included 2434 patients. Scores on all CLEFT-Q scales varied significantly with cleft subtype. Patients with unilateral or bilateral cleft lip and/or palate scored lower on all appearance scales compared with patients with cleft palate or unilateral incomplete cleft lip. Scores on the speech function and speech-related distress scales decreased with each progressive group in the Veau classification. Patients with complete bilateral cleft lip and palate scored lowest on the social, school, and psychological scales. CONCLUSIONS: Patient-reported outcomes measured with the CLEFT-Q vary significantly with cleft type. Visualizing multiple outcomes simultaneously with radar charts allows for an understanding of a patient's overall status in a single graph.