Use of a mobile application for self-management of pancreatic enzyme replacement therapy is associated with improved gastro-intestinal related quality of life in children with Cystic Fibrosis.

BACKGROUND: Most patients with cystic fibrosis (CF) suffer from pancreatic insufficiency (PI), leading to fat malabsorption, malnutrition, abdominal discomfort and impaired growth. Pancreatic enzyme replacement therapy (PERT) is effective, but evidence based guidelines for dose adjustment are lacking. A mobile app for self-management of PERT was developed in the context of the HORIZON 2020 project MyCyFAPP. It contains an algorithm to calculate individual PERT-doses for optimal fat digestion, based on in vitro and in vivo studies carried out in the same project. In addition, the app includes a symptoms diary, educational material, and it is linked to a web tool allowing health care professionals to evaluate patient's data and provide feedback. METHODS: A 6-month open label prospective multicenter interventional clinical trial was performed to assess effects of using the app on gastro-intestinal related quality of life (GI QOL), measured by the CF-PedsQL-GI (shortened, CF specific version of the Pediatric Quality of Life Inventory, Gastrointestinal Symptoms Module). RESULTS: One hundred and seventy-one patients with CF and PI between 2 and 18 years were recruited at 6 European CF centers. Self-reported CF-PedsQL-GI improved significantly from month 0 (M0) (84.3, 76.4-90.3) to month 6 (M6) (89.4, 80.35-93.5) (p< 0.0001). Similar improvements were reported by parents. Lower baseline CF-PedsQL-GI was associated with a greater improvement at M6 (p < 0.001). CONCLUSIONS: The results suggest that the MyCyFAPP may improve GI QOL for children with CF. This tool may help patients to improve self-management of PERT, especially those with considerable GI symptoms.

Rectal advancement flap plus adipose lipofilling (RAFAL) for the treatment of rectourethral fistulas after radical prostatectomy.

BACKGROUND: Rectourethral fistula (RUF) is a rare but significant complication after radical prostatectomy. Many different approaches have been used, but none of them has become the standard of care. METHODS: We present our series of seven patients treated with a transanal rectal advancement flap plus the injection of mesenchymal stem cells, to facilitate the healing of the fistula. Mesenchymal cells were obtained by a new mechanical device known as LIPOGEMS®. We called this technique RAFAL (rectal advancement flap plus adipose lipofilling). In all patients the RUF was a complication of laparoscopic radical prostatectomy. Fistula size ranged from 0.3 to 0.5 cm (median 0.4 cm). RESULTS: After a median follow-up of 53 months (range 6-163 months), 2 out of 7 patients experienced RUF recurrence. In both cases recurrence was successfully treated by the York-Mason technique in one case and by redo RAFAL in the other. Success rate of RAFAL was 71% (5 of 7). The total success rate of primary RAFAL and redo- RAFAL was 85.7% (6 of 7). No short- or long-term complications were seen. CONCLUSIONS: In our patient population this new procedure was safe and effective.

Predictors of protein-energy wasting in haemodialysis patients: a cross-sectional study.

BACKGROUND: Protein-energy wasting (PEW) is a highly prevalent condition in haemodialysis patients (HD). The potential usefulness of nutritional-inflammatory markers in the diagnosis of PEW in chronic kidney disease has not been established completely. We hypothesised that a combination of serum albumin, percentage of mid-arm muscle circumference and standard body weight comprises a better discriminator than either single marker of nutritional status in HD patients. METHODS: A cross-sectional study was performed in 80 HD patients. Patients were categorised in two groups: well-nourished and PEW. Logistic regression analysis was applied to corroborate the reliability of the three markers of PEW with all the nutritional-inflammatory markers analysed. RESULTS: PEW was identified in 52.5% of HD patients. Compared with the well-nourished patients, PEW patients had lower body mass index, serum pre-albumin and body cell mass (all P < 0.001) and higher C-reactive protein (s-CRP) (P < 0.01). Logistic regression analyses showed that the combination of the three criteria were significantly related with s-CRP >1 mg dL(-1) , phase angle <4°, and serum pre-albumin <30 mg dL(-1) (all P < 0.05). Other indicators, such as lymphocytes <20% and Charlson comorbidity index, were significantly involved (both P < 0.01). A receiver operating characteristic curve (area under the curve) of 0.86 (P < 0.001) was found. CONCLUSIONS: The combined utilisation of serum albumin, percentage of mid-arm muscle circumference and standard body weight as PEW markers appears to be useful for nutritional-inflammatory status assessment and adds predictive value to the traditional indicators. Larger studies are needed to achieve the reliability of these predictor combinations and their cut-off values in HD patients and other populations.

Hipertrigliceridemia secundaria a suplementación nutricional en enfermedad renal crónica avanzada / Hypertriglyceridemia secondary to nutritional supplementation in advanced chronic kidney disease

La enfermedad renal crónica avanzada (ERCA, estadío 4) es el cuadro clínico que cursa con un descenso importante del filtrado glomerular (FG: < 30 mL/min). Se presenta el caso clínico de una mujer de 81 años de edad con ERCA secundaria a uropatía obstructiva crónica y diagnosticada de incoordinación muscular orofaríngea-disfagia a sólidos y parcialmente a líquidos-. En los últimos 5 años, estaba en tratamiento con suplementación nutricional exclusiva. En el inicio de Unidad ERCA, presentaba hipertrigliceridemia mantenida y aumento de la fracción VLDLc y de la GGT sin patología hepática subyacente. La modificación de la pauta de suplementación nutricional adaptada a las necesidades individuales de energía y nutrientes en ERC, y considerando la cantidad de lípidos y fuente de ácidos grasos de la fórmula permitió normalizar los niveles de triglicéridos plasmáticos, la VLDLc y la GGT, sin deterioro de la función renal y del estado nutricional (AU)

Advanced chronic kidney disease (ACKD, stage 4) is the clinical condition with a severe decrease in glomerular filtration rate (GFR < 30 mL/min). We report the case of an ACKD woman aged 81 with secondary to chronic obstructive uropathy and diagnosed oropharyngeal muscular incoordination -dysphagia to solids and liquids partially-. In the last 5 years, was treated with oral nutritional supplements exclusively. At the beginning of ACKD Unit, presented sustained hypertriglyceridemia and increased VLDLc fraction and GGT, without underlying liver disease. The modification of oral nutritional supplementation regimen tailored to individual needs energy and nutrients in ACKD, by considering the amount of lipids and fatty acid source of the formula, allowed normalize the levels of plasma triglycerides, VLDLc and GGT, without deterioration loss of renal function and nutritional status (AU)

[A case of primitive amyloid goiter]. / Gozzo amiloide primitivo. Case report.

Amyloid goiter is a rare pathology due to massive amyloid infiltration of thyroid tissue, which cause diffuse or localized enlargement of the gland. It can be totally asymptomatic or cause only non-specific symptoms (compression of adjacent structures, tracheal deviation). Thyroid disfunction (hypothyroidism or hyperthyroidism) is rare. It is important to differentiate primitive amyloid goiter from other kinds of amyloid infiltration of the thyroid, such as in systemic amyloidosis. The correct diagnosis can be difficult because of the common presence of amyloid in thyroid neoplasms, such as medullary thyroid carcinoma (MTC). The Authors present a case of primitive amyloid goiter, that needed immunohistochemical analysis for a correct diagnosis, and a review of the concerning literature.

¿Son necesarias las consultas específicas prediálisis? Objetivo: cuidado integral del paciente con Enfermedad Renal Crónica (ERC) / Are specific pre-dialysis clinical visit necessary? Objective: comprehensive care of patients with Chronic Renal disease (CRD)

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[Colorectal carcinoma metastasis in livers infected with hepatitis B or C virus]. / Metastasi epatiche da carcinoma colo-rettale in pazienti con infezione da epatite B o C.

AIM: Metastases from colorectal cancers rarely occur in injured livers, however in western countries this phenomenon has not been investigated in patients with various forms of chronic hepatitis. Therefore in this study we evaluated the incidence of synchronous hepatic metastases of colorectal carcinomas in patients with hepatitis B (HBV) or C (HBC) infection. METHODS: Six hundred and thirty patients undergoing surgical treatment for colorectal carcinomas were analysed: the clinicopathological data of 87 patients with HBV or HCV infection (there were 29 patients with hepatitis C infection and 58 with hepatitis B infection) were compared to those of 543 non infected patients. RESULTS: Patients distribution was similar in both groups in terms of gender, age, type of operative procedures performed, histological grading and lymph node metastases. Stage I, II or III tumours were similarly represented in non infected and infected patients, while stage IV tumours were 33.1% in the non infection group and 17.2% in the infection group (P < 0.001). At the time of surgery, synchronous extrahepatic metastases were present in 14.9% of non infected patients and 15% of infected patients, while synchronous hepatic metastases were found in 32% and 17.2% of patients respectively (P < 0.01). CONCLUSIONS: Results of our study show that synchronous hepatic metastases of colorectal cancers are less frequently observed in chronic HBV or HCV infected patients than in non infected patients, while the incidence of extrahepatic metastases is comparable in both groups, suggesting that virus-related mechanisms and specific liver mediated immunity may have a protective role against neoplastic cell colonization of the liver.

[Surgical treatment of breast cancer in the elderly]. / Trattamento chirurgico del carcinoma della mammella in età avanzata.

AIM: The aim of this study is to evaluate the prognosis and survival of patients aged over 70 years and affected by breast cancer. METHODS: From January 1994 through December 2000, 56 patients with breast cancer aged 70 years or older were submitted to surgical treatment. Associated diseases were present in 24 patients, while no patient showed distant metastases at the time of hospital admission. All patients underwent breast preserving surgery regardless the tumour size and in 31 subjects out of 56, the surgical procedure was performed under local anesthesia. An axillary lymphectomy was associated in 46 patients. According to the TNM staging system, tumours were classified as follows: 10 T1Nx, 18 T1N0, 9 T1N1, 7 T2N0, 10 T2N1 and 2 T3N1. RESULTS: There was no postoperative mortality and in 6 cases an axillary seroma was observed. Radiotherapy and tamoxifen treatment followed surgery in all cases. The median follow-up was 44 months. Nineteen patients (34%) died during the follow-up: 6 patients of cancer progression with a specific cancer-death of 10.7% while 13 patients (23.2%) died because of concurrent diseases. A local relapse (1.8%) was observed in a single patient 2 years after the primary surgical treatment and, at 3 years, 37 patients (66%) are alive and disease-free. Long-term survival was significantly related to the stage of disease at the time of surgery, while our data do not allow any conclusions concerning the impact of axillary dissection on long-term outcome. CONCLUSIONS: In conclusion, results for breast cancer therapy are comparable in old and young patients and therefore strategies and treatment protocols should be similar, breast preserving surgery followed by radiotherapy and ormonal treatment being ''the gold standard''.

[Occurrence of synchronous colorectal cancer metastasis in the cirrhotic or fatty liver]. / Metastasi epatiche sincrone da carcinoma colo-rettale in pazienti con cirrosi epatica o steatosi.

AIM: Metastases from colorectal cancers rarely occur in injured livers, however this phenomenon has not been fully investigated in patients with different degree of liver damage. Therefore in this study we evaluated the incidence of synchronous hepatic metastases in patients with fatty or cirrhotic liver submitted to surgery for colorectal neoplasms. METHODS: Seven hundred and forty-seven patients undergoing surgical treatment for colorectal neoplasms were evaluated: the clinicopathological data of 171 patients with liver cirrhosis and 33 with fatty liver were compared to those of 543 patients without liver damage. RESULTS: Gender, age, type of operative procedures performed and histological grading were similar in patients with or without liver damage. In patients with liver cirrhosis the incidence of stage II tumour was greater, while stage IV tumours (P < 0.001) and nodal involvement were significantly lower than in patients with non injured or fatty liver (P < 0.02 and P < 0.001 respectively) . At the time of surgery, synchronous hepatic metastases were present in 32% of patients with normal liver, in 15% of patients with fatty liver (P < 0.02) and in 4.7% of patients with liver cirrhosis (P < 0.001). CONCLUSIONS: Results of our study show that synchronous hepatic metastases of colorectal cancer are less frequently observed in patients with fatty or cirrhotic liver than in patients with non injured liver, indicating that chronically damaged livers are protected from the spread of secondary cancers.

Use of radioisotopic esophageal transit in the assessment of patients with symptoms of reflux and non-specific esophageal motor disorders.

The purposes of this study were to assess the esophageal clearance of a radioisotopic bolus in patients with symptoms of reflux and evaluate the impact of manometric abnormalities on scintigraphic esophageal transit. Esophageal clearance was assessed in a supine position and indicated by the retained radioactivity in the esophagus at 10, 20, 30 and 40 s after the ingestion of a liquid bolus labeled with 2 mCi 99 mTc-SC. The study included 214 consecutive patients with symptoms of reflux and 11 normal controls. The results were compared to the motility findings detected on manometry performed on a separate occasion. Esophageal manometry was normal in 93 patients. Nonspecific esophageal motor disorders were identified in 121 patients and were classified into: 'predominantly nonpropagated activity', 'predominantly low-amplitude peristaltic contractions' and 'miscellaneous disorders' diagnosed in 27, 47 and 47 patients, respectively. The radionuclide clearance was significantly delayed in the overall group of patients compared with that of normal controls (P < 0.001); in patients with reflux symptoms and nonspecific esophageal motor disorders compared with patients with reflux symptoms and 'normal manometry' (P < 0.01 at 20 s); and in patients with reflux symptoms and 'normal manometry' compared with the control group (P < 0.01 at 20 s). Abnormal radioisotope clearances were detected in 88% of patients with 'predominantly nonpropagated activity', in 70% of patients with 'predominantly low-amplitude peristaltic contractions' and in 57% of patients with 'miscellaneous disorders'. Radioisotopic esophageal clearance abnormalities are frequently observed in patients with reflux symptoms and are more likely to be associated to hypomotility disorders, i.e. nonpropagated motor activity or low-amplitude contractions.

[Concordance between body self-perception and BMI estimated in a population of volunteers recruited on the IV National Day of Obese People]. / Concordancia entre la autopercepción corporal y el IMC calculado en una población voluntaria captada el IV Día Nacional de la Persona Obesa.

OBJECTIVES: To assess the concordance between corporal self-perception and BMI calculated in a population of volunteers. SECONDARY OBJECTIVES: to know the percentage of overweight, normoweight and obese individuals in this population. SUBJECTS: The study includes 160 volunteers recruited at the Gómez Ulla and Severo Ochoa Hospitals in Madrid during the 4th edition of the "Obesity Day". ACTIONS: A total of 160 interviews were carried out, comprising a first group of 10 questions about how obesity is understood, how they classify themselves by self-perception (obese, overweight or normoweight individuals), the incidence of obesity in the family, living habits and the number of previous failed attempts to lose weight. In the case of a positive response to the last question, the survey is extended to 19 questions, analyzing the reasons, prescriptors, methods used, and weight loss results obtained. RESULTS: The mean Body Mass Index (BMI) of the interviewees was 26.2 +/- 4.97 kg/m2, with 27.45 +/- 5.4 kg/m2 for males and 25.76 +/- 4.7 kg/m2 for women. Overweight men represented 32.5%, with 27.3% overweight women, while obese men accounted for 25.6% and obese women amounted to 20.6%. The differences were not significant between sexes, normoweight, overweight and obesity groups. The concordance between self-perception and BMI using the kappa index was 0,464 for the total sample, 0.229 for males and 0.527 for women, and according to the valuation scale proposed by Landis and Koch, it would correspond to a moderate degree of agreement for the population in general, medium for men and moderate for women. CONCLUSIONS: Overweight and obesity affect a high percentage of the Spanish population (almost 50%). Women show greater concern about being overweight, are more likely to participate in prevention campaigns, and present a corporal perception more in line with their true weight. Preventive campaigns should be implemented to reach the male population better, taking into account that this population presents a greater cardiovascular risk. It is important to place the problem of obesity in the right terms, without any "obesophobia" leading to disorders of the eating behaviour, while indicating the importance of the risk attributable to obesity and excess weight.

Concordancia entre la autopercepción corporal y el IMC calculado en una población voluntaria captada el IV Día Nacional de la Persona Obesa / Concordance between corporal selfperception and BMI calculated in a population of volunteers recruited on the iv national day for obese people

Objetivos: Valorar la concordancia entre la autopercepción corporal y el IMC calculado en una población voluntaria. Como objetivos secundarios: conocer el porcentaje de sobrepeso, normopeso y obesidad en dicha población.Sujetos: El estudio abarca 160 sujetos voluntarios captados en los hospitales de Gómez Ulla y Severo Ochoa de Madrid durante la 4ª edición de la "Jornada de la persona obesa".Intervenciones: Se realizaron 160 encuestas que constaban de un primer grupo de 10 preguntas acerca de: la forma de entender la obesidad, cómo se clasifican ellos según su autopercepción (obesos, sobrepesos o normopesos), la incidencia de obesidad familiar, los hábitos de vida y el número de intentos previos de pérdida de peso. En el caso de una respuesta positiva a esta última pregunta la encuesta se amplía a 19 preguntas, teniendo en cuenta los motivos, fuentes de prescripción, métodos empleados y resultados obtenidos en la pérdida de peso. Resultados: El índice de masa corporal medio de los encuestados fue de 26,2 ñ 4,97 kg/m2, siendo de 27,45 ñ 5,4 kg/m2 para los varones y 25,76 ñ 4,7 kg/m2 para las mujeres. Hubo un 32,5 por ciento de varones y un 27,3 por ciento de mujeres con sobrepesos, así como un 25,6 por ciento de varones y un 20,6 por ciento de mujeres con obesidad. Las diferencias no fueron significativas entre sexos para normopeso, sobrepeso y obesidad.La concordancia entre autopercepción e IMC mediante el índice kappa fue de: 0,464 para el total de la muestra, 0,229 para varones y 0,527 para mujeres que según la escala de valoración propuesta por Landis y Koch correspondería a un grado de acuerdo moderado para la población en general, mediano para varones y moderado para mujeres.Conclusiones: El sobrepeso y la obesidad afecta a un elevado porcentaje de nuestra población (casi el 50 por ciento). Las mujeres presentan mayor preocupación por la obesidad, son más participativas en las campañas de prevención y representan una percepción corporal más concordante con su peso. Deben realizarse campañas de prevención que logren llegar mejor a la población de sexo masculino, presentando además esta población un riesgo cardiovascular mayor. Es importante situar el problema de la obesidad en su justa medida, sin "obesofobia" que induzca a trastornos de la conducta alimentaria, pero valorando el riesgo atribuible al sobrepeso y obesidad (AU)

Objectives: To assess the concordance between corporal self-perception and BMI calculated in a population of volunteers. Secondary objectives: to know the percentage of overweight, normoweight and obese individuals in this population. Subjects: The study includes 160 volunteers recruited at the Gómez Ulla and Severo Ochoa Hospitals in Madrid during the 4th edition of the “Obesity Day”. Actions: A total of 160 interviews were carried out, comprising a first group of 10 questions about how obesity is understood, how they classify themselves by self perception (obese, overweight or normoweight individuals), the incidence of obesity in the family, living habits and the number of previous failed attempts to lose weight. In the case of a positive response to the last question, the survey is extended to 19 questions, analyzing the reasons, prescriptors, methods used, and weight loss results obtained. Results: The mean Body Mass Index (BMI) of the interviewees was 26,2 ± 4,97 kg/m2, with 27,45 ± 5,4 kg/m2 for males and 25,76 ± 4,7 kg/m2 for women. Overweight men represented 32,5%, with 27,3% overweight women, while obese men accounted for 25,6% and obese women amounted to 20,6%. The differences were not significant between sexes, normoweight, overweight and obesity groups. The concordance between self-perception and BMI using the kappa index was 0,464 for the total sample, 0,229 for males and 0,527 for women, and according to the valuation scale proposed by Landis and Koch, it would correspond to a moderate degree of agreement for the population in general, medium for men and moderate for women. Conclusions: Overweight and obesity affect a high percentage of the Spanish population (almost 50%). Women show greater concern about being overweight, are more likely to participate in prevention campaigns, and present a corporal perception more in line with their true weight. Preventive campaigns should be implemented to reach the male population better, taking into account that this population presents a greater cardiovascular risk. It is important to place the problem of obesity in the right terms, without any “obesophobia” leading to disorders of the eating behaviour, while indicating the importance of the risk attributable to obesity and excess weight (AU)