Clinical, histomorphological and therapeutic features of the Van der Woude Syndrome: literature review and presentation of an unusual case.

BACKGROUND: Van der Woude syndrome (VWS), an autosomal dominant condition associated with lower lip pits and/or cleft palate, is caused by mutations in the interferon regulatory factor 6 gene (lRF6 gene). The genetic alterations identified to date that contribute to expression of the syndrome are chiefly mutations located on chromosome 1 (the largest of our chromosomes), mutations at p36 that codifies the gene GRHL (grainy-head transcriptor factor) and mutations involving IRF6 (interferon regulatory factor). With frequency ranging from 1:35,000 to 1:100,000, depending on ethnicity, gender, and socio-economic status, the syndrome accounts for about 2% of orofacial clefts. The clinical and histomorphological aspects of VWS are studied, and a case of heterozygous female twins of whom only one was affected with VWS is reported. CONCLUSION: This very rare case (no similar case has been reported to date) contributes further evidence on modifying factors in the expression of this condition.

Is ionized calcium a reliable predictor of hypocalcemia after total thyroidectomy? A before and after study.

Wanting to find a way of identifying patients suitable for early discharge after thyroidectomy, we set out to establish whether ionized calcium concentration is a better predictor of post-surgical hypocalcemia than total serum calcium. Data were analyzed to establish whether serum ionized calcium concentrations are correlated with total serum calcium levels and symptomatic hypocalcemia after thyroidectomy. Sixty-two patients undergoing total thyroidectomy at the Department of Surgical Sciences of the "Sapienza" University of Rome, Italy, in 2010. Ionized calcium was measured before (day 0) and after surgery (days 1, 2 and 60) in all the patients. These measurements were compared with preoperative (day 0) and postoperative total serum calcium levels (days 1, 2 and 60). The preoperative ionized calcium levels differed from the ionized calcium levels recorded on days 1 and 2; this pattern was not observed for the total calcium concentrations. Conversely, total calcium on days I and II correlated significantly with the various ionized calcium measurements. The presence of parathyroid glands in the surgical specimen did not seem to affect suitability for discharge. The statistical analysis showed that ionized calcium measurements are more reliable than total calcium measurements in the immediate and long-term follow-up of total thyroidectomy patients. Applying a 95% confidence interval we established reference values for both total serum calcium and ionized calcium, below which all patients develop postoperative symptomatic hypocalcemia. In conclusion, measurement of ionized calcium, as opposed to total calcium, should be strongly recommended in the immediate and longterm follow-up of total thyroidectomy patients.

Association between oral and fecal microbiome dysbiosis and treatment complications in pediatric patients undergoing allogeneic hematopoietic stem cell transplantation.

The oral and gastrointestinal mucosae represent the main targets of the toxic effect of chemo and/or radiotherapy administered during the conditioning regimen before hematopoietic stem cell transplant (HSCT). These harmful consequences and the immunological complications that may occur after the transplant (such as Graft versus Host Disease, GvHD) are responsible for the clinical symptoms associated with mucositis during the aplasia phase, like pain, nausea, vomiting, and diarrhea. These toxicities could play a critical role in the oral and gastrointestinal microbiomes during the post-transplant phase, and the degree of microbial dysbiosis and dysregulation among different bacterial species could also be crucial in intestinal mucosa homeostasis, altering the host's innate and adaptive immune responses and favoring abnormal immune responses responsible for the occurrence of GvHD. This prospective pediatric study aims to analyze longitudinally oral and gut microbiomes in 17 pediatric patients who received allogeneic HSCT for malignant and non-malignant diseases. The oral mucositis was mainly associated with an increased relative abundance of Fusobacteria, and Prevotella species, while Streptococcus descendants showed a negative correlation. The fecal microbiome of subjects affected by cutaneous acute GvHD (aGvHD) correlated with Proteobacteria. Oral mucosal microbiota undergoes changes after HSCT, Fusobacteria, and Prevotella represent bacterial species associated with mucositis and they could be the target for future therapeutic approaches, while fecal microbiome in patients with acute GvHD (aGvHD) revealed an increase of different class of Proteobacteria (Alphaproteobacteria and Deltaproteobacteria) and a negative correlation with the class of Gammaproteobacteria.

Duplex ultrasound and magnetic resonance imaging of the supra-aortic arches in patients with non recurrent inferior laryngeal nerve: a comparative study.

BACKGROUND: Non-recurrent inferior laryngeal nerve (NRILN) is usually discovered during thyroid surgery. It is often associated with vascular abnormalities that can be detected with magnetic resonance imaging (MRI) or duplex ultrasound scan. The aim of this study was to compare the diagnostic sensitivity of ultrasonography with MRI to identify the vascular abnormalities associated to NRILN. PATIENTS AND METHODS: We revised 2713 total thyroidectomies to select patients with NRILN. The NRILN was identified in 17 patients (0,6%). A postoperative ultrasonic duplex scanning and a MRI was performed in 15 cases as 2 patients refused to submit to the exams. RESULTS: At MRI an unique origin of common carotid trunk and a concomitant aberrant retroesophageal subclavian right artery was showed in 11 patients. In 2 cases vascular abnormality consisted in separated origin of supra-aortic arteries. At duplex ultrasound scan only in 2 patients was impossible to identify vascular abnormalities detected at MRI. Tthe diagnostic sensitivity of duplex ultrasound was 84,6%. CONCLUSIONS: Preoperative duplex ultrasound is a non invasive method with high diagnostic sensitivity that can easily complete the preoperative thyroid ultrasonography.

Complex odontoma at the upper right maxilla: Surgical management and histomorphological profile.

BACKGROUND: Odontomas are hamartomatous developmental malformations of the dental tissues. Usually asymptomatic, their presence is often revealed on routine radiographs. The study aimed to establish the efficacy of this conventional approach in treating odontomas, analysing clinical outcome, follow-up, and histomorphological profile. CASE REPORT: A case is presented with a review of the international literature. The patient, aged 8 years, had a complex odontoma localised on the front upper jaw. She was treated following the conventional surgical procedure. Post-operative course and healing were uneventful. Orthodontic treatment was necessary to realign the teeth. At the 12-month follow-up there was no recurrence or failure. Healing was excellent. CONCLUSION: Variations in normal tooth eruption are a common finding, but significant deviations from established norms should alert the clinician to further investigate the patient's health and development.

Mitochondrial DNA variability modulates mRNA and intra-mitochondrial protein levels of HSP60 and HSP75: experimental evidence from cybrid lines.

To explore possible relationships between mitochondrial DNA (mtDNA) polymorphism and the expression levels of stress-responder nuclear genes we assembled five cybrid cell lines by repopulating 143B.TK(-) cells, depleted of their own mtDNA (Rho(0) cells), with foreign mitochondria with different mtDNA sequences (lines H, J, T, U, X). We evaluated, at both basal and under heat stress conditions, gene expression (mRNA) and intra-mitochondrial protein levels of HSP60 and HSP75, two key components in cellular stress response. At basal conditions, the levels of HSP60 and HSP75 mRNA were lower in one cybrid (H) than in the others (p = 0.005 and p = 0.001, respectively). Under stress conditions, the H line over-expressed both genes, so that the inter-cybrid difference was abolished. Moreover, the HSP60 intra-mitochondrial protein levels differed among the cybrid lines (p = 0.001), with levels higher in H than in the other cybrid lines. On the whole, our results provide further experimental evidence that mtDNA variability influences the cell response to stressful conditions by modulating components involved in this response. Sentence summary of the article: the results reported in the present study provide important experimental evidence that in human cells mtDNA variability is able to influence the cellular response to heat stress by modulating both the transcription of genes involved in this response and their intra-mitochondrial protein levels.

Randomized study on the effects of different strategies of intermittent pneumatic compression for lower limb claudication.

BACKGROUND: The aim of the present study was to evaluate the efficacy of different strategies of intermittent pneumatic compression (IPC) for the treatment of lower limb claudication. METHODS: Five study groups were prospectively studied. Group 1: 9 patients not undergoing IPC; Group 2; six patients undergoing IPC 1 hour/thrice-a-day/4 months; Group 3: six patients undergoing IPC 2 hours/once-a-day/4 months; Group 4; six patients undergoing IPC 1 hour/thrice-a-day/2 months; Group 5: six patients undergoing IPC 2 hours/once-a-day/2 months. RESULTS: All patients completed the planned treatment schedule and stated a compliance of 33% in group 2, 83% in group 3, 66% in group 4 and 100% in group 5. Peak systolic velocity of the popliteal artery blood flow increased over baseline values particularly when IPC lasted 4 months (group 2: 85%, group 3: 81% vs. group 4: 76%, group 5: 73%). These beneficial effects lasted 10 months and vanished 14 months after the end of IPC treatment. The absolute claudication distance increased at the end of the treatment of 101% in group 2, 94% in group 3, 86% in group 4, and 83% in group 5, and it was still increased over the baseline values 14 months after the end of the treatment. No differences have been observed whether the treatment was performed once- or thrice-a-day. CONCLUSIONS: ICP treatment performed two hours once-a-day for four months provide excellent results with satisfactory treatment compliance. However, these effects are not durable and vanish about one year after the end of IPC treatment.

Uni-centric localization of Castleman's disease treated with laparoscopic and traditional approach. Report of two cases.

Castleman's disease (CD) is a rare lymphoproliferative disorder. Clinically CD has been subdivided in two forms: uni-centric and multicentric. The uni-centric type is limited to a single anatomic lymph-node-bearing region. The present report describes two cases of uni-centric CD: the first was an abdominal localization treated with a laparoscopic approach; the second was a submaxillary localization treated with a classical approach. In case 1 the laparoscopic approach permitted to reach diagnosis, not clear after diagnostic imaging procedures, and enabled a total and excellent resolution of the pathology because our patient, after eight months of follow up, has had no evidence of recurrence of the disease. In case 2 we want to highlight that CD should be considered in the differential diagnosis of a solitary neck mass and that the surgical treatment is diagnostic and curative at the same time.

Educación alimentaria en pacientes en dialisis / Nutritional education in dialysis patients

La enfermedad renal crónica terminal aumenta el riesgo cardiovascular y puede ocasionar defectos en la mineralización ósea. Para prevenir esto, se debe mantener el fósforo plasmático normal, que depende de la diálisis, los quelantes y la ingesta de fósforo, principalmente de origen inorgánico, incorporado mediante aditivos alimentarios. Las intervenciones nutricionales son pilares en el tratamiento de estos pacientes. El objetivo es facilitar estrategias alimentarias a un grupo de pacientes pediátricos en diálisis, mediante educación alimentaria nutricional, para aumentar el consumo de alimentos naturales, disminuyendo la ingesta de fósforo inorgánico especialmente de los productos cárnicos procesados. Materiales y métodos: se estudió una población pediátrica en diálisis. Se preparó un programa educativo con atención personalizada, instrucción alimentaria y seguimiento mensual, seguido de un taller. Resultados: n: 17 pacientes, edad decimal media de 12,3, 53% sexo masculino, 88% en hemodiálisis. Previo a la intervención el 64,7% consumía productos cárnicos procesados. Luego del taller el 58,8% disminuyó su consumo, el 41,2% aumentó la ingesta de preparaciones caseras, el 53% incorporó nuevos condimentos, de los cuales el 89% presentó al incorporarlos, mejor aceptación a las preparaciones. Conclusiones: la hiperfosfatemia está presente en alrededor del 50% de los pacientes en diálisis asociándose a un incremento entre 20% al 40% del riesgo de mortalidad. La presencia de fósforo oculto en los alimentos y la falta de adherencia hacen prioritario trabajar en programas educativos que favorezcan el aprendizaje colaborativo, centralizados en prácticas culinarias, para brindar herramientas que faciliten una alimentación natural, disminuyendo el consumo de ultraprocesados (AU)

Chronic end-stage renal disease increases the risk of cardiovascular disease and may lead to defects in bone mineralization. In order to prevent these risks, normal plasma phosphorus levels should be maintained. Achieving this goal depends on dialysis, chelators, and phosphorus intake, mainly of inorganic origin, incorporated through food supplements. Nutritional interventions are crucial in the treatment of these patients. The objective is to facilitate nutritional strategies to a group of pediatric dialysis patients, through food education, to increase the consumption of natural foods, decreasing the intake of inorganic phosphorus, especially from processed meat products. Materials and methods: a pediatric population undergoing dialysis was studied. An educational program was prepared with personalized care, nutritional instruction, and monthly follow-up visits, followed by a workshop. Results: n: 17 patients, mean age 12.3 years, 53% male, 88% on hemodialysis. Prior to the intervention, 64.7% consumed processed meat products. After the workshop, 58.8% decreased their consumption, 41.2% increased the intake of homemade food, 53% incorporated new seasonings, of whom 89% reported better acceptance of the preparations when they were incorporated. Conclusions: hyperphosphatemia is observed in around 50% of patients undergoing dialysis and is associated with a 20% to 40% increased risk of mortality. The presence of hidden phosphorus in food and the lack of adherence point to the need for the development of educational programs that promote collaborative learning, focusing on food-preparation practices. These programs should provide tools that facilitate a natural diet, reducing the consumption of ultra-processed food (AU)

Role of circulating endothelial progenitor cells in the reparative mechanisms of stable ischemic myocardium.

BACKGROUND: Mobilization of endothelial progenitor cells (EPCs) into circulation from bone marrow in patients with acute myocardial infarction has strong scientific evidence; less is known about EPC mobilization in patients with stable coronary artery disease (CAD). The aim of this study was to investigate the association of stable ischemic heart disease with EPC levels in tissue and blood. METHODS: Fifty-five consecutive patients admitted to a single treatment center for valve or coronary artery bypass grafting (CABG) surgeries were included in the study. Blood samples were collected in the morning before surgery and analyzed by flow-cytometry to determine peripheral EPC levels (EPC/ml). Tissue EPC (CD34+VEGFR2+) levels were assessed on a right atrial appendage segment. RESULTS: Mean age was 76±5years, 48% were men, and 53% had CAD The number of CD34+ VEGFR2+ cells in the tissue of patients with CAD was significantly higher (p<0.005) and circulating EPC showed a tendency to be reduced by approximately 20% in peripheral blood of patients with CAD when compared to those without CAD. CONCLUSION: Patients with stable CAD had higher EPC density values (EPC/mm2) and were more likely to have lower EPC blood levels when compare with normal controls.

Spontaneous and inoculated yeast populations dynamics and their effect on organoleptic characters of Vinsanto wine under different process conditions.

Evolution of the microbial composition during the production of Vinsanto wine was investigated under different fermentation conditions to determine their impact on the yeast population and the wine sensorial characteristics. Fermentations were carried out according to the traditional process in 50-l barrels. Different fermentation conditions were applied (yeast inoculum, "mother sediment" addition and temperature) to standardise the Vinsanto production process. Fermentations and products were monitored over the aging period by chemical, microbial and sensory evaluation. The low temperature at the beginning of the fermentations under traditional cellar conditions results in prolonged survival of the non-Saccharomyces yeast. In contrast, Saccharomyces yeast populations dominated throughout the fermentation when the temperature of the cellar was maintained at a constant 16-18 degrees C. Results indicate that inoculation with a commercial yeast strain and fermentation temperature strongly influence the evolution of Vinsanto wine. The "mother sediment" seems to have no direct role as a microbiological starter in Vinsanto production but, despite this, it does have a strong influence on the sensory attributes of the Vinsanto wine. Our work highlights the importance of managing the fermenting microflora to improve the sensorial characteristics of Vinsanto wine.

Evaluación de la técnica de conteo de grasas y ajuste de enzimas pancreáticas en un grupo de pacientes con insuficiencia pancreática secundaria a fibrosis quística con seguimiento en un hospital de alta complejidad / Evaluation of the fat counting technique and pancreatic enzyme adjustment in a group of patients with pancreatic insufficiency secondary to cystic fibrosis followed-up in a tertiary-level hospital

Objetivo: evaluar la efectividad de la técnica de conteo de grasas y ajuste de enzimas pancreáticas en un grupo de pacientes con insuficiencia pancreática secundaria a fibrosis quística (FQ). Materiales y métodos: En un grupo de pacientes con FQ, sin otra patología asociada, mayores de 1 año, con >10 000 UKD (unidades por kilo por día) de lipasa; se realizó educación y aplicación de técnica de conteo de grasas con ajuste enzimático, solicitando Van de Kamer y registro alimentario de 5 días durante la recolección de la muestra con un intervalo de 3 meses entre ambas determinaciones. Se evaluó la efectividad de la misma y las dosis de enzimas utilizadas mediante el porcentaje de excreción grasa (PEG), así como las variaciones en la cantidad de enzimas utilizadas y la ganancia de peso. Los datos se registraron en RED Cap (Research Electronic Data Capture) y se analizaron mediante Stata 12. Resultados: De un total de 21 pacientes, 16 completaron la intervención. El 50% presentó un índice de masa corporal (IMC) mayor del Plo 25 antes y después, un 87% alcanzó adecuación calórica mayor del 120% de la ingestas diarias recomendadas (RDA) al final, logrando un aumento promedio de z score de peso de 0,28 con una media inicial de 17 kg y final de 18,2 kg. En cuanto a la media del requerimiento enzimático fue de 14 800 UKD antes y 10 145 UKD después (z=0,002), asimismo el porcentaje de excreción grasa (PEG) tuvo una disminución del 38% (p=0,1705). Conclusiones: La implementación de la técnica de conteo de grasas y ajuste enzimático, podría ser una estrategia válida para aquellos pacientes con FQ que tienen dosis altas de enzimas e inadecuada ganancia de peso (AU)

Objective: To evaluate the effectiveness of the fat counting technique and pancreatic enzyme adjustment in a group of patients with pancreatic insufficiency secondary to cystic fibrosis (CF). Materials and methods: A group of patients with CF without other associated diseases, older than 1 year of age, lipase dose >10 000 UKD (units per kilo per day), received education on the fat counting technique with enzyme adjustment followed by its implementation of the intervention. Van de Kamer was requested and a 5-day food record was kept during the sample collection with an interval of 3 months between both measurements. The effectiveness of the technique and the enzyme doses used were evaluated based on the percentage of fat excretion (PFE), as well as the variations in the amount of enzymes used and weight gain. Data were recorded in RED Cap (Research Electronic Data Capture) and analyzed using Stata 12. Results: Of a total of 21 patients, 16 completed the intervention. Fifty percent had a body mass index (BMI) greater than Plo 25 before and after the intervention; 87% had achieved a caloric increase greater than 120% of the recommended daily intake (RDA) at the end of the study and an average increase in weight z score of 0.28 with an initial mean of 17 kg and a final mean of 18.2 kg. Mean enzyme requirement was 14 800 UKD before and 10 145 UKD after the intervention (z=0.002). PFE decreased by 38% (p=0.1705). Conclusions: The implementation of the technique of fat counting and enzyme adjustment may be a valid strategy for CF patients with high enzyme doses and inadequate weight gain. (AU)

Situación nutricional y acciones implementadas en niños internados en salas de cuidados intermedios y moderados de un hospital pediátrico de alta complejidad de la ciudad de Buenos Aires / Nutritional status and actions implemented in children admitted to intermediate and moderate care wards of a tertiary-care pediatric hospital in the city of Buenos Aires

Introducción: La malnutrición es un estado de deficiencia o exceso de nutrientes que provoca efectos nocivos y puede alterar el crecimiento aumentando la morbi-mortalidad. Materiales y métodos: estudio retrospectivo, descriptivo. Incluyó niños/as de 1-18 años hospitalizados entre 2016-2018. Se obtuvieron datos de caracterización de la muestra y antropométricos. La herramienta de tamizaje nutricional pediátrico (HTNP) se utilizó para detectar riesgo nutricional y en este subgrupo se analizó: variación de peso, intervención nutricional, complicaciones infecciosas y estadía hospitalaria. El análisis de variables se realizó con SPSS Statistics 20. Resultados: Se evaluaron 745 pacientes, 373 niñas (50,1%). Mediana de edad 7,3 años. Estancia hospitalaria media de 4 días (1-123). Se observó 5,9% emaciados, 56,4% eutróficos, 16,8% sobrepeso y 20,9% obesidad. Con baja talla 13%. Se detectó riesgo nutricional con HTNP en 50,7% de los ingresos. Las patologías de base más frecuentes fueron cardiopatías y neoplasias. En pacientes con riesgo nutricional: estadía hospitalaria media de 5 días, 13,5% cursó con infecciones intrahospitalarias, 68% mantuvo o aumentó de peso durante la internación, 13,5% requirió apoyo nutricional (más utilizado el gavage en 59%). Conclusiones: El niño hospitalizado se encuentra en una situación de vulnerabilidad, por lo que el tamizaje y evaluación nutricional resultan acciones claves para prevenir el deterioro nutricional. En los niños con malnutrición las acciones llevadas a cabo por el Nutricionista Clínico como integrante del equipo de atención, revisten un rol clave para promover y garantizar el derecho de los pacientes a la alimentación adecuada y así mejorar su condición nutricional. (AU)

Introduction: Malnutrition is a state of nutrient deficiency or excess that causes harmful effects and can alter growth increasing morbidity and mortality. Materials and methods: retrospective, descriptive study. Children aged 1-18 years admitted to the hospital between 2016-2018 were included. Sample characterization and anthropometric data were collected. The pediatric nutritional screening tool (PNST) was used to identify nutritional risk and in this subgroup we analyzed: weight variation, nutritional intervention, infectious complications, and length of hospital stay. The analysis of variables was performed with SPSS Statistics 20. Results: 745 patients were evaluated, 373 were girls (50.1%). Median age was 7.3 years. Mean hospital stay was 4 days (1- 123). Among the patients, 5.9% were emaciated, 56.4% eutrophic, 16.8% overweight, and 20.9% obese. Thirteen percent of the patients had short stature. Nutritional risk was detected using HTNP in 50.7% of the admitted patients. The most frequent underlying diseases were heart disease and cancer. In patients at nutritional risk: mean hospital stay was 5 days, 13.5% had hospital-acquired infections, 68% maintained or gained weight during the hospital stay, 13.5% required nutritional support (gavage was the most frequently used in 59%). Conclusions: Hospitalized children are in a vulnerable situation, therefore nutritional screening and evaluation are key actions to prevent nutritional deterioration. In children with malnutrition, the Clinical Nutritionist, as a member of the health care team, plays a key role in promoting and guaranteeing the right of patients to adequate food and thus improve their nutritional condition (AU)

[Acquired rectourethral fistula: case report]. / Fistola retto-uretrale acquisita: a proposito di un caso.

Rectourethral fistulas are an uncommon complication of urinary or rectal surgery, trauma, inflammatory disease, radiation therapy for prostate cancer; they represent an unique challenge for the surgeon. Although closure can occure spontaneously, most cases of acquired rectourethral fistula need surgical repair. Despite a century of surgical experience, no single approach has been universally accepted. We report a case of a rectourethral fistula occurred in a 73 year-old man after a radical retropubic prostatectomy and external beam irradiation for prostate cancer, successfully treated with perineal approach.

[Gastric lipoma presenting as intestinal obstruction]. / Occlusione intestinale alta da lipoma gastrico.

Gastric lipoma is a rare benign tumor. The symptoms are correlated with the size and the dimensions of neoplasm. It can be the cause of bleeding, gastroduodenal intussusception and intestinal obstruction, as in case reported and surgically treated. The Authors make a literature review to define the better diagnostic and surgical approach.

Tumor-induced hypophosphatemic osteomalacia associated with tertiary hyperparathyroidism: a case report.

BACKGROUND: Tumor-induced hypophosphatemic osteomalacia is a syndrome characterized by urinary phosphate wasting related to the presence of a slowly-growing tumor of mesenchymal origin. The characteristic laboratory findings are normal serum calcium, marked hypophosphatemia, increased serum alkaline phosphatase, markedly reduced renal tubular reabsorption of phosphorus and inappropriately low levels of 1,25-dihydroxyvitamin D [1,25-(OH)2D]. CASE PRESENTATION: A 65-year-old woman presented with a 17-year clinical history of musculoskeletal pain, muscular weakness in the pelvic girdle, spontaneous fractures and difficulty in walking. Over the ensuing years the patient suffered other multiple spontaneous fractures, surgically treated, and the muscular pains worsened until she became bedridden. During the years before hospital admission the patient received treatment with clodronate, oral calcium salts and vitamin D therapy. Standard laboratory, ultrasonography and scintigraphic findings provided a "convenient" diagnosis of primary hyperparathyroidism, but the low plasma level of phosphorus induced to perform an Indium111-octreotide scintigraphy. Scintigraphy visualized an area of pathologic increased signal uptake in the left groin, consistent with a mass containing a high density of somatostatin receptors. After surgery, histologic examination and immunostaining of the resected specimen indicated an hemangiopericytoma. Nevertheless, the persistently low blood phosphorus level, in association with the increased serum calcium and PTH levels, were attributed to the prolonged phosphate therapy the patient underwent over the years, and the persisting abnormal laboratory indexes indicated the development of a tertiary hyperparathyroidism. We performed a subtotal parathyroidectomy and intraoperative assay of serum PTH showed that levels had diminished by more than 80% from preoperative values. Over the ensuing months Ca+2, PTH and serum phosphorus values returned to normal, and the pain symptoms disappeared. CONCLUSIONS: Tumour-induced osteomalacia is a very rare syndrome associated in 5% of cases with tertiary hyperparathyroidism due to long-term therapy with phosphorus and vitamin D. The initial diagnosis of primary hyperparathyroidism, confirmed by the parathyroid MIBI-scintigraphy, would lead us to an inappropriate surgical treatment. Therefore we want to stress the importance of In111-octreotide scintigraphy in detecting tumours, rich in somatostatin receptors, in presence of an hypophosphatemic syndrome.

[Gangrene of Meckel's diverticulum in strangulated left inguinal hernia]. / Ernia inguinale sinistra strozzata con necrosi d'ansa e di diverticolo di Meckel.

We report a case of a 57-year-old woman admitted for abdominal pain and a not reducible mass in left inguino-abdominal region. With a diagnosis of strangulated inguinal hernia, the patient underwent urgent surgery. The surgical exploration showed a gangrenous intestinal loop with a Meckel's necrotic diverticulum. A small bowel resection (20 cm) was performed. The post-operative course was uneventful. This seems the first case reported in the literature of woman with a Meckel's diverticulum involved in a strangulated left inguinal hernia.

Tamizaje de riesgo nutricional en el niño hospitalizado: validación de una herramienta elaborada en un hospital de alta complejidad de Ciudad de Buenos Aires / Nutritional risk screening in hospitalized children: validation of a tool developed at a tertiary hospital in the City of Buenos Aires

Introducción: El niño hospitalizado se encuentra en estado de vulnerabilidad nutricional. El tamizaje nutricional permite identificar malnutrición y/o el riesgo de desarrollarla, para realizar un abordaje precoz. El Área de Alimentación del Hospital Garrahan desarrolló la "Herramienta de Tamizaje Nutricional Pediátrico" (HTNP), dada la falta de consenso sobre un estándar de oro y para ajustar criterios a la población asistida. El objetivo de esta investigación fue su validación para niños en cuidados intermedios/ moderados. Métodos: estudio prospectivo, descriptivo y transversal. Entre agosto de 2016 y abril de 2018 se reclutaron niños de 1 a 18 años internados en salas de cuidados intermedios/moderados seleccionadas, que cumplieran los criterios de inclusión. Se aplicaron: la HTNP y la valoración nutricional global subjetiva (VNGS) como prueba de comparación. La HTNP consta de tres criterios: Patología de base y motivo de internación- Disminución de peso- Deterioro de la actitud alimentaria. Se define riesgo nutricional si se cumple con dos criterios. Se analizaron: Sensibilidad, especificidad, valor predictivo positivo (VPP) y valor predictivo negativo (VPN), factibilidad y reproducibilidad. Resultados: Se evaluaron 745 niños (50,1% sexo femenino; mediana de edad: 7,2 años). La HTNP detectó riesgo nutricional en 50,7% (n378) de los niños y la VNGS en 48,7% (n363). La HTNP presentó: Sensibilidad 87,3% (IC95% 83,8-90,9), Especificidad 84,0% (IC95% 80,2-87,8), VPP 83,9% (IC95%: 80,0­87,7) y VPN 87,5% (IC95%: 83,9-91,0). Del análisis de reproducibilidad con dos evaluadores independientes (n42) se obtuvo coeficiente kappa de 0,91 (0,74-1,0) y 0,78 (0,5-1,0) respectivamente. Su implementación llevo un promedio de tres minutos y medio (1-5 minutos). Conclusión: La HTNP es un instrumento simple, reproducible, práctico y factible de implementar para identificar pacientes en riesgo nutricional (AU)

Introduction: Hospitalized children are nutritionally vulnerable. Nutritional screening may identify malnutrition and/or the risk of developing malnutrition in order to start early intervention. The Food Services Area of Garrahan Hospital has developed a "Pediatric Nutritional Screening Tool (PNST) because of the lack of consensus on a gold standard and to finetune the criteria to the care population. The aim of this study was to validate the tool in children in intermediate/moderate care. Methods: A prospective, descriptive, cross-sectional study was conducted. Between August 2016 and April 2018 children from 1 to 18 years of age who met the inclusion criteria were enrolled on selected intermediate/ moderate care wards. The PNST was administered together with the Subjective Global Assessment (SGA) as a comparison test. The PNST consists of three criteria: Underlying disease and reason for admission- Weight loss- Deterioration of eating behavior. A patient was defined as being at nutritional risk was defined if two criteria were met. Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV), feasibility, and reproducibility were analyzed. Results: 745 children (50.1% female; median age: 7.2 years) were assessed. The PNST detected nutritional risk in 50.7% (n378) and the SGA in 48,7% (n363) of the children. The PNST showed: Sensitivity 87.3% (95%CI: 83.8-90.9), specificity 84.0% (95%CI: 80.2-87.8), PPV 83.9% (95%CI: 80.0­87.7), and NPV 87.5% (95%CI: 83,9-91,0). In a reproducibility analysis with two independent evaluators (n42) kappa coefficients of 0.91 (0.74-1.0) and 0.78 (0.5-1.0) were obtained, respectively. Administration of the tool took a mean of 3.5 inutes (1-5 minutes). Conclusion: The PNST is a simple, reproducible, practical, and feasible tool to use for the identification of patients at nutritional risk (AU)

Prevalence of chronic cerebrospinal venous insufficiency in multiple sclerosis: a blinded sonographic evaluation.

OBJECTIVES: To verify the prevalence of chronic cerebrospinal venous insufficiency in patients affected by different clinical forms of multiple sclerosis and in healthy subjects using the Zamboni ultrasound protocol combined with M-mode ultrasound examination. MATERIALS AND METHODS: We enrolled 112 patients with multiple sclerosis and 67 healthy subjects from 20 to 67 years of age. All the patients underwent Duplex and color-Doppler sonography of the neck vessels, transcranial colour duplex sonography, M-mode study of the valve system and of venous abnormalities. Subjects were positive for chronic cerebrospinal venous insufficiency when at least two of five hemodynamic criteria of the Zamboni protocol were fulfilled. Chronic cerebrospinal venous insufficiency condition was further analyzed by a multivariate analysis including age, sex, disease duration, subtypes of multiple sclerosis and expanded disability status scale score as independent variables. RESULTS: No healthy subjects was positive for chronic cerebrospinal venous insufficiency, while in the sample of patients affected by multiple sclerosis the diagnosis was made in 59.8% of cases (p < 0.0001). The first criterion was the most frequent in patients affected by multiple sclerosis and chronic cerebrospinal venous insufficiency (respectively 54.4% and 76.1%, p < 0.001). The second, third and fourth criteria were never present in healthy subjects but were detected in patients with multiple sclerosis. The positivity of the second criterion was associated with diagnosis of chronic cerebrospinal venous insufficiency in 100% of cases. The third criterion had a prevalence of 52.2% in the subgroup of chronic cerebrospinal venous insufficiency patients. It was positive in 36 multiple sclerosis patients and was associated with chronic cerebrospinal venous insufficiency diagnosis in all cases except one. The multivariate analysis showed that age, disease duration, sex, subtypes of multiple sclerosis and expanded disability status scale score were not considered predictors of this haemodynamic condition. CONCLUSION: Chronic cerebrospinal venous insufficiency is a haemodynamic condition strongly associated with multiple sclerosis and is not found in normal controls. The addition of M-mode ultrasound to the diagnostic protocol allows improved observation of venous valve abnormalities.