Megalencephaly-capillary malformation syndrome and associated hydrocephalus: treatment options and revision of the literature.

PURPOSE: We describe our series of 4 patients with megalencephaly-capillary malformation syndrome (MCAP) and review the literature in order to assess the optimal treatment for the associated hydrocephalus. METHODS: We review our institutional series of hydrocephalus associated with MCAP and review the literature, analyzing the causes that could originate the hydrocephalus and the different types of treatments proposed for them. RESULTS: Of our patients treated with ventriculoperitoneal (VP) shunt, one suffered a surgical revision of the shunt and died due to a cranial trauma unrelated to her syndrome or the previous shunt surgery, and the other did not undergo surgical revisions until the end of her follow-up. Our patients treated with endoscopic third ventriculostomy (ETV) have improved their symptomatology and have not suffered of any complications related to the hydrocephalus after the ETV surgery. CONCLUSIONS: We update the treatment of MCAP-associated hydrocephalus and propose ETV as a valid treatment, as it seems a safe procedure with a low rate of complications.

Extractable and Non-Extractable Phenolics and Antioxidant Capacity of Mandarin Waste Dried at Different Temperatures.

The mandarin industry is generating more waste due to the increasing demand for juice. In this study, extractable and non-extractable phenolics as well as 2,2-diphenyl-1-picrylhydrazyl (DPPH), 2,2'-azino-bis(3-ethylbenzothiazoline-6-sulphonic acid) (ABTS), ferric reducing ability of plasma (FRAP), and oxygen radical absorbance capacity (ORAC) antioxidant activities in Satsuma mandarin waste dried at different temperatures were determined. The amounts of non-extractable total phenols, total flavonoids, and condensed tannins measured in mandarin waste dried at 120 °C were 39.4, 44.3, and 45.6 %, respectively, which were higher than those of fresh-mandarin waste. Dried mandarin waste is rich in extractable and non-extractable hesperidin (259.86 and 182.52 mg/g, respectively) and eriocitrin (85.12 and 197.24 mg/g, respectively), as well as non-extractable gallic acid (36.08 µg/g). The antioxidant capacities of extractable and non-extractable phenolics, from the highest to the lowest, were ABTS > ORAC > DPPH > FRAP and ORAC > ABTS > DPPH > FRAP, respectively. The information reported here may encourage mandarin industry operators to re-evaluate their by-products, extending the application of mandarin fruits and reducing waste.

Chemical composition, starch digestibility and antioxidant capacity of tortilla made with a blend of quality protein maize and black bean.

Tortilla and beans are the basic components in the diet of people in the urban and rural areas of Mexico. Quality protein maize is suggested for tortilla preparation because it presents an increase in lysine and tryptophan levels. Beans contain important amounts of dietary fiber. The objective of this study was to prepare tortilla with bean and assesses the chemical composition, starch digestibility and antioxidant capacity using a quality protein maize variety. Tortilla with bean had higher protein, ash, dietary fiber and resistant starch content, and lower digestible starch than control tortilla. The hydrolysis rate (60 to 50%) and the predicted glycemic index (88 to 80) of tortilla decreased with the addition of bean in the blend. Extractable polyphenols and proanthocyanidins were higher in the tortilla with bean than control tortilla. This pattern produced higher antioxidant capacity of tortilla with bean (17.6 µmol Trolox eq/g) than control tortilla (7.8 µmol Trolox eq/g). The addition of bean to tortilla modified the starch digestibility and antioxidant characteristics of tortilla, obtaining a product with nutraceutical characteristics.

Current recommendations for cancer surveillance in Gorlin syndrome: a report from the SIOPE host genome working group (SIOPE HGWG).

Gorlin syndrome (MIM 109,400), a cancer predisposition syndrome related to a constitutional pathogenic variation (PV) of a gene in the Sonic Hedgehog pathway (PTCH1 or SUFU), is associated with a broad spectrum of benign and malignant tumors. Basal cell carcinomas (BCC), odontogenic keratocysts and medulloblastomas are the main tumor types encountered, but meningiomas, ovarian or cardiac fibromas and sarcomas have also been described. The clinical features and tumor risks are different depending on the causative gene. Due to the rarity of this condition, there is little data on phenotype-genotype correlations. This report summarizes genotype-based recommendations for screening patients with PTCH1 and SUFU-related Gorlin syndrome, discussed during a workshop of the Host Genome Working Group of the European branch of the International Society of Pediatric Oncology (SIOPE HGWG) held in January 2020. In order to allow early detection of BCC, dermatologic examination should start at age 10 in PTCH1, and at age 20 in SUFU PV carriers. Odontogenic keratocyst screening, based on odontologic examination, should begin at age 2 with annual orthopantogram beginning around age 8 for PTCH1 PV carriers only. For medulloblastomas, repeated brain MRI from birth to 5 years should be proposed for SUFU PV carriers only. Brain MRI for meningiomas and pelvic ultrasound for ovarian fibromas should be offered to both PTCH1 and SUFU PV carriers. Follow-up of patients treated with radiotherapy should be prolonged and thorough because of the risk of secondary malignancies. Prospective evaluation of evidence of the effectiveness of these surveillance recommendations is required.

Results of induction chemotherapy in children older than 18 months with stage-4 neuroblastoma treated with an adaptive-to-response modified N7 protocol (mN7).

PURPOSE: We report the response rate in children older than 18 months with stage 4 Neuroblastoma, using a modified dose-intensive, response-adaptive, induction mN7 protocol. METHODS: From 2005 to 2012, 24 patients were treated with the mN7 protocol. Phase 1 included five MSKCC N7 cycles and surgery and two high-dose cyclophosphamide-topotecan (HD-CT) cycles for those who did not achieve complete remission (CR) and negative bone marrow (BM) minimal residual disease (MRD) status (CR+MRD-). Phase 2 consisted of myeloablative doses of topotecan, thiotepa and carboplatin plus hyperfractionated RT. Phase 3 included isotretinoin and 3F8 immunotherapy plus GM-CSF. BM MRD was monitored using GD2 synthase, PHOX2B and cyclin D1 mRNAs. RESULTS: After 3 cycles, all patients showed BM complete histological clearance and 6 (25 %) were MRD-. Twenty of 21 s-look surgeries achieved macroscopic complete resection. After 5 cycles and surgery, (123)I-MIBG scan was negative in 15 (62.5 %) cases, BM disease by histology was negative in 23 (96 %) and 10 (42 %) patients were MRD-. Twelve (50 %) pts were in CR, 2 in very good partial response (VGPR), 9 partial response (PR) and one had progressive disease. With 2 HD-CT extra cycles, 17 (71 %) pts achieved CR+MRD- status moving to phase 2. Overall and event-free survival at 3 years for the 17 patients who achieved CR+MRD- is 65 and 53 %, respectively, median follow-up 47 months. Seven (29 %) patients never achieved CR+MRD-. Univariate Cox regression analysis shows CR+MRD- status after mN7 induction as the only statistically significant prognostic factor to predict overall survival. CONCLUSIONS: mN7 induction regimen produced a CR+MRD- rate of 71 %. CR+MRD- status following induction was the only predictive marker of long-term survival.

Successful pregnancy in a 63-year-old woman.

OBJECTIVE: To report the occurrence of an unusual case of successful pregnancy achieved by oocyte donation in a woman > 60 years of age. DESIGN: Case report. SETTING: University-based assisted reproductive technology program. PATIENT(S): Sixty-three-year old nulligravida, married for 16 years to her 60-year-old husband. Throughout her infertility treatment, the patient was believed to be 10 years younger, as she claimed. She revealed her true age of 63 only upon being referred for obstetric care at 13 weeks of gestational age. INTERVENTION(S): Oocyte donation, IVF, embryo cryopreservation, and ET. MAIN OUTCOME MEASURE(S): Attainment of pregnancy and subsequent delivery. RESULT(S): The patient underwent two cycles of oocyte donation. During the second attempt, the fresh transfer resulted in a clinical miscarriage at approximately 8 weeks of gestational age. A subsequent transfer of three frozen-thawed embryos resulted in an ongoing singleton gestation. The pregnancy was complicated by gestational diabetes (controlled by diet) and mild pregnancy-induced hypertension. Delivery by cesarean section at 38 weeks of gestational age resulted in the birth of a healthy female infant weighing 2,844 g with Apgar scores of 9 and 9. CONCLUSION(S): This case demonstrates that the uterus is capable of supporting nidation and subsequent gestation for many years beyond natural menopause. It shows that other aspects of human physiology are capable of adapting to the stresses and changes of pregnancy sufficiently well to achieve a normal birth at the age of 63 years. This case also exemplifies the difficulty in attempting to regulate the age of recipients in oocyte donation. As in other aspects of human life, when age limits are applied to the provision of certain services, human beings whose age falls outside of these limits become motivated to deceive the providers of those services to avail themselves of the services.

Effect of cultivar and growing location on the trypsin inhibitors, tannins, and lectins of common beans (Phaseolus vulgaris L.) grown in the semiarid highlands of Mexico.

Trypsin inhibitors (TI), tannins, and lectins appear to have a role in preventing chronic diseases in humans. The genetic variability of these traits in common bean needs to be ascertained in order to increase levels through breeding. The variability of TI, tannin, and lectins was determined in five bean cultivars grown at five locations in Mexico. TI and tannins contents in colored beans that belong to the Jalisco race were higher (11.1-11.9 trypsin units inhibited (TUI)/mg and 29.0-38.1 mg catechin equivalent (CE)/g, respectively) than cultivars of the Durango race (7.9-8.3 TUI/mg and 16.8-19.9 CE/mg, respectively). Bayo Victoria, a Durango race cultivar, had three times more lectins than levels reported for soybean. Cultivar influenced TI and tannins contents (p < 0.001), whereas site affected lectins (p < 0.001). An increase in levels of TI and tannins could be enhanced through breeding.

Daily intake of a bean-fiber fortified bar reduces oxidative stress

as been proposed that the consumption of common beans (Phaseolus vulgaris L.) reduces cardiovascular risk, and prevents and controls both chronic and degenerative diseases. The aim of this study was to compare the antioxidant capacity of a bean-fiber fortified bar (BFB) versus a commercial bar (CB) in 60 Mexican men and women (18-65 years old), who were randomly distributed in two groups: BFB or CB; individuals consumed a bar a day for one month. Anthropometric data, food intake and blood samples were collected. Glucose tolerance (GTT), lipid profile (PL), and lipid peroxidation (TBARS) tests were performed; carbonyls groups in serum oxidized proteins were also measured. GTT and PL were not different between both groups in either the 15 or 30-day follow-up of bar consumption assessments. There were no significant differences in either TBARS or carbonyl concentration between groups; BFB group showed higher levels of serum lipid peroxidation in basal and fifteen days measurements; these levels decreased at the final evaluation: No differences were detected on carbonyl levels between groups. In conclusion, 30 days of fiber bean bar consumption did not alter glucose or PL levels, while, in the BFF group, oxidative stress decreased within 30 days of the consumption of the fortified bar(AU)

Se ha propuesto que el consumo de frijol común (Phaseolus vulgaris L.) reduce el riesgo cardiovascular, y previene y controla las enfermedades crónicas y degenerativas. El objetivo del presente estudio fue comparar la capacidad antioxidante de una barra fortificada con fibra de frijol (BFB) versus una barra comercial (CB) en 60 hombres y mujeres mexicanos (18-65 años de edad), quienes aleatoriamente fueron distribuidos en dos grupos: El grupo BFB y el CB que consumieron la barras fortificada con frijol y la barra comercial, respectivamente, durante un mes. Se recopilaron datos antropométricos, ingesta de alimentos y muestras de sangre. Se realizó prueba de tolerancia a la glucosa (GTT), el perfil de lípidos (PL), la peroxidación de lípidos (TBARS) y la cuantificación de carbonilos en proteínas oxidadas como pruebas de bioquímica sanguínea. GTT y PL no fueron diferentes entre ambos grupos en la evaluación de seguimiento de 15 y 30 días del consumo de la barra. No hubo diferencias significativas en los TBARS o la concentración de carbonilo entre los grupos, el grupo BFB mostró niveles más altos de peroxidación de lípidos en suero en la fase basal y a los quince días del consumo de la barra; curiosamente, estos niveles disminuyeron en la evaluación final. No se detectaron diferencias en los niveles de carbonilo entre los grupos. En conclusión, 30 días de consumo de barras de fibra de frijol no alteraron los niveles de glucosa o PL; mientras que, en el grupo BFB, el estrés oxidativo disminuyó a los 30 días del consumo de la barra fortificada(AU)

Physicochemical, nutritional, and functional characterization of fruits xoconostle (Opuntia matudae) pears from Central-México Region.

Xoconostle cv. Cuaresmeño (Opuntia matudae) has attracted domestic and international industry attention; however, variations of composition from xoconostle structures have not been evaluated. Industries discard the pulp (endocarp) and peel (pericarp) as wastes and utilize the skin (mesocarp), which is the edible portion. The physicochemical, nutritional, and functional characterization of structures from xoconostle pear from 3 major sites of production in Mexico were assessed. Skin yield ranged from 58% to 64% and was higher to that of peel (22% to 24%) and pulp (12% to 18%) yields. pH, degrees Brix, and acidity were similar among xoconostle structures. Total fiber showed by peel (18.23% to 20.37%) was 2-fold higher than that of skin. Protein and ether extract were higher in xoconostle pulp compared to that showed by peel and skin. Iron content of xoconostle peel (6 to 9.6 mg/100 g, DWB) was higher to that of skin and pulp and prickly pear pulp. Soluble phenols of peel (840 to 863 mg GAE/100 g, DWB) were almost similar to that of skin (919 to 986 mg GAE/100 g, dry weigh basis); meanwhile, ascorbic acid concentration of skin was 2-fold higher compared to that of peel. The phenolic fraction of xoconostle structures consisted of gallic, vanillic, and 4-hydroxybenzoic acids; catechin, epicatechin, and vanillin were also identified by high-performance liquid chromatography-didoe array detection (HPLC-DAD). Xoconostle peel showed higher antioxidant activity (TEAC) compared to that of skin (2-fold) and pulp (6-fold) of commonly consumed fruits and vegetables. The potential of xoconostle peel and pulp for the production of feed or food is promissory. Practical Application: Outstanding nutritional and functional properties of xoconostle cv. Cuaresmeño fruits are demonstrated. Increased consumption could contribute positively to improve the diet of rural and urban consumers. The high fiber, mineral, and antioxidant components of xoconostle peel and pulp suggest that these fruit structures, which are currently discarded as waste, have promissory use as feed or food by industry.

Antimutagenic and antioxidant activities of quebracho phenolics (Schinopsis balansae) recovered from tannery wastewaters.

Quebracho extracts are used in tannery due to their high concentration of phenolics. The Mexican tannery industry uses around 450 kg/m(3) of which, 150 kg/m(3) remains in wastewaters and are discharged in drain pipe systems or rivers. The quebracho phenolics recovered from tannery wastewater (QPTW) was characterized by HPLC. The antimutagenic and antioxidant activities as well as the microbiological quality were evaluated. Total phenolic content of QPTW was 621mg catechin equivalent/g sample. Gallic and protocatechuic acids were the major components characterized by HPLC. QPTW showed an inhibition range on aflatoxin B(1) mutagenicity from 16 to 60% and was dose-dependent. Antioxidant activity (defined as beta-carotene bleaching) of QPTW (64.4%) at a dose of 12.3mg/mL was similar to that of BHT (68.7%) at a dose of 0.33 mg/mL, but lower than Trolox (90.8% at a dose of 2.5mg/mL); meanwhile antiradical activity (measured as reduction of DPPH) (60.8%) was higher than that of BHT (50.8%) and Trolox (34.2%). Quebracho residues were demonstrated to be an outstanding source of phenolic acids and for research and industrial uses.

Results of induction chemotherapy in children older than 18 months with stage-4 neuroblastoma treated with an adaptive-to-response modified N7 protocol (mN7)

Purpose. We report the response rate in children older than 18 months with stage 4 Neuroblastoma, using a modified dose-intensive, response-adaptive, induction mN7 protocol. Methods. From 2005 to 2012, 24 patients were treated with the mN7 protocol. Phase 1 included five MSKCC N7 cycles and surgery and two high-dose cyclophosphamide-topotecan (HD-CT) cycles for those who did not achieve complete remission (CR) and negative bone marrow (BM) minimal residual disease (MRD) status (CR+MRD-). Phase 2 consisted of myeloablative doses of topotecan, thiotepa and carboplatin plus hyperfractionated RT. Phase 3 included isotretinoin and 3F8 immunotherapy plus GM-CSF. BM MRD was monitored using GD2 synthase, PHOX2B and cyclin D1 mRNAs. Results. After 3 cycles, all patients showed BM complete histological clearance and 6 (25 %) were MRD-. Twenty of 21 s-look surgeries achieved macroscopic complete resection. After 5 cycles and surgery, 123I-MIBG scan was negative in 15 (62.5 %) cases, BM disease by histology was negative in 23 (96 %) and 10 (42 %) patients were MRD-. Twelve (50 %) pts were in CR, 2 in very good partial response (VGPR), 9 partial response (PR) and one had progressive disease. With 2 HD-CT extra cycles, 17 (71 %) pts achieved CR+MRD- status moving to phase 2. Overall and event-free survival at 3 years for the 17 patients who achieved CR+MRD- is 65 and 53 %, respectively, median follow-up 47 months. Seven (29 %) patients never achieved CR+MRD-. Univariate Cox regression analysis shows CR+MRD- status after mN7 induction as the only statistically significant prognostic factor to predict overall survival. Conclusions. mN7 induction regimen produced a CR+MRD- rate of 71 %. CR+MRD- status following induction was the only predictive marker of long-term survival (AU)

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Effects of regular and decaffeinated coffee on fetal breathing and heart rate.

The effects of maternal consumption of regular or decaffeinated coffee on the fetus were determined in eight pregnant women at 32 to 36 weeks of gestation. This was a single-blind crossover study in which two cups of caffeinated or decaffeinated coffee were consumed after an overnight fast. The total maternal caffeine ingested was 454 +/- 4 mg for regular coffee and 12 +/- 0.4 mg for decaffeinated coffee. Maternal consumption of regular coffee was associated with a twofold increase in the incidence of fetal breathing activity and a significant fall in baseline fetal heart rate. Decaffeinated coffee also increased the incidence of fetal breathing activity and produced a slight reduction in fetal heart rate. These results indicate that maternal consumption of regular and decaffeinated coffee can stimulate fetal breathing. Moreover, these results suggest that caffeinated coffee can produce baseline shifts in fetal heart rate.

Follow-up of intrauterine transfused surviving children.

The long-term effects of intrauterine transfusion (IUT) are still being assessed since the procedure was first reported in 1963. Therefore, we undertook a follow-up study on the survivors of 57 infants who received IUT's between 1966 and 1975. Forty-two infants were born alive, but 15 died in the neonatal period. Overall survival was 27/57 (48%). An extensive follow-up examination was carried out at age 22 months to 10 years in 21/27 survivors. Height was below the third percentile in one child and head circumference above the mean and 2 SD in another. Of 21 children, 19 had normal vision, one was slightly myopic, and one had hyperopia. One child had unilateral high-frequency hearing loss. All had normal EEG's and none have mental retardation but 2/21 have "major" and 6/21 have "minor" neurologic signs. No evidence of passive antibody transfer was found, but minor disturbances of immunoglobulin levels were present.

Diagnóstico de presión arterial en niños sanos de edades entre menores o igual a un año y hasta 18 / Blood pressure diagnosis in children between the ages of 1 and 18 years

Con el objetivo de conocer los valores de tensión arterial en edades de 0 a 18 años, se realizó un ensayo clínico diagnóstico en cuatro consultorios del Policlínico Centro de la ciudad de Camagüey. Se determinó el peso en kilogramos y la talla en centímetros y se calculó la superficie corporal. La tensión arterial se realizó por el método auscultatorio indirecto, realizándose dos tomas de tensión arterial con intervalo de 5 minutos y se repitieron las mediciones a la semana. Para el procesamiento estadístico fue utilizado SPSS versión 10, en el que inicialmente se realizó el análisis descriptivo de las variables cuantitativas, calculándose la media aritmética y desviación estándar. Se realizó la distribución de percentiles para las presiones arteriales sistólicas y diastólicas según sexo y superficie corporal. La correlación de las tensiones arteriales sistólicas y diastólicas con la edad es significativa (p<0.05). También fue significativa (p<0.05) con respecto a la talla y superficie corporal. El diagnóstico de hipertensión sistólica por el método de la media fue de 1.1 por ciento, y la hipertensión diastólica fue de 5 por ciento por el método de los percentiles. Por el método de la media no se encontraron hipertensos. Se concluye que la tensión arterial aumenta proporcionalmente con la edad, la talla, el peso y la superficie corporal y los hipertensos se detectan en mayor proporción por el método de los percentiles (AU)