Publications by authors named "Fons J"

The tympanic membrane (i.e. eardrum) sits at the interface between the middle and external ear.

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The methodology for production of biologics is going through a paradigm shift from batch-wise operation to continuous production. Lot of efforts are focused on integration, intensification, and continuous operation for decreased foot-print, material, equipment, and increased productivity and product quality. These integrated continuous processes with on-line analytics become complex processes, which requires automation, monitoring, and control of the operation, even unmanned or remote, which means bioprocesses with high level of automation or even autonomous capabilities.

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A mesiodens is a supernumerary tooth located in the midline of the premaxilla. To investigate the genetic cause of mesiodens, clinical and radiographic examination were performed on 23 family members of a two-generation Hmong family. Whole exome sequencing (WES) or Sanger sequencing were performed in 22 family members and two unrelated Thai patients with mesiodens.

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High incidence of chronic otitis media is associated with human craniofacial syndromes, suggesting that defects in the formation of the middle ear and associated structures can have a knock-on effect on the susceptibility to middle ear inflammation. Patients with branchio-oto-renal (BOR) syndrome have several defects in the ear leading to both sensorineural and conductive hearing loss, including otitis media. 40% of BOR syndrome cases are due to haploinsufficiency, with mouse models affecting , mimicking many of the defects found in patients.

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The pinna (or auricle) is part of the external ear, acting to capture and funnel sound toward the middle ear. The pinna is defective in a number of craniofacial syndromes, including Lacrimo-auriculo-dento-digital (LADD) syndrome, which is caused by mutations in or its receptor . Here we study pinna defects in the knockout mouse.

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Defects in ear canal development can cause severe hearing loss as sound waves fail to reach the middle ear. Here, we reveal new mechanisms that control human canal development and highlight for the first time the complex system of canal closure and reopening. These processes can be perturbed in mutant mice and in explant culture, mimicking the defects associated with canal atresia.

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Background: The egg tooth is a vital structure allowing hatchlings to escape from the egg. In squamates (snakes and lizards), the egg tooth is a real tooth that develops within the oral cavity at the top of the upper jaw. Most squamates have a single large midline egg tooth at hatching, but a few families, such as Gekkonidae, have two egg teeth.

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With continued development of integrated and continuous downstream purification processes, tuning and optimization become increasingly complicated with additional parameters and codependent variables over the sequence. This article offers a novel perspective of nonlinear optimization of integrated sequences with regard to individual column sizes, flow rates, and scheduling. The problem setup itself is a versatile tool to be used in downstream design which is demonstrated in two case studies: a four-column integrated sequence and a continuously loaded twin-capture setup with five columns.

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During the formation of repetitive ectodermally derived organs such as mammary glands, lateral line and teeth, the tissue primordium iteratively initiates new structures. In the case of successional molar development, new teeth appear sequentially in the posterior region of the jaw from Sox2(+) cells in association with the posterior aspect of a pre-existing tooth. The sequence of molar development is well known, however, the epithelial topography involved in the formation of a new tooth is unclear.

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Measuring the gross alpha activity in water samples is a rapid, straightforward way of determining whether the water might contain a radionuclide concentration whose consumption would imply a total indicative dose (TID) greater than some reference limit - currently set at 0.1 mSv/y in Europe. There are several methods used for such measurements.

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Introduction And Hypothesis: The prevalence of levator avulsion after vaginal delivery ranges from 10 to 30 %. To our knowledge, no previous studies have used anorectal manometry (AM) to assess this injury's impact on the functionality of the anal sphincter complex. We hypothesized that women with levator avulsion have lower manometric pressures.

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Dent's disease is an X-linked proximal tubulopathy characterized by low-molecular-weight proteinuria, hypercalciuria, nephrocalcinosis, nephrolithiasis and progressive renal failure. This disorder is frequently caused by mutations in the CLCN5 gene encoding the electrogenic chloride/proton exchanger ClC-5. Occasionally, Dent's disease has been associated to atypical cases of asymptomatic proteinuria with focal glomerulosclerosis.

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The determination of gross alpha, gross beta and (226)Ra activity in natural waters is useful in a wide range of environmental studies. Furthermore, gross alpha and gross beta parameters are included in international legislation on the quality of drinking water [Council Directive 98/83/EC]. In this work, a low-background liquid scintillation counter (Wallac, Quantulus 1220) was used to simultaneously determine gross alpha, gross beta and (226)Ra activity in natural water samples.

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The aim of the present work was to compare the results obtained with gross alpha methods such as evaporation, co-precipitation and total evaporation by liquid scintillation counting and to check whether these results are representative of the real total alpha activity concentration on the sample. The study was carried out on eight natural waters with very different radioactive characteristics. For all the samples uranium ((238)U, (235)U, and (234)U), radium ((226)Ra and (224)Ra), (210)Po, and (232)Th isotopes were also assayed by using radiochemical separation and alpha spectrometry in order to determine the sum of the activities of these alpha emitters.

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Introduction: There is still insufficient scientific evidence on which is the best technique to perform the anastomosis -intracorporeal (IC) or extracorporeal (EC)- in right laparoscopic hemicolectomy. The objective of the present study is to determine whether there are differences to compare in both techniques.

Material And Methods: A study was performed on a prospective patient series subjected to right laparoscopic hemicolectomy in our Hospital.

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Objective: To analyse the influence of several prenatal and neonatal risk factors in the development of enamel defects in low birth weight children.

Subjects And Methods: Children between 4 and 5 years of age (n = 102) were classified into: Group 1) 52 low birth weight (<2500 g); Group 2) 50 normal birth weight (>or=2500 g). Medical history, prenatal and neonatal variables were collected.

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We have studied 62 children with urinary infection simultaneously by means of micturitional cystoureterography (MCU) and indirect isotopic cystography. We confirm a correlation between the results of the MCU and in the indirect isotopic cystography in 86% of the ureters explored, whilst the results of the MCU and of the direct isotopic cystography coincided in 92% of the ureters explored. We discuss the advantages and drawbacks of isotopic cystography, concluding that it is a suitable method for the diagnosis and evolutive control of vesicoureteral reflux in infancy.

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Twenty-three children with idiopathic hypercalciuria and 7 control children were studied. Patients were classified into three groups according to the response to an oral calcium load. Five children displayed absorptive hypercalciuria (UCa/Cr 0.

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