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PubMed2004

Pharyngeal-cervical-brachial variant Guillain-Barré syndrome in a child.

The pharyngeal-cervical-brachial variant of Guillain-Barré syndrome is uncommon but well recognized in the adult literature. Patients have weakness in a pharyngeal-cervical-brachial distribution with relative lower limb sparing. We describe a 12-year-old boy with predominantly pharyngeal-cervical-brachial weakness and subsequent respiratory failure. Owing to prominent bulbar symptoms, he was initially misdiagnosed as having epiglottitis. This case illustrates that the clinical spectrum of Guillain-Barré syndrome in children includes the pharyngeal-cervical-brachial variant, which is distinct from Miller-Fisher syndrome. Atypical Guillain-Barré syndrome should be considered in the differential diagnosis of a child presenting with bulbar palsy and/or respiratory failure.

Child↗

PubMed1998

Airway protection in sleeping infants in response to pharyngeal fluid stimulation in the supine position.

This study was designed to evaluate upper airway protective mechanisms in response to pharyngeal fluid stimulation in healthy term and preterm infants at term equivalent age. Five term and seven preterm infants were studied and the following recorded: sleep state, cardiorespiratory function, and swallowing. Infusions of 0.9% saline and sterile water of volumes of 0.04, 0.2, and 0.35 mL were made during active (AS) and quiet sleep (QS). The effect of these variables on apnea (> or = 2 and > or = 5 s), swallowing, and arousal was examined. After pharyngeal infusion, apnea of > or = 2 and > or = 5 s did not change from spontaneous rates for both term and preterm infants. The most common response to pharyngeal infusion was swallowing. In AS, swallowing occurred after 65 and 73% and in QS after 40 and 64% of infusions in term and preterm infants, respectively. Swallowing was volume-related and occurred significantly more often in term infants after larger infusions of 0.35 and 0.2 mL (83 and 67%) compared with the 0.04 mL (19%) and after 0.2 mL compared with 0.04 mL for preterm infants (94 and 44%). At 0.2 mL, this was significantly higher in preterm compared with term infants (p < 0.01) and was the only significant difference between these infants. In response to pharyngeal fluid stimulation, airway defense in both full-term and preterm infants is maintained primarily by swallowing with no evidence of apnea.

Deglutition↗

PubMed2006

Sonic hedgehog is essential for first pharyngeal arch development.

The secreted protein sonic hedgehog (Shh) is essential for normal development of many organs. Targeted disruption of Shh in mouse leads to near complete absence of craniofacial skeletal elements at birth, and mutation of SHH in human causes holoprosencephaly (HPE), frequently associated with defects of derivatives of pharyngeal arches. To investigate the role of Shh signaling in early pharyngeal arch development, we analyzed Shh mutant embryos using molecular markers and found that the first pharyngeal arch (PA1) was specifically hypoplastic and fused in the midline, and remaining arches were well formed at embryonic day (E) 9.5. Molecular analyses using specific markers suggested that the growth of the maxillary arch and proximal mandibular arch was severely defective in Shh-null PA1, whereas the distal mandibular arch was less affected. TUNEL assay revealed an increase in the number of apoptotic signals in PA1 of Shh mutant embryos. Ectodermal expression of fibroblast growth factor (Fgf)-8, a cell survival factor for pharyngeal arch mesenchyme, was down-regulated in the PA1 of Shh mutants. Consistent with this observation, downstream transcriptional targets of Fgf8 signaling in neural crest-derived mesenchyme, including Barx1, goosecoid, and Dlx2, were also down-regulated in Shh-null PA1. These results demonstrate that epithelial-mesenchymal signaling and transcriptional events coordinated by Shh, partly via Fgf8, is essential for cell survival and tissue outgrowth of the developing PA1.

Animals↗

PubMed2006

Mesodermal expression of Tbx1 is necessary and sufficient for pharyngeal arch and cardiac outflow tract development.

The development of the segmented pharyngeal apparatus involves complex interaction of tissues derived from all three germ layers. The role of mesoderm is the least studied, perhaps because of its apparent lack of anatomical boundaries and positionally restricted gene expression. Here, we report that the mesoderm-specific deletion of Tbx1, a T-box transcription factor, caused severe pharyngeal patterning and cardiovascular defects, while mesoderm-specific restoration of Tbx1 expression in a mutant background corrected most of those defects in the mouse. We show that some organs, e.g. the thymus, require Tbx1 expression in the mesoderm and in the epithelia. In addition, these experiments revealed that different pharyngeal arches require Tbx1 in different tissues. Finally, we show that Tbx1 in the mesoderm is required to sustain cell proliferation. Thus, the mesodermal transcription program is not only crucial for cardiovascular development, but is also key in the development and patterning of pharyngeal endoderm.

Animals↗

PubMed2000

sucker encodes a zebrafish Endothelin-1 required for ventral pharyngeal arch development.

Mutation of sucker (suc) disrupts development of the lower jaw and other ventral cartilages in pharyngeal segments of the zebrafish head. Our sequencing, cosegregation and rescue results indicate that suc encodes an Endothelin-1 (Et-1). Like mouse and chick Et-1, suc/et-1 is expressed in a central core of arch paraxial mesoderm and in arch epithelia, both surface ectoderm and pharyngeal endoderm, but not in skeletogenic neural crest. Long before chondrogenesis, suc/et-1 mutant embryos have severe defects in ventral arch neural crest expression of dHAND, dlx2, msxE, gsc, dlx3 and EphA3 in the anterior arches. Dorsal expression patterns are unaffected. Later in development, suc/et-1 mutant embryos display defects in mesodermal and endodermal tissues of the pharynx. Ventral premyogenic condensations fail to express myoD, which correlates with a ventral muscle defect. Further, expression of shh in endoderm of the first pharyngeal pouch fails to extend as far laterally as in wild types. We use mosaic analyses to show that suc/et-1 functions nonautonomously in neural crest cells, and is thus required in the environment of postmigratory neural crest cells to specify ventral arch fates. Our mosaic analyses further show that suc/et-1 nonautonomously functions in mesendoderm for ventral arch muscle formation. Collectively our results support a model for dorsoventral patterning of the gnathostome pharyngeal arches in which Et-1 in the environment of the postmigratory cranial neural crest specifies the lower jaw and other ventral arch fates.

Amino Acid Sequence↗

PubMed2005

One-stage nasal and multi-level pharyngeal surgery for obstructive sleep apnoea: safety and efficacy.

A collapsible airway is often the common denominator in sleep-disordered breathing (SDB). The upper respiratory tract includes the nasal passage, nasopharynx, oral cavity, oropharynx, base-of-tongue region and the hypopharynx. It is believed that the highest amount of resistance in the upper respiratory tract is in the nasal cavities, and particularly the nasal valve. Most authors believe that when considering surgical options for patients with obstructive sleep apnoea (OSA) it is imperative to correct nasal pathology together with the other sites of airway obstruction. In this retrospective study, I sought to investigate the safety and efficacy of one-stage nasal and multi-level pharyngeal surgery. I compared two groups of patients: group 1, receiving one-stage nasal and multi-level pharyngeal surgery; and group 2, receiving only multi-level pharyngeal surgery. In group 1, nine out of 12 patients (75 per cent) met the criteria for surgical success, with a mean pre-operative apnoea-hypopnoea index (AHI) decreasing from 36.3 to 8.9 post-operatively (p<0.0002), while in group 2, 25 out of 40 patients met the surgical success criteria (62.5 per cent), with their mean AHI decreasing from 52.6 to 10.2 (p<0.0000). When comparing the surgical success rates between the two groups, it was not statistically significant, at p>0.106. There were no postoperative respiratory-related complications despite having bilateral nasal Merocel (tampon) packing in place (in group 1), and none of the patients in either group had any desaturation, hypoxaemia, apnoea or OSA-related complications. This series suggests that, with adequate post-operative monitoring, it is both safe and efficacious to perform both nasal and multi-level pharyngeal surgery in the one surgical session.

Adult↗

PubMed1988

Pharyngeal and oesophageal function in patients with diabetes mellitus and swallowing complaints.

Pharyngeal and oesophageal function were monitored using cine and video techniques in 18 diabetic patients with swallowing complaints. Morphological lesions had been excluded with double-contrast radiography. Abnormal pharyngeal function was present in 14 patients and abnormal oesophageal function in 10 patients. Eight of these patients had both pharyngeal and oesophageal dysfunction. Dysphagia does not seem to be a frequent symptom in diabetic patients but, when present, warrants a detailed analysis of pharyngeal and oesophageal function in order to reveal the underlying cause. We found cine and video techniques to be of value and we recommend them for use in functional analysis in these patients.

Adult↗

PubMed1988

Analysis of pressure generation and bolus transit during pharyngeal swallowing.

Current pharyngeal deglutition theory has stressed the role of the pharyngeal constrictors as producing a peristaltic wave responsible for bolus propulsion through the pharynx. This thesis presents data obtained using manofluorography which supports the significance of tongue and laryngeal motion in swallowing. The usage of the term peristalsis to describe the constrictor contraction is challenged. The results of this quantitative study of swallowing in normal subjects, laryngectomized patients, and patients with restricted tongue motion show that tongue driving pressure and the negative pressure developed in the pharyngeal esophageal segment appear more important than the peristaltic-like pressure of the constrictors. Bolus transit is really dependent upon these two pressures. This model for analysis has clinical significance because it permits quantification of the pharyngeal swallowing mechanism.

Deglutition↗

PubMed2004

[The influence of oral and pharyngeal phases on the swallowing dynamic].

BACKGROUND: The pharyngeal phase of swallowing has received more attention than oral phase although they are presumably interdependent. AIMS: 1. to evaluate, through the videofluoroscopic method, the oral phase of swallowing in order to observe the characteristics organization of the liquid bolus in healthy volunteers and the variations of this organization in exams of patient with dysphagia; 2. to establish the functional interrelation between both organization and ejection stages; 3. to verify the presence (or absence) of interference of the oral phase over the pharyngeal phase. CASUISTIC AND METHOD: Videofluoroscopic assessment has been performed in patients with stroke (15), patients with Parkinson disease (15) and in health volunteers (14). All of the studied individuals were submitted to the swallowing videofluoroscopic evaluation following protocol described by Junqueira and Costa. We privileged the incidence in right profile with middle of liquid contrast (solution of barium sulfate). RESULTS: According to the intra-oral organization of the contrasted bolus, we could classify the oral organization as: 1. closed, 2. open, that can be subdivided in open "anterior restricted" and "open expanded", 3. prolonged, and 4. unstable. The ejection can be defined as: 1. appropriate, 2. lentify, and 3. in two times. The correlation among the oral dynamics acted by the organization and pharyngeal phases can be noticed as: 1. appropriate, 2. adapted, and 3. altered. The organization and ejection types as well as the observed correlations are shown with statistics significance. CONCLUSIONS: The closed organization type was the one characterized as normal. The "open expanded", "prolonged" and "unstable" organization type can be associated with alteration in the swallowing process. The "lentify" and "two times" ejection types also point to swallowing physiology alteration. There are functional correlation between organization and oral ejection. The oral organization has influence, not only in the quality of the oral ejection, but also, in the effective dynamics of the pharyngeal phase.

Adult↗

PubMed1995

Pharyngeal flaps after one- and two-stage repair of the cleft palate: a 25-year review of 520 patients.

Between 1962 and 1976, a one-stage surgical procedure was performed on 328 cases of cleft lip and palate or isolated cleft palate. From 1977 to 1986, a two-stage surgical procedure was performed on 192 patients. After one-stage repair, 22 (6.7%) required a pharyngeal flap at the age of 6 years, and 13 (7%) required a flap after two-stage repair. When the patients were 14 years or older, 56 (17%) needed a flap after one-stage repair, and 14 (24%) required a flap after two-stage repair. Two-stage repair at 24 months and 5.2 years resulted in more patients in need of pharyngeal flaps than those who had repair at 12 to 18 months and 3 years. In both groups, at age 10, the incidence of flaps was approximately 20% after closure of isolated cleft palate involving the hard palate, with 11 to 12% in patients with unilateral cleft lip and palate (UCLP) or clefts of the soft palate only, and 14 to 16% in patients with bilateral cleft lip and palate (BCLP). The pharyngeal flap was more often used in girls than in boys, especially in cases with CP only. Approximately 85% of the flaps were carried out before the age of 10 years, and only a few flaps may have been related to involution of the adenoid. The incidence of fistulas was higher after one-stage repair of cleft lip and palate (CLP) (p < .05) and lower in patients with CP only (p < .05), compared with the results after two-stage repair. Pharyngeal flaps were slightly more common after two-stage repair.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1999

Attachment of Burkholderia pseudomallei to pharyngeal epithelial cells: a highly pathogenic bacteria with low attachment ability.

Respiratory infections are initiated by the attachment of bacteria to pharyngeal epithelial cells. We studied the attachment of Burkholderia pseudomallei to pharyngeal epithelial cells. After one, two, three, and four washes, there were 22.6+/-8.9, 15.7+/-7.0, 6.8+/-3.1, and 4.6+/-1.1 (mean+/-SD) attached bacteria/cell, respectively. If the bacterial concentration was maintained at 1 X 10(8) colony-forming units (cfu)/ml and three washes were done, at concentrations of 2.5 x 10(4), 5 X 10(4), and 1 x 10(5) cells/ml there were 9.9+/-3.6, 3.3+/-0.8, and 2.5+/-1.1 attached bacteria/cell, respectively. If the cell concentration was kept at 2.5 x 10(4) cells/ml and three washes were done, at bacterial concentrations of 1 x 10(5), 1 X 10(6), 1 X 10(7), 1 x 10(8), and 1 x 10(9) cfu/ml, there were 0.3+/-0.3, 0.6+/-0.6, 1.0+/-0.2, 5.1+/-2.3, and 9.6+/-1.9 attached bacteria/cell, respectively. There were 4.8+/-1.9, 5.5+/-2.5, 5.6+/-1.9, and 6.4+/-2.6 attached bacteria/cell at 0, 30, 120, and 240 min of incubation, respectively. Pharyngeal cells from 10 persons (seven men and three women, mean+/-SD age = 30.7+/-8.1 years, 12 experiments with a single isolate) showed that there were 7.8+/-4.3 attached bacteria/cell. It was found that the efficiency of attachment of this bacteria was very low (7.0+/-3.3 bacteria/cell). Electron microscopy revealed that there were no fimbriae but a thin capsular polysaccharide layer on the surface of B. pseudomallei. Attachment to pharyngeal epithelial cells appeared to be mediated by this structure.

Adult↗

PubMed1994

Effects of modified Cary and Blair medium on recovery of nonhemolytic Pasteurella haemolytica from Rocky Mountain bighorn sheep (Ovis canadensis canadensis) pharyngeal swabs.

Modified Cary and Blair transport medium (MCB) was evaluated for recovery of Pasteurella spp. from pharyngeal swabs of healthy Rocky Mountain bighorn sheep (Ovis canadensis canadensis). In experiment one, three pharyngeal swabs were collected from each of 25 bighorns. Pasteurella haemolytica was recovered from 21 of 25 swabs tested almost immediately and from 16 of 25 swabs held in MCB medium at about 22 C for 24 hr before testing (P > 0.10). Recovery of P. haemolytica decreased (P < 0.005) to 1 of 25 when swabs were held in MCB medium at about 22 C for 48 hr before testing. In experiment two, four pharyngeal swabs were collected from each of ten bighorns and held in MCB medium at about 5 C for < or = 5, 24, 48, or 72 hr prior to testing. Recovery was unaffected by storage at 5 C; P. haemolytica was isolated from all 40 of these samples. All Pasteurella spp. isolates were nonhemolytic P. haemolytica. In experiment one, most isolates were serotype 4; in experiment two, serotype 3 was most common. We propose that MCB medium is effective for transporting bighorn sheep pharyngeal swabs for P. haemolytica screening because it imposes minimal or no effect on recovery when held < or = 24 hr at 22 C or < or = 72 hr at 5 C.

Animals↗

PubMed1999

Hoxb-5 is expressed in gill arch 5 during pharyngeal arch development of flounder Paralichthys olivaceus embryos.

Hox genes are expressed in domains with clear anterior borders exhibiting 3'-->5' hierarchy in hindbrain and in the pharyngeal area commonly in vertebrate embryos. Teleost embryos form seven pharyngeal arches, the mandibular arch, hyoid arch and the gill arches 1-5. We previously reported that, in Japanese flounder (Paralichthys olivaceus) embryos, Hoxd-4 is expressed from rhombomere 7 to the spinal cord in the central nervous system and at gill arches 2-5. At present, the hierarchy of Hox genes at gill arches 3-5 of teleost fish is unclear. Here, we investigated the expression domains of Hoxb-5 in the flounder embryo by whole-mount in situ hybridization to gain insight into the Hox code at gill arches. The initial signal indicating Hoxb-5 expression was identified in the spinal cord at hatching, corresponding with the prim-5 stage of zebrafish. Then, intense signals were detected from the anterior part of the spinal cord and from the posterior part of the pharyngeal area at 36 h after hatching. By serially sectioning the hybridized embryos, it was found that signal in the pharyngeal area came from the most posterior gill arch 5. Therefore, it is speculated that Hoxb-5 functions in regional identification of gill arch 5 in this teleost.

Animals↗

PubMed1975

Acoustic features of pharyngeal /s/ fricatives produced by speakers with cleft palate.

Acoustic features of pharyngeal /s/ substitutions spoken by persons with cleft palate were measured using digital computer processing and analysis techniques. Average short-time spectral characteristics of pharyngeal /s/ sounds produced by four speakers with cleft palate were compared with spectral features of normally gestured /s/ sounds spoken by two normal speakers. The output spectral characterized by a set of distinctive acoustic properties that differed markedly from normal /s/ sounds gestured in the front of the mouth. One consistent feature of pharyngeal /s/'s was the presence of multiple spectral maxima. Pharyngealization of /s/ consonants gave rise to spectral outputs with energy maxima appropriate to high F1 values which were relatively close to F2, as well as to relatively strong, low frequency excitation of F2 speakers with cleft palate were, in general, comparable to those of /h/ fricatives spoken by speakers of Arabic.

Acoustics↗

PubMed1975

Transverse pharyngeal flaps: a dynamic repair for velopharyngeal insufficiency.

During the past three years, forty-eight patients, ranging from five to thirty years of age, have undergone bilateral transverse pharyngeal pedicles to the palate. Thirty-nine patients had not had pharyngeal flaps prior to this procedure and the remaining nine patients had failure of production of acceptable speech after undergoing inferiorly or superiorly based flap operations. One patient suffered separation of the suture line. Dramatic improvement in speech has been noted in the remaining forty-seven patients. While it is difficult to compare the speech results to other procedures, a significant finding has been achievement of satisfactory speech in all nine patients referred for correction after failure with superiorly or inferiorly based flaps. Muscle survival, as evidenced by consistent electromyograph studies, demonstrated functioning double pharyngeal sphincters to control airway shunting during rapid spontaneous speech. The use of transverse pharyngeal flaps offers an obturator effect and the added benefit of active muscle for airstream control in speech function to correct nasality in cleft palate patients. Preservation of the integrity of the neuromuscular component of these pedicles assures survival of the tissue mass for its obturator effect. A modified procedure has been adapted for patients with failure of production of acceptable speech, after using inferiorly or superiorly based flaps.

Adolescent↗

PubMed1975

Location of the levator veli palatini insertion following levator retropositioning, palatal pushback, and pharyngeal flap procedures.

A long-term cineradiographic follow-up study of twenty patients with velo-pharyngeal incompetence fails to demonstrate predictable retrodisplacement of the levator insertion following combined levator retropositioning, pushback, and pharyngeal flap procedures. Simple levator retropositioning gave posterior displacement in the two patients evaluated. Patients with normal or posterior levator insertions pre-operatively all demonstrated post-operative anterior displacement following pharyngeal flap procedures, either alone or in combination with pushback. Anterior levator displacement may be the result of scar contraction or division of the levator sling (during insetting of a pharyngeal flap). Pre-operative and post-operative speech evaluation demonstrated substantial improvement in all except 3 patients; 2 of the poor speech results were patients with demonstrated levator retrodisplacement on post-operative cineradiography.

Adolescent↗

PubMed2002

[Study on the status of oral pharyngeal carriage of Haemophilus influenzae in healthy preschool children in Fuzhou city].

OBJECTIVE: To study the status of oral pharyngeal carriage and characteristics of Haemophilus influenzae (Hi) in healthy preschool children in Fuzhou. METHODS: Six hundred and three healthy children in two representative kindergartens in Fuzhou were studied as research subjects, and the rates of oral pharyngeal carriage of Hi were studied in four seasons. All Hi strains were serotyped and biotyped. RESULTS: The oral pharyngeal carriage of Hi in day care nursery healthy children were 36.7% in winter, 18.0% in autumn, 12.4% in summer and 10.9% in spring respectively. Serotype Hib was preponderant in autumn (6.9%). In winter, the carriage rates of NTHi and Hib were 17.1%, 5.4% respectively. The carriage rates of other serotypes were low. Biotype VII and VIII were preponderant in autumn, spring and summer but biotype VII and VIII were decreasing evidently in winter. CONCLUSION: There was evident seasonal difference in the rates of oral pharyngeal carriage and type of Hi in healthy preschool children. The carriage rate of Hi strains was high in autumn and winter. Results suggested that while the inoculation of Hib-binding bacterial vaccine was expanded the study on new bacterial vaccine of Hi still needs to be augmented.

Carrier State↗

PubMed2005

Does velopharyngeal closure pattern affect the success of pharyngeal flap pharyngoplasty?

Historically at The Hospital for Sick Children in Toronto, pharyngeal flap pharyngoplasty has been the treatment of choice for treatment of velopharyngeal insufficiency, regardless of velopharyngeal closure pattern. The authors hypothesize that pharyngeal flap pharyngoplasty is more effective in treating velopharyngeal insufficiency in patients with circular or sagittal velopharyngeal closure and less effective in treating the coronal closure pattern. Ninety-three patients who underwent superiorly based pharyngeal flap surgery for velopharyngeal insufficiency were evaluated in a retrospective chart review. Closure pattern was determined preoperatively by nasopharyngoscopy or multiview videofluoroscopy. Nasalance was assessed preoperatively and at 6 weeks and 1 year postoperatively. Nasalance during nonnasal speech was decreased on average, for all closure patterns, postoperatively. However, a significantly higher percentage of patients were corrected to normal nasalance scores in thenoncoronal group than in the coronal group (57 percent versus 35 percent, respectively) at 1 year postoperatively (p < 0.05). Surgical overcorrection, as determined by postoperative hyponasality, occurred at a rate of 13 percent in the coronal group versus 7 percent in the noncoronal group (not statistically significant). The results demonstrate that hypernasality in patients with a coronal velopharyngeal closure pattern can be improved by pharyngeal flap pharyngoplasty. This procedure, however, is more frequently effective in correcting noncoronal closure pattern velopharyngeal insufficiency than coronal pattern velopharyngeal insufficiency. The authors are now more selective in their approach to the management of velopharyngeal insufficiency and are more inclined to treat coronal pattern velopharyngeal insufficiency with sphincter pharyngoplasty.

Adolescent↗