ENT Infection Research Articles

Study of Risk Factors for Nasopharyngeal Carcinoma: About 214 Cases

Nasopharyngeal carcinoma (NPC) is a malignant tumour of the nasopharyngeal epithelium. It is very aggressive; with geographical distribution, placing Morocco in an intermediate incidence zone; and multifactorial etiology. We report here 214 cases of NPC followed within the Radiation Oncology Department of the Mohammed VI University Hospital in Marrakech, 84.58% of whom presented with at least one risk factor. The most incriminated was ENT infection (41.12%). NPC, due to its characteristics, constitutes a public health problem that requires primary prevention. Knowledge of risk factors would make it easier to raise public awareness.

Incidence of pediatric tonsillitis, otitis and upper respiratory infectious entities in the pre and post COVID-19 quarantine eras

Background At this point of the COVID-19 pandemic, with the worldwide loosening of health restrictions, there has been an observed jump in infectious load especially of the upper airways. Aims/Objectives: To shed light on children’s immunity and potential health risks after the COVID-19 pandemic. Methods A retrospective chart review from May 2019 to January 2022. Pediatric patients with a discharge diagnosis suggestive of an upper respiratory or ENT infection were included. The sample was divided into three groups according to the date of presentation. Results A total 4356 patients were diagnosed with ENT infectious aetiology. The mean age was 4.69 years. The three periods studied were: Period-1 (May 2019–January 2020), period-2 (February 2020–April 2021) and period-3 (May 2021–January 2022). The distribution of adenoiditis and MEE is the same across all periods (p > .05). The incidence of URTI, AOM and tonsillitis were significantly highest during period-3 followed by period-1, which in turn was significantly higher than during period-2 (p < .05). The incidence of sinusitis was the highest during period-3 (p < .001). Conclusion There seems to be a heightened susceptibility to acute infection in children after the pandemic. Significance: It is important to keep in mind the changes in microbiota and implement measures to promote healthy gut flora, timely vaccination, and prompt medical interventions. Summary Box What is already known: We already know that quarantine has significantly decreased infectious load especially in children. This study adds an objective assessment of this decrease with an assessment of the infectious load post-quarantine. This study is a model for future pandemics on the importance of vaccinations and the importance of microbiota changes after pandemics.

A simple infection? A pictorial review of rare but serious complication of ENT infection in children

A simple infection? A pictorial review of rare but serious complication of ENT infection in children

Особенности иммунореактивности организма в постковидном периоде у полиморбидных пациентов с хроническими очагами инфекции ЛОР-органов и полости рта

The high level of morbidity and labor losses among the population during the epidemic of a new coronavirus infection is largely determined by the protracted course of the post-COVID syndrome, which is often complicated by the exacerbation of chronic foci of infection of the upper respiratory tract and oral cavity. Clinical manifestations of a developed immunodeficiency state require an assessment of the characteristics of the body’s immunoreactivity in patients who have undergone COVID-19 in order to develop targeted programs for their immunorehabilitation and prevent manifestations of chronic focal infection, which was the purpose of the study. Immunological examination of 37 patients aged 42 to 60 years (53 ± 3.8 years), 24 women and 13 men, in the post-COVID period with exacerbation of chronic ENT infection (nasopharyngitis, sinusitis, otitis media, laryngitis) and dental pathology (generalized inflammatory periodontal disease, apical granulomas) showed the formation of an immunological imbalance with a quantitative deficiency of T-helpers with a high level of cytotoxic T-lymphocytes and NK cells expressing the alpha chain of the CD8 antigen as well as cytokine-producing NK cells against the background of a pronounced decrease in the resources of alpha and gamma interferon and a high level of Ig E and CIC in the blood. The established immunological prerequisites for comorbid infection, allergization, autoimmunization, and chronic sequel of inflammatory response motivated approaches to the rehabilitation of patients in the post-COVID period and the prevention of activation of chronic foci of infection in the ENT organs and oral cavity through sequential detoxification and immunocorrection. The positive experience of using bioactive preparations of natural origin with a complex detoxifying, immunotropic, antioxidant, and adaptogenic action is presented.

P064 Henoch-Schonlein Purpura: experience of a general pediatric center

Abstract Background Henoch-Schonlein purpura (HSP) is the most classic dysimmune vasculitis in children, related to an abnormal response of an immature immune system following an external antigen attack. Digestive involvement in HSP conditions the immediate vital prognosis and severe renal failure conditions the long-term functional prognosis. The Objective is to identify the epidemiological, clinical, disease course and therapeutic particularities of patients with HSP with a review of the literature. Material and method The authors reported the results of a retrospective study of cases of patients with HSP hospitalized and treated in the 02 pediatric departments of the wilaya of Annaba in Algeria (Clinique Sainte Thérèse, Annaba hospital university center and EHS El Bouni) over a period of 02 years. Information was obtained from hospital records. Results 24 cases of HSP were identified with a sex ratio of 1.4 (14 boys and 10 girls) and a hospital incidence of 1.66%. The average age was 6.6 years. Half of patients was hospitalized during the autumn-winter period, 67% of whom had an ENT infection before their hospitalizations. 17% of cases have personal history of HSP (the oldest was 5 years ago and the most recent 10 days before hospitalization). 7 patients underwent surgeries prior to hospitalization. Skin lesions were found in all patients. Joint, digestive and renal involvement were recorded in 50%, 80% and 25% respectively. Gastrointestinal bleeding has been observed in one patient. Only 1 patient with renal impairment progressed to chronic renal failure and only 1 patient had confirmed hypertension. A rare association with viral hepatitis A has been noted in one patient. Conclusion HSP remains a benign condition, easy to recognize clinically. Its immediate prognosis depends on the digestive complications and its long-term on nephropathy which may expose to the risk of chronic renal failure.

COVID-19 and severe ENT infections in pediatric patients. IS there a relationship?

BackgroundPreliminary evidence suggests that children are just as likely to become infected with SARS-CoV-2 as adults but are less prone to developing severe clinical conditions. However, there are pediatric inflammatory conditions that have also been encountered. The aim of this report is to determine whether there is a relationship between COVID-19 and severe infections in the ear, nose, throat, and deep cervical area (ENT) in pediatric populations. Materials and methodsA compilation was made of all the cases of ENT area infections in the pediatric population per month attended to at the Niño Jesús University Children's Hospital from January 2010 to June 2020. Endemic channels and dispersion analysis were designed to analyze the incidence presented in the year 2020, compared to what was expected based on historical data from 2010 to 2019. Then, an epidemiological interview was conducted of the close contacts of COVID-19 of the children who presented a severe ENT infection in 2020. Finally, a serological test of IgG antibodies was performed on all of them to find out if they had overcome the COVID- 19. Results620 patients from 1022 were eligible for the study. We observed a significant outbreak in the incidence of complicated mastoiditis and deep cervical infections with complications in the year 2020 (13 patients) linked to the COVID-19 pandemic. From these patients, 54% had been confirmed or had high suspicion of close contact with COVID-19.15.4% of children were positive in serological tests for IgG antibodies. ConclusionThere has been a significant increase in mastoiditis and deep cervical infections with complications in the first four months of 2020, which constitutes an outbreak. A considerable number (54%) of these complicated infections were related to close contact with COVID-19. Still, only 15.4% were positive in serological tests for IgG antibodies, so we cannot establish a direct categorical relationship. The limitations in primary care due to a shortage of human resources in dealing with the pandemic's initial onslaught and changes in help-seeking behavior could explain increased complicated infections.

Pasteurella multocida acute epiglottitis

IntroductionApart from cases related to direct inoculation, pasteurellosis is a rare opportunistic infection occurring in predisposed subjects. Close contact with domestic animals, usually cats, is generally reported. Localized ENT forms are possible and are due to oropharyngeal carriage. Case reportWe present the case of a patient with no notable history, who presented with laryngeal dyspnea and hyperthermia leading to a diagnosis of acute epiglottitis. Bacteremia was detected and blood cultures were positive for Pasteurella multocida. Treatment consisted of the standard treatment for acute epiglottitis with hospitalisation and intravenous antibiotics. DiscussionThis patient presented a history of animal exposure, but no other known risk factors. The activity spectrum of antibiotic therapy for epiglottitis should include H. influenzae and this case illustrates the diversity of the micro-organisms potentially involved. Immunosuppression or another chronic disease does not appear to be a prerequisite for ENT infection.

Current trends in prescribing antimicrobial drugs on patients suffering from ENT infection in a teaching hospital in Khulna

Background: Inappropriate use of antimicrobials results in development of resistance to antibiotics.&#x0D; Aims and objectives: The present study was aimed at evaluating the current trends in prescribing antimicrobials in patient sufferings from ENT infection.&#x0D; Methods: A descriptive cross sectional study was carried out at ENT out-patient department of Khulna Medical College Hospital for three months in which data were collected from 500 prescriptions in customized proforma and analyzed using the World Health Organization (WHO) prescribing indicators.&#x0D; Results: Majority of the patients were females (55%) which was commonly seen between the age group of 15-24 years (39.2%).The most common diseases reported was Recurrent tonsillitis (16.4%) followed by Pharyngitis (15%), CSOM (13.2%). Commonly prescribed oral antimicrobials were â-lactams(39.72%) followed by fluroquinolones 16.16% and macrolides(4.98%). Among the â-lactams, Cefuroxime+clavulenic acid(20.39%) was more used followed by amoxicillin+clavulanic acid(15.55%), and among fluroquinolones was ciprofloxacin 9.81%and among macrolides was azithromycin(4.98%). 57.70% patients received single antimicrobials whereas 42.3% received multiple antimicrobials. Average number of antimicrobials prescribed per patient being 1.32. Concomitant therapy included antihistamines(22.93%), Nasal decongestant(21.56%). Average number of drugs per prescription was 3.07. All drugs were prescribed in brand names. Oral and topical preparations were frequently prescribed rather injection prescribed was nil. Percentage of encounters with an antibiotic prescribed was100%. and only 28..72% drugs were prescribed from Essential Drug List of Bangladesh 2008.&#x0D; Conclusion: Prescribing multiple antimicrobials in this study without definite culture and sensitivity reports make the study less rational in accordance with the standard guidelines.&#x0D; J Dhaka Medical College, Vol. 27, No.2, October, 2018, Page 155-160

594. A Multi-Centered Study of the Clinical and Molecular Epidemiology of AmpC Cephalosporinase-Producing (AmpC) Enterobacteriaceae (Ent) Infections in Children

BackgroundAmpC producing Ent are an important cause of multidrug-resistant (MDR) infections in pediatrics. Since most AmpC Ent studies have been conducted in adults, we characterized the molecular epidemiology of AmpC Ent strains with transmissible resistance and identified factors associated with AmpC Ent infections in children.MethodsA case–control study of children (0–18 years) at 4 Chicago hospitals during 2011–18 was performed. Cases were 44 children with infections due to Ent harboring an AmpC as detected by DNA microarray (Check-Points®). PCR, DNA sequencing, MLST, and phylogenetic analyses were performed. Controls (ctrls) were 132 children with expanded-spectrum cephalosporin-susceptible Ent infections matched by age and hospital. Demographics; residence; comorbidities; device, antibiotic, and healthcare exposures were evaluated. Predictors of AmpC Ent infection were assessed by logistic regression.ResultsThe median age of AmpC Ent patients was 3.0 years; 50% were male. Of blaAmpC genes, 68% were blaACT/MIR-type and 25% blaCMY-type. Predominant organisms were Enterobacter cloacae (59%) and Escherichia coli (32%); 27% of AmpC E. cloacae belonged to ST114 and 62% co-harbored a blaESBL gene, predominately blaSHV (94%). Most AmpC E. coli strains were unrelated; 71% carried blaCMY, 64% belonged to phylogroups B2/D, and 50% co-harbored blaCTX-M. On bivariate analysis vs. ctrls, AmpC Ent infections were more likely to be respiratory (39% vs. 18%, P < 0.01) and less likely to be urinary tract (41% vs. 67%, P < 0.01) or community-acquired (14% vs. 33%, P < 0.02). By multivariable analysis, children with AmpC Ent infections were more likely to be nonwhite, non-black, non-Hispanic (OR 4.7, CI 1.4–16.1) and have infections due to Enterobacter (OR 7.7, CI 3.5–17). Differences in gender, healthcare location, residential neighborhood, antibiotic exposures, comorbidities, devices or outcomes were not found.ConclusionAmpC Ent infections often had healthcare onset, were due to Enterobacter, and occurred in nonwhite, non-black, non-Hispanic children. AmpC Ent commonly co-harbored blaSHV and blaCTX-M ESBL genes which affects therapeutic options and suggests the need for contact precautions. Control of AmpC Ent infections in children will require validating sources and risk factors.Disclosures All authors: No reported disclosures.

The Grisel's syndrome: A non-traumatic subluxation of the atlantoaxial joint

IntroductionGrisel's syndrome consists in rotational subluxation of C1–C2 following ENT infection or surgery. There is no consensus on management. We present 2 cases requiring surgical treatment in our center. Case reportsTwo 10-year-old patients presented torticollis with cervical pain resistant to medical treatment, with onset a few months after tonsillectomy. In both cases, radiological assessment, comprising CT scan and MRI, showed Fielding–Hawkins type-3 C1–C2 rotational subluxation, without ligament lesion. After failure of conservative treatment, posterior reaming, realignment, C1–C2 arthrodesis using lateral masses and pars interarticularis screws and bone graft achieved good fusion and immediate spinal stability in all planes of the atlantoaxial complex. DiscussionGrisel's syndrome consists in non-traumatic subluxation of the atlantoaxial joint with intact atlantoaxial ligaments. Initial pharyngeal inflammation spreads to the prevertebral fascia via direct connections between the periodontoidal venous plexus and pharyngovertebral veins, inducing fasciitis that leads to abnormal relaxation of the atlantoaxial ligaments and reactional muscle contraction with ankylosis. This phenomenon, appearing gradually and insidiously over a period of a few weeks, creates a frozen joint with ankylosis. Medical treatment with NSAIDs, muscle relaxants, and immobilization is usually sufficient; cervical traction may be needed. Surgical treatment by C1–C2 arthrodesis is indicated in case of failure of medical management or onset of neurologic signs. ConclusionClose collaboration between pediatricians, ENT surgeons and neurosurgeons is essential for early diagnosis and management, which is the main prognostic factor for successful medical treatment, avoiding surgery.

Antibiotherapy management in respiratory infections in ambulatory in Tunisia

Acute respiratory tract infections are the commonest acute conditions managed in general practice and the main causes of antibiotic use. In Tunisia, the rates of antibiotic prescriptions remain high in general practice. In order to assess antibiotics prescribing practices of 23 general practitioners when facing an ENT or a respiratory tract infection, we conducted an observational study from February 1st, 2014 to November 25th, 2014.&#x0D; The study involved 374 patients, 45.6 mean age of years, among them 193 men (51.6%) and 174 (51.9%) with comorbidities. Tonsillitis, acute maxillary sinusitis and acute bronchitis are the main diagnosis, accounting for over 90% of ENT and respiratory tract infection. The diagnosis of these infections was based on clinical criteria, additional tests were prescribed for only 75 patients (20%).&#x0D; An antibiotic was prescribed for 360 patients (96.2%). β-lactams were the main prescribed class of antibiotics (58.3%). The antibiotic treatment was considered as inappropriate in 42.5% of prescriptions. The main causes were the prescription of amoxicillin-clavulanic acid during tonsillitis, non-antipneumococcal fluoroquinolones, when streptococci or streptococcus pneumoniae are presumed to be responsible for infection (tonsillitis, acute maxillary sinusitis, pneumonia), and unnecessary combination of antibiotics.&#x0D; A concomitant treatment was prescribed for 365 patients (97.6%). The most prescribed drugs were antipyretics (71.4%), corticosteroids (44.7%) and mucolytic (30.2%).&#x0D; The outcome was favorable in 333 cases (89%) and unfavorable in 26 cases (7.1%). The total average cost of an infectious episode was 79.776 ± 36.988 TND (45 ± 20 USD)., with a minimum and maximum values respectively 19.5 and 315.5 TND (35 to 558 USD).&#x0D; This study highlights the frequency of antibiotics prescription (96.2%) during supposed viral infections such as acute bronchitis, tonsillitis or when antibiotic prescription has to be debatable such as in acute otitis media, sinusitis maxillary or exacerbations of COPD.&#x0D; The over-use of antibiotics is an important concern in many countries of the region leading to emergence of bacterial resistance. National guidelines should be developed in order to promote rational use of antibiotics.

Calculating hospital length of stay using the Hospital Episode Statistics; a comparison of methodologies

BackgroundAccurate calculation of hospital length of stay (LOS) from the English Hospital Episode Statistics (HES) is important for a wide range of audit and research purposes. The two methodologies which are commonly used to achieve this differ in their accuracy and complexity. We compare these methods and make recommendations on when each is most appropriate.MethodsWe calculated LOS using continuous inpatient spells (CIPS), which link care spanning across multiple hospitals, and spells, which do not, for six conditions with short (dyspepsia or other stomach function, ENT infection), medium (dehydration and gastroenteritis, perforated or bleeding ulcer), and long (stroke, fractured proximal femur) average LOS. We examined how inter-area comparisons (i.e. benchmarking) and temporal trends differed. We defined a classification system for spells and explored the causes of differences.ResultsStroke LOS was 16.5 days using CIPS but 24% (95% CI: 23, 24) lower, at 12.6 days, using spells. Smaller differences existed for shorter-LOS conditions including dehydration and gastroenteritis (4.5 vs. 4.2 days) and ENT infection (0.9 vs. 0.8 days). Typical patient pathways differed markedly between areas and have evolved over time. One area had the third shortest stroke LOS (out of 151) using spells but the fourth longest using CIPS. These issues were most profound for stroke and fractured proximal femur, as patients were frequently transferred to a separate hospital for rehabilitation, however important disparities also existed for conditions with simpler secondary care pathways (e.g. ENT infections, dehydration and gastroenteritis).ConclusionsSpell-based LOS is widely used by researchers and national reporting organisations, including the Health and Social Care Information Centre, however it can substantially underestimate the time patients spend in hospital. A widespread shift to a CIPS methodology is required to improve the quality of LOS estimates and the robustness of research and benchmarking findings. This is vital when investigating clinical areas with typically long, complex patient pathways. Researchers should ensure that their LOS calculation methodology is fully described and explicitly acknowledge weaknesses when appropriate.

A survey of prescription pattern of antibiotic drugs on patients suffering from ENT infection within Dhaka metropolis

Background: This survey was designed to assess and evaluate the prescription pattern of antibiotic drugs on patients suffering from ENT infection within Dhaka metropolis.Methods: A cross sectional, observational and prospective study was conducted from January to June 2015 in the out-patients (OPD) and in-patients (IPD) of ENT department at different general and specialized government and private hospitals (Dhaka Medical College and Hospital, Sir Solimullah Medical College Mitford Hospital, Holy Family Red Crescent Medical College and Hospital, Dhaka Community Medical College and Hospital) within Dhaka city.300 prescriptions were collected and randomly evaluated for this present study.Results: Out of 300 patients, 220 (73.33%) were male and 80 (26.67%) were female (including children and adults) where most of the patients were outpatients 262 (87.33%). In the patients information section it was observed that approximately 93.33% prescription contained antibiotic drugs and almost all prescription (100%) contained antibiotic drugs along with other drugs such as 80.67%PPI (proton pump inhibitors), 76.67% analgesic and 51.33% vitamin / iron supplements. From this analysis we found that 19.33% prescription contained single antibiotic drug, 80.67% contained two antibiotic drugs and no prescription contained more than two antibiotic drugs. Most of the prescribed drugs were administered orally (12% capsule, 80% tablet form). Out of 473 prescribed antibiotic drugs majority of them lie underβ-Lactam (54%) class followed by cephalosporin (46.33%) class in which maximum drugs (92%) were prescribed by their brand names.Conclusions: Prescribing more than one antibiotics was commonly encountered in this study indicating the occurrence of polypharmacy. Interventions to rectify over prescription of antibiotics, use of brand names, inadequate labelling of drugs is necessary to improve rational drug use. Standard treatment guidelines, hospital formulary, and educational intervention become essential to modify this behaviour to benefit the patient.

Cerebellopontine angle subdural empyema in a 2-years old patient with bilateral mastoiditis - a life-threatening condition. Case presentation and review of literature

AbstractInfratentorial subdural empyema is a life-threatening condition, the common source being an ear infection. We present a 2-year old boy treated for bilateral mastoiditis, with infratentorial-left cerebellopontine angle subdural empyema. Clinical presentation encompassed a systemic febrile illness, headaches, and a stiff neck. Empyema was diagnosed with computed tomography and magnetic resonance imaging. The patient was successfully treated with surgery and appropriate antibiotics. Empyema should be considered in patients with ENT infection associated with neurological signs that suggest a posterior fossa lesion.

Prevalence of uncontrolled allergic rhinitis in Wuhan, China: a prospective cohort study.

Allergic rhinitis (AR) is a highly prevalent disease that affects the quality of life, especially in the "severe chronic upper airway disease" (SCUAD) group of patients who still have severe symptoms after adequate treatment. This study investigated the prevalence of uncontrolled AR and SCUAD consulting in the Allergy Department of Tongji Hospital, Wuhan, China. In this prospective cohort study, all patients consulting for AR were prospectively assessed using visual analog scale (VAS) and Allergic Rhinitis Control Test (ARCT) and put on standardized treatment based on the Allergic Rhinitis and Its Impact on Asthma (ARIA) guidelines. After 15 days, they were reevaluated by a telephone interview using a numerical scale (NS) and ARCT. A score of ARCT of <20 defined uncontrolled AR and a score of NS of ≥5 at day 15 defined SCUAD patients. A total of 252 patients were included. Moderate/severe AR (VAS ≥ 5) was diagnosed in 82.9% of the patients which had an impact on sleep (86.9%), work life (84.9%), social activities (81%), and physical activities (90.1%). Patients with uncontrolled AR (27.7%) at day 15 more frequently presented a higher weight (p = 0.042), history of ear, nose, and throat (ENT) infection or antibiotics intake for respiratory infection in the last 12 months (62.3% versus 45.6%; p = 0.018), smoking (15.9% versus 6.7%; p = 0.024), and smell disturbance (26.1% versus 11.7%; p = 0.005). Patients with SCUAD (24.5%) more frequently presented a history of ENT infection or antibiotics intake for respiratory infection in the last 12 months (63.9% versus 45.7%; p = 0.014) and smell disturbance (27.9% versus 11.7%; p = 0.003), and less commonly had atopic dermatitis (13.1% versus 28.2%; p = 0.017). Uncontrolled AR and SCUAD patients are numerous. VAS and ARCT are simple and quantitative methods and self-completion questionnaires that can be used for a global evaluation of the severity and control of AR.

Therapy of sinus venous thrombosis in childhood – A standardised treatment protocol – Call for participation

Aims: Cerebral sinus venous thrombosis (CSVT) is a potentially life-threatening disease causing 13–22% of all strokes in childhood, in two thirds associated with infection (mainly ENT infection). Clinical manifestations may be subtle and diagnosis delayed because of the wide spectrum of clinical signs. In adults, the first line treatment is anticoagulation – i.v. heparin or LMWH – based on controlled trials. Randomised controlled trials focusing on therapeutic guidelines for children with CSVT are still lacking. Actually recommendations for AC are given considering literature analysis, mainly based on study results in adults.

S258 – Rapid Intracranial Spread of ENT Infection in B-thalassaemia

Objectives 1) To find out the duration of onset of intracranial complications following middle ear and paranasal sinus infection in B-thalassaemia. 2) To find out the contributory factors for such complications. Methods A retrospective study of thalassaemic children who suffered from intracranial complication following ear and sinus infections between Jan '02- Oct '04, was done at a tertiary hospital Male', Maldives. Follow-up data were obtained from National Thalassaemia Centre. Patients' demographic pattern, thalassaemic status, Desferroxamine therapy, duration of the ear/nose symptom, the intracranial complications, the treatment and the outcomes were noted. We didn't include 3 patients, for whom full data were not available. Results We found the records of a series of 5 consecutive patients out of 94 thalassaemic patients which were referred with different ear and nose infections and were treated for intracranial complications. All patients were in the age group of 10–15 years. All were female. Spread of infection from the ear was more common than the spread from sinuses (3 out of 5 patients). The intracranial complications developed in these patients within few days (&lt;5 days) of presentation in the clinic. All of the patients were on regular blood transfusion with desferroxamine therapy but none had spleenectomy. Most common organism found was klebsiella. Conclusions Persistent anaemia, repeated transfusion, iron overload, and hypersplenism are the major contributory factors for frequent infections in thalassaemics. Natural thinning of the bony cortex and remodelling of skull in thalassaemics can be considered as an additional factor for the rapid development of intracranial complications.

Down syndrome: otolaryngological effects of rapid maxillary expansion

Phenotypical Down syndrome includes pharyngeal and maxillary hypoplasia and, frequently, constricted maxillary arch with nasal obstruction. This clinical trial assessed the effects of rapid maxillary expansion on ENT disorders in 24 children with Down syndrome randomly allocated to receive either rapid maxillary expansion or not. Each group received ENT and speech therapy assessments before expansion and after the device had been removed. In the rapid maxillary expansion group, the yearly ENT infection rate was reduced when assessed after device removal (p < 0.01). The parents of rapid maxillary expansion children reported a reduction in respiratory obstruction symptoms. Audiological assessment revealed improvements in the rapid maxillary expansion group (p < 0.01). Cephalometry showed increased maxillary width in the rapid maxillary expansion group. Rapid maxillary expansion resulted in a reduction in hearing loss, yearly rate of ENT infections and parentally assessed symptoms of upper airway obstruction, compared with no treatment. These findings are probably related to expanded oronasal space, due to rapid maxillary expansion.

Pegfilgrastim in Congenital Neutropenia: An Observational Survey from the French SCN Registry.

PegFilgrastim is registered in France since 2002. Its pharmacokinetic characteristic allows the possibility to limit the number of injections in patients with congenital neutropenia receiving long term G-CSF therapy. To date, no report has been issued about the outcome of patients with congenital neutropenia receiving this drug. At the cut-off date of July 31th, 2007, 339 patients with congenital neutropenia had been included in the French Severe chronic neutropenia (Severe congenital neutropenia n=142, WHIM n=16, Cyclic n=75; GSDIb n=16 SDS n=90). Eight patients (SCN n=4, Cyclic n=3 and GSDIb n=1) had been identified to receive PegFilgrastim. Median age at PegFilgrastim onset was 18.2 years (min 6.1-max 37.6). In 7 cases, PegFilgrastim was given in the continuation of Filgrastim (n=3) or Lenograstim(4), with a median delay of 13 years after G-CSF onset and in one case, PegFilgrastim was the initial prescription of cytokine. Dose of PegFilgrastim was usually a vial per injection, except in one child (1/2vials) resulting in a dose per injection between 90 and 286 μg/kg per injection. The rhythm of PegFilgrastim injection varied from 1 injection every 7 days until 1 injection every 14 days, with discontinuation period. The median duration (between onset of PegFilgrastim and the last dose) was 0.7 year (min 0.3–2.7). The efficiency measured by ANC criteria appears to better than G-CSF (fig 1: baseline Median ANC 156/mm3, Under G-CSF 501/mm3, Under PegFilgrastim 1901/mm3/ Kruskall Wallis test p=0.02). Severe infection was never reported under PegFilgrastim, but neither in the previous G-CSF therapeutic periods for the 7 patients. More frequent ENT infection was observed in one patient who consider the PegFilgrastim as less efficient than G-CSF. The side effects appeared to be more frequent compare to the G-CSF therapy. Bone pains were reported in 7 patients, anemia and thrombocytopenia (WHO gr II) in one, urticarian chronic skin rash in 3. Finally, at the last update, 7 patients had withdrawn PegFilgrastim, and had resumed G-CSF therapy. Only one patient is still receiving PegFilgrastim. In conclusion, PegFilgrastim appeared efficient in Congenital neutropenia, but more frequent short term side effects were observed compare to standard G-CSF therapy, resulting in a limited quality of life improvement, leading to drug withdrawn in most of the patients. [Display omitted]

Frontal Ataxia in Childhood

Frontal ataxia may be the result of a unilateral frontal lesion. In this report three cases are presented with ataxia due to right frontal lesions. One case concerns a boy presenting with an unsteady gait and titubation of the trunk, mimicking developmental disequilibrium and with complex partial seizures. It proved to be caused by a small right-sided cavernoma in the middle frontal gyrus. After surgical intervention the symptoms and the seizures disappeared. Two subsequent cases concern teenage patients presenting with headache after an ENT infection and on physical examination mild dysmetric function of the upper limbs and slight disequilibrium, due to right-sided frontal lobe abscesses. After neurosurgical and antibiotic therapy the symptoms were relieved. The frontal origin of ataxia should be considered in children presenting with a "cerebellar syndrome". Frontal gait disorders consist of a clinical pattern of different gait disorders. The syndrome has been mentioned in the literature under different names. Our patients show signs compatible with the term frontal disequilibrium, a clinical pattern of frontal gait disorder. This assumes walking problems characterized by loss of control of motor planning, leading to imbalance. Remarkably, frontal ataxia may mimic developmental delay as demonstrated in the first case and may be the leading mild symptom in extensive frontal lobe damage as demonstrated by the two other cases. We suppose that frontal ataxia is the result of a disturbance in the cerebellar-frontal circuitries and an impairment of executive and planning functions of the basal ganglia-frontal lobe circuitry.