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Biomedical subjects

B R Maddern

Publications and source records attributed to B R Maddern.

11 recordsLinked to original sources

Cochlear implantation in Jervell and Lange-Nielsen syndrome.

Jervell and Lange-Nielsen syndrome represents a rare, autosomal recessive cause of congenital deafness. Affected patients have a characteristic prolongation of the QT interval on electrocardiogram, along with cardiac arrhythmias, recurrent syncopal episodes, and a predisposition to sudden death. We present the first reported case of cochlear implantation in a child with Jervell and Lange-Nielsen syndrome. Special perioperative precautions were required, including cardiac monitoring for 48 hours, perioperative beta blockade, and special anesthetic considerations. Because sudden patient death has been associated with exposures to loud sounds in patients with prolonged QT syndrome without hearing loss (Ward-Romano syndrome), initial cochlear implant stimulation was performed with cardiac monitoring. This patient has responded well to cochlear implantation with 11 months of implant use. The patient has achieved limited open-set word comprehension and significantly improved speech, as is expected for her age. Although the patient had no problems with cardiac arrhythmias at surgery, she has since experienced a syncopal episode requiring placement of an automatic pacemaker and defibrillator. The implant has continued to work well despite the pacemaker and defibrillator. Cochlear implantation can be relatively safely performed in patients with Jervell and Lange-Nielsen syndrome, provided proper precautions are followed.

Child, Preschool↗

A role for tonsillectomy in the treatment of psoriasis?

Our objective was to determine whether tonsillectomy is beneficial in the treatment of recurrent childhood guttate psoriasis that is associated with recurrent streptococcal pharyngitis and tonsillitis. We retrospectively reviewed the cases of two children who were referred to our facility for treatment of repeated exacerbations of psoriasis and recurrent streptococcal pharyngotonsillitis. Both patients experienced a significant improvement in their psoriasis after undergoing adenotonsillectomy, and both were completely free of psoriatic outbreaks after 16 months of follow-up. We conclude that tonsillectomy appears to be of benefit in the treatment of children with recurrent guttate psoriasis and recurrent streptococcal pharyngotonsillitis, and we hope that further investigation will be undertaken.

Child↗

Pediatric voice disorders.

Voice disorders are common among children, estimated to range from 6% to 23% in school-aged children. The various causes of voice disorders are presented in this article in addition to a discussion of normal laryngeal voice and development. Also presented are the causes of abnormal voice and brief discussion of a framework for multidisciplinary assessment and treatment of pediatric voice disorder.

Child↗

Obstructive sleep apnea syndrome in sickle cell disease.

We present a series of 21 children with obstructive sleep apnea syndrome and sickle cell disease (SCD). Thirteen patients underwent adenotonsillectomy with resolution of symptoms and improvement in alveolar hypoventilation. Adenotonsillectomy involves significantly increased risks in patients with SCD. We present our protocol for management of these difficult surgical patients, including vigorous hydration and transfusion therapy. Polysomnography has proven valuable both in diagnosis and in identifying severely affected patients. Increased end tidal alveolar carbon dioxide during sleep has proven to be a significant predictor of disease, while oxygen saturation monitoring was shown to be unreliable. The incidence of adenotonsillar hypertrophy (ATH) in SCD appears increased and not related to infectious diseases. We suggest that ATH represents a part of the natural course of compensatory lymphoid tissue enlargement in children with SCD.

Adenoidectomy↗

Posttracheotomy granulation tissue managed by carbon dioxide laser excision.

The problem of accumulation of granulation tissue and scar at the superior edge of the tracheostoma is a frequent problem in the management of chronic tracheotomy patients. This traditionally has been managed by cup forceps excision or by eversion through the tracheostoma with a skin hook and blind resection. These methods often lead to hemorrhage, and incomplete removal in a bloody field. We have used the carbon dioxide laser via a bronchoscope for ablation of the granulation tissue and/or scar at the stomal edge and at the tracheotomy tube proximal tip without morbidity in 13 pediatric cases. With experience, removal with the laser often proceeds more quickly than conventional methods. The scar and granulation tissue are excised under direct vision with minimal hemorrhage. We believe this to be a reasonable alternative in the management of this recurring problem.

Adolescent↗

Lingual thyroid in a young infant presenting as airway obstruction: report of a case.

A case of lingual thyroid causing airway obstruction in a 3-month-old infant is reported. Endoscopic examination for stridor disclosed an obstructing 4 X 3 cm base of tongue lesion. Nuclear isotope scanning confirmed this to be the only thyroid tissue present in this patient. A transoral excision using the CO2 laser was performed without complication. Only one other case of airway obstruction in the young infant secondary to lingual thyroid has been reported, that being the original case description in 1869. A review of the embryology and discussion of the suggested therapies is presented. The rationale for use of the CO2 laser for this lesion is discussed.

Airway Obstruction↗

A double-blind, placebo-controlled clinical trial of the effect of chlorpheniramine on the response of the nasal airway, middle ear and eustachian tube to provocative rhinovirus challenge.

This paper presents the results of a randomized, double-blind, placebo-controlled study of the efficacy of chlorpheniramine in relieving the symptoms and attenuating the pathophysiologic correlates of a rhinovirus "common cold." Forty healthy, adult, nonatopic subjects were randomly assigned to one of two treatment groups: active drug and placebo. On study Day 0, all subjects were challenged intranasally with rhinovirus type 39 (dose = 100 TCID50). Subjects were cloistered from Day 2 to Day 7, at which time they were treated with either chlorpheniramine or placebo. From 3 days before challenge to study Day 19, subjects had nasal patency assessed by rhinomanometry, eustachian tube function assessed by the 9-step test and sonotubometry, middle ear pressure assessed by tympanometry and nasal clearance assessed by the dyed-saccharin technique. Symptom diaries were maintained throughout the period of follow-up. During cloister, symptoms also were scored by interview, nasal secretions were quantified and nasal washings were performed for viral culture. Results showed that 19 (95%) subjects in the active-treatment group and 18 (90%) subjects in the placebo-treatment group shed virus. Symptomatic colds were observed in 63% of the active-treated and 83% of the placebo-treated subjects. Symptoms increased on Day 1 and peaked at Days 4 to 5. Detrimental changes in other measured functions consistent with those previously reported were observed. During the period of treatment, significant differences in the average symptom scores favoring the active-treatment group were observed for sneezing. Also, weight of expelled secretions was greater and mucociliary clearance rate less on some cloister days for the placebo-treated group. No significant differences between treatment groups in the objective measures of nasal congestion or the response of the middle ear and eustachian tube were documented.

Acoustic Impedance Tests↗