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Biomedical subjects

B N Benjamin

Publications and source records attributed to B N Benjamin.

7 recordsLinked to original sources

Salivary gland choristoma of the middle ear.

A 5-year-old female with a so-called "salivary gland choristoma" in the middle ear is reported, believed to represent the nineteenth such case in the literature. The nineteen cases are reviewed. The only consistent symptom was deafness. Aural discharge, tinnitus and otalgia occurred occasionally. The findings included a middle ear mass, conduction deafness and sometimes facial and external ear developmental disorders. At surgery a lobulated firm mass in the posterosuperior tympanum was usually found, being mobile and often pedunculated from a dehiscent part of the horizontal facial nerve. Ossicular and outer ear developmental abnormalities were common.

Child, Preschool↗

Pathology of Tangier disease.

Two cases of Tangier disease are described in children from families unrelated to each other. Necropsy in one case, the first to be reported in this condition, showed large collections of cholesterol-laden macrophages in tonsils, thymus, lymph nodes, and colon, and moderate numbers in pyelonephritic scars and ureter. As the storage cells may be scanty in marrow, jejunum, and liver, the rectum is suggested as the site of choice for biopsy. The diagnosis was confirmed by demonstrating the absence of alpha-lipoproteins from the plasma of the living child, and by finding low plasma levels in both parents of both cases. The disease can be distinguished from other lipidoses by differences in the predominant sites of storage, staining reactions, and serum lipid studies.

Adolescent↗

Neonatal vocal cord paralysis.

The consultants agree that surgery is a common cause of unilateral vocal cord paralysis in neonates. In the absence of a history of surgery, they would evaluate a neonate for cardiovascular or central nervous system anomalies. None believes a relationship between laryngomalacia and vocal cord paralysis exists. But there is disagreement regarding the additional steps required to evaluate this child. The recommendations include endoscopy under general anesthesia with assessment of cricoarytenoid mobility, evaluation for other congenital anomalies, and observation of laryngeal dynamics (Dr. Benjamin), neurologic examination (Dr. Bailey), and no further testing (Dr. Gray). Laryngeal EMG in an infant is not an established technique and none of the consultants routinely performs this test. However, EMGs are part of the research protocol for one physician (Dr. Gray). The consensus is that aspiration is unlikely to be a problem in this case. However, if aspiration does occur, all would recommend conservative treatment. Feedings should be thickened and anti-reflux precautions taken. None was convinced that severe aspiration would be a problem. However, given the need for more aggressive treatment, the considerations would include collagen or Teflon injections or a tracheotomy (Dr. Gray) or a Nissen fundoplication, nasogastric tube feedings, or a gastrostomy (Dr. Bailey). Only one consultant would defer further treatment (Dr. Benjamin). The prognosis is generally good. Two consultants (Drs. Benjamin and Bailey) would follow a child with vocal cord paralysis by periodically repeating a laryngoscopic examination. A reinnervation procedure would be considered by one consultant at the age of 3 if the voice remains weak (Dr. Gray).

Diagnosis, Differential↗

Congenital agastria.

Congenital agastria has never before been reported. Congenital microgastria is rare but is well documented. We have cared for a Melanesian child in whom there was no radiological, anatomical, or physiological evidence of either a rudimentary stomach or a pylorus. The esophagus joined the first part of the duodenum directly. At this esophago-duodenal junction there was microscopic evidence of fundic-type gastric mucosa. The intestine was normally rotated and there was a functioning spleen. The child also had a severe micrognathia and a cleft soft palate and required a tracheostomy to relieve upper airway obstruction. The combination of micrognathia and agastria presented a difficult nutritional delivery problem. A jejunal pouch was constructed and placed between the esophagus and duodenum to provide some storage capacity. The child grew and developed well for almost 3 years. He suffered three episodes of severe acute enteritis, probably related to lack of the protective acid-pepsin barrier against ingested microorganisms. Unfortunately, the third episode of acute enteritis caused his death 3 months after returning home to his own country.

Child, Preschool↗

Alpha-interferon (Wellferon) as an adjunct to standard surgical therapy in the management of recurrent respiratory papillomatosis.

Ten patients received lymphoblastoid alpha-interferon (Wellferon) in a crossover study so that Wellferon and standard microsurgical laryngeal laser therapy could be compared to laser therapy alone. Wellferon was administered initially at an intravenous high dose of 15 megaunits/m2 for 5 days followed by a daily dose of 2 megaunits/m2 subcutaneously for 6 months. Dosage was adjusted according to predefined toxicity. One patient was withdrawn from the study. Of the others, all but one received over 75% of the planned total dose. At follow-up of the nine assessable patients, complete remission was achieved in two of them, partial remission in four, and no response in the remainder. The two complete remissions were sustained for 2 years, but the four partial remissions were not sustained. Thus, a role for alpha-interferon in the kind of regimen used here remains to be established.

Combined Modality Therapy↗