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Biomedical subjects

S S Johnston

Publications and source records attributed to S S Johnston.

14 recordsLinked to original sources

Evaluating the morphological competence of children with severe speech and physical impairments.

Reports present mixed findings on the extent to which the development of receptive language skills in children with severe speech and physical impairments (SSPI) is compromised by their difficulty with speaking (V. W. Berninger & B. M. Gans, 1986; D. V. M. Bishop, B. Byers Brown, & J. Robson, 1990; O. Udwin & W. Yule, 1990). In this study, grammaticality judgments were used to measure the sensitivity of 4 school-age children with SSPI to different morphological errors. These errors included violations of agreement between the subject and auxiliary verbs (e.g., she are falling), the marking of aspect (e.g., she is play the horn), and the marking of past tense on regular and irregular verbs (e.g., he jump, he fall, he falled). Performance of the participants with SSPI was compared to groups of typically developing children and adults. Results indicated that children in the SSPI and control groups made similar judgments. All groups showed high levels of sensitivity to agreement violations, aspect-marking errors, and tense-marking errors involving irregular verbs. Participants with SSPI had greater difficulty detecting tense-marking errors involving regular verbs. Implications for improving clinical assessments within this population are discussed.

Age Factors↗

Meningitis and midline facial deformity.

A baby with unilateral cleft lip, midline cleft palate and hypertelorism developed meningitis in the first 48 h of life. Examination of the nasopharynx showed a soft tissue mass, which was confirmed as a basal encephalocele by computed tomography. There was also congenital hydrocephalus and the corpus callosum was absent. Surgical treatment included repair of the anterior basal skull defect, repair of the lip and palate, and ventriculo-peritoneal shunt. There is currently evidence of developmental delay and right-sided visual impairment due to Morning Glory syndrome. This case demonstrates that basal encephalocele should be considered in any baby with midline facial deformity who develops meningitis.

Cleft Lip↗

Retinopathy of prematurity in surfactant treated infants.

Seventy six babies of less than 1500 g birth weight who had surfactant replacement therapy for severe respiratory distress syndrome were studied to assess the presence and stage of subsequent retinopathy of prematurity (ROP). A control group of 90 babies, matched for birth weight and gestational age, who did not have surfactant therapy were also studied. Threshold ROP or greater was found in 1.7% of the surfactant group and 7.8% of the controls. For the babies of less than 1000 g birth weight 4.0% of the surfactant babies and 16.3% of the controls reached threshold disease or greater. It is concluded that surfactant therapy is not associated with an increased incidence or severity of severe ROP in this preterm population.

Biological Products↗

Surfactant replacement therapy and the prevalence of acute retinopathy of prematurity.

To determine if surfactant replacement treatment is associated with an increase in the prevalence of retinopathy of prematurity (ROP) we studied 76 preterm babies who were treated with porcine surfactant (Curosurf) for severe respiratory distress syndrome from 1985 to 1990. Babies were first examined by indirect ophthalmoscopy at the equivalent of 32 weeks post-menstrual age and subsequently at 2-week intervals until discharge from hospital. Findings were documented according to the International Classification of ROP. Sixty-two (82%) babies survived to discharge, 7 survivors were not examined due to transfer elsewhere. Acute ROP developed in 14 (29%) of the 49 babies examined (7 stage I, 4 stage II, 2 stage III, and 1 stage IV); one baby required cryotherapy. No baby of birthweight greater than 1,500 g developed ROP. The prevalence of ROP was similar to that reported for non-surfactant-treated very-low-birthweight babies. We conclude that Curosurf treatment does not increase the risk of acute ROP in surviving very-low-birthweight babies.

Biological Products↗

The Morning Glory syndrome associated with sphenoethmoidal encephalocele.

A 10-month-old infant was referred to the ophthalmic department of the Royal Victoria Hospital for assessment. Pregnancy and delivery had been normal. At birth, cleft lip, cleft palate, hypertelorism and a soft tissue mass in the mouth were noted. At two days of age he developed meningitis, axial computerized tomography showed a sphenoethmoidal encephalocele, and agenesis of the corpus callosum. The basal encephalocele was surgically repaired. On examination in the ophthalmic department he was able to follow a light with the left eye. He perceived light with the right eye but did not follow. There was a manifest right divergent squint, measuring -10 degrees, and a right afferent nerve defect. Examination under anaesthetic was performed. The right fundus showed a Morning Glory syndrome. The disc was pink and deeply excavated, and surrounded by a ring of chorioretinal pigmentary disturbance. There was a central tuft of glial tissue. The left fundus was normal. The association of Morning Glory syndrome and basal encephalocele is rare; four previous cases have been reported. In all patients there were associated mid-facial congenital anomalies such as cleft lip and cleft palate. The presence of a basal encephalocele should be suspected if the Morning Glory syndrome occurs in association with mid-facial congenital anomalies.

Abnormalities, Multiple↗