Eyelid opening in preterm neonates.

The frequency and patterns of eyelid opening were examined in a group of neonates (gestational age 24-35 weeks). Observations were spread over the 24 hour period to obtain a complete pattern of events. Mean eyelid closure was 74%.

Validation of a photographic method of measuring corneal diameter.

A photographic method for measuring corneal diameter using the Medical-Nikkor f200 mm lens is described. Measurements were compared with those obtained by calipers (46 eyes of 25 patients) and by placing a ruler either near the eye (123 eyes of 64 patients) or on the nose (98 eyes of 55 patients). Over all we found good correlation between photographic and caliper measurements (r = 0.

HLA class III haplotypes in multicase rheumatoid arthritis families.

The class III complement proteins (C2, BF, C4A, and C4B) were studied in 57 multicase rheumatoid arthritis (RA) families. When the gene frequencies for RA probands were compared to a normal control panel (162 haplotypes), a significantly higher frequency of the rare variant C4B*3 was observed (p less than 0.05).

Complement system protein C4 and susceptibility to hydralazine-induced systemic lupus erythematosus.

21 patients with systemic lupus erythematosus induced by long-term treatment with hydralazine were investigated to see whether susceptibility to this syndrome was associated with deficiency of the classical pathway complement protein, C4. 16 of 21 (76%) patients had one or more C4 null (ie, non-productive) alleles compared with 35 of 82 normal subjects (43%). This difference was significant.

Are severe acute retinopathy of prematurity and severe periventricular leucomalacia both ischaemic insults?

Over a period of 20 months six preterm infants have been seen who developed severe acute retinopathy of prematurity (ROP) and who also had ultrasound evidence of extensive cerebral parenchymal changes compatible with severe periventricular leucomalacia. Only one of these infants had a birthweight of less than 1000 g, and their gestational ages ranged from 27 to 30 weeks. The association between these two important complications of preterm birth has led us to postulate that an episode of hypoperfusion of the cerebral circulation sufficient to result in cerebral ischaemia could also reduce an already compromised ocular blood flow and further exacerbate retinal ischaemia, thereby increasing the severity of ROP.

Comparison of a new non-contact tonometer with Goldmann applanation.

A comparison of a new non-contact tonometer (Keeler Pulsair: Keeler UK Ltd) with the Goldmann applanation tonometer is reported. Measurements of intraocular pressure were obtained from 182 eyes of 94 patients. At low pressures (less than 10 mmHg) the candidate tonometer tended to overestimate pressures obtained with the Goldmann tonometer whilst at high pressures (greater than 19 mmHg) those obtained by Goldmann applanation were underestimated.

HLA-DR4 associated Dw types in rheumatoid arthritis.

Frequencies of HLA-DR4 and its related Dw types were compared between randomly selected normal controls and the index cases of multiplex rheumatoid arthritis (RA) families. A DR4 frequency of 68.3% was observed in index cases (n = 57) compared to 31.

Epidemiology of retinopathy of prematurity.

The incidence and severity of retinopathy of prematurity (ROP) were studied prospectively in a geographically defined area of the East Midlands. Over 23 months, 505 infants weighing 1700 g or less at birth to mothers resident in this area were studied. Acute ROP developed in 248 (49.

Effects of nursery illumination on frequency of eyelid opening and state in preterm neonates.

The behaviour of a group of premature neonates was observed under three conditions of ambient illumination defined as either DARK, LIGHT or BRIGHT (mean intensities: 0.9, 109 and 1370 lux). Trends in the data suggested that increasing the level of illumination resulted in a reduction in the proportion of time spent with eyelids open and of the amount of time spent in awake states.

Heterogeneity in the gene locus for steroid 21-hydroxylase deficiency.

DNA was analysed from 33 patients with congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency. In each case Southern blots were prepared from a number of restriction enzyme digests and hybridised with probes for both the 21-hydroxylase and the adjacent fourth component of complement (C4). Evidence for deletion of the active 21-hydroxylase gene (CYP21B) was found in 13 cases and in 10 of these the deletion included the adjacent C4B gene, leading to a hybrid CYP21A/CYP21B gene.

Amblyopia--factors influencing age of presentation.

In the 47 months from September, 1981, 1531 new cases of amblyopia were identified in the ophthalmic clinics of Leicestershire. Amblyopia was due to strabismus in 689 (45%), combined strabismus and anisometropia in 540 (35%), anisometropia alone in 252 (17%), and form deprivation in 50 (3%). The median age at presentation for strabismic amblyopia (3.

Do we need to measure the vision of children?

With the advent of the acuity card procedure, it is now possible to measure quantitatively the vision of infants and young children in a routine clinical setting. Over a 19 month period 1177 tests were performed on 586 patients; ages ranged from 0.5 weeks to 23 years.

C4 complement allotypes in juvenile dermatomyositis.

Twenty probands with juvenile dermatomyositis and their relatives were studied to determine the inherited segregation patterns of class I, II, and III HLA region markers including C4A, C4B, Bf, and C2 complement polymorphisms. The extended haplotype B8, DR3, C4A*Q0, C4B*1, C2*C, and Bf*S was present in 13 of the 20 probands. Three other probands also carried a haplotype with a null allele for C4A and two further probands carried a null allele for C4B; only two probands had no detectable C4 null allele.

Grating and recognition acuities of young amblyopes.

The visual acuities of 36 young amblyopes were determined by (a) conventional recognition tests (near and distance) and (b) an adapted grating acuity card procedure. Considerable agreement between the estimates of acuity obtained with each method was demonstrated, which was generally less than, or equal to, the mean difference between adjacent Snellen lines (4.5 c deg-1).

Is the geniculostriate system a prerequisite for nystagmus?

The time of onset of congenital horizontal nystagmus is usually known. We present a group of infants in whom this was determined objectively. In two, vertical nystagmus in the neonatal period became horizontally directed, at 4 months in one, and between 7 and 9 months of age in the second.

Light transmission through the human eyelid: in vivo measurement.

We describe an in vivo measurement technique for determining the transmission of light through the human eyelid and present data from three human subjects. The relevance of the transmission measurements are discussed in relation to (1) clinical electrophysiology and (2) the effects of light on the developing visual system.

Antigenic determinants expressed by human C4 allotypes; a study of 325 families provides evidence for the structural antigenic model.

The antigenic determinants of human C4 have been defined by human IgG antisera, Rodgers (Rg) and Chido (Ch), in hemagglutination-inhibition assays (HAI). Eight (2 Rg and 6 Ch) are of high frequency, greater than 90%, and 1, WH, is of low frequency, 15%. The phenotypic combinations are complex; generally, C4A expresses Rg, and C4B has Ch, but reverse antigenicities have been established both by HAI and by sequence data of selected C4 allotypes.

Family study of the major histocompatibility complex in HLA DR3 negative patients with systemic lupus erythematosus.

Susceptibility to systemic lupus erythematosus (SLE) is known to be governed by genes in the HLA region of the 6th chromosome. From previous studies it has not been possible to distinguish between the effects of null genes for the complement component C4 and HLA-DR3, because of the marked linkage disequilibrium between DR3 and a null allele of C4A (C4A QO) in caucasoid populations. We report here an immunogenetic study of 44 cases of SLE, selected because they were DR3 negative.

Inherited deficiency of erythrocyte complement receptor type 1 does not cause susceptibility to systemic lupus erythematosus.

There is a deficiency of complement receptor type 1 (CR1) on the erythrocytes of patients with systemic lupus erythematosus (SLE). This receptor is involved in the processing of immune complexes. Whether the deficiency is inherited or acquired has been the subject of controversy.

Neuraminidase deficiency: case report and review of the phenotype.

A 12 year old boy with neuraminidase deficiency (sialidosis, mucolipidosis I) is described. His clinical features included coarse facies, cherry red spot, ataxia, myoclonus, and dysotosis multiplex. The level of neuraminidase activity in cultured fibroblasts was very low and intermediate levels were observed in both parents.

Sudden-onset squint.

Differentiating a sudden-onset concomitant squint from a paralytic squint in an ill child can be difficult, but the distinction is important in view of the neurological implications of the latter. Furthermore, the two types may co-exist, and four such cases are described. The causes of sudden-onset squint are discussed.