THE PSYCH OLOG I CAL CHARACTER I STI CS OF INFANTILE HVPERCALCAEMR A PRELIMINARY INVESTIGATION -

Rosemary Arnold William Yule Neil Martin

Infantile hypercalcaemia (IHC) is a syndrome which may be associated with mental handicap. Since the formation of The Infantile Hypercalcaemia Foundation, a parent support group, research has be.en stimulated, and a recent study (Martin et al. 1984) has clarified some of the clinical features of the syndrome. Although raTe (approximately 1:50,000 total live births) the condition may lead to long-term morbidity. I t has been frequently noted that the children often have a peculiar psychological profile and associated with this may also have special learning needs. Bennett et al. (1978) attempted to define this psychological profile for a few cases, and the study reported here extends their observations by defining the psychological attributes of a small cohort of the children affiliated to The Infantile Hypercalcaemia Foundation.

Clinical features of infantile hypercalcaemia Failure to thrive caused by hypercalcaemia was first described independently by Fanconi et al. (1952) and Lightwood (1952). It was soon obvious that cases varied in severity and prognosis. Cases as described by Lightwood presented with anorexia, vomiting, loss of weight and constipation. Some, but not all, had received above- average intake of vitamin D and serum calcium levels exceeded 3mmol/l. Treat- ment with low-calcium and vitamin diets

in most cases led to prompt symptomatic relief and complete resolution of the condition. Delay in mental development if present was only temporary and these cases were therefore described as ‘mild’.

The ‘severe’ form, as first described by Fanconi et al. (1952), presented in a similar fashion but certain other features also were evident. These included the elfin facies as elaborated on by Joseph and Parrott ( 1958); renal impairment; osteoslcerosis, particularly retognisable at the ends of the long bones and base of the skull; and stenotic lesions of the major arteries, in particular the aorta, peripheral pulmonary arteries and renal arteries. The infants’ N

despite treatment with the same low- 2 calcium diets, they remained mentally 6

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9 2 handicappedand frequently had persistent

cardiovascular and/or renal impairment. Although most cases did fall into these

and ‘severe’ was not always so clear-cut and intermediate cases have been described (Joseph and Parrott 1958). Whether these two forms of hypercalcaemia in fact form part of a continuum of severity is still open to question and largely depends to what extent the hypercalcaemia per se is the

The r61e of calcium and vitamin D in the pathogenesis of IHC is not clear. In his excellent review of the subject, Fraser

s g two groups, the distinction between ‘mild’ * L, d

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(1967) was unable to reach any definite conclusion as to the r81e of vitamin D in producing hypercalcaemia-the main in- criminating evidence is epidemiological. In the 1950s, paediatricians in the United Kingdom were concerned by an apparent epidemic of IHC. A working party was set up and subsequently recommended a reduction in the vitamin D fortification of baby milks and cereals (Lightwood et al. 1956). A later report concluded that there was some evidence that these measures had caused a fall in the incidence of IHc, although they noted that there was not a close temporal relationship between the two events (Oppt 1964). Moreover, in the majority of cases it was not possible to document excessive intake of vitamin D, either by the mother during pregnancy or by the infant before diagnosis. The theory of vitamin D sensitivity was therefore proposed (Lightwood 1953) but has never been elucidated.

Williams et al. (1961) described four children with mental handicap, peculiar facies and narrowing of the aorta above the aortic valves (supravalvular aortic stenosis). The connection between IHC and Williams’ syndrome was soon recognised by Black and Bonham-Carter (1963) who drew attention to the similarities in the facial features and clinical symptoms. It has now been clearly shown that the syndromes are very closely linked, if not identical (Martin et al. 1984). Jones and Smith (1975) suggested that the syndrome should be extended and adopted the term Williams’ elfin facies syndrome. This extension to include children with facial similarities and mental handicap may be clinically useful but also can lead to imprecision in any attempt to define the cause of IHC or describe its psychological profile.

In conclusion, IHC is a rare but significant cause of morbidity in child- hood. There appear to be mild and severe forms but intermediate cases may exist. Vitamin D has been implicated in its pathogenesis but the evidence is not conclusive. The concept of vitamin D sensitivity has been postulated to explain certain discrepancies in the clinical picture but no biochemical data exist to confirm this hypothesis.

Psychological characteristics of infantile hypercalcaemia Although the psychological characteristics of IHC children have rarely been appraised systematically, caregivers have made some common observations. Von Arnim and Engel (1964) presented four case studies from which four common features were apparent. First, the children appeared to have an unusual command of language in that they used complex structures in spontaneous speech. Second, they had difficulty in establishing relationships with peers but were excessively friendly towards adults. Third, they displayed signs of unreasonable anxiety. Fourth, although severely mentally retarded, they gave the impression that they were more intelligent. The members of the IHC Foundation have made similar observations and have also commented on the children’s hyper- sensitivity to noise. They suggest that these characteristics present difficulties in inte- grating the children into family and school life.

Bennett et al. (1978) reported results on the MaCarthy Scales of Children’s Abilities (McCarthy 1972) for seven children diagnosed as having Williams’ elfin facies syndrome. Over-all, the average cognitive index was 53.9 (range 30 to 81). (The McCarthy cognitive index is stan- dardised to a mean of 100, SD of 16.) Considering the individual subscales, the seven children performed best on Verbal, Memory and Quantitative scales, and significantly poorer on Perceptual per- formance and Motor. Bennett et al. concluded that this confirmed previous clinical impressions that Williams’ children were especially poor on gross and fine motor skills.

However, there is insufficient evidence to claim the existence of a psychological syndrome. Since the features have not been defined and described, it is not clear whether they are characteristic of all IHC children or indeed whether they are peculiar to the group. The research into spina bifida (Anderson and Spain 1978) highlights such problems of classification. Spina bifida children were thought to have a characteristic speech pattern but later systematic investigation did not sub- stantiate the claim. They also appeared to experience difficulties in relating to their

TABLE 1 Clinical features of the sample

Documented hypercalcaemia

Date of Vomiting (max serum Low-calcium Cardiovascular Inguinal Sex Age birth in infancy calcium mrnol/l) diet disorders hernia

1 F 2 M 3* M 4 F 5 M 6 M 7 F

9 10* F I 1 M 12 F 13 M 14 F IS* M 16 F 17 M 18 M 19* F 20 M 21 F 22 M 23 M

8 t ;

2m 4m 4m I m 8m 8m 2m 2m 6m 6m Om 5m Om Im 6m

I l m 2m Im 2m 5m 8m Om lm

19.03.74 2 1.02.74 12.01.74 05.05.73 17.10.72 23.09.72 15.04.72 27.03.72 29.1 1.71 26.11.71 16.05.7 I 21.01.71 01.06.70 26.04.70 20.12.69 06.07.69 24.03.69 20.03.69 15.03.69 29.01.69 18.10.68 05.06.68 02.05.68

J J

J J J J J J J

J

J J J J J J J J J

Js

J J J

J J J J J

J J

J J J

J

SVAS

SVAS SVAS SVAS SVAS** SVAS

SVAS SVAS SVAS

W A S

SVAS

SVAS/PPAS** SVAS SVAS** SVAS SVAS PPAS**

*Not seen by Dr. Martin. **Confirmed by cardiac catheterisation. ?Not diagnosed IHC by Dr. Martin. SVAS: Supravalvular aortic stenosis; PPAS: Peripheral pulmonary artery stenosis.

peers while being uninhibited in approach- ing adults. Anderson and Spain have suggested that this behaviour may simply be a function of the limited opportunities for interaction with peers that are available to handicapped children.

In view of the current status of knowledge about IHC, this study addressed itself to three questions: (1) Do the children display common psychological characteristics? (2) How do they differ from children of normal intelligence? (3) How do they differ from other retarded children?

Method The sample was drawn from the membership of the IHC Foundation which holds the only register of affected children in Great Britain. All but one or two of the 23 cases met the selection criteria subsequently suggested by Martin et a/. (1984). All the children had received a diagnosis of severe IHC from their paediatrician. (19 of the children sub-

sequently participated in the paediatric study reported by Martin et a/. 1984.)

Twenty-three of the 25 children on the register between the ages of seven and 12 years were assessed. The sample comprised 10 females and 13 males with a mean age of 10 years 4 months. The age distribution and variability in symptomatology is illustrated by Table I. 83 per cent of the sample had persistently vomited in infancy; 78 per cent had cardiovascular disorders and 30 per cent had received treatment for herniae.

The sample was assessed as follows: (1) Cognitive functioning was determined using the Wechsler Intelligence Scale for Children-Revised (Wechsler 1976). (2) Age-related scores for expressive language and verbal comprehension were derived from the Reynell Development Language Scales-Revised (Reynell 1977). (3) Social and emotional adjustment was assessed on the basis of responses to the Rutter Scales (Rutter et a/. 1970) and the AAMD Adaptive Behaviour Scale (Nihira et a/. 1975).

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52

The Rutter Scales for parents (A) and teachers (B) comprise a series of behavioural descriptions indicative of deviant behaviour. They were first used to screen all the 10-year-olds living on the Isle of Wight. The scales therefore have an extensive data-base for children of both normal and retarded intelligence. Both scales yield a score which, if it exceeds a specified cut-off point, suggests psychiatric disturbance. The responses can be further analysed to determine the nature of the disturbance as either neurotic or anti- social. Rutter et al. (1975) found that 60 per cent of the children whose score exceeded the cut-off point were diagnosed as having psychiatric disorder on full assessment. Normative data also exists for a hyper- activity subscale (Schachar et al. 1981). High scores are not necessarily indicative of a hyperkinetic syndrome but suggest management difficulties for caregivers.

The AAMD Adaptive Behaviour Scale is used to structure a parental interview to establish the degree of personal indepen- dence in daily living enjoyed by the children, and the extent of their mal- adaptive behaviour. Each subscale has a number of domains that provide a comprehensive description of the children’s behaviour. Normative data are available for children of all ages who are institutionalised and have varying degrees of mental retardation.

During the parental interview the children’s hypersensitivity to noise was explored. Unfortunately, normative data for the purpose of comparison are not available but data on prevalence is a prerequisite to further investigations.

Results Cognitive functioning One child did not score on a minimum of three performance subtests, so that no IQs could be computed. A further six children’s computed IQs fell below the basal level of 40. The Full-scale IQs of the remaining 16 children ranged from 40 to 72. Over the whole sample, the median Full-scale rQ was 42. Only one child had a Full-scale IQ in Wechsler’s ‘Borderline’ range. Five had IQs in the range 5 1 to 70; 17 had IQs below 50.

Verbal IQS ranged from 45 to 75 with a median of 49, Performance IQs from 45 to

73 with a median of 47.10 children failed to score above the floor of the Performance scale and only four children failed to score above the floor on the Verbal scale. However, when Verbal IQS exceeded Performance IQs, the differences were found not to be reliable (Sattler 1974). Over-all, there is no support for Verbal functioning being superior to Performance functioning.

Patterns of intra-individual differences were explored through a comparison between each pair of subtest means. Significant values are given in Table 11. The sample performed relatively well on Picture Completion, Comprehension and Object Assembly in contrast with their performance on Coding, Arithmetic and Information. These differences can be partially explained by the children’s poor fine motor control and their distractibility. Since there were considerable inter- individual differences these comparisons should be viewed with caution.

The full-scale scores were examined further to determine the homogeneity of scores in the group. Chi-square goodness- of-fit tests were used to test the hypothesis that the distribution was normal. The hypothesis was rejected (p>O.O5) for the 16 cases for whom an accurate score was established. However, when estimated scores for the remaining seven children were included the distribution did not significantly differ from normal. There- fore, the question of homogeneity remains open.

Language skills The distribution of age-related scores for verbal comprehension and expressive language on the Reynell Developmental Language Scale is illustrated in Figures 1 and 2 respectively. The language skills of three children exceeded the seven-year ceiling of the test while those of the remainder ranged from three to seven years. The mean score on Expressive language was 5 years 9 months and on Verbal comprehension was 5 years 5 months. Since much of the vocabulary and structure assessed on the Reynell scale have been acquired by the middle of the fourth year the range of scores does not represent a large difference in skills. All children used sentences of three words or

1ABLE I1 Significant differences between pairs of WISC-R Subtest scores of the sample

1 2 3 4 5 6 7 8 9 10

I . Information 2. Similarities

3 . Arithmetic 4. Vocabulary

5. Comprehension

6. Picture completion

7. Picture arrangement 8. Block design

9. Object assembly

10. Coding

2 .08 (p<O ' 05)

2 .97 (p<O.OI)

2 .47 (p<0.05)

3 .46 2.13 (p<O.Ol) (p<0.05)

2.54 (p<O. 05)

2.41 (p<O.OS)

2.13 (p<O 05)

2.59 (p<O, 05)

4 .58

4.35 (p<O. 01 )

(p<O.OI)

2 . I8 ( p<O .05)

2.87 (p<O. 01)

Rows indicate significantly higher means. t-values are in the body of the table (2-tail test).

more but few mature sentence forms were recorded. Indeed, only one child uttered a complex sentence containing two or more main verbs. All children were able to describe action depicted but their sentences varied in content and length. Although the range of scores was similar for both scales, there was a significant tendency (p<O* 05) for children to attain higher expressive language scores.

In addition to formal testing, other information about the children's speech was gained from observations during assessment and from comments of parents and teachers. The children were not excessively talkative during their assess- ment but in 18 cases care-givers remarked on their loquacity. They reported that the children talked excessively to gain adult attention, often talking nonsense or appearing to mimic others.

Social and emotional adjustment The scores of 19 children exceeded the cut- off point on one or both of the Rutter scales; 12 showed disturbed behaviour at home or school while seven were disturbed in both places (see Table 111). The proportion of children displaying disturbed behaviour was compared with the proportions found by Rutter et al. (1970) in a group of 10-year-olds with normal intelligence and a group of children with IQs below 70. On both scales the IHC

7 8 9 10 1 1 12 13 Chronological age

Fig. 1. Average expressive language age on Reynell Developmental Language Scales for different age groups of IHC sample.

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7 8 9 10 1 1 12 13

Chronological age

Fig. 2. Average verbal comprehension age on Reynell Developmental Language Scales for different age groups of the IHC sample.

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TABLE 111 Behavioural disturbance on parent and teacher questionnaires for normal, retarded and IHC children

Normal intelligence Retarded IHC No. % No. % No. %

Parents' questionnaire A(2) Disturbance (13 t) 1 1 7 . 7 17 30.4 12 52. 2 Neurotic 3 2 .1 8 14.4 7 30.4 Anti-social 4 2 . 8 7 12.5 4 17.4 Undesignated 4 2 1 N 143 56 23

Teachers' questionnaire B(2) Disturbance (9 +) 14 9 . 5 23 41.8 14 60.9 Neurotic 7 4 . 8 10 18.2 8 34.8 Anti-social 7 4 . 4 1 1 20 .0 3 13.0 Undesignated 2 3 N 147 55 23

TABLE IV Pervasive and situational hvoeractivitv in normal, retarded and IHC children

Normal intelligence Retarded IHC No. % No. % No. %

Non-hyperactive 1265 84 .2 20 58 .8 3 13.0 Situationally hyperactive 213 14.2 7 20.6 12 5 2 . 2 Pervasively hyperactive 24 1 . 6 7 20.6 8 34.8 N I502 34 23

54

children differ significantly from those of normal intelligence (p<O.Ol) but they do not differ from the intellectually retarded. No association was found between type of deviant behaviour and level of intellectual functioning. Therefore, the IHC sample did not differ significantly from other retarded children either in the proportion showing disturbed behaviour or in the type of disturbance.

Scores on the hyperactivity subscale were also computed. The incidence of situational and pervasive hyperactivity was determined for the sample and compared with normal and retarded groups (Schachar et al. 1981) (see Table IV). 20 (87 per cent) of the IHC sample were overactive at home or school or in both settings. The proportion of the IHC group

displaying hyperactivity was significantly (p<O.Ol) different from both the normal children (15.8 per cent) and the intellec- tually retarded children (41 * 2 per cent). The differences were particularly notice- able with respect to pervasive hyperactivity.

In view of the observations made by von Arnim and Engel (1964) the Rutter scales were also scrutinised for evidence of undue anxiety and poor peer relationships. The percentage of normal, retarded and IHC children displaying different types of neurotic behaviour at both home and school is given in Table V. At home the I H C children appear to display similar levels of neurotic behaviour as other retarded children; they only differ significantly (p<0.05) in the number experiencing sleeping difficulties. At school, they do not

appear to differ from their peers although significantly more tearfulness was reported.

The incidence of indicators of poor peer relationships is given in Table VI. The IHC sample differ significantly from other retarded children in the number who are reported to be solitary both at home (p<O.Ol) and at school (p<0.05). Significantly more parents of IHC children report that their children are not much liked by other children (p<O.O5).

Scores for the AAMD Adaptive Be- haviour Scale were computed and mean percentile ranks were derived for each domain (Figs. 3 and 4). Scores for personal independence in daily living compare well with those of the normative sample of retarded children living in institutions. However, they do fall below the median in two domains, Independent Functioning and Self Direction. The majority of the IHC children need supervision in eating, toileting and dressing and they do not display initiative in choosing activities and persisting with them. Despite a superior level of independent functioning compared with retarded children living in institu- tions, the IHC sample enjoy little independence compared with siblings of normal intelligence and they therefore present their parents with management problems. The children do not use money, cannot tell the time and are not able to go out alone. They d o not participate well in group activities and display a degree of social immaturity in their uninhibited approach to adults.

Although the extent of maladaptive behaviour does not differ greatly from the normative sample, close scrutinity indicates areas of difficulty. Hyperactivity is confirmed in 22 cases and there is a high incidence of psychological disturbance (22 cases). It seems that the children feel secure in a familiar environment but are easily upset by criticism and frustration, and consequently demand excessive attention and praise.

Hypersensitivity to noise Twenty children were reported to be hypersensitive to electrical noises such as lawn mowers, vacuum cleaners, pneumatic drills and sudden noises such as bursting balloons or exploding fireworks. On

TABLE V Incidence of neurotic behavioun displayed by the IHC and Rutter samples

Statements Gen. pop. Retarded IHC (%) (%) (%)

Parents' scale A(2) Has stomach ache or 31.75 46.4 47.82 vomiting Has tears on arrival 0.95 5 . 7 4.34 at school or refused to go into building Is there any sleeping 17.95 11.45 *30.43 difficulty? Often worried, 37.3 48.15 39.13 worried about many things Tends to be fearful or 25 . O 45.25 65.28 afraid of new things or new situations N 3064 54 23

Teachers' scale B(2)

worries about many things

miserable, unhappy, tearful or distressed Tends to be fearful 20.05 48.95 56.52 or afraid of new things or new situations

arrival at school or has refused to come into the building in the past 12 months N 3426 5 5 23

Often worried, 23.0 48.95 60.87

Often appears 8.15 23.6 26.01

Has had tears on 0.85 0.00 '13.04

*Significant differences.

' O 0 1

I I I Ill I V v VI V I I V l l l I X x

I Independent functioning I I Physical development Ill Economic activity IV Language development V Numbers and time VI Domestic activity V I I Vocational activity V l l l Self direction I X Responsibility X Socialisation

Fig. 3. Mean percentile ranks for the IHC sample for personal independence in daily living. 55

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TABLE VI Indicators of poor peer relationships displayed by the IHC and Rutter samples

Statements Gen. pop. Relarded IHC (%) (%) (9%)

Parents' scale A(2) Frequently fights or 10.25 35.25 30.43 is extremely quarrel- some with other children Not much liked by 4.8 13.15 34.78 other children

own-rather solitary Tends to be on 23.1 35.8 82.61

~~ ~~

Teachers' scale B(2) Frequently fights or 9.05 19.75 21.74 is extremely quarrel- some with other children Not much liked by 13.15 25.1 26.09 other children Tends to be on 15.85 27.65 60.87 own-rather solitary

hearing such noises, younger children tend to cry or scream while older children either block their ears or avoid the noise. Parents recalled that the children had always displayed this sensitivity. The children were also reported to enjoy some sounds. Listening to music is a favourite pastime for many.

Discussion The purpose of this study was to describe some psychological characteristics of children with infantile hypercalcaemia. It was viewed as a preliminary to medical investigations into aetiology and symp- tomatology as well as a prerequisite to advising parents and other caregivers on prognosis and behavioural management. It extends data collected by Bennett et al. (1978).

Although the test results indicate inter- individual differences, it is possible to make broad generalisations about the psychological characteristics of IHC child- ren. These are likely to be of use to those who counsel parents and care-givers about the future level of functioning of their children; IQ scores fall within the ranges of mild and moderate retardation. Since there were large individual differences in subtest

scores, it is not appropriate to generalise about specific cognitive skills but, unlike Bennett et ~ l . (1978), no significant difference was found between Verbal and Performance IQ scores. The sample's performance was characterised by poor fine motor co-ordination and poor concentration. The different findings of the two studies may be due to Bennett and colleagues' smaller sample or differences in the tests employed. As noted earlier, 10 of the 23 children failed to score at the floor of the WISC-R Performance scale and this may have obscured difficulties in this area.

The majority of the children's language skills were also found to be retarded, although all children were able to produce and respond to simple sentences. This finding is not in accord with the observations made by von Arnim and Engel (1964) and the Parents Association. The difference may be attributable to two factors. First, the children's relatively superior expressive language skills may lead care-givers to believe that over-all levels of language functioning are superior. Second, the data presented here are generated from standardised testing whereas earlier reports were based on unsystematic naturalistic observations. It may be that either the children perform better in more familiar settings or they are able to use their talent of mimicry in less- structured settings. Clearly, there is a need for further investigation of language skills.

The level of social and emotional functioning found in this study is not in full accord with previous observations. On the basis of the checklists used in the study, the IHC children are broadly similar to the normative retarded samples. There is little evidence for the claim that the children are unduly anxious when they are compared with those of a similar intellectual level. In addition, the IHc sample does not differ from other mentally retarded children in the incidence of neurotic and anti-social behaviour which could be indicative of psychiatric disorder. However, differences were found, some of which have been observed previously. The incidence of hyperactivity in the sample was signifi- cantly higher than in the normative sample. In addition, significantly more children with IHC were reported to be solitary both at home and school. Another

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6 3 a 5 2 3

I

I I I I l l IV V V I VI I V l l l IX X XI XI1 X l l l XIV

I I I l l IV v VI V I I V l l l IX x X I XI1 X l l l XIV

Violent and destructive behaviour Anti-social behaviour Rebellious behaviour Untrustworthy Withdrawal Stereotyped behaviour and odd mannerisms Inappropriate interpersonal manners Unacceptable vocal habits Unacceptable or eccentric habits Self-abusive behaviour Hyperactive tendencies Sexual I y aberrant behaviour Psychological disturbances Use of medications

d

Fig. 4. Mean percentile ranks for the IHC sample for maladaptive behaviour.

problem reported by parents was the behaviour arising from the hypersensitivity to noise.

The study cannot make an immediate contribution to research into the aetiology of infantile hypercalcaemia; nevertheless, it does indicate that affected children have global, long-standing cognitive impair- ment. The failure to identify specific cognitive deficits is not surprising because moderate mental retardation is character- ised by widespread neurophysiological maldevelopment and patterns of behaviour are not closely associated with neurological lesions. However, it is possible that further studies of psychological functioning could establish more specific disorders in infantile hypercalcaemia. There is a need for systematic comparative studies of cognitive functioning in which the level of investigation should be specific cognitive skills rather than the global, ill-defined skills assessed in intelligence tests.

The pattern of the children's behavioural disturbances suggest that environmental and social factors are significant. The contribution of such factors could be established from the outcome of inter- ventions designed to modify specific behaviours. The obvious target for a

behavioural programme is hyperactivity. It is possible that it prevents children from reaching their potential and it may also contribute to poor social functioning. The preferred technique is the use of positive reinforcement for staying seated (see for example Hemsley and Carr 1980) followed by reinforcement for visual fixation (see for example Maier and Hogg 1974). Reinforcers for IHC children could include adult attention and conversation although there is no apparent reason why sweets or tokens should not be successful.

In addition to individual interventions, the total environment of IHC children needs to be considered. Their pattern of psychological functioning does not im- mediately suggest any particular type of special school. The intellectual level of many falls on the borderline between mild and moderate mental handicap whereas their expressive language skills might suggest a higher level of intellectual and social functioning. Their social functioning is also deceptive in that they appear to relate well to adults but not to their peers.

In conclusion, infantile hypercalcaemia is associated with a number of behaviour patterns but there are considerable individual differences. All children do not 57

58

display all characteristics, and behaviour patterns are unlikely to be exclusive to IHC children. Nevertheless, there would seem to a discernible syndrome which requires further exploration and understanding

enthusiasm laid the groundwork for the investigation. Our sincere thanks are due to the parents, children and teachers who participated in the study, and to the late Ms. Bernie Spain for her advice. We are grateful to Dr. 1. B. Pless for very helpful comments on an earlier dratt of this paper.

before intervention programmes can be developed and causal factors identified.

Acknowledgements This study was undertaken by the first author under the supervision of the second as part of the requirements for the degree of M.Phil. We are grateful to the Infantile Hypercalcaemia Foundation who funded this study, and especially to Lady Cynthia Cooper whose foresight, energy and

Authors' Appointments Rosemary Arnold, Clinical Psychologist, Glenside Hospital, Blackberry Hill, Stapleton, Bristol. *William Yule, Reader in Applied Child Psychology, Institute of Child Psychiatry, De Crespigny Park, London SE5 8AF. Neil Martin, Research Paediatrician, Department of Child Health, The London Hospital, Whitechapel, London.

*Correspondence to second author

SUMMARY '

A sample of 23 children aged seven to 12 years with diagnoses of infantile hypercalcaemia was assessed on a battery of psychological tests and rating scales in an attempt to describe the psychological characteristics of this disorder. All children were found to have a mild or moderate degree of mental handicap. As a group, their expressive language skills were superior to their verbal comprehension. They displayed a high degree of behaviour and emotional disorders, most notably in terms of high levels of activity. Most children were reported as being highly sensitive to noise. This study clarifies some of the psychological correlates of infantile hypercalcaemia and some implications for management are suggested.

R ~ S U M E Caractiristiques psychologiques de I'hypercalcPmie infantile: une investigation preliminaire Une batterie de tests psychologiques et d'tchelles de niveau a t t t proposte a un Cchantillon de 23 enfants, ig t s de sept a 12 ans, chez qui avait tt6 port& un diagnostic d'hypercalctmie infantile, dans le but de dkcrire les caracttristiques psychologiques de ce trouble. Un degrt ltger ou modtr t de handicap mental a t t t observt chez tous les enfants. En tant que groupe, la capacitt expressive du langage ttait suptrieure comprehension verbale. Les enfants prtsentaient un degrt Clevt de troubles comportementaux et tmotifs, tout sptcialement en terme de haut niveau d'activitk Une haute sensibilitt au bruit a t t t relevte chez la plupart des enfants. Cette ttude prtcise quelques une des caractkres psychologiques de I'hypercalctmie infantile et les auteurs suggkrent quelques implications thtrapeutiques.

la

ZUSAMMENFASSUNG Die psychologischen Charakteristika der Hypercalcaemie im Sauglingsalter: eine vorlaufige Untersuchung 23 Kinder im Alter zwischen sieben und 12 Jahren mit der Diagnose Hypercalcaemie im Sauglingsalter wurden anhand psychologischer Tests und Bewertungsskalen untersucht, um die psychologischen Charakteristika dieser Erkrankung herauszufinden. Alle Kinder hatten eine leichte bis mittelgradige geistige Behinderung. Als Gruppe war ihre expressive Sprache besser als ihr Wortverstandnis. Sie zeigten im hohem M a e Verhaltens- und Gemiitsstorungen, was sich besonders in Hyperaktivitat aul3erte. Von den meisten Kindern wurde berichtet, dap sie auperst gerauschempfindlich seien. Diese Studie stellt einige psychologische Korrelate der Hypercalcaemie im Sauglingsalter dar und macht einige Vorschlage zu ihrer behandlung.

RESUMEN Caractreristicas psicoldgicas de la hipercalcemia infantil: investigacidn preliminar Una muestra de 23 nifios de siete a 12 afios con el diagn6stico de hipercalcernia infantil fueron examinados con una bateria de tests psicol6gicos y escalas de porcentajes con el objeto de describir las caracteristicas psicologicos de esta alteracibn. Se ha116 que todos 10s nifios tenian una dificultad mental leve o moderada. Como grupos sus habilidades de lenquaje expresivo eran superiores a su comprensibn verbal. Mostraban un alto grado de alteraciones emocionales y de conducta, sobre todo en ttrminos de unos mayores niveles de actividad. Se vi6 que la mayoria de nifios eran altamente sensibles a1 ruido. El estudio clarifica alguna de las correlaciones psicol6gicas de la hipercalcemia infantil. Se sugieren algunas implicaciones para su manejo.

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Bifida. London: Methuen. severe infantile hypercalcaemia.' Lancet. 11, 745-749.

Williams' elfin facies syndrome: The psychologica! Fanconi, G., Giardet, P., Schlesinger, B., Butler, H,., protile as an aid in syndrome identification, Black, J. S. (1952) 'Chronische Hypercalcamie Pediatrics. 61, 303-306. kombiniert mit Oteosklerose, Hyperazotamie,

Bennett, F. C., Laveck, B., Sells, C. J. (1978) 'The

Minderwuschs and kongenital Missbildungen.’ Helvetica Paediatrica Acta, I, 3 14-334.

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