Dear Editor,

We thank Dr Hadzic for his comment on our paper(1). However, we disagree with Dr Hadzic’s difficulties with our conclusions for the following reasons:

1. Our research suggests that over 2.5% of children five years of age and older may have five or more spider naevi. At present, if we were to screen our population of approximately one million children and adolescents in the Republic of Ireland and investigate all of those with more than five spider naevi for the presence of liver disease, then we would screen 25,000 children. Clearly, this is not tenable. The only conclusion that can reasonably be drawn from our study is that, as stated, the “presence of multiple spider naevi in an otherwise well child may not warrant further laboratory investigation”. Clearly, the clinical context will be essential in determining whether the child does or does not require further investigation.

2. At no stage, did we advocate “dismissing” the clinical sign of spider naevi in children. However, our paper clearly points to the fact that the presence of numbers of lesions in children is neither sensitive nor specific for the presence of underlying chronic liver disease and that this should be taken into account when interpreting their presence or absence in an individual child.

We, like Dr Hadzic and other hepatologists, will continue to examine children for stigmata of chronic liver disease, including spider naevi. The presence or absence of these lesions will be interpreted within the clinical context for an individual child. Professor Alex Mowat suggested that it was difficult to interpret the relevance of anything up to ten spider naevi in children(2). He also stated that the development of many and enlargement of older ones was suggestive of liver disease. Perhaps this latter observation, not specifically studied in our cross sectional cohort, is worthy of validation within a large group of children with evolving chronic liver disease.

References:

(1) Finn SM, Rowland M, Lawlor, F et al. The significance of spider naevi in children. Arch Dis Child 2006;91:604-5).

(2) Mowat AP (ed). Liver disorders in childhood. 2nd Ed Butterworth & Co, London. 1987; 260.

Dear Editor,

There is no doubt that language is contextual. Disorder as a term, while innocuously descriptive to one, may hold a negative connotation for another, especially those afflicted with whatever said disorder.

Regarding brain sex, I agree with the Consensus Statement on Management of Intersex Disorders (CoSMID) that “Structure of the brain is not currently useful for gender assignment.” Quantification and identification of brain sex haven't been at all forthcoming and fruitful on humans. A prodigious amount of neurons and synaptic connections that have to differentiate and develop would appear to, according to my statistical conjecture, render brain sex and other brain attributes variable (and study incredibly difficult) due to the immense complexity of the process. Gender that, then, combines brain sex and social and environmental factors would also at least, in line with brain sex that influences it, be variable. Sex, if one limits it to the genitalia and perhaps secondary sex characteristics, is dimorphic, as most persons get one of two possibilities when differentiation is complete - that is, if one ignores considerations of size and appearance, which some don't, but I do because functionality, to me, is paramount.

The authors of CoSMID appear to be focused on sex. Brain sex has already been ruled out by their statement on brain structure (see previous quote). Gender, in turn, is too variable to be predictable, and if one reads CoSMID, it is obvious it is not their focus. Most of their focus is at the level of the genitalia. As such, I believe sex was the main consideration for the designation "Disorders of Sexual Development" (DSD).

I take issue with the use of "development." I think it should be "differentiation" - that is, Disorders of Sexual Differentiation - as most DSD problems originate at that level. Development would be from puberty onwards. "Sexual development," in this case, may imply brain development because differentiation and development for the brain can be used interchangeably, but, again, I believe the focus was on sex.

I can only imagine, perhaps not even that, how it must feel to have a condition and it be labeled a "disorder." In a medical setting, however, "disorder" need not be stigmatizing or expedite surgical intervention or hormone replacement. I understand that medical professionals have set the stage with past controversial interventions that will cause them to be viewed with suspicion, but on the other hand we must call a spade a spade. From what is understood about sexual differentiation (and development), DSD cannot be anything other than what the term claims - disorders. Substituting "variation" for "disorder" colloquially is of no consequence, besides the assuagement of the afflicted, which admittedly is important, but scientifically, one would have to wonder about adaptation. If the focus were brain sex or gender, this might be an interesting academic and protracted discussion, but since it is sex (for reasons stated earlier), there are really only one of two possibilities, as most other possibilities tend to be dysfunctional or nonfunctional, leading to infertility. An adaptive benefit, even neutrality, seems doubtful. None of these considerations, however, must necessarily lead to medical intervention or management.

Again, I realize the need to be sensitive to the perceptions of the afflicted ("afflicted" probably will also be seen as a wrong choice of word), but I hope with this to somewhat lessen the supposed stigma "disorder" bears.

Sincerely,

M.A.

Dear Editor,

The new intersex terminology scheme, based on Disorders of Sex Development (DSD) and discussed in the consensus statement by Hughes et al [1], seems to offer the following benefits:

a) it covers a wide range of intersex conditions without using the archaic and stigmatising hermaphrodite and male/female pseudo- hermaphrodite terms, b) it should end the problem of the term intersex being used by some clinicians to mean ambiguous genitalia (and being extrapolated by the media to mean gender identity problems in all cases) when it in fact includes conditions like Complete Androgen Insensitivity Syndrome (CAIS) and Swyer syndrome with a totally female phenotype, c) it should abolish the assumption, on the part of the uninformed, that all affected people are "between the two sexes", d) it should reduce the problem of people using nouns to label patients ("She's a male pseudo- hermaphrodite" or "She's a genetic male", as if her whole being/identity is governed by this), e) it allows inclusion of non-intersex XX-female conditions like Mayer Rokitansky Kuster Hauser syndrome (MRKH) which share some common features (such as absence of uterus/vagina) with XY-female intersex conditions, f) it provides a terminology with which clinicians and funding bodies will feel comfortable and which has fewer political/activism associations in the eyes of parents, and g) it possibly differentiates intersex from transsexuality more clearly (but no doubt the trans community will try to annexe the DSD term).

The downside could be:

a) the choice of the term disorder is bad news. One might say, in its defence, that DSD is merely an umbrella term, and that the name of an individual diagnosis would be used wherever possible, but DSD's position at the top of the naming hierarchy makes it hard to ignore, b) some adults have come to like the more socio-political, whole-person terms and may ask why those who have been advocating a non-medicalising "proud to be intersex" philosophy over the last 10-15 years now seem to be advocating terms based on pathology, c) its reduction to a 3-letter acronym is possibly unfortunate as it allows use of DSD as a cover-up term (to avoid having to say the word 'disorder'? or 'sex'?) and d) there was lack of wide consultation on the new scheme amongst international peer support groups.

On 8 August 2006 one of our UK members had her story published in the Independent newspaper, an account in which she uses the term intersex. When asked if she knew that the term was likely to change to DSD (in clinical circles at least), she replied, "Disorders of... is horrible. Just adds to the sense that we are wrong. I like intersex."

So the overall structure of the scheme seems a significant advance on what has hitherto been available, although certain terms used within it seem retrograde in nature. Like Milton Diamond and Hazel Beh [2], my first reaction was why not use Variations of Sex Development, but I then learnt that VSD is already in use for Ventriculo-Septal Defect. Was Variations of Reproductive Development (VRD) considered, especially since many people might take issue with the notion that their sex is disordered, whereas they might accept that they have a variation in some aspect/s of their reproductive tract?

Another important issue discussed in Chicago is improved patient care. We have worked since the mid-1990s with selected UK clinicians to promote a patient-centred, multi-disciplinary approach to intersex patient care of the sort recommended in the Consensus statement. The first meeting to which we were invited, to discuss this with clinicians within the University College London Hospital NHS Trust, was on 6 Aug 1997; and the multi-disciplinary clinic there has been successfully running for a number of years now, with a high proportion of our members attending. So the recommended paradigm is already up and running in the UK.

Margaret Simmonds

Androgen Insensitivity Syndrome Support Group (AISSG) UK
www.medhelp.org/www/ais

References:

1] I A Hughes, C Houk, S F Ahmed, P A Lee LWPES/ESPE Consensus Group Consensus statement on management of intersex disorders Arch Dis Child 2006; 91: 554-563

2] Milton Diamond, Hazel G. Beh Variations of Sex Development Instead of Disorders of Sex Development Arch Dis Child 2006, Electronic Letter, 27 July 2006

This paper demonstrates deep understanding of economics but little feeling for the reality of early infancy.[1] Any paediatrician will know this is the most vulnerable time of life. Any parent will tell you how hard it can be looking after an infant in the first three months. The assumption that health visitors home visits and mothers visits to the GP “that the entire contact would be devoted to infant crying and sleeping” underestimates the work that is or needs to be done in these consultations.

Apart from the crying or lack of sleep the health visitor or GP will have to consider the presence of any physical condition, the physical, psychological and social welfare of the mother and the father. At least one in ten women experience depression in the first weeks or months after giving birth.[2] There is an inverse relationship between the age of infants and deaths from child abuse.[3] The injuries found on the head and neck of young infants who suffer physical abuse are commonly associated with a history of difficulty with sleeping or crying. A randomised trial in the USA showed statistically significant reduction in child abuse and neglect when the mothers were visited by nurses during pregnancy and infancy.[4]

Morris et al inform us that the £65 million spent by the (UK) NHS on babies that cry or do not sleep would buy over 13 million doses of Viagra (sildenafil). It is of regret that the authors did not discuss how persistent crying or lack of sleep could lead to child abuse. Perhaps in future work they could calculate the cost of one shaken baby to the NHS and society.

Dr D V Lang
Associate Specialist Child Health/Audiology
Royal Cornwall Hospitals Trust
Pendragon House, Treliske
Cornwall PL15 3LS, UK

References
(1) Morris S, et al. Economic evaluation of strategies for managing crying and sleeping problems. Arch Dis Child 2001;84:15-19.

(2) The management of postnatal depression [Review]. Drug Ther Bull 2000 May;38(5):33-7.

(3) Hobbs CJ, Hanks HGI, Wynne JM. Child abuse and neglect. A clinicians handbook (2nd ed). London: Churchill Livingstone, 1999.

(4) Olds DL, et al. Long term effects of home visitation on maternal life course and child abuse and neglect. Fifteen-year follow up of a randomised trial. JAMA 1997;278:637-43.