Dear Editor

The recent article by Ng et al. and the accompanying commentary [1] address the notion that girls with precocious puberty should undergo cranial imaging to exclude a CNS malformation.

One consideration not discussed in that article or the commentary is that hypothalamic hamartomas can be part of a malformation syndrome, most commonly Pallister -Hall syndrome.[2] Because this disorder can be subtle (minimal post- axial polydactyly with metacarpal fusion, bifid epiglottis, and anal stenosis) it can be missed. The disorder is inherited in an autosomal dominant pattern, so the recurrence risks may be substantial. Conversely, if a child with any of these anomalies develops precocious puberty, there is no doubt that cranial imaging is indicated.[3]

The issue of surgery for hypothalamic hamartomas is challenging. My experience with Pallister-Hall syndrome combined with published data on children with non-syndromic hypothalamic hamartomas [4] show that these lesions are malformations, not tumors. No patients in these series have experienced visual axis compromise in spite of having enormous hamartomas, some as large as 4 cm and associated with developmental distortion of the optic tracts and chiasm. In several instances, I have urged families to refuse planned surgical interventions and these children have done well. Although the commentary focuses on the cost of the MRIs, the greater cost may be that of unnecessary neurosurgery and its subsequent morbidity, sometimes including lifelong hormonal replacement. Avoiding one unnecessary neurosurgical procedure may save more than the cost of 100 MRIs.

Note: The opinion expressed here is that of the author and does not necessarily represent the opinion of the institutions with which he is affiliated.

References

(1) Ng SM, Kumar Y, Cody D, et al. Cranial MRI scans are indicated in all girls with central precocious puberty. Arch Dis Child 2003 88(5): 414-417.

(2) Biesecker LG, Graham JM Jr. Pallister-Hall syndrome. J Med Genet 1996 Jul;33(7):585-9.

(3) Biesecker LG, Abbott M, Allen J, et al. Report from the workshop on Pallister-Hall syndrome and related phenotypes. Am J Med Genet 1996 Oct 2;65(1):76-81.

(4) Feuillan PP, Jones JV, Barnes KM, et al. Boys with precocious puberty due to hypothalamic hamartoma: reproductive axis after discontinuation of gonadotropin-releasing hormone analog therapy. J Clin Endocrinol Metab 2000 Nov;85(11):4036-8.

Dear Editor

I am writing in response to the article on fever in returned travellers [1] that highlights the disappointing uptake of malaria chemoprophylaxis in travellers.

I wish to point out the practical problems associated with malaria chemoprophylaxis in children. The medications used for chemoprophylaxis varies depending on the area travelled to. A combination of weekly chloroquine and daily proguanil is the standard recommendation for travellers to South Asia.[2] Mefloquine is recommended when travelling to areas of chloroquine resistant falciparum malaria but is associated with a lot of side effects. Maloprim (a combination of pyrimethamine and dapsone) is the only other chemoprophylaxis for falciparum malaria chemoprophylaxis in children under 12 years of age. Chloroquine is the only medicine available in syrup form, but the syrup is extremely bitter. All the other medications are available in tablet forms and have to be crushed and given to children under 2 years of age. The recommended duration of chemoprophylaxis is 1 week prior to travel to 4 weeks after return to UK. [3] Many families residing in the UK and visiting their countries of origin stay abroad for a minimum of three to four weeks. This would mean that the desirable duration of chemoprophylaxis would vary between seven to eight weeks. As we can imagine, it can be incredibly difficult to get a young child under 2 years of age to comply with this child unfriendly chemoprophylaxis for this length of time. It is highly likely that many parents would try for a while and give up. This would account for the poor uptake of malaria chemoprophylaxis.

We can only improve the compliance to malaria chemoprophylaxis by production of more child friendly medications or an effective malaria vaccine. This will reduce the cases of imported malaria in children in the United Kingdom.

References

(1) NS West, FAI Riordan. Fever in returned travellers: a prospective review of hospital admissions for a 2 ½ year period. Arch Dis Child 2003; 88:432-434.

(2) British National Formulary. Prophylaxis against malaria.

(3) PHLS, Malaria reference laboratory. Guidelines for prevention of malaria-2001.

Dear Editor

Zhu points out the favorable effects of the One Child Family Policy (OCFP) on China’s demographic problems and on the population.[1] In our opinion, the considerable, unfavorable consequences of the OCFP are not sufficiently taken into consideration in the article. One of the major problems is the gender imbalance of Chinese population. The gender ratio mentioned by Zhu refers to the entire population, but the data regarding the gender ratio at birth (GRB) show a much more alarming situation. According to the official China’s 2000 Census data, that Zhu does not mention, 117 male children are born every 100 female babies in China, and in some provinces –such as in Hainan- the GRB (male:female) rises up to 135:100.[2] GRBs over 113:100 have been reported in China’s official figures since the late 1980s [3] and the phenomenon seems therefore to be increasing. It’s widely recognized that this abnormal GRB is the direct consequence of the OCFP: in a culture in which a strong traditional preference for boys exists, the OCFP determined the spread of prenatal sex selection practices against female fetuses, through the use of ultrasounds and selective abortion. The underreporting of female births, that Zhu presents as a cause of the abnormal gender ratio, has not been documented. If it existed and was so relevant as to skew the GRB by 10%, we should hypothesize that also the data about the decline of fertility rate in China are substantially biased. Although the OCFP does not seem to be the cause of negative effects on health and psychological conditions for the many only children born in China following the introduction of the policy,[4] yet it is likely to cause them social problems in the next years. Indeed, if China’s family planning policy is not modified so as to reduce GRB, in the next future about one sixth of Chinese young male adults -and up to one third in some rural areas- won’t have the possibility to create a family and to have children, due to the lack of women to marry.

References

(1) Zhu W X, The One Child Family Policy. Arch Dis Child 2003;88:463-464. (2) Plafker T. Sex selection in China sees 117 boys born for every 100 girls. BMJ 2002;324:1233.

(3) Gu B, Roy K. Sex Ratio at Birth in China, with Reference to Other Areas in East Asia: What We Know. Asia-Pacific Population Journal 1995;10:17-42.

(4) Hesketh T, Qu J D, Tomkins A. Health effects of family size: cross sectional survey in Chinese adolescents. Arch Dis Child 2003;88:467-471.

Dear Editor

We concur with Drs Colver and Sethumadhavan that the term diplegia has limited clinical value as a way to communicate about children with cerebral palsy and that use of the term should generally be discouraged.[1]

The definition of diplegia is imprecise and requires judgment; the dividing lines between diplegia and quadriplegia, or between diplegia and hemiplegia with ‘some’ involvement of the non-hemiplegic side, are very unclear; and there is no evidence of inter-observer reliability to demonstrate that people can make these clinical distinctions accurately. Further, we agree with their recommendation about using the classification system developed by the Surveillance of Cerebral Palsy in Europe (SCPE) to arrive at a description of the ‘impairment’,[2] although we remain unaware of any evaluation of the inter-rater reliability of that system.

However, we are concerned that the SCPE system does not currently encompass a reliable measure of functional ability. We propose that in order to describe children with cerebral palsy appropriately for the purposes of epidemiological or clinical studies the SCPE classification needs to be supplemented with a more dependable measure of the child’s functional ability (in effect, of the ‘severity’ of disability). This would describe, for example, whether the child can sit, stand or walk unsupported or with assistive devices. The Gross Motor Function Classification System (GMFCS) was developed explicitly for this purpose and its validity and reliability have been demonstrated.[3] The GMFCS provides a means to describe the functional ability of children with cerebral palsy at different ages in one of five explicitly defined levels. Children in Level I can perform most or all the activities of their age- matched peers, albeit with some difficulty with speed, balance, and coordination; children in Level V have difficulty controlling their head and trunk posture in most positions and achieve little or no voluntary control of movement. The recently published Motor Development Curves report significantly different patterns of gross motor development in children with cerebral palsy from a longitudinal study, classified according to each of the five levels of the GMFCS.[4] Since the initial publication in 1997 the GMFCS has had a major impact (over 50 citations) and has been utilised across the spectrum of epidemiology and clinical research.[5]

In summary, we agree that ‘the term diplegia should be abandoned’ but argue that most epidemiological or clinical studies require information on the functional ability of children. The Gross Motor Function Classification System is the condition-specific scale of ‘gross motor functional ability’ for children with cerebral palsy and should be used to supplement the SCPE classification of ‘impairment’. Furthermore we are optimistic that reaching an international consensus on clear and reliable operational definitions of clinical pictures might provide a basis for assessing differences in aetiology and neuropathology on the theme of developmental motor impairment.

References

(1) Colver AF, Sethumadhavan T. The term diplegia should be abandoned. Arch Dis Child 2003;88:286-90.

(2) Anon. Surveillance of cerebral palsy in Europe: a collaboration of cerebral palsy surveys and registers. Dev Med Child Neurol 2000;42:816-24.

(3) Palisano R, Rosenbaum P, Walter S, Russell D, Wood E, Galuppi B. Development and reliability of a system to classify gross motor function in children with cerebral palsy. Dev Med Child Neurol 1997;39:214-23.

(4) Rosenbaum PL, Walter SD, Hanna SE, Palisano RJ, Russell DJ, Raina P et al. Prognosis for Gross Motor Function in Cerebral Palsy Creation of Motor Development Curves. JAMA 2002;288:1357-63.

(5) Morris C, Bartlett D. Gross Motor Function Classification System: impact and utility. (submitted for publication).