Henoch Schonlein purpura is a relatively common and well recognized paediatric condition. We report a case of 2 monozygotic twins that presented with typical Henoch Schonlein symptoms, starting simultaneously. Both children had positive throat cultures for Streptococcus pyogenes and skin biopsies typical for HS disease. Their genotype was determined and compared with studies suggesting predisposition according to HLA typing.
of Smilari et al entitled &dquo;Holoprosencephaly (Lobar Form) Associated With Bilateral Vocal Cord Palsy&dquo; (J Child Neurol 2001;16:932-934). We find no evidence, in either the text or the figures, to support a diagnosis of holoprosencephaly, lobar or any other anatomic variant. We agree with the authors that the imaging is consistent with the diagnosis of agenesis or the corpus callosum and apparent absence of the septum pellucidum, which always accompanies total callosal agenesis (actually, remnants of the septum sometimes can be demonstrated microscopically).The statement &dquo;Holoprosencephaly is a disorder of ventral induction&dquo; is true of some, but not all, of the six known genetic forms of this complex malformation, such as Sonic hedgehog (SHH) mutations ; Zic2 has a dorsalizing induction or gradient, and mutations of this gene produce holoprosencephalies very similar to those owing to SHH mutations. Since no genetic studies are described in this report, the conclusion that it is a &dquo;ventral inductive disorder&dquo; is speculative. The authors' term &dquo;vocal cord palsy&dquo; is incorrect. Muscles suffer paralysis, and nerves suffer &dquo;palsies&dquo; (eg, recurrent largyngeal nerve). The vocal cords themselves are fibroelastic, nonmuscular structures, and it is the largyngeal muscles that control their tension and that become paralyzed. chance to thoroughly review our articlel and to double check for possible slips or mistakes. We were pleased to see that our brief communication roused such deep interest also because we are aware of the great competence of both authors. However, our clinical experience has taught us that in clinical practice the strict dogmas that our colleagues would like to suggest do not exist. We respectfully reply to their comments as follows:1. Regarding the lack of support for the diagnosis of holoprosencephaly in the text and figures: A brief communication does not always allow the authors to fully detail the case history and incorporate multiple illustrations. The diagnosis of lobar holoprosencephaly in case 1 was straightforward, and we thought that the neuroimaging picture in Figure 2, A and B, along with our statement on diagnosis in the text, was sufficient documentation. 2. Regarding our statement, &dquo;Holoprosencephaly is a disorder of ventral induction&dquo;: We are obviously aware that underexpression of ventralizing gradients is the major (not the only) cause of holoprosencephaly in animals and also in humans,2 as reported in the same issue's review by However, such considerations did not prevent Drs Sarnats' themselves-in their recently proposed molecular genetic classification of central nervous system malformations4>5-from classifying holoprosencephaly solely under the category of &dquo;disorders of ventralizing gradient in the neural tube&dquo; (underexpression of ventralizing genes) (Table 2, ILB.4,4 and Table 1, H,B.4).' In addition, the lesson learned from Sonic hedgehog gene abnormalities as a cause of holoprosencephaly is that clinical exp...
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