A report of two cases

British authors are the natural heirs to Charles Dickens and Graham Greene.) Nevertheless there are advantages to British readers in a British book especially, for instance, in the chapter on social paediatrics and occasionally as regards therapeutics. The new Forfar and Arneil is, like its predecessors, a good book. There are many new authors and much of it has been completely rewritten. Each chapter has a long list of references, many of them to further reading in books and important 'landmark' papers rather than to very recent original work, and that seems appropriate for a textbook of this nature. It is up to date, including references to topics such as oesophageal pH monitoring in gastrooesophageal reflux, the value of steroids in bacterial meningitis, a full account of the peroxisomal disorders, the significance of dystrophin in muscular dystrophy, the importance of vitamin A in reducing mortality in third world countries, and the use of sumatriptan in migraine and of vigabatrin and lamotrigine in epilepsy. Surprisingly, neither in this nor in Nelson's textbook could I find any reference to Angelman's syndrome. Some topics chosen at random such as Prader-Willi syndrome or haemolytic uraemic syndrome were discussed more fully in the British textbook. There is a surprising omission, however, and that is the chapter on disorders ofear, nose, and throat. Adenoids, glue ear or secretory otitis media, tonsillitis, and tonsillectomy have all been dropped from the index and deafness is dealt with in the chapter on neurology. If you look up 'tonsils' you will be referred to any entry which reads: 'Tonsils: size, presence of infection, exudate, pitting, peritonsillar swelling?'. The index entry 'ear, nose and throat problems' refers to a brief account of such problems in children with cleft palate. It is one of those crazy facts of modern life that the American book costs £60, the British £125. Who will read this new edition? I shall, and I suppose many other consultant paediatricians will also use it as a standard reference book. Undergraduate students will need a more compact text for their basic reading but should use it to look up specific topics, and doctors studying for the MRCP in paediatrics should make a systematic assault on it to provide them with a personal database to be supplemented by journals and monographs. As a textbook of British paediatrics it has no competition and it remains an excellent work of reference which must be available in all paediatric departments in Britain, but the absence of the chapter on ear, nose, and throat disorders must surely be an error and there is clearly a marketing problem for British publishers faced with American competition. If we want British textbooks, and I would not like to think that the time will be forced upon us when we shall have no choice, then it looks as if we must be prepared to pay for the privilege. DOUG ADDY Consultant paediatrician

https://adc.bmj.com/content/archdischild/68/3/435.2.full.pdf

Syndromes of the Head and Neck

Pierre Robin syndrome

Melkersson-Rosenthal syndrome (MRS) in children is a rare condition, clinically characterised by a triad of synchronous or metachronous symptoms: recurrent peripheral facial palsy, relapsing orofacial oedema, and a fissured tongue; the most recent review published on the topic has reported 30 published patients. The aetiology of this disease is still unclear. However, genetic factors, as well as alterations in immune functions, infections, and allergic reactions have been postulated. We report three children suffering from MRS and perform a literature review of paediatric cases. Taking into account that clinical and laboratoristical criteria for the diagnosis of MRS are lacking, this syndrome is probably underestimated, and we suggest increasing awareness of such a rare syndrome. Close multidisciplinary follow-up of these children with a team composed by paediatricians, neurologists, neuro-ophthalmologists, dermatologists, and otolaryngologists is crucial to guarantee exhaustive management and treatment success, while minimising relapses.

Melkersson⁻Rosenthal Syndrome in Childhood: Report of Three Paediatric Cases and a Review of the Literature.

Neuromyelitis Optica (NMO) is an autoimmune disease with a higher prevalence in non-European populations. Because the Mexican population resulted from the admixture between mainly Native American and European populations, we used genome-wide microarray, HLA high-resolution typing and AQP4 gene sequencing data to analyze genetic ancestry and to seek genetic variants conferring NMO susceptibility in admixed Mexican patients. A total of 164 Mexican NMO patients and 1,208 controls were included. On average, NMO patients had a higher proportion of Native American ancestry than controls (68.1% vs 58.6%; p = 5 × 10–6). GWAS identified a HLA region associated with NMO, led by rs9272219 (OR = 2.48, P = 8 × 10–10). Class II HLA alleles HLA-DQB1*03:01, -DRB1*08:02, -DRB1*16:02, -DRB1*14:06 and -DQB1*04:02 showed the most significant associations with NMO risk. Local ancestry estimates suggest that all the NMO-associated alleles within the HLA region are of Native American origin. No novel or missense variants in the AQP4 gene were found in Mexican patients with NMO or multiple sclerosis. To our knowledge, this is the first study supporting the notion that Native American ancestry significantly contributes to NMO susceptibility in an admixed population, and is consistent with differences in NMO epidemiology in Mexico and Latin America.

Native American ancestry significantly contributes to neuromyelitis optica susceptibility in the admixed Mexican population

Malformaciones craneofaciales. De las bases moleculares al tratamiento quirúrgico

Síndrome de Melkersson–Rosenthal

Intro and aim The morbidity at the donor-site in the free fibula flap is a well-studied fact in the adult population, but in children, there are few reports of the long-term adverse effects at the donor site. In this study, we evaluate donor-site morbidity, complications, and functional outcome in pediatric patients undergoing free fibula flap harvest for mandibular or maxillary reconstruction, and the relation between pain and walking abilities. Methods A retrospective study of 22 pediatric patients undergoing free fibula flap harvest for mandibular and maxillary reconstruction between 2003 and 2014 was conducted. A certified point evaluation system was used, analyzing several factors like pain, walking ability, restriction in activities, gait alteration, paresthesia, and cosmetic appearance, and the relationship between variables. Results Mean age follow up of patient was 8.5 years (range 2-13 years). Pain was reported in 27% (n = 6), paresthesia and numbness 9% (n = 2), walking ability alteration in 50% (n = 11), restriction in activities in 18% (n = 4), gait alteration in 23% (n = 5), and cosmetic appearance alteration in the 14% (n = 3). We compare the pain and the gait alteration and did not find any relation between pain and gait alteration (P = 0.6016) and there was no relation between restriction in activities and walking ability (P = 0.1455). Conclusion Free fibula flap is the gold standard for head and neck reconstruction, but the morbidity at the donor site, especially in a growing child, should not be ignored or minimized when counseling patients and their families.More studies, with more population and with a long-term follow-up should be carried out to determine the impact on the daily activities on the pediatric population.

Donor-Site Morbidity Following Free Fibula Flap Harvest for Mandibular or Maxillary Reconstruction in Pediatric Patients.

Summary The finding of the appendix inside an hernial sac is called “Amyand hernia”. The global incidence is 0.28 to 1%. Clinical manifestations are the presence of an inflamed inguinal mass, tense, hypersensitivensible, with variable size, non-reducible, and associated to abdominal pain, vomit and very rarely true appendicitis manifestations. Surgical treatment depends on the case presentation and the intraoperative fin

Hernia de Amyand gigante

La equinococosis o hidatidosis es una parasitosis causada por helmintos del genero Echinococcus. El pulmon representa el segundo sitio mas comun de localizacion. En esta comunicacion se presentan dos casos de hidatidosis pulmonar en pacientes masculinos dedicados a la agricultura y a la crianza de ganado ovino. En ambos casos se realizo exitosamente tratamiento quirurgico, el cual permitio la resolucion de la enfermedad. La importancia de esta comunicacion reside en la escasa frecuencia con que se reporta esta enfermedad en nuestro pais

/pdf/hidatidosis-pulmonar-reporte-de-dos-casos-clinicos-5gf4gmymwd.pdf

Damián Palafox

Papers

Malformaciones craneofaciales. De las bases moleculares al tratamiento quirúrgico

Síndrome de Melkersson–Rosenthal

Donor-Site Morbidity Following Free Fibula Flap Harvest for Mandibular or Maxillary Reconstruction in Pediatric Patients.

Hernia de Amyand gigante

Hidatidosis pulmonar. Reporte de dos casos clínicos