[Anhydrotic ectodermal dysplasia associated to mannose-binding lectin deficiency]. / Displasia ectodérmica anhidrótica asociada a déficit de lectina de unión a manosa.

Mannose-binding lectin (MBL) is a serum protein of the innate immune system. MBL enhances opsonophagocytosis by binding to carbohydrates expressed by multiple pathogens. MBL deficiency is due to polymorphisms in the structural and promoter sequences of the MBL2 gene and is associated with variety of recurrent infections, including respiratory tract infections. We present a case of anhidrotic ectodermal dysplasia associated with severe mannose-binding lectin deficiency, never described in patients with anhidrotic ectodermal dysplasia.

[Retropharyngeal and parapharyngeal abscess: experience in a tertiary-care center in Seville during the last decade]. / Abscesos retrofaríngeo y parafaríngeo: experiencia en hospital terciario de Sevilla durante la última década.

INTRODUCTION: Retropharyngeal and parapharyngeal abscesses are rare but associated with significant morbidity and potential mortality. This study reviews our experience in the diagnosis and management of retro- and parapharyngeal abscesses and compares children treated conservatively with those undergoing surgical intervention. MATERIAL AND METHODS: A retrospective analysis of children diagnosed with retro- and parapharyngeal abscess from 2000 to 2009 in our tertiary-care centre. RESULTS: Thirty-one children were identified. There were 17 retropharyngeal abscesses and 11 parapharyngeal abscesses; 3 children suffered from both conditions. The mean annual frequency increased significantly from 1.4 cases/year during 2000-2004 to 4.8 cases/year during 2005-2009 (P=.006). Median age was 3 years (range 1-10). A total of 18 (58%) children had received pre-admission oral antibiotics (beta-lactams in 84%). Clinical findings at presentation were: fever (93%), cervical lymphadenopathy (93%), neck pain (90%), torticollis (74%), odynophagia (64%), trismus (32%), drooling (22%) and stridor (6%). Thirteen (42%) children underwent surgical intervention, of those, microbiological culture was positive in 8 children; S. pyogenes being the most commonly isolated organism (n=4). All the patients received parenteral antibiotic therapy. There were no significant differences in the length of hospital stay, complication or recurrence rates between children treated conservatively compared to those undergoing surgical intervention. CONCLUSIONS: Retro- and parapharyngeal abscesses were increasingly observed during the 2(nd) part of the study period. The majority of children (58%) were treated conservatively with excellent clinical response. Indication for surgical intervention should be made based on the clinical presentation and response to antibiotic therapy.

Brucellosis infection presenting with cholestasis.

Brucellosis is a relatively common disease in the Mediterranean area and may present with prolonged fever without focus, however it remains an important diagnostic challenge to most pediatricians. We report the case of a 10-year-old male patient who presented with fever without a focus of 10-day duration, hepatomegaly, ascites, a small elevation in transaminases and acute-phase reactants indicating cholestasis, leukopenia, and thrombocytopenia. Imaging tests showed many small, rounded, hypodense focal lesions in the liver and spleen. After eliminating a wide range of diseases, positive results for the Rose Bengal test and indirect immunofluorescence assay for Brucella melitensis made it possible to establish a diagnosis of hepatosplenic brucellosis. A review of the family history revealed direct contact with farm animals. The patient made good progress on treatment with doxycycline and streptomycin, with complete resolution of both clinical symptoms and imaging signs. The prevalence of brucellosis is gradually increasing, mainly due to migratory movements. It should always be eliminated as a source of unknown fever in endemic areas and should also be taken into account in other geographical areas where it is not common whenever a patient presents with prolonged fever and unspecific symptoms. Standard therapy is highly effective, even in relapse cases, and early diagnosis leads to a rapid recovery and favorable outcome. The unusual presentation in the case reported here reminds us that it is possible to encounter a Brucella infection in a case of fever without a focus, irrespective of the existence of a clear epidemiological history, which is very often omitted by the family. All differential diagnostic protocols for fever without a focus should include a diagnostic test for Brucella in order to achieve early detection of the disease and initiate therapy promptly.

[Acute mastoiditis: experience in a tertiary-care center in the South of Spain during 1999-2008 period]. / Mastoiditis aguda: experiencia en los últimos 10 años en hospital terciario del sur de España.

INTRODUCTION: An increase in both the incidence and severity of acute mastoiditis (AM) has been recently recorded in many different geographical areas. Causes remain unclear. This study aims to evaluate our clinical and epidemiological data in paediatric patients with AM and to compare them with recent reports. MATERIAL AND METHODS: Retrospective chart review of 145 patients diagnosed of AM from 1999 to 2008 in our tertiary-care centre, including clinical, epidemiological, microbiological, treatment and outcome data. RESULTS: The annual incidence showed a changeable trend throughout the study period. The average number of cases was 14.5 cases per year, with a median age of 3 years, and 57.9% males. A total of 53.8% received pre-admission oral antibiotics, mainly beta-lactamics. Most frequent presenting clinical findings were fever (77.9%), ear displacement (73.8%), otalgia (71.7%), and postauricular swelling (70.3%). Microbiological cultures were performed in 53 cases; S. pneumoniae was the most isolated microorganism. CT scans were performed in 56.6% cases. All patients received parenteral antibiotic treatment with a median duration of treatment of 5 days. Surgery was performed on 32.6%. Complications were seen in 20% of patients: 13.1% had extracranial complications, and 8.2% had intracranial complications. A significant increase in intracranial complications was detected in the second half of the study period. CONCLUSIONS: A changeable trend in the annual incidence of AM throughout the time of study was observed. A higher proportion of intracranial complications was detected in the last five years of the study period.

[Unusually high frequency of complications in acute otitis media]. / Frecuencia inusualmente elevada de complicaciones secundarias a otitis media aguda.

INTRODUCTION: Intracranial complications (ICC) secondary to otitis media are unusual but potentially life-threatening. We report an unusually high frequency of these events, and describe their clinical and epidemiological features. MATERIAL AND METHOD: A retrospective study of all pediatric patients with ICC admitted to our tertiary hospital from April 2004 to November 2007. RESULTS: Eight patients had ICC: Sigmoid sinus thrombosis (4), lateral sinus thrombosis (1), meningitis (2), epidural abscess (1), otitic hydrocephalus (1). Four of the patients had acute mastoiditis. Pre-admission oral antibiotics were administered in 87.5% of the patients. Fever, otalgia, headache and VI and VII cranial nerves paralysis were the most frequent symptoms associated. The microorganism could be isolated in 3 patients: S pyogenes (2) and P mirabilis (1); the remaining patients had negative cultures. All of the patients received broad-spectrum parenteral antibiotics and 2 of them underwent a radical mastoidectomy. CONCLUSIONS: We report an unusually high incidence of cranial complications secondary to acute otitis media. Multicenter studies are needed in order to assess whether there is an increase in the incidence of these events.

[Meningitis due to haemophilus influenzae type f]. / Meningitis por Haemophilus influenzae tipo f.

We report an immunocompetent 5-month-old boy with Haemophilus influenzae type f (Hif) meningitis. The patient had previously been immunized with two doses of Hib conjugate vaccine (PRP-T). Vaccination failure was initially suspected based on Gram stain report. The results of culture identified a non-b Haemophilus influenzae capsular serotype (Hif).Non-Hib serotypes should be considered as potential pathogenic agents in children under the age of 5 years with invasive diseases. An adequate epidemiological surveillance system would be helpful in detecting the role of these non-b Hif serotypes as significant pathogens, which appear to be on the increase.

[IgG2 deficiency. Its clinical correlation with recurrent otitis media in childhood]. / Déficit de IgG2. Su correlación clínica con otitis medias de repetición en la infancia.

IgG2 deficiency has been reported in two recent years as an antibody deficiency associated mainly with recurrent acute and chronic ORL and lower respiratory and recurrent otitis media in children evaluated. We discuss the most rational approach to the treatment with special reference to the replacement therapy with intravenous immunoglobulin.