[Haemophilus influenzae type b invasive disease in Catalonia (1996)]. / Enfermedad invasiva por Haemophilus influenzae tipo b en Cataluña (1996).

BACKGROUND: The purpose of this study was to find out the incidence and characteristics of H. influenzae type b invasive disease (HibID) in Catalonia, Spain. MATERIAL AND METHODS: An active surveillance of H. influenzae isolated from normally sterile sites was carried out during 1996. Microbiology laboratories of hospitals of Catalonia were periodically contacted by telephone. The serotype of all the strains was studied. RESULTS: The incidence of H. influenzae invasive disease (HIID) was 7.1 per 100,000 in children under 5 years and 1.0 per 100,000 in those over 5 years. The incidence of serotype b was 6.4 per 100,000 children under 5 years and 0.2 above this age. Only three strains belonged to types other than b (d, e and f). CONCLUSIONS: The incidence of HIbID is uncommon in Catalonia, lower than that registered in the prevaccine era in other countries and regions of the same geographical area.

[Cardiovascular manifestations of children's collagenosis (author's transl)]. / Trastornos cardiovasculares en las colagenosis infantiles.

Cardiovascular manifestations of children's collagenosis is revised. Incidence varies in different entities from exceptional to constitute the main clinical characteristic of the disease. The pathogeny of the cardiac manifestations is diverse; heart primary inflammation, arterial hypertension, pulmonary hypertension, etc. The most frequent peripheric vascular symptom is Raynaud's phenomenon. Cardiac symptoms include: tachycardia, cardiac murmurs and dysrhythmias, etc., which are inespecific and appear when the cardiac lesion is important existing a disagreement between high incidence of alterations as there are found in autopsy and the rarity when the clinic diagnosis of cardiopathy was made.

[Marginality, ethnic groups and health]. / Marginalidad, grupos étnicos y salud.

Main marginated ethnic groups in Span are to be found among gypsies and 3rd world immigrants. The first group include about 250,000 persons and the second group more tan half a million people. Their origins and their being past of the less fortunate social layers made them a group of health risk. Pediatric pathologies are those favored by socio-economic shortcomings as well as hygienic-sanitary deficiencies. Imported pediatric pathologies have a small incident.

PIP: Marginalized ethnic groups in Spain consist primarily of some 250,000 gypsies and over half million immigrants from Third World countries who began to seek work in newly industrialized Spain beginning around 1970. The numbers of immigrants doubled between 1970-85 and immigration continues from Latin America, Africa south of the Sahara, North America, and the Philippines. Over 300,000 are believed to be undocumented, and around 23% are under 16 years old. This large scale immigration in a traditional country of emigration has created previously unknown problems for the health services. Health personnel still lack adequate knowledge of the cultural patterns, illnesses and needs of these groups, and frequent difficulties of communication complicate problems. Immigrants frequently initiate contact with the health services in search of treatment for their children, who are considered a population at risk. Imported diseases from the region of origin progressively lose importance as the immigrants are assimilated, typically into the least prosperous classes. Their illnesses lose specificity, and perinatal and early childhood problems emerge as the predominant concerns. Imported problems may include genetically determined illnesses such as sickle cell anemia, infectious and parasitic diseases prevalent in the region of origin, or pathologies contracted directly from indigenous medical treatments. Ailments of children acquired in Spain include the normal childhood diseases, with respiratory and gastrointestinal infections most common, dehydration caused by dietary errors, rickets, chronic lead poisoning caused by old paint in the dwelling, and accidents. Pathologies of adaptation may include other diseases but are typically psychic disorders resulting from the 2 greatest disadvantages facing immigrants: poverty and racism. Greater perinatal morbidity and mortality and more frequent and longer hospitalizations of infants and children are 2 consequences. The degree of marginalization experienced by gypsies in Spain depends somewhat on their region of residence. In recent years they have tended to cluster in periurban zones outside the large cities, where their neighbors live in similar poverty and share the same diseases of deprivation. Pediatric health problems are similar to those acquired in Spain by immigrant children. Gypsies have higher rates of fertility and infant mortality. Gypsy children are at higher risk of tuberculosis, malnutrition, accidents, dental caries, sand drug consumption, and they are less likely to be immunized. They are a recognized risk group for iron deficiency anemia and lead poisoning, and their rates of hospitalization and consultations in emergency rooms are elevated.

[Intrahepatic portal venous hypoplasia in Alagille's syndrome]. / Hypoplasie veineuse portale intrahépatique dans le syndrome d'Alagille.

One new case of arteriohepatic dysplasia (Alagille's syndrome) is reported. The case showed signs of progressive portal hypertension with the liver biopsy revealing evidence of hypoplasia of the intrahepatic portal venous branches without evident cirrhosis, next to the characteristic paucity of intrahepatic bile ducts. This venous abnormality might be responsible for the unfavourable outcome observed in some cases with Alagille's syndrome and corresponds to a severe form of the disease.

[Male pseudohermaphroditism due to deficit in the conversion of cholesterol to delta5 pregnenolone (author's transl)]. / Pseudohermafroditismo masculino por defecto en la conversión del colesterol en delta5 pregnenolona.

One patient, aged 13 months, considered phenotipically a female, was admitted with a picture of acute dehydration. Familial history (one sister dead from the same picture at the age of 18 months), clinical data (several episodes of dehydration, dark skin and mucosae, and slight abnormalities in the external genitalia), hormonal examinations (low plasmatic levels of cortisol, aldosterone, androgens and low urinary excretion of 17 hydroxycorticoids, dehydroepiandrosterone and ethiocholanolone), chromosomal examination (karyotype XY) and histological data (normal testis) suggested a diagnosis of male pseudohermaphroditism with complete feminization due to an abnormal conversion of cholesterol in delta5 pregnenolone. Late appearance of the first episode of dehydration, particularly intense cutaneous pigmentation and statural growth and bone maturation both unaffected, are some particular traits of this patient.

Gram-negative germs infections in infancy.

The incidence of systemic or local infections due to gram-negative bacilli in an Infant Ward from September 1969 to December 1976 was 7.9%. The 29.34% were septicemia, most of them as epidemic outbreaks caused by Pseudomonas aeruginosa, Klebsiella-Enterobacter and Serratia marcescens. Two facts are to be emphasized: an almost complete disappearance of systemic infections with Pseudomonas starting from 1972, and the global predominance of the group Klebsiella-Enterobacter, particularly evident from 1970 to 1972.

Paraneoplasic precocious puberty. Report of a new case with hepatoblastoma and review of the literature.

A new case of precocious puberty secondary to production of human chorionic gonadotropin (HCG) by an hepatoblastoma was studied in an 8-month-old infant. A review was made of the 35 cases of paraneoplasic precocious puberty previously reported in the literature. The most frequent cause is hepatoblastoma, which was responsible for 18 cases. There are nine reports of mediastinal teratoma, six of which were observed in patients with Klinefelter Syndrome. In another six cases, the paraneoplasic syndrome was attributed to intracranial tumoration, and two of these patients showed teratoma with areas of choriocarcinoma, whereas germinoma was suspected for clinical reasons in another three. Lastly, one case of presacral teratoma and one of retroperitoneal carcinoma were reported. The differential characteristics of paraneoplasic precocious puberty are: almost exclusive occurrence in males, because of tumoral production of HCG, moderate or absent increase of testicle size, hyperplasia of Leydig's cells without spermatogenesis, and rapidly progressive signs of puberty.

Acute otitis media in childhood: a study of 20,532 cases.

We present a study of acute otitis media among 20,532 Spanish children during a 6-month period from January through June 1991. The study was done by distributing a questionnaire to all Spanish pediatricians. Of the patients 51.7% were girls and 48.3% boys; 68.7% of all children were younger than 5 years. The most frequent symptoms were otalgia or irritability, 92.7%; fever, 63.5%; otorrhea, 24.9%; vomiting, 21.4%; and another concomitant upper respiratory tract infection, 65.5%. Otitis was bilateral in 45.6% of the cases. The most frequently used antibiotic was cefixime (38%), followed by amoxicillin-clavulanate (22.2%), amoxicillin (15.3%), 2nd-generation cephalosporins (13.5%) and macrolides (8.8%). In 11.8% of the patients, a change in the initial antibiotic therapy was required due to adverse effects. Resolution of symptoms occurred in 77.8% of the patients.

Congenital methemoglobin-reductase (cytochrome b5 reductase) deficiency associated with mental retardation in a Spanish girl.

Methemoglobinemia and mental retardation associated with NADH-diaphorase deficiency was found in a 2-year-old girl of Spanish origin. She showed no NADH-diaphorase activity in either erythrocytes or leukocytes, but electrophoretic studies of the hemolysate showed traces of an enzyme with normal mobility. Cytochrome b5 reductase activity was also found to be absent in the leukocytes of the propostius. Intermediate NADH-diaphorase activity was found in erythrocytes and leukocytes in her parents and her sister in accordance with the autosomal recessive mode of inheritance of this enzymopathy. The relationship between a generalized cytochrome b5 reductase deficiency and the progressive neurological involvement in our patient is discussed briefly.