[Acute epiglottitis caused by Haemophilus influenzae type b in children: presentation of 21 cases].

Background: The aim of this paper is to review the epidemiological, clinical and therapeutical characteristics of Haemophilus influenzae type b (Hib) epiglottitis in children at a time when an efficient and safe vaccination is available.

Methods: The clinical histories of 21 children admitted to Children's Hospital La Fe (1971-1996) with a clinical diagnosis of epiglottitis and isolation of the microorganism in blood cultures (20 cases) and surface culture of the epiglotis (one case) are reviewed.

Results: The annual average was 4/100,000 children under 5 years of age.

Neurofibromatosis type 1 in children: MR imaging and follow-up studies of central nervous system findings.

Purpose: To determine the frequency, evolution and diagnostic impact of characteristic central nervous system MR imaging lesions in children with neurofibromatosis type 1 (NF1).

Subjects: We reviewed 89 children with established or clinically suspected disease. A final diagnosis of NF1 was made in 72 (age range, 10 months to 14 years).

[Treatment of tobacco use. Pediatric aspects].

Objective: To divulge among pediatricians therapeutic modalities against tobacco use.

Methods: Literature review of therapeutic modalities, their efficacy indications, secondary effects and scientific value. Relapse-related factors are analyzed.

Sonographic findings in aneurysmal bone cyst in children: correlation with computed tomography findings.

Purpose: This study compares the value of sonography and computed tomography (CT) in assessing fluid-fluid levels (FFLs) in aneurysmal bone cysts (ABCs) in children.

Methods: Five children 4-13 years old with ABC in pubis, humerus, fibula, calcaneus, and ilium-ischium were studied between January 1995 and December 1996. Plain radiography, sonography, and CT were performed.

[Our experience in portal cavernomatosis in childhood: cross-sectional study and analysis of results].

A retrospective and transversal study of our patients with portal cavernomatosis is developed, with the aim of comparing the evolution and the present status of the patients that have undergone different therapeutical approaches. Between 1975 and 1995, 15 patients with portal cavernomatosis have been treated. Personal history, signs and symptoms at the moment of diagnosis, and treatment were analyzed.

[Solid ovarian tumors in childhood].

We evaluate our experience in solid ovarian tumors at the Children's Hospital La Fe. The main aims of the study were finding out relevant clinical data for a correct clinical approach, and the optimal surgical attitude in order to obtain tumor relieving with preservation of fertility when possible. A review of 28 girls under 14 years with solid ovarian tumors was carried out.

Cantrell's pentalogy. Report of four cases and their management.

Four neonates with the uncommon Cantrell's pentalogy seen in our hospital within a 6-month period without any apparent factor in common are described, with a review of the world literature. The first case was diagnosed prenatally at 15 weeks' gestation, the earliest intrauterine finding in the literature. The occurrence of imperforate anus with the syndrome, observed in one of the cases, has not been previously reported.

[Demyelinating diseases in childhood: diagnostic contribution of magnetic resonance].

Magnetic resonance imaging (MRI) exhibits a high sensitivity to detect white matter alteration but lacks specificity to characterize the disorder. We focus on the differential MR finding In two supposed autoimmune demyelinating diseases: multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM). In ADEM, the associated finding of T2 prolongation in deep gray matter, specially the thalamic involvement, is a useful distinguishing feature.

[Granulocyte recovery and early infection complications in oncologic pediatric patients treated with hematopoietic stem cell transplantation].

Objective: Infections during the early post-BMT period are a major cause of morbidity in BMT recipients. We report our experience in a single institution.

Patients And Methods: Clinical reports from all BMT patients between 1989-1995 were reviewed retrospectively.

Single aberrant umbilical artery in a fetus with severe caudal defects: sirenomelia or caudal dysgenesis.

We describe a 1,000-g twin fetus with absent kidneys and ureters, anal atresia and minimal evidence of external genitalia, and hypoplastic lower limbs with absent feet. A postmortem arteriogram showed a large single umbilical artery in direct continuation with the abdominal aorta, a unique anomaly almost always related to sirenomelia. We discuss the possible diagnosis of this case as sirenomelia or caudal dysgenesis, and the controversy as to whether they are two related or separate entities.

[Wandering spleen syndrome in childhood].

Wandering spleen is a clinical condition in which an absence of ligaments and a long vascular pedicle allows the spleen to move freely in the abdomen. It is a rare entity in children but the exactly incidence is unknown because most of them are asymptomatic. The most common form of presentation is acute, chronic or intermittent abdominal pain caused by spleen torsion.

Congenital intrahepatic portocaval shunt associated with trimethylaminuria.

A case of congenital portal-systemic shunting due to an intrahepatic connection diagnosed by ultrasound scanning and color Doppler in an 8-month-old girl is reported. She began to manifest trimethylaminuria 3 years later. At 7 years of age, she is asymptomatic without therapeutic measures except for diet.

[Latex allergy in children with myelomeningocele. Incidence and associated factors].

Rationale: The purpose of our work is to know the prevalence of allergies to latex in patients with myelomeningocele (MMC) and to evaluate any potential factors influencing its development.

Methods: The study included 100 children with mean age 7.5 +/- 4.

[Sturge-Weber syndrome: experience with 14 cases].

Objective: The objective of this study was to review the cases of Sturge-Weber syndrome (SWS) diagnosed and followed-up in our center over the last 25 year period in order to evaluate their clinical characteristics, evolution and therapeutical response.

Patients And Methods: A retrospective review of the records of patients diagnosed of SSW (facial nevus flammeus at least over the first branch of the trigeminal nerve and ipsilateral leptomeningeal angioma) was performed.

Results: Fifteen patients were found in our records, with one of them being excluded from the study due to the lack of follow-up.

Congenital intrahepatic portocaval shunt associated with trimethylaminuria.

A case of congenital portal-systemic shunting due to an intrahepatic connection diagnosed by ultrasound scanning and color Doppler in an 8-month-old girl is reported. She began to manifest trimethylaminuria 3 years later. At 7 years of age, she is asymptomatic without therapeutic measures except for diet.

[Ureteral ectopia in pediatric age: treatment and evolution in 55 cases].

In the last twenty years 55 children with ureteral ectopia were diagnosed and treated in our Hospital. Thirty seven of them were female (67.3%) and they presented the urinary tract infection like the most frequent symptomatology.

[The use of color doppler sonography of the acute scrotum in children].

The differential diagnosis of the causes of acute scrotum only by clinical means, is often very difficult and supposes the risk of making mistakes, which leaves to several unnecessary surgical explorations. By Colour Doppler Sonography we get some information about testicular flow that helps us in the diagnosis. During the last two years 72 consecutive cases of acute scrotum in children younger than 13 years old, were seen in our hospital emergency department.

[The prolapse of the rectum. Treatment with fibrin adhesive].

Injection of sclerosing solutions into the rectal submucosa or into the perirectal space is one of the many therapies designed to correct rectal prolapse. Since 1994, four patients affected with this condition have been treated with fibrin adhesive percutaneous injection. Up to now, this product had not been used to this aim.