[Castleman disease]. / Enfermedad de Castleman.

Castleman disease or angiofollicular hyperplasia is a rare disorder included in the group of lymphoproliferative disorders. This entity was originally described by Castleman in 1956. The etiology remains unknown but it is postulated to be a reactive lymphoid hyperplasia due to chronic antigenic stimulation caused by a viral infection. The disease presents in young adults and is more frequent in women; it is exceptionally rare in the pediatric age group. It is classified into two clinical groups (localized disease and disseminated disease) and there are two histologic variants (hyaline-vascular and plasma cell Castleman disease). Localized disease is usually asymptomatic, has a good prognosis, and is the most common presentation in pediatric patients, usually corresponding to highly vascularized mediastinal masses. Resection of the mass, which is curative, is associated with a high risk of blood loss. Recently, preoperative arteriography with embolization has been used satisfactorily in the preoperative management of these tumors. We present a case of localized Castleman disease in a 12-year-old girl satisfactorily treated with embolization before curative resection.

Enfermedad de Castleman / Castleman disease

La enfermedad de Castleman o hiperplasia angiofolicular es una entidad poco frecuente enmarcada dentro de los trastornos linfoproliferativos. Esta enfermedad fue descrita inicialmente por Castleman en 1956. Su etiología es desconocida, pero se cree que responde a una estimulación antigénica crónica de probable etiología viral. Se presenta en adultos jóvenes, más frecuente en mujeres, y es excepcional en la edad pediátrica. Clínicamente se distinguen dos formas de enfermedad: una localizada y otra multicéntrica. La forma localizada, de buen pronóstico, asintomática al diagnóstico, es la forma de presentación más frecuente en la edad pediátrica y por lo general corresponde a masas mediastínicas muy vascularizadas. La resección de la lesión, que es curativa, se asocia a un alto riesgo de sangrado. Recientemente se ha introducido la arteriografía con embolización prequirúrgica para minimizar el riesgo de sangrado en el acto quirúrgico. Se presenta un caso de enfermedad de Castleman localizada en una paciente de 12 años en la que se utilizó con éxito la angioembolización prequirúrgica

Castleman disease or angiofollicular hyperplasia is a rare disorder included in the group of lymphoproliferative disorders. This entity was originally described by Castleman in 1956. The etiology remains unknown but it is postulated to be a reactive lymphoid hyperplasia due to chronic antigenic stimulation caused by a viral infection. The disease presents in young adults and is more frequent in women; it is exceptionally rare in the pediatric age group. It is classified into two clinical groups (localized disease and disseminated disease) and there are two histologic variants (hyaline-vascular and plasma cell Castleman disease). Localized disease is usually asymptomatic, has a good prognosis, and is the most common presentation in pediatric patients, usually corresponding to highly vascularized mediastinal masses. Resection of the mass, which is curative, is associated with a high risk of blood loss. Recently, preoperative arteriography with embolization has been used satisfactorily in the preoperative management of these tumors. We present a case of localized Castleman disease in a 12-year-old girl satisfactorily treated with embolization before curative resection

¿Qué sucede en la vía aérea que justifique este tipo de asma? / No disponible

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[Diagnosis and surgical treatment for male pseudohermaphroditism in a multidisciplinary unit of intersexual conditions]. / Diagnóstico y tratamiento quirúrgico del pseudohermafroditismo masculino en una unidad multidisciplinaria de estados intersexuales.

The birth of a child with ambiguous genitalia represents a very stressing situation for the family, and afterwards has great social and psychological repercussion for the patient itself. Until now, the sex assignation is being done according to the phenotype. Now, with the molecular diagnosis of the genes that play a role in the sexual development, the assignation must also take into account the prognosis of response to androgens. The aim of this work is to review the 40 male pseudohermaphroditism cases controlled in our hospital and the genetic molecular diagnosis done in 19 cases, thus obtaining the certainty diagnosis. In 15 patients the mutations were located in the AR gene (androgen receptor). In 2 cases the mutation affected the SRD5A2 gene (deficiency of 5a-reductase) and in the other 2 cases it affected the HSD17BIII gene (deficiency of 17-ketoreductase). If the mutations affect the AR gene they must be assigned to the feminine sex, because of the impossibility of virilisation at puberty (lack of response to androgens). If the mutations are located in the other 2 genes they can be assigned to the masculine sex, since in puberty, they will present good response to androgens and will virilize. The molecular diagnosis offers us also the possibility to establish a prenatal diagnosis and to offer genetic advice.

[Sudden death in a 4-month-old infant associated with anomalous origin of the left coronary artery]. / Muerte súbita en un lactante de 4 meses asociada a origen anómalo de arteria coronaria izquierda.

We describe the autopsy findings of a 4 months-old boy, who died suddenly after an episode of high airway infection of 3 days time. Autopsy examination showed cardiomegaly (80 g) with widening of the left cavities and thick and white endocardial surface, besides a severe thinning of the cardiac apex at the level of the left ventricle. Left coronary origin was in the pulmonary artery trunk. Histologically, the myocardium shows endomyocardial fibroelastosis and also multiple and extensive areas of old and recent infarcts in the left ventricle. The collateral coronary arteries, were increased in number, and branches showed a marked intimal oedema and a reduction of the luminal diameter.

[Adenomatous cystic pulmonary malformations: presentation of 26 cases]. / Malformación adenomatoidea quística pulmonar. Presentación de 26 casos.

We present our experience of 26 cases of cystic adenomatoid malformations of the lung (CAM), treated in the Department of Pediatric Surgery in our hospital between 1967-1991. There were two clinical pictures: one neonatal severe respiratory distress and successive repeated pulmonary infection appearing after the patient's first year of life and requiring both urgent diagnosis and treatment. Embryological development determines the pathologic classification of this entity in 3 types. Basic examinations by image are analyzed, bearing in mind their diagnostic value and the patterns they show. After analysing all the conditioning factors, no explanation has been found to the different course that this affectation (< 1 month and > 1 year of age respectively). Normally, neonatal mortality is closely related to other malformations, particularly to cardiovascular ones. Differential diagnosis is very important in the neonatal period, especially with regard to diaphragmatic hernia, lobar emphysema and pulmonary cysts. All these cases have been verified and classified by means of a pathologic study, which has shown the need for surgical operation. In the follow up of the patients no alteration has been noticed in the pulmonary function.

[Congenital lobar emphysema: report of 38 cases]. / Enfisema lobar congénito. Presentación de 38 casos.

We present our experience of 38 patients suffering from congenital lobar emphysema (CLE) treated in the Department of Pediatric Surgery in our hospital between 1966-1991. 22 of these patients had to undergo surgical correction due to the severity of their respiratory symptoms. The other 16 presented mild respiratory symptoms: 8 of them received conservative treatment and the remainin 8 had to be operated on due to broncial compression caused by the vascular malformation. In most cases the main symptoms were dyspnea and cyanosis, which indicate the severity of the process. 20 cases presented during the patient's first month of life, 10 within the first 6 months and the remaining 8 between the first 6 months and 5 years. The different aetiologics forms in our study are analysed and compared with those described in the literature. The main diagnostic tested and revied, specially radiology tested led to a correct differential diagnosis and subsequently to an adequate therapeutic treatment. It is possible to carry out other tests since they do not actually help diagnosis, we have considered them unnecessary. All the surgical operations used are analysed as well as the complications observed. The clinical course of the patients has proved to be particularly good, since there was no mortality and the morbility rate was low.

[Intensification of the initial treatment in acute lymphoblastic leukemias of middle and high risk in children]. / Intensificación del tratamiento inicial en las leucemias agudas linfoblásticas de medio y alto riesgo en el niño.

The aim of study LAL 17/84 was to reduce the number of relapses by intensifying initial treatment. Patients were classified as high (HR) and standard-risk (SR) groups following the established risk index. Early response, after 2 weeks of treatment was also considered. SR protocol includes a ten-week induction and CNS prophylaxis phase: in the first 5 weeks, prednisolone (PRED), vincristine (VCR), daunorubicin (DAUNO) and asparaginase (ASPAR) are given; in the last 5 weeks two intravenous infusions of Ara-C and three of intermediate dose (ID) methotrexate (MTX) are administered simultaneously with 5 it injections of both drugs. CNS prophylaxis is completed with 5 further it injections: one at the beginning and 4 at monthly intervals from the 3rd to the 6th months. In the HR protocol, the initial phase lasts 16 weeks: in the first 5 the same drugs as in SR are used but doses of PRED and ASPAR are higher. CNS prophylaxis includes holocranial radiotherapy (18 Gy), 2 iv infusions of Ara-C and a further 2 of MTX ID and 6 it doses of MTX and Ara-C. Finally, a consolidation phase of PRED, VCR (3 doses), tenoposide (3 doses) and cyclophosphamide is given. Complementary chemotherapy in both protocols consists of daily mercaptopurine and weekly MTX for 2 years; moreover, the randomized half of the patients received monthly reinforcements with PRED, VCR (and DAUNO in HR group) for 4 months. Between Oct. 83 and Nov. 87,87 patients with ages between 3 months and 15 years were enrolled; 86 are evaluable. All achieved remission.(ABSTRACT TRUNCATED AT 250 WORDS)

[Cystic adenomatoid malformation of the lung (author's transl)]. / Malformación adenomatoidea quística congénita del pulmón.

Four cases of congenital cystic adenomatoid malformation are presented. The age of the patients ranged from 1 day to 7 years. All cases were treated surgically and 3 of them survived. The only death in the group corresponded to the neonatal case. The clinical, radiological and patological features of congenital cystic adenomatoid malformation are also reviewed. Occasionally the clinical manifestations will be hardly noticeable or non existent and the malformation may be diagnosed beyond the neonatal period in the course of a radiological examination performed as routine or because of repeated respiratory infections. Surgery is the treatment of choice. In the group of neonates where the cystic adenomatoid malformation doesn't cause respiratory difficulty the authors feel that it might be advisable to postpone surgery until a later date.