Biomedical subjects
A Marqués Gubern
Publications and source records attributed to A Marqués Gubern.
[Hepatoblastoma today. Our experience].
UNLABELLED: Prognosis of hepatoblastoma in children has improved in the last few years particularly because of preoperative chemotherapy and total surgical removal. The aim of this study was to present the evolution of HB therapy at our centre. Since 1966, 44 patients have been treated. The first 20 cases (group I) were treated with chemotherapy alone. The next 19 patients (1975-1989) (group II) underwent preoperative chemotherapy and delayed surgery. After 1990, 5 patients (group III) were treated with protocolized preoperative chemotherapy and accurate surgery (SIOPEL-1). RESULTS: survival rates for the three groups were: 0%, 15% and 100%, respectively. CONCLUSIONS: HB differs from hepatocellular carcinoma pathologically and in onset of symptoms and prognosis. SIOPEL-1 results showed that preoperative chemotherapy aids tumour involution and therefore facilitates resectability and that accurate surgical technique permits safe removal of huge masses. Acceptance of these pillars for treatment of HB is currently essential for paediatric surgeons.
[The first year of life of children with myelodysplasia: a multicenter study of 393 cases].
A multicentric study about the first year of life of myelodisplastic children, was done. Family, pregnancy, delivery, newborn somatometry, and anatomical-pathological and terapeuthical aspects were review in 393 patients born before july 1992. Annual prevalence vary from 6 (1973) to 28 (1984). The families clinical history was not significant. There were 9 (2,3%) gemelar pregnancies, but both children were affected only in one case. Prenatal ultrasound was not diagnostic in 61%-84% of patients are meningomyeloceles, the anatomical level of myelodisplasia was lumbar and or sacral in 98%. There was hydrocephalus in 59%, hip dislocation in 24%, feet deformities in 50%, upper urinary tract dilatation in 12%, vesicoureteral reflux in 15%. In total 825 surgical procedures were performed (M = 2); the neurosurgery was 94% of them.
[Spina bifida: management after 25 years].
The authors present their experience of 468 patients with spina bifida treated in a period of 25 years. They discuss the philosophy of the management of the severe neural tube defects based in the follow up of these patients. This attitude is based in the prevention that could be: 1) following Smithells theory; 2) trying a prenatal diagnostic before 20 weeks of pregnancy, and 3) doing a selection of spina bifida that could be operated.
[Thyroid cancer in childhood: review of 7 cases].
Thyroid carcinoma in childhood is a rare, but nevertheless malignant disease, with a high incidence of nodal metastasis. Seven children, 6 boys and 1 girl, aged 4 to 9 years with thyroid carcinoma were treated at the Hospital Infantil Valle de Hebrón of Barcelona between 1965 and 1990. The diagnosis was established by cervical nodal biopsy in all cases except one. Papillary carcinoma was found in 4 cases, follicular type in 2 cases and one case of mixed carcinoma was diagnosed. Surgical treatment consisted in lobectomy on the affected side, followed by total thyroidectomy on the opposite side and bilateral resection of involved nodes one or more times. In two patients, treatment with sodium-iodine I131 showed active tissue in the thorax and wound. Both were treated effectively. All patients are alive without recurrences during a follow-up period of 1 to 23 years. Main complications were: temporary recurrence of the nerve injury in two patients and a controlled hypoparathyroidism in another two cases. In all cases, treatment with L-thyroxine was required. Thyroid carcinoma in children carries a considerably better prognosis than in adult patients. We must suspect thyroid carcinoma and indicate biopsy in all cases of cervical nodes resistant to antibiotic treatment. Early diagnosis, total thyroidectomy, radioiodine treatment and hormone administration permit radical cure even in the case of metastatic disease.
[Neoplasms during the 1st year of life: a study of 116 cases treated during the last 10 years].
116 infants under one year of age (14 per 100) with malignancies including 35 neuroblastomas, 23 retinoblastomas, 14 Wilm's tumours, 10 hepatoblastomas, 10 brain tumours, 9 germ cell tumours, 8 histiocytic and 7 soft tissue sarcomas, were treated in the last ten years. Hepatoblastoma is the highest relative incidence tumor in the first year of life and with brain tumours has the worst prognosis (50 and 40 per 100, respectively). The disease-free survival rate is most than 80 per 100 in neuro and nephroblastoma with medical and surgical treatment. Familiar incidence in Wilm's tumor is 42 per 100 and chemotherapy side effects are 23 per 100. Familiar incidence in neuroblastoma is 20 per 100 and chemotherapy side effects 21 per 100. In conclusion, in children with malignancies under one year of age have good prognosis (75 per 100 survival at five years), with lower surgical complications rate. Early diagnosis and response to chemotherapy, the side effects of which are considerable and should be strictly controlled, are key factors in the better prognosis and increased life expectancy in this group of patients.
[Anomalous course of a lipomeningocele].
A 9 months old male with a lipomeningocele developed progressive flaccid paraparesis with loss of deep tendon reflexes and sensation to pink prick on the lower extremities. The clinical picture was secondary to a tethered spinal cord syndrome associated to the lipomeningocele. Surgical untethering of the spinal cord was followed by clinical recovery over a 3 months period.
[The origin of malformations].
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[Children throughout art history].
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[The prevention and prenatal diagnosis of neural tube defects].
The AA. provide with the schemes for the study of Neural Tube Defects (D.T.N.) yet the continuation and the prevention of NTD'S clinical cases. Also, the AA. show a protocol of action. It is indispensable for all the Spina Bifida Sections to work together by means of an Orthogenesis and Familiar Planning Unit.
[Embryonal fatty tumors. Lipoblastoma-lipoblastomatosis].
The authors present four cases. The first case is a diaphragmatic-mediastinal, the second one is a cervical-mediastinal one, the third case has a buttock, the last one is a case of hip-thigh. Their ages are one, eight and three years old and the fourth case is seven days old. They appear in axilla, neck, thoracic wall and fat prevertebral tissue, in below three year old children. Despite their benignity, the tumors grow very fast. The complete extirpation is fundamental to avoid relapses, which are presented approximately in a 15 for 100 of the cases. Mutilations are not necessary, since it is a benign tumor. Finally, the authors amply review the literature. They differentiate the concepts of lipoma, hibernoma and lipoblastoma.
[The reality of pediatric oncology].
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[Surgical treatment in tumors of the anterior mediastinum].
Anterior mediastinal tumours (A.M.T.) suppose a diagnostic problem owing to nonspecific clinical signs and symptoms. In the last twenty years, we have seen ten patients with A.M.T., 50 per 100 of which were fortuitous radiologic findings and the remaining 50 per 100 were diagnosed from nonspecific symptoms. Diagnostic imaging techniques were used to classify the anterior location of the tumours. Posterolateral thoracotomy was performed in the first six patients and sternotomy in the remaining four. In conclusion, sternotomy provides a wider surgical field and is to be recommended in A.M.T. surgery. Low morbidity and mortality support an early decision for thoracic surgery.
[Lipomeningocele: review of 44 cases].
On a review of 500 spina bifidas, the authors presents 44 lypomeningoceles (8.8%). During de first two years of age a clinical follow-up with a T.A.C. and myelography is performed. Depending on the symptoms the surgical intervention is performed after two years of age. The authors described the surgical technique. Urological, gastroenterological and orthopedics sequelae are presented and reviewed.
[The publications of Cirugía Pediátrica].
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[Primary cervical tumors of the sympathetic nervous system. Report of 8 cases].
The authors present his experience in the treatment of 8 patients with primary tumors of cervical sympathetic ganglions. The presence of a hard, painless, adherent and slow evolution latero-cervical mass is the first sign in 7 cases. The other one, a neuroblastoma, had a Horner's syndrome. The authors expose the methods of diagnostic and staging, some characteristics of surgical technique, chemotherapy treatment and follow-up methods (TAC, Gammagraphy with i-MIBG and Tumoral Markers). This imply a survival in the 100% of cases.