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Biomedical subjects

A Navajas Gutiérrez

Publications and source records attributed to A Navajas Gutiérrez.

At least 19 recordsLinked to original sources
PubMed2002

[Difficulties in the diagnosis of familial hemophagocytic lymphohistiocytosis].

Familial hemophagocytic lymphohistiocytosis (FHL) is characterized by proliferation and non-malignant activation of histiocytes and T lymphocytes in the reticuloendothelial system. Diagnostic guidelines include fever, splenomegaly, cytopenia, hypertriglyceridemia and/or hypofibrinogenemia with hemophagocytosis in the bone marrow, spleen or lymph nodes. In many patients diagnosis is difficult due to the lack of diagnostic criteria, hemophagocytosis, variability of clinical presentation, spontaneous improvement and the absence of a specific marker of the disease. When there is strong clinical suspicion of FHL, chemotherapy and immunosuppressor treatment should be started early to achieve complete cure and should be followed by hematopoietic stem cell transplantation. We present the case of a 2-month-old girl who presented fever, anemia and thrombocytopenia, enlarged liver and spleen, hyperferritinemia, hypertriglyceridemia, and hypertransaminasemia without the finding of hemophagocytosis in bone marrow. Two of the girl's relatives had died of fulminant hepatic failure of unknown etiology. The patient improved spontaneously but presented reactivation of the disease 3 weeks later and died after splenic biopsy.

Fatal Outcome↗
PubMed2000

[Hereditary spherocytosis in neonates. Review of our caseload].

AIM: Review of hereditary spherocytosis diagnosed in infants younger than two months and their follow up. PATIENTS AND METHODS: Retrospective study of 18 infants younger than two months diagnosed from 1973 to 1995. RESULTS: Diagnosis was established in the first week of life in 50% of the patients. Hereditary pattern was autosomic dominant in 94% of the cases. Anaemia was observed in all the patients and hyperbilirubinemia in only 44%, although the latter was the clinical presentation in patients diagnosed at younger age. Exchange transfusion was performed in 3 children (1 with the severe form and 2 with the typical form of the disease). During the first 6 months of age, 55% of infants presented hemolytic crises that required transfusion in 91% of them. Both periodicity of crises and transfusions decreased to 38 and 44% respectively after the first year. Splenectomy was performed in the 3 children with severe forms and in 6 with typical forms (mean age 8 years and 3 months). No cholecystectomy was required so far. CONCLUSIONS: The authors believe that neonatal spherocytosis does not implicate worse prognosis at follow up. Blood support is higher during the first year of life. Elective splenectomy depends on age and transfusional requirements.

Female↗
PubMed1998

[Ovarian tumors in childhood: apropos of a review of cases].

OBJECTIVE: Our objective was to evaluate the incidence and behavior of ovarian tumors in our population. PATIENTS AND METHODS: Between 1984 and 1994, all clinical charts with the diagnosis of ovarian tumors were reviewed retrospectively. Of 158 tumors in females below 14 years of age, 7 were located in the ovary (4.4%). Clinical presentation, diagnostic methods, histology, treatment and survival were evaluated. RESULTS: The mean age of the patients was 11.3 years (range: 7 months-13.5 years). One child was asymptomatic, 4 complained of abdominal pain, 3 with vomiting and 3 with urinary symptoms. Palpable abdominal masses were found in all, with abdominal distension in 2, ascytes, general malaise and precocious puberty each in one case. Alpha-fetoprotein was elevated in 3 cases and CA 125 in 1. Image studies revealed pleural effusion in one X-ray abdominal mass in 6 abdominal X-rays, which was confirmed by echography in all girls. Histology revealed benign teratoma (3), endodermal sinus tumor (1), malignant teratoma (1), granulosa cell tumor (1) and mixed teratoma and dysgerminoma (1). Treatment consisted in surgical resection (7), total in 5 cases (stage I) and subtotal in 2 (one malignant teratoma and one TSE, stages III). Chemotherapy was added initially in 2 stage III tumors and after relapse in 3. Radiotherapy was not given. Six girls survived. CONCLUSIONS: We comment on the low incidence of these tumors, which can be well diagnosed by using echography. Mixed histology may be misdiagnosed. Chemotherapy may rescue the worse prognostic cases.

Adolescent↗
PubMed1997

[Neuroblastoma in children under than 1 year of age].

OBJECTIVE: Our objective was to carry out a prospective multicenter study of neuroblastoma patients diagnosed between 0 and 12 months of age. PATIENTS AND METHODS: Diagnostic procedures included histology, catecholamine excretion, bone marrow cytology and MIBG-scan. Staging was evaluated according to the INSS classification. After 1992, Simada criteria were used and also N-myc amplification, DNA index and P-glycoprotein determinations in tumoral tissue. The surgical technique employed and complications derived from it were also evaluated. The patients were treated according to stage with multicenter Spanish protocols N-I-87 and N-II-92. Overall survival and event free survival were calculated by actuarial methods. RESULTS: Between October 1987 and June 1992, a total of 140 infants less than one year of age were registered and diagnosed of neuroblastoma, representing 40% of all neuroblastoma cases. Median age was 0.3 years and 73% were less than 6 months of age at diagnosis. The most frequent stage was 1 (35%) followed by 4-S (20%). The frequency of unfavorable prognostic factors was the following: LDH (21%), NSE (14%), ferritin (18%), Shimada (7%), DNA (35%), NMA (3%), TrakA (23%), P-glycoprotein (19%). Surgery was performed in 133 children: total resection was reported in 94 and > 90% in another 22 cases. Complications attributed to surgery occurred in 12% of the cases. Chemotherapy was given in 73 cases and radiotherapy in 7. The five year total survival is 91% and the event free survival 88%. Survival by stages: Stage 1 = 91%, stage 2A = 88%, stage 2B = 100%, stage 3 = 84%, stage 4 = 56% and stage 4-S = 100%. CONCLUSIONS: 1) The majority of neuroblastoma cases in infants less than one year old are diagnosed before six months of age. 2) For this age group stages 1 and 4-S are the most frequently observed. 3) Unfavorable biological factors are less frequent than for children over one year of age and are associated with disseminated disease (advanced stage). 4) The outcome is excellent, except for stage 4 patients. The cases in stage 1 and 2 may be treated by surgery alone. Chemotherapy may be of benefit for stage 3 patients.

Age Distribution↗
PubMed1992

[Nutritional study of children with oncologic diseases].

From October 1989 to June 1990 we have evaluated at diagnosis and after six months of treatment the nutritional status of 21 oncologic patients younger than 14 years. At diagnosis, 14% of the children showed slight malnutrition on anthropometric evaluation, although there were biochemical data of protein malnutrition in 47% of the cases. Children with abnormal anthropometric measurements and those with a high risk of becoming malnourished (infants with advanced abdominal diseases) were given dietary supplements. In the follow-up evaluation, 90% of the patients showed normal somatic indexes and 76% showed recuperation in their protein values. The recovery of the nutritional status was most important in the group of patients with Acute Lymphoblastic Leukemia.

Adolescent↗
PubMed1991

[Second solid tumors in childhood. Review based on three cases].

As children with cancer survive longer, the incidence of second malignant neoplasms has increased considerably. We describe here three cases of second solid tumors after 12, 8 and 2 years of initial diagnosis of cancer: one osteosarcoma of left maxilla in a previously treated child with bilateral retinoblastoma, a temporal astrocytoma associated with acute lymphoblastic leukemia and a glioblastoma multiforme in a girl with neurofibromatosis de Von Recklinghausen, after Non Hodgkin lymphoma, respectively. We review the literature about the influence of genetic, immunologic and therapeutic factors involved in the appearance of these second tumors.

Astrocytoma↗
PubMed1990

[Familial hemophagocytic lymphohistiocytosis: survival of a case treated by polychemotherapy].

A case of familial hemophagocytic limphohistiocytosis (FHLH) is presented in a 16 months old infant, with clinical picture of prolonged fever, cutaneous purpura, generalized lymphadenopathy and progressive hepatosplenomegaly and laboratory of severe pancytopenia, hypofibrinogenemia and hypertriglyceridemia, with elevation of the pre-beta fraction in the lipidogram, all this compatible with this disease. She also showed bone marrow and ganglionar biopsy infiltrated by histiocytic cells with hemophagocytosis. The patient received chemotherapy with cycles VP-16, vincristine (VCR) and intrathecal methotrexate (MTX), alternating every two-three weeks with VACP, during one year, with resolution of clinical and laboratory parameters after two months of this treatment without relapses. The survival from diagnosis is 39 months.

Drug Therapy, Combination↗