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I Astigarraga Aguirre

Publications and source records attributed to I Astigarraga Aguirre.

11 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↗
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

[Increased survival rates of children with cystic fibrosis].

Seventy-two patients with cystic fibrosis were under care between January 1st 1972 and December 31st 1988, and 75 until now (July 1989). Fifty are alive, 24 died and 1 was lost to follow-up. The number of alive patients under control at the end of every year rose from 5 in 1972 up to 47 in 1988 and 50 at present. In order to assess the progress of survival rates, we compared two periods: 1972-1980 and 1981-1988. Twenty-eight were under control during the first period and 16 (57%) died, these figures for the second period being 55 and 8 (14%) (p 0.001) respectively. A remarkable increase in cumulative survival rates during the second period was observed at ages 1 (96% vs 68%), 5 (94% vs 55%) and 10 (86% vs 28%). Greater experience and closer follow-up, together with more aggressive treatment are likely to account for the increase in survival. It is vital that reference cystic fibrosis centers should be set-up in our country for the management of so complex a disease as 'cystic fibrosis.

Adolescent↗