ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

Thrombotic thrombocytopenic purpura (TTP) is a severe multisystem disorder characterized by microangiopathic hemolysis, central nervous system and renal dysfunction, and a very poor prognosis. Recently, however, plasma exchange or infusion therapy has proven effective in the majority of patients with TTP. We report a patient who developed TTP several years after splencctomy for hereditary spherocytosis. Despite aggressive therapy with plasmapheresis (PP). plasma infusion, antiplatelet drugs, and corticosteroids, the patient had progression of TTP that eventually resulted in his death. The occurrence of TTP in an asplenic patient with an intrinsic red cell disorder, a previously unreported association, may predict a poor prognosis.

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

To assess the neuroendocrine function of long-term survivors of childhood hematologic malignancies. 10 patients who had acute lymphocytic leukemia and two who had non-Hodgkins lymphoma (NHL) (mean age 13.5 ± 1 year) were studied, who were treated with similar chemotherapeutic regimens with or without 2,400 rads of prophylactic cranial irradiation. Pharmacologic growth hormone (GH) stimulation tests and three graded doses of the GH-releasing hormone (1–40-OH-GRH, 0.1, 0.3, and 1 μg/kg) were administered. Venous sampling for GH and gonadotropin determinations was done at 20-min intervals for 24 h, and a new computerized pulse detection algorithm was used to analyze pulses. All the patients who had neuroendocrine abnormalities were in the cranially irradiated group. Two of the 12 patients were GH deficient, and had abnormal 24-h secretory profiles, blunted GH responses to pharmacologic stimuli, and minimal responses to the three doses of GRH. The pulsatile properties of luteinizing hormone (LH) were normal in 10 of the 12 nongonadally irradiated patients, irrespective of previous cranial irradiation and pubertal stage, when compared with available normative data. We conclude (a) that GH deficiency is a common finding among children who receive 2,400 rads of cranial irradiation, and that 24-h GH secretory profiles and standard pharmacologic GH stimulation tests are sensitive indicators of the dysfunction; (b) that treatment with GH should be reserved for children with GH deficiency, documented either by pharmacologic stimuli or by 24-h studies, and should not be used in every short child that received relatively modest doses of cranial irradiation; (c) that in our two GH-deficient patients, the dysfunction may not be hypothalamic, and (d) that the prepubertal gonadotrophs are relatively resistant to the biologic effects of modest doses (2,400 rads) of ionizing radiation.

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

Diaziquone (aziridinylbenzoquinone, AZQ) was given by 30-min infusion at 25 mg/m2/day on a daily x 5 schedule to 16 children with acute lymphoblastic leukemia (ALL) in bone marrow relapse. 16 children with acute nonlym-phocytic leukemia (ANLL) in bone marrow relapse, and I child with chronic myelocytic leukemia in blast crisis. None of the children achieved bone marrow remission. Five children (four with ALL and one with ANLL) were also evaluable for the response of central nervous system leukemia; all had a significant reduction in the cerebro-spinal fluid blast count. Mild transient transaminase elevation was commonly seen. Grade 3 and 4 hyperbiliru-binemia was seen in association with sepsis. AZQ was ineffective for induction of bone marrow remission as utilized in this study.

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

Hereditary hemochromatosis is the most common cause of iron overload in adults and is probably the second most common cause of iron overload in children in the United States next to transfusional overload. Serious morbidity from this disorder of iron absorption can occur in early as well as in middle and advanced age, iron overload having been reported in children with hereditary hemochromatosis as early as 2 years of age. Younger persons differ from older persons in that the risk for iron loading in females appears to be equal to the risk for males, in contrast to a preponderance of males among older patients. Also, younger patients frequently demonstrate cardiac and gonadal involvement, with cardiac failure commonly leading to death, whereas older patients are more likely to have liver involvement and diabetes mellitus, with liver failure and hepatoma commonly leading to death. Because early diagnosis and treatment can prevent the toxicities of iron overload, appropriate screening can be lifesaving. Transferrin saturation is the most reliable screening test. Liver biopsy with objective measurement of hepatic iron stores is the most important diagnostic criterion at present, although reliable noninvasive methods for quantitating body iron are being developed. Young individuals who should be screened for iron overload include patients with cardiac myopathies, hypo-gonadism, amenorrhea, loss of libido, diabetes mellitus. other endocrine disorders, cirrhosis of the liver, and arthritis, as well as the siblings, parents, and children of patients with hereditary hemochromatosis or iron loading of unknown cause.

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

A 7-week-old infant with methemoglobinemia, hemolytic anemia, and inadequate weight gain was found to have aCampylobacter jejunigastrointestinal tract infection. Known etiologies of methemoglobinemia such as oxidative drug exposure, deficiency of NADH-methemoglobin reductase, and hemoglobin M disorder were excluded. The patient had a twin brother (probably identical) who had neither methemoglobinemia nor stool cultures positive forC. jejuni.The twin essentially served as an experimental control, making other environmental or genetic causes, of methemoglobinemia unlikely in the patient. Both the methemoglobinemia and theC. jejuniinfection responded to adequate treatment with erythromycin. The association of aC. jejuniinfection with methemoglobinemia is discussed in light of previous associations of enteritis and methemoglobinemia in infants.

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

Serum ferritin is known to be one of the tumor markers for neuroblastoma. Serum ferritin is elevated in most children with neuroblastoma who are in stages III or IV, but it is not elevated in those in stage I or II. It has also been observed that iron load caused by blood transfusion shows a greater effect on serum ferritin levels than tumor activity due to neuroblastoma. Thus, serum ferritin increases in a linear fashion in children given repeated blood transfusions but the levels increase exponentially in children who have bulky neuroblastomas. Thus, serum ferritin values must be interpreted with caution in patients with advanced stages of neuroblastoma who inevitably require blood transfusions. The authors propose that the pretransfusion and posttransfusion serum ferritin values be in comparison with other tumor markers such as urinary VMA, urinary HVA, and neuron specific enolase. The serum ferritin level should not be used as a sole indicator of tumor activity in neuroblastoma.

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

The Children's Cancer Study Group's (CCG) clinical trials in acute lymphoblastic leukemia (ALL) prior to 1981 consistently demonstrated that patients presenting with a white blood cell count (WBC) >50,000/μl or the “lymphoma syndrome” had a <40% 3-year event-free survival (EFS). The Berlin Frankfurt Munster (BFM) 76/79 study suggested that the prognosis of these patients could be improved. Before testing this therapy in a randomized setting, 29 CCG institutions used it for treatment of 209 newly diagnosed children with ALL and an initial WBC >50,000/μl or the lymphoma syndrome. In the intensive phases of therapy, 77% of cumulative parenteral doses and 55% of cumulative oral doses were within 10% of protocol requirements or were modified appropriately for reported toxicity. One hundred ninety-five patients achieved remission (93.3%). Eleven patients died in remission (5.6%)—10 during the intensive reinduction/reconsolidation phase. The 4-year EFS (±1 SD) was 62% (±3.7%) with a median follow-up of 40 months. Only one patient has had an isolated CNS relapse. These results appear superior to past CCG studies for high-risk patients and extend observations made from studies of similar therapy.

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

The syndrome of thrombocytopenia with absent radii (TAR) is a rare congenital syndrome in which the characteristic hematological findings are hypomegakaryocytic thrombocytopenia, periodic leukemoid reactions, and eosinophilia. The thrombocytopenia usually presents at birth or during the neonatal period and may be profound, accounting for significant morbidity. However, resolution of the thrombocytopenia usually occurs after the first year. In this review of 100 cases, the clinical manifestations, pathophysiology, genetics, and treatment are discussed in detail. With the increased understanding of the natural history and pathophysiology of the hypomegakaryocytic thrombocytopenia in the TAR syndrome, in conjunction with the advances in supportive therapy discussed, the outlook for patients with this syndrome has become quite good.

ISSN:0192-8562    

出版商:OVID    

年代:1988

数据来源: OVID