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Clonality in autoimmune diseases 总被引:1,自引:0,他引:1
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Most infants with hemophilia have no bleeding in the neonatal period even if birth trauma occurs. The explanation for this lack of bleeding in the first few days of life in most hemophiliacs is unknown. Maternal factors VIII and IX fail to cross the placenta and cannot, therefore, protect the neonate. There have, however, been an increasing number of reports of severe neonatal bleeding in hemophiliac neonates. Herein, a case of severe neonatal bleeding responsible for hypovolemic shock and disseminated intravascular coagulation masking the hemophilia and delaying its diagnosis is reported. Transfusion of twice the total globular mass and exchange-transfusion were required. Hemorrhagic gastric necrosis occurred, requiring subtotal gastrectomy. The diagnosis of severe hemophilia A (factor VIII = 1%) was established only at 17 days of age. At the age of five months, the child developed a dumping syndrome which improved under appropriate dietary therapy and finally resolved. Outcome was favorable and at the evaluation at two years of age the child was leading a normal life. This case underlines the difficulty of the diagnosis of hemophilia at birth. When there is no family history of bleeding, the diagnosis of hemophilia is usually missed in the neonatal period and established only later or retrospectively. Factors VIII and IX should consequently be measured in male neonates with unusual bleeding and an increased activated partial thromboplastin time, even if disseminated intravascular coagulation is present. Prompt diagnosis and initiation of specific therapy may lessen acute morbidity and prevent long-term sequelae in affected infants. 相似文献
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Y Leskinen JP Salenius T Lehtim?ki H Huhtala H Saha 《American journal of kidney diseases》2002,40(3):472-479
BACKGROUND: Knowledge of the prevalence of peripheral arterial disease (PAD) in patients with chronic renal failure (CRF) is limited because of a lack of uniformity in disease definition and recognition. Furthermore, little is known of the prevalence of medial arterial calcification (MAC) in patients with CRF. Our goal is to study the prevalence of PAD and MAC defined by ankle brachial index (ABI) or toe brachial index (TBI) measurements in a Finnish population of patients with CRF consisting of predialysis and dialysis patients, as well as renal transplant recipients. METHODS: We examined 136 patients with CRF and 59 control subjects. Fifty-nine of the patients with CRF had moderate to severe predialysis CRF, 36 patients were on dialysis treatment, and 41 were renal transplant recipients. Mean age of patients was 51.9 +/- 11.5 years, and 39 patients (29%) had diabetes. ABI and TBI were measured by means of photoplethysmography. The definition of PAD required an ABI value of 0.90 or less, a TBI value of 0.60 or less, or a previous positive lower-extremity angiogram result. ABI values of 1.3 or greater or incompressible arteries at ankle level indicated MAC. The presence of claudication was determined by an interview. RESULTS: Prevalences of PAD on this study were 22.0% in patients with predialysis CRF, 30.6% in patients on dialysis treatment, 14.6% in renal transplant recipients, and 1.7% in the control group (P = 0.001). Prevalences of MAC were 23.7%, 41.7%, 23.1%, and 3.4% (P < 0.001), respectively. Only 9 patients had claudication, and 6 of those patients had PAD. CONCLUSION: Both asymptomatic PAD and MAC are common in patients with CRF. Therefore, we recommend the use of both ABI and TBI measurements in the evaluation of PAD in patients with CRF. 相似文献