Recognition of this disease and differentiation from juvenile idiopathic arthritis is important to avoid unnecessary investigations and treatment. Follow-up of renal function is indicated.
Haemophilia has an average incidence of about 1 in 5000 live male births [1]. The age-adjusted prevalence is 13.4 cases/100 000 males. Haemophilia A counts for 79%, haemophilia B counts for 21%.There are no guidelines for the treatment of haemophilia in premature born infants. Treatment is merely based on experience with older children and case reports, of which there are only two in the literature [1,2].We describe the management and outcome of haemophilia in three premature infants.Two twin boys were born at 33 weeks gestation. Mother was a known carrier of haemophilia A. Both boys had a factor VIII (FVIII) activity of 2% and received (50 IU kg )1 ) FVIII (rFVIII, Advate Ò ) once per day, from 7 h after birth, because of prematurity and the associated increased risk of an intracranial haemorrhage (ICH).In the first boy, FVIII suppletion was increased to 50 IU kg )1 twice a day for 5 days during a sepsis period because of the risk of developing sepsisrelated disseminated intravascular coagulation (DIC). Coagulation studies then showed a peak plasma FVIII level of 180% and normal values of activated partial thromboplastin time (APTT), prothrombin time (PT) and platelet count. Fibrinogen was elevated at 5.4 g L )1 (normal 1.25-3.00 g L )1 ) and D-dimers were elevated at 1640 ng mL )1 (normal <200 ng mL )1 ). On day 12, the frequency of rFVIII administration was decreased to 50 IE kg )1 once a day. This was continued until removal of all venous catheters on day 17. No further bleeding complications occurred. A cerebral ultrasound on day 23 showed no signs of ICH. On day 28 the patient was discharged. Plasma sampling showed trough levels of 25% and 103% on days 2 and 11, respectively. Peak plasma FVIII levels were 180% (day 9) and 170% (day 11). FVIII recovery was 94%. At age 18 months no FVIII inhibitors were demonstrated in the plasma.In the second boy rFVIII administration was raised to twice a day 50 IU kg )1 because of suspected sepsis on day 4. On day 8 he became very ill due to enterobacter cloacae sepsis with a pulmonary and intracranial haemorrhage. Because of rapid progression of the sepsis no further blood investigations were obtained. The last measured FVIII level was 81%, 2½ h after factor administration on that day. He died on day 9 from respiratory and circulatory failure.The third male infant was born at 31 weeks gestation and presented with prolonged bleeding after a scalp pH-measurement before birth. There was an anaemia with haemoglobin declining from 7.0 to 3.5 mmol L )1 , 7 h after birth. Platelet counts were 54 · 10 9 L )1 , APTT was 156 s (normal <90 s) and PT was 29 s (normal 11-18 s). DIC was present with decreased fibrinogen (0.86 g L )1 ) and elevated D-dimers (1940 ng mL )1 ). Packed cells and fresh frozen plasma were administered. Coagulation studies performed 16 h after birth because of persistent heavy bleeding from the skull lesion, showed a factor IX (FIX) activity of 6% (normal 5-45%). This was relatively low compared with the measured values of coagulation factors II, V and VIII th...
We present a case study of a newborn girl with a left-sided congenital diaphragmatic hernia and a myocardial infarction (MI). The occurrence of MI in newborns has been associated with cardiac malformations and abnormalities of the coronary arteries or thromboembolization. In our patient, echocardiography revealed left ventricular dysfunction, persistent pulmonary hypertension and an inferolateral MI. Within 24 hours cardiac function and oxygenation did not improve and the baby died. Autopsy showed massive MI, a small aortic valve and ascending aorta, and hypoplasia of the distal aortic arch and isthmus. An accessory spleen was also present. The present report underlines the importance of cardiac evaluation in patients with congenital diaphragmatic hernia.
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