Successful treatment with prothrombin complex concentrate of postoperative bleeding in a haemophiliac with a factor VIII inhibitor.

Abstract

thyroid, and increased lipofuscin deposition. Increasing use of minocycline could produce additional examples.Pigmentation of the thyroid need not cause functional upset. However, nuclear pyknosis indicates epithelial damage, and the studies of Benitz et al showed that minocycline has an antithyroid effect. Patients on long-term minocycline should have their thyroid function monitored.1 Benitz, K F, et al, Toxicology and Applied Pharmacology, 1967, 11, 150. 2 Borel, D M, and Reddy, J K, Archives of Pathology, 1973, 96, 269. 3 Ryan, G B, et al, Brain, 1970, 93, 617. 4 Without treatment the mortality from neonatal thyrotoxicosis is 120.' In the case reported here the fetus was recognised as being at risk when the mother was five months pregnant. Carbimazole was given to the mother in an effort to control the fetal thyroid. Case historyA 29-year-old woman had a partial thyroidectomy at the age of 11 for Graves's disease. At 16 she became hypothyroid and was started on thyroxine 0-3 mg daily.When she was 21 she aborted a macerated female fetus at 28 weeks. She had not had any antenatal care. The fetus weighed 1868 g, and at necropsy a goitre and an enlarged left ventricle of the heart were noted. At the age of 23 she gave birth to a 36-week boy weighing 2160 g and 43 cm long. During the first day he developed exophthalmos. The second day his heart rate was 200/min and his spleen became palpable. Treatment was started with carbimazole, Lugol's solution, hydrocortisone, and digoxin but he died four days after birth. His weight at death was 1760 g. Necropsy showed slight jaundice, an enlarged (7-5 g) and slightly nodular thyroid, and increased heart weight (25 g), with dilatation of both ventricles.The patient was first seen at this hospital in July 1973 when she was five months pregnant. She was euthyroid but had bilateral exophthalmos and diplopia. As she was likely to have another affected baby she was given carbimazole 5 mg three times a day and her dose of thyroxine was increased to 0-4 mg daily. On this regimen she felt well during the rest of her pregnancy and remained euthyroid. At 37 weeks she was delivered of a healthy girl. The infant's heart rate was 140/min just before delivery and 130/min immediately afterwards. The Apgar score was 10 at one minute. She weighed 3420 g, was 52-1 cm long, and her head circumference was 34-3 cm. Cord blood hormone levels are shown in the table.The child was placed in the special baby care unit. The day after delivery she developed a tachycardia of 140-150/min, was noted to be feeding voraciously and sweating, and her eyes appeared prominent. Thyroid and spleen were palpable. Treatment with carbimazole 0 75 mg three times daily was initiated and continued in reducing doses for three weeks, by which time her heart rate had fallen to 120/min and her weight had risen to 3810 g. Conversion: SI to traditional units-Thyroxine: 1 nmol/1 0 08 tug/100 ml. Triiodothyronine: 1 nmol/1 z 0-7 ng/ml. was found, but the concentration of LATS protector (27 U/ml) was consistent with tha...

Factor VIII Inhibitors in Mild and Moderate-severity Haemophilia A

“…Although previous reports have emphasised the low-titre and transient nature of the inhibitors described (7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17), our survey has detected high-level inhibitors two thirds of which disappeared spontaneously, or after immune-tolerance induction after a median of 12 months, whilst the remainder persisted after a median 99 months of follow-up. Neither persistence of the inhibitor nor inhibitor titre were predicted by the factor VIII genotype, family members being dissimilar in these respects.…”

“…Inhibitors also arise in patients with mild or moderate severity haemophilia A but are uncommon. The prevalence of inhibitors in this group has been estimated to be between 3 and 13%, (1,5,6) but very few have been fully described in the literature (7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). The inhibitors reported commonly arose following intensive replacement therapy and were usually reported to be transient and of low titre (8,9,11,12,(14)(15)(16)(17).…”

Inhibitors to Factor VIII in a Family with Mild Hemophilia: Molecular Characterization and Response to Factor VIII and Desmopressin

“…A recent study (1) which evaluated prospectively the development of inhibitors in 81 previously untreated hemophiliacs infused with recombinant FVIII showed that whereas the prevalence of inhibitors was 28% in 49 patients with severe hemophilia A and 13% in 15 moderate hemophiliacs, no inhibitor developed among 17 patients with mild hemophilia (FVIII greater than 5 U/dl). In the past inhibitors have been reported in 10 patients with mild hemophilia (2)(3)(4)(5)(6)(7)(8)(9)(10)(11) and in all but two cases (5,8) they were of low titer and transient, usually detected following intensive FVIII replacement therapy. In other cases the onset of inhibitor causes a fall of plasma FVIII levels, a concomitant increased frequency and severity of hemorrhages (11) and an anamnestic increase in titer following replacement therapy.…”

Failure of factor VIII inhibitor bypassing activity (Feiba) to secure haemostasis in haemophilic patients with antibodies.

“…Preparations purposely made to contain 'activated' factors have also been produced, for example, factor VIII inhibitor bypassing activity (Feiba, Immuno, Austria), and it has been claimed that these also exert a therapeutic effect even in the case of external bleeding (Preston Received for publication 12 April 197812 April et al, 1977. Recently, it has been claimed that nonactivated or 'routine' factor IX preparations (eg, Konyne, Cutter; Proplex, Hyland) may also be effective (Abildgaard et al, 1976;Kelly and Penner, 1976;Lowe et al, 1976). In contrast to these reports Pollock and Lewis (1976) found no benefit from using these products in one patient.…”