Factor x deficiency: an unusual cause of spontaneous intracranial bleeding.
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Biomedical subjects
Publications and source records attributed to N S Menon.
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We studied 19 infants with a mean age of 3.8 months who presented with features consistent with acute lead encephalopathy following the use of traditional medicines. All presented with convulsions; CT scans of the brain on admission showed brain oedema in four, atrophy in four and normal findings in 11. Cerebrospinal fluid analysis in nine patients showed pleocytosis in six and a high protein content in eight. The median lead level in these 19 infants which encephalopathy was 3.6 mumol/l (74.5 micrograms/dl). Seven had a mean lead level of only 2.7 mumol/l (56.9 micrograms/dl) which is much below 70 micrograms/dl, the level usually proposed as the threshold for encephalopathy. Thirteen infants developed brain damage during follow-up; statistical analysis correlated the lead level at 2 months post chelation with an abnormal neurological outcome. Our findings indicate that in very young infants acute lead encephalopathy may occur at lead level lower than previously reported.
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We report on an infant with multiple congenital anomalies, tetralogy of Fallot, and Karyotype 45,X,t(Y;18)(q12;11.2). The infant's anomalies are consistent with a del(18p) syndrome, except for the exceptional severity of the heart defect.
Accidental mebendazole poisoning in an 8-week-old infant and respiratory arrest with tachyarrhythmia associated with continuous seizures is reported. Exchange transfusion was undertaken as a life-saving measure. Mebendazole, like piperazine citrate, has considerable neurotoxicity, especially in infancy, and we propose the use of exchange transfusion as a means of mebendazole elimination in infants.
The primary concern of all involved in the management of radiotherapeutic patients is that the tumor dose delivered to each and every patient in one institution is identical to that delivered anywhere else. Despite the advocacy 1,2,3,4 for the calibration of high energy photon beams at 5 cm depth or beyond in a water phantom to reduce the effect of electron "contamination" from collimators, filters, applicators, etc., the "in-air" method of calibration is still in vogue in many institutions in the United States and elsewhere. The introduction of S.I. units in radiology is likely to create ambiguity in proper conversion factors from exposure to absorbed dose which can be avoided if field instruments are calibrated in terms of absorbed dose in water under specified conditions. The present study shows that the estimated error can be as much as 7% low when the "in-air" method of calibration is used instead of measurements in a large water phantom at 5 cm depth for cobalt-60 photon beams and hence recommends that the procedure for "in-air" calibration for high energy photon beam should be discontinued.
Four neonates with congenital Factor X deficiency presented soon after birth with bleeding episodes. Two of the newborns had intracranial hemorrhages; one of them also had antenatal ventricular dilatation and postnatal hydrocephalus and died of massive intracerebral hemorrhage at four months. One patient was lost for follow up. The two surviving infants were followed up for four years and two years respectively, while on replacement therapy with three injections of 40 units/kg prothrombin complex a month. In spite of markedly elevated prothrombin time and partial thromboplastin time, these two infants remain free of major bleeding manifestations except for troublesome petechiae and ecchymoses. A schedule for substitution therapy with Factor X is proposed for infants and children to prevent bleeding in severe Factor X deficiency.