The Bleeding Child; Is It NAI? a E Thomas
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1163 PERSONAL PRACTICE Arch Dis Child: first published as 10.1136/adc.2003.034538 on 19 November 2004. Downloaded from The bleeding child; is it NAI? A E Thomas ............................................................................................................................... Arch Dis Child 2004;89:1163–1167. doi: 10.1136/adc.2003.034538 As a paediatric haematologist, the question of whether a Presenting complaint The history of the presenting complaint will child has been abused or whether they might have a include questions as to how the injury occurred bleeding diathesis is a question that I am regularly asked. and an assessment made as to whether this When I first became a consultant, I would often find that not explains the injuries or bleeding seen. Guidance is given that abuse should be suspected if there is enough information was available; for example, significant bruising or bleeding with no history incomplete histories had been taken or investigations were of trauma or a history inconsistent with the incomplete and difficult to interpret. This inevitably led to severity of the injury,3 but in a child with a bleeding diathesis, this may be precisely how the delays in confirming the cause of the bleeding and meant child presents. Where a child has bruising in a that if parents or carers contested a diagnosis of abuse, recognisable pattern such as a belt or hand, then excluding a bleeding disorder was extremely difficult. I was suspected abuse must be reported regardless of the results of laboratory tests.4 However, finger- also aware that carers of several of my patients with tip bruising can be seen in children with a haemophilia or other bleeding disorders had initially been bleeding diathesis from normal physical interac- under suspicion of abuse, most usually at the time of the tion. A drug history should also be elicited, particularly one of anticoagulant use. first few presentations. By highlighting important questions in history taking, having a specific haematological screen Past history Past history of bleeding and haemostatic chal- for children being investigated for bleeding in the context lenges add important information and specific of non-accidental injury, and encouraging discussion of questions should be asked about birth; for abnormal results with a haematologist, these difficulties example, whether there was a cephalhaema- toma, unexpected bleeding from the umbilical can, for the most part, be avoided. stump, or bruising after intramuscular injections. ........................................................................... Active children usually have bruises on their shins, but not normally on unexposed areas. Persistent mucocutaneous bleeding such as gum bleeding or epistaxis (often bilateral) in addition s doctors who care for children, we have a to bruising might indicate thrombocytopenia, a responsibility to be aware of signs and platelet disorder, or von Willebrand disease. http://adc.bmj.com/ Asymptoms suggestive of child abuse, including non-accidental injury. Equally how- Significant haemostatic challenges ever, we must also recognise that medical and Significant haemostatic challenges will include physical conditions may simulate abuse and that operations such as circumcision, tonsillectomy, appropriate measures need to be taken to rule or removal of teeth. Bleeding response to injury out these conditions. Haemostatic abnormalities such as a bitten tongue or a wound that requires are manifest usually as bruising or other bleed- stitching can yield useful information. In girls, on October 1, 2021 by guest. Protected copyright. ing into the skin or mucosal membranes, and menstrual loss may be an indicator of a bleeding similarly cutaneous lesions are by far the most diathesis. Bleeding may be due to other disease common presenting features of child abuse.1 An states that affect haemostasis such as hepatocel- incorrect diagnosis of child abuse can be devas- lular dysfunction, renal disease, or malabsorp- tating for the family and if a serious underlying tion. blood disorder is later identified, regaining the trust of that family may be very difficult. Delay in Family history diagnosis of a condition such as haemophilia will A family history of bleeding may be apparent and mean that no treatment can be offered which is more often seen in dominantly inherited or X- may lead to increased morbidity and even linked conditions such as haemophilia A or B. However, haemophilia A, the commonest type of ....................... mortality. It is important to remember, however, haemophilia, arises as a result of spontaneous that diagnosis of a bleeding disorder does not Correspondence to: mutation in about 30% of cases and therefore rule out abuse2 and that these children will be at Dr A E Thomas, family history will be lacking.5 The sex of the Department of greater risk of bleeding secondary to abuse. Haematology, Royal patient and the age also help to distinguish Hospital for Sick Children, between possible causes of bleeding. X-linked Edinburgh EH9 1LF, UK; CLINICAL HISTORY AND PRESENTATION disorders such as haemophilia A and B usually [email protected]. When a child presents with bruising or bleeding, occur in males. Such disorders may occur in girls nhs.uk the main differential diagnoses are physiological but unless consanguinity or Turner’s syndrome is Accepted 6 April 2004 or accidental bleeding, non-accidental injury, or present, the chances are very small. Extreme ....................... a bleeding diathesis. Lyonisation of the X chromosome can also result www.archdischild.com 1164 Thomas in girls being clinically affected; therefore, although such commoner causes of bleeding and to exclude or confirm some Arch Dis Child: first published as 10.1136/adc.2003.034538 on 19 November 2004. Downloaded from diagnoses are unlikely, they should be tested for as part of a of the rarer causes for the safety and management of the full evaluation. The age at presentation influences the child. Further investigation might be needed if no explana- likelihood of a particular cause of a bleeding diathesis. A tion of the bleeding is found or no admission of non- patient with a severe congenital bleeding diathesis is unlikely accidental injury is made. to present for the first time as an adolescent. It must also be remembered that the plasma concentration of many of the Venepuncture; reducing investigation artefact coagulation and fibrinolytic proteins are age dependent and A set of screening investigations should be performed on therefore appropriate normal ranges must be used for blood taken from a single venepuncture, and analysed interpretation. Ideally the laboratory should establish normal without delay by laboratories experienced in handling small ranges for age using their reagents and methods for at least samples. Artefact can significantly distort results and lead to the more common parameters measured. Details of ethnic misdiagnosis. The utmost care must be exerted in the way the origin and consanguinity should be taken as certain disorders specimens are taken and handled prior to processing. Ideally, are more frequent in particular groups; for example, factor XI they should be taken by a person experienced in paediatric deficiency in those of Ashkenazy Jewish descent6 and phlebotomy, with the minimum of venous stasis and during recessive disorders are more common in consanguineous core working hours. families. Pitfalls in specimen collection Examination of the child Common pitfalls in specimen collection and processing When examining the child, the general health and state of include poor venepuncture technique, whereby there is the child should be assessed. contamination by tissue fluid or air bubbles leading to activation of the sample in vitro which can result in both Bruises prolongation or shortening of clotting times as well as If the child is presenting with bruising, particular note thrombocytopenia. If blood is taken from a cannula which should be made of the distribution and size of the bruises. has been heparinised, heparin contamination can occur, The age of a bruise is very difficult to ascertain with certainty resulting in prolongation of the activated partial thrombo- and depends on the integrity of the coagulation system plastin time (aPTT). Over or under filling of the specimen and vessels and the force and location of the injury.7 tube will result in an altered ratio of the anticoagulant Accompanying tissue swelling and abrasion may be present (citrate) to plasma, giving shortened or prolonged clotting in more recent bruises. Bruises of different ages are seen both times respectively. Prolonged clotting times may also be seen in abuse and in children with a bleeding diathesis. The in severe polycythaemia where there is a reduced plasma pattern of bruising should be recorded, in particular if marks volume. Inappropriate storage or transport of the specimen indicate use of an object such as a belt or flex. As has been may result in activation of the sample or loss of factor noted above, fingertip bruising is not infrequently found in activity. children with a bleeding diathesis. The distribution of bleeding may indicate a diagnosis of Henoch-Scho¨nlein First line investigations purpura, which presents as symmetrical bruising on extensor Initial tests should include a coagulation screen consisting of surfaces. It is due to a vasculitis rather than a coagulopathy, a prothrombin time (PT), activated partial thromboplastin giving a normal coagulation screen and