Mutilations Due to Medical Disorders in Children

Soft-tissue and bone necrosis, although rare in childhood, occasionally occur in the course of infectious diseases, either viral or bacterial, and seem to be the result of hypoperfusion on a background of disseminated intravascular coagulation. Treatment consists in correction of septic shock and control of necrosis. Necrosis, once started, shows extraordinarily rapid evolution, leading to soft-tissue and bone destruction and resulting in anatomic, functional, psychological, and social handicaps. Ten mutilated children were treated from January 1986 to January 1999 in Hospital de Dona Estefaˆ nia, Lisbon, Portugal. One was recovering from hemolytic-uremic syndrome with a severe combined immunodeficiency, another malnourished, anemic child had malaria, and three had chicken pox (in one case complicated by meningococcal septicemia). There were three cases of meningococcal and two of pyocyanic septicemia (one in a burned child and one in a patient with infectious mononucleosis). The lower limbs (knee,leg, foot) were involved in five cases, the face (ear, nose, lip) in four, the perineum in three, the pelvis (inguinal region, iliac crest) in two, the axilla in one, and the upper limb (radius, hand) in two. Primary prevention is based on early recognition of risk factors and timely correction. Secondary prevention consists of immediate etiologic and thrombolytic treatment to restrict the area of necrosis. Tertiary prevention relies on adequate rehabilitation with physiotherapy and secondary operations to obtain the best possible functional and esthetic result.

Síndrome Hemolítico-Urémico

Os síndromes de mieroangiopatia trombótica - Púrpura Trombótica Trombocitopénica (PTT) e Síndrome Hemolftico Urémico (SHU) - são caracterizados por anemia hemolftica microan giopática, trombocitopénia, alterações da função renal, febre e anomalias do sistema nervoso central. Actualmente são considerados como dois extremos de um espectro contínuo designado PTT- SHU. São doenças raras com uma taxa de mortalidade elevada, apesar dos avanços na terapêutica. Os autores descrevem um caso de síndrome hemolítico urémico num adulto jovem em que o curso inicial e a primeira biópsia sugeriam bom prognóstico. Contudo a recaída precoce e o aparecimento de hipertensão arterial grave implicaram um desfecho fatal em 6 meses. A este propósito os autores fazem uma revisão de alguns dos aspectos mais recentes da patogénese e tratamento deste síndrome.

Warm Antibody Autoimmune Haemolytic Anaemia Associated with Ovarian Teratoma

The ovarian cystic teratoma is a rare cause of autoimmune haemolytic anaemia by warm antibodies, resistant to corticotherapy, with few case reports published in the medical literature. We present a case of a 45-year-old woman admitted to hospital due to general weakness. Laboratory studies revealed macrocytic anaemia, biochemical parameters of haemolysis and peripheral spherocytosis. The direct Coombs test was positive. Viral serologies, anti-nuclear antibodies, anti-double-stranded DNA antibodies and β2-microglobulin were negative. CT scan of the thorax, abdomen and pelvis showed a heterogeneous right anexial lesion. The patient was treated with corticotherapy without improvement of anaemia. Regression of extra-vascular haemolysis and normalisation of haemoglobin was obtained only after laparoscopic splenectomy and right ooforectomy, and the histopathology of the right anexial mass revealed a cystic teratoma. Previously published cases controlled the haemolysis by surgically removing the lesion associated with splenectomy.