Sudanese journal of paediatrics
-
Immune thrombocytopenia (ITP) is an acquired hematological disorder that is developed secondary to the production of auto-antibodies against platelets leading to isolated thrombocytopenia, in the absence of other causes of thrombocytopenia such as drugs, infections, malignancy, or other autoimmune diseases [1-6]. ITP commonly affects children between one and seven years of age. Severe life threatening bleeding is rare (0.2-0.9%) [7-12]. ⋯ Initial management options for newly diagnosed childhood ITP include; observation only, the use of intravenous immunoglobulin (IVIG), steroids, anti-D immunoglobulin, each alone or in combination [6, 19.] Children who develop chronic ITP may benefit from splenectomy [19, 20-24]. Rituximab, a chimeric monoclonal antibody (anti-CD20), may lead to complete remission, and defers the need for splenectomy [25-27]. Recently, the thrombopoietin (TPO) agonists (Romiplostim and Eltrombopag) produced very good response in adult and pediatric patients with severe chronic ITP [28-30].
-
Craniopagus conjoined twins represent a rare phenom- enon of congenital malformation/ dysmorphism. The clinical pathology of this complex entity is reviewed and placed in perspective. ⋯ Successful separation of craniopagus twins remains a rarity, however modern neurosurgical tech- niques have created opportunities for successful sepa- ration and brought hope for a normal survival of these children who in the past were often left as historical footnotes or put on display as oddities of nature. We report on a craniopagus conjoined twins from Sudan who had been successfully separated.
-
Case Reports
Chronic immune thrombocytopenia in a child responding only to thrombopoietin receptor agonist.
Immune thrombocytopenia (ITP) is an acquired hematological disease in which the body produces antibodies against its own platelets leading to platelet destruction resulting in isolated thrombocytopenia. Childhood ITP may enter complete remission in the majority of cases within six months from diagnosis. However, 20-30% of affected children may develop chronic ITP (lasting for more than 12 months). ⋯ Here is a case report on an 8 ½ -year-old boy with refractory chronic ITP who failed therapy with IVIG, corticosteroids, splenectomy and Rituximab. He showed excellent response to treatment with TPO receptor agonist (Romiplostim). His platelet count increased from less than 10 x10(3)/dl and maintained between 100x10(3)/dl to 200x10(3)/dl after few weeks of starting Romiplostim therapy.
-
Acute viral bronchiolitis is one of the leading causes of worldwide admission of children under 2 years of age during winter months. There is a lack of consensus regarding the clinical definition of acute viral bronchiolitis in children and hence the management varies across the globe. ⋯ The available evidence in the worldwide literature suggests that supportive and symptomatic management is still the mainstay of management in this condition. The key to reducing the morbidity and mortality in children with RSV bronchiolitis is through prevention of infection through immunoprophylaxis especially in high-risk children.