ABSTRACT
Paediatric plastic bronchitis (PB) is a rare disease characterized by the presence or expectoration of branching airway casts usually in children with cardiac conditions and allergy. It is thought to be due to obstruction or altered drainage of the lymphatics in those with cardiac conditions. Obstruction can be diffuse and thus fatal if untreated. Less than 600 cases have been described in literature and just one in our region in a patient with sickle cell disease. We present a case of a 7-year-old female with acute symptoms suggestive of a lower respiratory tract infection and left hemithorax opacification and bronchial casts on bronchoscopy.
ABSTRACT
Severe Combined Immunodeficiency (SCID) involves the lymphocyte lineage and mimics Human Immunodeficiency Virus (HIV) disease common in our region, making it difficult to diagnose and manage effectively. SCID in East Africa stands underdiagnosed because of lack of awareness and diagnostic resources. A case series of three SCID patients admitted to a Tertiary Paediatric Centre in Kenya between 2016 and 2019. The clinical presentations, laboratory findings, management and outcome for each were studied. Three cases were diagnosed between the ages of 4 to 15 months. Two of them were male and one was a female. All had a history of previous sibling death. There was no parental consanguinity. All presented with pneumonia. One of them had vaccine acquired Rotavirus infection and a persistent generalised maculopapular rash. The T, B cell profile was T- B- in two and T- B+ in one case and the immunoglobulins were reduced in all. All the cases were fatal. Thus, Primary immunodeficiency disorders are prevalent in East Africa. A proper clinical history, examination and laboratory tests like a haemogram, peripheral blood film can aid to suspect and diagnose SCID even with limited resources.
