Myeloid sarcoma in a child with acute myeloblastic leukaemia

We report a rare occurrence of myeloid sarcoma in a 7 years old child with acute myeloblastic leukaemia [AML - FAB type M[2]]. He presented with fever, generalized weakness, bilateral proptosis and left parotid swelling. CT scan revealed a mass in paranasal sinuses extending into brain and retro-orbital region. Diagnosis of AML M[2] was made on bone marrow aspiration and special stains. Induction therapy for AML was given according to standard protocol. The extramedullary lesion as well as the acute leukaemia went into complete remission

Michelin tire syndrome

We describe a 5-year-old girl who has symmetrical ringed skin folds associated with underlying smooth muscle hamartoma. Cutaneous findings in this child resemble those in other reported cases of Michelin tire syndrome. In addition to the skin changes she has multiple phenotypical anomalies. These include distinctive facial dysmorphia, and genital, ophthalmologic, skeletal and central nervous system anomalies

Study of coeliac disease in children

A study was conducted in Paeds Department of CMH Lahore to collect clinical data related to coeliac disease. Over a period of 12 months [Jan 1994- Dec 1994] 25 [0.21%] cases were diagnosed to be suffering from coeliac disease, out of a total of 11813 patients seen in the out patient department. The revised criteria of European Society of Gastroentrology And Nutrition [ESPGAN] 1990 was followed for diagnosis i.e. a flat mucosa followed by a clinical response to gluten exclusion from the diet. The mean age at the time of diagnosis was 5.5 years and male female ratio was 1.77:1. The major clinical features included failure to thrive, chronic diarrhoea, muscle wasting, anaemia, abdominal distension, anorexia, while one case had hypocalcaemic tetany and another anasarca. All the cases showed total or partial villous atrophy on jejunal biopsy and subsequently responded to gluten exclusion from the diet. Three cases had recurrences of symptoms on follow up due to poor dietary compliance. However, they responded well on a supervised gluten free diet. It is concluded that Coeliac Disease does occur in our population. Jejunal biopsy remains the mainstay of diagnosis. Response to diet therapy is sufficient to confirm the diagnosis