Mucopolysaccharidosis type I (MPS I H, Hurler syndrome) is a rare autosomal recessive inborn deficiency in the metabolism of glycosaminoglycans (GAGs) heparan sulfate and dermatan sulfate, resulting from deficiency of Alpha-L-iduronidase enzyme. This condition is characterized by accumulation of incompletely degraded glycosaminoglycans into various organs of body, which leads to impairment of organs and body functions. Such children appear nearly normal at birth; however, if left untreated, show a progressive mental and physical deterioration leading to death due to cardiorespiratory failure before the second decade of life. Pedodontists have a role for early diagnosis, rendering corrective and preventive treatment to the developing dentition, and referring the patient to the concerned specialities. An interesting case of a seven year old boy with a combination of skeletal, neurological, ophthalmologic, oro-dental and radiological findings of this diverse and devastating clinical entity with MPS I-(Hurler syndrome) has been presented here in this case report.

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Surgical closure of Atrial Septal Defects (ASD) and Patent Ductus Arteriosus (PDA) can be performed successfully with low mortality. However, the morbidity associated with general anaesthesia, thoracotomy, cardiopulmonary bypass, postoperative monitoring in the intensive care unit, several days of hospital stay and the requirement of blood products is considerable. The expense associated with this morbidity, operative scar and the psychologic trauma to the patient and parents are additional disadvantages of surgery. Hence, the closure of these defects by transcatheter methods with various devices has been evaluated worldwide. We report the initial experience at our centre with closure of secundum ASDs and large PDAs with the Amplatzer Septal Occluder and Amplatzer Duct Occluder.

A 32-year-old male patient, a case of critical calcific mitral stenosis (following closed mitral valvotomy in 1989) was admitted for mitral valve replacement in September 2001. In hospital, he developed cardiogenic shock, pulmonary oedema and oliguria precluding surgery. An emergency percutaneous transatrial balloon mitral commissurotomy as a life-saving procedure in a valve with unfavourable morphology and 'balloon impasse' is discussed.

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