Primary neurological manifestations of HIV in children.

Human immunodeficiency virus type 1 (HIV-1) causes various diseases in different age groups. Neurological manifestations of HIV are common and add to morbidity and mortality. It was previously thought that the central nervous system (CNS) was involved only in the advanced stages of the disease. However, recent evidence supports pathological involvement of the CNS from initial viral entry. Some of the CNS manifestations in children share similarities to neurologic disorders of HIV-infected adult patients, while others are unique to the pediatric population. Many HIV-related neurologic complications seen in adults are rarely encountered in children with AIDS and vice versa. However, with recent advances in the treatment, more HIV-infected children are surviving into adulthood. A systematic review of the available literature was performed to study the manifestations, causes, outcomes, and treatment of primary neurologic disorders in children with HIV. Online databases (Ovid Medline, Embase and PubMed), websites from the World Health Organization, commercial search engines, including Google, and chapters on HIV in standard textbooks of pediatrics and medicine were reviewed. HIV-associated neurological syndromes can be classified into four types: primary HIV neurological diseases, treatment-related neurological diseases, adverse neurological effects of antiretroviral therapy and secondary/opportunistic neurological illness. These conditions are not mutually exclusive and may co-exist in a given patient. This narrative review will focus mainly on the primary neurological manifestations of HIV in children.

Branchio-oto-renal syndrome presenting with syndrome of hyporeninemic hypoaldosteronism.

Branchio-oto-renal (BOR) syndrome is an autosomal dominant, clinically heterogeneous disorder characterized by branchial arch anomalies, hearing impairment, and renal malformations. We report the case of a 10-year-old boy with BOR syndrome who presented with hyperkalemic hyperchloremic metabolic acidosis due to hyporeninemic hypoaldosteronism. The child also had mental retardation and spastic diplegia which have hitherto not been described in BOR syndrome.

Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner's syndrome.

We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD) with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner's syndrome).