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.

Unilateral pulmonary venous atresia: A rare cause of recurrent hemoptysis.

Hemoptysis is a rare but distressing complaint in children. Pulmonary venous atresia (PVA) is a rare cause of recurrent hemoptysis in children. We report a 3-year-old boy with recurrent hemoptysis for 2 years. The child had tachycardia, tachypnea, tender hepatomegaly, and left-sided decreased air entry with crepitations. Coagulation and Koch's workup was negative. X-ray of the chest showed a small left hemithorax. Computed tomography with angiography showed left unilateral PVA with pulmonary artery hypoplasia and dysplastic left lung. The child has been posted for left pneumonectomy. Although uncommon, PVA should be diagnosed early to prevent life-threatening complications such as hemoptysis and pulmonary hypertension. The patient can be managed conservatively or surgically depending on the severity. Early diagnosis and intervention helps in reducing morbidity and mortality.

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).

A Case of Acinetobacter Septic Pulmonary Embolism in an Infant.

Case Characteristics. An 11-month-old girl presented with fever and breathlessness for 5 days. Patient had respiratory distress with bilateral coarse crepitations. Chest radiograph revealed diffuse infiltrations in the right lung with thick walled cavities in mid and lower zone. Computed tomography showed multiple cystic spaces and emboli. Blood culture grew Acinetobacter species. Intervention. Patient was treated with Meropenem and Vancomycin. Outcome. Complete clinical and radiological recovery was seen in child. Message. Blood cultures and CT of the chest are invaluable in the evaluation of a patient with suspected septic pulmonary embolism. With early diagnosis and appropriate antimicrobial therapy, complete recovery can be expected in patients with septic pulmonary embolism.

Distal renal tubular acidosis in a child with HIV infection.

Renal tubular acidosis (RTA) is not uncommon in HIV-infected children with advanced disease, and has been described mainly due to nephrotoxic anti-retroviral therapy and in association with prophylaxis or treatment of Pneumocystis carinii pneumonia with co-trimoxazole. We describe an 8-year-old boy, newly diagnosed to have HIV infection, who presented with distal RTA. There were no features of chronic RTA in the form of rickets or nephrocalcinosis, making an inherited form unlikely.

Neurocysticercosis-induced hemiballismus in a child.

Neurocysticercosis lesions can occur in the basal ganglia, but most of these are clinically silent. Neurocysticercosis manifesting as movement disorders is extremely uncommon. The authors report a case of neurocysticercosis in an 11-year-old girl presenting with right hemiballismus (a clinical manifestation not yet reported). Magnetic resonance imaging of the brain confirmed the solitary neurocysticercosis lesion in the left thalamus. The child was symptomatic for 5 years and improved dramatically within 2 days of starting definitive therapy for neurocysticercosis (albendazole and prednisolone).

Neonatal presentation of a rare metabolic liver disease.

Tyrosinemia is a rare paediatric metabolic liver disorder. A 15-days-old neonate born of a third degree consanguineous marriage presented with jaundice due to tyrosinemia, which progressed to fatal hepatic encephalopathy. The diagnosis was based on very high alpha-fetoprotein level, with urine aminoacidogram revealing tyrosine spot and liver biopsy depicting cirrhosis. Very early neonatal presentation and rapid progression were the unusual features of this case.

Pneumonias de repetição em ambulatório de pneumologia pediátrica: conceito e prevalência / Recurrent penumonia in a pediaatric pulmonology outpatient unit: concept and prevalence

Objetivo: Determinar a prevalência de pneumonias de repetição na demanda de consultas de primeira vez encaminhadas ao ambulatório de Penumologia Pediátrica do Instituto de Puericultura e Pediaatria Martagão Gesteira da UFRJ para esclarecimentos diagnóstico e revisar o conceito de pneumonias de repetição na literatura pediátrica. Método: Os dados foram obtidos através da avaliação de consultas desse tipo no período de 01/01/95 a 30/04/97. Resultados: De um total de 638 consultas, 101 foram encaminhadas com o diagnóstico presuntivo de pneumonias de repetição. Em apenas 39,6 por cento, o motivo do encaminhamento coincidiu com o conceito de pneumonias de repetição adotado pelo Serviço. Conclusão; Os autores concluíram que o conceito de pneumonias de repetição deve ser melhor esclarecido e difundido entre os médicos pediatras, pois cabe a eles a decisão de encaminhar ao especialista os pacientes com essa queixa. Cabe ressaltar a valorização do exame radiológico normal entre os episódis agudos para a caracterização do quadro de pnemonias de repetição, visando melhor determinação dos pacientes que posteriormente necessitarão de encaminhamento ao especialista para prosseguimento da investigação diagnóstica

[Recurrent pneumonia in a pediatric pulmonology outpatient unit: concept and prevalence] / Pneumonias de repetição em ambulatório de pneumologia pediátrica: conceito e prevalência.

OBJECTIVE: This study aimed to determine the prevalence of recurrent episodes of pneumonia in patients referred to the pulmonology outpatient unit at "Serviço de Pneumologia Pediatrica" of the Instituto de Puericultura e Pediatria Martagão Gesteira/UFRJ and to review the recurrent concept in accordance to medical references currently available. METHODS: Data were obtained by reviewing all documented initial appointments from January 1st, 1995 up to April 30th, 1997. RESULTS: One hundred and one visits out of six hundred and thirty eight appointments with suspected diagnosis of recurrent episodes of pneumonia were studied. In less than 40% of this population the initial diagnosis was in accordance with the criteria usually required in our unit to perform such diagnosis. CONCLUSIONS: We concluded that the recurrent nature of pneumonia episodes should be further clarified and discussed with pediatricians because it is up to them to refer such patients to specialists. Furthermore we emphasize the importance of normal chest roentgenograms taken between acute episodes so that "recurrent pneumonia" can be adequately characterized. This may help identify those patients who need subsequent evaluation by specialists regarding complementary diagnosis.