Osteoporosis and multiple fractures in an antiretroviral-naive, HIV-positive child.

As a result of the increased incidence of osteopenia and osteoporosis in HIV-infected patients, numerous publications have suggested that there may be a link between bone metabolism alterations and HIV infection. The early bone loss seen in these patients was initially attributed to the use of highly active antiretroviral treatment (HAART) that included protease inhibitors. Recent studies, however, have suggested that it may be a direct consequence of the viral infection on bone metabolism, persistent activation of pro-inflammatory cytokines (TNFa), or altered vitamin D metabolism secondary to the virus, combined with subsequent factors (e.g., antiretroviral treatment) that aggravate the bone demineralization. We present an antiretroviral-naive 6-year-old girl with vertically transmitted HIV infection who presented with severe osteoporosis and multiple pathological fractures of the vertebrae, ribs, and upper and lower limbs. The child was treated with HAART, appropriate nutritional support for her age, physiotherapy and rehabilitation, calcium and vitamin D supplements, and alendronate therapy. After 6 weeks of treatment, the intense pain and muscle atrophy had disappeared and she was able to walk unassisted. At 6 months, bone mass had increased by 72%. The interest of this case lies in the presence of severe osteoporosis and multiple pathological fractures in an HIVinfected naive child. To date, this condition has only been described in patients treated with antiretrovirals. Moreover, this is the first reported HIV-positive pediatric patient treated with bisphosphonates, which proved to be highly successful.

[The use of polymerase chain reaction in the diagnosis of invasive meningococcal disease]. / La reacción en cadena de la polimerasa en el diagnóstico de la enfermedad meningocócica invasiva.

INTRODUCTION AND OBJECTIVES AND AIMS: Invasive meningococcal disease (IMD) remains a serious public health problem. Although culture is the gold standard, previous antibiotic therapy reduces its sensibility. The aim of this study is the epidemiological analysis of IMD in our area, to assess the usefulness of polymerase chain reaction (PCR) to increase its diagnostic accuracy,and to show the association of antibiotic administration with the negative result of the culture. PATIENTS AND METHODS: A retrospective study was conducted on all children younger than 16 years with microbiologically (positive culture and/or PCR) confirmed IMD, admitted to our hospital between 2004-2012. RESULTS: Seventy-five patients were included, of whom 52% had sepsis, 30.7% meningitis, and 17.3% with both of them. PCR was positive in all samples, whereas a positive was seen 50.7% of the cultures. Previously administered antibiotic was documented in 40 patients (53.3%), and 40% of them were confirmed by PCR only. CONCLUSIONS: PCR was the only test providing evidence for IMD diagnosis and serogroup determination in almost 39% of cases.

Pulsed Doppler patterns of left atrial flow in mitral regurgitation.

A prospective pulsed Doppler study of 55 patients was undertaken to detect and quantitate mitral regurgitation. Systolic left atrial flow dispersion indicated mitral regurgitation. Doppler study was feasible in nearly 95% of patients. Sensitivity, specificity and predictive accuracy were 89%, 84% and 88%, respectively. Three methods were used to quantitate mitral regurgitation: (1) maximal depth level of systolic left atrial flow, (2) left atrial flow patterns immediately behind the mitral valve closure line (I, protosystolic; II, gradual decrease in intensity of left atrial flow signal throughout systole; and III, broad flow dispersion occupying all systole); and (3) a score system that combined these 2 methods. Results showed that flow patterns behind the mitral valve closure line correlate with angiographic assessment of mitral regurgitation. This provided the simplest and most practical method of determining the severity of mitral regurgitation.

Caroli's disease versus polycystic hepatic disease. Differential diagnosis with Tc-99m DISIDA scintigraphy.

Four patients with multiple hepatic cysts were studied: two were diagnosed with Caroli's disease (CD) and two with polycystic hepatic disease (PHD). In CD, hepatic scintigraphy with Tc-99m DISIDA showed areas of focally increased radiotracer accumulation that persisted more than 120 minutes, whereas in PHD, areas of focally decreased radiotracer accumulation were observed with normal liver washout and biliary excretion. When multiple hepatic cysts are shown by abdominal echography or CT scan, hepatic scintigraphy with Tc-99m DISIDA should be performed. This examination is safe and noninvasive, and permits differential diagnosis between CD and PHD.

[The Spanish Society of Paediatric Infectious Diseases (SEIP) recommendations on the diagnosis and management of invasive candidiasis]. / Recomendaciones de la Sociedad Española de Infectología Pediátrica sobre diagnóstico y tratamiento de la candidiasis invasiva.

Candida yeasts are ubiquitous commensals, which can cause opportunistic infection in any location of the body. The source of infection may be both endogenous and exogenous. Invasive candidiasis encompasses different entities ranging from invasive candidiasis to disseminated multiorgan infection. Invasive candidiasis is the third leading cause of nosocomial bloodstream infection and the fourth of all nosocomial infections. It is also the most common invasive fungal infection in non-neutropenic critically ill patients, with a remarkable increase in the last 20 years owing to the increased survival of these patients and to more complex diagnostic, therapeutic and surgical procedures. Its incidence in infants, according to recent reviews, stands at 38.8 cases/100,000 children younger than 1 year. Candida albicans remains the most frequent isolate in invasive infections, although infections caused by other species have risen in the last years, such as C. kruzsei, C. glabrata and C. parapsilosis; the latter causing invasive candidiasis mainly associated with central venous catheter management, especially in neonatal units. The overall mortality of invasive candidiasis is high, with 30-day mortality reaching 20-44% in some series involving paediatric patients. This report provides an update on incidence, epidemiology, clinical presentation, diagnosis, treatment and outcome of invasive infection by Candida spp. in the paediatric patient.

[Emerging invasive fungal infections]. / Infecciones fúngicas invasivas emergentes.

The frequency and diversity of invasive fungal infections has changed over the last 25 years. The emergence of less common, but medically important fungi has increased, and the children at risk has expanded, with the inclusion of medical conditions such as cancer, mainly haematological malignancy or stem cell transplant, immunosuppressive therapy, prolonged neutropenia, and T-cell immunodeficiency. Among mould infections, fusariosis and phaeohyphomycosis (Dematiaceous fungi) have been increasingly reported in this group of patients. To successfully manage these challenging infections, it is imperative that paediatricians and sub-specialists remain aware of the optimal and timely diagnosis and therapeutic options. Unlike other common mycoses that cause human disease, there no simple antigen or serological tests available to detect these pathogens in tissue or blood. The outcome for these disseminate, and often refractory fungal infections in neutropenic patients and transplant recipients remains extremely poor, requiring early and aggressive therapy. Unfortunately there are no guidelines outlining the choices for optimal therapy in the treatment of paediatric invasive fungal infections do not exist, and on the other hand are limited paediatric data available comparing antifungal agents in children with proven, probable or suspected invasive fungal infection. The options for treatment rest mainly on some adult guidelines that comment on the treatment of these emerging and uncommon important fungi in children. Despite the sparse clinical trials available on treatment and its poor outcome, options for treatment of invasive fungal infections have increased with the advance of new antifungal agents, with improved tolerability and increased range of activity. The epidemiology, clinical manifestations, diagnosis and treatment of fusariosis and phaeohyphomycosis are discussed in this article.

[Spanish Paediatric Infectious Diseases Society consensus document on the treatment of fungal infections based on the immune response]. / Documento de consenso de la Sociedad de Infectología Pediátrica sobre el tratamiento de la infección fúngica basado en la respuesta inmunológica.

Despite the emergence of new diagnostic and therapeutic methods, invasive fungal infections are still a major cause of morbidity and mortality in immunocompromised and critical patients. Therefore, adjuvant treatments to the standard antifungal therapy are being investigated, with immunity-based therapy being one of the most important. Both immunomodulatory (dendritic and T cells transfusions, colony stimulating factors, interferón-gamma, interleukin 12, fungal vaccines, transfer factors and certain drugs such as chloroquine) and immunotherapeutic modalities (granulocyte transfusions, monoclonal antibodies and intravenous immunoglobulin) have been described. This document aims to summarise currently available data on immunity-based therapy of fungal infections and to provide basic knowledge on the immune response to fungal infections. This helps to understand how, in selected cases, immunity-based therapy may improve the response to standard antifungal treatment. The potential indications of immunity-based therapy in the paediatric patient are reviewed, although there is still a lack of scientific evidence for its use in children.

A new diagnostic approach to the Dubin-Johnson syndrome.

Dubin-Johnson syndrome (DJS), a congenital metabolic disorder of bilirubin excretion, was classically diagnosed by the bromsulfalein (BSP) curve and needle hepatic biopsy methods. We present three cases of DJS and propose a new diagnostic approach which could conceivably become a substitute for more aggressive techniques. The results of the 24-h urine coproporphyrin determination and 99mTc-Disofenin scintigraphy gave, together, enough data for an accurate diagnosis.

[Endocardial monophasic action potentials obtained by means of suction catheters]. / Los potenciales de acción monofásicas endocárdicas obtenidos mediante catéteres de succión.

Endocardial monophasic active potentials (M.A.P.) recorded by suction --tip intracardiac catheters constitute a new research-- method in cardiac arrythmias, electrophysiology and pharmacology. The characteristics of MAP at auricular and ventricular levels are analyzed. The limitations of the method in its incapacity to record the MAP of conduction tissues is discussed. This method of recording MAP is evaluated with antiarrythmic drugs of tipes I, II, III and IV. The results are identical to recordings performed with intracellular microelectrodes.