Nonlethal, attenuated, transfusion-associated graft-versus-host disease in an immunocompromised child: case report and review of the literature.

BACKGROUND: Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare complication of transfusion of nonirradiated blood components. It usually affects children in high-risk groups, including those who have primary immunodeficiencies (PIDs). It usually presents with skin, hepatic, digestive, and hematologic involvement and is normally fatal. CASE REPORT: We report the case of a nonlethal, attenuated, TA-GVHD in a 7-month-old boy. The disease was marked by the presence of a severe rash but lacked all the other usual manifestations. We speculate that the unusually benign course of this disease, which has normally a fulminant course, was due to the fact that this child was under high-dose corticotherapy at the time of the engraftment. This fortunate coincidence led to the survival of this child and allowed the diagnosis of a combined immunodeficiency. CONCLUSION: A high index of suspicion is required for the diagnosis and proper management of PID. The administration of nonirradiated blood components in the first year of life, sometimes before the clinical suspicion of a PID, is of great concern. TA-GVHD may be more prevalent than reported in the literature and it is possibly a nonidentified cause of death in recipients with unexplained death and nondiagnosed PID.

Methadone intoxication in a child: toxic encephalopathy?

Methadone is used in the treatment of opioid addiction. Acute intoxication can lead to severe consequences and can even be lethal. In several case reports and small series, a presumably toxic leukoencephalopathy is described resulting from inhalation of heroin. We present the case of a 3-year-old boy who ingested methadone accidentally. In a coma with acute obstructive hydrocephalus owing to massive cerebellar edema and supratentorial lesions, he was successfully treated with methylprednisolone and cerebrospinal fluid external drainage. To our knowledge, this is the first report of an encephalopathy associated with synthetic opioid intoxication.

Lupus myelopathy in a child.

A 5-year-old female developed, after a 7-month period of fever, anorexia, weight loss, and a transitory cutaneous erythematous eruption, a severe acute transverse myelopathy, with a partial recovery of motor and sensory function. She had positive antinuclear and antidouble-stranded DNA antibodies but no antiphospholipid antibodies. Six months later she had massive proteinuria and restarted treatment with steroids and cyclophosphamide. Our patient is one of the youngest reported with lupus myelopathy. We discuss the clinical presentation, the magnetic resonance imaging findings, and other relevant laboratory studies of this rare but serious complication of systemic lupus erythematosus.

Health-related quality of life of pediatric intensive care survivors.

OBJECTIVE: To assess the health-related quality of life (HRQoL) of children surviving to pediatric intensive care discharge. METHODS: A prospective evaluation of HRQoL at admission and 6 months later was carried out with children aged 6 years or more, admitted to three tertiary pediatric intensive care units (PICUs) from May 2002 to June 2004. HRQoL was measured with the Health Utilities Index Mark 3 (HUI3) questionnaire, administered to a child's proxy. RESULTS: From the 517 eligible admissions, 44 children died in the PICU (8.5%) and 320 cases were evaluated at admission; among those, follow-up data were available in 252 cases. There were no statistically significant differences between preadmission and follow-up HUI3 global scores (medians [interquartile range] of 0.86 [0.42-1.00] and 0.83 [0.45-1.00]; p = 0.674, respectively). At the individual level, 21% of children had their HRQoL unchanged, improvement was seen in 40% and deterioration in 38% of the cases. Severe disability before admission (HUI3 global score < 0.70) was present in 36% of the cases, with improvement at the 6-month follow-up in 60% of them. Among those with deterioration of HRQoL at follow-up, 45% were trauma victims. CONCLUSIONS: Although the HRQoL was globally similar in both evaluations, several differences were found at the individual level. Children with low preadmission HRQoL (severe disability) may benefit from pediatric intensive care, since many of these children improved their HRQoL compared to preadmission status.

[Sickle cell disease and cerebrovascular stroke: a preventable event]. / Anemia de células falciformes e acidente vascular cerebral: uma história evitável.

INTRODUCTION: About 75% of the children presenting with cerebrovascular stroke have an identified cause. For black children, the most common underlying condition is sickle cell disease. This case report describes a preventable natural history, with an unusual presenting feature. CASE PRESENTATION: 27th month black infant without relevant background, presented with an acute focal neurological deficit. Brain computed tomography scan showed extensive acute infarction, and magnetic resonance also revealed previous silent cerebral infarct. Blood sample showed normocytic anemia and spontaneous sickling of the red blood cells, with 87% hemoglobin S. Exchange transfusion was made. CONCLUSION: Stroke, as a sickle cell disease complication, can occur at early ages and being the presenting sign. We consider that it would be worth spread the antenatal hemoglobinopathy screening programme, and evaluating the cost-effectiveness of a neonatal screening programme for sickle cell disease in Portugal.

Successful Handling of Disseminated BCG Disease in a Child with Severe Combined Immunodeficiency.

In high-burden countries, Mycobacterium bovis Bacillus Calmette-Guérin (BCG) vaccine is administered in newborn to prevent severe Mycobacterium tuberculosis infection. Because life-threatening disseminated BCG disease may occur in children with primary immunodeficiency, vaccination strategy against tuberculosis should be redefined in non-high-burden countries. We report the case of a patient with X-linked severe combined immunodeficiency (SCID) who developed disseminated BCG disease, highlighting the specific strategies adopted.

[Hereditary pancreatitis in a child]. / Pancreatite hereditária na criança.

Hereditary pancreatitis is defined as a family history of two or more relatives with pancreatitis and clinical, biochemical, or radiologic evidence of pancreatitis. This is the fourth family described with hereditary pancreatitis related to mutation c.364C>T (p.R122C) of PRSS1 gene. The index case was a four year old child who had had his first episode of abdominal pain at age three. At that time he was admitted in hospital for two days and he improved with analgesic treatment only. One year later, in a second similar episode, he had been diagnosed with pancreatitis. His father was submitted to pancreato-duodenectomy (Whipple procedure) when he was 27 years old due to recurrent pancreatitis since age 19. Paternal grandfather and the parents of this grandfather had been diagnosed with diabetes mellitus. The mutation R122C was present in heterozigoty, in the exon 3 of PRSS1 gene, in the index case and also in his father. The importance of a long term follow-up is highlighted, taking into consideration the risk of ductal pancreatic adenocarcinoma.

[Lúpus erythematosus]. / Lúpus Eritematoso Sistémico.

Systemic Lúpus Erythematosus (SLE) is an auto-imune disorder, with multiple organ involvement, characterized by vascular and connective tissue inflammation, as well as antinuclear antibodies (ANA). We report a case of a black teenager with a past of arthritis of knees, fever and astenia during the year previous to this admission. She was admitted to the Pediatric Intensive Care Unit (PICU) for bilateral Streptococcus pneumoniae pneumonia complicated by pleural effusion. She also had generalized edema and macroscopic hematúria. She was started on antibiotics, with improvement. The studies were compatible with active SLE, poliserositis, nephrotic proteinúria, lupic nephritis class IV and serious anemia. During the admission, she had symptomatic hypertension with tonic-clonic seizure. She was transferred to Italy, clinically stable, medicated with steroids, anti-hypertensive and anti-convulsant drugs.

[Paediatric Burkitt lymphoma presenting as acute pancreatitis]. / Pancreatite aguda como manifestação de linfoma de Burkitt em idade pediátrica.

Pancreatitis in children and adolescents is uncommon and its causes are more varied in this age group than in adults. A tumoral aetiology is particularly rare. We present the case of a 13-year-old boy who was admitted to our Intensive Care Unit with the diagnosis of acute pancreatitis, bilateral pleural effusion and ascites. Serial sonographic and computed tomography evaluations were suggestive of an infiltrative process of the gastric wall. Endoscopy showed an infiltrative tumor of the gastric mucosa and duodenum with a giant ulcer. Biopsies were compatible with Burkitt Lymphoma confirmed by ascitic fluid cytology and cytometry. Because of severe progressive cholestasis a temporary biliary stent was placed in the common bile duct. After staging (stage III), the patient was treated according to FAB LMB 96 chemotherapy protocol, achieving complete remission. Acute pancreatitis and cholestasis are rare presentations of lymphoma in the paediatric age group.

Qualidade de vida relacionada à saúde de sobreviventes à terapia intensiva pediátrica / Health-related quality of life of pediatric intensive care survivors

OBJETIVO: Avaliar a qualidade de vida relacionada à saúde (QVRS) de crianças sobreviventes à alta da terapia intensiva pediátrica. MÉTODOS: Foi realizada uma avaliação prospectiva da QVRS na admissão e após 6 meses em crianças com idade igual ou superior a 6 anos, internadas em três unidades de terapia intensiva pediátricas (UTIPs) terciárias de maio de 2002 a junho de 2004. A QVRS foi avaliada com o questionário Health Utilities Index Mark 3 (HUI3), aplicado a um representante da criança. RESULTADOS: Das 517 admissões elegíveis, 44 crianças faleceram na UTIP (8,5 por cento) e 320 casos foram avaliados na admissão; entre eles, foi possível realizar o seguimento de 252 casos. Não foram encontradas diferenças estatisticamente significativas entre os escores globais do HUI3 antes da admissão e no seguimento [medianas (intervalo interquartil) de 0,86 (0,42-1,00) e 0,83 (0,45-1,00); p = 0,674, respectivamente]. No âmbito individual, 21 por cento das crianças não apresentaram mudanças na QVRS, foi observada melhora em 40 por cento e agravamento em 38 por cento dos casos. Deficiência grave antes da admissão (escore global do HUI3 <  0,70) esteve presente em 36 por cento dos casos, com melhora no seguimento aos 6 meses em 60 por cento deles. Entre aqueles que apresentaram agravamento da QVRS no seguimento, 45 por cento eram vítimas de trauma. CONCLUSÕES: Embora a QVRS seja globalmente semelhante nas duas avaliações, foram encontradas várias diferenças no âmbito individual. As crianças com baixa QVRS antes da admissão (deficiência grave) podem se beneficiar da terapia intensiva pediátrica, visto que muitas dessas crianças melhoraram a QVRS, em comparação com seu estado pré-admissão.

OBJECTIVE: To assess the health-related quality of life (HRQoL) of children surviving to pediatric intensive care discharge. METHODS: A prospective evaluation of HRQoL at admission and 6 months later was carried out with children aged 6 years or more, admitted to three tertiary pediatric intensive care units (PICUs) from May 2002 to June 2004. HRQoL was measured with the Health Utilities Index Mark 3 (HUI3) questionnaire, administered to a child’s proxy. RESULTS: From the 517 eligible admissions, 44 children died in the PICU (8.5 percent) and 320 cases were evaluated at admission; among those, follow-up data were available in 252 cases. There were no statistically significant differences between preadmission and follow-up HUI3 global scores (medians [interquartile range] of 0.86 [0.42-1.00] and 0.83 [0.45-1.00]; p = 0.674, respectively). At the individual level, 21 percent of children had their HRQoL unchanged, improvement was seen in 40 percent and deterioration in 38 percent of the cases. Severe disability before admission (HUI3 global score < 0.70) was present in 36 percent of the cases, with improvement at the 6-month follow-up in 60 percent of them. Among those with deterioration of HRQoL at follow-up, 45 percent were trauma victims. CONCLUSIONS: Although the HRQoL was globally similar in both evaluations, several differences were found at the individual level. Children with low preadmission HRQoL (severe disability) may benefit from pediatric intensive care, since many of these children improved their HRQoL compared to preadmission status.

Disección carotídea en un niño con neumonía y empiema / Carotid dissection in a child with pneumonia and empyema

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