Central nervous system lymphoma in children

This paper describes the rare MR and CT features of central nervous system (CNS) lymphoma in immunocompetent children and in survivors of childhood acute lymphoblastic leukemia (ALL) and discusses the causative role of cranial irradiation and/or leukoencephalopathy preceding central nervous system (CNS) lymphoma in survivors of childhood leukemia. The authors reviewed MR and CT scans of 3 children with biopsy-proved CNS lymphoma. One child had tumor infiltration within the optic nerve sheaths and optic chiasm by previously known non-Hodgkin lymphoma. In 2 patients, CNS lymphoma developed 8 and 10 years after initial ALL treatment. In both cases CNS lymphoma was preceded by cranial irradiation and leukoencephalopathy. A single lesion was present in 3 out of 4 patients. All lesions were isointense or hypointense on the T1-weighted images relative to gray matter and showed homogeneous enhancement. One lesion was centered in the central gray matter, one lesion was centered within a cerebral hemisphere, one lesion was in optic nerve, and there were 2 parasellar lesions. CNS lymphoma has a variable appearance in children. Knowledge of risk factors in children may help in the early recognition of disease, allowing for timely intervention. This may prompt early biopsy or a conservative management in the appropriate clinical setting.     

Miliary tuberculosis in children: a review of 94 cases.

This is a retrospective review of the clinical, radiologic and laboratory features of 94 cases of childhood miliary tuberculosis seen during a 5-year period, 1985 to 1989. A history of Bacillus Calmette-Guérin vaccination was documented in 88% of children. The median age at presentation was 10.5 months, 52% of cases occurring in those younger than 1 year. The presenting symptoms were nonspecific: cough (72%); fever (61%); loss of appetite and weight (40%); and diarrhea and vomiting (33%). The main presenting signs were hepatomegaly (82%), splenomegaly (54%), lymphadenopathy (46%) and pyrexia (39%). Most of the patients were malnourished and anergic. Meningitis occurred in 19% of patients and this was the only significant risk factor identified for mortality, the overall case fatality rate being 14%. The diagnosis in the vast majority was made on the clinical presentation supported by a classic miliary pattern on chest roentgenogram (91% of cases). Mycobacterium tuberculosis was cultured in 33% of cases. In addition a review of hospital admissions from 1981 to 1989 revealed that annually miliary tuberculosis in children and adults accounted for 8.3 and 1.3%, respectively, of all tuberculosis admissions. This study confirms that miliary tuberculosis is a relatively common complication of tuberculosis in young children.     

North American Indian cirrhosis in children: a review of 30 cases

BACKGROUND: North American Indian childhood cirrhosis (NAIC) is a distinct, rapidly evolving form of familial cholestasis found in aboriginal children from northwestern Quebec. This is a retrospective review of the 30 patients treated in Quebec since the discovery of NAIC in 1970. METHODS: The clinical records and histologic samples from 30 patients were reviewed. Extensive metabolic, biochemical, viral, genetic, and radiologic studies were performed in most patients. RESULTS: Genetic analysis suggests autosomal recessive inheritance and a carrier frequency of 10% in this population. Gene mapping studies showed that the NAIC gene is located on chromosome 16q22. Typically, patients have neonatal cholestatic jaundice (70%) or hepatosplenomegaly (20%) with resolution of clinical jaundice by age 1 year but persistent direct hyperbilirubinemia. Portal hypertension was documented in 29 patients (91%). Variceal bleeding (15 patients, 50%) occurred as early as age 10 months. Surgical portosystemic shunting was performed in 13 of these 15 patients (87%); 4 (31%) rebled after 1 to 5 years. Fourteen patients died (47%). In 10 (71%), liver disease was the cause. Four children died of liver failure before liver transplantation became available. In transplanted livers, no recurrence of NAIC was observed after 1 to 10 years. Recognized infectious, metabolic, toxic, autoimmune, and obstructive causes of cirrhosis have been eliminated. The histologic features of NAIC show early bile duct proliferation and rapid development of portal fibrosis and biliary cirrhosis, suggesting a cholangiopathic phenomenon. CONCLUSION: Together with gene mapping studies showing that the NAIC gene is different from those of other familial cholestases, these observations suggest that NAIC is a distinct entity that could be classified as "progressive familial cholangiopathy."     

Central nervous system manifestations of HIV infection in children

Vertically transmitted HIV infection is a major problem in the developing world due to the poor availability of antiretroviral agents to pregnant women. HIV is a neurotrophic virus and causes devastating neurological insults to the immature brain. The effects of the virus are further compounded by the opportunistic infections and neoplasms that occur as a result of the associated immune suppression. This review focuses on the imaging features of HIV infection and its complications in the central nervous system.     

Enterobacter cloacae bacteremia in children: a review of 30 cases in 12 years

A review was performed of the 30 cases of pediatric Enterobacter cloacae (EBC) bacteremia which occurred at our institution during a 12-year period. These 30 cases represented 88% of all cases in which EBC was isolated by blood culture (four other instances were considered contaminants); the rate of isolation of this organism relative to all positive blood cultures was 0.6%. There were 14 patients less than 12 months of age, with 10 less than 2 months of age. Infection was nosocomially-acquired in 17 cases. At the time the positive blood culture was obtained, 5 patients were afebrile, and 8 patients (five immunocompromised) had been receiving parenteral antibiotic therapy to which the organism exhibited in-vitro sensitivity for at least 24 hours. EBC was a constituent of polymicrobial bacteremia in 6 cases; in 5 instances the associated organisms were also gram-negative bacteria. There were a total of 33 underlying medical conditions or foci of infection associated with EBC bacteremia identified in 27 patients, the most common of which were immune-deficiency state (17) and gastrointestinal tract lesions (6). There were 3 patients who died. EBC bacteremia is a relatively rare pediatric infection. It is commonly nosocomially-acquired, and afflicts children who are younger-aged or compromised by underlying medical problems.     

Modern imaging of tuberculosis in children: thoracic,central nervous system and abdominal tuberculosis

Tuberculosis (TB) can affect any organ in the body. Children are a high-risk group for contracting the disease and pose a constant challenge to clinicians with regard to making a definitive diagnosis. Radiologists are playing a more active role in diagnosing TB, and armed with more accurate diagnostic investigations such as CT and MRI, they must face the cost implications as well as technical limitations. This review aims to guide the reader through the modern imaging techniques useful for diagnosing TB of the thorax, central nervous system and abdomen in children. The more specific features of each modality in the particular anatomical regions are highlighted.     

小儿肺炎支原体感染的神经系统损害

最近三十年 ,从流行病学和临床观察 ,采用最敏感的诊断技术显示 ,城市人群中肺炎支原体感染很为常见。Hornsleth等人应用补体 -固定抗体测定 ,6～ 1 1个月婴儿4 2 %血清抗体阳性 ,1～ 9岁抗体阳性率为 6 7%。 Brunner等应用敏感的放射免疫沉淀试验检测血清抗体 ,2 5～ 6 0个月婴幼儿 6 7%阳性 ,97%的 1 7岁及以上人群抗体阳性 〔1〕。以上数字表明 ,人群中肺炎支原体感染率相当高。儿童肺炎支原体感染主要引起肺炎 ( 71 % )、中耳炎 ( 1 4 % )、咽炎( 1 0 % ) ,大约 1 5 %可无症状或表现轻度上呼吸道感染〔1 ,2〕。以肺炎为例支原体分别占…     

Central nervous system processing of emotions in children with faecal incontinence

Aim: Faecal incontinence (FI) is a common disorder involving both the enteric (ENS) and central nervous systems (CNS). The aim of the study is to analyze neurophysiologically the central processing of emotions in children with FI, healthy controls and children with Attention‐deficit hyperactivity disorder (ADHD). Methods: Fourteen children with FI and constipation, nine with non‐retentive FI, 15 controls and 13 children with ADHD were examined. The methods included a physical exam, sonography, Child Behavior Checklist, a psychiatric interview and intelligence test. Acoustic evoked potentials were recorded according to standardized methodology. For the event‐related potentials, 80 neutral, 40 positive and 40 negative pictures from the International Affective Picture System (IAPS), and 40 pictures depicting faeces were presented. Results: Children with FI had significantly more intense responses for most stimuli over the frontal, central and parietal regions compared to controls. Stool pictures did not evoke stronger responses than other stimuli. Children with constipation elicited stronger responses. Children with ADHD did not differ from controls. Acoustic evoked potentials were comparable in all groups. Conclusions: Children with FI have increased responses in the processing of emotions. These can be interpreted as a neurobiological vulnerability, possibly due to the association of the ENS and CNS.     

Central nervous system leukemia

With one exception, the risk and severity of neurotoxicity is directly proportional to the number of therapeutic modalities used. Three are worse than two, and two are worse than one. Combinations of therapeutic modalities which include CNS RT appear to be the most neurotoxic. The least neurotoxic combination of two modalities appears to be the IT MTX with high-dose intravenous MTX. Thus far, high-dose MTX appear to be the safest single modality, in terms of acute, subacute, and delayed neurotoxicity. The improved outcome in intellectual and academic performance in the NCI-191/CCG-134P conjoint trial of the CCSG and the Pediatric Branch described above (see section of Presymptomatic CNS Therapy) appears to confirm this observation. Whether triple IT chemotherapy will have the same result remains to be established. If CNS RT must be combined with MTX therapy, the least neurotoxic approach appears to be to administer these modalities in sequence, IT MTX, or high-dose intravenous MTX followed by CNS RT. MTX given during or after CNS RT appears from the clinical data to be more likely to produce severe neurologic sequelae. An ultimate goal would be to avoid both ionizing RT and IT chemotherapy. To this end, the NCI/CCSG study has demonstrated that this may be possible, except for those patients who are at the highest risk for CNS relapse despite conventional CNS prophylaxis. Meanwhile, for presymptomatic therapy, either cranial RT (18 Gy total dose at 120-180 cGy per day) in conjunction with IT MTX, or frequent IT chemotherapy with MTX, cytarabine, and hydrocortisone combined and administered throughout induction, consolidation, and maintenance is eminently justified in the majority of children with ALL. On a worldwide basis, chemoradiotherapy with cranial RT and IT MTX remains the established method of preventing overt CNS leukemia. The benefits of this intervention, in terms of prevention of symptomatic CNS leukemia, prolongation of complete remission, and increased cure rates, are clearly worth the risks.     

Central nervous system tumors

Central nervous system (CNS) tumors comprise 15% to 20% of all malignancies occurring in childhood and adolescence. They may present in a myriad of ways, often delaying diagnosis. Symptoms and signs depend on the growth rate of the tumor, its location in the central nervous system (CNS), and the age of the child. This article describes the presentation, diagnosis and management of these tumors.