Biomedical subjects
Publications and source records attributed to V Novelli.
Children presenting with disseminated viral infections should be carefully investigated because they almost invariably have an underlying immunodeficiency. A child is reported who had disseminated cytomegalovirus and a novel form of severe combined immunodeficiency with abnormal expression of the common leucocyte antigen, CD45.
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A family clinic providing specialist paediatric and adult medical, testing, counselling, and terminal care services for families living with HIV was set up at a paediatric tertiary care hospital in London in 1991. During the first five years, until April 1996, 185 children from 149 families attended, including 119 infected children, of whom 32 have died. Only 5% of mothers were born in the UK; the rest were born in 24 different countries, the majority in sub-Saharan Africa. Less than a quarter of children were cared for by both parents, 61% by mothers alone, and 11% by guardians or foster parents. Of the adult attendees, 76% were women, and more than half were untested when they first attended the clinic. Provision of a family planning service within the family clinic was initiated as a result of women presenting with unplanned pregnancies. Shared care with local clinics is increasing, but with the complexity around the management of paediatric HIV infection, particularly with regard to antiretroviral treatments, there is need for continued specialist input. Coordination among specialist and locally based family services is required to provide flexible, accessible, and up to date care for families living with HIV infection in London.
BACKGROUND: Kawasaki disease (KD) is a systemic vasculitis of childhood with widespread vascular endothelial damage in the acute stage. Long-term complications, such as myocardial infarction and death, are recognized, but the extent and nature of late vascular abnormalities that might predispose to these events have not been studied. METHODS AND RESULTS: We used high-resolution ultrasound to study endothelial function in the brachial artery of 20 patients 5 to 17 years after acute KD (median, 11 years) and compared findings with those in 20 age- and sex-matched control subjects. Vascular responses to reactive hyperemia (with flow increase leading to endothelium-dependent dilation) and to sublingual glyceryl trinitrate (GTN; endothelium-independent dilation) were recorded. The relationship between endothelium-dependent vascular responses and features of the endothelium acute illness was examined. There was no difference in baseline vessel diameter, degree of reactive hyperemia, or response to GTN between patients and control subjects. In contrast, flow-mediated dilation was markedly reduced in KD patients compared with control subjects (3.1% versus 9.4%; P < .001). Late endothelium-dependent responses were not related to features of the acute illness. CONCLUSIONS: Abnormalities of systemic endothelial function are present many years after resolution of acute KD, even in patients without detectable early coronary artery involvement. Because this may be an important factor in the genesis of late vascular complications, long-term follow-up of all patients with KD is indicated.
BACKGROUND/AIMS: The aims of alpha-interferon treatment for chronic viral liver infections are clearance of the virus and healing of the disease. Hepatocellular carcinoma is a complication of viral cirrhosis; but it is not yet known whether treatment of viral cirrhosis with alpha-interferon prevents this complication. METHODS: The incidence and the risk (Cox regression analysis) of developing hepatocellular carcinoma were calculated in 347 patients with hepatic cirrhosis; 227 (34 hepatitis B virus and 193 hepatitis C virus related) were treated with alpha-interferon and 120 (28 hepatitis B virus and 92 hepatitis C virus) did not receive this treatment, in order to evaluate the efficacy of alpha-interferon in the prevention of hepatocellular carcinoma. In all patients, the cirrhosis was well compensated (Child A). RESULTS: Over mean follow-up periods of 49 months for hepatitis B virus and 32 months for hepatitis C virus, 20/347 patients (6/62 hepatitis B virus and 14/285 hepatitis C virus) developed hepatocellular carcinoma. The risk of developing this tumor was significantly greater in males (p < 0.007) and in patients not treated with alpha-interferon (p < 0.01). The Relative Risk of developing hepatocellular carcinoma increased significantly (p < 0.0002) with each passing year. In patients with hepatic cirrhosis secondary to hepatitis B virus infections, the risk did not seem to be modified by alpha-interferon treatment, even though a greater, but not significant risk (Relative Risk = 4.9; p = 0.3) was calculated for untreated patients; in contrast, in hepatitis C virus-related cirrhosis, this risk was reduced by a factor of 4.0 (p = 0.04). The tumor developed only in non-responder patients regardless of virus type. After adjustment for confounding factors (sex, age, alcohol consumption, cigarette smoking), a statistically significant (p < 0.025) effect of interferon treatment in preventing hepatocellular carcinoma was still demonstrated when responders were matched with controls, but not when responders were compared with non-responders. CONCLUSIONS: These results show that, in addition to its ability to halt the progression of viral-induced liver disease, alpha-interferon is also of benefit in patients with hepatitis C virus cirrhosis who respond to this treatment by lowering their risk of developing hepatocellular carcinoma.
This study identified the ENT symptoms of 66 HIV infected children over an 8 year period (1986-1993) at Great Ormond Street Hospital for Children. The incidence, nature and age of onset of ENT symptoms were investigated; 91% of the children had ENT symptoms, the most common being cervical lymphadenopathy, oro-oesophageal candidiasis and otitis media. The HIV infected children suffered from the common ENT diseases of childhood. They also presented with specific conditions such as diffuse parotid swelling. Therefore, their clinical features differed from HIV infected adults as well as non-infected children. An increasing incidence of paediatric HIV infection was demonstrated by the study. Most were due to vertical transmission. ENT surgeons are likely to see more HIV infected children in future, either with the usual ENT diseases of childhood (to which they seem more susceptible) or with HIV-specific conditions. Although the diagnosis of HIV may be known, the ENT condition could be the initial presentation suggestive of immunodeficiency.
AIDS related Kaposi's sarcoma is commonly seen in homosexual men, only occasionally in men and women with heterosexually acquired HIV, and extremely rarely in children. The case of an HIV infected mother and her vertically infected child who both developed visceral Kaposi's sarcoma is reported. It is proposed that the putative Kaposi's sarcoma agent may also be transmitted vertically.
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Cytomegalovirus retinitis is common in adults with AIDS but has been reported infrequently in children with perinatally acquired HIV infection. The cases are presented of two infants with vertically acquired HIV infection who developed disseminated cytomegalovirus infection and retinitis, and who posed difficult management issues.
There appears to be an increase in imported cases of visceral leishmaniasis in Northern Europe; many are children infected on holiday in the Mediterranean. Making the diagnosis in young children can be difficult especially when an adequate travel history is not obtained at presentation. Two infants with visceral leishmaniasis are presented who were initially felt to have alternative diagnoses and who subsequently responded dramatically to a short course of liposomal amphotericin B (AmBisome).
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When an infant develops acute respiratory failure of sufficient severity to necessitate supportive mechanical ventilation a cause should always be sought. A chest radiograph showing predominantly interstitial lung disease and an infant's failure to respond to standard antibiotic treatment are indications for non-bronchoscopic bronchoalveolar lavage. If P carinii pneumonia is diagnosed a congenital immunodeficiency should be sought and the parents counselled about HIV infection. Earlier investigation may be indicated by features of immunodeficiency when taking a history, performing a general examination, or analysing the results of basic haematological testing.
The aim of this study was to evaluate the efficacy of human lymphoblastoid interferon-alpha treatment in chronic sporadic-type non-A, non-B hepatitis. We also aimed to determine if histological or liver function data could predict either response or relapse. Sixty patients with chronic sporadic-type non-A, non-B hepatitis were randomized in two groups of 30. One group was treated with interferon-alpha (3 MU thrice weekly) for one year; the other group was untreated controls. The treated group was followed for another year after interferon withdrawal. Liver function tests were performed during treatment. Liver biopsy was carried out before and a year after randomization. We evaluated rate of response [normalization of alanine aminotransferase (ALT) levels for at least three consecutive months] and rate of relapse (ALT rebound after therapy suspension). We also looked at possible predictive factors for response and relapse. In the treatment group the rate of response was 55% (16/29). No control patient exhibited ALT normalization. Among the responders, 31% (5/16) relapsed after interferon withdrawal. Low gamma GT and female sex are positive predictive factors of response (P < 0.01 and P < 0.02 respectively). Presence of portal and periportal inflammation at the second liver biopsy was correlated with relapse (P < 0.05). In conclusion, human lymphoblastoid interferon-alpha treatment for one year is beneficial in patients suffering from chronic sporadic-type non-A, non-B hepatitis. Low pretreatment gamma GT levels and female sex are positive predictors of response in this patient population.(ABSTRACT TRUNCATED AT 250 WORDS)
In order to review the clinical course, laboratory findings, and outcome of children with vertically acquired HIV infection and Pneumocystis carinii pneumonia, questionnaires were sent to paediatricians in the British Isles who had reported P carinii pneumonia and HIV infection through the British Paediatric Surveillance Unit (BPSU). Paediatric reports from the BPSU are linked to reports of pregnancies in HIV positive women and laboratory reports. P carinii pneumonia was the most frequently reported AIDS indicator disease at AIDS diagnosis, occurring in 22/56 (40%) children born in the British Isles; in a further two children P carinii pneumonia occurred after another AIDS indicator disease. The median age at P carinii pneumonia diagnosis was 4.1 (1.4-27.3) months and in 48% it occurred with other AIDS indicator diseases. Despite intensive treatment the three month survival was only 38%. The nine children surviving P carinii pneumonia subsequently developed further AIDS indicator diseases, in particular HIV encephalopathy and four have since died. P carinii pneumonia was present at AIDS diagnosis in 65% of children developing AIDS in the first year of life and caused 82% of infant deaths. Most children were not known to be at risk of HIV until they presented with P carinii pneumonia. Children with HIV infection develop P carinii pneumonia at an early age and have a poor outcome. Increased awareness of the condition is required to initiate early treatment. Prevention may be a compelling incentive for screening in pregnancy, but further study is required to quantify the risks and benefits of initiating early P carinii pneumonia prophylaxis as well as the impact this might have on life expectancy.
The advent of bile acid therapy has shed some light on the mechanisms involved in determining bile lipid secretion. The administration of cholelytic bile acids results in a lowering of cholesterol percent molar and saturation index due to a reduction in cholesterol secretion. Studies carried out after administration of bile acids showed initially that biliary cholesterol secretion rates were dependent on the hydrophobic/hydrophilic balance of the prevailing bile acid present in bile. However, more detailed investigations showed that some bile acids (cholic and chenodeoxycholic acids) did not follow this rule because of the presence of other mechanisms involved in determining biliary cholesterol secretion and a possible link between cholesterol synthesis and biliary cholesterol secretion. Several different human models have been used in more recent studies to arrive at a better understanding of the mechanisms involved in determining bile lipid secretion: obese patients, obese patients in rapid weight loss, patients with non-familial hypercholesterolemia and primary biliary cirrhosis. The findings in these studies indicate how modifications in biliary lipid secretion can easily be induced when there are changes in the relative amounts of bile acids. These changes may bring about modifications in intestinal absorption, liver synthesis, and secretion of cholesterol and bile acids that could possibly lead to the formation of lithogenic bile and subsequently to cholesterol gallstones.
Lactobin-R is a commercial hyperimmune bovine colostrum with potent anticryptosporidial activity. It was administered to a 4 year old child with AIDS and severe diarrhoea associated with cryptosporidiosis. There was significant clinical improvement in the diarrhoea and permanent elimination of the parasite from the gut as assessed through serial jejunal biopsy and stool specimens.
Official methods for the determination of 2-furaldehyde in spirits involve for a spectrophotometric evaluation, which is characterized by poor specificity. Gas chromatographic evaluations have also been proposed, which offer a much higher sensitivity, particularly when capillary columns are used. In this paper a high-performance liquid chromatographic (HPLC) method based on the formation of the 2,4-dinitrophenylhydrazones of carbonyl compounds and subsequent reversed-phase separation of these derivatives is described. Derivatization is carried out by utilizing an acidic solution of 2,4-dinitrophenylhydrazine in acetonitrile. Precipitation of the derivatives is avoided and direct injection of the sample into the HPLC system is allowed. The determination offers a high specificity and a detection limit of the order of 10(-8) mol/l. Accuracy and reproducibility data are presented.