Efficacy of Helicobacter pylori eradication on platelet recovery in children with chronic idiopathic thrombocytopenic purpura

Aim: Little is known about the influence of environmental factors on the epidemiology of idiopathic thrombocytopenic purpura (ITP). The role of Helicobacter pylori infection in relation to the development and/or persistence of ITP in infected patients remains controversial. Therapy used for eradicating H. pylori has led to a rise in platelet counts in a significant number of adult patients, but few paediatric studies have been undertaken to evaluate such treatment. The aim of this prospective study was to determine the prevalence of H. pylori and to evaluate whether H. pylori eradication can induce chronic ITP regression in children. Methods: To investigate new, non-invasive techniques for diagnosis of H. pylori infection, an enzyme immunoassay for H. pylori antigens in faeces (HpSA) was evaluated. Patient eligibility criteria included isolated thrombocytopenia (≤50 × 10[Formula: See Text] L[Formula: See Text]) for more than 6 months without any identifiable cause and either normal or increased marrow megakaryocytes. H. pylori status was monitored before eradication and 4, 12, and 24 mo, after the end of treatment using Premier Platinum HpSA. Results: In this study we evaluated 22 chronic ITP patients, 9 of whom were infected with H. pylori. Using repeated HpSA testing, we demonstrated for eradication of H. pylori after treatment in all infected patients. Five of the nine patients had increased platelet counts that persisted throughout the follow-up period.

Conclusion: These results should stimulate additional research into the involvement of H. pylori infection in chronic ITP in childhood. This approach may offer an accepted algorithm at least for some of these patients.     

Beta-glucuronidase in the diagnosis of bacterial meningitis and response to treatment

Aim: β-Glucuronidase activity is increased in the cerebrospinal fluid (CSF) of patients with bacterial meningitis. The aim of this study was to investigate the β-glucuronidase activity in the cell-free CSF of bacterial meningitis and its course during treatment, and compare it with other CSF parameters. Methods: The β-glucuronidase activity, cell number, protein concentration and CSF/blood glucose ratio were measured in 43 consecutive infants and children with bacterial meningitis, and 97 control subjects. Patients had one or two follow-up lumbar punctures. Results: The β-glucuronidase activity was increased early in bacterial meningitis, even when the other CSF parameters were undisturbed. Before treatment, the median activity in affected children was 136 μmoles 4-methylumbelliferone l ^-1  h ^-1 (range 44-826) and in controls 14 (7-23). In all patients who improved, the activity was lower in the follow-up CSF samples. Six to 12 h after starting treatment, the median activity was already reduced by 59%. The other CSF parameters showed a variability during the first 24 h of treatment independently of the course of the disease. Multiple comparisons of the CSF parameters in 17 patients who had two follow-up punctures showed that the β-glucuronidase activity was the best prognostic index.

Conclusion: β-Glucuronidase activity in the CSF is a reliable indicator of bacterial meningitis, which can identify the response to treatment early in the course of illness. The enzyme activity is increased early in the disease, even when the other laboratory parameters from the CSF remain normal.     

Short-term ceftriaxone therapy for treatment of severe non-typhoidal Salmonella enterocolitis

Aim: To evaluate the clinical efficacy of a short-course ceftriaxone therapy in the treatment of paediatric patients with severe non-typhoidal Salmonella enterocolitis. Methods: During a 1 y period, all paediatric patients who were suspected having Salmonella enterocolitis by the presentation of bloody and/or mucoid diarrhoea with or without fever were eligible for the study. Patients with either negative stool cultures or bacteraemia were excluded. Severe enterocolitis was defined as a bloody and/or mucoid diarrhoea associated with high fever persisting for longer than 48 h and signs of moderate or severe dehydration. The patients with severe enterocolitis were assigned to treatment with ceftriaxone (50 mg kg^-1 d^-1) for 3-5 d, while the rest were given supportive treatment only. Before treatment all study patients received blood testing for white blood cell (WBC) count, C-reactive protein (CRP) level and blood culture. The duration of the fevers was recorded. Patients were followed up after clinical recovery for the possibility of relapse. Results: Seventy-three patients with culture-confirmed Salmonella enterocolitis without bacteraemia were analysed. The duration of fever was longer in severe cases who were treated with ceftriaxone than those who were not. However, rapid defervescene was found after short-course ceftriaxone therapy in those patients with severe enterocolitis. CRP was significantly higher in severe cases. There was no significant difference in the WBC count between the two groups of patients. No relapse was found in these patients.

Conclusion: High CRP, prolonged high fever and signs of moderate or severe dehydration appear appropriate to define severe cases of Salmonella enterocolitis. Short-course ceftriaxone therapy is clinically beneficial to these patients. Neither clinical nor microbiological relapse was seen after therapy.     

Retrospective evaluation of long-term efficacy and safety of splenectomy in chronic idiopathic thrombocytopenic purpura in children

Aim: To review the long-term efficacy and safety of splenectomy in children with chronic idiopathic thrombocytopenic purpura (cITP). Patients and methods: Data from 33 splenectomized children were retrospectively analysed (median follow up period: 18.8 y from the removal of the spleen). The median age of children at splenectomy was 12 y and the median ITP duration 3.3 y. Indications for splenectomy were: persistent severe thrombocytopenia with extensive purpura, epistaxis and/or gum bleeds, menorrhagia (n = 5) and severe or recurrent haemorrhage from various sites (n = 11). Results: Eighty-five per cent of the patients showed an excellent (n = 26) or partial response to splenectomy. Five children (15%), all females, failed to respond. Of the responders, 25% experienced a transient recurrence of thrombocytopenia within 6 mo to 4 y from splenectomy. The mortality rate due to severe sepsis was 3%. However, the majority of the splenectomized patients have not so far suffered any severe or mild bacterial infection, despite incomplete vaccination and/or antibiotic prophylaxis.

Conclusion: Splenectomy remains the only effective therapeutic modality for children with cITP, although it is associated with transient recurrence and rarely with post-splenectomy sepsis, which could be fatal. Nonetheless, splenectomy should be the last treatment option for the cITP patient, after all available therapeutic modalities have been exhausted and the child still remains profoundly thrombocytopenic and symptomatic.     

Impact of inhaled corticosteroids on the risk of early Pseudomonas aeruginosa acquisition in cystic fibrosis

Aim: To investigate the role of inhaled corticosteroids (IC) on the risk of Pseudomonas aeruginosa acquisition before the age of 10 y in cystic fibrosis (CF) patients. Methods: For each subject the cumulative dose kg^-1 of IC received for each year of age was calculated until the end of follow-up. The age at CF diagnosis, the nutritional status (NS) and the number of respiratory exacerbations (RE) were used as surrogate measures for the severity of CF. Results: A total of 83 patients (40 M, 43 F) entered the study. Their median length of follow-up was 4.4 y, for a total of 386 person-years at risk. Twenty-three patients acquired P. aeruginosa at a median age of 4.6 y (range 0.4-9.9 y). The estimated survival without P. aeruginosa acquisition was 65% at 10 y of age. The effect of different risk factors (IC, NS, RE and age at CF diagnosis) on the probability of P. aeruginosa acquisition was evaluated: none of them was significantly associated with the risk of P. aeruginosa acquisition. In particular, patients receiving very high cumulative doses of IC (4th quartile) had a non-significantly increased risk of P. aeruginosa acquisition compared with those receiving low doses of IC (1st quartile) (hazard ratio = 1.73, 95% confidence limits 0.40-7.38).

Conclusion: This retrospective study was not able to demonstrate any role of IC in increasing the risk of P. aeruginosa acquisition. This complication seems to occur at a constant pace that is independent of CF severity and age. Prospective multi-institutional randomized studies are needed to investigate the effects of high-dose IC in CF patients.     

Effect of conservative management on the prevalence of surgery in patients with adolescent idiopathic scoliosis

STUDY DESIGN: Retrospective analysis of outcome in terms of prevalence of surgery for adolescent idiopathic scoliosis in patients receiving conservative management. OBJECTIVES: To determine whether a centre with an active policy of conservative management has fewer patients who eventually undergo surgery for adolescent idiopathic scoliosis than a centre where the practice is non-intervention. BACKGROUND DATA: The efficacy of orthoses for the treatment of idiopathic scoliosis was called into question in a recent publication. Because the prevalence of surgery in an untreated group of patients (28.1%) was not significantly different from that in a braced group (22.4%), the authors concluded that bracing appears to make no difference. Based on prior experience, this conclusion is questioned. METHODS: Since 1991, bracing and physical therapy have been recommended for children with adolescent idiopathic scoliosis at a centre in Barcelona, Spain. The scoliosis database was searched for patients with adolescent idiopathic scoliosis who were at least 15 years of age at last review and who had adequate documentation of the Cobb angle. The prevalence of surgery was compared with that of published data from a centre where the practice is non-intervention. RESULTS: From a total of 106 braced cases out of which 97 were followed up, six cases (5.6%) ultimately underwent spinal fusion. A worst case analysis, which assumes that all nine cases that were lost to follow-up had operations, brings the uppermost number of cases that could have undergone spinal fusion to 15 (14.1%). Either percentage is significant statistically when compared to the 28.1% reported surgeries from the centre with the policy of non-intervention. CONCLUSIONS: If conservative management does reduce the proportion of children with adolescent idiopathic scoliosis that require surgery, it can be said to provide a real and meaningful advantage to both the patients and the community. It is contended that conservative methods of treatment should never be ruled out from scoliosis management, because they can and do offer a viable alternative to those patients who cannot or will not opt for surgical treatment.     

Medical follow-up visits in adults 5-25 years after treatment for childhood acute leukaemia, lymphoma or Wilms' tumour

Aim: One aspect of organizing medical follow-up for adult survivors of childhood cancer is to determine to what extent the former patient experiences a need for health services. In the present paper, we studied how the healthcare needs, both subjectively and objectively, were fulfilled for our former patients. Methods: 335 survivors over 18 y of age, with a follow-up time of more than 5 y after completion of therapy, were sent a questionnaire probing their present use of health services. Results: The response rate was 73%. A majority (60%) of the survivors had no regular follow-up visits, and 42% of these reported that they missed not having one. More than one third were thus far dissatisfied with the follow-up programme. Only 3% of those who had regular follow-ups found them “unnecessary”. Complaints subjectively related to their diseases or treatments were reported by 47%. Out of all responders, 34% did not miss having regular follow-up visits. Neither perceived disease-related complaints nor radiation therapy was a predictor for having a scheduled follow-up visit.

Conclusion: In the absence of a long-term follow-up programme, many survivors were not receiving proper medical healthcare, whether from their perspective or from a professional one.     

Mother-infant skin-to-skin contact after delivery results in early recognition of own mother's milk odour

Aim: To determine the effects of mother-infant skin-to-skin contact immediately after birth on infant recognition of their own mother's milk odour and breastfeeding duration until 1 y of age. Methods: Sixty healthy, full-term neonates were randomly assigned to group A with skin-to-skin contact and group B without. One and 4 d after birth, infant responses to the following odour stimuli were observed: own mother's milk, another mother's milk, formula, orange juice and distilled water. Infant facial action was videotaped and the frequency of mouthing movements was evaluated for each stimulus. Nutritional assessment, focused particularly on breastfeeding, was performed every 3 mo on participating infants. Statistical analysis comparing the frequency of mouthing movements with the aforementioned five different odour exposures was performed by ANOVA with Fisher's PLSD. Kaplan-Meier analysis with a log-rank test was used to compare breastfeeding rates between groups. Results: Infants in both groups responded differently to mother's milk odour (either their own or another mother's milk) compared to the other stimuli on days 1 and 4. However, infants in group A demonstrated a larger difference in mouthing movements between their own and another mother's milk odour at 4 d of age (2.6 ± 1.6) compared to infants in group B (0.9 ± 2.0, p = 0.01). Infants in group A were breastfed an average of 1.9 mo longer than the others.

Conclusion: Our study provides evidence that mother-infant skin-to-skin contact for more than 50 min immediately after birth results in enhanced infant recognition of their own mother's milk odour and longer breastfeeding duration.     

Nontuberculous mycobacterial adenitis: effectiveness of chemotherapy following incomplete excision

Background: Management of lymphadenopathy caused by nontuberculous mycobacteria (NTM) is primarily surgical. Where this cannot achieve sufficient clearance of infected nodes, chemotherapy is often given. Aim: This study compared results of surgery alone with surgery followed by chemotherapy in instances where there was incomplete surgical removal of diseased tissue. Methods: Chemotherapy comprised azithromycin 10 mg/kg and rifabutin 6 mg/kg both given once daily for 6 mo. Ninety-eight children with NTM infection were seen in the period 1990-2004. Sixty-eight cases with adenopathy where “time to healing” (discharge stopped and inflammation settled) was known were available to compare response to treatment. Results: The median (range) “time to healing” in weeks for 43 patients who had surgery alone was: incision and drainage (I&D)/curettage 6 (1-72) (n=10); excision 3 (1-28) (n=22); and from the last operation of multiple (repeat) surgery 3 (1-40) (n=11). For 25 patients who required chemotherapy in addition to surgery, the median (range) “time to healing” in weeks was I&D/curettage 10 (1-40) (n=17), excision 14 (8-20) (n=2) and multiple surgery 29 (2-88) (n=6).

Conclusion: In children with adenitis due to NTM, where surgical resection is followed by continued discharge and inflammation, chemotherapy should be considered before further surgery is undertaken.     

Upper endoscopic findings in children with active juvenile chronic arthritis

Aim: To investigate the association between gastroduodenal mucosal damage and symptoms of the digestive tract in children with juvenile chronic arthritis (JCA) Methods: This was a prospective, open, non-randomized study. Gastroscopy was performed on 45 children with active JCA in 1996-2000. Gastrointestinal symptoms before and during the treatment were noted, as was the length of antirheumatic medication, for which the data were retrospectively assessed. Plasma haemoglobin (Hb) and mean corpuscular volume (MCV) levels and erythrocyte sedimentation rate (ESR) were analysed. Mucosal biopsies were obtained for histology and Helicobacter pylori culture. All patients were taking non-steroidal anti-inflammatory drugs (NSAIDs) and 11 (24.4%) were on peroral steroids; 16 (35.6%) were receiving hydorxychloroquine, 9 salazopyrine, 5 myocrisine and 14 methotrexate. Results: Seven children (15.6%) were found to have active inflammation in their gastric and/or duodenal mucosa, two having ulcers and two being infected with H. pylori. Abnormal endoscopic findings were more common in symptomatic children (n = 24) than in children without symptoms (n = 21) (75% vs 38%, p = 0.017). There was no clear association between the Hb or MCV level and the degree of gastroduodenal inflammation (p = 0.98 and 0.7, respectively). Significantly more children (66.6% vs 33.3%) experienced abdominal pain after beginning medical therapy than before therapy (p = 0.02).

Conclusion: Endoscopic evaluation of patients with JCA and receiving NSAIDs should be considered at least in symptomatic cases.     

Behavioural effects of phenylalanine-free amino acid tablet supplementation in intellectually disabled adults with untreated phenylketonuria

Aim: To evaluate the effects of phenylalanine (Phe)-free essential amino acid (AA) tablets enriched in tyrosine and tryptophan on the performance of intellectually disabled adult patients with untreated phenylketonuria (PKU). Methods: Phe-free AA tablets and placebo tablets were administered to 19 untreated PKU subjects on a normal diet for 6 mo in a prospective double-blinded crossover study. The adaptive behaviour of the patients was tested prior to the study and at 6 and 12 mo after the start, using a simplified version of the Vineland Adaptive Behaviour Scale. For each sub-domain, the patients were rated either “0” (for poor performance) or “1” (for good performance). Neurological signs and symptoms and specific behavioural characteristics were recorded monthly by caretakers. Every 6 mo, neurological examination of the patients was performed, and the caretakers were interviewed. The statistical significance of the results was tested by means of the Fisher's exact and Wilcoxon tests. Results: The most significant changes were an improved concentration and the development of a meaningful smile, which were observed in 44% and 43% of the patients on AA tablet treatment, respectively, but not patients on placebo. Other important but less significant changes included increased awareness of external stimuli (63%) and less self-injury (43%), and 40% were smiling and laughing occasionally. The mean overall rating increased from an initial value of 6.3 to 10.1 in patients when on AA tablet treatment (p=0.002), and to 7.0 in patients when on placebo (p=0.068). The difference between active AA treatment and placebo was statistically significant (p=0.027).

Conclusions: This pilot study suggests that Phe-free AA tablets enriched in tyrosine and tryptophan may improve the quality of life in some intellectually disabled adults with untreated PKU.     

Better care of immature infants; has it influenced long‐term pulmonary outcome?

Aims: To assess whether lung function in late childhood had improved in subjects born extremely prematurely in the early 1990s compared to the early 1980s, and whether neonatal factors in the respective periods had different impact on long-term pulmonary outcome. Design: Population-based, controlled cohort study. Lung function was determined in 81 of 86 (94%) eligible subjects born with gestational age ≤28 weeks or birthweight ≤1000 g in Western Norway in 1982-85 (n=46) and 1991-92 (n=35), and in 81 matched control subjects born at term. Results: The incidence of bronchopulmonary dysplasia was similar in the two periods. At follow-up, airway obstruction, hyper-responsiveness and pulmonary hyperinflation were similarly increased in both preterm cohorts compared to matched controls. Furthermore, current lung function was similarly related to neonatal respiratory disease in both birth-cohorts: FEV₁ was reduced with respectively 18.6% and 18.7% of predicted in preterms dependent on supplemental oxygen at 36 weeks postmenstrual age. Lack of antenatal treatment with corticosteroids and prolonged neonatal oxygen treatment predicted similar significant airway obstruction in the two birth-cohorts.
Conclusion: Preterms born in different eras of neonatology had similar long-term decreases in lung function. Long periods of oxygen supplementation are still required to salvage immature infants, and airway obstruction may still be a common long-term outcome.     

Graves' disease in children and adolescents: response to long-term treatment

Background: Optimal treatment of Graves' disease in paediatric patients is still a matter of controversy. Antithyroid drugs, radioiodine and thyroidectomy are the three therapeutic options available. Aim: To report our experience of long-term medical treatment and outcome of paediatric Graves' disease. Methods: A 5-y-long medical protocol was implemented in 20 children and adolescents with Graves' disease. All patients received antithyroid drugs as the first therapeutic option; patients who did not enter long-term remission received I¹³¹ and/or surgery as the definitive treatment. Results: The mean age at diagnosis was 12.1±4 y. Only two patients were males, both presenting concomitant type 1 diabetes. Mean follow-up was 13.8±5.5 y. Forty per cent of patients achieved long-term remission with low antithyroid drugs doses (mean treatment time: 5.4±1.4 y). Six patients received I¹³¹ as definitive treatment and another six underwent surgery after completing medical treatment for 6.8±4.1 and 5.1±2 y, respectively. No patients requiring high antithyroid drugs doses to maintain euthyroidism reached long-term remission and needed I¹³¹ and/or surgery.

Conclusion: Implementation of a long-term antithyroid drug protocol achieved 40% long-term remissions in paediatric patients with Graves' disease. Need for maintained high doses of antithyroid drugs could be considered a predictive factor for no remission. When permanent remission was not obtained by medical treatment, I¹³¹and/or surgery allowed healing in all cases.     

Perinatal risk factors of adverse outcome in very preterm children: a role of initial treatment of respiratory insufficiency?

Aim: To investigate risk factors of adverse outcome in a cohort of very preterm children treated mainly with nasal continuous positive airway pressure (CPAP) during the neonatal course. Methods: In Denmark, preterm children are treated with nasal CPAP as a first approach to respiratory support. A national prospective study of all infants with a birthweight below 1000 g or a gestational age below 28 wk born in 1994-1995 was initiated to evaluate this approach. Of the 269 surviving children 164 (61%) were not treated with mechanical ventilation in the neonatal period. A follow-up of the children at 5 y of age was conducted. Data from the neonatal period and the 5-y follow-up were analysed. Results: In multivariate analyses including 250 children, a severely abnormal neonatal brain ultrasound scan was predictive of cerebral palsy (OR = 19.9, CI 95%: 6.1-64.8) and intellectual disability (OR = 6.2, CI 95%: 2.3-16.5). A high Clinical Risk Index for Babies (CRIB) score (OR = 2.4, CI 95%: 1.1-5.5) and chronic lung disease (OR = 2.8, CI 95%: 1.2-6.9) were predictive of intellectual disability. In univariate analyses mechanical ventilation was associated with cerebral palsy (OR = 4.3, CI 95%: 1.7-10.8) and intellectual disability (OR = 2.2, CI 95%: 1.2-4.2), but the associations became insignificant in multivariate analyses including chronic lung disease and a severely abnormal ultrasound scan.

Conclusion: The associations between neonatal risk factors and adverse outcome in our cohort were very similar to those found in other cohorts with another initial treatment of respiratory insufficiency. We found no significant adverse effects of mechanical ventilation beyond what could be explained by associations with chronic lung disease and IVH 3-4/PVL.     

Weekly oral vitamin K prophylaxis in Denmark

Aim: To evaluate oral vitamin K prophylaxis at birth by giving 2 mg phytomenadione, followed by weekly oral vitamin K prophylaxis; 1 mg was administered by the parents until 3 mo of age. Methods: A total of 507 850 live babies were born in Denmark during the study period, November 1992 to June 2000. Of these infants, 78% and 22% received oral and intra-muscular prophylaxis, respectively; i.e. about 396 000 neonates received oral prophylaxis at birth. Weekly oral prophylaxis was recommended for all infants as long as they were mainly breastfed. A survey of possible cases of vitamin K deficiency bleeding (VKDB) was carried out by repeated questionnaires to all Danish paediatric departments and by checking the National Patient Register. Results: No cases of VKDB were revealed, i.e. the incidence was 0-0.9:100 000 (95% CI). The questionnaires were used to evaluate compliance with the regimen. Parents of 274 infants participated. A dose of vitamin K was regarded as having been given if the infant received a drop of vitamin K or was mostly formula-fed that week, and the prophylaxis was regarded as completed if the infant had received at least 9 doses. Compliance was good, with 94% of the infants completing the course of prophylaxis.

Conclusion: Weekly oral vitamin K supplementation during the first 3 mo of life was an efficient prophylaxis against VKBD. Parental compliance with the regimen was good.     

Lower leg growth suppression caused by inhaled glucocorticoids is not accompanied by reduced thickness of the cutis or subcutis

Background: Exogenous glucocorticoids suppress short-term lower leg growth in children as assessed by knemometry. The knemometric measurements, however, may be confounded by reductions in the thickness of the cutis and subcutis over the knee. Aim: To assess whether inhaled glucocorticoid-induced suppression of short-term growth is accompanied by changes in the thickness of the cutis and subcutis. Methods: The study was a randomized, controlled, crossover trial with 1 wk treatment, run-in and washout periods. Active treatment was inhaled budesonide 200 μg twice daily. Short-term growth was assessed by knemometry, and the thickness of the cutis and subcutis over the knee, on the volar forearm and abdomen was measured by 20 MHz B-mode ultrasound. Material: Nineteen children with asthma aged 7 to 13 y. Results: Lower leg growth was significantly reduced during budesonide treatment (0.27 mm/wk) compared to the treatment-free period (0.54 mm/wk) (p = 0.02, 95%: -0.50 to -0.05). Variations in the thickness of the cutis were seen during budesonide treatment (mean ± SEM): -0.01 ± 0.03 mm over the knee, -0.02 ± 0.02 mm on the forearm and 0.01 ± 0.02 mm on the abdomen. The variations in the total thickness of the cutis and subcutis were -0.05 ± 0.12 mm, 0.06 ± 0.12 mm and -0.06 ± 0.10 mm during budesonide treatment. The variations in thickness of the cutis or subcutis were not statistically different during budesonide treatment and the treatment free period in any anatomical location.

Conclusions: Short-term lower leg growth suppression induced by inhaled glucocorticoids is not confounded by variations in thickness of cutis or subcutis. The present observations further establishes knemometry as a reliable tool for assessment of the risk of growth suppression of inhaled glucocorticoids in children with asthma.     

Abnormalities in plasma fatty acid composition in human immunodeficiency virus-infected children treated with protease inhibitors

Aim: To study plasma fatty acid composition in human immunodeficiency virus-infected children treated with protease inhibitors and its relation with other components of the metabolic syndrome observed after this therapy. Design: Cross-sectional study from collected clinical database. Subjects: 17 children with HIV infection treated with protease inhibitors. Nine patients received ritonavir (20-30 mg/kg/d) and the remaining eight received nelfinavir (60-90 mg/kg/d). Duration of protease inhibitors treatment was 711±208 d. As controls, we used 112 matched blood samples from apparently healthy children admitted for minor surgical procedures. Methods: Plasma fatty acids were determined using a Hewlett Packard GC 5890 gas chromatograph. Results: Plasma levels of cholesterol and triglycerides and insulin-like growth factor 1 (IGF-1) tended to be high in protease inhibitor-treated patients. Plasma content of ω6 long-chain polyunsaturated fatty acids and, in particular, of the highly unsaturated 22:4ω6 and 22:5ω6, was significantly increased. Also, infected children had increased Δ6 and Δ4 desaturase activities and decreased Δ5 desaturase activity. Significant correlations were present between plasma IGF-1 level and plasma triglycerides, plasminogen activator inhibitor-1 activity and Δ6 desaturase activity.

Conclusion: HIV-infected, protease inhibitor-treated children exhibit a metabolic syndrome which is associated with significant changes in plasma fatty acid composition. These changes are similar to those observed in situations of insulin resistance and are linked to variations in plasma IGF-1 concentration.     

Methicillin-resistant Staphylococcus aureus bacteraemia in neonatal intensive care units: an analysis of 90 episodes

Aim: To delineate the clinical features of methicillin-resistant Staphylococcus aureus (MRSA) bacteraemia in infants hospitalized at the neonatal intensive care unit. Methods: Episodes of MRSA bacteraemia in Chang Gung Children's Hospital neonatal intensive care unit from 1997 to 1999 were reviewed for incidence, predisposing factors, clinical presentations, treatment and outcome. Results: Ninety episodes of MRSA bacteraemia were identified. The overall rate of MRSA bacteraemia was 1.05 per 1000 patient days during the 3-y period. Most of the patients were premature infants (76%), with prior operation or invasive procedures (39%), had an indwelling intravascular catheter (79%) and exposure to antibiotic therapy (96%). A localized cutaneous infection was found in 53.3% of the episodes. The most common clinical diagnoses were catheter-related infections (54.4%), skin and soft tissue infections (21.1%), bacteraemia without a focus (20%) and pneumonia (16.7%). Metastatic infection occurred in 18% of these infants. Among the patients treated with vancomycin for ≤14 d, 88.7% did not develop any complications, and 11.3% developed a recurrence.

Conclusions: MRSA is an established pathogen in our NICU. MRSA bacteraemia in the neonates predominantly presented as catheter-related infections, and metastatic infections were not infrequently seen. In uncomplicated MRSA bacteraemia, treatment with vancomycin for ≤14 d seems to be adequate.     

Thiamine-responsive megaloblastic anaemia syndrome: long-term follow-up and mutation analysis of seven families

Aim: Thiamine-responsive megaloblastic anaemia syndrome (TRMA) is the association of diabetes mellitus, anaemia and deafness, due to mutations in SLC19A2, encoding a thiamine transporter protein. This is a unique monogenic form of vitamin-dependent diabetes for which there is limited long-term data. We aimed to study genotype-phenotype relationships and long-term follow-up in our cohort. Methods: We have studied 13 patients from seven families and have follow-up data for a median of 9 y (2-30 y). Results: All patients originated from Kashmir or Punjab, and presented with non-immune, insulin-deficient diabetes mellitus, sensorineural deafness and a variable anaemia in the first 5 y of life, the anaemia progressing to megaloblastic and sideroblastic changes in the bone marrow. The anaemia and diabetes mellitus responded to oral thiamine hydrochloride 25 mg/d, but during puberty thiamine supplements became ineffective, and almost all patients require insulin therapy and regular blood transfusions in adulthood. All patients are homozygous for mutations in the SLC19A2 gene. We have identified a novel missense mutation (T158R) that was excluded in 100 control alleles.

Conclusion: Diabetes in this syndrome is due to an insulin insufficiency that initially responds to thiamine supplements; however, most patients become fully insulin dependent after puberty. A mutation screening strategy is feasible and likely to identify mutations in almost all cases.     

Hypercontractile heart failure caused by catecholamine therapy in premature neonates

Catecholamine treatment for hypotension is common practice in neonatal intensive care units. In the presence of left ventricular hypertrophy, the positive inotropic action of catecholamines with mainly β-receptor specificity can lead to excessive hypercontractility and paradoxical hypotension. This reports presents two cases of hypercontractile heart failure during β-agonist treatment in very low birthweight (VLBW) infants. Both patients (27 wk, 5 d; and 26 wk, 6 d of gestation) underwent surgical arterial duct ligation and coarctation repair. After operation they developed arterial hypotension that failed to respond to epinephrine (adrenaline) or dobutamine treatment. Echocardiography demonstrated a complete systolic obliteration of the left ventricular cavity. Epinephrine and dobutamine were stopped, and norepinephrine (noradrenaline) was successfully used to control the hypotension.

Conclusion: Treatment of hypotension with β-agonists in VLBW infants with left ventricular hypertrophy can lead to hypercontractility and left ventricular obliteration with paradoxical hypotension. Careful echocardiographic monitoring is indicated in such cases.