Adrenocortical suppression increases the risk of relapse in nephrotic syndrome.

OBJECTIVE: Children with nephrotic syndrome (NS) are usually treated with long-term low dose alternate day prednisolone with or without glucocorticoid sparing therapy, such as levamisole or ciclosporin, to maintain remission. The degree of hypothalamic-pituitary-adrenal axis (HPA) suppression with such therapeutic strategies has not been studied systematically. HPA suppression could cause a relapse or adrenal crisis. STUDY DESIGN: To study the risks of HPA suppression, a modified low dose synacthen test (0.5 mug) was administered to 32 patients (22 male,10 female) with a mean age of 9.7 years (range 3.8-17.6 years) with NS receiving long-term alternate day prednisolone for over 12 months. Twelve patients received alternate day prednisolone, 11 alternate prednisolone+levamisole and nine alternate prednisolone+ciclosporin. All patients were followed up for 3 years and the relapse rate noted. RESULTS: 20/32 (62.5%) patients had a peak serum cortisol concentration of <500 nmol/l, which suggested suboptimal cortisol secretion and possible HPA suppression. 10/12 children in the prednisolone group and 8/11 in the levamisole group had a suboptimal cortisol response compared with 2/9 in the ciclosporin group. During follow-up, the 20 children who had a suboptimal cortisol response had significantly more relapses (95 relapses) compared to the 12 children with a normal cortisol response who had 24 relapses (p = 0.01). CONCLUSIONS: Children with NS receiving long-term alternate day prednisolone therapy are at risk of developing HPA suppression and should be evaluated using the modified synacthen test. Children with evidence of HPA suppression are at a greater risk of relapse.     

Prednisolone versus dexamethasone in croup: a randomised equivalence trial

Background

Croup remains a common respiratory problem presenting to emergency departments. A single oral treatment of oral dexamethasone results in improved outcome. Prednisolone has similar pharmacokinetic properties and has a significant advantage in that it is commercially available in liquid preparations.

Objective

To ascertain whether a single oral dose of prednisolone was equivalent to a single oral dose of dexamethasone (matched for potency) in children with mild to moderate croup.

Design

A double blind, randomised, controlled equivalence trial

Setting

Tertiary paediatric emergency department.

Patients

133 children aged 3 to 142 months presenting with mild to moderate croup.

Interventions

Children received either a single oral dose of dexamethasone 0.15 mg/kg or single oral dose of prednisolone 1 mg/kg.

Outcome

The main outcome measure was unscheduled re‐presentation to medical care as determined by telephone follow up at 7 to 10 days. Croup score, adrenaline (epinephrine) use, time spent in the emergency department, and duration of croup and viral symptoms were secondary outcome measures.

Results

Children treated with prednisolone were more likely to re‐present: 19 of 65 children (29%) reattended medical care compared with 5 of 68 (7%) from the dexamethasone group. The confidence intervals around this 22% difference in outcome were 8% to 35%, outside the 0% to 7.5% range of equivalence. There were no significant differences in other outcome measures.

Conclusion

A single oral dose of prednisolone is less effective than a single oral dose of dexamethasone in reducing unscheduled re‐presentation to medical care in children with mild to moderate croup.     

No increased risk of relapse after meningococcal C conjugate vaccine in nephrotic syndrome

Objectives

To investigate whether meningococcal C conjugate vaccine (MCCV) caused relapse in children with steroid‐responsive nephrotic syndrome.

Design

A population‐based study was conducted using an active surveillance system, developed to assess adverse events following vaccination, which linked hospital record information on relapses of nephrotic syndrome to community child health population MCCV data. An ecological study looking at hospital admissions for nephrotic syndrome in different age cohorts of children before and after the MCCV introductory campaign was also carried out.

Settings

South East England, and England and Wales.

Patients

52 children having 162 relapses of nephrotic syndrome. Also, all hospital admissions of children aged 2–18 years with steroid‐responsive nephrotic syndrome in England and Wales between 1995 and 2003, relating admissions to when MCCV was introduced in specific age cohorts.

Main outcome measures and analysis method

Self‐controlled case series analysis looking for increased risk of relapse following MCCV and changes in admission rates for nephrotic syndrome (incidence ratio) following the introduction of MCCV to different age cohorts of children.

Results

There was no increased risk of relapse following MCCV in the self‐control case series, where a relative incidence of 0.95 (95% confidence interval (CI) 0.61–1.47) was found in the 6‐month post‐vaccination period, or in the ecological study, which gave an incidence rate ratio of 1.05 (95% CI 0.95 to 1.15) for the quarter when MCCV was introduced and the following two quarters.

Conclusions

We found no association between MCCV and nephrotic syndrome, which is therefore not a contraindication to meningococcal vaccination.     

Infant feeding in the second 6 months of life related to iron status: an observational study

Objective

To investigate the relationship between iron status in infancy and type of milk and weaning solids consumed.

Design

An observational cohort study.

Setting

928 term infants from the Avon Longitudinal Study of Parents and Children in 1993–94.

Methods

Haemoglobin and ferritin concentrations at 8 and 12 months were assessed in relation to type and quantity of milk intake at 8 months.

Results

By WHO criteria, 22.7% of the infants were anaemic at 8 months and 18.1% at 12 months. More breast‐ than formula‐fed infants were anaemic at 8 and 12 months. Cows'' milk as the main drink was associated with increased anaemia at 12 months and low ferritin at 8 and 12 months. No association was found between any nutrients and haemoglobin concentrations. Protein and non‐haem iron intakes were positively associated with ferritin concentrations and calcium intake negatively. This effect was more marked in infants being fed cows'' milk. More than 25% of infants in the breast milk and cows'' milk groups and 41% of infants having >6 breast feeds per day had iron intakes below the lower reference nutrient intake. Feeding cows'' milk or formula above 600 ml or >6 breast feeds per day was associated with lower intakes of solids.

Conclusions

Both breast and cows'' milk feeding were associated with higher levels of anaemia. Satisfactory iron intake from solids in later infancy is more likely if formula intake is <600 ml per day and breast feeds are limited to <6 feeds per day. Cows'' milk should be strongly discouraged as a main drink before 12 months.     

Prevalence of hypothalamic-pituitary-adrenal axis suppression in children treated for asthma with inhaled corticosteroid

OBJECTIVE:

To determine the prevalence of hypothalamic-pituitary-adrenal (HPA) axis suppression in asthmatic children on inhaled corticosteroids (ICS).

METHODS:

Clinical and demographic variables were recorded on preconstructed, standardized forms. HPA axis suppression was measured by morning serum cortisol levels and confirmed by low-dose adrenocorticotropic hormone stimulation testing.

RESULTS:

In total, 214 children participated. Twenty children (9.3%, 95% CI 5.3% to 13.4%) had HPA axis suppression. Odds of HPA axis suppression increased with ICS dose (OR 1.005, 95% CI 1.003 to 1.009, P<0.001). All children with HPA axis suppression were on a medium or lower dose of ICS for their age (200 μg/day to 500 μg/day). HPA axis suppression was not predicted by drug type, dose duration, concomitant use of long-acting beta-agonist or nasal steroid, or clinical features.

CONCLUSION:

Laboratory evidence of HPA axis suppression exists in children taking ICS for asthma. Children should be regularly screened for the presence of HPA axis suppression when treated with high-dose ICS (>500 μg/day). Consideration should be given to screening children on medium-dose ICS.     

Adrenal responses to low dose synthetic ACTH (Synacthen) in children receiving high dose inhaled fluticasone

Background and Aims

Clinical adrenal insufficiency has been reported with doses of inhaled fluticasone proprionate (FP) >400 μg/day, the maximum dose licensed for use in children with asthma. Following two cases of serious adrenal insufficiency (one fatal) attributed to FP, adrenal function was evaluated in children receiving FP outwith the licensed dose.

Methods

Children recorded as prescribed FP ⩾500 μg/day were invited to attend for assessment. Adrenal function was measured using the low dose Synacthen test (500 ng/1.73 m² intravenously) and was categorised as: biochemically normal (peak cortisol response >500 nmol/l); impaired (peak cortisol ⩽500 nmol/l); or flat (peak cortisol ⩽500 nmol/l with increment of <200 nmol/l and basal morning cortisol <200 nmol/l).

Results

A total of 422 children had been receiving FP alone or in combination with salmeterol; 202 were not investigated (137 FP within license; 24 FP discontinued); 220 attended and 217 (age 2.6–19.3 years) were successfully tested. Of 194 receiving FP ⩾500 μg/day, six had flat responses, 82 impaired responses, 104 were normal, and in 2 the LDST was unsuccessful. Apart from the index child, the other five with flat responses were asymptomatic; a further child with impairment (peak cortisol 296 nmol/l) had encephalopathic symptoms with borderline hypoglycaemia during an intercurrent illness. The six with flat responses and the symptomatic child were all receiving FP doses of ⩾ 1000 μg/day.

Conclusion

Overall, flat adrenal responses in association with FP occurred in 2.8% of children tested, all receiving ⩾1000 μg/day, while impaired responses were seen in 39.6%. Children on above licence FP doses should have adrenal function monitoring as well as a written plan for emergency steroid replacement.     

PFAPA syndrome: new clinical aspects disclosed

Background

The recently described PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis) syndrome is characterised by periodic fever, aphthous stomatitis, pharyngitis and adenitis. However, there are currently relatively few data on the natural history of this syndrome.

Objective

To describe the presentation, clinical course, doctors'' awareness, therapeutic response and long‐term follow‐up of children with PFAPA syndrome.

Methods

Children with PFAPA syndrome referred over a 5‐year period (from January 1999 to January 2004) were enrolled in the study. Data were gathered from medical records, parents'' interviews, physical examination and telephone calls.

Results

54 patients with PFAPA syndrome were evaluated. Our patients had a higher rate of abdominal pain (65%) and a lower rate of aphthous stomatitis (39%) than those in previous reports. Four different patterns of disease evolution were identified, including the relatively common (n = 14, 26%) and newly described course of alternating remissions and relapses. The remissions lasted 8.5 months on average (range 4–36 months). Diagnosis was established by primary paediatricians in 30 of 54 (56%) patients. However, a substantial delay in diagnosis was apparent (mean 15 months). Episodes were curtailed by a much lower dose of prednisone or equivalent corticosteroid (mean 0.6 mg/kg/day, range 0.15–1.5 mg/kg/day) than reported previously. Tonsillectomy was successful in the prevention of recurrence of further episodes in all six patients who underwent the procedure.

Conclusions

We describe several new characteristics of PFAPA syndrome in children, contributing to our knowledge of this relatively unrecognised but troublesome syndrome. Early diagnosis and appropriate treatment can markedly improve the quality of life of both patients and families.PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis) syndrome is characterised by recurrent episodes of fever associated with cervical adenitis, pharyngitis and aphthous stomatitis. The disease belongs to the group of periodic fever syndromes characterised by short episodes of illness that recur regularly for several years, alternating with healthy periods. The syndrome was first described in 1987¹ and the acronym PFAPA was subsequently coined in 1989.² Currently, most of the information gathered about the syndrome is based on only a few reports.^1,2,3,4This study was undertaken to investigate the epidemiological data, clinical course and long‐term follow‐up of children with PFAPA syndrome in central Israel. We were especially interested in aspects that were not described in previous reports, such as doctors'' awareness of the syndrome, adherence to corticosteroid treatment, effective corticosteroid dosage, unique side effects of corticosteroid treatment and a possible correlation between childcare attendance and occurrence of PFAPA syndrome.     

Vitamin K deficiency bleeding in Great Britain and Ireland: British Paediatric Surveillance Unit Surveys, 1993 94 and 2001-02

Objective

To conduct and report monitoring of vitamin K deficiency bleeding (VKDB) in Great Britain and Ireland following the 1988–90 survey (VKDB‐90).

Design

Two 2‐year surveys conducted during 1993–4 (VKDB‐94) and 2001–02 (VKDB‐02).

Setting

Data collected from all consultant paediatricians in Great Britain and Ireland.

Patients

All infants presenting with bleeding resulting from vitamin K (VK) deficiency.

Main outcome measures

Incidence of VKDB, related mortality/morbidity and VK prophylaxis recommended/received, noting predisposing features.

Results

Compared with previous studies, VKDB‐02 found fewer cases of VKDB (RR: 0.27 (95% CI: 0.12 to 0.59), p<0.001) with no deaths, no long‐term morbidity and reduced incidence among those receiving any oral dosing (RR: 0.24 (95% CI: 0.06 to 1.01), p<0.059). Breast‐fed infants accounted for the vast majority of cases. The number receiving no prophylaxis fell consecutively over time: 20 of 27 in VKDB‐90, 10 of 32 in VKDB‐94 and 4 (because of parental refusal) of 7 in VKDB‐02. Seven received one oral dose of VK in VKDB‐90, 16 in VKDB‐94 and none in VKDB‐02. Underlying liver disease was found in six cases in VKDB‐90, 12 in VKDB‐94 and one in VKDB‐02.

Conclusions

In the most recent survey, the incidence of VKDB was about one third that in the two earlier studies. Late onset VKDB remains virtually confined to breast‐fed infants who have received either no VK or just one oral dose. The effectiveness of oral prophylaxis regimens has improved over the last 15 years, but parental refusal of prophylaxis has become more problematic.     

Is omitting post urinary‐tract‐infection renal ultrasound safe after normal antenatal ultrasound? An observational study

Background

Guidelines recommend obtaining a renal ultrasonogram (RUS) for young children after a first urinary tract infection (UTI).

Objective

The aim of the current study was to assess the concordance of prenatal and post‐UTI RUS findings in children with a first simple UTI.

Methods

This was a prospective study and included all children aged 5 years or younger who were hospitalised with a first simple UTI (determined as clinical response and normalisation of temperature within 48 h on initiation of antibacterial therapy with no complications). Data were collected from each child regarding the results of prenatal and post‐UTI RUS.

Results

Overall, 250 children were included in the study and the results of late‐pregnancy and post‐UTI RUS were available for 84% (n = 209). Complete concordance between the two RUS was demonstrated in 96% (n = 201). The predictive value of normal antenatal to normal post‐UTI RUS was 96% (95% CI: 93% to 99%). These results include four children with mild transient pelvic dilatation. In eight children in whom renal anomalies were demonstrated only in post‐UTI RUS, the influence of these anomalies on the children''s management was negligible.

Conclusions

Prenatal‐RUS have been performed in most children <5 years old hospitalised with a first simple UTI. Concordance with post‐infection tests is very high. Findings which appear only in post‐infectious RUS usually have negligible effects on children''s management. Thus, in such children with normal antenatal RUS omitting post‐UTI RUS could be considered.     

Independent risk factors for chronic cyclosporine induced nephropathy in children with nephrotic syndrome

Background

Cyclosporine A (CsA) has been widely used in children with steroid dependent and steroid resistant nephrotic syndrome (NS) because of its efficacy in relieving these patients from systemic side effects of steroids. However, its long term use is controversial, since chronic CsA induced nephropathy (CsAN) may develop in a considerable number of patients.

Aims and Methods

In order to clarify the risk factors for the development of CsAN, the clinical characteristics of children with steroid dependent or steroid resistant NS taking CsA (target blood trough levels 50–150 ng/ml) for more than six months, managed at a single centre, were retrospectively analysed.

Results

Thirteen of 30 children (24 boys and 6 girls) taking CsA (mean duration 43 months, range 6–144) had CsAN defined as the presence of CsA associated arteriopathy with or without striped tubulointerstitial lesions. The multivariate analysis revealed that CsA treatment for more than 36 months and an age younger than 5 years at the start of CsA treatment were independent risk factors for the development of CsAN. The univariate analysis also showed that patients with CsAN had more frequent relapses during CsA treatment than those without CsAN.

Conclusion

An alternative treatment should be seriously considered after a 36 month administration of CsA in order to prevent CsAN. Data also suggest that CsA treatment in children younger than 5 years should be avoided if possible.     

Comparison of alteplase and heparin in maintaining the patency of paediatric central venous haemodialysis lines: a randomised controlled trial

Objectives

To determine whether the tissue plasminogen activator, alteplase, is more effective than heparin in preventing blood clots developing in children''s haemodialysis central lines between dialysis sessions.

Design

A prospective double‐blind, within‐patient multiperiod cross‐over controlled trial of instilling a “lock” of either heparin 5000 U/ml or alteplase 1 mg/ml into the central lines of two children haemodialysed twice weekly, and seven dialysed thrice weekly, over 10 weeks.

Setting

A UK paediatric nephrology unit.

Main outcome measures

Weight of blood clot aspirated from the line at the start of the next dialysis session.

Results

The odds of a clot forming was 2.4 times greater with heparin than alteplase (95% CI 1.4 to 4.0; p = 0.001), and when present they were 1.9 times heavier (31 vs 15 mg; 95% CI 1.5 to 2.4; p<0.0005). There was no effect of inter‐dialytic interval. One child required an alteplase infusion to clear a blocked line following a heparin lock. We subsequently changed our routine locks from heparin to alteplase. Comparing the year before and after that change, the incidence of blocked lines requiring an alteplase or urokinase infusion fell from 2.7 to 1.2 per child (p<0.03), and the need for surgical replacements from 0.7 to nil (p<0.02).

Conclusion

Alteplase is significantly more effective than heparin in preventing clot formation in central haemodialysis lines. This reduces morbidity and improves preservation of central venous access. It is more expensive, though relatively economic if packaged into syringes and stored frozen until needed, but reduces the costs of unblocking or replacing clotted lines.     

Nasopharyngeal aspiration for diagnosis of pulmonary tuberculosis

Background

Confirmation of pulmonary tuberculosis (PTB) in young children is difficult as they seldom expectorate sputum.

Aim

To compare sputa obtained by nasopharyngeal aspiration and by sputum induction for staining and culture of Mycobacterium tuberculosis.

Patients and methods

Patients from Mulago Hospital, Kampala with symptoms suggestive of PTB were considered for inclusion in the study. Those with a positive tuberculin test and/or a chest radiograph compatible with tuberculosis were recruited. Infection with human immunodeficiency virus (HIV) was confirmed by duplicate enzyme‐labelled immunosorbent assay or in children <15 months by polymerase chain reaction (PCR). Direct PCR was undertaken on 82 nasopharyngeal aspirates.

Results

Of 438 patients referred, 94 were recruited over a period of 5 months. Median (range) age was 48 (4–144) months. Of 63 patients tested, 69.8% were infected with HIV. Paired and uncontaminated culture results were available for 88 patients and smear results for 94 patients. Nasopharyngeal aspirates were smear‐positive in 8.5% and culture‐positive in 23.9%. Induced sputa were smear‐positive in 9.6% and culture positive in 21.6%. Overall, 10.6% were smear‐positive, 25.5% were culture‐positive and 26.6% had smear and/or culture confirmed tuberculosis. Direct PCR on nasopharyngeal aspirates had a sensitivity of 62% and specificity of 98% for confirmation of culture‐positive tuberculosis.

Conclusions

Nasopharyngeal aspiration is a useful, safe and low‐technology method for confirmation of PTB and, like sputum induction, can be undertaken in outpatient clinics.     

Severe receptive language disorder in childhood--familial aspects and long-term outcomes: results from a Scottish study

Background and aims

Little is known about the familial characteristics of children with severe receptive specific language impairment (SLI). Affected children are more likely to have long‐term problems than those with expressive SLI but to date they have only been described as small cohorts within SLI populations. We therefore aimed to describe the clinical and familial characteristics of severe receptive SLI as defined by a rigorous phenotype and to establish whether non‐word repetition showed a relationship with language impairment in these families.

Methods

Cross‐sectional study of children who met ICD‐10 (F80.2) criteria for receptive SLI at school entry, their siblings and genetic parents with standardised measures of language and non‐verbal IQ, phonological auditory memory and speech sound inventory.

Results

At a mean of 6 years after school entry with a severe receptive SLI, the 58 participants had a normal mean and standard deviation non‐verbal IQ, but only 3% (two) had attained language measures in the normal range. One third still had severe receptive language impairment. One third of siblings not known to be affected had language levels outside the normal range. Phonological auditory memory was impaired in most family members.

Conclusion

Severe receptive SLI is nearly always associated with an equally severe reduction in expressive language skills. Language impairment in siblings may go undetected and yet they are at high risk. Family members had weak phonological auditory memory skills, suggesting that this could be a marker for language acquisition difficulties. Receptive SLI rarely resolves and trials of therapy are urgently needed.     

Randomised trial of a parenting intervention during neonatal intensive care

Objective

To evaluate the influence of parenting intervention on maternal responsiveness and infant neurobehavioural development following a very premature birth.

Design

Cluster‐randomised controlled trial, with a crossover design and three‐month washout period.

Setting

Six neonatal intensive care units.

Patients

Infants born <32 weeks'' gestation.

Intervention

The Parent Baby Interaction Programme (PBIP) is a supportive, educational intervention delivered by research nurses in the neonatal intensive care unit, with optional home follow‐up for up to six weeks after discharge.

Main outcome measures

Parenting stress at 3 months adjusted age, as measured by the Parenting Stress Index (PSI). Other outcomes included the Neurobehavioural Assessment of the Preterm Infant (NAPI) and maternal interaction as assessed by the Nursing Child Assessment Teaching Scale (NCATS) and the responsivity subscale for Home Observation for Measurement of the Environment (HOME).

Results

112 infants were recruited in the intervention phases and 121 in the control phases. Mean standardised NAPI scores at 35 weeks did not differ between the PBIP and control groups. Both groups had low but similar NCATS caregiver scores before discharge (36.6 in the PBIP group and 37.4 in control, adjusted mean difference −0.7, 95% CI −2.7 to 1.4). At three months, adjusted age mean PSI scores for the PBIP group were 71.9 compared with 67.1 for controls (adjusted mean difference 3.8, 95% CI −4.7 to 12.4). NCATS scores and HOME responsivity scores were similarly distributed between the groups.

Conclusion

This early, nurse‐delivered, parent‐focused interaction programme intervention had no measurable effects on short‐term infant neurobehavioural function, mother–child interaction or parenting stresses.     

Ethnic and gender differences in body fat in British schoolchildren as measured by DXA

Background

There are known to be ethnic differences in body composition in adults which are related to ethnic differences in adult disease.

Objectives

To evaluate gender and ethnic differences in percentage body fat in British schoolchildren and to compare these differences with classification of obesity using body mass index (BMI) criteria.

Design

A cross‐sectional study of 1251 healthy children and adolescents aged 5–18 years from white, South Asian and African‐Caribbean ethnic groups. Percentage body fat was determined by dual x ray absorptiometry and the subjects classified using BMI criteria for overweight and obesity.

Results

Significant gender differences in percentage body fat were seen, with girls having higher values from the age of 5 years. Girls had 3.8% higher percentage body fat at 5 years of age increasing to 12.9% at 18 years of age. Significant ethnic differences were found, with South Asian girls and boys having the highest percentage body fat from 5 and 7 years of age, respectively. These differences increased with age, being most significant in the teenage years. Although South Asian girls and boys were over‐represented in the group containing children with more than 25% body fat (p<0.0001, χ² test), African‐Caribbean subjects were more likely to be classified as obese using BMI criteria.

Conclusions

There are clear gender and ethnic differences in percentage body fat in British schoolchildren which may relate to known differences in the risk of type 2 diabetes in adolescence and adulthood. BMI criteria for defining overweight and obesity do not accurately identify ethnic differences in body fat.     

Prevalence rates for constipation and faecal and urinary incontinence

Objective

To evaluate the prevalence rates for constipation and faecal and urinary incontinence in children attending primary care clinics in the United States.

Methods

Retrospective review of case records of all children, 4–17 years of age, seen for at least one health maintenance visit during a 6 month period and followed from birth or within the first 6 months of age in our clinics. We reviewed all charts for constipation, faecal incontinence and urinary incontinence.

Results

We included 482 children in the study, after excluding 39 children with chronic diseases. The prevalence rate for constipation was 22.6% and was similar in boys and girls. The constipation was functional in 18% and acute in 4.6%. The prevalence rate for faecal incontinence (⩾1/week) was 4.4%. The faecal incontinence was associated with constipation in 95% of our children. The prevalence rate for urinary incontinence was 10.5%; 3.3% for daytime only, 1.8% for daytime with night‐time and 5.4% for night‐time urinary incontinence. Faecal and urinary incontinence were significantly more commonly observed in children with constipation than in children without constipation.

Conclusion

The prevalence rates were 22.6% for constipation, 4.4% for faecal incontinence and 10.5% for urinary incontinence in a US primary care clinic. Children with constipation had higher prevalence rates for faecal and urinary incontinence than children without constipation. Boys with constipation had higher rates of faecal incontinence than girls with constipation.     

Severity of meningococcal infections is related to anthropometrical parameters

Background

Invasive meningococcal infections remain an important cause of death in children. In addition, malnutrition has been classically associated with increased severity of infectious diseases. However, in our experience lethal meningococcaemia in clinically malnourished children is extremely rare. Our purpose was to determine whether there is an association between nutritional status and outcome in children with invasive meningococcal infection.

Methods

We carried out an observational study and prospectively determined anthropometrical parameters in 127 children aged 1 month to 4 years with invasive meningococcal infection seen in our inpatient facilities from August 1999 to May 2004. Severity and survival were the clinical end points analysed.

Results

Children with severe disease had higher weight for age (1.02 vs −0.19) and height for age (1.12 vs −0.58) z scores than those with non‐severe disease. Non‐survivors had higher weight for age (0.90 vs −0.16) and height for age (0.73 vs −0.57) z scores than survivors. Clinical and biological variables usually accepted as predictors of high mortality or severity in patients with meningococcal infection were not significantly associated with weight for age and height for age z scores.

Conclusion

In the present prospective series of children with invasive meningococcal disease, severity and death were linked to anthropometrical parameters and seemed to be associated with a very good nutritional status, which confirmed our previous uncontrolled observations.     

High prevalence of vitamin D deficiency in newborn infants of high-risk mothers

Objective

To determine the prevalence of vitamin D deficiency in newborn infants of mothers at risk of vitamin D deficiency because of dark skin or the wearing of concealing clothes (such as a veil) compared with a group presumed not to be at risk. A second aim was to correlate these newborn infants'' vitamin D concentrations with biochemical parameters of vitamin D metabolism and bone turnover at birth.

Design

A prospective study conducted between April 2004 and February 2006 including women delivering during this period and their newborn infants.

Setting

The outpatient clinic of the obstetrics department, Sint Franciscus Gasthuis, Rotterdam, the Netherlands.

Patients

Eighty seven newborn infants of healthy mothers with either dark skin and/or concealing clothing (risk group) or light skin (control group).

Results

We found a significant difference in the prevalence of vitamin D deficiency (25‐hydroxyvitamin D₃ <25 nmol/l) between newborn infants of mothers at risk and those of mothers in the control group (63.3% vs 15.8%; p<0.001). Mean alkaline phosphatase concentrations were significantly higher in the at risk group.

Conclusions

Newborn infants of mothers with dark skin or wearing concealing clothes are at great risk of vitamin D deficiency at birth. The clinical implications are unknown. Further research is necessary to determine the long‐term consequences of maternal and neonatal vitamin D deficiency so that guidelines on vitamin D supplementation during pregnancy can be issued.     

Congenital adrenal hyperplasia due to 21-hydroxylase deficiency is associated with a prolonged gestational age

Background

The timing of parturition in most mammals is thought to be linked to a late gestational rise in corticosteroid production by the fetal adrenal gland. We hypothesised that gestational age would be prolonged in our patients with impaired cortisol production secondary to congenital adrenal hyperplasia (CAH) due to 21‐hydroxylase deficiency.

Methods

We compared the gestational age of patients affected by salt‐wasting CAH due to 21‐hydroxylase deficiency (born 1978–2004; n = 31) with that of children with congenital hypothyroidism (born 1981–2003; n = 30) and a control group of short normal children (born 1980–2002; n = 120). Each group was compared with national (England 2002–3) and regional (2003–4) data on gestational age from hospital episode statistics. Post‐term delivery was defined as birth beyond 41 completed weeks.

Results

National statistics reveal a frequency of 4.4% for singleton deliveries beyond 41 weeks. In our region the frequency was 4.6%. In the group of children with CAH, the frequency of post‐term delivery was 19.3% (p<0.001). In patients with congenital hypothyroidism, the frequency was 13.3% (p = 0.02). The proportion of short children who did not have a recognised endocrinopathy born post term was comparable to national and regional data at 6.7%.

Conclusions

A prolonged gestation is more likely in pregnancies where the fetus has the salt‐wasting form of CAH. This may be due to impaired cortisol production, although other changes in steroidogenesis may also be contributory.