Diagnostic assessment of haemorrhagic rash and fever

AIMS—To establish criteria for early distinction between meningococcal disease and other conditions with similar clinical features, and to identify other causes for haemorrhagic rashes accompanied by fever.
METHODS—In a prospective study, 264 infants and children hospitalised with fever and skin haemorrhages were studied.
RESULTS—We identified an aetiological agent in 28%: 15% had meningococcal disease, 2% another invasive bacterial infection, 7% enterovirus infection, and 4% adenovirus infection. Five clinical variables distinguished between meningococcal disease and other conditions on admission: (1) skin haemorrhages of characteristic appearance; (2) universal distribution of skin haemorrhages; (3) maximum diameter of one or more skin haemorrhages greater than 2 mm; (4) poor general condition (using a standardised observation scheme); and (5) nuchal rigidity. If any two or more of these clinical variables were present, the probability of identifying a patient with meningococcal disease was 97% and the false positive rate was only 12%. This diagnostic algorithm did not identify children in whom septicaemia was caused by other bacterial species.

     

Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders

AIMS—To review the precipitating events, clinical features, treatment, and outcome of macrophage activation syndrome (MAS).
METHODS—Retrospective review of cases of MAS from a prospectively collected database of children with rheumatic diseases from 1980to 2000.
RESULTS—Nine patients (eight girls) were considered to have evidence of MAS. The primary diagnosis was systemic onset juvenile idiopathic arthritis in seven, enthesitis related arthritis in one, and chronic infantile neurological cutaneous articular syndrome in one. Mean age of onset was 5.7 years, and duration prior to MAS, 4.2 years. No medication was identified as a trigger. Eight had infections prior to MAS; specific infectious agents were identified in four. High grade fever, new onset hepatosplenomegaly, and lymphadenopathy were common clinical features. Platelet counts fell dramatically, from an average of 346 to 99 × 10⁹/l. Mean erythrocyte sedimentation rate (in three patients) fell from 115 to 28 mm/h. Eight had abnormal liver function during the disease course, and six had coagulopathy. Bone marrow examination supported the diagnosis with definite haemophagocytosis in four of seven. All received high dose steroids (eight intravenous, one oral), five cyclosporin, two cyclophosphamide, and one antithymocyte globulin. Two of three patients with significant renal impairment died.
CONCLUSION—MAS is a rare and potentially fatal complication of childhood rheumatic disorders. Most of our patients were female, and most cases were preceded by infection. Bone marrow studies support the diagnosis. Deranged renal function may be a poor prognostic sign. Aggressive early therapy is essential.
     

Usefulness of antinuclear antibody testing to screen for rheumatic diseases

OBJECTIVE—To assess the usefulness of the indirect immunofluorescence antinuclear antibody test (FANA) using human laryngeal epithelial carcinoma cells as nuclear substrate, to screen for childhood rheumatic diseases.
STUDY DESIGN—A review of all FANA tests performed on children at British Columbia''s Children''s Hospital between 7 March 1991 and 31 July 1995.
RESULTS—FANA tests were positive at titres of 1:20 or greater in 41% of all subjects tested, and in 65% of all subjects in whom the diagnosis was obtained. FANA positivity occurred in 67% of those with a rheumatic disease, compared with 64% of those with a non-rheumatic disease (p=0.4). More girls had high titre FANA positivity than boys independent of whether or not they had a rheumatic disease (p=0.05). At a screening serum dilution of 1:40 a positive test has a sensitivity of only 0.63, and a positive predictive value of only 0.33 for any rheumatic disease. For systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), or overlap syndrome at a screening dilution of 1:40 the test has a very high sensitivity of 0.98, but a very low positive predictive value of only 0.10,the test having slightly better characteristics for boys than girls.
CONCLUSION—Although a negative FANA test makes a diagnosis of SLE or MCTD extremely unlikely, a positive test even at moderately high titres of 1:160 or higher is found so frequently in children without a rheumatic disease that a positive result has little or no diagnostic value. It is suggested that a screening serum dilution of 1:160 or 1:320 would increase the usefulness of the test, by decreasing false positive tests, without significantly increasing false negative tests for SLE or MCTD, and would have the potential for considerable cost savings.

     

Paediatric presentation of type 2 neurofibromatosis

BACKGROUND—Neurofibromatosis type 2 (NF2) is a highly penetrant autosomal dominant condition predisposing affected individuals to schwannomas and meningiomas. The proportion of children presenting with meningioma or schwannoma who have NF2 is not well described, and neither is the mode of presentation in most children with the inherited disease.
AIMS—To determine the frequency of childhood meningioma and schwannoma cases caused by NF2 and the mode of presentation.
METHODS—The records of the Manchester Children''s Tumour Registry from 1954 were searched for cases of meningioma and schwannoma. Paediatric presentation in a large UK series of NF2 was also studied.
RESULTS—18% (61/334) of patients with NF2 on the UK database presented in the paediatric age group (0-15 years), frequently with the symptoms of an isolated tumour. More than half had no family history to alert the clinician to their susceptibility. Three of 22 children presenting with a meningioma on the Manchester Children''s Tumour Registry have gone on to develop classic features of NF2.
CONCLUSIONS—Clinicians should suspect NF2 in children presenting with meningioma, schwannoma, and skin features, such as neurofibromas/schwannomas, but fewer than 6 café au lait patches, who thus fall short of a diagnosis of neurofibromatosis type 1.
     

The first attack of acute rheumatic fever in childhood--clinical and laboratory profile

One hundred consecutive cases of 'first attack' of acute rheumatic fever were studied. There were 52 males and 48 females, constituting 1.12% of total hospital admissions. Nearly 10% of children were below the age of 5 years, stressing the early onset of rheumatic fever in tropics. Only 47% gave a definite history of overcrowding at home. Sore throat was present in 67%, overt arthritis in 66%, carditis in 57%, arthralgia alone in 22% of which 45.45% had carditis. Small joint involvement was noticed in 23% of cases of which 73.91% had carditis. Only 33.33% had congestive cardiac failure. Ten per cent of children had chorea, while subcutaneous nodules were seen in 4% of cases, all of whom had associated carditis. Erythrocyte sedimentation rate (ESR) showed good correlation with clinical profile. Throat cultures were positive for beta hemolytic streptococci only in 12% of cases. Anti-streptolysin 'O' (ASO) titre showed significant titres on 68% of cases, anti-deoxyribonuclease "B" (ADN-B) in 69.32%, antibody to group A carbohydrate (ACHO) in 70.65%. ASO, ADN-B, and ACHO titres together gave 87.5% positivity while estimations in paired sera showed ASO 79.54%, ADN-B 82.27% and ASO, ADN-B together 99.92% significant titres. Study of blood groups showed A group children to be more vulnerable to rheumatic fever (37.5%) and rheumatic carditis (47.37%). Mortality in the present study was nil.     

Pancreatic exocrine and endocrine function after pancreatectomy for persistent hyperinsulinaemic hypoglycaemia of infancy

OBJECTIVE—To investigate mortality of children diagnosed with insulin dependent diabetes mellitus (IDDM) and to identify common factors before death.
DESIGN—Follow up of a population based cohort of children diagnosed with IDDM to ascertain deaths.
SETTING—Children were diagnosed in Yorkshire but followed up throughout the United Kingdom.
SUBJECTS—From the Yorkshire Children''s Diabetes Register details of 1854 children aged 0-16 years (1978-93) were submitted to the NHS Central Register.
MAIN OUTCOME MEASURE—Notification and causes of death.
RESULTS—98.3% of cases were traced and 26 deaths identified. Follow up ranged from 1-18 years (median 9.3 years), providing 17 350 person-years of IDDM. Fifteen deaths (58%) were attributed to diabetes or its complications; 11 (42%) were unrelated and included one suicide. For mortality from all causes, the standardised mortality ratio (SMR) of 247 (95% confidence interval (CI) 163 to 362) was significantly increased for those under 34 years. The largest number of deaths (n = 10) occurred in the 15-19 year age range, with an SMR of 442 (95% CI 209 to 802). Case note examination showed a clear tendency towards poor diabetic control, and worries over control were expressed before death by health care professionals.
CONCLUSIONS—Despite advances in treatment, IDDM still carries an increased mortality for young people, particularly in the "transition" age range.

     

Diagnosis, classification, and management of erythema multiforme and Stevens-Johnson syndrome

BACKGROUND—In adults, erythema multiforme (EM) is thought to be mainly related to herpes infection and Stevens-Johnson syndrome (SJS) to drug reactions.
AIMS—To investigate this hypothesis in children, and to review our experience in the management of these patients.
METHODS—A retrospective analysis of 77 paediatric cases of EM or SJS admitted to the Children''s Hospital in Bordeaux between 1974and 1998.
RESULTS—Thirty five cases, inadequately documented or misdiagnosed mostly as urticarias or non-EM drug reactions were excluded. Among the remaining 42 patients (14 girls and 28 boys), 22 had EM (11EM minor and 11 EM major), 17 had SJS, and three had isolated mucous membrane involvement and were classified separately. Childhood EM was mostly related to herpes infection and SJS to infectious agents, especially Mycoplasma pneumoniae. Only two cases were firmly attributed to drugs (antibiotics). No patient died. EM and SJS sequelae were minor and steroids were of no overall benefit.
CONCLUSION—In paediatric practice EM is frequently misdiagnosed. The proposal that SJS is drug related in adults does not apply to children, and in our recruitment EM and SJS are mostly triggered by infectious agents. The course of both diseases, even though dramatic at onset, leads to low morbidity and mortality when appropriate symptomatic treatment is given.

     

The prognosis of childhood abdominal migraine

AIMS—To determine the clinical course of childhood abdominal migraine, seven to 10 years after the diagnosis.
METHODS—A total of 54 children with abdominal migraine were studied; 35 were identified from a population survey carried out on Aberdeen schoolchildren between 1991 and 1993, and 19 from outpatient records of children in the same age group who had attended the Royal Aberdeen Children''s Hospital. Controls were 54 children who did not have abdominal pain in childhood, matched for age and sex, obtained from either the population survey or the patient administration system. Main outcome measures were presence or resolution of abdominal migraine and past or present history of headache fulfilling the International Headache Society (IHS) criteria for the diagnosis of migraine.
RESULTS—Abdominal migraine had resolved in 31 cases (61%). Seventy per cent of cases with abdominal migraine were either current (52%) or previous (18%) sufferers from headaches that fulfilled the IHS criteria for migraine, compared to 20% of the controls.
CONCLUSIONS—These results support the concept of abdominal migraine as a migraine prodrome, and suggest that our diagnostic criteria for the condition are robust.

     

What do parents of wheezy children understand by "wheeze"?

BACKGROUND—Reported wheeze is the cornerstone of asthma diagnosis.
AIMS—To determine what parents understand by wheeze.
METHODS—Two studies were undertaken: (1) Parents of clinic attendees with reported wheeze (n=160) were asked by questionnaire what they understood by "wheeze" and how they knew their child was wheezy. Responses were compared to definitions of wheeze in 12epidemiology studies and their response options. (2) The extent of agreement of parents'' reports (n=139) of acute wheezing in their children and clinicians'' findings of "wheeze" and "asthma" was examined.
RESULTS—(1) "Sound" and "difficulty in breathing" were perceived central to "wheeze". "What you hear" was not selected by 23% (95% confidence interval (CI) 16-30%). "Whistling" was mentioned by 11% (CI 6-15%) but featured in 11 of 12 epidemiology questionnaires. (2) There was les than 50% agreement between parents'' and clinicians'' reports of wheeze and asthma.
CONCLUSIONS—Conceptual understandings of "wheeze" for parents of children with reported wheeze are different from epidemiology definitions. Parents'' reports of acute wheeze and clinicians'' findings also differ.

     

The prognosis of cyclical vomiting syndrome

AIMS—The medium term prognosis of cyclical vomiting syndrome (CVS) was studied to determine the proportion of affected individuals who had gone on to develop headaches fulfilling the International Headache Society criteria for migraine.
METHODS—Twenty six (76%) of 34 CVS sufferers identified from the authors'' clinical records were traced, and all agreed to participate. Each child was matched to a control, and telephone interviews were conducted using a standardised questionnaire.
RESULTS—Thirteen (50%) of the subjects had continuing CVS and/or migraine headaches while the remainder were currently asymptomatic. The prevalence of past or present migraine headaches in subjects (46%) was significantly higher than in the control population (12%).
CONCLUSION—Results support the concept that CVS is closely related to migraine.

     

Birth weight and cognitive function at age 11 years: the Scottish Mental Survey 1932

AIMS—To examine the relation between birth weight and cognitive function at age 11 years, and to examine whether this relation is independent of social class.
METHODS—Retrospective cohort study based on birth records from 1921 and cognitive function measured while at school at age 11 in 1932.Subjects were 985 live singletons born in the Edinburgh Royal Maternity and Simpson Memorial Hospital in 1921. Moray House Test scores from the Scottish Mental Survey 1932 were traced on 449of these children.
RESULTS—Mean score on Moray House Test increased from 30.6 at a birth weight of <2500 g to 44.7 at 4001-4500 g, after correcting for gestational age, maternal age, parity, social class, and legitimacy of birth. Multiple regression showed that 15.6% of the variance in Moray House Test score is contributed by a combination of social class (6.6%), birth weight (3.8%), child''s exact age (2.4%), maternal parity (2.0%), and illegitimacy (1.5%). Structural equation modelling confirmed the independent contribution from each of these variables in predicting cognitive ability. A model in which birth weight acted as a mediator of social class had poor fit statistics.
CONCLUSION—In this 1921 birth cohort, social class and birth weight have independent effects on cognitive function at age 11. Future research will relate these childhood data to health and cognition in old age.

     

Complications of varicella in a defined central European population

AIMS—To describe complications of varicella requiring hospitalisation in a defined population (canton of Bern) and to compare the hospitalisation rates for varicella with published data.
METHODS—Retrospective analysis of hospital records of patients less than 16 years of age admitted with complications of varicella to the hospitals serving this population (University Children''s Hospital of Bern and the Wildermeth Children''s Hospital of Biel, Switzerland), and calculation of hospitalisation rates for varicella and its complications based on birth rates and varicella antibody prevalence rates.
RESULTS—From 1986 to 1996, 113 cases (median age, 5.6 years) were identified. Younger siblings were overrepresented (odds ratio (OR), 1.42; 95% confidence interval (CI), 1.09 to 1.84). Central nervous system (CNS) complications (26 patients; 23%) were found predominantly in previously healthy children (relative risk, 7.1; 95% CI, 1.01to 49.86). Group A β haemolytic streptococci were recovered from only one of 35 patients with bacterial complications. The hospitalisation rates for primary varicella (9.2/10⁴ cases; 95% CI, 7.4 to 11/10⁴), skin infections (2.0/10⁴ cases; 95% CI, 1.2 to 2.9/10⁴), and pneumonia (0.8/10⁴ cases; 95% CI, 0.3 to 1.3/10⁴) were significantly lower than reported previously. The CNS complication rate (2.2/10⁴ cases; 95% CI, 1.3 to 3.1/10⁴) was among the highest rates reported.
CONCLUSIONS—The low hospitalisation rate in comparison with studies from elsewhere indicates that there is a large regional variability in complications associated with varicella. Such data should be taken into consideration when local varicella immunisation strategies are developed.

     

Faecal candida and diarrhoea

BACKGROUND—Candida species are frequently isolated from stools of children with diarrhoea but are not proven enteropathogens. It is hypothesised that faecal candida causes diarrhoea.
AIMS—To determine the prevalence of faecal candida in childhood diarrhoea and the relation between faecal yeasts and diarrhoea.
METHODS—Comparison of clinical and laboratory data, including quantitative stool culture for yeasts from 107 children hospitalised with diarrhoea and 67 age matched controls without diarrhoea.
RESULTS—Yeast species, predominantly candida, were identified in the stools of 43 children (39%) with diarrhoea and 26 (36%) without diarrhoea. The concentration of candida was positively associated with recent antibiotic use (p = 0.03) and with the presence of another enteric pathogen (p < 0.005), but not with patient age, nutritional status, or duration of diarrhoea.
CONCLUSION—Candida species do not cause childhood diarrhoea in well nourished children.

     

The abnormal nucleus as a cause of congenital facial palsy

BACKGROUND—Congenital facial palsy (CFP) is clinically defined as facial palsy present at birth. It is associated with considerable disfigurement and causes functional and emotional problems for the affected child. The aetiology of the majority of cases however, remains elusive.
AIMS—To investigate the role of a neuroanatomical abnormality as a cause of unilateral CFP.
METHODS—Magnetic resonance imaging (MRI) scans were performed on 21 patients with unilateral CFP. Fifteen patients had unilateral CFP only; six suffered from syndromes which can include unilateral CFP.
RESULTS—Of the 15 patients with unilateral CFP only, four (27%) had an abnormal nucleus or an abnormal weighting of this area on the MRI scan, compared to one (17%) of the remaining six patients.
CONCLUSION—Developmental abnormalities of the facial nucleus itself constitute an important, and previously ignored, cause of monosymptomatic unilateral CFP.

     

Outcome of psychiatric intervention in factitious illness by proxy (Munchausen's syndrome by proxy)

OBJECTIVE—To determine the outcome for children after psychiatric intervention in cases of factitious illness by proxy.
METHODS—All 17 children from 16 families, selected for admission to the Park Hospital Oxford family unit 1992-96 were followed up after a mean of 27 months. Information was obtained on the children and their carers from general practitioners, social workers or both; 13 of the children and carers were interviewed.
RESULTS—All patients were at the severe end of the abuse spectrum; 12 involving direct induction of illness, 1 tampering with samples to mimic illness, and 4 fabrication of symptoms. The biological mother was the abuser in all cases. Four children and their parents had been initially admitted for assessment, and 13 for treatment to decide whether family reunification was viable. The 4 assessments clarified diagnosis, enabling improved care plans to be made. Of the 13 treatment cases, 10 were reunited with parents after a mean of 71/2 weeks'' admission, whereas 3 were discharged to out of home care. There was a further episode of induced illness in 1 of the reunited children. Although some mothers had continuing mental health difficulties, only 1 of the other reunited cases had appreciable parent-child relationship difficulties (not requiring referral to psychiatric services). The children did well in their development, growth, and adjustment.
CONCLUSION—Family reunification is feasible for certain cases, but long term follow up is necessary to ensure the child''s safety and to identify deterioration in parent''s mental health. The outcome for reunited children compared well with reported untreated cases.

     

Risk assessment of renal cortical scarring with urinary tract infection by clinical features and ultrasonography

AIMS—To address some of the issues in the ongoing debate over the optimal diagnostic imaging following childhood urinary tract infection (UTI), by determining the risk of missing renal cortical scarring which would be detected on a technetium-99m dimercaptosuccinic acid (DMSA) gold standard if ultrasound alone were used, factoring for clinical features (upper or lower tract), UTI recurrence, and age group (infants, preschool, or school age).
METHODS—Details of UTI clinical features and recurrence were recorded for 990 children with a proven UTI, and their DMSA and ultrasound results were compared for each kidney.
RESULTS—The risks of missing DMSA scarring varied between 0.4% (school age children with solitary lower tract UTI) and 11.1% (infants with recurrent upper tract UTI).
CONCLUSIONS—UTI clinical features are important in assessing the need for DMSA imaging. Current UK imaging guidelines are endorsed, although preschool children with solitary lower tract UTI remain a controversial group and more attention needs to focused on children with recurrent UTI.