Skinfold measurements in children with cystic fibrosis: monitoring fat-free mass and exercise effects

Monitoring fat free mass (FFM), an indicator of nutritional status and a predictor of exercise performance in children, is particularly important in patients with cystic fibrosis (CF). We assessed validity of the skinfold method for measuring FFM, and its changes with exercise training, in children with CF. A total of 14 children with moderately severe symptoms of CF (age 10–18 years) were followed longitudinally and measured three times, before (at 0 and 6 months) and after exercise training (at 12 months). Separately, single measurements were conducted in 12 children with mild symptoms of CF and in 13 healthy controls. FFM was calculated from four skinfold measurements, and compared with estimations from total body water measured with deuterium dilution. The FFM calculated from skinfolds was 1.7% (P < 0.05) and 3.3% (P < 0.005) higher than that estimated with deuterium oxide dilution in patients with CF and controls, respectively. Limits of agreement were similar in patients with moderate and mild symptoms and in controls. The measurements in patients with moderate symptoms showed similar bias and limits of agreement at 6 and 12 months as compared to 0 months. Changes in FFM measured with both methods were significantly correlated before exercise (r = 0.82, P < 0.0005), and after exercise training (r = 0.60, P < 0.05). Conclusion In children with cystic fibrosis, skinfold measurements are applicable to monitor fat free mass irrespective of clinical severity of the disease, and repeated measurements at intervals of 6 months are applicable to monitor changes in fat free mass during exercise training. Received: 15 September 1998 / Accepted in revised form: 22 February 1999     

Precipitants in 42 cases of erythema multiforme

A total of 42 children with erythema multiforme (aged 0.1 to 15.8 years, median 6.1 years) were treated between 1978 and 1997 at the Department of Paediatrics, University of Bern, Switzerland. Antecedent infections were noted in 30 cases: Mycoplasma pneumoniae infection (n = 14), acute upper respiratory tract disease (n = 10) and herpes simplex infection (n = 6). Four cases were associated with antecedent medication (n = 3) or immunization (n = 1). In 12 of the 30 patients in whom erythema multiforme followed an infectious disease, drugs described in the literature as inducers of erythema multiforme had been given for symptoms not suggestive of the condition. In the remaining eight children no precipitating agent could be detected. Conclusion In this survey infections were found as a definite or at least presumptive trigger of erythema multiforme in 71% of cases. Drugs (including immunization) implicated as triggers of erythema multiforme played a definite causative role in 10% and a presumptive role in a further 29% of patients. In 19% of patients an associated condition was not diagnosed. Received: 1 September 1998 / Accepted in revised form: 16 April 1999     

Severe pneumonia in a remote hilly area: Integrated management of childhood illness

Objective: This study aims to profile children with severe pneumonia in the perspective of Integrated Management of Childhood Illness (IMCI) strategy in a resource-constrained environment.Methods: 115 consecutive children, aged 2 months to 10 yr, hospitalized with severe pneumonia were prospectively evaluated between May 1997 and June 1998 at a civil hospital in the northern hilly state of India.Results: All children had tachypnea and lower chest wall indrawing. Grunting was observed in 39.7%, inability to drink in 16.5%, and cyanosis in 1.7% cases. Radiological investigation was carried out only in 90 children that included abnormal chest radiographs (CXRs) in 76.6% cases. Feeding malpractices, vaccination inconsistencies, exposure hazards to smoking, micronutrient as well as macronutrient deficiencies, treatment from unqualified practitioners, inconsequential involvement of health care workers, predominant burden on mothers in the care of sick children, failure to recognize signs and symptoms of pneumonia by parents at home, lack of oxygen facilities, problems of accessibility and less faith on primary health care services were widely prevalent bottlenecks for effective implementation of 3 components of IMCI.Conclusion: Our study offers practical insights that can be useful in customizing IMCI to needs of children with pneumonia in a resource-constrained environment.     

Uncommon manifestations of shunt infection

Shunt infection is one of the commonest and most troublesome cause of shunt failure. According to the etiology and clinical presentations it has been divided into external and internal types.Method : We hereby present four pediatric cases, who presented with atypical manifestations not coinciding with the above mentioned types of shunt infection. Three patients developed round, globular or diffuse fluctuant CSF filled swellings at the burr hole site, one of whom had involvement of the entire pericatheteral extent upto the abdomen.Result : The fourth child developed spontaneous expulsion of ventricular end of shunt tube through the dehiscent and infected chest wall incision site.Conclusion : First three of the above children were diagnosed as having internal type of shunt infections, while the fourth had mixed manifestations of external and internal type     

Myelofibrosis: an unusual presentation of vitamin D-deficient rickets

We present the case of a breast-fed 5-month-old infant who presented with pancytopenia, secondary to intense myelofibrosis during the winter months because of undiagnosed rickets. The patient responded to oral vitamin D with rapid resolution of symptoms. Secondary hyperparathyroidism was the probable cause of the myelofibrosis. Conclusion Although nutritional rickets remains a problem in developing countries, children in northern climates in industrialized countries may also be at risk. Rickets must be considered when assessing myelofibrosis in a very young child. Received: 3 February 1998 / Accepted in revised form: 24 February 1999     

Anthropometric indicators of human immunodeficiency virus infection in infants with early and late symptoms in the first months of life

Standardised growth indices (Z-scores of weight-for-age, WA, length-for-age, LA, weight-for-length, WL, according to the reference data of the World Health Organization) have been compared for the first 4 months of life among 119 infants born to mothers affected by the type-1 human immunodeficiency virus (HIV). Infants were subdivided according to their HIV serostatus and the clinical expression of the disease. Uninfected status (n = 92), late (≥6 months, n = 18) and early (≤3 months, n = 9) onset of symptoms among the HIV infected defined three groups. Infants with early symptoms showed the lowest median WA and LA Z-scores at all times and the LA difference with their uninfected counterparts was already significant at birth. Infants with late symptoms showed early differences in WL and then in WA also compared with the uninfected ones. A<−0.40 LA Z-score at birth gave a 5.9 relative risk (RR) to be an infant with early symptoms (95% CI = 1.2−27.4) while a negative WL Z-score at 2 months of age gave a 4.2 RR for the HIV seropositivity (95% CI = 2.1−8.3). Conclusion Linear growth is the first parameter to be negatively affected among human immunodeficiency virus seropositive infants with early symptoms. In infants with late symptoms the lack of rapid WA and WL increase found among uninfected patients may be viewed as an early anthropometric indicator of HIV status. Received: 12 January 1998 / Accepted: 2 February 1998     

Leptospirosis in children in Mumbai slums

Objective : There is limited data available on symptomatic leptospirosis in Indian children. We report an outbreak of leptospirosis that occurred in children living in slums following heavy rainfall and flooding. This hospital — based prospective study was conducted from July to August 2001.Methods : Diagnosis of acute leptospirosis was suspected by following the Indian Leptospirosis Society working definition for leptospirosis. Diagnosis was confirmed by detecting anti —Leptospira antibodies, using either aLeptospira genus — specific latex agglutination assay or a dipstick assay or by a macroscopic slide agglutination test.Result : Thirty (32%) out of 93 children admitted had acute leptospirosis. Fever, bodyache, chills, abdominal pain, headache, vomiting, cough, hepatosplenomegaly, edema and crepitations were the common presenting signs and symptoms. Twenty — two children had anicteric leptospirosis and 8 had Weil disease. Response to penicillin treatment was good in all except in one child with Weil disease who died of renal failure within 3 hours of admission.Conclusion : Leptospirosis has emerged as an infectious disease in Mumbai. During monsoon, parents should ensure that their child does not have contact with the contaminated flood water.     

Chest CT findings in pediatric Wegener’s granulomatosis

Background Although pulmonary involvement occurs in the majority of children and adolescents with Wegener’s granulomatosis (WG), relatively little has been published regarding the CT imaging manifestations in this group of patients. Objective To determine the frequency and types of chest CT abnormalities in active pediatric WG (pWG). Materials and methods The study was a retrospective examination of 29 chest CT examinations performed at diagnosis (n=14) and during disease flares (n=15) in 18 children. Results The most common abnormalities were nodules (seen in 90% of examinations), ground-glass opacification (52%), and air-space opacification (45%). Of examinations with nodules, 73% demonstrated nodules >5 mm in diameter and 69% demonstrated more than five nodules; 17% had cavitary lesions. The only abnormality with a significant difference in prevalence between diagnosis and disease flares was air-space opacification, present in 71% and 20%, respectively (P < 0.01). Conclusions In accordance with the findings of published adult studies and at variance with those of prior pediatric studies, our findings indicate that chest CT abnormalities in active pWG are frequent, most commonly comprising nodules and ground-glass opacification, which may be difficult to detect on plain radiography. We therefore advocate the routine use of chest CT for all affected patients, both at the time of presentation and during disease flares.     

Respiratory muscle rigidity in a preterm infant after use of fentanyl during Caesarean section

Fentanyl is in many neonatal intensive care units the sedative of choice. One side-effect is, however, the possibility of muscle and/or chest wall rigidity. A pregnant woman with a critical pulmonary valve stenosis had a balloon dilatation at 26 weeks of gestation. She was put on propranolol, but went into a severe cardiac failure. In week 31, she developed pregnancy induced hypertension. Periodically absent diastolic flow in the umbilical cord was demonstrated. A Caesarean section was performed using fentanyl as analgesia. A boy weighing 1440 g, had a 1 min Apgar score of 3 without respiratory movements. Mask ventilation was tried, but chest wall expansion was not achieved despite using high pressures. He was intubated and positive pressure ventilation attempted, with the same result. Despite the use of high pressures up to 60–70 cm H₂O, no chest movement could be achieved. An intravenous line was established in order to give naloxone and pancuronium. Just before the drugs were given, chest wall movements were achieved and the heart rate normalized. Conclusion This is the first report on chest wall rigidity in a neonate after administration of fentanyl to the mother during Caesarean section. Received: 15 August 1997 and in revised form 30 January 1998 / Accepted 3 February 1998     

Changes in attention with hypo- and hyperglycaemia in children with insulin dependent diabetes mellitus

We compared the results of a computerized attention test (TOVA) in 38 children with insulin dependent diabetes mellitus in relation to various spontaneously occurring blood glucose levels. Testing was performed at the following blood glucose levels: <3.3 mmol/l (hypoglycaemia), 3.3–8.3 mmol/l (normoglycaemia) and >8.3 mmol/l (hyperglycaemia) . The attention (sum of errors and response time) varied significantly with the blood glucose level (P=0.002). The highest number of errors of omission and the longest response time was observed during the test run with hypoglycaemia. Age, sex, age at manifestation of the disease, metabolic control and the results of the intelligence test had no significant influence on these results. We found that attention in children with diabetes was significantly reduced compared to TOVA norms especially during mild hypoglycaemia (P<0.001). Irrespective of the blood glucose levels, reaction time and the variability of the reaction time differed significantly between TOVA norms and diabetic children (P<0.01). Conclusion In children with diabetes mellitus a significant reduction in attention was found at mild hypoglycaemia but as well at low normal blood glucose levels. Attention deficits due to transient lowering of blood glucose may therefore occur in diabetic children even before they are aware of hypoglycaemic symptoms. Received: 24 November 1997 / Accepted: 2 March 1998     

Mild closed head injury in children compared to traumatic fractured bone; neurobehavioural sequelae in daily life 2 years after the accident

Two years after an accident resulting in either a mild head injury or a fractured bone, two groups of 22 children each, aged 4–14 years, were examined for the existence of any neurobehavioural symptoms by means of a standardized questionnaire filled out by their caretakers. Selection of the children was based on reports of the Accident and Emergency Department in 1 year. Significantly more symptoms were reported after mild head injury. The main symptoms reported were headache, dizziness, fatigue and memory problems. The total number of symptoms in the children with mild head injury exceeded four times this in the group of children with a fractured bone. Conclusion Even 2 years after a mild head injury there are still residual symptoms in daily life. Received: 23 April 1998 / Accepted in revised form: 28 October 1998     

Factors influencing the spread of pertussis in households

The objective of this study was to compare the spread of pertussis in children and adults being secondary contacts after household exposure. The study was nested in an efficacy trial of an acellular pertussis vaccine. The spread of the disease was also monitored with respect to gender and antibiotic therapy. A total of 453 index cases, of which 133 were monitored for adult disease, fulfilled the WHO definition of pertussis. They had contacts to 173 unvaccinated children aged 6–47 months, and a total of 101 adults with pertussis were followed. Detection of the bacteria, or a significant increase of specific antibodies confirmed the diagnosis. Secondary spread of the disease was assumed, when a household member coughed for 7 days or more and had laboratory evidence for pertussis. Crude attack rates (AR) were 69% in children and 31% in adults (P < 0.05). AR in children were independent of gender but more women than men (P = 0.02) were affected in those households where the index case was a child. Erythromycin treatment of the index case reduced the AR in exposed toddlers from 80% to 57% (P = 0.06), and in exposed adults from 40% to 21% (P= 0.2). Erythromycin therapy in contacts did not alter the clinical course of the disease significantly. Conclusions In a household study of pertussis, 69% of children and 31% of adults (more women than men) contracted the disease. Erythromycin reduced the number of infections in household contacts, but did not alter the clinical course in those who contracted pertussis. Received: 12 June 1997 / Accepted in revised form: 29 September 1997     

Fetomaternal haemorrhage and prenatal intracranial bleeding: two more causes of blueberry muffin baby

Blueberry muffin lesions are associated with prenatal infections, severe and chronic anemia and neoplastic infiltrative diseases. In the first two instances they represent postnatal re-expression of cutaneous haematopoiesis, in the latter they are cutaneous localizations of a neoplastic disease. Chronic prenatal anaemia leading to blueberry muffin lesions in the neonate has been reported in association with severe haemolytic anaemia such as congenital spherocytosis, Rhesus haemolytic disease and ABO incompatibility, or in anaemia caused by twin-to-twin transfusion. We present two more causes of prenatal anaemia leading to blueberry muffin lesions: chronic fetomaternal haemorrhage and severe intracranial bleeding. Conclusion In any blueberry muffin baby with profound anaemia, chronic fetomaternal haemorrhage and severe internal bleeding should be included in the differential diagnosis. Skin biopsy must be performed to rule out neoplastic infiltrative diseases. Received: 14 November 1997 / Accepted: 18 June 1998     

Gastro-intestinal bleeding caused by leiomyoma of the small intestine in a child with neurofibromatosis

Gastro-intestinal bleeding is an uncommon presentation in children with neurofibromatosis. Gastro-intestinal involvement caused by jejunal leiomyoma has only been described in adults. To the best of our knowledge, this is the first paediatric case of jejunal leiomyoma associated with neurofibromatosis. We present a 10-year-old girl with a 9-month history of anaemia and low gastro-intestinal bleeding. Abdominal sonography and small bowel series showed a submucosal mass in the proximal jejunum. On surgery, a submucosal tumour was excised and histological examination suggested a diagnosis of “smooth muscle tumour of undetermined malignant potential”. There were no recurrence of symptoms for 4 years after the operation. Conclusion Jejunal leiomyoma should be considered in a child with neurofibromatosis presenting with gastro-intestinal bleeding. Received: 27 May 1997 / Accepted in revised form: 14 October 1998     

Computed tomography scan assessment of lung disease in primary immunodeficiencies

A wide spectrum of lung disease can complicate primary immunodeficiencies and early recognition influences management and prognosis. Computed tomography (CT) especially high resolution computed tomography (HRCT) has been shown to detect lung disease in adult immunodeficient patients often when the chest radiograph (CXR) is normal, but this has not been studied in children. Twenty-five CT scans [10 HRCT] and CXRs were reviewed in 23 children [14 male, 9 female] with primary immunodeficiency. Eighteen [72%] of the CT scans were abnormal, bronchiectasis being the commonest finding present in eight CT scans in patients with antibody deficiency. In eight cases CT scan revealed changes not seen on CXR (bronchiectasis;interstitial changes; small parenchymal nodules; air trapping;and a small upper lobe cyst) which influenced treatment in six cases. Conclusion CT scans have a valuable role in assessing lung disease in children with primary immunodeficiencies and will detect important changes not visible on CXR. Received: 9 December 1997 / Accepted in revised form: 3 June 1998     

Impact assessment of mass measles vaccination

Objective : The mass measles vaccination campaign was conducted in the slums of Surat City, in Gujarat State, as a part of urban measles control initiative in India. One dose each of the vaccine was administered to children in the age range of 9–59 months residing in these slums, regardless of their previous vaccination status.Methods : One year later, (October 2000), the present study was carried out in order to assess the impact of the mass vaccination campaign on the vaccination coverage and on the incidence of measles by comparing the findings with those of the baseline survey carried out in May98. This was a retrospective study with a recall period of the preceding year. 3147 children under five were studied in thirty slum clusters selected by the cluster sampling method. The parentsJcaretakers of these children were interviewed for information on any episode of fever with rash conforming to the case definition.Result : The incidence rate for measles declined from 7.7 percent reported in the baseline (May 1998) to 3.5 percent in the impact assessment study. The incidence was 8 times higher in unvaccinated children. The mean and median age at contracting the illness increased from 26 + 14.2 months and 26 months in the baseline to 30.9 ± 14.7 months and 30 months respectively in the impact assessment. The vaccination coverage had improved from 48.3 percent to 73.7 percent following the campaign.Conclusion : The compaign increased vaccination coverage decreased disease incidence and caused a shift towards higher age-groups in vaccinated children.     

The role of acoustic rhinometry in the diagnosis of adenoidal hypertrophy in pre-school children

Adenoidal hypertrophy is a common problem in pre-school children and diagnosis depends mostly on evaluation of clinical symptoms and signs. Investigative techniques to assess adenoidal size often do not add to this information. Recent reports have suggested a role for acoustic rhinometry in this situation. A total of 49 children consecutively referred to the Department of Otorhinolaryngology, University of Mainz, with evidence of adenoidal enlargement underwent acoustic rhinometry pre- and postoperatively and were compared to an age-matched control group from a local kindergarten school. Adenoidal size was visually estimated at surgery and questionnaires were completed by parents of symptomatic children. Acoustic rhinometry was not able to differentiate controls (mean nasopharyngeal cross-sectional area 1.34 +/− 0.47 cm², n = 35) from symptomatic children admitted for adenoidectomy (mean nasopharyngeal cross-sectional area 1.66 +/− 0.83 cm², n = 42, P = 0.53). Acoustic rhinometry was advantageous for patients with adenoidal hypertrophy in two situations. Firstly a subgroup of patients with complete nasopharyngeal obstruction could be identified (P = 0.03) and secondly all patients with a postnasal space less than 1.2 cm² clinically benefited from adenoidectomy. Conclusions Acoustic rhinometry, in general, is not suitable for assessing adenoidal size in pre-school children. Physical limitations of currently available acoustic rhinometers are likely to explain the limited clinical value of this investigative technique. Received: 26 February 1998 / Accepted in revised form: 22 June 1998     

Early and severe neurological features in a Wilson disease patient compound heterozygous for two frameshift mutations

We describe a patient with Wilson disease who presented at 11 years of age with neurological symptoms and subsequent rapid progression of neurological impairment but absent hepatic manifestations. Molecular analysis showed compound heterozygosity for two frameshift mutations, 2299insC and 214delAT, which most likely result in an absent or inactive protein product. Mutation-phenotypic analysis indicates that this genotype does not explain the severe phenotype, suggesting the presence of modifying factors. Conclusion Wilson disease may present even in childhood or adolescence with neurological abnormalities in the absence of hepatic manifestations. Received: 22 October 1996 / Accepted in revised form: 31 July 1997     

When do children with optic pathway tumours need treatment? An oncological perspective in 106 patients treated in a single centre

Progression patterns of optic pathway tumours (OPT) need to be precisely defined for treatment planning. In patients with neurofibromatosis type 1 (NF1), this disease is usually indolent and the available literature rarely reports progression after the age of 6 years. In patients without NF1, the disease course seems to be less favourable. We reviewed the clinical and radiological files of 106 children referred to our institution for the treatment of a symptomatic OPT since 1980. NF1 was present in 51 of them. Progression patterns in children with NF1 differed markedly from those in the other patients. A total of 83 children had tumour extension beyond the chiasm (Dodge type III). Children with NF1 had progressive tumours later during follow-up (47% after the age of 6 years), had more often proptosis and infiltrating tumours but less frequently nystagmus or increased intracranial pressure. 32 children were not treated at diagnosis because they had only mild symptoms related to the OPT. In these patients, progression occurred more often in children without than with NF1 (12/12 versus 12/20 respectively, P=0.04). A high number of patients needed treatment for progression or severe symptoms after 6 years of age. Of the patients, 33% needed treatment for progression or severe symptoms after 6 years of age. Conclusion Progression patterns of optic pathway tumours in children with neurofibromatosis type 1 differ markedly from those in other patients. This study emphasises the need for prolonged follow-up of children with optic pathway tumours, especially in neurofibromatosis type 1. Received: 1 December 1999 / Accepted: 29 March 2000     

Maximal expiratory pressure in residential and non-residential school children

Objective :Maximal Expiratory Pressure (MEP) is a powerful indicator for respiratory muscle strength, which reflects the ability of a person to cough effectively.Methods: The present study was designed on healthy respiratory symptom free children of a Residential – Sainik (50) and Non-Residential (44) school children (aged 12-14 yrs) of Bijapur, to evaluate MEP in relation to their physical anthropometry. MEP (mm Hg) was determined by using a modified Black’s apparatus, which is significantly higher in Residential-Sainik school children in comparison to Non-Residential school children (p<0.01).Results: In both groups, MEP showed positive correlation with height (Residential, r=0.72, p<0.001; Non-Residential, r=0.73, p<0.001); weight (Residential, r=0.73, p<0.001; Non-Residential, r=0.69, p<0.001), BSA (Residential, r=0.79, p<0.001; Non-Residential, r=0.72, p<0.001) and BMI (Residential, r = 0.48, p<0.01; Non-Residential,r=0.34, p<0.05), and also revealed a higher MEP in children with greater chest expansion.Conclusion: Possibly, routine physical exercise in residential Sainik school children attribute to their better respiratory muscle strength.