Vitamin D in pregnancy-time for action: a paediatric audit.

Maternal vitamin D deficiency is a significant public health issue. This retrospective multicentre audit was undertaken to identify the scale of maternal vitamin D deficiency in London, using infant vitamin D deficiency as a surrogate marker. During January 2006 to June 2008, 74 infants presented with symptomatic vitamin D deficiency, a prevalence of 1.6 per 1000 deliveries in London. Of these, 49% were hypocalcaemic at presentation and 27% had hypocalcaemic seizures. A telephone survey in June 2008 showed that no London National Health Service antenatal units had departmental guidelines on vitamin D. This audit is presented to raise awareness of current recommendations and the sequelae of maternal vitamin D deficiency.

Congenital diaphragmatic hernia misdiagnosed as tension pneumothorax.

Drainage of the pleural space is undertaken as an emergency when there is respiratory distress and the radiological appearance of mediastinal shift. We present two cases in which the initial diagnosis was tension pneumothorax and chest drains were inserted. In both cases, the diagnosis turned out to be congenital diaphragmatic hernia.

Primary pulmonary lymphangiectasis in a premature infant: resolution following intensive care.

Primary pulmonary lymphangiectasis is a rare disease, often described as fatal within the neonatal period. We report on an infant who developed severe bilateral chylous pleural effusions with computed tomography (CT) features of pulmonary lymphangiectasis. She recovered spontaneously with supportive treatment only. We discuss aspects of her intensive care which might have contributed to the good outcome, and conclude that modern intensive care may allow spontaneous recovery in conditions described in the literature as having a very poor outcome.

The value of ultrafast computed tomography in the investigation of pediatric chest disease.

Ultrafast computed tomography (CT) can be performed in almost all children with little or no sedation. The benefit has to be balanced against financial cost and radiation dose. Since observing a steady increase in numbers of scans performed, we analyzed the contribution to management of 106 pediatric chest CT scans performed over a 12-month period. Forty-eight of 106 yielded a positive diagnosis and a further 43/106 provided clearly useful information. CT was most useful in children with chronic productive cough (21/48 scans showed bronchiectasis) and suspected interstitial lung disease. It was least useful in the preoperative assessment of empyema complicating community-acquired pneumonia (0/11 scans giving information that changed management). We conclude that the increased ease of performance of chest CT in children has not led to a large number of inappropriate requests. In the large majority of cases, diagnostically useful information was provided.

Complications of modified Blalock-Taussig shunts mimicking pulmonary disease.

The modified Blalock-Taussig shunt is commonly performed as early palliation in cyanotic congenital heart disease. To highlight unusual diagnostic problems in such patients, two cases are reported in whom shunt complications were initially diagnosed as lobar pneumonia and tuberculosis, respectively. The children, an 8 month old boy and a 3 year old boy, had false aneurysm secondary to infection of the graft and dilatation of the left pulmonary artery caused by blood flow through the shunt, respectively.

The use of cyclosporin in corticosteroid dependent asthma.

Five children with severe asthma requiring at least 10 mg of prednisolone daily were treated with cyclosporin. Three were weaned from prednisolone, but one quickly relapsed. One girl had her steroid dose lowered but suffered unacceptable hirsutism and one child failed to respond. Cyclosporin may be useful in refractory childhood asthma. A prospective study is required to confirm this.

ISABEL: a web-based differential diagnostic aid for paediatrics: results from an initial performance evaluation.

AIMS: To test the clinical accuracy of a web based differential diagnostic tool (ISABEL) for a set of case histories collected during a two stage evaluation. SETTING: acute paediatric units in two teaching and two district general hospitals in the southeast of England. MATERIALS: sets of summary clinical features from both stages, and the diagnoses expected for these features from stage I (hypothetical cases provided by participating clinicians in August 2000) and final diagnoses for cases in stage II (children presenting to participating acute paediatric units between October and December 2000). MAIN OUTCOME MEASURE: presence of the expected or final diagnosis in the ISABEL output list. RESULTS: A total of 99 hypothetical cases from stage I and 100 real life cases from stage II were included in the study. Cases from stage II covered a range of paediatric specialties (n = 14) and final diagnoses (n = 55). ISABEL displayed the diagnosis expected by the clinician in 90/99 hypothetical cases (91%). In stage II evaluation, ISABEL displayed the final diagnosis in 83/87 real cases (95%). CONCLUSION: ISABEL showed acceptable clinical accuracy in producing the final diagnosis for a variety of real as well as hypothetical case scenarios.

Open lung biopsy for diffuse interstitial lung disease in children.

The aim of the study was to investigate the contribution that open lung biopsy makes to the management of children with diffuse interstitial lung disease and to review the procedure-related morbidity in comparison with published literature on other biopsy techniques. The authors reviewed the case notes and histology of patients under 18 yrs who had had an open lung biopsy in 1991-1998 for investigation of diffuse interstitial lung disease. The majority of patients returned from theatre breathing spontaneously and without an intercostal drain. Three out of 27 suffered a complication related to the biopsy that required intervention. A clear histological diagnosis was reached in 25/27 patients resulting in a change of management in 15/27. The most common histological patterns were nonspecific interstitial pneumonitis which generally had a favourable prognosis and follicular bronchiolitis/lymphocytic interstitial pneumonitis where prognosis was largely dependent on that of an underlying systemic disorder. It is concluded that open lung biopsy makes a substantial contribution to the management of diffuse interstitial lung disease in children and considering both diagnostic yield and safety, remains the biopsy technique of choice.

Primary ciliary dyskinesia: age at diagnosis and symptom history.

UNLABELLED: Age at diagnosis and the symptom history of children with primary ciliary dyskinesia (PCD) are described by reviewing the case notes in the paediatric PCD clinic. Mean age at diagnosis was 4.4 y despite a history of neonatal respiratory distress in 37/55 cases, situs inversus in 38/55 cases and early onset troublesome rhinitis in 42/55. CONCLUSION: Diagnosis of PCD is often delayed despite the presence of typical symptoms early in life. The key clinical features of unexplained neonatal respiratory distress, early onset rhinitis, situs inversus and a productive cough are highlighted, which, especially when occurring in combination, makes early referral for specific testing for PCD mandatory.

Incarcerated inguinal hernia in premature babies--a report of two cases.

UNLABELLED: We highlight the morbidity of incarcerated inguinal hernia in very low birthweight (VLBW) infants by presenting a report of two cases. Our aim is to raise the question of the optimal timing of surgery when this common problem presents on the neonatal unit. In each of our cases the hernia was diagnosed but surgery was delayed, as per normal policy on the unit, pending growth of the baby and improvement in respiratory status. Both babies suffered significant morbidity when the hernias subsequently became acutely incarcerated. CONCLUSION: In conclusion, we question whether the widespread practice of delayed surgery for inguinal hernia in VLBW infants should be reconsidered.

Diagnostic accuracy of thin-section CT and chest radiography of pediatric interstitial lung disease.

OBJECTIVE: We assessed the accuracy of thin-section CT and chest radiography to diagnose pediatric interstitial lung disease. MATERIALS AND METHODS: We identified 20 infants, boys, and girls (age range, 1 month to 14 years) with histopathologic confirmation of interstitial lung disease. Six boys and girls without interstitial lung disease were also included. Two observers independently assessed chest radiograph and CT images. The observers stated the most likely diagnosis and a differential diagnosis. We evaluated individual CT features and their distribution. RESULTS: Observers' diagnoses on CT images were correct (first choice or differential) in 66% of observations versus 45% on chest radiographs (p < 0.025). Correct diagnoses were made on first choice in 61% of CT observations versus 34% on chest radiographs (p < 0.005). Observers were confident (versus uncertain) in 42% of the CT observations versus 18% on chest radiographs; of the confident diagnoses made on CT, 91% were correct. CT interpretations were most accurate in the diagnosis of pulmonary alveolar proteinosis, congenital lymphangiectasia, and idiopathic pulmonary hemosiderosis. All healthy patients examined with CT were correctly identified as such. We noted a distinctive CT pattern in three patients with nonspecific interstitial pneumonitis and one patient with desquamative interstitial pneumonitis; the CT pattern consisted of upper zone predominant honeycombing on a background of ground-glass attenuation. CONCLUSION: A higher proportion of pediatric interstitial lung diseases can be diagnosed on thin-section CT than on chest radiographs. In our study, confident and correct diagnoses were made more frequently with CT than with chest radiographs.

Imaging and laboratory investigation in herpes simplex encephalitis.

A 14 day old baby presented with signs of an acute encephalitis. Clinically, herpes simplex encephalitis (HSE) was suspected. Early MRI and EEG were normal and there was rapid clinical improvement. A negative polymerase chain reaction (PCR) result on the initial CSF sample seemed to make HSE most unlikely. This diagnosis was subsequently proved after demonstration of specific antibody production using immunoelectrophoresis of the CSF. The child had extensive damage to brain tissue. The need for sequential analysis of CSF in making or refuting this diagnosis is illustrated.