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Paediatric Surveillance Unit (paediatric + surveillance_unit)
Selected AbstractsThe clinical presentation of mitochondrial diseases in children with progressive intellectual and neurological deterioration: a national, prospective, population-based studyDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 5 2010CHRISTOPHER M VERITY Aim, Our aim was to study the clinical presentation, mode of diagnosis, and epidemiology of mitochondrial disorders in children from the UK who have progressive intellectual and neurological deterioration (PIND). Method, Since April 1997, we have identified patients aged 16 years or younger with suspected PIND through the monthly notification card sent to all UK consultant paediatricians by the British Paediatric Surveillance Unit. Clinical details obtained from reporting paediatricians are classified by an Expert Group. Results, By July 2008, 2493 cases of PIND had been reported, among which there were 112 children (69 males, 43 females) with mitochondrial diseases presenting between birth and 14 years 7 months (median 12mo), divided into 13 subgroups. In some instances, clinical features were characteristic of mitochondrial disease, but many children presented non-specifically with combinations of developmental delay, hypotonia, failure to thrive, and seizures; 16 children had multisystem disease at presentation. Mortality was high: 40 children had died. Blood and/or cerebrospinal fluid lactate measurements were abnormal in 87 children, and 47 of 78 brain magnetic resonance images showed increased basal ganglia signal. Definite diagnoses were usually made by muscle enzyme or genetic studies. Interpretation, This is a unique population-based study of the mitochondrial disorders that cause childhood neurodegenerative disease. It provides detailed information about the clinical presentation and investigation of these complex cases. [source] Symptomatic infant characteristics of congenital cytomegalovirus disease in AustraliaJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 8 2005Sian C Munro Background: Human cytomegalovirus (CMV) is the most common cause of viral intrauterine infection. In utero transmission can occur during primary maternal infection, reactivation or reinfection of seropositive mothers. Objective: To describe the aetiology and clinical features of infants diagnosed with congenital CMV and to document maternal factors that were presented. Methods: Active national surveillance was initiated in 1999 in collaboration with the Australian Paediatric Surveillance Unit. Results: Monthly notifications resulted in 70 cases of congenital CMV being identified between 1999 and 2003. Nearly all of the cases were symptomatic with the most common clinical sequelae reported in infected infants being jaundice, thrombocytopaenia, hepatomegaly, petechiae, purpura and splenomegaly. Almost half (43.5%) of the infants had central nervous system (CNS) complications, such as microcephaly, chorioretinitis, sensorineural hearing loss, intracranial calcifications, developmental delay or seizures, with over half presenting two or more CNS abnormalities. Maternal febrile illness was noted in 54.8% of the cases. The majority of mothers were primiparous (46.4%) or secundiparous (39.3%), indicating two different population groups at risk of primary CMV infection. Conclusion: This study documents symptomatic congenital CMV cases in Australia. [source] Congenital and neonatal varicella in AustraliaJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 2 2000JM Forrest Objective: To establish the incidence and severity of congenital and neonatal varicella in Australia. Methodology: Demographic and clinical details were obtained by postal questionnaire regarding cases notified to the Australian Paediatric Surveillance Unit by over 930 participating clinicians in 1995,97 inclusive. Results: Seven cases of congenital varicella (1 : 107 000 pregnancies/year) followed maternal infection at 8,26 weeks: five had defects, two did not. Four of the seven infants with congenital varicella developed herpes zoster in the first 15 months of life. Forty-four infants had neonatal varicella (1 : 17 000 pregnancies/year). Conclusion: There is an ongoing, albeit low, incidence of congenital and neonatal varicella in Australia. [source] A nationwide study on hospital admissions due to dehydration in exclusively breastfed infants in the Netherlands: its incidence, clinical characteristics, treatment and outcomeACTA PAEDIATRICA, Issue 5 2009Rolf AA Pelleboer Abstract Aims: To estimate the incidence and clinical characteristics in hospital admissions due to dehydration or undernutrition and their laboratory evaluation and treatment outcome in exclusively breastfed infants. Methods: All hospital admissions during the first 3 months of life assessed by the Dutch Paediatric Surveillance Unit (DPSU) between mid 2003 and mid 2005. Results: Nationwide 158 cases reported, correspond to an incidence of 58/y/100 000 breastfed infants; it is lower for severe dehydration at risk for hypernatraemia; 20/y/100 000. Sixty-five per cent of cases were <2 weeks old, their median weight loss was 9.3% and median age at admission 5 days; Serum sodium value was measured in only 12% of all cases. Insufficient volume intake and inadequate growth were most frequently reported (61% and 41%). Lethargy, jaundice or clinical dehydration was scored in 11,25%, seizures or shock in 3%. A breast pump at home was used in only 31%. In the hospital breast pumps were available (82%) as lactation consultants (73%). For treatment 65% was offered formula, in 30% by nasogastric drip. Most admissions lasted up to 3 days, all recovered fully and 33% were breastfed exclusively at discharge. Conclusion: The incidence of severe dehydration in the Netherlands is relatively low. With extended use of breast pumps at home it could be lower. To prevent complications, we recommend applying a reference weight chart, a full clinical examination and more extensive screening of serum sodium and glucose. [source] | ||||||||||