Pre-pregnancy obesity and risk of congenital abnormalities of the kidney and urinary tract (CAKUT)-systematic review, meta-analysis and ecological study.

BACKGROUND: There is increasing evidence that maternal obesity is associated with several structural birth defects. Congenital abnormalities of the kidney and urinary tract (CAKUT) account for 30 to 50% of children starting kidney replacement therapy (KRT). We conducted a systematic review, meta-analysis and ecological study to explore the relationship between maternal obesity and CAKUT. METHODS: A systematic literature search was conducted in EMBASE, MEDLINE, Global Health, The Cochrane Library, Scopus and Web of Science. Study quality was assessed for bias and confounding. A meta-analysis using a random effect model was carried out to obtain a summary odds ratio (OR) and 95% confidence interval (CI). In the ecological study, country-level data were used to examine the correlation of secular trends in female obesity, CAKUT incidence and incidence of KRT. RESULTS: Eight epidemiological studies were included in the review-4 cohort studies and 4 case-control studies-7 of which were included in the meta-analysis. There was evidence of a positive association between obesity during pregnancy and the risk of CAKUT, with a summary OR = 1.14 (1.02-1.27). No association was seen with overweight, nor a dose response with increasing obesity. There was an increasing trend in countries' proportion of female obesity and an increasing trend in reported CAKUT incidence with specific rises seen in congenital hydronephrosis (CH) and multicystic kidney dysplasia (MCKD). CONCLUSIONS: Our findings suggest that pre-pregnancy obesity may be associated with increased risk of CAKUT at population level. Graphical abstract.

Can childhood obesity influence later chronic kidney disease?

Childhood overweight and obesity affects more and more children. Whilst associations of childhood overweight with later outcomes such as hypertension, diabetes and cardiovascular disease have been well documented, less is known about the association of childhood overweight and obesity with kidney disease. We review the existing evidence for the association of childhood obesity with markers of childhood and adult kidney disease. Whilst there is some evidence for an association, studies have not been able to distinguish between childhood being a sensitive time to develop later kidney problems, or whether observed associations of childhood obesity with poor outcomes are driven by greater lifelong exposure to obesity.

Do English NHS Microbiology laboratories offer adequate services for the diagnosis of UTI in children? Healthcare Quality Improvement Partnership (HQIP) Audit of Standard Operational Procedures.

The National Institute of Care Excellence (NICE) 2007 guidance CG54, on urinary tract infection (UTI) in children, states that clinicians should use urgent microscopy and culture as the preferred method for diagnosing UTI in the hospital setting for severe illness in children under 3 years old and from the GP setting in children under 3 years old with intermediate risk of severe illness. NICE also recommends that all 'infants and children with atypical UTI (including non-Escherichia coli infections) should have renal imaging after a first infection'. We surveyed all microbiology laboratories in England with Clinical Pathology Accreditation to determine standard operating procedures (SOPs) for urgent microscopy, culture and reporting of children's urine and to ascertain whether the SOPs facilitate compliance with NICE guidance. We undertook a computer search in six microbiology laboratories in south-west England to determine urine submissions and urine reports in children under 3 years. Seventy-three per cent of laboratories (110/150) participated. Enterobacteriaceae that were not E. coli were reported only as coliforms (rather than non-E. coli coliforms) by 61% (67/110) of laboratories. Eighty-eight per cent of laboratories (97/110) provided urgent microscopy for hospital and 54% for general practice (GP) paediatric urines; 61% of laboratories (confidence interval 52-70%) cultured 1 µl volume of urine, which equates to one colony if the bacterial load is 106 c.f.u. l(-1). Only 22% (24/110) of laboratories reported non-E. coli coliforms and provided urgent microscopy for both hospital and GP childhood urines; only three laboratories also cultured a 5 µl volume of urine. Only one of six laboratories in our submission audit had a significant increase in urine submissions and urines reported from children less than 3 years old between the predicted pre-2007 level in the absence of guidance and the 2008 level following publication of the NICE guidance. Less than a quarter of laboratories were providing a service that would allow clinicians to fully comply with the first line recommendations in the 2007 NICE UTI in children guidance. Laboratory urine submission report figures suggest that the guidance has not led to an increase in diagnosis of UTI in children under 3 years old.

Implementation of NICE guidance on urinary tract infections in children in primary and secondary care.

AIM: To audit compliance with the 2007 National Institute of Clinical Excellence guidelines on the management of urinary tract infection in children under the age of 16 years across primary and secondary care services in England. METHODS: A retrospective multisite audit of 10 general practice, 3 paediatric, 2 paediatric emergency and 2 emergency general units. Four distinct geographical areas were represented. Data were collected between 1 January 2010 and 31 December 2010. Six criteria were audited, which focused on the following: improving the rate of diagnosis, management of the very young child with UTI and selection of children for imaging. RESULTS: A total of 1149 children were audited (682 from primary care and 467 from secondary care). Overall compliance was as follows: criterion 1: 28%; criterion 2: 68%; criterion 3: 89%; criterion 4: 43%; criterion 5 (comprising 12 subcriteria): 13% and for criterion 6: 45%. CONCLUSION: The results indicate significant shortcomings in the implementation of NICE guidance on childhood UTI in England. The guidance is complex and this makes its implementation challenging. It was difficult to identify children presenting with nonspecific fever from clinical data systems. Adequate IT systems throughout the NHS are a key step to improving implementation of this and other NICE guidance.

Dent's disease complicated by an acute Budd-Chiari syndrome.

We present the case of a young boy with Dent's disease, identified as having a mutation in the kidney-specific chloride-proton antitransporter CLCN5 during investigation for nephrotic-range proteinuria. He went on to develop growth hormone deficiency and was treated with recombinant growth hormone. He later presented acutely with hepatorenal failure and thrombotic occlusion of the middle and right hepatic veins consistent with a diagnosis of Budd-Chiari syndrome, which required a prolonged period of intensive care. The diagnosis of Dent's disease should be considered early in boys with nephrotic-range proteinuria in the absence of clinical oedema and hypoalbuminaemia to allow for the timely introduction of strategies, such as a high-citrate diet, to preserve renal function. The measurement of urinary ß-2 microglobulin has been shown by this case to be a more reliable and specific marker of tubular dysfunction than the urinary retinol-binding protein.

GPs should evaluate all children following UTI.

Ten per cent of girls and 3% of boys will have had a UTI by 16 years of age. The majority are acute, isolated illnesses that resolve quickly, with no long-term implications for the patient. However, UTIs may be associated with underlying congenital abnormalities, and recurrent infections can lead to renal scarring. UTI is defined as bacteriuria in the presence of symptoms. Asymptomatic bacteriuria does not require treatment or investigation. The presentation of UTI is extremely variable. The only way to differentiate a UTI from a viral infection is by testing the urine and this should be carried out within 24 hours in children with non-specific fever. UTIs can also present with vomiting, failure to thrive or persistent irritability. A urine infection in the presence of any of the above symptoms is a pyelonephritis (upper UTI). Children may also present with classical symptoms of cystitis (lower UTI) such as urinary frequency, dysuria and abdominal pain. Most children with UTI, even if febrile, can be managed in the community. If the initial assessment shows a high risk of serious illness, there should be an urgent referral to a paediatrician. The same applies to infants under three months with suspected UTI. It is better to obtain a urine sample by the clean catch method, rather than using urine pads or bags. Leucocyte esterase and nitrite dipsticks are not reliable in children under three, so a negative dipstick does not rule out UTI. Not every child needs to be referred after a first UTI. However, they should all be evaluated to help determine which require renal imaging as well as identifying triggers for recurrence. GPs are central to the identification of children at risk of renal pathology. All children who are diagnosed and treated for a UTI must be assessed for risk of renal abnormalities and/or recurrence.

Ocular presentation of pediatric Miller-Fisher syndrome.

The authors describe a case of Miller-Fisher syndrome in a child who presented to the ophthalmology department with bilateral abducens nerve palsies. Miller-Fisher syndrome is an important differential diagnosis in any case of bilateral sixth nerve palsies but should only be definitively diagnosed once tumors, infections, and other neurological diseases have been conclusively ruled out.

A genetic cause for neonatal encephalopathy: incontinentia pigmenti with NEMO mutation.

UNLABELLED: Incontinentia pigmenti (IP) is not generally recognized as a cause of neonatal encephalopathy. A full-term infant developed a rash and encephalopathy with lesions in the basal ganglia and periventricular white matter 3 days after a normal delivery. Typical skin changes of IP were confirmed by histology and mutation analysis of the NFkappaB essential modulator (NEMO) gene. CONCLUSION: The mechanism of brain injury appears to be increased apoptosis after inflammation and this condition should be included in differential diagnosis of neonatal encephalopathy if skin lesions are present.