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.

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.

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.

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.

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.

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.

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.