Denis Gill

Clinical presentation of type 1 diabetes

Abstract:  Objective:  To identify the presenting features of type 1 diabetes in a national incident cohort aged under 15 yr, the duration of symptoms, the occurrence of diabetic ketoacidosis (DKA) at presentation, and the frequency of a family history of diabetes.

Good outcome in anti-glomerular basement membrane nephritis

We report a 6-year-old boy with anti-glomerular basement membrane nephritis (Goodpastures syndrome). Intensive treatment with plasmaphaeresis and immunosuppression resulted in clearance of antibody and good recovery of renal function.

PLAGIOCEPHALY, BRACHYCEPHALY AND CRANIAL ORTHOTIC DEVICES: MISSHAPEN HEADS AND HELMETS

The apparent prevalence of plagiocephaly has increased in recent years as indicated by referrals to paediatricians, neurosurgeons and craniofacial surgeons for advice and management.1 For the purposes of this article, we are referring to posterior deformational plagiocephaly (PDP) as shown in fig 1 and deformational brachycephaly (DB) as shown in fig 2. Synonyms used to describe PDP are shown in box 1. PDP is seen more frequently on the right side, has a male preponderance and is related to intrauterine position. Increasing foetal size may be a contributory factor. The asymmetry of PDP is frequently accentuated postnatally by the infant’s preference of sleeping on the flattened side. ### Box 1 Synonyms Figure 1 Non-synostotic occipital plagiocephaly or posterior deformational plagiocephaly (present at birth). Figure 2 Deformational brachycephaly (acquired due to supine sleeping). The problem of PDP has been increased by the “back to sleep” programme for the prevention of sudden infant death syndrome (SIDS).2 3 This has resulted in noticeable DB (fig 2) and accentuation of PDP. We were unable to find current population figures on the incidence of either PDP or DB. PDP is asymmetrical, while DB is symmetrical. PDP is present at birth, while DB is acquired as a result of a supine sleep position. Companies promoting cranial orthotic devices (CODs) are active on the internet and in the lay press, and one company had a stand at the RCPCH meeting in York in 2008. It would appear that CODs are being widely promoted and used in the USA. At least six companies and orthotists are providing a service in the UK, with some trusts providing CODs on the NHS. The most balanced and informative internet site is, in our opinion, that of Great Ormond Street Hospital.4 In an effort to inform ourselves on current practice, in 2006 we sent a …

Sporting activity following kidney transplantation.

Abstract. A postal questionnaire on sporting activity following renal transplantation revealed that most units encouraged participation in the majority of sports, but counselled against contact sports such as rugby football, boxing, and Asian martial arts.

Cyanide poisoning in the post-transplantation patient—a cautionary tale

There have been few reported cases of cyanide toxicity following treatment with sodium nitroprusside. We report on the case of a paediatric patient who had received sodium nitroprusside for intractable hypertension in the post-operative period, resulting in cyanide toxicity. Treatment with sodium thiosulphate, sodium nitrate and haemodialysis resulted in the elimination of cyanide from the circulation. The patient made a full recovery with no neurological sequelae.

Emergency management of meningococcal disease.

Editor,—Pollard et al presented a comprehensive personal view on the emergency management of meningococcal disease.1 I wish however to take issue with one point concerning lumbar puncture. Lumbar puncture should certainly be deferred in certain instances but should not be avoided as could be interpreted from the article. All children with suspected meningitis should, in my opinion, have a lumbar puncture at some stage in their illness. The reasons for lumbar puncture include: • : the presence or absence of meningitis should influence the choice and, perhaps, duration of antibiotic treatment • : the presence or absence of meningitis should influence fluid management once the initial shock is treated • : accurate anatomical diagnosis of meningitis is important for epidemiological purposes • : the presence or absence of meningitis is very relevant to neurodevelopmental prognosis and possible hearing impairment.  I increasingly meet paediatric trainees who seem to accept that a clinical and polymerase chain reaction based diagnosis of meningitis is sufficient. I would prefer if Pollard et al replaced (in the figure) the capitalised order DO NOT LUMBAR PUNCTURE (sic) with the instructions DEFER LUMBAR PUNCTURE and discuss its performance later …

Meningococcal infection in children.

SummaryTWO hundred and seventy five cases of meningococcal meningitis and/or septicaemia treated at the Children’s Hospital Temple Street, Dublin between January 1970 and December 1986 were retrospectively reviewed. Peak incidence was in 1981 (50 cases) and these cases were reviewed in greater detail. The mortality declined from 20 per cent for the 5 year period from 1972–1976 to 2.2 per cent from 1982–1986. The overall mortality 1970–1986 was 7.3 per cent. Over 90 per cent of meningococcal infections were caused by Group B neisseria meningitidis. The disease was seen most frequently during the winter months.The average number of deaths attributed to meningococcal infection in Ireland between 1970 and 1984 was 15 annually. About three quarters of these deaths occurred in children aged under 5 years.It is concluded that meningococcal infection remains common in north Dublin and that the national meningococcal mortality to young children should be a cause for concern.

Acute transient myositis of childhood

SummarySix cases of acute transient myositis, mainly affecting the calf muscles in school age children, are described. Each had a viral prodromal illness usually involving the upper respiratory tract; one was recovering from varicella. Serum creatine phosphokinase was markedly elevated in all six children and most had a leucopaenia at the time of diagnosis. Recovery was spontaneous, with a simultaneous fall in creatine phosphokinase.

Bloom's syndrome: case report.

SummaryA girl with Blooms syndrome is reported—the first case reported from Ireland. The outcome was death from acute myelomonoblastic leukaemia. The relationship of this syndrome with malignancy is discussed.

Pediatric Renal Transplantation in a Highly Sensitised Child—8 Years On

Highly sensitised children have markedly reduced chances of receiving a successful deceased donor renal transplant, increased risk of rejection, and decreased graft survival. There is limited experience with the long-term followup of children who have undergone desensitization. Following 2 failed transplants, our patient was highly sensitised. She had some immunological response to intravenous immunoglobulin (IVIg) but this was not sustained. We developed a protocol involving sequential therapies with rituximab, IVIg, and plasma exchange. Immunosuppressant therapy at transplantation consisted of basiliximab, tacrolimus, mycophenolate mofetil, and steroids. At the time of transplantation, historical crossmatch was ignored. Current CDC crossmatch was negative, but T and B cell flow crossmatch was positive, due to donor-specific HLA Class I antibodies. Further plasma exchange and immunoglobulin therapy were given pre- and postoperatively. Our patient received a deceased donor-kidney-bearing HLA antigens to which she originally had antibodies, which would have precluded transplant. The graft kidney continues to function well 8 years posttransplant.