Measles outbreak in Dublin, 2000.

BACKGROUND: An outbreak of measles occurred in Ireland between December 1999 and July 2000. The majority of cases were in north Dublin, the catchment area of The Children's University Hospital (TCUH). METHODS: Details of all of the 111 children attending the hospital with a diagnosis of measles between December 1999 and July 2000 were prospectively entered into a database. Charts were subsequently reviewed to extract epidemiologic and clinical details. National figures were obtained from the National Disease Surveillance Centre. RESULTS: In the study period 355 attended TCUH with a serologic or clinical diagnosis of measles, and 111 were admitted (47% female, 53% male). The main indications for admission were dehydration in 79%, pneumonia or pneumonitis in 47% and tracheitis in 32%. Thirteen children (11.7% of those admitted) required treatment in the intensive care unit, and in 7 of these mechanical ventilation was necessary. There were 3 deaths as a result of measles. Public health measures to curb spread of the disease included promotion of immunization for susceptible children nationally and recommending administration of measles-mumps-rubella vaccine (MMR) from the age of 6 months, in North Dublin. CONCLUSION: This outbreak of measles posed a major challenge to the hospital and the community for the first half of 2000. The national MMR immunization rate before the outbreak was gravely suboptimal at 79%, whereas the rate in North Dublin, the catchment area of TCUH, was <70%. Three children died as a result of a vaccine-preventable illness.

Childhood posterior reversible encephalopathy syndrome.

Posterior Reversible Encephalopathy Syndrome (PRES) is a clinicoradiologic syndrome characterised clinically by headaches, altered consciousness, visual disturbances and seizures and radiological changes which can resolve. However left untreated it can be fatal and not all cases are reversible. It can occur in many settings, the most common being hypertensive crisis. We discuss the clinical and radiological features of this increasingly diagnosed condition among children and current thinking on its pathogenesis. A brief case is used to highlight the variable presentation of PRES. PRES is often unsuspected by the clinician and radiologists may be first to suggest the diagnosis. Accurate assessment including blood pressure measurement, appropriate imaging and rapid treatment is required to avoid a devastating outcome.

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.

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.

Clinical presentation of type 1 diabetes.

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. METHODS: A prospective study was undertaken of incident cases of type 1 diabetes using an active monthly reporting card system from January 1, 1997 to December 31, 1998 in the Republic of Ireland. Follow-up questionnaires were distributed to pediatricians nationally. RESULTS: Two hundred and eighty-three incident cases were identified. Polyuria, polydipsia and weight loss were the main presenting symptoms in all age categories. Nocturnal enuresis was reported in 19% under 5 yr and in 31% aged 5-9.99 yr. Constipation was noted in five patients and in 10.4% under 5 yr of age. The median duration of symptoms was highest in the youngest (under 2 yr) and oldest (10-14.99 yr) age categories. Presentation in moderate/severe DKA occurred in 25% overall and six of nine of those aged under 2 yr. A family history of type 1 diabetes in a first-degree relative was found in 10.2%. CONCLUSIONS: This study confirms the abrupt onset of type 1 diabetes, the absence of a family history, and the importance of the classical symptoms of polyuria, polydipsia, and weight loss in the majority of cases. It reveals secondary enuresis as an important symptom, especially in those under 10 yr, and constipation in the under 5 yr age group. The very young (under 2 yr) are more difficult to diagnose, have more variability of symptom duration, and are more likely to present in moderate/severe DKA. A high index of suspicion aids early diagnosis.

Ethical principles and operational guidelines for good clinical practice in paediatric research. Recommendations of the Ethics Working Group of the Confederation of European Specialists in Paediatrics (CESP).

A child has the full right of protection of his/her life by provision of optional medical care. There is a need in paediatrics for better evidence based practice founded on quality research into efficacy and safety of children's medications. To protect the best interests of the child one must balance the ethical demand to do clinical studies with the necessity to avoid doing harm. To achieve this end good clinical practice in paediatric research demands that studies comply with the Declaration of Helsinki, ICH topic E11, EU Directives and other relevant international guidelines. Evident differences in physiology, pharmacology, pharmacokinetics and pharmacodynamics between children of differing ages and between children and adults demand properly constructed and conducted studies that respect the special somatic, emotional and mental needs of children. To justify any research project one must balance the benefit/risk ratio, provide experienced, competent personnel and infracture, obtain adequate informed consent/assent, and have the study evaluated and approved by an ethics committee containing expertise on the rights and needs of children.