Irish neonatal mortality statistics for 2004 and over the past 17 years: how do we compare internationally?

In the past 17 years neonatal mortality survey has provided important data on the trends in deaths of all live born infants born in Southern Ireland who are greater than 500 g birth weight and who die within the first 28 days of life. The aims of this study were to report neonatal mortality data for Southern Ireland for 2004, to examine trends in neonatal mortality over the past 17 years and compare Irish Neonatal Mortality rates to other countries around the world. The neonatal mortality rate for 2004 was 2.9/1000 with a corrected NMR of 1.9/1000. The response rate to the survey was 100%. Prematurity is now the leading cause of neonatal mortality representing a change from previous years. Deaths related to asphyxia have remained largely unchanged. When compared to international figures Ireland compares favourably to other countries around the world.

Modes of death in neonatal intensive care units.

With the ever-increasing availability of aggressive medical treatment and technical support, neonatologists are offered an increasing ability to prolong life. While "end-of-life" decisions within NICUs have been studied internationally, there is limited data available for Ireland. Through the auspices of the Irish Faculty of Paediatrics 2002 Neonatal Mortality Ouestionnaire, decisions made around the time of death in Irish Neonatal Intensive Care Units were examined. The overall response rate to the questionnaire was 96% (n=25). One hundred and eighty seven deaths were reported for 2002. Information pertaining to the mode of death was available in 53% of cases. Seventy seven percent of those paediatricians who answered this question, reported either withdrawing or withholding treatment in babies thought to have a hopeless outcome, with the greatest proportion of these deaths occurring in premature infants (n=30) and babies with congenital defects (n=40).

Irish neonatal mortality--12 years on.

All Paediatricians in Ireland were requested to fill a questionnaire which dealt with data pertaining to all live-born infants over 500 g birth weight who died within the first 28 days of life in 1999. Deaths were categorized according to Wigglesworth Classification into Lethal Malformation, Immaturity (Prematurity), Asphyxia and "Specific". Each hospital and patient was assigned a unique ID number. Data from questionnaires was entered into a database. Results were compared with those previously published by Counahan and Clarke for 1987. Twenty one of the 23 paediatric centres in Ireland replied giving a response rate of 91%. The over-all number of neonatal deaths for 1999 was 186 compared to 310 for 1987. One hundred and two (55%) were < 1500 grams and 61 (33%) < 1000 g. The principal causes of death for 1999 were Congenital Malformations 39%, (n=72), Prematurity 37% (n=69) and Asphyxia 3% (n=5). This compares to figures of 39%, 40% and 8%, respectively for 1987. The overall Neonatal Mortality Rate for 1999 was 3.48/1000 with a Corrected Neonatal Mortality Rate of 2.1/1000. The Neonatal Mortality Rate and Corrected Neonatal Mortality Rate for 1987 were 5.3/1000 and 3.3/1000 respectively. The decrease in Neonatal Mortality in the past 12 years in Ireland is to be applauded. However it can lead to a false sense of security regarding standards of neonatal care. While the death of Extremely Low-Birth-Weight infants can now often be prevented, in many cases it is merely deferred to the post-neonatal period. Furthermore, no national data is available on the long term outcome and neurodevelopmental status of preterm or asphyxiated infants who survive. The possibility of establishing an Irish Neonatal Morbidity Register, aimed at improving the effectiveness and efficiency of medical care for newborn infants, must now be explored.

Contact and nutrient caregiving effects on newborn infant pain responses.

To understand how the 'caregiving context' could affect responses to procedural pain, the authors sought to determine whether (1) the combined effects of sweet taste and holding (caregiving contact) were greater than the effects of either alone, (2) any combined effects were additive or interactive, and (3) the interventions had similar effects on behavioral (crying and facial activity) and physiological (heart rate, vagal tone) responses to the heel-stick procedure in newborn infants in a randomized two-factorial intervention trial. Eighty-five normally developing newborn infants were studied with a mean gestational age of 39.4 weeks on the 2nd or 3rd day of life. Infants were randomized in blocks of eight to receive (1) no holding and water taste (control participants), (2) no holding and sucrose taste (sucrose group), (3) holding and water taste (holding group), or (4) holding and sucrose taste (holding and sucrose group). Crying was reduced significantly by taste and holding, and the interventions combined additively. Facial activity was only significantly reduced by holding. For physiological measures, the interventions interacted with each other and preintervention levels to reduce heart rate and lower vagal tone more during the procedure in infants in whom heart rate and vagal tone were higher before intervention. Consequently, sweet taste and holding interventions combined in complex ways when acting on different behavioral and physiological response systems to modify stressful pain experiences. The results suggest that providing a caregiving context when painful procedures are performed may be a simple and practical method of reducing pain experience in infants, and that no one measure captures these effects.

Dermatitis Herpetiformis: a review of fifty-four patients.

A review of 54 patients with dermatitis herpetiformis presenting between 1984-1993 to The Regional Centre of Dermatology, Mater Misericordiae Hospital was undertaken. All patients had skin lesions clinically and histologically typical of dermatitis herpetiformis. Deposition of granular IgA at the dermoepidermal junction on direct immunofluorescence was present in each case. The average age of onset was 41.8 yr, patients having symptoms for an average of 1.6 yr before diagnosis. Six patients had a prior history of coeliac disease. Two patients had a family history of dermatitis herpetiformis, a father and son who were both propositi in this study. Small bowel biopsy was performed on 35 patients, 71.4 per cent of them showing evidence of villous atrophy. All patients were controlled on a gluten free diet or by dapsone or a combination of these. None of the patients experienced serious adverse effects of therapy, nor did any develop lymphoma of the small bowel with a mean follow up period of 4.2 yr (range 1-10 yr).

Carbon 13-labeled urea breath test for the diagnosis of Helicobacter pylori infection in children.

Helicobacter pylori infection is mainly acquired in childhood, and studies on the epidemiology of this infection depend on the availability of a noninvasive diagnostic test for use in children. The aim of this study was to determine whether the carbon 13-labeled urea breath test (UBT) can be used in children by evaluating: (1) its sensitivity and specificity compared with either culture or both rapid urease test and histologic examination, (2) whether a test meal or a prolonged fast is required, (3) the usefulness after treatment for H. pylori. Eighty-eight children (mean age, 10.6 +/- 4.19 years) who were undergoing upper endoscopy were studied while fasting, not fasting, and after treatment. Children were given 50 mg of 13C-urea if they weighed less than 50 kg or 75 mg of 13C-urea if they weighed more than 50 kg with 50 mg of a glucose polymer solution in 7.5 ml of water. Breath samples were collected at baseline and at 15, 30, 45, and 60 minutes. In 63 fasting children the UBT was 100% sensitive and 97.6% specific at 30 minutes with a cutoff value of 3.5 delta 13CO2 per mil. Nonfasting tests in 23 children, performed between 1 and 2 hours after their usual meal, were 100% sensitive and 91.6% specific. In 13 children fed directly before the UBT, the sensitivity of the test was reduced to 50%. Thirty minutes was the optimal sampling time. There was a significant decrease in specificity when samples were obtained at 15 minutes, possibly caused by the interference of oral urease-producing organisms. The test was 100% sensitive and specific in 20 children after treatment for H. pylori infection. The UBT is a highly sensitive and specific test for the diagnosis of H. pylori infection in children. Neither a prolonged fast nor a test meal is required.

Gastric metaplasia and duodenal ulcer disease in children infected by Helicobacter pylori.

BACKGROUND: Helicobacter pylori infection of the gastric mucosa is vital in the pathogenesis of duodenal ulcer disease. H pylori will only colonise gastric epithelium and its association with duodenal disease is therefore not easily explained. AIMS: To determine if gastric metaplasia in the duodenum increases the risk of duodenal ulcer disease in children infected with H pylori. PATIENTS: All children undergoing upper endoscopy over a 20 month period in a children's hospital in Ireland. METHODS: Two biopsy specimens were obtained from the antral mucosa and two from the first part of the duodenum. One antral biopsy specimen was used in a rapid urease test (Clo Test). Biopsy sections were stained with haematoxylin and eosin and also with cresyl violet for identification of H pylori. Periodic acid Schiff (PAS) stain was performed to identify areas of gastric metaplasia. RESULTS: Gastric and duodenal biopsy specimens were obtained from 148 patients (M:F 1:2:1). Twenty five children (17%) had H pylori positive gastritis. Thirty four children (23%) had gastric metaplasia in the duodenum. Nine per cent of children under the age of 8 years had gastric metaplasia compared with 38% in those 12 years of age or over (p < 0.005). Seven children had duodenal ulcer disease. Gastric metaplasia was present in six of seven (86%) children with duodenal ulcer disease compared with 28 of 141 (20%) without ulceration (p < 0.001). While both H pylori and gastric metaplasia were each significant risk factors for duodenal ulcer disease, the combined presence of both factors was associated with a pronounced increase in duodenal ulcer disease. Duodenal ulcer disease occurred in over 50% of children with both H pylori infection and gastric metaplasia. In contrast duodenal disease did not occur in children (0 of 100) when both were absent. CONCLUSION: The presence of gastric metaplasia in the duodenum is the major risk factor for duodenal ulcer disease in patients colonised by H pylori.

Preparing the bowel for colonoscopy.

Bowel preparation methods for total colonoscopy in children generally involve whole gut irrigation with electrolyte lavage solutions, which in most children will require hospitalisation for nasogastric tube administration. The aim of the study was to determine the efficacy of oral bisacodyl combined with a single phosphate enema as a bowel preparation regimen in children. In an open prospective trial, 30 children (aged 18 months-15 years) were given oral bisacodyl on each morning of the two days before colonoscopy. The children were maintained on a normal diet. A phosphate enema was administered on the morning of the procedure. The adequacy of bowel preparation was graded as grade I if no faecal material was encountered, grade II if small amounts of faecal material were present in scattered locations, and grade III if there was poor preparation with faecal material precluding satisfactory visualisation of the bowel mucosa. Eight children (26.6%) had minor abdominal cramps when taking bisacodyl, but all had a previous history of similar pain. Five children (16.6%), all under 5 years of age, cried during the administration of phosphate enema. Bowel preparation was considered excellent (grade I) in 26 (86.6%) and good (grade II) in four (13.3%). In all patients adequate visualisation of the bowel mucosa was obtained. Oral bisacodyl combined with a single phosphate enema provides an ideal method of preparing the bowel for total colonoscopy. This preparation allows colonoscopy to be carried out as a day case procedure in children while maintaining them on a normal diet.

Copper-associated liver disease in childhood.

BACKGROUND/AIMS: Indian childhood cirrhosis is associated with high liver copper concentrations and progressive liver disease with a high mortality. Early treatment with penicillamine was found to reduce mortality and reverse liver damage. We aimed to define the clinical features of copper-associated liver disease outwith the Indian subcontinent and encourage the earlier consideration of the syndrome in cryptogenic liver disease. METHODS: Three European children presented between 10 and 29 months of age with abdominal distension, pyrexia and hepatosplenomegaly. Over 1-5 weeks their condition deteriorated rapidly due to liver failure. Two died within 2 months of onset and one received a successful liver transplant. In two cases consideration of the diagnosis occurred only on examination of the liver after orthotopic liver transplant or death. Light microscopy was used, with haematoxylin and eosin, reticulin and orcein stains. Tissue, plasma and water copper levels were measured by flame atomic absorption spectrometry. RESULTS: All had micronodular cirrhosis and severe hepatocellular necrosis with Mallory bodies and copious-orcein positive material. Liver copper concentrations ranged from 1100-1310 micrograms/g dry weight. For two patients domestic water with high copper content had been used for the preparation of feeds. No environmental source of excess copper could be identified in the third case. CONCLUSIONS: We suggest that the above condition, which is called Indian childhood cirrhosis in the Indian subcontinent and Copper Storage Disease elsewhere, would be better named 'Copper-Associated Liver Disease in Childhood', emphasising the need to consider this disorder in unexplained liver disease and to seek possible sources of excessive copper intake.

Isolated cerebral thrombo-embolism and Crohn disease.

Arterial thrombo-embolism is an unusual complication in inflammatory bowel disease in adults and children. Cerebral arterial disease has been reported on only a few occasions. There are only two previous case reports of arterial disease occurring in a child with Crohn disease. However in both instances the arterial disease was part of a generalised Takayasu arteritis which resulted in multi-organ involvement. This report describes a 14-year-old boy who developed seizures before a histological diagnosis of Crohn disease was made. These seizures were the result of a vascular lesion which was confined to the right middle cerebral artery. CONCLUSION. Crohn disease as well as ulcerative colitis should be considered as a possible cause of cerebrovascular accidents in children.

Unilateral Beau's lines in childhood reflex sympathetic dystrophy.

Reflex sympathetic dystrophy is characterized by severe pain and autonomic dysfunction in a limb, usually after an injury. We describe a patient with childhood reflex sympathetic dystrophy with unilateral Beau's lines on the nails of the affected hand. Unilateral Beau's lines have not been described previously in this condition to our knowledge, and we discuss their possible pathogenesis.

Association of symptoms with Helicobacter pylori infection in children.

The aim of this study was to determine prospectively whether Helicobacter pylori-associated gastritis is associated with specific symptoms by evaluating whether these symptoms are relieved by treatment of the infection. Symptoms resolved after the eradication of H. pylori in only three of eight children with H. pylori-associated gastritis alone, in comparison with all six children with duodenal ulcer disease.

Sleep position and SIDS in Irish infants.

A retrospective case-controlled study has compared the frequency of different sleeping positions in a group of 97 SIDS infants and a group of 98 control infants. The results show a clear excess of SIDS deaths among Irish infants sleeping in the prone (lying on abdomen) position with the risk of a SIDS increased 2.3 fold comparing prone to side and 10.5 fold comparing prone to back positions. Among the SIDS group 79% of infants were lying prone when found dead compared to 25% of the control group (chi square = 64.3, p < 0.001) with only 7% and 9% sleeping on their sides and backs respectively (control group 48% and 27% respectively). Irish parents should be advised to avoid placing infants in the prone position (on abdomen) to sleep as this position is associated with a significantly increased risk of the Sudden Infant Death Syndrome.

Surfactant therapy in respiratory distress syndrome: the effect of a learning curve in improving outcome.

A retrospective study of all premature neonates who received artificial surfactant (Curosurf) at the Rotunda Hospital was performed. The period from October 1990 to June 1992 (n = 48) was compared with the initial experience from June 1987 to January 1988 (n = 15). In the initial period mortality rate was 67% in surfactant treated infants, and use of surfactant was not associated with an improvement in outcome compared with the previous six years. In the more recent period overall mortality was 21%. Overall survival in normally formed very low birthweight infants improved from 59% in 1986 to 86% in 1991-1992. Improvement in survival rates was most noticeable in infants with birthweight 750-999 grams, with survival increasing from 44% (before introduction of surfactant treatment) to 91% (in 1991-1992). It is probable that a certain level of experience with use of surfactant is required before optimal effects can be obtained.