Studies have been made in 1407 patients of the causes, the organs involved and the outcome of injury to the abdomen in patients needing admission to hospital in an area of Southern Sweden, between 1950 and the end of 1979. The proportion of female patients and those aged over 60 increased significantly. The seasonal distribution of the injuries showed significant change, with a drop in the initially high frequency sustained during the summer months. Penetrating injuries were rare, but increased in the 1970s. Road traffic accidents as the cause of abdominal injuries rose to a maximum of 56 per cent in the late 1960s. The numbers of injured organs and the frequency of other associated injuries rose gradually until the mid-1970s, after which there was a slight decrease. The spleen, liver and large blood vessels were the organs which were increasingly often injured. The annual incidence of various visceral injuries per 100 000 population was calculated. The number of patients with a delay of at least 24 hours before operation fell significantly and there was a tendency to shorter hospital stay. The mortality curve showed a peak in the late 1960s.
AIM: Aplastic anaemia following hepatitis may develop in as many as 1 of 3 patients with non-A, non-B and non-C hepatitis. Several causative factors have been discussed, such as viral infections and autoimmunity. Here we describe the natural history of this condition in 7 children and investigate possible hepatitis-causing agents. METHODS: We reviewed the medical records, bone marrow and liver biopsies of 7 children with severe hepatitis, with or without liver failure, who subsequently had developed aplastic anaemia. RESULTS: The median time from onset of hepatic symptoms until diagnosed onset of aplasia was 54 days. No associated viral infections could be identified. On liver biopsy, a majority had lobular inflammation but lacked signs of autoimmune hepatitis, findings compatible with a viral aetiology. Three of 6 children had low reticulocyte counts already at onset of hepatitis. All, but one patient is alive at median follow-up of 8 years. CONCLUSION: The unknown pathogenetic mechanism appears to target liver and bone marrow simultaneously, because half of the children concomitantly had low reticulocyte counts and severe liver failure.
To determine short-term outcome for children with acute liver failure (ALF) as it relates to cause, clinical status, and patient demographics and to determine prognostic factors.
A prospective, multicenter case study collecting demographic, clinical, laboratory, and short-term outcome data on children from birth to 18 years with ALF. Patients without encephalopathy were included if the prothrombin time and international normalized ratio remained > or = 20 seconds and/or >2, respectively, despite vitamin K. Primary outcome measures 3 weeks after study entry were death, death after transplantation, alive with native liver, and alive with transplanted organ.
The cause of ALF in 348 children included acute acetaminophen toxicity (14%), metabolic disease (10%), autoimmune liver disease (6%), non-acetaminophen drug-related hepatotoxicity (5%), infections (6%), other diagnosed conditions (10%); 49% were indeterminate. Outcome varied between patient sub-groups; 20% with non-acetaminophen ALF died or underwent liver transplantation and never had clinical encephalopathy.
Causes of ALF in children differ from in adults. Clinical encephalopathy may not be present in children. The high percentage of indeterminate cases provides an opportunity for investigation.
Acute liver failure (ALF) is a life-threatening condition in the absence of preexisting liver disease in children. The main clinical presentation comprises hepatic dysfunction, elevated liver biochemical values, and coagulopathy. The etiology of ALF remains unclear in most affected children; however, the recent identification of mutations in the neuroblastoma amplified sequence (NBAS) gene in autosomal recessively inherited ALF has shed light on the cause of a subgroup of fever-triggered pediatric ALF episodes. Previously, biallelic mutations in NBAS have been reported to be associated with a syndrome comprising short stature, optic atrophy, and Pelger-Huët anomaly (SOPH) specifically occurring in the Yakut population. No hepatic phenotype has been observed in individuals with this disorder who all carry the homozygous NBAS founder mutation c.5741G>A [p.(Arg1914His)]. We present the case of a 4-year-old girl with the cardinal features of SOPH syndrome: characteristic facial dysmorphism, postnatal growth retardation, delay of bone age, slender long bones, optic atrophy, and Pelger-Huët anomaly. During the first 2 years of her life, a series of infections with episodes of fever were accompanied by elevated liver enzyme levels, but hyperammonemia, hypoglycemia, coagulopathy, or encephalopathy suggestive of acute and severe liver disease were never observed. Whole exome sequencing in the patient revealed compound heterozygosity of the 2 NBAS variants, p.(Arg1914His) and p.(Glu943*). This case highlights the variability of clinical presentation associated with NBAS deficiency. Absence of severe liver problems in this case and SOPH-affected Yakut subjects suggests that individuals carrying the NBAS missense mutation p.(Arg1914His) are less susceptible to developing ALF.
OBJECTIVE: To determine the relationship between cardiac size and various measures of adiposity in children and the occurrence of coronary fatty streaks in relation to childhood obesity. DESIGN: A retrospective study based on 210 medico-legal autopsies of 5-15 y old children who had suffered violent death in the Province of Oulu, Finland, in 1970-1995. METHODS: Autopsy reports with attached clinical data and police records were recovered from the archives of the Department of Forensic Medicine, University of Oulu, Finland. All 5-15 y old individuals who had died of external trauma and were not documented as having had any chronic illnesses were chosen for investigation. The following data were extracted from the papers: age, sex, height, body weight, heart weight, liver weight, total weight of the kidneys, thickness of the abdominal subcutaneous fat and description of the coronary arteries. Heart weight was indexed to height2.7, and liver weight and kidney weight were indexed to body weight, body surface area and height. The ponderal index (body weight/height3) was calculated, and relative body weight was determined as the percentage deviation of the weight from the mean weight for height according to a growth chart developed for Finnish children. RESULTS: The average absolute heart weight and heart weight/height2.7 were significantly higher in the boys than in the girls over 12 y of age. Abdominal subcutaneous fat was thicker in the girls, but there were no significant differences in the other indicators of obesity. After adjusting for age, there were significant positive correlations between the ponderal index and heart weight/height2.7 in both the boys (P = 0.000) and the girls (P = 0.038), between relative body weight and heart weight/height2.7 in the boys (P = 0.000) and the girls (P = 0.027), and between abdominal subcutaneous fat thickness and heart weight/height2.7 in the boys (P = 0.045) but not in the girls (P = 0.234). Multiple linear regression analysis showed the ponderal index to be a significant predictor of heart weight/height2.7. Coronary intimal fatty streaks were documented in six individuals (2.9%), five of them having a ponderal index above the average and all of them having a relative weight above the average. Kidney weights were higher in the boys and were positively correlated with the measures of obesity and with heart weight/height2.7. CONCLUSIONS: The results point to an important role of excess body weight and adiposity in the development of early myocardial and coronary changes in childhood: coronary fatty streaks were not found at all in the leanest individuals in this relatively large group and the cardiac size adjusted for body size indicated hypertrophy with increasing adiposity. Prospective autopsy studies are needed in order to determine whether adiposity and the regional distribution of fat, especially intra-abdominal fat accumulation, are associated with the earliest signs of myocardial hypertrophy and the initial phase of lesion development in the arterial intima of children.
We studied age at diagnosis and disease progression of cystic fibrosis (CF) patients with a new study design, using data of 119 patients extracted from Stockholm CF Centre registry. Risk factors for overall morbidity and for lung, liver and nutritional morbidity were investigated separately using time to event methodology (Kaplan-Meier curves, proportional hazards regression). The patients were followed from: (i) healthy at diagnosis to morbidity, (ii) diagnosis with symptoms of morbidity to being free of morbidity, and (iii) free of morbidity to relapse of morbidity. Median age at diagnosis was 5.0 months. Of the patients with overall morbidity at diagnosis 50% became free of morbidity after 4.8 years; however, the patients above the age of 24 months at diagnosis had a reduced chance of becoming free of morbidity (crude hazard ratio 0.14 [95 % confidence interval 0.04, 0.45]) compared with those with diagnosis between the ages of 2 and 12 months (P
Excessive alcohol consumption is a leading preventable cause of death in the United States and has substantial public health impact on American Indian and Alaska Native (AI/AN) populations. To estimate the average annual number of alcohol-attributable deaths (AADs) and years of potential life lost (YPLLs) among AI/ANs in the United States, CDC analyzed 2001-2005 data (the most recent data available), using death certificate data and CDC Alcohol-Related Disease Impact (ARDI) software. This report summarizes the results of that analysis, which indicated that AADs accounted for 11.7% of all AI/AN deaths, that the age-adjusted AAD rate for AI/ANs was approximately twice that of the U.S. general population, and that AI/ANs lose 6.4 more years of potential life per AAD compared with persons in the U.S. general population (36.3 versus 29.9 years). These findings underscore the importance of implementing effective population-based interventions to prevent excessive alcohol consumption and to reduce alcohol-attributable morbidity and mortality among AI/ANs.