RESUMO
We report three patients with intestinal microvillous dystrophy, two of whom were siblings. The relatively delayed clinical presentation and the lack of classical microvillous inclusions distinguish these cases from the previously described microvillous inclusion disease (MVID). There appears to be an underrecognized spectrum of microvillous disorders leading to fatal intractable secretory diarrhea in infants. In our three cases the diagnosis was suggested by periodic acid-Schiff (PAS) and alkaline phosphatase preparations of a jejunal biopsy specimen showing thinning or absence of brush border staining, which was confirmed by electron microscopy. The latter showed poorly developed and haphazardly arranged microvilli with intracytoplasmic vesicular bodies but no true inclusions. As in MVID, the prognosis of intestinal microvillous dystrophy is poor. The occurrence of the disease in two siblings of consanguinous parents suggests an autosomal recessive inheritance, and like MVID, genetic counselling of affected families is essential.
Assuntos
Enteropatias/patologia , Microvilosidades/patologia , Feminino , Humanos , Recém-Nascido , Masculino , Microscopia EletrônicaRESUMO
Diarrhoea morbidity data were collected prospectively over 22 months from a cohort of young children living in a deprived community in rural Zimbabwe. Despite the general high prevalence of diarrhoeal disease, there was considerable individual variability in attack rates. Risk factors associated with high diarrhoea frequency were therefore sought by a questionnaire study on feeding, environmental, educational and socio-economic factors. This was supported by observation of living conditions, and water and sanitation facilities. Surprisingly, no association was found between diarrhoeal morbidity and any of these factors, suggesting that other factors such as individual hygiene behaviour or individual susceptibility to diarrhoea may play a role in determining the observed differences in diarrhoea rates in this community.
Assuntos
Diarreia Infantil/etiologia , Diarreia Infantil/epidemiologia , Feminino , Humanos , Higiene , Lactente , Masculino , Prevalência , Estudos Prospectivos , Fatores de Risco , População Rural , Fatores Socioeconômicos , Zimbábue/epidemiologiaRESUMO
The evidence indicating a mucosal source for the jejunal fluid lactase activity of children is so far inconclusive. Samples of jejunal mucosa and the adjacent fluid were obtained simultaneously from 15 children. Lactase activity was measured at pH 5.9 in mucosa and fluid. Fluid activities showed a significant positive correlation with the activity of the corresponding mucosal homogenate but a stronger correlation was found with an enterocyte microvillous membrane fraction prepared from the same homogenate (r = 0.807 and 0.889, respectively). Kinetic and pH optima studies were consistent with a microvillous membrane origin. Fluid activity and pH optimum were not changed detectably when measured in the presence of an enterocyte lysosomal acid lactase inhibitor. Jejunal fluid lactase activity and its properties closely reflect the microvillous membrane enzyme. Lysosomal acid lactase does not contribute measurably to the total lactase activity of jejunal fluid.
Assuntos
Líquidos Corporais/enzimologia , Mucosa Intestinal/enzimologia , Jejuno/enzimologia , beta-Galactosidase/metabolismo , Adolescente , Criança , Pré-Escolar , Humanos , Concentração de Íons de Hidrogênio , Lactente , Cinética , Lactase , Lisossomos/enzimologia , Microvilosidades/enzimologiaRESUMO
Nutritional status and 'well-being' were compared prospectively in 39 children (mean age 8.1 years) who received nutritional support following bone marrow transplantion (BMT): 20 received enteral tube feeding (ETF; six received parenteral nutrition [PN] subsequently) and 19 with oral mucositis received PN (one received ETF subsequently). Poor nutritional status (height for age and/or weight for height and/or mid-arm circumference z-scores <-1) was present in 18 patients and was associated with a longer hospital stay (P = 0. 01). Both ETF and PN groups were comparable with respect to age, pretransplant nutritional status and conditioning regimens. No significant deterioration in anthropometric indices in either group occurred following BMT. However, significant correlations were found between the duration of ETF (and not PN) and improvements in nutritional status. Furthermore, PN was associated with more frequent exocrine pancreatic insufficiency than ETF (P = 0.001). Oral mucositis was associated with poorer 'well being' at the start of PN compared with ETF (P < 0.0001), but this was reversed by the end of PN. Bone marrow recovery, hospital stay and positive blood cultures were similar in the two groups. Hypomagnesaemia, hypophosphataemia and biochemical zinc deficiency were common in both groups but hypoalbuminaemia and biochemical selenium deficiency were worse in the PN group. In conclusion, both ETF and PN are effective in maintaining nutritional status post-BMT. When ETF is tolerated, it is associated with better nutritional response. With the existing ETF and PN regimens close monitoring of the trace element and mineral status is required.
Assuntos
Transplante de Medula Óssea , Apoio Nutricional , Criança , Diarreia/etiologia , Nutrição Enteral/efeitos adversos , Feminino , Nível de Saúde , Humanos , Tempo de Internação , Masculino , Distúrbios Nutricionais/complicações , Estado Nutricional , Nutrição Parenteral/efeitos adversos , Estudos Prospectivos , Albumina Sérica/metabolismoRESUMO
The aim of this study was to assess the frequency and importance of biochemical abnormality related to parenteral nutrition (PN) in a group of infants, and to devise an appropriate policy for routine biochemical surveillance. A standard monitoring protocol based on widely published guidelines was applied to 30 consecutive patients (age 3 days-3 years) referred to a children's hospital nutritional care team for PN. No serious biochemical abnormalities were observed to arise simply as a consequence of PN. Electrolyte disturbance most commonly occurred before starting PN. Biochemical abnormality was most likely to be found in patients with abnormal fluid and electrolyte losses. Protocols for biochemical surveillance during PN err on the side of caution and often suggest frequent and comprehensive testing. In stable patients such as the surgical newborn, this is both expensive and unnecessary, and simpler monitoring regimes may be used with safety.
RESUMO
We review the pathophysiology of intestinal water and electrolyte transport leading to diarrhoea, the currently available pharmacological strategies for its treatment, and the economic implications of such treatments. Diarrhoea occurs most frequently and is associated with highest mortality in children under 5. Oral rehydration therapy (ORT) is the cornerstone of its management. The safety and efficacy of ORT in the prevention of death from dehydration, both in field and also in hospital settings, are now well established. Because it is also inexpensive, ORT is widely applicable worldwide. More recently, rice-based ORT has emerged, based on well known traditional remedies for diarrhoea in southeast Asia and the Far East. Rice-based ORT has the advantage of being more culturally acceptable, readily available even in rural homes in developing countries, and is more effective in reducing stool output and the duration of diarrhoea, compared with conventional glucose-electrolyte solutions such as World Health Organization ORT. For infants, the well known antidiarrhoeal properties of human milk needs emphasis for a variety of reasons including economic ones. Data concerning the economic benefits to a nations' health budget as a result of nationwide implementation of oral rehydration solution (ORS) use are limited. Available data from individual centres in developing countries, if projected to national level, would incur considerable economic advantage. Except for a few notable infections such as shigellosis, cholera, amoebiasis and giardiasis, the widespread use of antibiotics in acute diarrhoea, still a common practice in many developing countries, has no proven value and may be detrimental. The economic implications of antibiotic abuse in the treatment of diarrhoea in developing countries is enormous. Despite the availability of a wide spectrum of pharmacological agents for diarrhoea reviewed in this article, only a few such agents are of proven clinical efficacy: corticosteroids, aminosalicylates and immunosuppressants in the treatment of inflammatory bowel disease and opioid derivatives such as loperamide which may be useful in protracted diarrhoea in children and in disorders where rapid gastrointestinal transit is the main cause of diarrhoea. Opioids are not recommended for acute infective diarrhoea in childhood. Octreotide, a somatostatin analogue, is reported to be useful in the treatment of secretory diarrhoea due to noninfective causes and in the treatment of intractable diarrhoea associated with AIDS. Its high cost and need for parenteral administration prevent its wider application.(ABSTRACT TRUNCATED AT 400 WORDS)
Assuntos
Diarreia/economia , Diarreia/terapia , Corticosteroides/uso terapêutico , Agonistas alfa-Adrenérgicos/uso terapêutico , Alcaloides/uso terapêutico , Ácidos Aminossalicílicos/uso terapêutico , Antibacterianos/uso terapêutico , Anti-Inflamatórios não Esteroides/uso terapêutico , Antidiarreicos/uso terapêutico , Bromocriptina/uso terapêutico , Diarreia/fisiopatologia , Dietoterapia , Farmacoeconomia , Hidratação , Humanos , Imunossupressores/uso terapêutico , Metais/uso terapêutico , Entorpecentes/uso terapêutico , Ácidos Nicotínicos/uso terapêutico , Fenotiazinas/uso terapêutico , Somatostatina/uso terapêuticoRESUMO
Traveller's diarrhoea (TD) is an enteric infective disease seen in 30 to 50% of the 30 million people who travel annually from the developed world to developing countries. It is the commonest disorder affecting international travellers. Although usually trivial, a significant minority of patients may develop a more protracted disease with an attendant increase in morbidity. This may then incur financial costs to the travellers themselves and to the healthcare systems of the host and origin countries. Advice regarding risk avoidance has so far proved ineffective in altering the behaviour of travellers and consequently the incidence of TD among them. Hard data are lacking on the cost effectiveness and cost benefit of prophylaxis and treatment of TD. Thus, we have attempted to quantify, as far as is possible, the total cost of TD and to set this against the potential savings from avoiding TD if prophylaxis or treatment options were universally employed. It must be noted that the financial benefit-cost ratio of an intervention may not be the most suitable measure of its desirability. However, such concepts as the success of travel are inherently difficult to quantify in economic terms. This analysis applies to travellers from the UK and economic variations between countries will after the outcomes obtained. Nevertheless, given these necessary constraints, all possible treatment options have a more favourable benefit-cost ratio than prophylaxis. In conclusion, the case for prophylaxis is not strong except in selected high risk groups, and limited self-treatment seems economically justified.
Assuntos
Diarreia/economia , Diarreia/terapia , Viagem , Diarreia/epidemiologia , HumanosRESUMO
OBJECTIVE: To assess the evidence that diarrhoea is an important cause of growth faltering in young children in developing countries. DESIGN: Prospective, longitudinal cohort study. SETTING: Worker's compounds on commercial farms in Shamva, rural Zimbabwe. SUBJECTS: 204 children < 12 months old were enrolled, 73 from birth. The median age at enrolment was 4 months. Eleven children died and 39 were lost to follow-up. INTERVENTIONS: Prospective weekly diarrhoea surveillance by farm health workers and monthly anthropometry. RESULTS: Growth faltering was severe, but there was little difference in average rates of growth between children with frequent diarrhoea and infrequent diarrhoea. The results of an interval-based data analysis were consistent with there being only a transient effect of diarrhoea on weight gain. Estimation of weight faltering following episodes of diarrhoea and the rate of return to the trend in the 9-14 month age range, indicated that weight loss associated with each episode was small (approximately 2%) and return to the child's trend was 90% complete within a month. At older ages than this, weight loss appeared to be less, and estimates were not statistically significant. CONCLUSIONS: These observations lend weight to the hypothesis that recurrent episodes of diarrhoea are not a potent cause of growth faltering in early childhood except in a small minority of largely catastrophic cases. Inadequate food intake is a more plausible explanation.
PIP: In Zimbabwe, health workers collected data on diarrhea incidence every week and anthropometric data once a month from 204 children aged less than 12 months to examine the association between diarrhea and growth faltering. 73 children were enrolled at birth. 148 children were followed throughout the entire study. 11 children died (8 because of diarrhea or protein-energy malnutrition). 39 children were lost to follow-up. The children's parents were farm laborers who lived on large-scale commercial farms in Shamva district. Diarrhea incidence peaked between 13 and 18 months. In 91% of attacks, the diarrhea was watery rather than bloody. 31 children had more than 9 diarrhea episodes (high diarrhea frequency). 25 had no more than 4 diarrhea episodes (low diarrhea frequency). There was little difference in the children's mean weight and mean length from 1 to 30 months of age between high and low diarrhea frequency subjects. The average loss of overall growth per diarrhea episode in the age range 9-23 months was 51 g and 0.18 cm. In the age range of 9-14 months, weight loss after the diarrhea episode was 2.3% of body weight, and 90% of the sudden weight decline below the child's trend was recovered in 30 days. Weight loss was less than 2.3% among older children. A 2.3% weight loss in an 8 kg child is 180 g. Assuming that diarrhea is responsible for the entire weight loss (about 66 g/episode), the reduction in overall growth is about 120 g (1.5%). The total energy needed to accumulate 120 g is 480 kcal; thus, a child would require an additional 2-3 kcal/kg/day (a small amount) to gain 120 g. These findings support the hypothesis that recurrent diarrhea episodes do not induce growth faltering except in a few cases. Inadequate food intake is a more plausible explanation.
Assuntos
Diarreia Infantil/fisiopatologia , Crescimento , Antropometria , Fenômenos Fisiológicos da Nutrição Infantil , Estudos de Coortes , Diarreia Infantil/mortalidade , Humanos , Lactente , Estudos Longitudinais , Estudos Prospectivos , População Rural , ZimbábueRESUMO
In most adults who believe themselves to be food intolerant there is no objective supporting evidence. It has therefore been proposed that the misperception of intolerance to food is linked to psychiatric illness or personality disorder. This hypothesis was tested in a community-derived sample of individuals who attributed an adverse symptom to a type of food. A random mailing recruited 955 participants aged > or =18 years, of whom 232 perceived themselves to be food intolerant (PFI). All recruits were sent two questionnaires, the General Health Questionnaire-28 (GHQ-28) and the shortened version of the Eysenck Personality Questionnaire (EPQ-R). A total of 535 GHQ-28 and 518 EPQ-R forms were returned that were correctly completed, an overall response rate of 55%. For the subscales of the EPQ-R, neuroticism was greater in those with a PFI than those without. Women with a PFI were more extroverted than control women. For the GHQ-28 subscales, women with a PFI had significantly higher scores than control women on somatic symptoms, anxiety, insomnia, and severe depression. There was a greater percentage of psychiatric caseness among women with a PFI than among men with a PFI or control women. Nevertheless, this percentage was no greater than that reported among a reference sample derived from NHS and university staff. It is concluded that perceived food intolerance is associated with psychological distress in women with a PFI, and neurotic symptoms in both men and women with a PFI, but there is no greater prevalence of psychiatric disorder among women or men with a PFI than there is in some professional groups.
Assuntos
Hipersensibilidade Alimentar/psicologia , Transtornos Neuróticos/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Extroversão Psicológica , Feminino , Nível de Saúde , Humanos , Incidência , Introversão Psicológica , Masculino , Pessoa de Meia-Idade , Inventário de Personalidade/estatística & dados numéricos , Estudos de Amostragem , Autoavaliação (Psicologia) , Fatores Sexuais , Transtornos Somatoformes/epidemiologia , Inquéritos e Questionários , Reino Unido/epidemiologiaRESUMO
An ultrasonic technique was used to compare gastric emptying after a feed of expressed breast milk and formula milk in a blind, cross over study of preterm infants. Fourteen infants (median gestational age 33 weeks) were studied on 46 occasions. Each infant received a nasogastric feed of either expressed breast milk or formula milk, and the alternative at the next feed. Real time ultrasound images of the gastric antrum were obtained and measurements of antral cross sectional area (ACSA) were made before the feed and then sequentially after its completion until the ACSA returned to its prefeed value. The half emptying time (50% delta ACSA) was calculated as the time taken for the ACSA to decrease to half the maximum increment. On average, expressed breast milk emptied twice as fast as formula milk: mean 50% delta ACSA expressed breast milk 36 minutes; formula milk 72 minutes. The technique was reproducible and there was no significant difference between the emptying rates of feeds of the same type for an individual infant. These data show that breast milk has a major effect on gastric emptying, which may have important implications for preterm infants who have a feed intolerance due to delayed gastric emptying.
Assuntos
Esvaziamento Gástrico , Recém-Nascido Prematuro/fisiologia , Animais , Peso ao Nascer , Método Duplo-Cego , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Masculino , Leite , Leite Humano , Fatores de Tempo , UltrassonografiaRESUMO
Gastro-oesophageal reflux is common in preterm infants, but the role of gastric emptying as a causal factor has not been studied before. Gastric emptying was therefore measured in 19 healthy preterm infants (median gestational age 32 weeks) while concurrently measuring 24 hour lower oesophageal pH, using an antimony pH electrode, positioned manometrically. Real time ultrasonic images of the gastric antrum were obtained, and measurements of antral cross-sectional area (ACSA) were made immediately before a nasogastric feed and then during subsequent gastric emptying until ACSA returned to its pre-feed value. Half emptying time (50% delta ACSA) was calculated as the time taken for the ACSA to fall to half the maximal postprandial increment. Mean (SEM) reflux index for the group was 11.9 (2.0)%; number of reflux episodes per 24 hours: 15.4 (1.7); and number of reflux episodes longer than five minutes 5.5 (0.8). Average half emptying times for an individual infant were: median (range) 46 (18-105) minutes. There was no association between gastric emptying rates and any of the indices of gastro-oesophageal reflux, either during the entire 24 hour period for which the lower oesophageal pH was recorded, or in the postprandial periods after the feeds which were studied ultrasonically. Gastro-oesophageal reflux was also unrelated to feed volume and feed type. Asymptomatic gastro-oesophageal reflux is common in preterm infants, but gastric emptying time is not a determinant of it. Inappropriate relaxation of the lower oesophageal sphincter or abnormal oesophageal motility offer more plausible explanations.
Assuntos
Esvaziamento Gástrico/fisiologia , Refluxo Gastroesofágico/fisiopatologia , Doenças do Prematuro/fisiopatologia , Esôfago/metabolismo , Feminino , Humanos , Concentração de Íons de Hidrogênio , Alimentos Infantis , Recém-Nascido , Recém-Nascido Prematuro , Masculino , Antro Pilórico/diagnóstico por imagem , UltrassonografiaRESUMO
To test the hypothesis that relative pancreatic dysfunction is a determinant of catch up growth in small for gestational age (SGA) babies, 47 such babies (median gestation 38 weeks; range 27-41) and 41 appropriate for gestational age (AGA) babies matched for sex, race, and gestational age were recruited. Anthropometry was performed within 48 hours of birth and at 6 months. Faecal chymotrypsin activities were measured at 0-2 days, 14 days, 6 weeks and 6 months. At 6 months 30 SGA infants and 25 AGA infants were remeasured. In each group, median stool chymotrypsin activities doubled between 0-2 days and 6 months (9.0-25.5 IU/g SGA group; 11.6-25.3 IU/g AGA group). SGA babies had significantly lower chymotrypsin activities at 14 days (10.9 U/g) than AGA babies (15.5 U/g). In the SGA group faecal chymotrypsin activities at 0-2 days were strongly correlated with both catch up weight and with catch up length when corrected for the effects of birthweight. These data show that impaired pancreatic exocrine function at birth is associated with severe intrauterine malnutrition and with impaired catch up growth during the first 6 months of life.
Assuntos
Quimotripsina/análise , Recém-Nascido Pequeno para a Idade Gestacional/crescimento & desenvolvimento , Pâncreas/fisiopatologia , Estudos de Casos e Controles , Fezes/enzimologia , Seguimentos , Humanos , Lactente , Recém-NascidoRESUMO
AIM: To assess the efficacy of cisapride in reducing ileus persisting to the tenth postoperative day after neonatal abdominal surgery. METHODS: A prospective, randomised, double blind trial comparing rectal cisapride (1.4-2.3 mg/kg/day) with placebo over seven days was undertaken in 33 neonates. RESULTS: Seven of 12 (58%) patients receiving placebo and eight of 11 (73%) receiving cisapride achieved a first sustained feed during treatment. Of those receiving cisapride, the first sustained feed occurred at 2.3 days (SEM 0.6) compared with 4.7 days (SEM 0.8) with placebo. By the seventh day the mean daily net enteral balance was 69 (SEM 18) ml/kg in the cisapride subgroup and 17 (SEM 8) ml/kg for those receiving placebo. Stool was passed on 6.3 (SEM 0.4) treatment days in the cisapride subgroup compared with 4.1 (SEM 1.0) treatment days in the placebo subgroup. CONCLUSION: Cisapride is effective in neonates with a prolonged ileus after abdominal surgery.
Assuntos
Fármacos Gastrointestinais/uso terapêutico , Motilidade Gastrointestinal/efeitos dos fármacos , Obstrução Intestinal/tratamento farmacológico , Piperidinas/uso terapêutico , Complicações Pós-Operatórias/tratamento farmacológico , Administração Retal , Cisaprida , Método Duplo-Cego , Humanos , Recém-Nascido , Estudos ProspectivosRESUMO
The steatocrit is a simple and easily repeated assay for measuring the fat content of infants' stools. However, we and others have experienced technical difficulties in its use. Three modifications were therefore made to the original procedure: incorporation of a lipid-soluble dye, improved homogenization and a heating step. The modified method was used to measure the stool fat content of young children with and without clinical steatorrhoea. Validation of the modified steatocrit is presented, together with examples of its application to both clinical research and clinical practice.
Assuntos
Fezes/química , Lipídeos/análise , Doença Celíaca/metabolismo , Pré-Escolar , Corantes , Interpretação Estatística de Dados , Diarreia/terapia , Gorduras na Dieta/administração & dosagem , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Métodos , Distribuição Aleatória , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , TemperaturaRESUMO
This is a case report of a previously asymptomatic 11-year-old boy who developed chronic intestinal pseudo-obstruction. Barium studies revealed grossly disordered motility of the proximal small bowel, and ganglion cells in a gastric biopsy were mildly abnormal. Treatment with conventional prokinetic agents and gastrojejunostomy were ineffective. Intravenous cisapride induced an immediate remission, which has been maintained subsequently by rectal administration of the drug.
Assuntos
Pseudo-Obstrução Intestinal/tratamento farmacológico , Piperidinas/uso terapêutico , Administração Retal , Criança , Doença Crônica , Cisaprida , Humanos , Injeções Intravenosas , Pseudo-Obstrução Intestinal/patologia , Intestino Delgado/patologia , Masculino , Piperidinas/administração & dosagemRESUMO
To investigate the possibility that small intestinal dysmotility is a cause of long-standing and persistent symptoms in patients with malrotation, we retrospectively reviewed 94 patients operated on for intestinal malrotation. In 50 patients operated on during the neonatal period, associated abnormalities were common (24%) and all presented with obstructive symptoms; only three (6%) had continuing mild symptoms the postneonatal period (aged 1 month to 1 year), five of whom (22%) had other abnormalities. Recurrent vomiting was the most common presentation (11/23); only 5 patients (21%) had acute obstruction. Response to operation was good in 18 (78%) and two had persistant symptoms; both died. Twenty-one patients presented beyond infancy (aged greater than 1 year), only 19 of whom had symptoms of less than 2 months' duration. Eight (47%) of those with long-standing symptoms had no relief from operation. Small bowel motility was recorded manometrically in 4 patients with long-standing symptoms. The findings suggest that small intestinal dysmotility may be common in patients with malrotation and persistant symptoms.
Assuntos
Motilidade Gastrointestinal , Intestinos/anormalidades , Adolescente , Criança , Pré-Escolar , Humanos , Lactente , Recém-Nascido , Obstrução Intestinal/etiologia , Intestinos/cirurgia , Recidiva , Estudos RetrospectivosRESUMO
The long-term complications of loose stools and failure to thrive following resection of the ileocecal region with end-to-end ileocolic or jejunocolic anastomosis are well recognized. We report four cases of a previously undescribed insidious complication that has developed in 8% of our patients after a latent period of many years following the primary operation; that of perianastomotic ulceration leading to severe iron deficiency anemia.
Assuntos
Colo/cirurgia , Doenças do Colo/etiologia , Doenças do Íleo/etiologia , Íleo/cirurgia , Complicações Pós-Operatórias , Úlcera/etiologia , Adolescente , Anastomose Cirúrgica/efeitos adversos , Anemia Hipocrômica/etiologia , Criança , Pré-Escolar , Colo/patologia , Doenças do Colo/patologia , Diarreia/etiologia , Feminino , Seguimentos , Humanos , Doenças do Íleo/patologia , Íleo/patologia , Lactente , Masculino , Complicações Pós-Operatórias/patologia , Úlcera/patologiaRESUMO
The medical records of 74 neonates dependent on parenteral nutrition for at least 21 days after emergency abdominal surgery (performed between 1988 and 1992) were reviewed respectively. The role of enteral starvation, prematurity, composition and duration of parenteral nutrition, and sepsis in the evolution of parenteral nutrition-related cholestasis was evaluated by multiple regression analysis. The most important factors for cholestasis were low gestational age (median, 34 weeks), early exposure to parenteral nutrition, and sepsis. Episodes of sepsis were associated with a 30% increase in the bilirubin level. Enteral starvation and composition and the duration of parenteral nutrition solutions did not correlate significantly with the development of cholestasis. Prevention of sepsis should be the priority in minimising cholestasis in postsurgical neonates who are dependent on parenteral nutrition.
Assuntos
Colestase/etiologia , Doenças do Prematuro/cirurgia , Nutrição Parenteral Total , Cuidados Pós-Operatórios , Complicações Pós-Operatórias/etiologia , Abdome/cirurgia , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Análise Multivariada , Fatores de Risco , Sepse/etiologiaRESUMO
The hepatic histology and clinical status of 37 children on long-term parenteral nutrition (PN) referred for consideration of small bowel transplantation were determined. Seventy five percent of the children had splenomegaly and plasma bilirubin level of greater than 100 mumol/L. All of 21 children who underwent liver biopsy, had increased fibrosis, but only half had established cirrhosis. Thirty-one children were considered to be in need of transplantation (combined liver and bowel transplant, 29; isolated bowel transplant, 2), but only 13 were stable enough to be placed on the transplant list. Seven out of the thirteen children waiting have died because of lack of size-matched organs, and the overall mortality rate of the 37 children was 70%. The main risk factors for death within 6 months were bilirubin level of greater than 100 mumol/L, splenomegaly, and cirrhosis (P = .01). The natural history of PN-associated liver disease is that of progressive liver failure and death 6 to 12 months after onset of cholestasis, defined as bilirubin level of greater than 100 mumol/L. The development of cirrhosis occurs after the onset of jaundice, so early referral may also permit some children to be offered isolated bowel transplantation, which has better outcome than combined liver and bowel transplantation.
Assuntos
Enteropatias/complicações , Intestino Delgado/transplante , Cirrose Hepática/cirurgia , Transplante de Fígado , Nutrição Parenteral/efeitos adversos , Adolescente , Criança , Pré-Escolar , Humanos , Lactente , Enteropatias/terapia , Cirrose Hepática/etiologia , Seleção de Pacientes , Prognóstico , Taxa de Sobrevida , Fatores de TempoRESUMO
The protean manifestations of child abuse continue to cause diagnostic difficulty. Recent observations of the high mortality in victims of Munchausen syndrome by proxy, and their siblings, reinforce the need for early diagnosis and appropriate intervention. We report the nasal manifestations which unmasked Munchausen syndrome by proxy in an infant who presented with intestinal and peri-orifical signs masquerading as Crohn's disease. The possibility of Munchausen syndrome by proxy should be considered in an infant with persistent nasal excoriation presenting as part of an undiagnosed illness.