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1.
The incidence of transient hypogammaglobulinaemia of infancy (THI) detected in a major paediatric centre over a 10 year period was examined. A total of 2468 subjects less than 2 years of age had an IgG measurement taken between July 1979 and March 1990. Subjects with known immunodeficiencies were excluded. Fifteen patients were classified as having THI with an initial IgG level less than the fifth centile followed by a second measurement within the normal range. A further 24 patients were identified as having possible THI with a single low IgG concentration. There were 60,174 live births each year in Victoria in the years 1979-88. This gives an incidence of proved THI of 23 per 10(6) births, and including proved and probable THI an incidence of 61 per 10(6) live births. Of those patients with proved THI 12/15 had symptoms of either atopic disease or food allergy/intolerance and three had gastrointestinal symptoms without any evidence of atopic disease. At presentation 12/15 (80%) were IgA deficient and 9/15 had IgM concentrations less than the 20th centile for age. It is suggested that in view of the preponderance of atopic and food intolerant patients that subclinical protein loss from the bowel due to allergic inflammation may be a contributing factor to the development of THI in some patients.  相似文献   

2.
As part of a population based regional review of all neonatal readmissions, the incidence of dehydration with hypernatraemia in exclusively breast fed infants was estimated. All readmissions to hospital in the first month of life during 1998 from a population of 32 015 live births were reviewed. Eight of 907 readmissions met the case definition, giving an incidence of at least 2.5 per 10 000 live births. Serum sodium at readmission varied from 150 to 175 mmol/l. One infant had convulsions. The sole explanation for hypernatraemia was unsuccessful breast feeding in all cases. The eight cases are compared with the 65 cases published in the literature since 1979. Presentation, incidence, risk factors, pathophysiology, treatment, and prevention are discussed.  相似文献   

3.
Hypernatraemic dehydration and breast feeding: a population study.   总被引:2,自引:0,他引:2  
As part of a population based regional review of all neonatal readmissions, the incidence of dehydration with hypernatraemia in exclusively breast fed infants was estimated. All readmissions to hospital in the first month of life during 1998 from a population of 32 015 live births were reviewed. Eight of 907 readmissions met the case definition, giving an incidence of at least 2.5 per 10 000 live births. Serum sodium at readmission varied from 150 to 175 mmol/l. One infant had convulsions. The sole explanation for hypernatraemia was unsuccessful breast feeding in all cases. The eight cases are compared with the 65 cases published in the literature since 1979. Presentation, incidence, risk factors, pathophysiology, treatment, and prevention are discussed.  相似文献   

4.
The records of 62,107 neonates cared for at three neonatal referral units between 1979 and 1985 were analysed with regard to the number of lumbar punctures (LP) performed and the results obtained. The mean perinatal mortality rate was 9.3/1000 live births and the neonatal mortality rate was 5.9/1000. The units were comparible in this respect. Two hundred and sixty three (0.4%) babies weighed less than 1.0 kg at birth and 510 (0.8%) babies weighed 1.0–1.5 kg. A total of 1554 LPs were performed on 1084 babies, 1.7% of the whole population. Seventeen babies were diagnosed as having meningitis following CSF examination; 5 weighed less than 1.5 kg (VLBW) (incidence of meningitis 6.5/100 births) and 12 weighed more than 1.5 kg (incidence 0.2/1000 live births). An LP was performed on 44% of VLBW babies and 1.2% of babies weighing more than 1.5 kg. In both groups approximately 1.5% of CSF samples were positive. Eight of the 17 positive results were obtained during the first 3 postnatal days. Only one VLBW baby had meningitis on the 1st day of life (1.3/1000). In view of the low incidence of meningitis and the risk factors associated with an LP it is questionable whether this procedure should be a routine investigation in the VLBW newborn.  相似文献   

5.
Between 1982 and 1988, familial hypophosphataemic rickets (FHR) was diagnosed in 24 children, in nine during screening of the families of index patients. The average annual incidence was 0.2/1000 live births. There were 16 boys and 8 girls in 10 families, of which nine had more than one affected child. Their ages at the onset of the disease ranged between 10 months and 14 years (mean 6.9 yrs). Growth retardation and bowing of the legs were the most prominent features, observed in all index patients and in four of the patients diagnosed by screening. Treatment with 1 alpha-hydroxyvitamin D3 and phosphates was associated with acceleration of growth in all children, healing of rickets in 21, and normalization of the serum phosphate in 22. Two children with late diagnosis are now older than 16 years with a final height below the 3rd centile. Three more pubertal children are also shorter than the 3rd centile. In areas where nutritional rickets is common, FHR is likely to be missed and the treatment delayed with grave consequences; in particular, growth retardation and bone deformity.  相似文献   

6.
By screening the patient list of four Sicilian centers of gastroenterology and those with gluten-free product consumption, 1074 patients (607 females and 467 males) with celiac disease, diagnosed between 1975 and 1989, were identified. A maximum cumulative incidence rate by birth cohort was reached in 1986 (1.65/1000). When the incidence rate was adjusted for the years of follow-up, the actual standardized rate was 3 cases per 1000 live births. Growth failure and chronic diarrhea were the most common symptoms, but a diminishing trend for chronic diarrhea was observed when symptoms were distributed by year of diagnosis. Even though 61.1% of all cases were diagnosed within six months from the onset of symptoms, mean age at diagnosis showed an increasing trend, from less than two years to approximately four years of age. The results of our study showed an increasing incidence of celiac disease due to diagnosis of less typical cases at an older age and also to a steady increase in the rate of diagnosis of cases with a classic clinical picture.  相似文献   

7.
Among 229,846 children born alive between 1971 and 1980 in the districts Erfurt and Suhl--this figure coincide with the total number of live births a year in the GDR--1638 patients with structural congenital heart disease were identified. The study is based on an analysis of cardiac patients of this districts and autopsy protocols of all deceased children of the 10-year period. Additional attempt has been made to assess the proportion of cases in adults undergoing cardiac catheterisation with congenital heart disease, undetected in childhood. In our study we found malformations of the heart in 7.13 of 1,000 live births. There is agreement with the results of foreign studies. It is evident, however, that the result requires correction to 8-10/1,000. Our study of the incidence of congenital heart disease in children born between 1971 and 1980 concludes that over the 10-year period the overall incidence has remained constant.  相似文献   

8.
The purpose of this study was to determine whether cow milk-specific antibody responses correlated with the development of clinical tolerance in cow milk-allergic children. Double-blind, placebo-controlled food challenges were performed annually in 29 patients with cow milk allergy. Clinical reactivity was lost in 11 (38%) of 29 patients. The median age for all patients at the time of diagnosis by these food challenges was 3 years; more than 80% of patients in each group had atopic dermatitis as part of their presenting symptoms. Casein-specific and beta-lactoglobulin-specific IgE, IgG, IgG1, and IgG4 antibody concentrations were analyzed in all patients at regular intervals. In the patients becoming clinically tolerant to cow milk, the IgE-specific antibody concentrations and IgE/IgG-specific ratios for both milk proteins were lower initially and decreased significantly with time, in comparison with those in the group who retained clinical sensitivity. The concentrations of IgG1- and IgG4-specific antibody to casein and the IgE/IgG1 and IgE/IgG4 ratios for both casein and beta-lactoglobulin were significantly less in the patients losing clinical reactivity. No differences in the IgG-specific concentrations were observed in either group at any of the evaluation times noted above. Monitoring similar casein-specific and beta-lactoglobulin-specific IgE concentrations and IgE/IgG ratios may help predict which patients will ultimately lose their clinical reactivity to cow milk.  相似文献   

9.
BACKGROUND: Transient hypogammaglobulinemia of infancy (THI) results from a delay in the maturation of immunoglobulin production. METHODS: The clinical and immunologic data of 40 patients with THI are presented. Clinically, the majority of patients presented with recurrent respiratory infections and otitis media, bronchitis and/or bronchial asthma and recurrent gastroenteritis. RESULTS: Ten of 40 children had isolated low IgG; isolated low IgA and isolated low IgM were detected in one and three patients, respectively. At initial evaluation, levels of all three immunoglobluins were low in nine patients. Ten patients had diminished IgG and IgA levels, six had diminished IgA and IgM levels and one had low IgG and IgM levels. Two patients were given intravenous immunoglobulin replacement therapy for 1 year. None of the patients has experienced serious infections during their follow-up period. CONCLUSIONS: Prospective evaluation of patients (age range 5-60 months) revealed that immunoglobulin levels in 33 patients recovered before 36 months of age. Seven patients still had low immunoglobluin levels at 40-57 months of age. Three of these patients had low levels of both IgG and IgA, while two patients had low IgM levels and a further two patients had low IgA levels.  相似文献   

10.
Abstract Sixtynine children with case histories of food intolerance and 30 food tolerant children with atopic dermatitis have been investigated regarding serum IgE levels and IgE-, IgG, and IgA-antibodies to some common foods. Children with food intolerance had significantly higher IgE levels and to a larger extent specific IgE antibodies to the tested allergens. IgE antibodies to cow's milk were found in 71% of the children with histories of cow's milk allergy but occurred also in similar titers in 27% of milk tolerant children with other food allergies. IgE antibodies to egg-white occurred in 88% of egg allergies, but low and moderate titers were also found in 17% of children without food intolerance. However, all children with high titers had symptoms of egg allergy. IgE antibodies to the fish allergen were only found in fish allergic children while IgE antibodies to soy-bean and green peas were found less consistently. The level of serum IgA antibodies to milk was similar in both groups. The IgG antibody titers to all tested food antigens seemed to parallel the IgE antibody titer to the same food. It was not possible to correlate the IgG antibody titers to symptoms.  相似文献   

11.
AIMS: To review the results of long term high dose intravenous immunoglobulin treatment. METHODS: 162 treatment years in 18 patients with hypogammaglobulinaemia who received intravenous immunoglobulin treatment between 1973 and 1993 were reviewed. RESULTS: A mean dose of 0.42 g/kg immunoglobulin resulted in a mean trough IgG concentration on the 23.5th centile for age. The subjects enjoyed a good standard of health. Infection rates were similar to the general paediatric population and a similar pattern of infections occurred. There were only 0.06 episodes of pneumonia and 0.11 hospital admissions per year of treatment. The development of chronic pulmonary disease was significantly related to trough IgG concentrations less than the 10th centile (p < 0.009), however, this developed in only two children after the start of treatment. All children had normal growth parameters. Adverse reactions to immunoglobulin infusions reduced from 9.1% to 0.8% after the introduction of low pH modified intravenous immunoglobulin in 1986. Although minor, transient increases in liver transaminase values were common; none of the 11 patients tested by hepatitis C polymerase chain reaction were positive. CONCLUSION: Children with hypogammaglobulinaemia who are receiving replacement treatment grow normally and have an infection rate similar to that of non-immunodeficient children. No evidence of transmission of hepatitis C virus by the Commonwealth Serum Laboratories immunoglobulin was found.  相似文献   

12.
National immunodeficiency registers in several countries have reported prevalence but not incidence rates for severe combined immunodeficiency (SCID). The objective of this study was to document the incidence and type of SCID in Australia, the age and clinical features at presentation and short-term management. The Australian Paediatric Surveillance Unit conducts active, monthly, national surveillance of rare disorders with reporting by child-health specialists. Between May 1995 and December 2001, clinicians provided clinical and laboratory data on children newly diagnosed with SCID. Thirty-three incident cases of SCID were identified [incidence 1.8/10(5) live births per annum; 95% confidence interval (CI) 1.2-2.5]. Twenty-six children had classical SCID (1.45/10(5) live births; 95% CI, 0.9-2.0) and 20 (77%) of these were boys. Classical SCID was X-linked in 13 children, autosomal recessive (AR), not further classified in four, and attributed to adenosine deaminase deficiency (ADA) in four, interleukin-7 receptor alpha chain deficiency in one, Ommen syndrome in two, and Di George syndrome in two. Twenty-one (81%) received bone marrow/stem cell transplantation, three of whom died between 1 and 4 months after transplantation (two ADA deficient, one AR). Seven children had atypical SCID, five of whom died within 1-4 yr of diagnosis. Most children with SCID presented with failure to thrive and recurrent infections and there was no significant delay between presentation and diagnosis. The reported national incidence of classical SCID in Australia (1.45/10(5) or approximately 4 cases per year or 1/69,000 live births) is consistent with the rate (1.4/10(5)) previously reported in Victoria. Diagnosis is rarely delayed, and transplantation is associated with good short-term survival.  相似文献   

13.
Sixtynine children with case histories of food intolerance and 30 food tolerant children with atopic dermatitis have been investigated regarding serum IgE levels and IgE-, IgG, and IgA-antibodies to some common foods. Children with food intolerance had significantly higher IgE levels and to a larger extent specific IgE antibodies to the tested allergens. IgE antibodies to cow's milk were found in 71% of the children with histories of cow's milk allergy but occurred also in similar titers in 27% of milk tolerant children with other food allergies. IgE antibodies to egg-white occurred in 88% of egg allergies, but low and moderate titers were also found in 17% of children without food intolerance. However, all children with high titers had symptoms of egg allergy. IgE antibodies to the fish allergen were only found in fish allergic children while IgE antibodies to the fish allergen were only found in fish allergic children while IgE antibodies to soy-bean and green peas were found less consistently. The level of serum IgA antibodies to milk was similar in both groups. The IgG antibody titers to all tested food antigens seemed to parallel the IgE antibody titer to the same food. It was not possible to correlate the IgG antibody titers to symptoms.  相似文献   

14.
Mucopolysaccharidosis type 1 (MPS1) is an autosomal recessive disorder with severe, moderate and mild phenotypes: Hurler, Hurler-Scheie and Scheie syndromes. We estimated incidence (2001-2006) and prevalence (2002 census) of MPS1 in the Irish Republic (ROI) using population data, database and chart review of all live MPS1 patients attending two specialised centres. Patient genotypes, ethnicity, province of origin, age at diagnosis and presenting features were recorded. Thirty-one patients (14 females, 17 males) were alive, 27 of whom were <15 years. Twenty-six patients had Hurler syndrome, four had Hurler-Scheie and one had Scheie syndrome. The birth incidence was 1 in 26 206 births with a carrier frequency of 1 in 81. Of note, 19/26 (73%) Hurler patients were Irish Travellers. Amongst Irish Travellers the incidence was 1 in 371 with a carrier frequency of 1 in 10. This is the highest recorded incidence worldwide. Given the morbidity and mortality associated with delayed treatment we recommend targeted newborn screening for this population.  相似文献   

15.
ABSTRACT. In a survey carried out to determine the incidence of Duchenne muscular dystrophy in South East Norway, 38 cases were ascertained among 149 975 live male births during the years 1968–1978. The overall incidence is 1:3917 in live male births (25.33×10-5). This incidence is compared with those in other studies. There is an apparent drop in incidence in the last cohort studied, and the possible role of genetic councelling and antenatal sexing are discussed.  相似文献   

16.
Group B streptococcus (GBS) is a common cause of early-onset sepsis in neonates. The most recent reviews describing incidence, diagnosis, treatment, and outcome evaluated data on patients from the early 1980s. To obtain current information about this disease, we retrospectively evaluated data on neonates with GBS early-onset sepsis from nine hospitals in the United States between Jan. 1, 1987, and Dec. 31, 1989. There were 245 infants with GBS bacteremia identified among 61,809 live births, resulting in an incidence of 0.32%. Ninety-six infants (39%) were preterm (less than 38 weeks of gestational age). Maternal risk factors for infected preterm and term infants were similar. Antibiotics were administered during parturition in 10% of infants with bacteremia. Mothers of preterm infants received antibiotics up to 48 hours before delivery; mothers of term infants received antibiotics less than 4 hours before delivery. All preterm infants with bacteremia had symptoms; 22% of term infants with bacteremia had no symptoms. Group B streptococcal meningitis was confirmed in 6.3% of infants. Although 86% survived, GBS sepsis increased the birth weight-specific mortality rate up to eightfold in preterm infants and more than 40-fold in term infants. Although the incidence of GBS early-onset sepsis is not changing, we speculate that the improved birth weight-specific survival rate and the changing clinical presentation are due to improved intrapartum and neonatal management.  相似文献   

17.
Low birthweight (LBW) infants are at risk of increased mortality, impaired growth, and increased morbidity. We studied the progress of all LBW infants (less than 2.5 kg) born in a West African village where there is a resident pediatrician and midwife. There were 426 live births during the 8 years studied of which 42 (10 per cent) were of LBW. There were 17 infant deaths (IMR = 40/1000). Mean birthweight and gestation of infants dying in the first year were significantly lower than those of all live birth (2.33 kg v. 2.99 kg, P less than 0.001; 36.9 w v. 38.9 w, P less than 0.05). The mean weights of the 32 surviving LBW infants (15 preterm, 17 full term, small for gestational age) were compared with those of 32 full term, appropriate for gestational age matched controls over the first year. Growth velocities of paired groups were identical. The significant differences in weight at 1 year (P less than 0.005) reflected those at birth (P less than 0.001). There were no significant differences in clinic attendance rate or incidence of diarrhoea and respiratory disease between the four groups. We conclude that LBW is a strong risk factor for infant mortality, but not for diarrhoeal and respiratory morbidity in a community with ready access to obstetric and pediatric care.  相似文献   

18.
Revised intrauterine growth curves for an Australian hospital population   总被引:5,自引:0,他引:5  
ABSTRACT. Intrauterine growth curves require periodic revision because of changes in population, socio-economic factors and technology used in obstetric care. Anthropometric measurements were derived from consecutive livebirths in the Royal Women's Hospital in 1979 and all those born before 35 weeks in 1977 and 1978; at gestational ages less than 30 weeks, data previously published was also incorporated. Infants were included if an ultrasonic examination of the uterus had been performed in the first 20 weeks of pregnancy or gestation based on "certain" menstrual history was confirmed clinically. From data on 3120 infants, intrauterine growth curves from 24--42 weeks' gestation were prepared; compared with births in the same hospital in 1966, there was generally an elevation of all centiles, particularly so for the 10th centile for weight after 37 weeks' gestation. Factors in the infant such as sex and ethnic origin and also maternal factors, especially pregnancy weight and height should be considered when using standard intrauterine growth charts.  相似文献   

19.
Of 6,274 consecutive live-births during 1975–78, 170 (2.7 percent) infants were diagnosed to have 241 major congenital malformations. The incidence of malformations among preterm was almost twice (5.3 per cent), while two-thirds of infants with internal malformations weighed less than 50th centile for their gestational age at birth. Musculoskeletal defects accounted for one-third of all malformations. Out of systemic defects, central nervous system anomalies were most frequent (12.9 per cent) while other system defects (except respiratory system) had an identical frequency of about 10 per cent. The incidence of neural tube defects was found to be 7 per 1000 total births. Among individual anomalies, club foot, spina bifida, polydactyly, left-to-right cardiac shunt, clef lip ±cleft plate, hydrocele, Down syndrome and congenital dislocation of hips had a frequency greater than 1 per 1000 live-births.  相似文献   

20.
A positive association between Helicobacter pylori antibodies and food allergy presenting with gastrointestinal symptoms has recently been reported. A subset of a H. pylori strain possesses an antigen, CagA, as a virulence factor. Anti‐ H. pylori and anti‐CagA IgG titre have been determined in children with atopic dermatitis (AD) as the sole clinical manifestation of food allergy. In this study, thirty patients with AD as the sole clinical manifestation of food allergy were examined (group A). For comparative purposes, 30 patients affected by food allergy with gastrointestinal symptoms (group B) and 30 affected by atopic asthma (group C) were studied. Anti‐ H. pylori and anti‐CagA immunoglobulin G (IgG) were determined in all individuals by means of the enzyme‐linked immunosorbent assay. The anti‐ H. pylori IgG titre was significantly higher in group A and group B vs. group C (p < 0.05); no significant difference was detected between group A and group B (p > 0.05). No significant difference in anti‐CagA titre was found between the groups. These data demonstrate a positive association between H. pylori antibodies and AD as the sole manifestation of food allergy. Further investigations are needed to evaluate the cause–effect relationship between H. pylori seropositivity and AD.  相似文献   

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