Liver disease in alpha 1-antitrypsin deficiency. A retrospective analysis of the influence of early breast- vs bottle-feeding

We identified children with alpha 1-antitrypsin deficiency from the medical records of the Massachusetts General Hospital and Children's Hospital, Boston, and investigated their early feeding history. Between 1969 and 1983, forty children with the deficiency were seen at one or both hospitals. Clinical information was obtained from hospital records and from questionnaires mailed to the parents. Complete morbidity, mortality, and early feeding data were obtained for 32 of the children who were born at 38 to 42 weeks' gestation and whose weights were appropriate for gestational age. We compared the presence of severe liver disease and the death rate of those who had been exclusively breast-fed for one month with those who had been bottle-fed. Severe liver disease was present in eight (40%) of bottle-fed and one (8%) of breast-fed infants. Twenty-four of the 32 infants were still alive at the termination of the study; 12 had been breast-fed and 12 bottle-fed during their first month of life. All eight deceased infants had been bottle-fed. The mortality rate in the bottle-fed group was significantly greater than that of the breast-fed group. Our study suggests that breast-feeding may offer some protection against severe liver disease and early death in infants with alpha 1-antitrypsin deficiency.     

Cognitive development of Yu-Cheng ("oil disease") children prenatally exposed to heat-degraded PCBs.

OBJECTIVE--To compare the cognitive development in Taiwanese children who had been exposed prenatally to high levels of heat-degraded polychlorinated biphenyls (PCBs) with control children who were exposed to background levels. The disorder was called Yu-Cheng, "oil disease," in Taiwan. DESIGN--Matched-pair cohort study. SETTING--Communities in central Taiwan in which there had been a cooking-oil contamination and mass poisoning by heat-degraded PCBs in 1978 through 1979. PARTICIPANTS--One hundred eighteen children born between June 1978 and March 1985 during or after their mothers' consumption of contaminated rice oil; 118 children matched for age, sex, neighborhood, maternal age, and parental education and occupational class; and 15 older siblings of exposed children, born before the poisoning. MAIN OUTCOME MEASURES--Cognitive development measured from 1985 through 1990 using the Chinese versions of the Stanford-Binet test and the Wechsler Intelligence Scale for Children, Revised, RESULTS--The exposed children scored approximately 5 points lower on the Stanford-Binet test at the ages of 4 and 5 years and approximately 5 points lower on the Wechsler Intelligence Scale for Children, Revised, at the ages of 6 and 7 years. Children born up to 6 years after their mothers' exposure were as affected as children born within a year or two after exposure when examined at 6 and 7 years of age. Older siblings resembled the control children. CONCLUSION--Children prenatally exposed to heat-degraded PCBs had poorer cognitive development than their matched controls. The effect persisted in the children up to the age of 7 years, and children born long after the exposure were still affected.     

Head injuries to children riding bicycles

This article examines the characteristics of 150 children who were admitted to Brisbane hospitals with head injuries that were caused by bicycle riding. These children constitute over 20% of all the children with head injuries who were admitted to hospital; accidents involving bicycle riding are the second largest single cause of head injury in these children, after falls. There was one death in the group, and three children were moderately disabled nine months after the injury. These four children all had Glasgow coma scores of less than 9 on their admission to hospital. Only two of the 150 children wore helmets. A survey of a random subgroup of children who had sustained head injuries confirmed our opinion that many children owned helmets but did not wear them for fear of peer derision. It is suggested that this refusal can be overcome by a combination of legislative enforcement, helmet design and promotion.     

Follow up study of children born elsewhere but attending schools in Seascale, West Cumbria (schools cohort)

Records on 1546 children who were identified as having attended schools in Seascale up to November 1984 and were born since 1950 but not in the civil parish were studied. These children lived in or near Seascale for a period of time while they were attending one or more of three local schools and are an additional group to the 1068 children who were identified as born to mothers resident in Seascale in an accompanying study. Even though some of the schoolchildren apparently remained in the village for a short period only all but 7% were followed up through the National Health Service Central Register. Mortality among these children to 30 June 1986 is comparable to that expected at national rates. From all causes there were 10 observed deaths compared with 12.69 expected--a ratio of 0.79 (95% confidence interval 0.38 to 1.45)--and from cancer one observed death compared with 2.04 expected--a ratio of 0.49 (95% CI 0.01 to 2.73). No deaths from leukaemia or lymphoma were reported, but only 0.83 was expected. Since 1971 (the year when cases of cancer were first notified to the NHS Central Register) three non-fatal cases of cancer were reported, including two lymphomas, compared with 2.04 expected and two cases of carcinoma in situ of the cervix compared with 1.79 expected. In addition, there was a case of leukaemia among the schoolchildren which was known previously and had been diagnosed in 1968. There is an interesting difference between the results of this study and the results of the study of children born to mothers who were resident in Seascale. In the latter study there was an excess of leukaemia and of other cancers, but a similar finding is not apparent among children who spent some time at schools in Seascale but were born elsewhere. This raises the question of whether one or more aetiological factors in childhood cancer were acting on a locality specific basis before birth or early in life. This cannot be answered from these cohort studies, but it is hoped that the case-control study that is under way in West Cumbria will provide relevant information.     

Are all born equal? Incidence of febrile convulsions by seasons of birth

To test whether the seasons of birth had an effect on subsequent experience of illness, details were obtained of all Sheffield children born between 1973 and 1977 who were admitted to hospital before their second birthday with a first febrile convulsion. Analysis by date of birth in consecutive 28-day cohorts showed that the incidence of febrile convulsions ranged from 2.5 per thousand live births to 30.2 per thousand in different "month" cohorts. Statistically significant variations were noted in the incidence rates in relation to season and year of birth. The implication is that even large scale epidemiological studies which have been confined to children born in a particular week or month may not be representative of the whole child population.     

Malformations in infants of very low birth weight

The incidence of lethal, serious and trivial malformations was determined in a consecutive series of 675 infants who weightd 1500 g or less (very low birth weight infants). All patients were born in one maternity hospital. Most of the 169 survivors were followed until at least five years of age. Of these children, 15 had a trivial malformation that either disappeared spontaneously or was corrected surgically. A further five children have a significant or serious residual defect. There were 506 perinatal and infant deaths and 51 of the babies who died had malformations confirmed at necropsy. In 30 of these, death was inevitable. There were 10 seriously malformed infants for whom survival was possible, but in six cases, the diagnosis was obvious at birth. The patients were treated between 1966 and 1970 when techniques of intensive care were evolving. Consequently, some of the infants who died would have survived with the treatment now available. Amongst the perinatal deaths in this report, only 2.0% had a serious malformation compatible with survival.     

Children born after elective induction of labour

A comparison was made between the performance at eight years of age of 63 children who were born after elective induction of labour, and 29 spontaneously born children. The children had been tested previously at five years of age. Tests of reading and arithmetic achievement, visuomotor coordination, aural-visual coordination, auditory discrimination, and behavioural rating scales were administered. On two of the 12 measures (reading comprehension and euroticism) the induced children performed better than the spontaneously born. On all other measures there were no significant differences. There appeared to be no evidence of specific learning disabilities or behavioural problems among the induced children.     

Extremely-low-birthweight infants: neurological, psychological, growth and health status beyond five years of age

Of 60 consecutive survivors of birth weight 500-999 g, who were born in one tertiary perinatal centre from 1977 to 1980, 59 infants were assessed by a multidisciplinary team at two years of age (corrected for prematurity) and 58 children were evaluated when aged at least five years. At the latter examination, 9% of the 58 children who were assessed were severely disabled; 17% had a mild or moderate disability; and 74% had no important disability. For the 53 children who were tested, the means for the three scales of the Wechsler Preschool and Primary Scales of Intelligence were just above the test mean. The psychologist noted behavioural problems during her assessment in 50% of children, and 29% of mothers reported behavioural problems which could interfere with schooling. At the age of five years and over, five (9%) children had cerebral palsy and one child was deaf. Twenty-two (38%) children had a visual impairment, although only one child was blind; the detection of retinopathy of prematurity in the nursery was an important risk factor. Health problems with readmissions to hospital and suboptimal growth were present in many children at two years of age and frequently these problems persisted to five years of age. Although only four (7%) children were too disabled to attend a normal school, apprehension exists that many of the other children may later encounter educational difficulties. At the two-years' assessment, ascertainment of cerebral palsy had not been complete or entirely accurate and the Mental Developmental Index of the Bayley Scale tended to underestimate the later psychological performance.     

Why are patients with acute stroke admitted to hospital?

Data on 515 consecutive patients registered with the Oxfordshire Community Stroke Project were used to compare the characteristics of those patients who were admitted to hospital within one month after their first stroke with those who remained in the community during that time. Twenty eight patients had their stroke while in hospital for other conditions, and of the remaining 487, 266 were admitted. Though patients with a severe neurological deficit were significantly more likely to be admitted, 47 out of 202 such patients were managed in the community. In a substudy of 162 consecutive patients the general practitioners' reasons for either arranging admission to hospital or continuing with community care in the first week after the stroke were ascertained. Sixty patients were admitted. The only reason for admission was diagnostic uncertainty in five cases (though this was a contributing factor in 25) and to provide nursing or general, non-medical care in 25. Patients who lived alone were more likely to be admitted. All 12 patients who presented directly to the casualty department were admitted, though only five had had a severe stroke. A stroke service that provides a facility for rapid outpatient and domiciliary diagnosis as well as a rapidly acting domiciliary nursing team might reduce the number of patients with stroke admitted to hospital without adversely affecting the quality of patient care: this should be properly evaluated.     

Legionnaires' disease caused by Legionella dumoffii in distilled water.

Five cases of Legionnaires' disease caused by Legionella dumoffii were identified within an 11-month period in a hospital in the Quebec City area. In four cases bacterial isolates were obtained from clinical specimens, and in one case seroconversion was demonstrated. All the patients had been admitted to hospital within 10 days before diagnosis. Two of the patients were immunosuppressed children. Only 1 of the 40 hot water samples from the hospital yielded L. dumoffii; however, 6 of 11 distilled water samples contained the bacterium. All the patients had been exposed to distilled water, four through respiratory therapy equipment and one through a room humidifier. Following the use of sterile distilled water in the apparatus, no further cases were identified. This is the first reported outbreak of Legionnaires' disease caused by L. dumoffii, and it is the first time that nosocomial legionellosis has been linked to contaminated distilled water in Canada.     

33例单胎试管婴儿1岁内生长发育情况分析

目的　对比分析单胎试管婴儿与自然受孕儿 1岁内生长发育情况。方法　对 2001年 1月~2004年 7月在北京大学深圳医院儿童保健门诊随访的单胎试管婴儿 (33例) 和自然受孕儿 (33例) 进行常规检查, 采用WHO推荐的 0~6岁儿童身高、体重参考值及评价标准进行评价。试管婴儿和自然受孕儿均进行了新生儿甲状腺功能低下、苯丙酮尿症筛查, 第 6、12个月查血常规, 分析二者生长发育的差异。结果　单胎试管婴儿与自然受孕儿的新生儿甲状腺功能低下、苯丙酮尿症筛查结果均正常; 1岁内体格发育情况间差别无显著性意义 (P>0 .05 ); 丹佛发育筛查法(DDST) 筛查的结果间差别无显著性意义 (P>0. 05 ); 两者在 6、12月龄时血红蛋白评价结果间差别无显著性意义(P>0. 05)。结论　单胎试管婴儿在 1岁内的生长发育与自然受孕儿比较无显著性差异。     

Hepatitis B vaccination in Indian children: Seroprotection and age-related change in antibody titres

BackgroundChildhood immunization against hepatitis B is one of the most effective strategies for reducing the global burden of chronic hepatitis B infection and its sequelae. There are limited data from India on both the anti-Hep B antibody titres in children after vaccination and the age-related decline in the titres. This study was planned to estimate the proportion of children in the age group of 1–10 years who develop protective levels of anti-hepatitis B antibodies after childhood vaccination and to examine the change in antibody titres with age in these children.MethodsA hospital-based cross-sectional study was carried out in children admitted to the hospital for various ailments. Basic demographic data, vaccination history and HBsAg status of the mother were recorded. All the enrolled children were evaluated for HBsAg and anti hepatitis B surface antibody (anti-HBS) titres. Institutional ethical clearance was obtained, and informed consent from the parents of the children was taken before drawing samples.ResultsWe found that 68.86% Confidence Interval ((CI): 59.8–76.8%) of the children showed protective antibody titres after vaccination, while 31.14% (CI: 23.1–40.2%) of the children had titres less than 10 IU/L. Although 100% of children in the age group from birth to three years had titres more than 10 IU/L, this percentage showed a decline across the age groups, and 60% of children aged 9–10 years had titres less than 10 IU/L.ConclusionChildhood vaccination against hepatitis B is effective in 68% children, and the antibody levels showed a steady decline with increasing age.     

Prognosis of children who are carriers of hepatitis B

Fifteen children who had become positive for hepatitis B surface antigen (HBsAg) by perinatal transmission were traced and re-examined after a mean of 8.1 years; all had been born in England to mothers from ethnic minorities who were carriers of HBsAg. Fourteen of the children remained carriers of HBsAg; of these, more girls than boys developed antibody to hepatitis B e antigen (anti-HBe). Those children whose transaminase activities had been above normal within the first three years of life were more likely to have developed anti-HBe. The earlier production of anti-HBe suggests that girls have a more effective immune response. Increased transaminase activity early in the course of asymptomatic carriage of HBsAg may be a favourable prognostic sign.     

Salmonellose chez les enfants: Etude de 95 cas à l''Hôpital Ste-Justine, Montréal, en 1963-1964

A retrospective study was done in children in whom salmonellosis was confirmed by laboratory findings with the aim of reviewing etiology, epidemiology, clinical manifestations and therapy. The 15 serotypes most frequently isolated from stool, and in exceptional cases from urine, are discussed. If patients with typhoid fever are excluded, only one patient (who subsequently died) had a blood culture positive for Salmonella, specifically S. enteritidis.

No seasonal or other peaks of incidence were noted. Age appeared to be important; of 81 patients with gastroenteritis, 30 were less than 6 months old.

Two children in the older age group developed complications; one with appendicitis required surgery.

Ten strains of Salmonella out of 23 tested by the disc method showed in vitro resistance to ampicillin on primary isolation.

Acquired in vitro resistance to one or more antibiotics appeared to develop with six Salmonella strains reisolated from patients after or during antibiotic treatment.

In several children the stool cultures remained positive after clinical signs had disappeared. These findings strongly suggest that, even though antibiotic therapy may improve the symptoms of Salmonella infection, it does not decrease the number of carriers during the convalescent period.

     

Limits of confidentiality.

A patient is described, a woman of 60, who at that age was diagnosed as having Huntington's chorea, a genetic disease which is transmitted as an autosomal dominant. She had one daughter, who was married and lived abroad. The patient's mother and maternal aunt, it emerged during the consultations, had had the disease, and shortly after the diagnosis had been made the patient's sister was admitted to hospital with a repiratory condition, and in the course of treatment Huntington's chorea was diagnosed in her also. She had one daughter. Throughout, the problem was not only that of treating the patient herself, but the situation of all those of the family affected in any way. The patient's husband had not known of her medical history at the time of the marriage. At no time had the daughter been told. The discussion, in which seven doctors, a moral philosopher, and a social worker took part, centered on the basic issue of confidentiality in the case of a disease, from which would stem problems for the affected family through several generations. All were agreed that in these circumstances those affected must be told, but differed as to who should make some of the subsequent decisions. Some thought that having told of the diagnosis and its implications, the doctor should leave further decisions to those concerned; others that in a disease such as Huntington's chorea one is looking at part of a whole chain of genetic material, and, although the individual patient or potential patient is of course important, he or she must be seen in the genetic setting of the community. The participants also considered the psychological.     

Measles in a two-months age baby and his mother in Juba, Sudan

Measles in children 2-5 months of age is a new phenomena not only in Equatorial Juba but in the Sudan at large. It is the first time a baby of two months of age and his mother who had never contracted measles till the age of 17 years have been admitted to Sabah Children's Hospital in Juba. This occurrence may indicate as in other areas where immunisation programmes have been carried out increasing numbers of people will not encounter measles till they reach adolescence or adult life. This could lead to infants being born without usually transmitted antibodies, hence develop infection with measles at an earlier age than has been usual in the past.     

Risk factors for persistent middle-ear effusions. Otitis media, catarrh, cigarette smoke exposure, and atopy

To ascertain risk factors for persistent middle-ear effusions (PMEE), we interviewed the parents of two groups of children. The first consisted of 76 children with PMEE who were admitted to the hospital for tympanostomy-tube insertion. The second, a control group, consisted of 76 children admitted for other types of surgery, who were matched for age, sex, season, and surgical ward. Nearly all (97%) of the children admitted for insertion of tympanostomy tubes had one or more episodes of suppurative otitis media. Only 59% of the control children had previous ear infections. Frequent ear infections sharply increased the risk for persistent effusions. Catarrh, household cigarette smoke exposure, and atopy also occurred more frequently in children with PMEE. The risk for middle-ear effusions was greatest when these three factors were all present. The avoidance of daily exposure to domestic tobacco smoke and, if atopic, of specific allergens should be included in the medical treatment of children with PMEE.     

Hospitalisation patterns in children from 10 aboriginal communities in the Northern Territory.

OBJECTIVE: To describe childhood hospitalisation patterns in rural Aboriginal communities in the Northern Territory. DESIGN: Longitudinal data for 1976-1985 were collected retrospectively between March 1986 and December 1987. COHORT: All children born between 1 January 1976 and 31 December 1985 and identified in records at 10 community health centres were included in the study, except for 30 children who were residents of more than one community. Records of hospital admissions were ascertained for the remaining 2254 children until five years of age or 31 December 1985, whichever was earlier. RESULTS: Mean admission rates for Aboriginal children aged 0-1 year were two to three times the national average, and there was a fourfold excess in the number of days in hospital and the number of deaths before the age of five years for Aboriginal infants. Admission rates varied by community, age, year and sex: at 0-6 months of age, rates for communities ranged from a minimum of 0.21 to a maximum of 1.46 admissions per child-year at risk and mean rates increased from 0.50 per year in 1976 to 1.05 in 1985; mean admission rates declined from 0.84 per child-year at age 0-6 months to 0.12 at age 37-60 months. Boys were admitted to hospital more frequently than girls. For communities, hospital stay ranged from a minimum of 2.7 to a maximum of 15.3 hospital days per child-year at risk. CONCLUSIONS: Differences of up to sevenfold in hospitalisation rates between communities highlight the potential for primary and secondary prevention of childhood disease in Aboriginal communities.     

Cancer risk among children of atomic bomb survivors. A review of RERF epidemiologic studies. Radiation Effects Research Foundation

This article summarizes recent epidemiologic studies of cancer risk among the children of atomic bomb survivors conducted at the Radiation Effects Research Foundation. These children include two groups: (1) the in utero-exposed children (ie, those born to mothers who had been pregnant at the time of the bombings of Hiroshima and Nagasaki) and (2) the F1 population, which was conceived after the atomic-bombings and born to parents of whom one or both were atomic bomb survivors. Although from 1950 to 1984 only 18 cancer cases were identified among the in utero sample, cancer risk did appear to significantly increase as maternal uterine dose increased. However, since the observed cases are too few in number to allow a site-specific review, the increased cancer risk cannot be definitively attributed to atomic bomb radiation, as yet. For those members of the F1 population who were less than 20 years old between 1946 and 1982, cancer risk did not appear to increase significantly as parental gonadal dose increased. Follow-up of this population will continue to determine if the patterns of adult-onset cancer are altered.     

Ecchymoses: an unusual manifestation of toxocariasis in children

OBJECTIVE: To investigate the aetiology of multiple ecchymoses associated with eosinophilia. SETTING: Professorial Paediatric Unit (PPU) at Lady Ridgeway Hospital (LRH) for children, Colombo. DESIGN: Prospective study from July 1998 to April 2000. MATERIALS AND METHODS: Patients admitted to the PPU with multiple ecchymoses associated with an absolute eosinophilia (> 1 x 10(9)/1) were investigated further to determine the possible aetiology; bleeding time, clotting time, platelet count, platelet function tests, stools examination and toxocara antibody tests were performed. Patients who were positive for toxocara were treated either with thiabendazole or albendazole. Patients who had geohelminth infestations were treated with mebendazole. RESULTS: 14 children (11 boys) were studied. 7 were between 1 and 5 years, and 7 were above 5 years of age. The mean eosinophil count was 4.3 x 10(9)/1. All patients had a prolonged bleeding time, but clotting time and platelet counts (mean value 220 x 10(9)/1) were normal. Platelet function tests were done in 5 patients. Twelve patients were positive for toxocariasis and the other 2 were positive for ascariasis. All children who were positive for toxocariasis had contact with pet cats or dogs. Six patients were treated with thiabendazole and 6 with albendazole. The two patients who had ascariasis were treated with mebendazole. Post-treatment mean eosinophil count was 0.63 x 10(9)/1, and the bleeding time was 4 minutes. CONCLUSION: Toxocariasis could present with ecchymoses due to an acquired bleeding disorder. Awareness of this would help to investigate, diagnose and treat early, and lessen parental anxiety.