'Juvenile' myasthenia gravis in early infancy

A previously well infant developed severe muscle weakness and hypotonia at 6 months of age. This was reversed by anticholinesterase medication. However, she had subsequent further weakness and died at 10 months after an acute respiratory arrest. The clinical pattern was that of the 'juvenile' form of myasthenia gravis rather than the 'congenital' forms which have previously been described in early infancy.     

Cows' milk protein-sensitive enteropathy. An important factor in prolonging diarrhoea of acute infective enteritis in early infancy.

The possible role of cows'' milk protein in prolonging diarrhoea in very young infants with acute infective enteritis was studied in 14 infants, 9 under the age of 2 months and 5 older than 6 months. Bacterial pathogens were isolated from the stools of 4 infants from the younger age group. After appropriate initial treatment the infants were maintained on a cows'' milk protein-free formula. 6 weeks later jejunal biopsies were performed before and 24 hours after challenge with a low lactose cows'' milk protein formula. The immunoglobulin and complement levels in the serum and duodenal juice were also estimated at these times. Attempts to isolate bacterial and viral pathogens in stools were again made in all patients. The 5 older infants clinically tolerated cows'' milk protein and their pre- and postchallenge jejunal biopsies were within normal limits. However, significant histological changes were observed in the postchallenge jejunal biopsies of all 9 infants under 2 months of age. In addition, 5 of these infants developed diarrhoea. This suggests that the jejunal mucosa of very young infants previously fed a cows'' milk protein-based formula and who contract infective enteritis suffers damage when rechallenged with cows'' milk protein.     

Early identification of hearing loss: screening and surveillance methods.

Service monitoring data on the outcomes of health visitors'' screening for hearing loss at 8 months in West Berkshire indicate low sensitivity and low positive predictive value, despite efforts to improve the conduct of the screen. Nevertheless, data on a recent series of severely hearing impaired children indicate significantly earlier diagnosis than previously, due in part in the introduction of other service changes including neonatal ''at risk'' screening and surveillance using parental observation. For a trial period the traditional screening method for the detection of hearing loss in babies will be discontinued and effort concentrated on these alternative procedures.     

Chemotherapy of advanced neuroblastoma: does adriamycin contribute?

Between 1970 and 1977, 69 children with newly diagnosed stage III or IV neuroblastoma were treated with pulses of either cyclophosphamide and vincristine (CV) (n = 23), or cyclophosphamide, vincristine, and adriamycin (CVA) (n = 46). The ''complete'' and partial response rates were 35 and 22% to CV, and 43 and 26% to CVA. For ''complete'' responders the median time to relapse was 18 months for those treated with CV, and 17 months for those treated CVA; for partial responders the times were 5 and 7 months respectively. At 2 1/2 years only 17% of the CV patients and only 13% of the CVA patients were alive and free of disease, giving a 15% overall survival rate. The addition of adriamycin to cyclophosphamide and vincristine did not significantly improve the response rate, duration of response, or survival in these children with advanced neuroblastoma. The previously noted favourable effects of age less than 1 year at diagnosis and of female sex were confirmed. The equally poor survival for stage III and stage IV patients justifies the inclusion of stage III patients in a bad prognosis group.     

Prevention of anaemia in inner city toddlers by an iron supplemented cows' milk formula.

There are few data to support the use of follow-on formulas in infants from the age of 6 months. In a prospective trial in a deprived inner city area of Birmingham 100 infants who were already receiving pasteurised cows'' milk by 6 months of age were enrolled and randomised either to receive a follow-on formula or to continue on cows'' milk from 6 months until 18 months. At 18 months of age the follow-on formula group returned to cows'' milk and both groups were followed up until 24 months. Iron status, growth, and nutritional status were analysed at intervals of six months. At enrollment, no differences in haematological status were evident. However, by 12 months of age, 31% of the cows'' milk group were anaemic (haemoglobin concentration < 110 g/l) compared with only 3% of those receiving follow-on formulas. At 18 months, 33% of the cows'' milk group were anaemic compared with only 2% of the follow-on formula group and by 24 months of age none of the follow-on formula group was anaemic, whereas 26% in the cows'' milk group still had a haemoglobin of < 110 g/l. Mean corpuscular volume was significantly smaller and ferritin significantly lower in the cows'' milk group at 12, 18, and 24 months. Dietary iron intake was higher in the follow-on formula group at 12 and 18 months but not at 24 months, when both groups were back on cows'' milk. Infants and toddlers at high risk of iron deficiency are therefore unlikely to become anaemic if receiving a follow-on formula, although the relative merits of follow-on formula compared with an ordinary infant formula remain uncertain.     

Phase II trial of ifosfamide/mesna and mitoxantrone in previously treated patients with non-Hodgkin's lymphoma: Cancer and leukemia group B study 8753

Ifosfamide (2.0 g/m² intravenously/day × 3) with mesna (400 mg/m² intravenously q 4 h daily × 3) was combined with mitoxantrone (14 mg/m² intravenously × 1) and given on a 3 week schedule to patients with previously treated non-Hodkgin's lymphoma. In 45 eligible/evaluable patients, a 47% response rate (95% confidence interval: 32%, 62%) was achieved of which 25% were complete responses and 22% were partial responses. Median duration of remission was 10 months with 42% of patients in remission at 18 months. Hematologic toxicity (granulocytopenia) was the dose-limiting toxicity with severe or life-threatening granulocytopenia in 98% of patients at full protocol doses. Nausea/vomiting was seen in 73% of patients but was usually mild/moderate. Three patients had significant hematuria secondary to ifosfamide. Two patients had severe changes in the left ventricular ejection fraction (LVEF) secondary to mitoxantrone; and one patient had acute neurologic dysfunction secondary to ifosfamide. Ifosfamide/mesna can be safely combined with mitoxantrone at full doses in previously treated patients with non-Hodkgin's lymphoma. This combination should be considered for other trials in lymphoma. © 1992 Wiley-Liss, Inc.     

Breathing,sleep state,and rectal temperature oscillations.

Overheating may cause terminal apnoea and cot death. Rectal temperature and breathing patterns were examined in normal infants at home during the first 6 months of life. Twenty one infants had continuous overnight rectal temperature and breathing recordings for 429 nights (mean 20.4 nights, range 7-30) spaced over the first six months of life. Periods when breathing was ''regular'' were directly marked on single night records. Sleep state was determined from respiratory variables. ''Regular'' breathing was a reliable marker of ''quiet'' sleep (specificity 93%). The duration of ''quiet'' sleep increased from 6 to 22 minutes from two weeks to three months of age and then remained static, as did the proportion of sleep spent in the quiet phase (9% to 34%). Rectal temperature fell during 66% of quiet sleep and usually rose during rapid eye movement (REM) sleep. The drop in rectal temperature was maximal at the start of quiet sleep, whereas the maximum rise during REM sleep was reached after 10 to 15 minutes. Oscillations in rectal temperature are associated with changes in sleep and breathing state. The maturation of rectal temperature patterns during the first six months of life are closely related to a maturation of sleep state and breathing patterns.     

Prognostic value of immunological data,in vitro antibody production,and virus culture in vertical infection with HIV-1.

The prognostic value of immunological indices, in vitro antibody production, and virus culture pattern at 3 months of age was estimated in 35 infants infected by HIV-1 from a cohort of 298 babies born to HIV-1 seropositive mothers and followed up from birth. At 1 year old, 15 of these infants were classified as stage P-1 (according to the Centers for Disease Control classification) seven were P-2A, and seven had AIDS. Significantly higher CD8 percentages, lower percentages and absolute value of CD4, and lower CD4/CD8 ratios at 3 months were observed in infants with severe symptoms at 1 year of age when compared with those who were asymptomatic at this age. Seventy seven per cent of infants with a ''rapid'' virus culture when 3 months old had developed AIDS or had died by 1 year of age and only 8% of those with ''slow'' virus culture had AIDS when 1 year old. Moreover, 100% of those who were asymptomatic at 1 year had a slow virus culture at 3 months. Significant statistical association was found between the virus replication pattern at 3 months and the clinical stage at 1 year of age.     

A review of 404 'late walkers'.

A survey of all known 18 month old Oxfordshire children who had not yet walked unassisted and who were born in the four year period between July 1, 1976 and June 30, 1980 was carried out. A total of 275 children aged 18 months with no previously suspected cause for late walking were referred by health visitors; 257 of these children were assessed neurologically and developmentally by a paediatrician at home. Nine cases of cerebral palsy (3 X 5%) and 6 cases of minor neurological abnormality (2 X 3%) were newly diagnosed. A register of all other 18 month old ''late walkers'' (129) who were already known to paediatricians and were either normal (17) or had known causes for late walking (112) was compiled for the same four year period. The total incidence of pathology among all late walkers (404) in these two groups was 32%.     

Infant feeding in the second 6 months of life related to iron status: an observational study

Objective

To investigate the relationship between iron status in infancy and type of milk and weaning solids consumed.

Design

An observational cohort study.

Setting

928 term infants from the Avon Longitudinal Study of Parents and Children in 1993–94.

Methods

Haemoglobin and ferritin concentrations at 8 and 12 months were assessed in relation to type and quantity of milk intake at 8 months.

Results

By WHO criteria, 22.7% of the infants were anaemic at 8 months and 18.1% at 12 months. More breast‐ than formula‐fed infants were anaemic at 8 and 12 months. Cows'' milk as the main drink was associated with increased anaemia at 12 months and low ferritin at 8 and 12 months. No association was found between any nutrients and haemoglobin concentrations. Protein and non‐haem iron intakes were positively associated with ferritin concentrations and calcium intake negatively. This effect was more marked in infants being fed cows'' milk. More than 25% of infants in the breast milk and cows'' milk groups and 41% of infants having >6 breast feeds per day had iron intakes below the lower reference nutrient intake. Feeding cows'' milk or formula above 600 ml or >6 breast feeds per day was associated with lower intakes of solids.

Conclusions

Both breast and cows'' milk feeding were associated with higher levels of anaemia. Satisfactory iron intake from solids in later infancy is more likely if formula intake is <600 ml per day and breast feeds are limited to <6 feeds per day. Cows'' milk should be strongly discouraged as a main drink before 12 months.     

Behaviour in first year after drug dependent pregnancy.

Neurobehavioural development of a group of 35 infants of drug dependent mothers and of a reference group of 37 infants was compared. Two tailed testing showed that at the age of 3 months the infants of drug dependent mothers seemed to be more active than the reference group; at 6 months, however, no difference was found. On the infant behaviour temperament questionnaire, infants of drug dependent mothers tended to have slightly better scores for ''duration of orienting'' at the age of 9 months; five other dimensions of behaviour did not differ between the groups. The Bayley scales of infant development and neurological examination according to the method of Touwen did not show any significant differences among the groups at the ages of 6 or 12 months. At 12 months infants of drug dependent mothers had slightly but not significantly worse results on electroencephalography. Studied with multidisciplinary measurements, the development of infants of drug dependent mothers does not seem to diverge particularly from the development of the reference group.     

'Near miss' sudden infant death and obstructive apnoea.

A 4 month old girl presented with a ''near miss'' sudden infant death episode for which no cause was found. Obstructive sleep apnoea syndrome subsequently developed. All symptoms ceased after adenoidectomy at age 9 months.     

Demographic,socioeconomic, and sociocultural factors associated with any breastfeeding in homeless mothers

In high‐income countries, breastfeeding has been shown to be positively associated with socioeconomic position. However, less is known about breastfeeding practices and their associated factors among extremely disadvantaged populations. We aimed to assess the associations of cultural origins and socioeconomic factors with any breastfeeding initiation and duration in homeless families. We analyzed data from 456 children aged 6 months to 5 years from the cross‐sectional ENFAMS survey, conducted in 2013 among a random sample of homeless families in shelters in the Greater Paris area. Data were collected by bilingual interviewers in 17 languages. Four nested multivariable robust Poisson regression models were run in a hierarchical framework to determine the factors associated with breastfeeding initiation and with any breastfeeding for 6 months or more. Most of the children (86.0%) had previously been or were currently being breastfed at the time of the survey; 58.9% were fed with breast milk ≥6 months. A higher maternal age and African origin were positively associated with breastfeeding ≥6 months, although the relation to the region of origin was moderated by education level. Migration to escape war, unrest or other violence and the child''s birth in France were inversely associated with breastfeeding ≥6 months. Any breastfeeding by these homeless mothers seems influenced predominantly by their cultural origin and complicated by a difficult migration trajectory. The possible influence of poor material circumstances and cumulative hardship should encourage interventions targeted at homeless mothers that emphasize social/family support with a commitment to improving the family''s living conditions.     

Primary central nervous system Burkitt's lymphoma presenting as Guillain-Barré syndrome

A rare case of CNS Burkitt's lymphoma presenting as acute Guillain-Barré syndrome is presented. A 6-year-old previously healthy female presented with acute onset of limb and truncal weakness, involvement of ocular and bulbar nerves, and areflexia. The clinical diagnosis of Guillain-Barré syndrome prompted treatment with intravenous gammaglobulin with no response. A lumbar puncture following revealed marked pleocytosis, elevated protein, and decreased glucose. Immunological, cytological, and molecular studies of these cells confirmed the diagnosis of Burkitt's lymphoma IgM, kappa with t(8;14) and rearrangement of the J and kappa immunoglobulin chains. Aggressive systemic and intrathecal chemotherapy were started and within 5 days remission was achieved. The child is in complete remission 2 years from diagnosis. Although very rare, CNS lymphoma should be taken into account in every patient presenting with the clinical features of acute polyneuropathy. © 1994 Wiley-Liss, Inc.     

HIV with Juvenile Dermatomyositis

Dermatomyositis with HIV infection has been very rarely reported. The authors report an 8-y-old boy who presented with skin rashes and edema, muscle weakness and polymicrobial infection along with mild immunosupression. Diagnosis of dermatomyositis was established by raised enzymes, suggestive MRI and muscle biopsy findings. Child responded to systemic steroids and low dose weekly methotrexate.     

Adrenocortical Atrophy and Diffuse Cerebral Sclerosis

A boy, diagnosed as having Addison''s disease due to idiopathic atrophy of the adrenal glands at the age of 7 years, developed the first evidence of what was originally thought to be `Schilder''s disease'' at 8 years and 10 months. He died at 9 years and 11 months. There was a very striking family history of autoimmune disorders on the mother''s side. The clinical and pathological aspects of his case are outlined. Detailed studies of adrenal function during life showed a diminution in excretion of adrenal androgens and corticosteroids before therapy which was not evident from the 17-oxosteroid and 17-hydroxycorticosteroid assays in the resting state, though the initial diagnosis was based on the failure of ACTH to produce a rise in the 17-hydroxycorticosteroid excretion. No unusual or abnormal steroids were detected nor was there any disproportion between the androgen and corticosteroid excretion to suggest an adrenal enzyme deficiency. The biochemistry of the brain at necropsy revealed the changes expected in a demyelinating disorder, but the detection of abnormalities in the grey matter distinguished the condition from `Schilder''s disease'' in which the biochemistry of the grey matter is normal. There are 12 fully documented reports of boys with adrenocortical atrophy and diffuse cerebral sclerosis, and it is thought that this rare entity is inherited as an X-linked recessive characteristic. The two likely theories of causation involve either an error of metabolism common to the adrenal cortex and the brain, or the possibility that both the adrenal and brain pathology are due to an autoimmune disorder.     

孤独症谱系障碍儿童的诊断年龄及其影响因素

目的 研究孤独症谱系障碍（ASD）儿童的诊断年龄,探讨ASD诊断年龄的影响因素。方法 收集2011年2月至2017年7月于重庆医科大学附属儿童医院首次就诊后确诊为ASD的1691例儿童的临床资料。应用多元线性回归模型分析影响ASD诊断年龄的相关因素。结果 ASD诊断年龄平均为35±17个月（范围9~175个月）,其中24~35个月获得确诊者占比最高（46.13%,780/1 691）,其次为≥ 36个月（33.41%,565/1 691）。多元线性回归分析显示,有语言障碍、居住在主城区、父母学历高的ASD儿童诊断年龄较早（P < 0.05）。结论 儿童ASD的诊断年龄多在24~35个月。ASD诊断年龄与个体主要症状、居住地区和父母文化程度有关。     

Home monitoring for central apnoea.

Between July 1978 and December 1981, 64 infants thought to be at increased risk from sudden infant death syndrome (SIDS) were monitored at home for central apnoea. Twenty four of the infants had had a ''near miss'' episode at age, median (range), 6 (1 to 33) weeks, and of these infants 22 had had 335 alarms for apnoea by age 6 months. Stimulation by shaking was carried out on 38 occasions and bag and mask resuscitation on one. The remaining 40 infants were siblings of SIDS victims and of these, 35 were monitored from age 1 week (usually after discharge home). Thirty four of the SIDS siblings had had 573 alarms for apnoea by age 6 months: stimulation by shaking was carried out on 32 occasions and bag and mask resuscitation on one. The duration of home monitoring was, median (range), 34 (8 to 87) weeks for ''near miss'' infants and 45 (12 to 70) weeks for SIDS siblings. All infants survived. As part of an over all support system monitors were accepted and greatly appreciated by most parents, especially those with previous experience of SIDS. Home monitoring was practicable but the commitment in time and expertise was great and objective benefits to the infant remain unproved.     

Effects of elevated blood lead levels in preschool children in urban Vellore

Objectives

To study the burden and associated risk factors for elevated blood lead levels among pre-school children (15–24 months) in urban Vellore, and to study its effects on child cognition and anemia.

Design

An investigative study through Mal-ED cohort.

Setting

Eight adjacent urban slums in Vellore, Tamil Nadu.

Participants

251 babies recruited through Mal-ED Network.

Outcome measures

Blood lead levels using Graphite Furnace Atomic Absorption Spectrophotometry method at 15 and 24 mo; hemoglobin estimation by azidemethemoglobin method; cognitive levels using Bayley Scales of Infant Development III.

Results

Around 45% of children at 15 months and 46.4% at 24 months had elevated blood lead levels (>10 μg/dL). Among children who had elevated blood lead levels at 15 months, 69.2% (45/65) continued to have elevated levels at 24 months. After adjusting for potential confounders, children from houses having a piped drinking water supply and houses with mud or clay floors were at significantly higher risk of having elevated blood lead levels at 15 months. Thirty one percent (21/67) of the children with elevated blood lead levels had poor cognitive scores. Children with elevated blood lead levels at 15 months had higher risk (Adjusted OR 1.80; 95% CI 0.80–3.99) of having poorer cognitive scores at 24 months. More than half of the children (57%) were anemic at 15 months of age, and elevated blood lead levels were not significantly associated with anemia.

Conclusions

Elevated blood lead levels are common among preschool children living in urban slums of Vellore. Poorer conditions of the living environment are associated with elevated lead levels.