Evaluation of diagnostic tests for HIV infection in infants born to HIV-infected mothers in Switzerland.

Children born to HIV-infected women in Switzerland were tested every 3 months for HIV-reactive serum immunoglobulin (Ig) G, IgM and IgA antibodies by Western blot, viral antigen, virus replicating in T-lymphocyte cultures, and immunologic and clinical parameters. At birth, 27% were isolation-positive, 68% had IgM, 48% IgA and 10% circulating antigen. The proportion of IgM and IgA declined to about 18 and 27%, respectively, during the first 2 years. Detection of circulating antigen was less frequently positive than virus isolation in all age and disease groups. Clinical symptoms were only seen in infants or children who were or had been positive for IgM and/or IgA, but only 39% of children positive for these markers have developed disease so far. Clinical symptoms combined with signs of immunodeficiency were seen only in children who were isolation-positive or had evidence of HIV-reactive IgA or child-produced IgG. Absorption studies showed that Western blot-detected IgM and IgA antibodies were of two types: 42% were directed against various HIV proteins, while the rest represented rheumatoid-factor-like IgM or IgA binding to HIV-specific IgG. HIV-specific IgG antibodies were detected in all samples up to the age of 12 months and were still found in 83% of infants 13-18 months old. We observed weak HIV-specific IgG above the age of 15 months with no other signs of HIV infection, suggesting that the demonstration of antibodies in children beyond this age does not necessarily indicate HIV infection.

Increased carrying reduces infant crying: a randomized controlled trial.

The crying pattern of normal infants in industrialized societies is characterized by an overall increase until 6 weeks of age followed by a decline until 4 months of age with a preponderance of evening crying. We hypothesized that this "normal" crying could be reduced by supplemental carrying, that is, increased carrying throughout the day in addition to that which occurs during feeding and in response to crying. In a randomized controlled trial, 99 mother-infant pairs were assigned to an increased carrying or control group. At the time of peak crying (6 weeks of age), infants who received supplemental carrying cried and fussed 43% less (1.23 v 2.16 h/d) overall, and 51% less (0.63 v 1.28 hours) during the evening hours (4 PM to midnight). Similar but smaller decreases occurred at 4, 8, and 12 weeks of age. Decreased crying and fussing were associated with increased contentment and feeding frequency but no change in feeding duration or sleep. We conclude that supplemental carrying modifies "normal" crying by reducing the duration and altering the typical pattern of crying and fussing in the first 3 months of life. The relative lack of carrying in our society may predispose to crying and colic in normal infants.

Carrying as colic "therapy": a randomized controlled trial.

In healthy infants, crying behavior is reduced significantly by "supplemental" carrying; that is, increased carrying throughout the day in addition to that which occurs during feeding and in response to crying. To determine whether the recommendation to increase carrying would be effective as a therapy for colic, 66 mothers of infants 4 weeks of age or less who came to their pediatricians with complaints of crying problems ("colic") were randomized to receive standard pediatric advice (standard group) or standard advice plus the recommendation to increase supplemental carrying by 50% (supplemental group). Overall, the supplemental group carried their infants 6.1 hours/d throughout the intervention period, an increase of 2.2 hours/d (56%) more than that provided by the standard group. Despite this significant increase in carrying, there was no difference between groups in the duration or frequency of crying, fussing, or cry/fuss at any time throughout the intervention period. When the greatest treatment effect was expected at 6 weeks, the supplemental group infants cried only 3 minutes less per day (95% confidence interval: 37 minutes less to 32 minutes more per day). We conclude that, compared with standard pediatric advice to be "responsive," supplemental carrying does not reduce crying and fussing behavior further in infants who have colic. In marked contrast to healthy infants, this apparent resistance to increased carrying may indicate an important difference in state regulation and control in infants with colic.

[Langerhans cell histiocytosis of the orbits]. / Langerhans-Zell-Histiocytose der Orbita.

Langerhans cell Histiocytosis is an infrequent disease of the orbit in little children. It is sometimes recognized in a rather late stage. We present three cases and discuss the manifestations and the treatment.

A developmental approach to the management of children with sleep disturbances in the first three years of life.

A developmental approach to the management of sleep disturbances was successful in 85% (44/52) of the children (aged 2-36 months) within 2-6 weeks. No drugs were used. The approach was based on the following two principles: taking into account the characteristics of sleep behavior in infants and young children by setting up seven rules of normal sleep behavior, and dealing with the expectations and anxieties of the parents by means of a sleep chart.

[Management of at-risk children: parent-child relations in the first half year. A descriptive study]. / Betreuung von Risikokindern: Eltern-Kind-Beziehung im ersten Lebenshalbjahr. Eine deskriptive Studie.

Early development of parental behavior was studied in 10 infants born at risk. The infants were firstborn, suffered from prematurity and perinatal complications and were separated from their mothers for at least three days after birth. Semistructured videotaped interviews with the parents and developmental and neurological testing of the infants were performed prior to the dismissal from the neonatal unit, and at three and six months after term. Premature birth, perinatal complications and separation of mother and child had a strong impact on the emotional development of the parents affecting mothers and fathers differently. A number of pre-, peri- and postnatal factors are reported influencing the mother-father-infant triad as well as the marital relationship. The findings of this study indicate that pediatric care of infants born at risk requires knowledge about the emotional changes during early parenthood, the risk factors of parenting after premature delivery and perinatal complications, and, in particular, about the emotional adaptation of the parents under these circumstances.

Neonatal metatarsus adductus, joint mobility, axis and rotation of the lower extremity in preterm and term children 0-5 years of age.

A total of 484 premature children and a control group of 114 healthy term children underwent orthopaedic follow-up from birth to 5 years of age. At birth, metatarsus adductus was found to be more frequent in twins than in single infants (41% vs 16%; P less than 0.01), but occurred with equal frequency in single preterm and term infants (16% vs 12%). By 5 years of age, metatarsus adductus had resolved in all the term but only in 81% of the preterm children (P less than 0.05). In the preterm and term groups, knee axis (mean intermalleolar distance 22.0 mm vs 20.1 mm), tibial torsion (mean angle -1.2 degrees vs + 0.6 degrees) and angle of gait (mean angle + 1.5 degrees vs + 0.7 degrees) at 5 years were statistically insignificant. Hip function at 5 years was similar in normal preterm and term children but significantly decreased in preterm children with cerebral palsy, more so with regard to abduction (56 degrees vs 39 degrees, P less than 0.05) and extension (22 degrees vs 8 degrees, P less than 0.01). The difference between the sexes was insignificant in both the preterm and term groups.

Pseudotumour cerebri after varicella.

UNLABELLED: Thrombotic complications in children with varicella are well known. Transient protein S deficiency due to the presence of an anti-protein S antibody seems to be responsible at least in part. In an 8-year-old girl, pseudotumour cerebri with abducens palsy and ileofemoral vein thrombosis occurred 3 weeks after chickenpox. A transient elevation of anti-protein S auto-antibodies was identified. Under treatment with heparin the pseudotumour cerebri rapidly disappeared but the iliofemoral thrombosis persisted. CONCLUSION: Thrombotic complications after varicella might be mediated at least in part by antibodies against protein S. Early recognition of this condition permits appropriate treatment with recombinant tissue-type plasminogen activator.

Compensatory maturational deceleration of growth or "catch-down growth" in patients with congenital adrenal hyperplasia after delayed initiation of therapy.

The growth pattern is reported of 16 patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency in whom therapy was started after the age of 3 years. Treatment was initiated at a mean chronological age (CA) of 4.7 years (range 3.0-7.2) and at a mean bone age (BA) of 10.4 years (range 10.2-13.2). It consisted of hydrocortisone (mean dosage 26 mg/m2) or prednisone (8.7 mg/m2) in all, and of fluorohydrocortisone (0.05-0.1 mg daily) in five patients. At the last examination the mean duration of therapy was 5.2 years, the mean CA 10.0 years, and the mean BA 12.6 years. In 13 of the 16 patients a "catch-down growth" pattern was observed, which was characterised by a decrease in height (expressed as SDS) for CA, a deceleration of bone maturation and increase of height (SDS) for BA, and an improvement in predicted height (Bayley-Pinneau).

Group G streptococcus sacroilitis with sepsis in a 15-y-old adolescent.

Group G streptococci cause invasive infections of different tissues. Most infected patients have underlying diseases and are of adult age. Invasive group G streptococcal infections rarely occur in childhood and adolescence. A 15-y-old boy with a beta-haemolytic group G streptococcus sacroiliitis, sepsis and secondary pulmonary manifestations resembling an acute respiratory distress syndrome is described.

Infants born to mothers with antibodies against Borrelia burgdorferi at delivery.

A serological survey over a 1-year period of 1416 mothers at delivery and their 1434 offspring for the presence of anti-Borrelia burgdorferi antibodies revealed a prevalence of 0.85%. Clinically active Lyme disease during pregnancy was found in 1 of these 12 women with elevated titres and the child was born with a ventricular septal defect. Of six affected children, two had hyperbilirubinaemia, one muscular hypotonia, one was underweight for gestational age, one was macrocephalic, and one had supraventricular extrasystoles. Anomalous findings could not be attributed to B. burgdorferi due to a lack of serological evidence of intrauterine infection. Our data do not imply the need for serological screening in pregnancy, however, the importance of recognition and treatment of Lyme disease in pregnancy is emphasized.

Neurological outcome in high risk weight appropriate for gestational age preterm children at early school age.

Neurological development in preterm children with birth weight appropriate for gestational age is reported in two separate groups: a longitudinal study of 97 preterm children and 93 term children as a control group and a cross-sectional study of 249 preterm children. Both preterm groups were regarded as high risk with respect to number of outborns, distribution of gestational age and perinatal risk factors. Neurological outcome at 5-6 years of age in the majority of the preterm children was comparable to that of the term children. However, 15% of boys and 9% of girls in the preterm group were diagnosed as having cerebral palsy. Mild diplegia was most frequently observed; 4% of the children were severely impaired. Fourteen percent of the preterm vs 2% of the term boys and 6-9% of the preterm vs none of the term girls received motor therapy during early school age. There was a small but consistent sex difference in neurological outcome in favour of the term and preterm girls. Effects of drop out rate and of incompleteness of ascertainment are reported in detail.

Predicting developmental outcome at school age from infant tests of normal, at-risk and retarded infants.

The predictive validity of developmental testing was investigated in term, preterm and retarded children. Verbal, performance and locomotor development were assessed at various ages and individual development curves constructed. Inter-age correlations between development tests at nine to 24 months and intellectual assessment at seven years revealed a strong effect on prediction for age at testing and level of mental performance. Categorisation by level of mental performance demonstrated that at seven years 98.6 per cent of the children with developmental quotients (DQs) greater than 85 at 24 months achieved IQs greater than 85, while 98.7 per cent of the seven- to eight-year-old children with DQs greater than 85 at nine to 24 months achieved IQs greater than 85. The Griffiths language and performance scores and their combination were the strongest predictors of later intellectual functioning. The social score was of moderate significance, while the locomotor score had no predictive value. Analysis of individual development curves revealed that prediction was hampered in some children by factors not detectable by statistical analysis, such as dissociations in development, organic impairment and major life events. Among the prenatal, perinatal and postnatal variables studied, only socio-economic status was of predictive significance; its effect depended on the level of mental performance and was most marked in term children, but it had no effect on retarded children.

Prenatal diagnosis of Schwartz-Jampel syndrome with early manifestation.

A mother who had given birth to a child with Schwartz-Jampel syndrome (SJS) with neonatal manifestations (myotonia, congenital contractures, bowing of femora and tibiae) underwent ultrasonic fetal examination during the 17th and 19th week of her second pregnancy. Moderately decreased fetal motor activity and constant flexion of the fingers were observed at both examinations. In addition, there was mild bowing and shortening of the femora. At birth, the child presented with the characteristic pattern of SJS similar to her older brother. Prenatal ultrasonic diagnosis of Schwartz-Jampel syndrome is possible, at least for the form with neonatal onset of myotonia and contractures.

Infection with Staphylococcus aureus producing toxic shock syndrome (TSS) toxin-1 without TSS.

Relevant findings are reported in an 8-year-old boy with skin infection due to Staphylococcus aureus producing toxic shock syndrome toxin-1 without shock but with an increase in antibody titre against the toxin.

Stable human immunodeficiency virus encephalopathy in two infants receiving early intravenous gammaglobulin plus antimicrobial prophylaxis.

Two infants with human immunodeficiency virus (HIV) infection, encephalopathy, intrathecal anti-HIV IgG antibody production and (in one case) the presence of HIV antigen received monthly doses of intravenous gammaglobulin (IVGG) and daily antimicrobial prophylaxis starting at the ages of 6 and 9 months respectively. The follow-up over 15 and 12 months revealed a favourable course with remarkable improvement in visuo-spatial functions, receptive language, play behaviour and fine motor skills, as well as in muscle tone, pyramidal tract signs and vigilance in case 1, and near normalization in case 2. Viability of HIV in peripheral blood mononuclear cells, antigen in serum and cellular immunodeficiency, however, all remained unchanged. We suggest that neurological complications of encephalopathy in paediatric acquired immunodeficiency syndrome may have a slower progression when IVGG treatment plus antimicrobial prophylaxis is started early.

Effects of the long-acting somatostatin analogue SMS 201-995 in an infant with intractable diarrhea.

The long-acting somatostatin analogue SMS 201-995 was administered to a six-month-old infant with intractable diarrhea after failure of conventional treatment. During eight weeks of treatment, the secretory component of the diarrhea was positively influenced with a reduction of daily stool weight and stool sodium concentration. Plasma levels of growth hormone were markedly, and levels of insulin, IGF I, gastrin, pancreatic polypeptide, VIP, and neurotensin moderately decreased. Linear growth was also inhibited. The patient unexpectedly died from fulminant colitis at a time, when the dosage had been reduced from 18 to 3.5 micrograms/kg/day. The relationship, if any, between therapy with SMS 201-995 and the colitis remained unclear. It is concluded that SMS 201-995 can be effective in reducing secretory diarrhea in infants. However, further studies are necessary to assess the safety of its administration in this age group.

Immunological evaluation in the early diagnosis of prenatal or perinatal HIV infection.

A longitudinal evaluation was carried out of the clinical, infective, and immunological progress of 34 children (who were aged 6 to 68 months--mean 25 months at the time of writing) born to 31 mothers infected with human immunodeficiency virus (HIV), over a mean observation period of 13.4 months. Clinical symptoms, not always clearly related to HIV became apparent in 11 children, and preceding immune abnormalities were documented in two of them. In eight children culture for HIV was positive, and six of these were symptomatic. No cancers were diagnosed and none of the children died. Immune abnormalities including hypergammaglobulinaemia, IgG subclass deficiency, low serum IgA concentration, antibody deficiency, a decrease in the number of CD4+(T helper) cells, and defective cellular responses to antigens, were found in seven of the children in whom culture for HIV was positive; in two of four who had symptoms and in all four who were symptom free and in whom culture was negative for HIV but in whom HIV antibodies persisted and who were older than 15 months; and in three of nine who were symptom free and in whom culture was negative with loss of HIV antibodies. We conclude that serological diagnosis alone may be misleading and that additional immunological assessment may help to identify affected children. Analysis of humoral and cellular responses to antigens used for vaccination such as tetanus toxoid by measurement of specific antibodies and skin testing are simple and helpful in clinical practice.

Pericardial effusion as a sign of acquired hypothyroidism in children with Down syndrome.

In three children with Down syndrome and acquired hypothyroidism echocardiography was performed before and after the start of L-thyroxine treatment. Initial studies revealed pericardial effusions which resolved during treatment suggesting that they were caused by hypothyroidism. The incidence of hypothyroidism in Down syndrome is high, but the diagnosis is often missed for lack of specific clinical criteria. The finding of pericardial effusion by echocardiography may be essential in discovering thyroid dysfunction. The cases illustrate that regular thyroid function tests are important in Down syndrome.