bluuebunny/crossref_metadata_2025 huggingface dataset:
Can Maternal Dietary Supplements Help in Preventing Infant Malnutrition?
Authors: PRENTICE A. M.
Published: January 2008

Many nutritional intervention programmes throughout the world have targeted food supplements towards women during the “at risk” periods of pregnancy and lactation. Some of these programmes, together with a number of small‐scale scientific studies, have attempted to evaluate the efficacy of supplementation in terms of birth outcome or lactational performance. After reviewing the available evidence it is concluded: (a) that supplementation during late pregnancy can have a significant beneficial effect on birthweight in women who are genuinely “at risk” due to an inadequate home diet; (b) that statistical projections would predict that the increase in birthweight should be accompanied by a significant decrease in neonatal mortality; (c) that supplementation during lactation is most unlikely to increase breast‐milk output or significantly improve its composition except perhaps in extremely malnourished women. Since pregnant women form a small and easily identifiable target group, and since the potential benefits of extra food may be substantial, it is recommended that future efforts are focussed in this direction. Large‐scale effectiveness and cost‐benefit trials are required with neonatal mortality as the primary outcome.
Aspects of Anthropometric Evaluation of Malnutrition in Childhood
Authors: DURNIN J. V. G. A.
Published: January 2008

The evaluation of malnutrition by the use of anthropometry poses considerable problems if any meaningful information is to be acquired, particularly on individual children. It is possible to formulate reference values (17), for height and weight taking age into account, or perhaps as a simple ratio but there may be little relation between the assessment of malnutrition by this means and functional impairment. It is suggested in this paper that anthropometry in this context should include length or stature, weight, skinfold thicknesses (triceps, subscapular and suprailiac) and circumferences (head, upper arm, upper thigh). The reference values to be used in the assessment of malnutrition are not very satisfactory, and there are objections to the simple use of the NCHS (9) data. It would be more desirable to use values obtained on “well‐fed” indigenous populations, although these hardly exist at the present time. Well‐controlled surveys are urgently needed on length or stature, weight, skinfolds, and circumferences of appropriate populations if anthropometric evaluation of malnutrition is to be anything other than a crude assessment. At a more complex level, the relationship of these anthropometric variables to various functional tests also needs investigation.
Growth Hormone and Insulin‐Like Growth Factor I: Nutritional Pathophysiology and Therapeutic Potential
Authors: BIER DENNIS M.
Published: January 2008

The growth hormone—insulin‐like growth factor I axis has been appreciated for more than 30 years and the effects of malnutrition on this axis for more than 20 years. Over the last decade, advances in molecular biology have permitted enhanced understanding of feedback regulation between growth hormone and IGF‐I at the gene level, including limited information on nutritional influences. Similarly, the availability of recombinant human growth hormone has allowed controlled clinical studies demonstrating its net anabolic actions at hypocaloric dietary energy intake levels and its ability to enhance height velocity in children with various causes of diminished growth. Although investigational use of recombinant IGF‐I in humans has been limited, its actions are likely to complement those of growth hormone during periods of profound dietary energy deficit. From the information presented, two hypotheses are developed. First, recombinant IGF‐I administration will enhance substrate anabolic events during the acutely malnourished state when dietary intake is severely limited. Second, administration of recombinant human growth hormone will accelerate protein anabolism and catch‐up growth during the period of recovery from protein‐energy malnutrition. Given current clinical investigational tools and the availability of both recombinantly‐produced hormones, these are testable hypotheses.
Use of Soya‐Beans for the Dietary Prevention and Management of Malnutrition in Nigeria
Authors: ABIODUN P. O.
Published: January 2008

Protein‐energy malnutrition is still highly prevalent in the developing countries due to the decline in breast‐feeding and early introduction of overdiluted and often contaminated commercial milk products, use of weaning foods which are low in energy and nutrients, a high prevalence of diarrhoea and infections. An ideal food for the prevention and management of malnutrition should be of high nutritive value, acceptable to the children and their mothers, readily available at a cheap rate, easy to prepare and well tolerated both in health and disease. Soya‐beans fulfill these conditions. It is furthermore easily processed and can be combined with a variety of foods to increase nutritive value and make them ideal both as a staple and as a weaning food. Soy‐milk as well as a combination of soya‐beans with maize pap “soyogi”, have been found to be valuable in the management of malnutrition. In our preliminary results, home made “soyogi” was found to be as good as the more expensive commercial soy‐milk product in the dietary management of acute diarrhoea in children. The children on “soyogi” tended to have a shorter duration of diarrhoea, decreased number of stools, and increased weight gain. The use of soya‐beans both as soy‐milk and as “soyogi” among other formulations in the homes is recommended for prevention and management of malnutrition.
Biochemical Characteristics and Diagnosis of the Carbohydrate‐deficient Glycoprotein Syndrome
Authors: STIBLER HELENA , JAEKEN JAAK , KRISTIANSSON BENGT
Published: January 2008

29 patienxts with a new inherited complex developmental deficiency syndrome—the carbohydrate‐deficient glycoprotein syndrome—were studied biochemically. The most striking biochemical abnormality in these patients is the presence of secretory glycoproteins, that are deficient in their carbohydrate moieties. Serum transferrin shows the most pronounced carbohydrate defect, both quantitatively and qualitatively. Half of this glycoprotein is apparently missing two or four of its terminal trisaccharidcs—sialic acid, galactose and N‐acetylglucosamine—while the carbohydrate core appears to be intact. The abnormal transferrin is also present in the liver. This biochemical alteration is a highly specific marker of the syndrome, which can be diagnosed either qualitatively by isoelectric focusing of serum transferrin or quantitatively by the “carbohydrate‐deficient transferrin” (CDT) assay. In the CDT assay all of these patients have values approximately ten times above the reference level. The unique carbohydrate defect in secretory glycoproteins indicates that this disorder represents a new type of inborn error of glycoprotein metabolism. Studies of eleven enzymes in glycoprotein synthesis and catabolism have not revealed any deficiency of glycosidases or glycosyltransferases. The nature of the transferrin change and the cathepsin assays performed to date may suggest an as yet unidentified degradation abnormality.
Sural Nerve Pathology in the Carbohydrate‐deficient Glycoprotein Syndrome
Authors: NORDBORG C. , HAGBERG B. , KRISTIANSSON B.
Published: January 2008

Sural nerve biopsies from nine children with the carbohydrate deficient glycoprotein (CDG) syndrome were examined. The light and electron microscopical findings were consistent with a mild neuropathy of complex pathogenesis in all the children. The most striking finding was an attenuation of myelin sheaths and the presence of multivacuolar myelinoid bodies in the Schwann cells, indicating a change in myelin turnover. Degeneration of scattered unmyelinated fibres suggests that axons may also be primarily affected. Fibrillary‐crystalline cytoplasmic inclusions were found in endo‐and epineurial fibroblasts in all the cases. The neuropathological features support the contention that this syndrome represents a new type of error of macromolecular metabolism.
Pathogenic Mechanisms of Pubertal Growth Failure in Chronic Renal Failure
Authors: SCHAEFER F. , SCHÄRER K. , MEHLS O.
Published: January 2008

Patients with chronic renal failure (CRF) are prone to growth retardation throughout childhood (Fig. 1). In infancy, growth failure in uraemic patients is attributable mainly to reduced spontaneous food intake. During mid‐childhood, when statural growth is believed to be dependent primarily upon growth hormone (GH), growth may be variably affected by CRF, ranging from growth patterns parallel to standard centile curves to complete growth arrest. In late prepuberty particularly, growth rates may be much lower than normal, and the pubertal growth spurt is usually insufficient to produce a normal adult height. While nutritional supplementation and treatment with recombinant human GH offer promising options for the treatment of growth failure in infants and prepubertal children with CRF, increasing interest is now focusing on the problem of reduced pubertal growth in uraemia.
Insulin‐Like Growth Factors (IGFs) and IGF Binding Proteins in Chronic Renal Failure: Evidence for Reduced Secretion of IGFs
Authors: BLUM W.F.
Published: January 2008

To test the hypothesis that growth hormone (GH) insensitivity is responsible, amongst other mechanisms, for impaired growth in uraemic children, insulin‐like growth factor I (IGF‐I), IGF‐II, IGF binding protein‐1 (1GFBP‐1), IGFBP‐2 and IGFBP‐3 were measured by radioimmunoassay in normal control children, in patients with end‐stage renal failure (n = 51) and in patients with preterminal chronic renal failure (n = 11) and the production rate of IGF was calculated. A unique pattern of normal IGF‐I and IGF‐II levels and markedly increased levels of all three IGFBPs was present in uraemia. Measurement of free IGF‐II binding capacity, and affinity cross‐linking experiments showed that the excess immuno‐reactive IGFBP was able to bind IGFs. To explain the excess of unoccupied IGF binding sites in uraemia, a mathematical model was developed which describes the production of IGFs and their interaction with IGFBP. Calculations of IGF secretion rates suggested that production of IGF is two orders of magnitude lower in uraemic children than in control children, despite normal GH secretion. It is concluded that in uraemia there is a relative GH insensitivity with respect to IGF production.
Serum Binding Proteins for Growth Hormone: Origins, Regulation of Gene Expression and Possible Roles
Authors: HERINGTON A.C. , TIONG T.S. , YMER S.I.
Published: January 2008

The above discussion highlights the heterogeneity of the family of GH receptors/GHBPs and their mRNAs. Considerable uncertainty still exists as to the interrelationships between the various forms, the specific mechanisms for their generation and their possible significance in terms of modulating GH action and receptor function. Collectively, the regulatory data indicate that while the mRNAs encoding the membrane and soluble GH receptors/GHBPs in the rat are expressed by the same broad distribution of tissues, they can be differentially regulated. Such regulated expression implies a functional basis for production of GHBP. The wide tissue distribution of GHBP mRNA also suggests a role for GHBP as a paracrine/autocrine effector molecule, perhaps in addition to an endocrine role. Additional studies, both in vitro and in vivo, perhaps utilizing highly purified recombinant GHBP, will be required to provide more definitive information as to the true physiological role(s) of the circulating GHBPs.
Dramatic Early Postnatal Growth Failure in Children with Early Onset Growth Hormone Deficiency
Authors: CHATELAIN P.
Published: January 2008

From the large group of patients included in the Kabi Pharmacia International Growth Study, 58 were studied who were treated with growth hormone (GH) before the age of 2 years, after diagnosis of GH deficiency (GHD). The growth parameters calculated from these infants indicate dramatic early postnatal growth failure, thus suggesting that GH could participate in early postnatal growth. The intensity of growth failure in these infants with GHD suggests that early screening on clinical grounds is possible.
Puberty in Children with Idiopathic Growth Hormone Deficiency on Growth Hormone Treatment: Preliminary Analysis of the Data from the Kabi Pharmacia International Growth Study
Authors: PRICE D.A.
Published: January 2008

In total, 524 children (335 boys, 189 girls) with idiopathic growth hormone deficiency (GHD) who had entered puberty after at least 1 year of growth hormone (GH) treatment were studied. Spontaneous onset of puberty occurred in 319 boys and 174 girls, and puberty was induced in 16 boys and 15 girls. Median chronological and bone ages at onset of spontaneous puberty were 14.8 and 13.4 years in boys, and 13.3 and 11.8 years in girls, respectively. Age at onset of spontaneous puberty was correlated to age at start of GH treatment. Puberty was induced at a median chronological age of 16.0 years in boys and 14.4 years in girls. Height velocity increased in the year before spontaneous puberty in boys and girls, and rose further to a maximum (median, 8.3 cm/year) in the first year of puberty in boys. In girls, the maximum height velocity occurred in the year before puberty (median, 6.5 cm/year). Height velocity during the first year of puberty was inversely correlated to age at puberty onset (r= ‐0.56, p ‐ 0.0001). After medical induction of puberty, height velocities in the first year of puberty were 5.4 and 3.4 cm/year for boys and girls, respectively. The change in median height SDS from start of GH treatment to puberty onset was +0.7 in boys and +0.8 in girls, and when corrected for bone age was ‐0.3 in boys and +0.6 in girls. GH doses were decreased from the start of treatment to puberty onset, decreased further during the first 2 years of puberty in boys, but increased in the second year of puberty in girls. Height velocity was positively correlated to dose of GH in the first year of puberty in boys (r= 0.27, p= 0.002) and girls (r= 0.27, p= 0.02). The relationship between dose and height velocity, when controlled for age by covariance analysis, was lost in girls but maintained in boys. In a subgroup of boys who were younger at diagnosis, the dose‐height velocity relationship was greater (r= 0.59, p= 0.001) than for the total group. In the first year of pubertal growth in boys with idiopathic GHD on GH treatment, there is a dose‐response relationship.
Breast feeding duration in consecutive offspring: a prospective study from southern Brazil
Authors: Victora Cesar G , Huttly Sharon R , Barros Fernando C , Vaughan J Patrick
Published: January 2008

The association between breast feeding duration in two consecutive pregnancies was studied in a prospective study in southern Brazil. In a population‐based sample of 5960 women giving birth in 1982, 1386 delivered a second child within 4 years. The data were analyzed using life table techniques. The duration of breast feeding of the second child increased directly according to the duration the previous child had been breast fed. In particular, when the previous child had been breast fed for 6 months or more, the subsequent child was clearly more likely to be breast fed. However, when the previous child had been breast fed for under 6 months, the differences among subsequent children disappeared after 3–6 months. These differences were still present after stratification by family income, maternal education and parity. Mothers with a previous unsuccessful or problematic breast feeding experience should receive special priority in promotion campaigns.
Characteristics and prognosis of hospital‐treated obstructive bronchitis in children aged less than two years
Authors: Wennergren G , Hansson S , Engström I , Jodal U , Åmark M , Brolin I , Juto P
Published: January 2008

In a prospective study 101 children aged less than 2 years (median age 10 months), were examined the first time they were admitted to a paediatric ward for asthmatic symptoms. Two‐thirds were boys and 58 had parents or siblings with allergic symptoms. During winter‐spring, respiratory syncytial (RS) virus was verified in 50% of children. Other viral agents were adenovirus, parainfluenza 3, coxsackie B 2, ECHO 6 and rotavirus. At the acute stage, 54% of the children displayed changes on pulmonary X‐ray. The total IgE value was + 2 SD score units in 14 children. At reinvestigation after 3–4.5 years, when the children were aged 3.3–6.3 years, 53% were free from asthmatic symptoms; the median age for the last episode was 2 years. A total of 33% had mild asthma, 8% moderate and 6% severe asthma. The factors which correlated significantly with persistent asthma were: (1) The need for daily medication for at least 6 months. (2) A young age in conjunction with the first wheezing episode and on the first admission to a paediatric ward because of asthmatic symptoms. (3) Other past or present atopic symptoms. Heredity, tobacco smoking at home, having a furry pet, RS virus infection, or high total IgE at the time of the first admission did not correlate significantly with the persistence of asthma 3–4.5 years later. The results emphasize the good overall prognosis of wheezing in early childhood, even when the wheezing is severe enough to lead to inpatient treatment.
Dominant role for osmolality in the efficacy of glucose and glycine‐containing oral rehydration solutions: studies in a rat model of secretory diarrhoea
Authors: Ferreira RMC Cunha , Elliott EJ , Watson AJM , Brennan E , Walker‐Smith JA , Farthing MJG
Published: January 2008

The effect of osmolality on the efficacy of oral rehydration solutions (ORS) and the contribution of the amino acid glycine to water absorption from ORS have been studied in an animal model of secretory diarrhoea. After exposure to pure cholera toxin, rat small intestine (excluding the duodenum) was perfused in situ with seven different ORS. All ORS were derived from a “basic” solution containing Na 50, K 25, Cl 75 and glucose 50 mmol/l to which 25 or 50 mmol/l of glycine, glucose, or mannitol was added. All ORS reversed water secretion to absorption, but maximum water absorption was obtained with the “basic” solution with an osmolality of 200 mOsm/kg. When the osmolality of the “basic” solution was raised to 225 and 250 mOsm/kg by adding mannitol, water absorption decreased. At each of these osmolalities, substitution of mannitol by glycine or glucose resulted in similar increases in water absorption, but all modifications compared unfavourably with the “basic” solution. Net sodium secretion occurred with all ORS tested, despite net water absorption. These findings in a perfusion model of rat small intestine suggest that osmolality is a key factor influencing the efficacy of ORS and that addition of a second substrate, such as glycine, has no beneficial effects. Our results suggest that there is a maximal rate for water absorption from the small intestine which is inversely related to the osmolality of the perfusate.
Transient colonization of the gut of newborn infants by orally administered bifidobacteria and lactobacilli
Authors: Bennet R , Nord CE , Zetterström R
Published: January 2008

We investigated if orally administered bifidobacteria and/or lactobacilli could be cultured from faeces of infants after antibiotic treatment, when these bacterial species are usually absent. Lyophilized Bifidobacterium longum, strain BB‐536, B. breve, strain BB‐576, or Lactobacillus acidophilus, strain LAC‐343, were used. Doses of 3 ± 109 cells of one strain, or a mixture of all three strains 3 ± 109 cells each were fed three times daily at mealtimes to 11 infants aged 0‐8 weeks. Treatment was started the first day after antibiotic treatment and was continued for 5 days. The bacterial species were isolated in 9 of 11, 7 of 10 and 2 of 9 specimens obtained on the last day of bifidobacteria or lactobacilli administration, 5 and 15 days thereafter, respectively. No side effects were noted.
Transfer of specific IgG and IgG subclasses to herpes simplex virus across the blood‐brain barrier and placenta in preterm and term newborns
Authors: Osuga T , Morishima T , Hanada N , Nishikawa K , Isobe K , Watanabe K
Published: January 2008

The kinetics of virus‐specific IgG subclasses (IgG 1‐4) among newborns and their mothers has not yet been determined. In this report, we examined anti‐herpes simplex virus IgG activities (HSV‐IgG) and its subclasses in CSF and serum of premature or term newborns without HSV infection and in the serum of their mothers using ELISA. We found that CSF/serum ratios of HSV‐IgG and IgG subclasses (IgG 1‐4) in newborns with a gestational age less than 38 weeks were higher than those of term newborns. These findings indicate that the blood‐brain barrier against HSV‐IgG and IgG subclasses is insufficient in newborns. Furthermore, we found that HSV‐IgG subclasses, which were transferred across the placenta and later transferred across the blood‐brain barrier had a tendency to be proportional to each of the maternal HSV‐IgG subclasses.
Hyperbilirubinemia, hypocarbia and periventricular leukomalacia in preterm infants: relationship to cerebral palsy
Authors: Ikonen RS , Janas MO , Koivikko MJ , Laippala P , Kuusinen EJ
Published: January 2008

This study comprised 103 preterm infants with a gestational age less than 33 weeks who were born in Tampere University Hospital and who were followed up to two years of age. Sixty‐four perinatal variables were compared to ultrasound findings in the neonatal period and neurologic handicap at the age of two years. Duration of hypocarbia (PCO2± 30 mmHg) during the first 72 h and hyperbilirubinemia (the mean level of serum total bilirubin) at three days of age were independently and significantly related to periventricular leukomalacia, but not directly to cerebral palsy. The only perinatal variables related independently and significantly to cerebral palsy at two years of age were periventricular leukomalacia and ventriculomegaly. According to these results, periventricular leukomalacia was the main predictor of cerebral palsy in preterm infants. In addition to hypocarbia, hyperbilirubinemia may also be involved in the pathogenesis of extensive (severe cystic) periventricular leukomalacia.
Narcotic and nicotine effects on the neonatal auditory system
Authors: Trammer Regina M , Aust Gottfried , Köster Karin , Obladen Michael
Published: January 2008

To evaluate narcotic and nicotine effects on the neonatal auditory system, brain stem auditory evoked responses were recorded in 15 infants prenatally exposed to both narcotics and nicotine (median gestational age 38.4 weeks, median birth weight 2820g), in 15 nicotine exposed infants (gestational age 40.0 weeks, birth weight 3000g) and in 24 healthy term infants (gestational age 40.1 weeks, birth weight 3310g) who served as controls. Whereas nicotine‐exposed and control infants were similar in all brain stem auditory evoked response measurements, narcotic/nicotine infants had bilaterally increased wave V latencies (left:p=0.005; right:p=0.04) and I‐V intervals (left:p=0.02; right:p=0.01) when compared to controls. Our findings suggest that prenatal narcotic but not nicotine exposure negatively affects maturation or integrity of the neonatal auditory brain stem tract and that neither narcotic nor nicotine exposure is associated with hearing loss in the neonate.
Participation of immune complexes in adenovirus infection
Authors: Mistchenko AS , Lenzi HL , Thompson FM , Mota EM , Vidaurreta S , Navari C , Grinstein S
Published: January 2008

To determine the participation of immune complexes during adenovirus infection, we evaluated serum and necropsy specimens of patients with confirmed adenovirus infection of the lower respiratory tract. In lung and kidney from seven dead patients, immunofluorescence revealed the presence of hexon, immunoglobulins and complement. These patients had clinical manifestations of kidney dysfunction. In dead patients (3/3 in whom serum was available) neither anti‐adenovirus antibodies nor adenovirus‐specific immune complexes could be found in the final stage of the infection. However, two of these patients had anti‐adenovirus antibodies and immune complexes in samples obtained early in the infection. Most patients (16/19) who survived the infection had circulating anti‐adenovirus antibodies. Half also had immune complexes specific for adenovirus in some moment of the illness. This suggests that immune complexes arise during respiratory infection by adenovirus, probably contributing to its clinical picture.
The role of cow's milk protein intolerance in steroid‐resistant nephrotic syndrome
Authors: Sieniawska M , Szymanik‐Grzelak H , Kowalewska M , Wasik M , Koleska D
Published: January 2008

The role of cow's milk protein intolerance in steroid‐resistant nephrotic syndrome was evaluated in 17 children. Cow's milk was excluded from the diet for at least 14 days without changing previously ineffective predinisone dosage. Six patients with minimal change or mesangial proliferation went into remission 3 to 8 days after elimination of cow's milk. After a period of 2‐3 weeks of remission, cow's milk challenge was positive in three patients. After one year on a cow's milk‐free diet, two of six patients became milk tolerant and are in remission of NS, one of six became steroid‐dependent, two of six are still unable to tolerate cow's milk and are in remission on a cow's milk‐free diet and one of six children was lost from observation. The role of cellular mechanisms in steroid‐resistant nephrotic syndrome is suggested.
Magnetic resonance imaging of the brain structures in the posterior fossa in retarded autistic children
Authors: Hashimoto T , Murakawa K , Miyazaki M , Tayama M , Kuroda Y
Published: January 2008

Midsagittal magnetic resonance images of the brains of retarded autistic children were compared to those of non‐autistic mental retardation patients and controls. We found that the whole brain stem and particularly two of its components (the midbrain and medulla oblongata) were significantly smaller in retarded autistic children and mental retardation cases than in control children. The pons area was significantly smaller in mental retardation cases as compared to control children but did not differ between autistic and control children. Moreover, there was no difference in the brain stem between retarded autistic children and mental retardation cases. We also noted no difference in the cerebellar vermis area among retarded autistic children, mental retardation cases and control children. The ratio of the midbrain to posterior fossa area was significantly smaller only in autistic patients. Although the significance of these results is unknown, further examination of autistic children with a normal IQ is necessary.
A 10‐year prognosis for childhood allergic rhinitis
Authors: Linna O , Kokkonen J , Lukin M
Published: January 2008

The prognosis of allergic rhinitis was studied in 154 children aged 3‐17 years at diagnosis by means of a detailed questionnaire administered 8‐11 years later. The symptoms had completely disappeared in only 15 (10%) patients. The conjunctival symptoms, however, had disappeared or were controlled successfully by topical drug therapy in almost all, and 77 (50%) were managing without medication for allergic rhinitis. Twenty‐five (23%) of the 110 children with seasonal allergic rhinitis had a perennial disease at follow‐up, in contrast to seven (16%) of 44 with perennial allergic rhinitis originally who had only seasonal symptoms at follow‐up. Asthma or wheezing had developed in 29 cases (19%) and was more common (p < 0.01) among those with perennial allergic rhinitis (15 of 44) than among those with seasonal allergic rhinitis (14 of 110). No significant association was found between age at onset of symptoms, family history of atopic disease or type of treatment for allergic rhinitis and allergic rhinitis still present at follow‐up or development of asthma during the observation period.
Evaluation of captopril versus reserpine and frusemide in treating hypertensive children with acute post‐streptococcal glomerulonephritis
Authors: Morsi Mohamed R , Madina Ekram H , Anglo Adel A , Soliman Ashraf T
Published: January 2008

We have evaluated the effect of oral captopril versus a combination of oral reserpine and frusemide in the treatment of 20 children with post‐streptococcal glomerulonephritis (APGN) with hypertension. Captopril produced a significantly greater reduction in systolic and diastolic blood pressure in both the standing and supine positions than reserpine + frusemide at 0.75, 1, 1.25, 8, 24, 48 and 72 h after initiating treatment. Neither postural hypotension nor reflex tachycardia accompanied the therapeutic effect of captopril. Blood urea, serum creatinine and creatinine clearance did not change significantly after therapy in either study group. Three days after initiating treatment, the 24 h urinary catecholamine output increased significantly in children who received captopril but did not change in children treated with frusemide and reserpine
Oral zinc as initial therapy in Wilson's disease: two years of continuous treatment in a 10‐year‐old child
Authors: Milanino R , Deganello A , Marrella M , Michielutti F , Moretti U , Pasqualicchio M , Tamassia G , Tatò L , Velo GP
Published: January 2008

Two years of continuous therapy promoted a significant overall amelioration in a 10‐year‐old boy affected by an hepatic form of Wilson's disease in which zinc sulphate was the sole therapy. In particular, liver function returned to normal and hepatic histology also improved. The parameters characterizing copper metabolism were kept under good control, and a decrease in copper concentration was found in both erythrocytes and liver. The copper balance study performed during the 25th month of treatment showed that oral zinc was still efficiently inhibiting the intestinal absorption of copper. No side effects have been reported so far.
Rett syndrome: the late infantile regression period‐a retrospective analysis of 91 cases
Authors: Engerström Ingegerd Witt
Published: January 2008

The late infantile regression period of Rett syndrome was analysed in a consecutively collected series of females, born 1945–87 (median 17 1/2 years), fulfilling the diagnostic criteria for classic Rett syndrome. For general information, data from 91 girls and women were used (group A), while more detailed analysis was based on the youngest 20, all born during the last decade (group B). Median ages at onset of developmental stagnation (stage I) were 11 (5–24) and 10 (5–18) months for groups A and B, respectively. Loss of acquired skills (stage II) began at 19 (12–36) and 17(13–25) months, and lasted for 19 (2–53) and 13 (2–32) months. Onset of the period of loss was distinctly marked in 43%, dramatic in 16% and insidious in 41%. Gross motor delay and aberrant behaviour raised the first suspicions of disease, while a more specific pattern comprising a triad of manifestations, i. e. contact/communication, hand use/skill, babble/words, characterized the loss of acquired skills. Although development invariably came to a definite break at a crucial stage of maturation, an “awakening” (stage III) and return of interest to act and interact astonishingly followed the period of withdrawal. Apraxia was found in all, but some use of the hands could be enticed in engaging and non‐demanding situations. The observed preference for use of the left hand (9/11), compared to the right (2/11), suggested a reflection of an underlying dysfunction/disease process, also displayed as spike activity in left central leads in EEGs from 13/20 girls.
Transverse myelitis following mumps in children
Authors: Nussinovitch M , Brand N , Frydman M , Varsano I
Published: January 2008

Although acute transverse myelitis is a rare complication of mumps, it is relatively well documented. We describe a child who developed mumps associated acute transverse myelitis and who subsequently recovered completely. To our knowledge, only 13 cases have been reported in children. This case is compared with 13 previously reported patients.
Psychological development in children born with very low birth weight after severe intrauterine growth retardation: a 10‐year follow‐up study
Authors: Smedler Ann‐Charlotte , Faxelius Gerd , Bremme Katarina , Lagerström Monica
Published: January 2008

Children born small for gestational age (SGA) and children having very low birth weight, less than 1500 g, are claimed to be at risk of developmental problems, even when obvious pathology and disability are absent. In this study, sensorimotor and cognitive development of 14 medically healthy, very‐low‐birth‐weight and small‐for‐gestational‐age children were investigated. The children were born at the Karolinska Hospital between 1979 and 1981. At the time of the assessment, the children were aged 8.7‐11.2 years. The assessment instruments included the Wechsler Intelligence Scale for Children, a modified version of the Bruininks‐Oseretsky Test of Motor Proficiency, as well as selected subtests from the Halstead‐Reitan Neuropsychological Battery and from the Southern California Tests of Sensory Integration. Information was also obtained from obstetric, neonatal and pediatric records, which included early developmental assessments. As a control group, 14 children were recruited and matched for age, sex and socio‐economic background. The very‐low‐birth‐weight‐small‐for‐gestational‐age group scored significantly lower on measures of visuospatial ability, non‐verbal reasoning, strategy formation and gross‐motor coordination. The group differences were largely attributable to the subnormal performance of eight of the very‐low‐birth‐weight‐small‐for‐gestational‐age children. These children, who also tended to be born earliest (<33 weeks), had a high incidence of behavioral and educational problems. These findings are consistent with the view that the very preterm infant develops a different neurobehavioral organization than a full‐term infant. Developmental deficits may become increasingly evident in the early school years.
Studies on an outbreak of neonatal diarrhea caused by EPEC 0127:H6 with plasmid analysis restriction analysis and outer membrane protein determination
Authors: Wu Shi‐Xiao , Peng R‐Q
Published: January 2008

An outbreak of enteropathogenic Escherichia coli (EPEC) 0127:H6 diarrhea occurred at two nurseries for the newborn in Chongqing in May 1987. Sixty‐nine neonates had diarrhea; two deaths resulted. The epidemic strains, carrying 1.5 and 60 Md plasmid DNA, had an identical restriction digest profile and the same outer membrane protein pattern and could produce localized adherence to HeLa, HEp‐2 and FL cells. The rates of contamination with EPEC 0127:H6 on medical staff's hands in these two nurseries were 11.8% and 8.7%, respectively, whereas 85 samples from milk, air and other sources were all negative for EPEC. The source of infection was the index case's mother who had had watery stools. Transmission of EPEC 0127:H6 from infant to infant took place by way of the fecal‐oral route, most likely via the hands of medical staff attending their care. We present the first case, confirmed by plasmid and restriction analyses and outer membrane protein determination, of a neonate who acquired EPEC during delivery through ingestion of organisms residing in the maternal birth canal.
Tracheostomy in pediatric patients
Authors: Puhakka HJ , Kero P , Valli P , Iisalo E
Published: January 2008

From 1978 to 1987, tracheostomy was performed on 33 children, 13 boys and 20 girls, with a male to female ratio of 0.65: 1. The mean age at the time of tracheostomy was 726 days, 76% of the children being under the age of two years. The incidence of pediatric tracheostomy per hospital admissions was 0.05%. Subglottic stenosis (13 children) and respiratory distress syndrome with prolonged endotracheal ventilation (11 children) were the most common indications for tracheostomy. The mean duration of prolonged endotracheal intubation before tracheostomy was 64 days, and that of tracheostomy treatment 117 days. During the tracheostomy period, five children died, but only one death was related to tracheostomy. The total rate of complications was 30%. We emphasize the importance of strict indications for pediatric tracheostomy.
Late sequelae of gonadal, mediastinal and oral teratomas in childhood
Authors: Lahdenne P
Published: January 2008

Thirty‐two patients with gonadal or non‐sacrococcygeal extragonadal teratomas who underwent operation in childhood were evaluated for different late sequelae. Follow‐up times ranged from 5.0 to 40 years. Physical abnormalities resulting from surgery were seen in eight patients who had had oral or mediastinal primary teratomas. Radiographs revealed multiple spina bifida occulta lesions in three patients. The males, but not the females, showed signs of gonadal dysfunction. Impaired semen quality or abnormal serum concentrations of testosterone or gonadotrophins were found in five of 10 adult men who had had testicular or mediastinal teratomas. Thus, some late sequelae may be predicted from the site of the primary teratoma, but gonadal dysfunction in males and vertebral anomalies may occur irrespective of where the teratoma is situated.
Thyroid dyshormonogenesis: severe hypothyroidism after normal neonatal thyroid stimulating hormone screening
Authors: Zegher F de , Vanderschueren‐Lodeweyckx M , Heinrichs C , Vliet G Van , Malvaux P
Published: January 2008

We report four children who presented no evidence of primary hypothyroidism in the neonatal period, either clinically (normal growth velocity) or biochemically (normal plasma levels of thyroid stimulating hormone and/or thyroid hormone). However, in early childhood, these children developed severe hypothyroidism due to dyshormonogenesis. We conclude that apparently normal thyroid function in the neonatal period does not preclude the development of severe hypothyroidism due to thyroid dyshormonogenesis later in childhood.
Intratracheal N‐acetylcysteine use in infants with chronic lung disease
Authors: Bibi H , Seifert B , Oullette M , Belik J
Published: January 2008

To evaluate the effect of intratracheal administration of N‐acetylcysteine (Mucomyst®) on the clinical status, pulmonary function and gas exchange in premature infants with chronic lung disease, we conducted a randomized, placebo‐controlled, crossover trial. Ten mechanically ventilated infants (gestational age 27 ± 1 week; postnatal age 22 ± 6 days) with clinical and radiological evidence of chronic lung disease and increased airway secretion were enroled in the study. Each infant received tracheal administration of 5% N‐acetylcysteine for one week and saline placebo every 4 h for another week. N‐acetylcysteine was associated with a 59 ± 26% increase in total airway resistance by the third day of treatment (p < 0.01). A two‐fold increase in airway resistance associated with an increased frequency of bradycardia and cyanosis spells was seen in two of the infants following three days of N‐acetylcysteine administration, with a rapid improvement in their condition when subsequently switched to saline. Overall, N‐acetylcysteine administration had no effect on the variables measured. We conclude that intratracheal administration of N‐acetylcysteine to liquefy airway mucus neither improves the clinical condition nor hastens recovery in premature infants with chronic lung disease and its administration may lead to increased total airway resistance and cyanotic spells. The present data do not support the use of N‐acetylcysteine as a mucolytic agent in premature infants with chronic lung disease.
Liver transplantation in tyrosinaemia type 1: the dilemma of timing the operation
Authors: Salt A , Barnes ND , Rolles K , Calne RY , Clayton PT , Leonard JV
Published: January 2008

Four children with tyrosinaemia type I received liver transplants. The metabolic disorder was corrected and all four had normal liver function on an unrestricted diet. Two children, transplanted at age five and seven years, proved to have occult hepatocellular carcinoma and both subsequently developed pulmonary metastases. One child was well 32 months after removal of a single pulmonary metastasis but the other child died with multiple metastases. The two younger children, transplanted at age 19 and 21 months, were well 28 and 44 months after operation, one after a second liver transplant. Our experience confirms the high risk of hepatocellular carcinoma in this disease and the potential value of early liver transplantation.
Clinical and electrophysiological reports in a case of early onset myotonia congenita (Thomsen's disease) successfully treated with mexiletine
Authors: Ceccarelli M , Rossi B , Siciliano G , Calevro L , Tarantino E
Published: January 2008

A sporadic event of myotonia congenita in a infant admitted to the Paediatric Clinic for frequent crises of apnoea, cyanosis, vomiting and difficult feeding is reported. EMG analysis was consistent with the dominant variety of myotonia congenita. Mexiletine therapy showed excellent results in reducing myotonic activity. It is worthwhile stressing that early symptoms may go unnoticed or may be misinterpreted and that information on the genetic form of the disease can be obtained also from the EMG analysis through a repetitive stimulation test.
Supplementation of an adapted formula with bovine lactoferrin. 2. Effects on serum iron, ferritin and zinc levels
Authors: Chierici R , Sawatzki G , Tamisari L , Volpato S , Vigi V
Published: January 2008

Breast milk provides an excellent supply of most nutrients for newborn infants. Infant formulae should be nutritionally comparable to breast milk especially with regard to critical nutrients like iron and other trace elements. Infant formulae supplemented with various amounts of bovine lactoferrin were given to two groups of infants. These infants were compared with infants receiving unsupplemented formula and breast‐fed infants. The effects of these diets on levels of haemoglobin, haematocrit, serum iron, ferritin and zinc were examined for a study period of 150 days. At birth, concentrations of iron, haemoglobin, haematocrit and zinc were comparable in all four feeding groups. The fact that the serum zinc level was not altered by lactoferrin supplementation appears to rule out an in‐vivo effect of lactoferrin on zinc nutrition of infants. Ferritin levels of breast‐fed infants were significantly higher than in non‐supplemented formula‐fed infants at day 30 and day 90. This difference was seen only at day 30, when comparing breast‐fed infants to lactoferrin‐supplemented formula‐fed infants. Comparing the infants receiving formulae, the formula supplemented with the higher amount of bovine lactoferrin induced significantly higher serum ferritin levels compared to the unsupplemented formula at day 90 and day 150. These observations favour the idea that lactoferrin may be involved in iron absorption. Since this effect was pronounced only after 90 days, it has to be discussed as to whether this effect is a convincing argument for supplementing infant formulae with bovine lactoferrin.
Temperature, metabolic adaptation and crying in healthy full‐term newborns cared for skin‐to‐skin or in a cot
Authors: Christensson K , Siles C , Moreno L , Belaustequi A , De La Fuente P , Lagercrantz H , Puyol P , Winberg J
Published: January 2008

The aim of the present study was to compare temperatures, metabolic adaptation and crying behavior in 50 healthy, full‐term, newborn infants who were randomized to be kept either skin‐to‐skin with the mother or next to the mother in a cot “separated”. The babies were studied during the first 90 min after birth. Axillary and skin temperatures were significantly higher in the skin‐to‐skin group; at 90 min after birth blood glucose was also significantly higher and the return towards zero of the negative base‐excess was more rapid as compared to the “separated” group. Babies kept in cots cried significantly more than those kept skin‐to‐skin with the mother. Keeping the baby skin‐to‐skin with the mother preserves energy and accelerates metabolic adaptation and may increase the well‐being of the newborn.
Cerebral and aortic blood flow velocity patterns in preterm infants receiving prophylactic surfactant treatment
Authors: van Bel Frank , de Winter Peter J , Wijnands Hannie BG , van de Bor Margot , Egberts Johannes
Published: January 2008

Blood velocity in the internal carotid artery (ICA) and in the descending thoracic aorta (DAo) was investigated used Duplex‐Doppler ultrasound in 14 infants of <30 weeks gestation, treated prophylactically with surfactant, and in 11 comparable infants with relatively mature lungs who served as controls. After surfactant administration, blood gases, pH or FiO2 were not different between the groups. Temporal mean blood velocity in the ICA was used as a relative measure of cerebral flow (TMFV‐cer), and its coefficient of variation (CV‐cer) was used to assess fluctuations in cerebral blood velocity. The pulsatility index (PI) in the ICA (PI‐cer) and DAo (PI‐DAo) was used to estimate if a left‐to‐right shunt was present. During surfactant instillation TMFV‐cer was abnormally low and CV‐cer indicated a fluctuating cerebral blood velocity. At 10 min after surfactant administration, TMFV‐cer of the treated infants was higher compared to the controls, while CV‐cer was stable in both groups. PI‐cer and PI‐DAo were abnormally high during the first hour of life after surfactant treatment, suggesting a left‐to‐right shunt without, however, clinical signs of a hemodynamically important ductus arteriosus. We suggest that cerebral perfusion is affected during and at 10 min after surfactant instillation. Left‐to‐right shunting appears to be a common event following surfactant treatment.
Human gastric lipase: ontogeny and variations in children
Authors: Sarles J , Moreau H , Verger R
Published: January 2008

The aim of this study was to investigate the precise origin of the acid pre‐duodenal lipase during human development and to evaluate its possible changes, at the tissue level, in children with gastritis or pancreatic insufficiency. Human gastric lipase appears around the 11 th week of gestation and increases slowly during pre‐ and postnatal development. It is localized in the fundus of the stomach without any lingual localization. Human gastric lipase reaches its adult level in the third month of life, and does not vary in relation to pancreatic insufficiency. It is only rarely impaired during gastritis.
The effect of dietary pectin on rapid catch‐up weight gain and urea kinetics in children recovering from severe undernutrition
Authors: Doherty J , Jackson Alan A
Published: January 2008

The rates of weight gain and urea kinetics were measured in 12 children receiving one of two energy dense, isonitrogenous formulae (711 kJ/kg/day, 170 kcal/kg/day) during recovery from severe undernutrition. Both formulations contained added arachis oil but in one a source of complex carbohydrate was added in the form of pectin (3.4% of total energy). The children taking the pectin diet had a rate of weight gain which was highly significantly less (7 g/kg/day) than the children not receiving pectin (14 g/kg/day). Urea production was significantly less on the pectin diet (0.37 ± 0.07 vs 0.55 ± 0.18 gN/kg/day). On the pectin diet there was a reduction in the rate of excretion of urea in urine and in the rate at which urea nitrogen was salvaged by the lower bowel, but these differences failed to reach statistical significance.