bluuebunny/crossref_metadata_2025 huggingface dataset:
Nested amplification protocol for the detection of Mycobacterium tuberculosis
Authors: Narita M , Matsuzono Y , Shibata M , Togashi T
Published: January 2008

Several methods for rapid diagnosis of tuberculosis have been devised through DNA amplification. However, the chemically strong cell wall of the species, the presumptively low numbers of organisms and their uneven distribution in clinical samples, and the lack of a “gold standard” for diagnosing tuberculosis, have hindered the routine clinical use of this method. In a pediatric patient group, these factors are more perplexing. To circumvent these problems, we made use of nested amplification and developed a standard protocol for extracting DNA from various forms of clinical samples which were suitable to our clinical laboratory. It is our impression that the overall sensitivity, including technical bias accompanying this method, is equal to, or at least greater than, that of culture. Most notably, the rapidity in obtaining results and the simplicity in handling, storage and transfer of samples are the principal advantages of this method.
Guillain‐Barré syndrome presenting with severe musculoskeletal pain
Authors: Manners Prudence J , Murray Kevin J
Published: January 2008

Three children with Guillain‐Barré syndrome are described who presented with musculoskeletal pain to an orthopaedic clinic or to a paediatric rheumatologist at a children's hospital. In each case, osteomyelitis of the spine was considered to be the most likely diagnosis and bone scans were performed in two of the three patients. Two of the three children required care in an intensive care unit, within hours of diagnosis. Outside of the speciality of neurology, the presentation of Guillain‐Barré syndrome with severe muscle pain is not generally well known. With greater awareness of this particular presentation, dangerous delays in diagnosis and inappropriate investigations will be minimized.
Bone marrow examination before steroids in thrombocytopenic purpura or arthritis
Authors: Reid MM
Published: January 2008

Corticosteroids were used to treat two children with presumed idiopathic thrombocytopenic purpura and one with juvenile rheumatoid arthritis without examination of the bone marrow. Of the two with presumed idiopathic thrombocytopenic purpura, one had Fanconi's anaemia and the other may have had aplastic anaemia. The third child had acute lymphoblastic leukaemia. The diagnosis of Fanconi's anaemia was delayed. A diagnostic and therapeutic dilemma was caused in the second case. In the third, delayed diagnosis and, perhaps, compromised outlook resulted. These three cases re‐emphasize the well aired caveats about the diagnosis of idiopathic thrombocytopenic purpura and juvenile rheumatoid arthritis and provide further support for the arguments of those who believe that if corticosteroids are to be used to treat such children, their bone marrow should be examined first.
Precision of gestational age assessment in the neonate
Authors: Gagliardi Luigi , Scimone Francesco , DelPrete Alberto , Petecca Cristina , Stival Giorgio , Pasinetti Giuliana , Teani Milena , Bianchi Paolo , Marani Miris , Duca PierGiorgio
Published: January 2008

The precision of the Ballard scale for assessing gestational age (GA) was evaluated in a consecutive sample of 227 preterm and/or low‐birth‐weight neonates. Each newborn was rated independently by two neonatologists and the difference in GA estimation between them was computed. The estimated precision was not high, the 95% tolerance interval estimate being as large as 7.4 weeks. The precision of the neurologic and physical parts of the scale was poorer than that of the complete scale (95% of differences less than 10.5 and 9.2 weeks respectively), and more influenced by the type of delivery. These findings are not unexpected from statistical theory, and cast doubts on the use of only the physical part of the Ballard scale in assessing GA, since greater accuracy could be accompanied by reduced precision.
Re‐evaluation of the whey protein/casein ratio of human milk
Authors: Kunz Clemens , Lönnerdal Bo
Published: January 2008

Total casein subunits as well as whey proteins were quantitated in human milk samples during lactation. Two independent methods were used: precipitation at pH 4.3 in the presence of Ca2+ followed by Kjeldahl analysis and polyacrylamide gradient gel electrophoresis (PAGGE) followed by densitometric scanning. Both methods yielded similar results: casein synthesis is low or absent in early lactation, then increases rapidly and subsequently decreases. The concentration of whey proteins decreases from early lactation and continues to fall. These changes result in a whey protein/casein ratio of about 90: 10 in early lactation, 60: 40 in mature milk and 50: 50 in late lactation. These observations indicate that the synthesis and/or secretion of caseins and whey proteins is regulated by different mechanisms. In addition, the relative proportion of the different β‐ and k‐casein subunits was found to vary throughout lactation.
Supplementation of an adapted formula with bovine lactoferrin: 1. Effect on the infant faecal flora
Authors: Roberts AK , Chierici R , Sawatzki G , Hill MJ , Volpato S , Vigi V
Published: January 2008

The development of the infant faecal flora was studied over the first three months of life in infants receiving breast milk, a modern adapted formula and adaptations of this formula. Breast‐fed infants developed a flora rich in Bifidobacterium sp. Facultative anaerobes were ubiquitous, but in relatively small numbers within the diet group. Other obligate anaerobes, such as Clostridium sp. and Bacteroides sp. were rarely isolated. Standard formula produced a flora rich in bifidobacteria, but the growth of facultative organisms was not suppressed by this diet. Clostridium sp. and Bacteroides sp. were more common in this feeding group. After the addition of lactoferrin at 10 mg/100 ml to the formula diet, a flora similar to that of the standard formula‐fed babies was achieved. Lactoferrin at 100 mg/100 ml was able to establish a “bifidus flora” in half of the babies given this formula, but only at age three months. Clostridium sp. and Bacteroides sp. were common faecal isolates from babies receiving both the lactoferrin diets.
Recombinant human granulocyte‐colony‐stimulating factor in the treatment of patients with chronic benign granulocytopenia and congenital agranulocytosis (Kostmann's syndrome)
Authors: Imashuku S , Tsuchida M , Sasaki M , Shimokawa T , Nakamura H , Matsuyama T , Taniguchi N , Oda M , Higuchi S , Ishimoto K , Kobayashi M , Ueda K , Tsukimoto I , Hanawa Y
Published: January 2008

Seven patients with chronic benign granulocytopenia and nine patients with congenital agranulocytosis, received consecutive seven‐day courses of recombinant human granulocyte‐colony stimulating factor at a starting dose of 50 μg/m2/day, subcutaneously. If there was no response the doses were increased to 300 μg/m2. All patients with chronic benign granulocytopenia responded rapidly at the minimum dose within 1–3 days after administration. By contrast, only three of the nine patients with congenital agranulocytosis responded within 1–7 days at this dose. Four patients with congenital agranulocytosis showed a response between days 7–19 at a dose of granulocyte‐colony‐stimulating factor 100‐200 μg/m2 but in the remaining two cases no response was obtained. The administration of granulocyte‐colony‐stimulating factor was shown to be safe and effective also in reducing infectious episodes in these patients. Previously it was reported that granulocyte‐colony‐stimulating factor 10–30 μg/kg/day was effective for patients with congenital agranulocytosis. These results indicate that patients with congenital agranulocytosis may require much higher doses of recombinant human granulocyte‐colony‐stimulating factor than patients with chronic benign granulocytopenia and that the response to ordinary doses of recombinant human granulocyte‐colony‐stimulating factor may be useful in differentiating between chronic benign granulocytopenia and congenital agranulocytosis.
Congenital dislocation of the hip: a prospective study comparing ultrasound and clinical examination
Authors: Rosendahl K , Markestad T , Lie RT
Published: January 2008

Screening for congenital dislocation of the hip by standard clinical methods and ultrasound was compared prospectively in 1503 newborns (1291 girls and 212 boys). A total of 82.8% of the hips (78.8% of the infants) had well developed acetabulae, 14.5% (17.2% of the infants) were shallow (immature) and 2.7% (4.1% of the infants) were dysplastic. Within 1‐3 months 96.7% of the infants with clinically stable, but immature hips normalized, while 3.3% did not improve or worsened, and therapy was therefore initiated. Indications for treatment included dysplastic and/or clinically unstable hips, and a higher number of newborns were treated during the study period than in 1982‐85 (31.2 vs 19.7 per 1000, p= 0.0002). Thirty‐seven percent of the patients had both clinical and ultrasound findings compatible with congenital dislocation of the hip, while the decision to treat was based on clinical findings alone in 25.0% and on ultrasound findings in 38.0%. Infants born during the study period of nine months had a low incidence of late congenital dislocation of the hip compared with our earlier reported results from 1982‐85 (0.9 vs 3.5 per 1000, p= 0.012). The study demonstrated major discrepancies between clinical and ultrasound evaluation of hips in the newborn, and the role of ultrasound in the screening for congenital disclocation of the hip requires further evaluation.
Reference intervals and developmental changes in urinary prostanoid excretion in healthy newborns, infants and children
Authors: Leonhardt A , Busch C , Schweer H , Seyberth HW
Published: January 2008

Urinary excretion of prostaglandins E2, F2α, E‐M (7α‐hydroxy‐5, 11‐diketotetranor‐prosta‐1, 16‐dioic acid), 6‐keto F1α, 2,3‐dinor‐6‐keto‐F1α, thromboxane B2, 2,3‐dinor‐thromboxane B2 and 11‐dehydro‐thromboxane B2 was determined by gas chromatography‐mass spectrometry in 83 healthy subjects aged one day to 37 years. The excretion rates of all prostanoids increased with advancing age. After correction for 1.73 m2 body surface area, only urinary excretion rates of prostaglandins E‐M and 6‐keto‐prostaglandin F1α depended on age. Reference intervals were calculated as the 10th and 90th percentiles for prostaglandins E2 (4‐27 ng/h/1.73 m2), F2α (23‐87 ng/h/1.73 m2), 2,3‐dinor‐6‐keto‐F1α (4‐19 ng/h/1.73 m2), thromboxane B2 (1‐21 ng/h/1.73 m2), 2,3‐dinor‐thromboxane B2 (8‐36 ng/h/1.73 m2) and 11‐dehydro‐thromboxane B2 (15‐87 ng/h/1.73 m2) in all subjects, and for prostaglandins E‐M and 6‐keto‐prostaglandin F1α in subjects aged 30 days or less (110‐1140 ng/h/1.73 m2 and 7‐23 ng/h/1.73 m2) and older than 30 days (62‐482 ng/h/1.73 m2 and 2‐12 ng/h/1.73 m2). High urinary excretion of prostaglandins E‐M and 6‐keto‐F1α during the newborn period and some distinct changes in urinary excretion of prostaglandin E2 and thromboxane B2 with advancing age suggest that these prostanoids might play a specific role during child development.
Modification of some markers of inflammation during treatment for acute respiratory exacerbation in cystic fibrosis
Authors: Valletta EA , Rigo A , Bonazzi L , Zanolla L , Mastella G
Published: January 2008

An objective approach for monitoring the treatment of acure pulmonary exacerbation in cystic fibrosis was evaluated. Eleven biochemical markers of inflammation (erythrocyte sedimentation rate, neutrophil count, C‐reactive protein, α‐I antitrypsin, haptoglobin, ceruloplasmin, fibronectin, α‐l glycoprotein, α‐2 macroglobulin, C3, granulocyte elastase and anti‐Pseudomonas IgG) were measured in blood serum and plasma from 46 cystic fibrosis patients with chronic Pseudomonas aeruginosa colonization before and after treatment. The overall outcome in each patient was evaluated by means of a pondered sum of clinical, chest X‐ray and lung function scores. Biochemical markers were related to the overall clinical improvement: haptoglobin, ceruloplasmin, fibronectin and α‐I glycoprotein showed a good sensitivity (64‐70%), specificity (60‐70%) and positive predictive value (86‐89%). Granulocyte elastase showed a similar sensitivity (67%) and positive predictive value (85%) but a lower specificity (33%). The negative predictive value was generally poor (32‐39%). Our data suggest that the combined measurement of some markers of inflammation and of conventional clinical parameters, may help in evaluating the efficacy of anti‐infective treatment in cystic fibrosis.
Health examination of preschool children in relation to school adjustment in grade I
Authors: Bondestam M , Hagelin E , Rydell A‐M , Westerlund M
Published: January 2008

The results of a collaborative study on health examination in preschool children, using data from child health care, preschool establishments and a teacher evaluation carried out six months after the start of school were compared. The analysis showed that many children with difficulties in motor function, behaviour and/or learning in grade I had been identified at the health examination. The results emphasize the value of collaboration between preschool establishments, child health care centres and schools for identification of children at risk of developing early adjustment problems.
The growth of low‐birth‐weight children
Authors: Elliman Alison , Bryan Elizabeth , Elliman Anthony , Walker Jean , Harvey David
Published: January 2008

The growth of 171 seven‐year‐old children, free from major disability, with a birth weight of 2000 g or less was examined and compared with that of their own parents and of normal‐birth‐weight peers. Measurements included height, weight, occipitofrontal circumference, biparietal, occipitofrontal and bi‐iliac diameters, triceps and subscapular skinfold thickness. Low‐birth‐weight children were shorter than their parents whereas those of normal birth weight were taller. For all parameters, particularly weight, the low‐birth‐weight children were significantly less well grown. There was no significant relationship between occipitofrontal circumference and intellectual ability.
Psychosocial growth failure: a positive response to growth hormone and placebo
Authors: Boulton TJC , Smith R , Single T
Published: January 2008

Seven children were diagnosed as having an emotional cause for growth failure. Pretreatment growth hormone secretion profiles during sleep were analysed using PULSAR. Mean (± SD) growth hormone concentration was 10.9 (4.4) mU/1, mean peak 19.6 (6.7) mU/1 and the peak‐to‐peak interval 147 (108) min. Mean (SEM) IGF‐1 was 1.08 (0.31). The seven children received a six‐month course of recombinant growth hormone in a double‐blind, crossover study using a dose of 1.2 U/kg/week (28 U/ m2/week). Daily placebo injections were given for the other six‐month epoch, with a one month washout period. The mean (SEM) growth velocity SD score after growth hormone administration was +4.66 (1.88) and after placebo ‐0.60 (0.69), each value being greater than the pretreatment value of ‐2.32 (0.122) (p < 0.0001 on analysis of variance). The change in IGF‐1 during growth hormone treatment was not significant. No significant changes in food energy or protein intake occurred.
Effect of phenylpropanolamine on incontinence in children with neurogenic bladders. A double‐blind crossover study
Authors: Åmark P , Beck O
Published: January 2008

In children with myelodysplasia and a low‐level spinal cord lesion (low lumbar‐sacral), detrusor hyperactivity together with dyssynergic urethral function forms the main pathophysiological basis for incontinence. Pharmacological treatment of incontinence due to neurogenic bladder dysfunction has been tried, mainly with anticholinergics and alpha‐adrenoceptor antagonists. In this study, the effects of the alpha‐adrenoceptor agonist phenylpropanolamine on urodynamic parameters and incontinence were investigated in 10 patients. Effects on incontinence were evaluated in a double‐blind crossover trial. Plasma concentrations of phenylpropanolamine were measured by means of gas chromatography‐mass spectrometry. Phenylpropanolamine reduced detrusor hyperactivity and improved continence, but the effect was not so pronounced as to make the patients continent.
Immunogenicity and immunomodulatory activity of bovine surfactant (SF‐RI 1)
Authors: Bartmann P , Bamberger U , Pohlandt F , Gortner L
Published: January 2008

Respiratory distress syndrome in preterm infants can be treated successfully by endotracheal administration of a bovine surfactant preparation (SF‐RI 1). Before the routine use of xenogenic surfactant preparations can be recommended, their immunogenicity as well as their in‐vivo and in‐vitro immunomodulatory activity have to be investigated. High titers of anti‐surfactant antibodies were detected by a sensitive ELISA after immunizing rats, rabbits and mice with SF‐RI 1. Repeated endotracheal administration of SF‐RI 1 resulted in a humoral antibody response in three out of eight rabbits. After treatment of 34 preterm infants with SF‐RI 1 (50‐200 mg/kg), a humoral immune response to SF‐RI 1 could not be detected. In‐vitro restimulation of peripheral blood lymphocytes with SF‐RI 1 after primary in‐vivo administration did not result in cell proliferation as measured by 3H‐thymidine incorporation. SF‐RI 1 did not stimulate peripheral blood lymphocytes of neonates in vitro. The mitogenic response of these cells to stimulation with PHA, ConA or PWM was heavily impaired in the presence of SF‐RI 1 concentrations increasing from 0.04 to 4 mg/ml. These data indicate that SF‐RI 1 is immunogenic and that it may have an influence on lymphocyte proliferation in vivo.
Growing up in Uppsala. Part II. Could adolescents with severe psychosocial problems have been identified by symptoms observed in school at age 10 years?
Authors: Mellbin T , Sundelin C , Vuille J‐C
Published: January 2008

The study comprised all 1715 children born in 1965 and resident in Uppsala at age 10 and 18 years. Data were collected through teacher interviews and analysis of school health records in grade 3 at the age of 10 years. The psychosocial burden up to the age of 18 years was assessed on the basis of all registered contacts with official institutions outside school (authorities for care of the handicapped, Department of Child Psychiatry, social agencies, legal authorities). Approximately 12% of the adolescents were clearly in a situation of manifest psychosocial risk on the threshold of adult life. These adolescents were assigned to five mutually exclusive problem groups comprising different sex distribution, symptoms and utilization of institutional care. The analysis of the relationship between data from grade 3 and the psychosocial burden up to 18 years of age showed that the information available to the school did not permit reasonably secure predictions of the child's psychosocial situation at the end of adolescence. Observations in school of pre‐adolescent children cannot be used as a basis for risk‐group strategies aiming at concentrating early treatment measures and resources to a restricted number of children at risk. However, the prognosis is apparently serious for a limited number of 10‐year‐olds with serious problems in school.
Recurrent nodular haemangiomas in Klippel‐Tréaunay syndrome
Authors: Illum Niels , Nielsen Henrik Winther , Skov Birgit Guldhammer
Published: January 2008

A one‐year‐old child had hypertrophy of the left leg and an unusual constellation of a naevus flammeus and superficial enlarged veins of the trunk together with successive appearance and involution since birth of numerous nodular elements located in the naevus and in the surrounding normal skin. Microscopic examination of these elements showed haemangiomas with capillaries, cavernous channels and lymphangiomatous components. The benign nature of transient nodular elements located to the trunk and the lack of associated visceral vascular malformations in the Klippel‐Trénaunay syndrome are documented.
The primary empty‐sella syndrome and diabetes insipidus in a child
Authors: Hung Wellington , Fitz Charles R
Published: January 2008

The empty‐sella syndrome is uncommon in pediatric patients. Hypothalamic‐pituitary dysfunction is common in these patients but involvement of the posterior pituitary gland is very rare. We report a seven‐year‐old girl with empty‐sella syndrome who first developed arginine‐vasopressin deficient diabetes insipidus and then anterior pituitary gland deficiency. The empty‐sella syndrome should be included among the causes of arginine‐vasopressin deficient diabetes insipidus in pediatric patients.
Breast feeding and the dietary habits of children in rural Somalia
Authors: Ibrahim MM , Persson LÅ , Omar MM , Wall S
Published: January 2008

Breast feeding and dietary habits were studied prospectively in a cohort of children under the age of five years in a rural Somali community. The median duration of breast feeding was 19.5 months. However, all the children also received cow's milk by cup from the first day of life and onwards. Energy supplements (mainly sugar and oil) as well as additional water were given daily from early infancy. Staples, protein‐rich foods (beans and meat), vegetables and fruits were usually introduced when the children reached the age of 12‐18 months. There was a seasonal variation with the lowest intake of protein‐rich and vitamin‐rich foods during the rains in May to June. Thus, there was a complete absence of exclusive breast feeding. Energy‐reinforced cow's milk and human milk dominated the diet up to the age of one year. Staples were mixed with oil and supplemented with milk, thereby leading to a much higher energy density in the complementary food than is usually the case in African communities.
Cardiovascular effects of carbon dioxide in ventilated preterm infants
Authors: Fenton AC , Woods KL , Leanage R , Abu‐Harb M , Levene MI , Evans DH , Field DJ
Published: January 2008

Sick preterm infants may, under certain conditions, demonstrate blood pressure passive cerebral blood flow in response to changes in arterial carbon dioxide tension. Blood pressure in turn depends on cardiac output and peripheral resistance. A Doppler technique for assessing cardiac output compared favourably in terms of reproducibility to a thermodilution technique in a group of infants undergoing cardiac catheterization for congenital heart disease. Doppler was subsequently used to monitor changes in cardiac output following an increase in arterial carbon dioxide tension of 1 kPa in 25 ventilated preterm infants. Blood pressure increased significantly (p= 0.006). However, heart rate did not change significantly (p= 0.16) and, in addition, both stroke and minute volume decreased (p= 0.023, p= 0.02, respectively). This suggests that accompanying changes in components of peripheral resistance exert important effects on blood pressure in the preterm neonate in response to changes in arterial carbon dioxide tension.
Chronic active hepatitis with renal tubular acidosis presenting as hypokalemic periodic paralysis with respiratory failure
Authors: Koul PA , Saleem SM
Published: January 2008

A child with chronic active hepatitis and renal tubular acidosis, presenting with periodic hypokalemic paralysis resulting in respiratory failure, is described.
The status of lactose absorption in Hong Kong Chinese children
Authors: Tadesse K , Leung DTY , Yuen RCF
Published: January 2008

Lactose malabsorption was investigated in 169 Chinese children aged between two and 16 years using the breath hydrogen test. The challenge was either lactose solution (1 g/kg) or cow's milk (10 ml/kg). Overall, 68% of the children showed a significant increase in breath hydrogen following the lactose challenge while only 17% showed an increase after the cow's milk challenge and 13% after both challenges. The number of malabsorbers increased significantly (p < 0.001) with age and no associated gastrointestinal symptoms or signs were found in any of the children following the challenges, suggesting a gradual and partial loss of intestinal lactase activity. We conclude that the prevalence of lactose malabsorption in Hong Kong children is very high using the standard lactose tolerance test but when a more realistic amount of lactose and a natural medium such as a glass of milk is used as the challenge, the number of malabsorbers becomes small and clinically insignificant.
Neuroradiological findings in children with congenital myotonic dystrophy
Authors: Tanabe Y , Iai M , Tamai K , Fujimoto N , Sugita K
Published: January 2008

We studied seven children with congenital myotonic dystrophy, aged 2.1–8.3 years, and the results of computed tomography and magnetic resonance imaging of the brain were analyzed and neurological development was assessed from the neonatal period. We found that ventricular dilatation that had been seen on the first day of life in two of three infants had not progressed in sequential follow‐up computed tomography scans taken at intervals of one to six years. Also, in T2‐weighted magnetic resonance imagings, areas of periventricular hyperintensity were identified in all children, as well as areas of subcortical hyperintensity in one child. Further, an asphyxial episode had occurred at birth in five patients and the extent of the periventricular hyperintensity was found to correlate significantly with Apgar scores, indicating that the degree of perinatal asphyxia that had occurred was responsible for the abnormalities uncovered by the magnetic resonance imagings. However, there was no correlation between the neurodevelopmental outcome and the extent of the periventricular hyperintensity or ventriculomegaly. Therefore, in patients with congenital myotonic dystrophy, a neonatal episode of asphyxia can be responsible for a finding of periventricular hyperintensity, but it is unlikely that an integral part of the mental retardation is attributable to brain damage due to perinatal asphyxia.
Phenytoin‐induced IgG2 and IgG4 deficiencies in a patient with epilepsy
Authors: Ishizaka A , Nakanishi M , Kasahara E , Mizutani K , Sakiyama Y , Matsumoto S
Published: January 2008

A five‐year‐old girl with epilepsy and recurrent respiratory infections was investigated for serum IgG subclass concentrations. She was diagnosed as having a combined deficiency of IgG2 and IgG4 with a decreased serum concentration of IgA and IgG3 and was given replacement therapy with iv immunoglobulins. Since then, she has been free from respiratory infections. After phenytoin therapy was stopped, IgG subclass deficiency improved. This case describes the further action of phenytoin on the immune system, adding IgG subclass deficiency to the list.
Congenital sideroblastic anaemia with intrauterine symptoms and early lethal outcome
Authors: Andersen K , Kaad PH
Published: January 2008

Sideroblastic anaemia is a rare disease, which most often presents in early childhood. A case of ringed sideroblastic anaemia with onset in early foetal life in a female infant, resulting in severe intrauterine symptoms, is reported. Six weeks after birth a bone marrow examination revealed a large amount of typical ringed sideroblasts, thus establishing the diagnosis. In spite of repeated blood transfusions, the haemoglobin content gradually decreased. The condition was refractory to pyridoxal phosphate treatment and continued to deteriorate with lethal outcome four months after birth.
Neonatal polycythemia in appropriately grown infants of hypertensive mothers
Authors: Kurlat I , Sola A
Published: January 2008

Neonatal polycythemia is a frequent finding after pregnancies complicated by diabetes and by maternal hypertension with intrauterine growth retardation (IUGR). It is still unclear if the association of polycythemia with hypertension is the result of IUGR or of hypertension per se. To establish the incidence of neonatal polycythemia in populations at risk, we analyzed the results of hematocrit values obtained from 1592 neonates born consecutively at the Hospital de Clinicas, Buenos Aires. Capillary hematocrit values were obtained by heel stick before 6 h of age. When the values were 65% or greater, new samples were obtained from an antecubital vein. The risk of polycythemia in appropriately grown infants of hypertensive mothers was 12.6‐fold greater than the risk in the general population. These data show that maternal hypertension poses a significant risk for polycythemia, regardless of fetal growth. We suggest that, to prevent possible sequela, hematocrit is measured routinely in all infants of hypertensive mothers for prompt diagnosis and treatment.
Treatment of hypercholesterolemia in children and adolescents
Authors: Koletzko Berthold , Kupke Ingeborg , Wendel Udo
Published: January 2008

In patients with severe genetic hypercholesterolemia, therapeutic reduction of elevated serum total cholesterol and LDL cholesterol should begin in early childhood to lower the risks of cardiovascular disease later in life. We evaluated the effects of outpatient therapy with diet alone and with combined diet and drug therapy in children and adolescents with hypercholesterolemia of apparent dominant inheritance. Serum lipid values before and during dietary treatment were available in 35 patients (mean age at start of treatment 7.9 years, range 2.0–17.6 years) followed for an average duration of 17.5 months (range 4–70 months). A comparison between untreated state and combined therapy with diet and cholestyramine was possible in 14 patients (mean age 8.6 years, range 2.4–17.0 years) followed for 27.9 months (range 4–97 months). Dietary modification achieved by repeated counseling and training lowered serum total cholesterol by mean (± SE) 11.7 ± 1.9% (p < 0.0001) and LDL cholesterol by 17.3 ± 3.5% (p < 0.0001). However, five of 35 patients did not show an appreciable effect of therapy (cholesterol reduction < 5%), possibly because of non‐compliance. Diet combined with cholestryramine in an average dose of 0.36 g/kg body weight/day reduced total cholesterol by 33.0 ± 2.4% (p < 0.0001) and LDL cholesterol by 37.5 ± 4.3% (p < 0.0001) and was effective in all patients. Both forms of treatment had no effect on serum triglycerides and HLD cholesterol. No serious side effects were noted, and percentile values for weight and height remained unchanged in all but three obese children. We conclude that age‐adjusted dietary and drug treatment is acceptable and effective in children and adolescents with hypercholesterolemia and consequently should be applied in this patient group.
Ganciclovir therapy of congenital cytomegalovirus disease
Authors: Reigstad Hallvard , Bjerknes Robert , Markestad Trond , Myrmel Helge
Published: January 2008

A newborn with cytomegalovirus disease with cerebral involvement was treated with ganciclovir for 21 days. The treatment resulted in only temporary cessation of virus shedding in the urine and probably had no ameliorating effect on the long‐term clinical outcome.
Intracardiac rhabdomyomas in neonates: report of three cases
Authors: Björkhem G , Lundström N‐R , Lingman G
Published: January 2008

Three cases of intracardiac rhabdomyomas in neonates are presented. All were diagnosed by ultrasound, one prenatally and two directly after birth. Two of the patients were operated on during the neonatal period. The operations were carried out on the ultrasound findings alone. One patient was considered inoperable and died at three weeks of age. The two surviving patients have no cardiac symptoms and involution of the tumors has taken place. Two of the three patients have shown signs of tuberous sclerosis.
Shoshin beriberi in an infant of a thiamine‐deficient mother
Authors: Debuse Peter J
Published: January 2008

The classical form of thiamine deficiency in children is comprised of peripheral neuropathy, encephalopathy and high‐output cardiac failure, predominantly right‐sided. “Shoshin beriberi” cardiac failure has a different presentation, with vasoconstriction, hypotension and severe metabolic acidosis. A three‐month breast‐fed infant developed these features (biochemical tests confirmed the diagnosis). His mother, although non‐symptomatic, had biochemical evidence of thiamine deficiency.
Hospital care techniques resulting in intact survival of a 380‐g infant
Authors: Ginsberg Harley G , Goldsmith Jay P , Stedman Charles M
Published: January 2008

Intact survival of infants delivered before completion of the 26th week of gestation or weighing less than 500 g is a well known phenomenon. We recently cared for an infant whose birth weight was 380 g, making her one of the smallest survivors in the United States. Her hospitalization (including expenses), the techniques of our minimal intervention protocol and her 20‐month (corrected) follow‐up are presented together with a discussion of the moral, economic and social implications involved in the care of such an infant.
Mortality associated with acute watery diarrhea, dysentery and persistent diarrhea in rural North India
Authors: Bhandari N , Bhan MK , Sazawal S
Published: January 2008

Mortality associated with diarrhea was investigated in a longitudinally followed cohort of children under six years of age in rural North India. During the follow‐up, 1663 episodes of diarrhea and 23 diarrhea related deaths were recorded in 1467 children followed up for 20 months. The case fatality rate was 0.56% for acute watery diarrhea, 4.27% for dysentery and 11.94% for non‐dysenteric persistent diarrhea. Most of the episodes lasted less than a week; 5.2% became persistent (duration > 14 days). The case fatality rate was similar in episodes of one and two weeks’duration (0.64% and 0.8%) and increased to 13.95% for persistent episodes. Of the total 86 persistent episodes, 22.1% were dysenteric; the case fatality rate for such dysenteric persistent episodes was 21.1% and for watery persistent diarrhea 11.4%. Diarrheal attack rates were similar among different nutritional groups, but diarrheal case fatality rates progressively increased with increasing severity of malnutrition, these were 24 times higher in children with severe malnutrition (7.48%) compared to those normally nourished (0.31%). With availability and use of oral rehydration therapy, dysentery and persistent diarrhea emerge as major causes of diarrhea related mortality, with underlying malnutrition as a key associated factor.
Persistent diarrhea associated with AIDS
Authors: Keusch GT , Thea DM , Kamenga M , Kakanda K , Mbala M , Brown C , Davachi F
Published: January 2008

Chronic diarrhea and wasting are very common manifestations of AIDS in adults in developing countries. Etiologic studies show that protozoa (including Cryptosporidium parvum, Isospora belli, and Enterocytozoon bieniusi) and Mycobacterium avium‐intracellulara are the most frequently identified pathogens. Limited data in children suggest that common enteric pathogens are equally as likely in HIV+ and HIV‐ babies. Preliminary analysis of an ongoing longitudinal study of 469 babies born to mothers with known HIV serostatus in Kinshasa, Zaire, reveals progression of acute to persistent diarrhea is six times greater in HIV+ compared to HIV‐ babies, and 3.5 times greater in HIV‐ babies born of HIV+ mothers in comparison to HIV‐ babies with HIV‐ mothers. HIV+ babies were also at greater risk than HIV‐ babies to have recurrent episodes of diarrhea (RR = 2.3). Fifty percent of the deaths were due to acute or persistent diarrhea, and were strongly associated with HIV infection. Efforts to improve child survival in AIDS infected populations will need to address HIV infections in both mothers and infants.
Household behaviors in the management of diarrhea and their relevance for persistent diarrhea
Authors: Bentley Margaret E
Published: January 2008

There has been a great deal of research in recent years on household behaviors related to diarrhea management. Most of the available data on household diarrhea management, however, pertains to acute diarrheal episodes. There is a dearth of knowledge concerning household and caretaker behaviors when the diarrhea is of longer duration. This paper briefly reviews some of what has been learned about household behaviors in the management of acute diarrhea and discusses its relevance for persistent diarrhea. Based upon what has been learned from anthropological studies of acute diarrhea and the little that is known about caretaker behavior during persistent diarrhea, a hypothetical model of the interactions among household behavior and characteristics of diarrheal episodes is presented. The model argues that maternal (or caretaker) concern increases with diarrheal duration, and that changes in behavior, both adaptive and maladaptive, are more likely to occur during an episode of persistent diarrhea, compared to acute. In some cases, these actions may directly influence the outcome of the episode. There is a need to better understand household case management behaviors through the continuum of diarrheal duration and the effect of these behavioral factors on episode outcome. This information is necessary in order to communicate effective messages to caretakers about what they should know and what they should do when persistent diarrhea occurs.
Small intestinal mucosal histology in the syndrome of persistent diarrhoea and malnutrition: a review
Authors: Sullivan Peter B , Marsh Michael N
Published: January 2008

Prolonged injury to the small intestinal mucosa is probably the final common pathway by which a variety of noxious influences—nutritional, infective and possibly allergic—perpetuate the syndrome of persistent diarrhoea in children in developing countries. Animal studies have helped to separate the individual effects of malnutrition and diarrhoea on the gut in a way that is not possible in the clinical situation. Early studies in children provided somewhat subjective or semi‐quantitative data on intestinal morphology. More recently, the application of computer‐assisted quantitative morphological techniques to intestinal mucosae from children with persistent diarrhoea have revealed a spectrum of changes consistent with a cell‐mediated immune form of damage. The nature of the antigens that provoke these responses remains to be elucidated. Several reports indicate that in children with persistent diarrhoea clinical severity and prognosis do not necessarily correlate with the degree of small intestinal mucosal damage. Nutritional rehabilitation can be shown to produce a demonstrable improvement in small intestinal crypt cell proliferative activity in children with persistent diarrhoea. It is not yet known for how long nutritional rehabilitation should be continued to ensure complete recovery of the intestinal damage following persistent diarrhoea.
Dietary protein sensitivity: is it an important risk factor for persistent diarrhea?
Authors: Snyder John D
Published: January 2008

Although the causes of persistent diarrhea are largely unknown, dietary protein sensitivity has been identified as one possible mechanism. Since nutritional therapy appears to be an essential component of treatment for persistent diarrhea, a clearer understanding of the importance of dietary protein allergy is needed to provide optimal dietary recommendations. This paper reviews the current concepts of the pathophysiology, clinical features and criteria for diagnosis of dietary protein sensitivity. Evaluation of the results of several clinical studies indicates that dietary protein sensitivity does occur especially in young infants fed cows’milk but that it is an uncommon event, especially in older infants and children.
Nutritional management of persistent diarrhea: factors predicting clinical outcome
Authors: Bhutta Zulfiqar A , Molla Abdul M , Issani Zeenat , Badruddin Salma , Hendricks Kristy , Snyder John D
Published: January 2008

In a prospective study we randomized 102 male children (age 6‐36 months) with persistent diarrhea to receive a traditional rice‐lentil (Khitchri) diet and yogurt (K‐Y) or a soy formula (alone for seven days and then in combination with K‐Y for seven days). Of 73 children satisfying the study criteria, 66 were successfully treated and there were 7 treatment failures. There was poor concordance between parental accounts of severity of diarrhea or vomiting and that observed after admission. Significant risk factors associated with treatment failure included younger age (p<0.005) and vomiting at presentation (p<0.02). The greatest number of risk factors associated with delayed recovery (<10 days) were identified during an initial evaluation period (the first 8 h after admission). These included greater severity of watery diarrhoea (p<0.01) and increased ORS intake (p<0.02). Our data suggest that an initial evaluation period, including objective observations, may identify children with persistent diarrhea who are at greatest need of hospitalization.
Near‐infrared interactance in assessing superficial body fat in exclusively breast‐fed, full‐term neonates
Authors: Kasa N , Heinonen KM
Published: January 2008

Near‐infared interactance was used to study changes in the percentage of superficial body fat in 91 healthy, breast‐fed, full‐term neonates during the first five days of life (group 1) and subsequently at two and four weeks of age in 28 infants (group 2). Near‐infrared interactance was recorded as optical densities (ODs) at 937 nm and 947 nm. OD ratio agreed acceptably with fat content determined chemically in 16 pork meat samples. No correlation between OD ratio and skinfold thickness (measured at the same site in the subscapular area) was found. In group 1, no change was found in OD ratio, and maximum weight loss (at two days of age) was only 3%. In group 2, percentage of superficial body fat (estimated via OD ratios) increased by only 2%, despite normal weight gain (26% increase from birth weight). Breast feeding thus maintains adequate weight gain without accelerating superficial fat accumulation during the neonatal period.
Bilirubin bound to cells does not form photoisomers
Authors: Christensen T , Kinn G
Published: January 2008

Cultured cells from one human and one murine cell line were incubated with bilirubin by different methods that allowed bilirubin to be bound to cells. The cells were irradiated with visible light of different wavelengths. Bilirubin bound to human serum albumin was also irradiated with light. After irradiation, bilirubin and its photoisomers were extracted and analyzed by HPLC. No photoisomers were found in samples of irradiated cells, while the types and amounts of photoisomers that were expected from the literature were found in samples of irradiated bilirubin/albumin mixtures. We conclude that the formation of therapeutically active photoisomers during phototherapy most probably does not take place in skin cells, but most likely in bilirubin bound to albumin in the vessels or in the interstitial space.
Towards an equal distribution of health? Socioeconomic and regional differences of the secular trend of the age of menarche in Finland from 1979 to 1989
Authors: Rimpelä AH , Rimpelä MK
Published: January 2008

The purpose of this study was to investigate if a secular trend towards an earlier age of menarche still existed in Finland in the 1980s and if social class and regional differences observed previously in the mean age of menarche had disappeared. Questionnaires were mailed to nationwide representative samples of 16‐ and 18‐year‐old girls every other year from 1979 to 1989. At the national level, the secular trend towards an earlier menarche was not observed in the 1980s but the trend was significant among girls living in the North‐West and rural areas. Clearly observed regional and urban‐rural differences in 1979 disappeared in the 1980s. Social class differences persisted: farmers' daughters had a higher mean age of menarche than those of other occupational groups. Adolescents in the 1980s displayed a more even distribution of health than the cohorts born before them. Improved welfare of the Finnish society and reorganization of the primary health care are probable explanations.
Renal function in hyperparathyroidism with complicating nephrocalcinosis
Authors: Eaton DG Murdoch , Hewitt CAH
Published: January 2008

Primary hyperparathyroidism in childhood is rare. Long‐standing hypercalcaemia is reported to result in severe and irreversible renal damage. However, renal complications of hyperparathyroidism, particularly nephrocalcinosis are uncommon. We report a 12‐year‐old girl presenting after three years with extensive nephrocalcinosis and with rapid recovery of renal function.
Kinetics of hypogammaglobulinemia in patients with common variable immunodeficiency
Authors: Kato Yoshihiro , Kondo Naomi , Motoyoshi Fumiaki , Ozawa Takeshi , Agata Hiroatsu , Orii Tadao
Published: January 2008

We investigated the production of immunoglobulin (Ig) in six patients with common variable immunodeficiency and analyzed the courses of their levels of serum Ig for a period of 8–13 years. In all patients, levels of serum IgM, IgG and IgA were markedly low at the first examination, except for the IgM level in one patient. Improvement of serum Ig levels was observed in the patients in whom Ig production of non‐T cells at the first examination and Cμ gene expression had been detected to a slight degree, but serum Ig levels remained low in the patients in whom Ig production of non‐T cells and Cμ gene expression had not been detected. Our results suggest that some hypogammaglobulinemia in common variable immunodeficiency develops or improves with age.
Hereditary properdin deficiency in three families of Tunisian Jews
Authors: Schlesinger M , Mashal U , Levy J , Fishelson Z
Published: January 2008

Hereditary properdin deficiency is a rare genetic disorder of the complement system. Three propositi and six additional family members with properdin deficiency have been found following analysis of the hemolytic activity of the classical (CH50) and the alternative (AP50) complement pathways in the sera of 101 survivors of meningococcal infections and 59 survivors of severe pneumococcal and Haemophilus influenza infections. All the properdin‐deficient individuals had undetectable levels of properdin by radial immunodiffusion and by Western blotting. They belonged to three non‐related families of Tunisian Jews who came from different parts of Tunisia. Two patients had a meningococcal infection at 15 and 16 years of age, respectively, and one had Haemophilus influenza meningitis at 1.5 years of age. In contrast to the fulminant and fatal course of meningococcal infection which was previously described in some properdin‐deficient patients, our patients had a relatively mild disease. Properdin deficiency may not be as rare as previously thought. Analysis of AP50, in addition to CH50, in sera of patients who had meningococcal infection, will probably disclose many more cases of hereditary properdin deficiency. In addition, our findings indicate that, as in other complement abnormalities, hereditary properdin deficiency may also be associated with the ethnic origin of the patient.
Severe hyperlipoproteinemia in congenital nephrotic syndrome of the Finnish type: effect of dialysis and kidney transplantation
Authors: Salo Matti K , Jaakkola Olli , Solakivi Tiina , Ylä‐Herttuala Seppo
Published: January 2008

Two children with congenital nephrosis of the Finnish type were studied successively at the three stages of the disease: (A) nephrosis, (B) renal insufficiency/peritoneal dialysis and (C) post‐transplantation; two additional patients were studied at two stages. Plasma lipoprotein profiles were determined by density gradient ultracentrifugation and lipids by enzymatic methods. Stage A was characterized by hyperchylomicronemia, low high density lipoprotein (HDL) cholesterol and the presence of dense low density lipoprotein (LDL) and HDL particles. Total cholesterol and triglycerides showed great daily variation (5–14 and 5–33 mmol/l, respectively). During stage B, hyperlipidemia weakened. Yet HDL concentration remained low and the concentration of intermediate density lipoproteins (IDL) increased. At stage C, hyperlipidemia had almost subsided, but the presence of IDL persisted. In conclusion, severe hyperlipoproteinemia of congenital nephrosis at the nephrotic stage is attenuated during renal insufficiency and dialysis, and essentially normalizes after kidney transplantation. Yet the presence of IDL implies an increased risk of atherosclerosis.