bluuebunny/crossref_metadata_2025 huggingface dataset:
Biosynthetic Human Growth Hormone in the Treatment of Growth Hormone Deficiency
Authors: HOLCOMBE J.H. , CONFORTI P.M. , WONG A.C. , THOMPSON R.G. , DRAPER M.W.
Published: January 2008

ABSTRACT. A total of 309 previously untreated children with growth hormone deficiency (GHD) (219 boys, 90 girls; mean age 8.4 ± 3.9 years, range 1.5‐19 years) were treated for up to 3 years in an ongoing trial designed to examine the long‐term efficacy and safety of biosynthetic somatropin (rhGH). The children were treated with rhGH, 0.06 mg/kg (0.16 IU/kg) three times weekly. In the prepubertal children, the mean height velocity increased during the first year from 3.8 ± 1.8 cm/year to 8.9 ± 2.2 cm/year (n = 188). During the second and third years, their height velocities were 7.1 ± 1.1 (n = 147) and 6.3 ± 1.2 cm/year (n ‐ 64), respectively. The height velocity SDS increased from ‐2.5 ± 1.9 before treatment to 3.1 ± 2.6 during the first year of treatment in the prepubertal children. The mean pretreatment height velociity in those with idiopathic GHD (3.8 ± 1.6 cm/year) did not differ from that in children with organic GHD (3.8 ± 2.3 cm/year). In addition, the height velocities during the first year of therapy did not differ significantly with respect to the aetiology of GHD. For the children who entered puberty during the study, the mean height velocity increased from 3.0 ± 1.7 cm/year before treatment to 8.4 ± 2.3 cm/year during the first year of rhGH therapy. In the first year, the height velocity of children with a bone age of less than 5 years (9.4 ± 2.3 cm/year) was significantly greater than that in children with a bone age of 5‐10 years (8.4 ± 1.8 cm/year) or greater than 10 years (7.8 ± 2.2 cm/year: p= 0.001). However, the increase in height velocity above the pretreatment level did not differ between bone age groups (p= 0.13). During the study, the height SDS improved from ‐3.4 ± 1.4 before treatment to ‐2.1 ± 1.1 by the end of year 3. At the start of rhGH therapy, only 38 out of 309 (12.3%) children had heights above ‐2 SD, but by the end of the 3 years, 44 out of 99 children (44.4%) had heights above that level. Antibodies to GH were detected in 8 out of 304 children (2.6%), but none of these children showed attenuated growth as a result. The results of this ongoing study confirm that rhGH is a safe and effective therapy in children with GHD.
Anal Tonometry in the Neonatal Period for the Diagnosis of Hirschsprung's Disease
Authors: VERDER H. , PETERSEN W. , MAURITZEN K.
Published: January 2008

ABSTRACT. A double balloon technique was used for anal tonometry in 22 infants. The weight was less than 2500 g in 10 of the infants. Ileus or delayed passage of meconium was seen in 9 infants. Thirteen infants had not revealed any symptoms of intestinal obstruction during the first week of life. In all cases, except one, it was possible to demonstrate reflexes from the internal anal sphincter. In 18 infants normal curves were registered from the internal anal sphincter. None of these developed Hirschsprung's disease. In 3 infants a pathological pattern of contractions of the internal anal sphincter was demonstrated. Two of them proved to have Hirschsprung's disease. In the third case normal reflexes were registered after one year. Possibly the innervation of rectum and the internal anal sphincter was disrupted during the neonatal period in connection with enterocolitis and ileus, thus representing a reversible case of achalasia.
Cardiovascular Effects of Acute Bronchiolitis
Authors: SREERAM N. , WATSON J. G. , HUNTER S.
Published: January 2008

ABSTRACT. Twenty‐one children with normal hearts were studied during acute bronchiolitis. Doppler echocardiography showed tricuspid valve regurgitation in 11 patients, many of whom had evidence of raised pulmonary artery systolic pressure. Serial studies in those with severe infection showed that tricuspid regurgitation disappears with clinical improvement.
Longitudinal Study of Serum Cholesterol, Apolipoproteins and Sex Hormones During Puberty
Authors: STOŽICK$YA F. , SLAB$YA P. , VOLENIKOVÁ L.
Published: January 2008

ABSTRACT. In order to determine the age and sex dependency of some of the major risk factors for coronary heart disease during the period of sexual maturation a five‐year longitudinal study was carried out on 105 boys and 133 girls living in Plzeň, Czechoslovakia. Serum cholesterol was significantly elevated in 13‐year‐old girls (p<0.01), while no statistically significant sex‐related differences in total and lipoprotein cholesterol and apolipoprotein A‐I and apolipoprotein B levels were found in other age groups during the period of follow‐up. In boys the total cholesterol showed a constant and statistically significant decrease (p<0.01) between the ages 11 to 13 years, but tended to rise during the last two years of study. In girls, serum cholesterol decreased significantly between the ages 11 to 15 years (p<0.05). A statistically significant decrease in pre‐beta cholesterol was found in girls during puberty (p<0.05). The changes in beta lipoprotein cholesterol were similar to those for total cholesterol. The apolipoprotein A‐I concentration decreased significantly between 11 and 15 years in boys (p<0.05), while it underwent no substantial change in girls. The apolipoprotein B level decreased permanently and significantly (p<0.05) during the 5‐year period in girls. In boys, the concentration of this protein decreased between 11 and 14 years and increased during the 15th year when a value significantly higher than that for girls was attained (p<0.05). As a result of these changes, the apolipoprotein A‐I to apolipoprotein B ratio became higher in girls. In the last year of the study, attainment of the highest value of the testosterone to estradiol ratio in boys was associated with higher concentrations of total, pre‐beta, and beta cholesterol and apolipoprotein B compared with girls. On the other hand, the highest level of the estradiol to testosterone ratio, which was also attained by girls during the last year of the follow‐up period, was accompanied by higher concentrations of alpha cholesterol and apolipoprotein A‐I.
Comparison of Serum and Salivary Antibodies in Children Vaccinated with Oral Live or Parenteral Inactivated Poliovirus Vaccines of Different Antigen Concentrations
Authors: ZAMAN S. , CARLSSON B. , JALIL F. , MELLANDER L. , WEZEL A. L. VAN , BÖTTIGER M. , HANSON L. Å.
Published: January 2008

ABSTRACT. A new antigen‐rich inactivated poliovirus vaccine (IPV) in ordinary (IPV1), double (IPV2) and quadruple (IPV4) antigen concentrations was given in 2 doses to 6 and 18 week old Pakistani infants. The immune responses to poliovirus types 1 and 3 were compared to those in infants given three doses of oral poliovirus vaccine (OPV) at 6, 12 and 18 weeks of age. Enzyme‐linked immunosorbent assay, ELISA, was used to estimate IgC and IgA in serum and secretory IgA (SIgA) in saliva. Two to three years later, a follow‐up of the serum antibody response was carried out in the same infants using a microneutralization test. Serum IgG antibody responses to poliovirus type 1 antigen after two doses of IPV1, IPV2 and IPV4 were not significantly higher than the response after three doses of OPV at 21 weeks of age (p > 0.05). The serum IgC responses to poliovirus type 3 were similar to those against type 1 in all the groups. Mean neutralizing antibody titres to poliovirus type 1 was significantly higher in the IPV2 group than the rest of the groups (p<0.01). For type 3, these titres were highest but not significantly, in the IPV4 group (p > 0.05). This study shows that two doses of a new antigen‐rich IPV can give similar immediate serum antibody responses as OPV but higher late responses. SIgA antibodies in saliva were more efficiently induced by OPV after three doses than after 2 doses of IPV (p<0.05).
Congenital Structural Abnormalities in Biliary Atresia: Evidence for Etiopathogenic Heterogeneity and Therapeutic Implications
Authors: SILVEIRA THEMIS R. , SALZANO FRANCISCO M. , HOWARD EDWARD R. , MOWAT ALEX P.
Published: January 2008

ABSTRACT. The clinical, surgical, laboratory and histological data of 237 children with extrahepatic biliary atresia were reviewed. Forty‐seven patients (20%) had associated congenital anomalies, and of these, 28 had cardiovascular, 22 digestive and 19 splenic malformations. Of the 19 patients with splenic malformations, 13 showed the polysplenia syndrome and two had asplenia. Chromosome studies were performed in eight children, six having associated anomalies, and two of them showed karyotype abnormalities (46,XX,del 18 p‐ and 49,XXXXY). These observations indicated that biliary atresia could be subdivided into four distinct etiopathogenic subgroups, three involving a congenital form that could arise through a malformation, a disruption or a chromosome abnormality, and the remaining to agents active in the perinatal period (the acquired form). The surgical outcome in 171 patients operated on by an experienced surgeon was not influenced by the presence of anomalies but by the timing of surgery. Seventy‐one percent of 24 patients operated on by 8 weeks of age were jaundice‐free as opposed to only 34% of those who had later surgery (p<0.01)
Low Colostral IgA Associated with Cow's Milk Allergy
Authors: SAVILAHTI E. , TAINIO V.‐M. , SALMENPERÄ L. , ARJOMAA P. , KALLIO M. , PERHEENTUPA J. , SIIMES M.‐A.
Published: January 2008

ABSTRACT. During a nutritional study of 198 infants, seven became allergic to cow's milk. The seven infants showed acute cutaneous manifestations during cow's milk challenge tests in hospital and six had increased levels of IgE cow's milk‐specific antibodies. Neither in the development of the levels of immunoglobulins G, A and M, nor in that of the cow's milk‐specific antibodies of these isotypes did these seven patients differ from the remaining infants. Beta‐lactoglobulin content and levels of cow's milk‐, and beta‐lactoglobulin‐specific antibodies and of immunoglobulins A, G and M were measured in samples of colostrum and milk from the mothers of the seven infants with cow's milk allergy and from a comparison group (non‐atopic mothers of non‐atopic infants). The milk of the mothers whose infants became allergic to cow's milk contained less IgA through the lactation: 95% confidence intervals of the groups did not overlap. The difference was most marked in the colostrum. All other measurements were similar in the two groups. This suggests that an infant is more likely to develop cow's milk allergy if the mother's colostrum had a low total IgA content.
Second Malignant Neoplasms in Patients Treated for Childhood Leukemia: A Population‐based Cohort Study from the Nordic Countries
Authors: NYGAARD R. , GARWICZ S. , HALDORSEN T. , HERTZ H. , JONMUNDSSON G. K. , LANNING M. , MOE P. J. ,   For the Nordic Society of Pediatric Oncology and Hematology (NOPHO)
Published: January 2008

ABSTRACT.Among a cohort of 981 children who were followed up 4.3–26.5 years after cessation of antileukemic therapy, eight patients in remission of acute lymphoblastic leukemia (ALL) developed a distinctively new malignant disease. The second malignant neoplasms (SMN) included brain tumors, basal cell carcinomas, thyroid cancer, leiomyo‐sarcoma and finally rhabdomyosarcoma in a patient who also had suffered from Hodgkin's disease while still on antileukemic treatment. Cranial radiation had been given to 58.4% of the patients in the study group, which consisted of 895 ALL patients who had completed various chemotherapy protocols. With one exception, the SMN appeared after 7.5–16.5 years at a location previously exposed to radiotherapy (RT). The estimated cumulative risk of SMN appearing within 20 years after diagnosis was 2.9%, and the corresponding risk for cases with RT was 8.1% compared to 0.3% for those without (p= 0.05). In a Cox regression analysis, the incidence rate ratio of SMN between patients with and without RT was 6.7 (95% CI = 0.8, 57.7). Based on age‐, year‐ and sex‐specific cancer incidence figures for Norway, the overall standardized incidence rate ratio (SIR) of SMN after treatment for ALL was 5.9 (95% CI = 2.2, 12.9). The number of brain tumors among patients who had received cranial radiation was nearly 27 times greater than expected, whereas no such tumors were seen after chemotherapy. Individuals treated for childhood ALL are at increased risk of a new malignancy, and this seems mainly to be associated with previous irradiation.
Long‐Lasting Catch‐up Growth under Bio‐Methionyl Growth Hormone Treatment in an Infant with Isolated Growth Hormone Deficiency Type 1A1
Authors: LUCA FILIPPO DE , DUQUESNOY PHILIPPE , ARRIGO TERESA , LOMBARDO FORTUNATO , GOOSSENS MICHEL
Published: January 2008

ABSTRACT. This case report concerns a 7‐month‐old infant with severe height retardation (–5.0 SD), typical growth hormone (GH)‐deficient phenotype, and undetectable GH serum levels in response to three pharmacological stimuli. Diagnosis of isolated GH deficiency type 1A was confirmed by restriction endonuclease analysis of genomic DNA which pointed out GH‐N gene deletion. The introduction of bio‐methionyl‐GH therapy in this patient was followed by a transient and clinically irrelevant appearance of low binding capacity GH antibodies as well as by a long‐lasting catch‐up growth (42.2 cm) which is continuing 44 months after beginning of treatment. This atypical pattern confirms that immune and growth response to exogenous GH in isolated GH deficiency 1A may be very heterogeneous.
Juvenile Pemphigus Vulgaris
Authors: GRAFF‐LONNEVIG V. , KAAMAN T.
Published: January 2008

ABSTRACT. Pemphigus vulgaris is extremely rare in childhood. Only 35 cases of juvenile pemphigus vulgaris have been reported in the literature up until to now. During the pre‐steroid era, the outcome of the disease was usually regarded as fatal. Today, the prognosis has improved because of the possibility of early diagnosis by immunoflourescent techniques, determination of antibodies against epidermal intercellular substance and early therapy with systemic steroids. A case of juvenile pemphigus vulgaris with a favourable outcome in a 13‐year old girl is described.
Coordination in Low Birthweight Seven‐Year‐Olds
Authors: ELLIMAN ALISON M. , BRYAN ELIZABETH M. , ELLIMAN ANTHONY D. , WALKER JEAN , HARVEY DAVID R.
Published: January 2008

ABSTRACT. The coordination and laterality of a group of 171 seven‐year‐old children, free from major disability, with a birthweight of 2000 g or less, were examined and compared with those of normal birthweight peers. More low birthweight children were left‐handed and of mixed or undetermined hand, foot and eye dominance. Left‐handedness may adversely affect some areas of performance of normal birthweight but not of low birthweight children. Low birthweight children performed significantly less well in tests of both fine and gross motor coordination. Girls tended to perform better than boys in fine motor tests. In the low birthweight group there was a correlation between IQ and coordination.
Riding‐School Lung?:Allergic Alveolitis in an 11‐Year‐Old Girl
Authors: KRISTIANSEN J. D. , LAHOZ A. X.
Published: January 2008

ABSTRACT. Farmer's lung is a rural disease, caused by inhalation of airborne moulds and actinomycetes in the environment (farms). We describe to our knowledge the first case of farmer's long in an 11‐year‐old girl briefly exposed to Thermophilic actinomycetes at a riding‐school.
Protein Losing Enteropathy as a Manifestation of Henoch‐Schönlein Purpura
Authors: REIF SHIMON , JAIN ASHOK , SANTIAGO JOSE , ROSS THOMAS
Published: January 2008

ABSTRACT. Gastrointestinal manifestations of Henoch‐Schönlein purpura (HSP) commonly include abdominal pain and gastrointestinal bleeding. Hypoproteinemia and edema could be related to renal involvement. We report a 14‐year‐old boy with classical features of HSP manifestated with edema due to severe intestinal protein loss, measured by elevated fecal alpha 1 antitrypsin secretion. The protein losing enteropathy subsided with corticosteroid therapy.
Unusual Cause of Methadone Poisoning
Authors: GAYLE MICHAEL O. , RYAN C. ANTHONY , NAZARALI SHEMIN
Published: January 2008

ABSTRACT. A child with respiratory distress was found to have been given an antibiotic which was reconstituted with methadone. A delay in standard emergency room management led to a delay in diagnosis and treatment.
Pseudomonas Septicaemia in Apparently Healthy Children
Authors: WONG SIK‐NIN , TAM ALFRED YAT‐CHEUNG , YUNG RAYMOND WAI‐HUNG , KWAN ELAINE YIN‐WAH , TSOI NELSON NAI‐SHUN
Published: January 2008

ABSTRACT. Among 27 cases of Pseudomonas septicaemia in the Department of Paediatrics of Queen Mary Hospital from 1981 to 1988, we have identified 10 children without known predisposing causes before presentation and report their clinical features. Six were infants, of whom 4 developed shock on admission and died. Ecthyma gangrenosum was present in 4 patients. Pesudomonas aeruginosa was isolated in 8 patients. All isolates, except Ps. cepacia, were sensitive to gentamicin. One patient had cyclical neutropenia. Another had an appendicular abscess. Salmonella was cultured from the stool in one patient. Although Pseudomonas septicaemia is normally considered to be associated with underlying immunodeficiency, in 22% it occurred in previously healthy children. Mortality is high especially in infants who develop septicaemic shock. It is advisable to cover for Pseudomonas septicaemia with aminoglycosides or ceftazidime in sick septic infants.
Invasive Haemophilus influenzae Infections in Household Contacts of Patients with Haemophilus influenzae Meningitis and Epiglottitis
Authors: TROLLFORS B.
Published: January 2008

ABSTRACT. In a retrospective nation‐wide Swedish study of Haemophilus influenzae meningitis and epiglottitis, a total of 500 cases of culture‐verified meningitis and 841 cases of epiglottitis fulfilling strict clinical criteria were found during a three‐year period. Eight families with two cases of these infections occurring with an interval of less than 60 days were identified. Four of the secondary cases occurred in siblings and four in mothers of the index case. Two of the secondary cases were twins to the index case. This shows that the risk of developing invasive H. influenzae infections is considerably increased in both siblings and parents of patients with invasive infection. Rifampicin prophylaxis, which is recommended in the United States for family contacts, would, however, prevent less than 1 % of all invasive infections with H. influenzae.
Health and Nutrition in Newly Resettled Refugee Children from Chile and the Middle East
Authors: HJERN A. , KOÇTÜRK‐RUNEFORS T. , JEPPSON O. , TEGELMAN R. , HÖJER B. , ADLERCREUTZ H.
Published: January 2008

ABSTRACT. The general health and nutritional status of 105 refugee children from Chile and the Middle East were examined shortly after the arrival in Stockholm. A chronic medical condition was present in 10% and there were clinical signs of canes in 57% of the children. Iron deficiency was found in 15% of Chileans and 6% of Middle Easterners, but no other nutritional deficiencies were discovered. Stunting and wasting was rare and a significant catch‐up growth was observed in both groups in a follow‐up 18 months after resettlement. Obesity was common in the Chilean group on arrival in Sweden and increased further after resettlement. We conclude that chronic medical conditions, canes and obesity were the major somatic health problems in this sample of newly resettled refugee children.
Interactions between Infections, Malnutrition and Ironnutritional Status in Pakistani Infants A Longitudinal Study
Authors: JAVAID NOSHEEN , HASCHKE F. , PIETSCHNIG B. , SCHUSTER E. , HUEMER C. , SHEBAZ A. , GANESH P. , STEFFAN I. , HURREL R. , SECRETIN M. C.
Published: January 2008

The interactions between infections, malnutrition and poor iron nutritional status in infants at weaning ages are poorly defined. Therefore, four groups of infants from an area with a high incidence of malnutrition (Lahore, Pakistan) were enrolled in a prospective, randomized nutritional intervention study. Between 122 and 365 days of age, the infants from one community received either a milk cereal without iron fortification (n= 29), a milk cereal fortified with ferrous fumarate (7.5 mg/100 g; n= 30), or a milk cereal fortified with ferric‐pyrophosphate (7.5 mg/100 g; n= 27). Forty‐four infants from a neighbouring community did not receive a nutritional supplement and served as the control group. Calculated mean daily energy‐ and protein intake with the cereals was between 259–287 kcal, and 9.6–10.6 g at 12 months of age, respectively. Mean daily iron intake with the fortified cereals was between 4.1–5.1 mg at corresponding age. Nutritional supplementation resulted in significantly lower incidence of malnutrition and heigher weight gain. Incidence of acute diarrhoea was significantly (p<0.05) lower in the supplemented groups. The infants fed the iron‐fortified milk cereals had significantly higher hemoglobin (mean 10.4 vs. 9.8 gdl‐1) and serum ferritin (mean 13.3 vs. 8.5 ngml‐1) values than the infants fed the non‐fortified milk cereals. However, no differences in the incidence of infections were found between the supplemented groups. It is concluded that poor nutritional intake between 122 and 365 days of age substantially contributed to the high incidence of diarrhoea and malnutrition in Pakistani infants.
Analysis of Transferrin and α1‐Fetoprotein in Amniotic Fluid and Neonatal Serum: a Possible Means for Indirect Prenatal Diagnosis of the Carbohydrate‐deficient Glycoprotein Syndrome
Authors: STIBLER HELENA , KRISTIANSSON BENGT
Published: January 2008

A new type of inborn error of glycoprotein metabolism has recently been identified (CDG syndrome). The disease has systemic manifestations but mainly involves the nervous system. The most striking biochemical abnormality is the presence of secretory glycoproteins that are partially deficient in their carbohydrate moieties, the most pronounced of which is seen in serum transferrin. The basic genetic defect of the syndrome has not yet been identified. This study was carried out in order to provide a basis for indirect prenatal diagnosis by qualitative and quantitative analyses of a corresponding carbohydrate‐deficiency in transferrin and also in α1‐fetoprotein in amniotic fluid. On isoelectric focusing both glycoproteins normally showed a carbohydrate (sialic acid (‐dependent microheterogeneity in amniotic fluid as well as in neonatal serum which in transferrin differed from adult serum. Reference values of total transferrin (TT) and carbohydrate‐deficient isotransferrins (CDT) during gestation were determined. Analysis of the microheterogenity of these glycoproteins by isoelectric focusing or determination of the CDT/TT ratio in amniotic fluid might provide a possibility for prenatal diagnosis of this syndrome. It was also shown that neonatal diagnosis is possible by analysis of serum transferrin.
Food consumption and nutrient intake of one‐ to two‐year‐old Finnish children
Authors: Räsänen L , Ylönen K
Published: January 2008

Food consumpton data for 46, 1‐ to 2‐year‐old children in Helsinki were collected by means of 3‐day food records. The mean daily energy intake was 1242 kcal for boys and 1092 kcal for girls. Protein accounted for 16% (range 9‐24%), fat 33% (20‐50%), total carbohydrates 51% (35‐69%) and sucrose 12% (3‐33%) of total energy intake. The mean ratio of polyunsaturated and saturated fatty acids was 0.43 (0.11‐3.71). The average energy and nutrient intake met or exceeded the recommended amounts except for iron, zinc, fluorine and chromium. When the children were divided into four groups according to dietary fat content, the intake of several nutrients was lowest in the group with the highest fat content (40% or more of energy) even though energy intake did not differ. The wide variety observed, especially in the fat composition of the children's dicts, indicates a need to reconsider the recommendations concerning the diet of young children after they have made the transition to family food.
Thermal responses and heart rates of low‐birth‐weight premature babies during daily care on a heated, water‐filled mattress
Authors: Sarman Ihsan
Published: January 2008

Body temperatures, heart rates and resting oxygen consumptions were examined during routine nursing care in 12 premature babies treated alternately in incubators or on a heated, water‐filled mattress (HWM). The mean temperatures were significantly higher in the axilla (0.3°C;p0.05) and the foot (1.4°C;p< 0.001) during HWM care. The degree of maximal fall in various body temperatures during routine nursing procedures was the same for both treatments, whereas the time taken for the foot temperature to fall was 13 min shorter during HWM care (p< 0.001). The proportion of heart rates below 160 bpm when the babies were not disturbed was 7.4% greater during HWM treatment (p< 0.01). The resting oxygen consumption in babies treated on HWM was slightly lower (6.2 ± 0.4 vs 6.4 ± 0.5 ml/kg/min;p< 0.05). Treatment on the HWM seems to promote calm and comfort, since it reduces the amount of thermal stress and prolongs quiet resting periods.
Artificial renal and liver support in a severe hepatorenal syndrome of childhood
Authors: Alarabi AA , Danielson BG , Wikström B , Kreuger A , Tufveson G
Published: January 2008

This case report describes a special management approach in a child aged 4.5 years with a severe form of hepatorenal syndrome in which the final diagnosis was familial hemophagocytic lymphohistiocytosis (FHL). The patient presented with grade IV hepatic coma and acute renal failure (ARF). While the diagnosis was difficult at the time of admission, as well as during acute treatment, artificial liver support was established for elimination of bilirubin and other metabolic by‐products by using charcoal column plasma perfusion (CCPP) and bilirubin‐adsorbing resin column plasma perfusion (BRCPP). Serum levels of bilirubin showed a notable decrease after each of the four treatment sessions. Additional artificial renal function replacement by continuous arteriovenous hemofiltration and hemodialysis (CAVH, CAVHD, respectively) had a marked lowering effect on urea and creatinine serum concentrations. Both artificial liver and renal support contributed to the general clinical improvement and survival of the patient. Further experience in these therapies will be needed to establish better prognosis in such fatal or acute similar conditions.
Long‐term study of the immunodeficiency of Bloom's syndrome
Authors: Kondo Naomi , Motoyoshi Fumiaki , Mori Seiji , Kuwabara Naoki , Orii Tadao , German James
Published: January 2008

The immune state was evaluated over a 10‐year period in two individuals with Bloom's syndrome. In both patients, serum concentrations of IgM were markedly low. Mildly decreased serum concentrations of IgG and IgA increased significantly with age, whereas the IgM levels remained low. From assessments of B‐cell and T‐cell functions in pokeweed mitogen‐induced immunoglobulin production, the IgM deficiencies were thought to result from B‐cell dysfunction. T‐cell function appeared intact. Moreover, although the percentages of surface IgM‐bearing cells were not reduced, the numbers of IgM‐secreting cells were reduced. These findings suggest that the IgM deficiency is due to an abnormality in the maturation of surface IgM‐bearing B cells into IgM‐secreting cells.
Respiratory water loss and oxygen consumption in newborn infants during phototherapy
Authors: Kjartansson S , Hammarlund K , Riesenfeld T , Sedin G
Published: January 2008

Respiratory water loss was measured together with oxygen consumption (±O2) and carbon dioxide production (±CO2) in 11 full‐term and eight preterm infants (mean gestational age 34 weeks, range 31‐36 weeks) before and during 1 h of phototherapy. The method for determination of respiratory water loss, ±O2 and ±CO2 was based on an open flow‐through system with a mass spectrometer for measurement of gas concentrations. All infants were studied naked in an incubator with an ambient relative humidity of 50% and with a controlled environment with respect to temperature and air velocity. The infants were calm during the measurements. Before phototherapy, in term infants respiratory water loss was 4.4 (SD 0.7) mg/kg min and ±O2 5.9 (0.9) ml/kg min and in preterm infants respiratory water loss was 4.7 (0.8) mg/kg min and ±O2 6.1 (0.8) ml/kg min. No significant difference was found between values obtained during or after 1 h of phototherapy and those obtained before.
Sister chromatid exchanges in peripheral lymphocytes in newborns treated with phototherapy and vitamin E
Authors: Galla A , Kitsiou‐Tzeli S , Gourgiotis D , Hadjigeorgiou E , Yousef‐Ayash H , Kanarios J , Richardson K , Sinaniotis C
Published: January 2008

A study was undertaken to determine whether blue fluorescent light might affect the sister chromatid exchange (SCE) frequency of peripheral lymphocytes in icteric newborns undergoing continuous phototherapy treatment (72 h). Also, the potential preventive effect of vitamin E on SCE frequency was studied in a subgroup of 11 preterm and 9 fullterm newborns after daily administration of vitamin E (46.44 μmol/kg/d, im). The results revealed that only the preterm icteric newborns showed an increase in mean SCE frequency of peripheral lymphocytes after phototherapy (9%, p= 0.02), but in no case did the highest SCEs/cell ratio exceed the normal values. No correlation was found between the average SCE rate and birth weight, gestational age or bilirubin levels. Also, no difference in SCEs was observed between newborns treated or untreated with vitamin E.
Relationship between neonatal behavior and subsequent temperament
Authors: Tirosh E , Hard J , Abadi J , Berger A , Cohen A
Published: January 2008

The validity of the neonatal behavioral assessment scale in predicting later infant temperament is of theoretical and clinical importance. The scale was administered to 47 full‐term healthy neonates. Of these, 40 were followed and the infant characteristics questionnaire was given at four months of age. Significant correlations were revealed between the neonatal assessment items from the clusters of orientation and motor maturity and the temperament factors “fussy‐difficult” and “unpredictable”. Items from the cluster of autonomic stability correlated with the temperament factor “unadaptable.” These correlations possibly indicate that perceived temperament does reflect intrinsic infant qualities.
Hydrops fetalis associated with Ureaplasma urealyticum
Authors: Ollikainen J , Hiekkaniemi H , Korppi M , Katila M‐L , Heinonen K
Published: January 2008

A hydroptic newborn was born at 32 weeks' gestation and at the age of 14 h died of post‐asphyxial syndrome. Immunologic causes of hydrops fetalis were excluded, as were anomalies and chromosomal aberrations. Ureaplasma urealyticum was isolated in bronchial secretions, lung tissue and brain tissue of the newborn. Our findings suggest that U. urealyticum infection should be considered in the differential diagnosis of hydrops fetalis.
Role of non‐capsulated Haemophilus influenzae as a respiratory pathogen in children
Authors: Korppi M , Katila ML , Jääskeläinen J , Leinonen M
Published: January 2008

During a 12‐month surveillance period from 1981‐1982, non‐capsulated Haemophilus influenzae was detected in nasopharyngeal aspirates from 64 (14%) of the 449 children hospitalized for middle or lower respiratory infection. An antibody response to H. influenzae was indicated in 15(23%) of the 64 patients with H. influenzae present in nasopharyngeal aspirate and in 10(3%) of the 385 patients with a negative finding. Thus, serological evidence of H. influenzae infection was demonstrated in 25(6%) of all the 449 children with respiratory infection. Of 13 patients with cultures positive for H. influenzae acute otitis media, an antibody response was seen in only 4(30%) patients. H. influenzae infection was associated with infections caused by other microbes in 20 children (80%), with viral infections in 60% and with pneumococcal infections in 24% of cases. An infection focus was present in 15(79%) of the 25 patients with H. influenzae infection; pneumonia was present in 10 cases and acute otitis media in 9 cases. Non‐specific laboratory evidence of bacterial infection was seen in 11 patients (58%); C‐reactive protein was increased in 7 and erythrocyte sedimentation rate in 9 patients. It is concluded that non‐capsulated H. influenzae is a genuine respiratory pathogen in children. H. influenzae infections appear to be secondary to preceding viral or other bacterial infections in children who are carriers of this strain.
Growth during treatment of familial hypercholesterolemia
Authors: Hansen Dorthe , Michaelsen Kim Fleischer , Skovby Flemming
Published: January 2008

Treatment of hypercholesterolemic children with restriction of dietary saturated fat may result in an inadequate supply of energy for normal somatic growth. We examined the growth of 30 children with familial hypercholesterolemia, some of whom were also treated with colestipol, a bile acid‐binding resin. The median duration of treatment was 8.5 years in 13 patients on diet only, and 5.5 years + 3.5 years in 17 patients treated with diet followed by diet and colestipol. Statistically significant reductions in serum total cholesterol were obtained in both groups. The SD scores for both height/age and weight/age decreased by approximately 0.4 during dietary treatment (p<0.05), but were not affected by treatment with colestipol. These results document the risk of growth retardation during dietary treatment of children with familial hypercholesterolemia.
Metabolic control and diet in Finnish diabetic adolescents
Authors: Virtanen SM
Published: January 2008

The effects of dietary, clinical and demographic factors on metabolic control in 105 diabetic adolescents were studied. All patients had diabetes for longer than two years and a daily insulin dose greater than 0.5 IU/kg body weight. Low body mass index, high social class, high number of daily eating occasions, high day‐to‐day variation in energy intake, high number of urine tests and a long interval between insulin injection and eating were associated with good metabolic control. Many of these determinants reflect also the general compliance with the diabetic regimen. The results stress the importance of good coordination between insulin injections and eating habits.
Trans fatty acids may impair biosynthesis of long‐chain polyunsaturates and growth in man
Authors: Koletzko Berthold
Published: January 2008

Human diet contains large amounts of trans fatty acids originating primarily from hydrogenated fats. Consumption of trans fatty acids is considered safe for man, but side effects, including impaired biosynthesis of long‐chain polyunsaturated fatty acids with 20 and 22 carbon atoms and reduced growth have been observed in animals. We studied whether or not there are indications of untoward effects of trans fatty acids in 29 premature infants (birth weight 1700 ± 127 g, gestational age 33.6 ± 1.4 weeks, mean ± SD). Plasma samples obtained on day 4 of life were analysed for fatty acid composition. Trans octadecenoic acid and total trans fatty acids in plasma lipid fractions (% wt/wt) were not related to the precursor essential fatty acids linoleic and alpha‐linolenic acids but correlated inversely to n–3 and n–6 long‐chain polyunsaturated fatty acids and to the product/substrate ratios of long‐chain polyunsaturate biosynthesis. Trans fatty acids were also inversely correlated to birth weight but not to gestational age. These data indicate a potential impairment of essential fatty acid metabolism and early growth by trans isomers in man, and question the safety of high dietary trans isomer intakes during pregnancy and the perinatal period.
High‐dose iv human immunoglobulin in a case with infantile opsoclonus polymyoclonia syndrome
Authors: Sugie H , Sugie Y , Akimoto H , Endo K , Shirai M , Ito M
Published: January 2008

We report a 14‐month‐old male with infantile opsoclonus polymyoclonia syndrome. His clinical disabilities responded favorably to high‐dose human immunoglobulin therapy. High dose immunoglobulin therapy is one of the therapies for patients with this syndrome.
Albuminuric growth failure. A case of Munchausen syndrome by proxy
Authors: Lyall EGH , Stirling HF , Crofton PM , Kelnar CJH
Published: January 2008

A five‐year‐old girl presented with profound growth failure, lethargy, vomiting and acidosis. A diagnosis of Munchausen syndrome by proxy was made after the demonstration of egg albumin, diphenhydramine and phenothiazine metabolites in her urine. Growth improved dramatically, but a subsequent child in the family died of sudden infant death syndrome.
Comparison of fiberendoscopy and Watson capsule for small intestinal biopsy in infants and children
Authors: Gottrand F , Turck D , Mitchell V , Farriaux JP
Published: January 2008

We analyzed the results of 607 small bowel biopsies performed over a seven‐year period: 284 biopsies were obtained using a fiberendoscope and 323 biopsies using a Watson capsule. Three to six specimens were removed during endoscopy. The biopsy fragments obtained with the fiberendoscope were deeper than those obtained with the Watson capsule (p<0.0001) and were more often located in the duodenum (p <0.0001). The failure rate of the fiberendoscope biopsies (1%) was lower than for the Watson capsule biopsies (9%) (p <0.0001). Multiple biopsies increased the diagnostic value of fiberendoscopy which was 95% versus 85% for Watson capsule. The mean duration of the endoscopic procedure recorded in 30 children was 6.5 min for four to six samples, i. e. 1.5 min per biopsy specimen. Fiberendoscopy appears to be an efficient and safe method for performing small bowel biopsies in infants and children.
Greek immigrant children in southern Sweden in comparison with Greek and Swedish children. III. Energy and nutrient intake
Authors: Neiderud J , Philip I , Sjölin S
Published: January 2008

Greek immigrant children (GI) belonging to the second generation of immigrants in Sweden have been compared with Swedish children.(S) and Greek children in Greece (G) regarding energy and nutrient intake. Twenty‐four‐hour recalls were obtained in the homes of the families. The mean energy intake was the same in all three groups and met the Swedish Nutrition Recommendations. The energy distribution for protein, carbohydrates and fat was also similar. The fat intake was far above the recommended level in all groups. The GI and the G group had a significantly higher mean daily intake of monounsaturated fatty acids than the S group (p<0.05 and p<0.001, respectively). Children aged four to eight years in the GI group had a significantly higher nutrient density of retinol, vitamin D, riboflavin, vitamin B6, calcium, iron, magnesium and sucrose compared to the G group, but compared to the S group they had a lower nutrient density of retinol, vitamin D, ascorbic acid, niacin, vitamin B12 and selen. The GI children consumed more milk than the G children but as much as the S children and they had started to use enriched low‐fat milk in Sweden. In conclusion, the food quality in the GI group was better than in the G group and much the same as in the S group, and, with few exceptions, it met the Swedish Nutrition Recommendations.
The influence of the basal yellow colour of the skin at birth on later jaundice meter readings in mature newborn infants
Authors: Knudsen Aage
Published: January 2008

The yellow colour of the skin was measured just after birth in 100 mature newborns using a jaundice meter. The skin colour was significantly correlated to the cord bilirubin concentration (rho=0.26, p=0.009), but unrelated to cord reserve albumin concentration, cord albumin concentration, cord haemoglobin concentration, birth weight and gestational age. In 123 other mature newborns, the basal yellow colour of the skin was estimated on the basis of meter readings taken just after birth. Correction of meter readings taken on the third postnatal day for the basal yellow skin colour improved neither the correlation between the meter readings and the bilirubin concentration nor the ability of the meter readings to predict hyperbilirubinaemia.
Recombinant factor VIIa in an infant with haemophilia A and inhibitors
Authors: Tengborn L , Kjellman B , Elfstrand P‐O , Bågenholm T
Published: January 2008

Post‐traumatic bleeding from the gingiva in a 16‐month‐old boy with severe haemophilia A did not stop after treatment with factor VIII concentrate and tranexamic acid, and it was demonstrated that he had developed antibodies to factor VIII. Treatment with recombinant factor VIIa 60‐90 μg/kg body weight four to eight times daily, together with local measures, stopped the bleeding and no complications were seen.
Schistosoma haematobium: a neglected common parasitic disease of childhood in Nigeria. Incidence and intensity of infection
Authors: Bello AB , Edungbola LD
Published: January 2008

A prospective and cross‐sectional study was carried out in various communities in Kwara State, Nigeria, to access the status and implications of urinary schistosomiasis among schoolchildren. Of 425 pupils examined in nine communities, 193 (45.4%) were infected. Infection rates for boys (44.7%) and girls (47.9%) were not significantly different (p > 0.5). Children between 11 and 13 years of age had the highest incidence rates (33.6%). However, the percentage of children (25.9%) excreting at least 1000 eggs per 10 ml of urine sample during their first decade of life was significantly higher (p < 0.01) than for older pupils. The health implications of schistosomiasis acquired early in life, as in this study, are highlighted in the discussion.
Progressive idiopathic cholestasis presenting with profuse watery diarrhoea and recurrent infections (Byler's disease)
Authors: Winklhofer‐Roob BM , Shmerling DH , Solèr R , Briner J
Published: January 2008

The second child of healthy unrelated parents presented with chronic diarrhoea since the age of two months, initially associated with non‐characteristic liver involvement. Recurrent infections, severe failure to thrive and various metabolic deficiencies complicated the further course, as well as profuse watery diarrhoea with elevated regulatory gut peptides, responding only to somatostatin analog treatment. At 22 months of age, intermittent cholestasis with permanently normal serum gamma‐glutamyltransferase was evident. The child died of fulminant purulent meningitis at the age of three years six months. Liver histology showed intrahepatic cholestasis, bile duct paucity with focal proliferation as well as slight portal and intralobular fibrosis. The clinical, biochemical and histopathological findings were indicative of Byler's disease.
Septo‐optic dysplasia and growth hormone deficiency: accelerated pubertal maturation during GH therapy
Authors: Freude Susanna , Frisch H , Wimberger Daniela , Schober Edith , Häusler Gabriele , Waldhauser F , Aichner F
Published: January 2008

We report four patients (three male, one female) with septo‐optic dysplasia and growth hormone deficiency. All had GH therapy for a period of four to eight years until reaching final height. In all four cases bone maturation during puberty was accelerated (1.4 to 1.9 “years”/year), resulting in a final height which was clearly below the predicted height. The progress of pubertal stages was very short in all patients. In three patients TSH and prolactin release after TRH stimulation were increased. These data support a hypothalamic original of the endocrine disorder. Insufficient GH release, even after repeated GHRH stimulation, is in contrast to this assumption. In one case there was a late manifestation of neurohormonal diabetes insipidus, which indicates the possibility of later disease progression. MR imaging of the brain demonstrated variable malformation of the septum pellucidum, chiasma and nervus opticus or the pituitary gland, respectively.
Circulating levels of endothelin and atrial natriuretic factor during postnatal life
Authors: Kojima Takatsugu , Isozaki‐Fukuda Yuka , Takedatsu Misa , Hirata Yukio , Kobayashi Yohnosuke
Published: January 2008

We determined plasma endothelin‐1‐like immunoreactivity and atrial natriuretic factor concentrations in preterm and full‐term newborn infants during the first month of life. Plasma endothelin‐1‐like immunoreactivity levels were far greater than those of maternal blood in the newborn infants. The highest plasma endothelin‐1‐like immunoreactivity levels were observed on the first day of life (8.7 ± 3.7 pmol/l) and gradually decreased with age. There was no significant difference in plasma endothelin‐1‐like immunoreactivity levels between preterm and full‐term infants. A good correlation was observed between plasma endothelin‐1‐like immunoreactivity levels and atrial natriuretic factor concentrations (p < 0.01). These results suggest that circulating endothelin may regulate the cardiovascular system in the newborn period.
Anesthesia‐induced rhabdomyolysis in infants with unsuspected Duchenne dystrophy
Authors: Tang Thomas T , Oechler Herbert W , Siker Daniel , Segura Annette D , Franciosi Ralph A
Published: January 2008

Anesthesia‐induced rhabdomyolysis in infancy may represent an unsuspected Duchenne dystrophy. In order to establish the diagnosis of this genetic disease more definitively, a dystrophin test is a requisite following the conventional creatine kinase test and light and electron microscopies of the muscle biopsy.
Persistent diarrhea in Northeast Brazil: etiologies and interactions with malnutrition
Authors: Lima Aldo AM , Fang Guodong , Schorling John B , de Albuquerque Licio , McAuliffe Jay F , Mota Sulivan , Leite Roberio , Guerrant Richard L
Published: January 2008

With the improved control of acute diarrheal illness mortality with oral rehydration therapy, persistent diarrhea is now emerging as a major cause of childhood mortality in tropical developing areas like the impoverished populations in Brazil's Northeast. “Graveyard surveillance” in the rural community of Guaiuba in northeastern Brazil revealed fully half of the 70% diarrhea mortality was due to persistent diarrheal illnesses. Furthermore, 11% of 14 or more diarrheal illnesses per child per year in an urban slum in Fortaleza persisted beyond 14 days, a definition that clearly identified the high risk children for heavy diarrhea burdens. Not only did heavy diarrhea burdens ablate the key “catch‐up” growth seen in severely malnourished children and in children following previous diarrheal illnesses, but malnutrition significantly predisposed children to a greater incidence and duration of diarrhea as well as a greater incidence of persistent diarrhea. Etiologic studies of 37 children presenting with persistent diarrhea to Hospital das Clinicas in Fortaleza revealed that Cryptosporidium (in 13%) and enteroadherent E. coli (36% with aggregative, 29% with diffuse and 13% with localized adherence to HEp‐2 cells) were the predominant potential pathogens found in the stool or upper small bowel. These findings suggest that persistent diarrhea is emerging as an important health problem in Brazil's Northeast, that it identifies a high risk child for heavy diarrhea burdens, that important interactions occur with malnutrition and that Cryptosporidium and enteroadherent E. coli warrant further study as potential etiologies of this major cause of morbidity and mortality.
The child's disease (Doença de criança): popular paradigm of persistent diarrhea?
Authors: Nations M
Published: January 2008

According to popular report, the folk illness, Doença de criança (The Child's Disease) is the leading cause of infant and childhood death in Northeast Brazil; most health professionals, however, are unaware it exists. Doença de criança is described based on ethnographic interviews with 50 traditional healers and 50 bereaved mothers whose children have died from the condition. Doença de criança is a sociocultural label developed by the popular medical system and applied to the end stages of severe childhood infectious diseases and malnutrition against a background of grinding poverty. Doença de criança is discussed as an analog to persistent diarrhea. Practical treatment and management implications of this relationship are presented. Doença de criança, most importantly, offers a new, holistic paradigm for the future study of persistent diarrhea.