bluuebunny/crossref_metadata_2025 huggingface dataset:
Somatostatin and its Physiological Significance in Regulating the Episodic Secretion of Growth Hormone in the Rat
Authors: ROBINSON I.C.A.F. , JEFFERY S. , CLARK R.G.
Published: January 2008

ABSTRACT. Somatostatin (SS) is a powerful inhibitor of growth hormone (GH) secretion both in vitro and in vivo, and is generally regarded as having a negative influence on GH secretion and growth. In the conscious rat, the GH secretory pattern, the response to repeated injections of GH‐releasing hormone (GHRH), and the feedback mechanism by which GH regulates its own release are all sexually dimorphic and probably reflect a sexually dimorphic pattern of SS release. Prolonged infusions of SS initially block GHRH‐induced GH release but responses begin to break through with time. Withdrawal of SS induces a rebound release of GH in vivo, largely dependent on the release of endogenous GHRH. Prolonged exposure to intermittent infusions of SS in normal female rats produces a paradoxical growth response by inducing regular peaks of GH secretion whereas continuous infusions of SS do not. This response is GH‐dependent, and is not due to other effects of SS since intermittent infusions of SS do not increase growth in dwarf rats which are deficient in pituitary GH. The sex differences in GH secretion are also reflected in the expression of several GH‐dependent liver enzymes, one of which, carbonic anhydrase III, responds to manipulations of the endogenous secretory pattern by SS in both normal and dwarf rats, and appears to be sensitive to differences in basal, rather than peak, GH levels.
Recombinant Human Growth Hormone Treatment of Children with Chronic Renal Failure: Update 1990
Authors: FINE R.N.
Published: January 2008

ABSTRACT.Nine children with growth retardation due to chronic renal failure were treated with recombinant human growth hormone (rhGH) for 12‐36 months. Results demonstrated a significant increase in height velocity at each 12‐month interval compared with that achieved during the year prior to treatment. However, the increase in bone age was no greater than the increase in chronological age during the period of treatment. The mean calculated creatinine clearance did not decrease significantly during the 36 months of treatment; however, two patients required institution of dialysis at 18 and 30 months following initiation of rhGH treatment. There was no exacerbation of the glucose intolerance of uraemia following treatment. Currently, 6 of 7 patients who have been treated for more than 24 months have achieved sufficient acceleration in height velocity to attain an SDS of less than ‐2.00 and are above the 5th centile for chronological age on the growth curve. These updated data indicate that rhGH treatment of growth retarded children with chronic renal failure continues to result in accelerated height velocity during the second and third year of treatment, and demonstrate the potential for such children to achieve normal stature (±2 SD) for chronological age despite the continued presence of chronic renal failure.
Treatment of Growth Hormone Deficient Patients with Recombinant Somatropin for 1 year: Results of a Chinese Multicentre Trial
Authors: SHI Y.F. , BAO X.L. , WANG D.F. , CHEN R.G. , CHUN Y.
Published: January 2008

ABSTRACT. Fifty‐nine patients with idiopathic growth hormone deficiency were included in a Chinese multicentre trial of recombinant somatropin, 0.5‐0.7 IU/kg/week s.c. given in six or seven divided doses. The height velocity increased from 2.8 ±1.0 cm/year to 13.1 ± 2.5 cm/year during 1 year of treatment, and typical catch‐up growth was observed. The increase in height SDS for chronological age was significant, but the increase in height SDS for bone age was not statistically significant. No adverse reactions to the treatment were recorded. Recombinant somatropin was shown to be very effective and safe during the first year of therapy in patients with growth hormone deficiency.
Medulloblastoma in Nordic Children. I
Authors: CLAUSEN N. , GARWICS S. , GLOMSTEIN A. , JONMUNDSSON G. , KRUUS S. , YSSING M.
Published: January 2008

ABSTRACT. The average annual incidence of histologically verified medulloblastomas was 5.4×10‐6 among all 4.9 million Nordic children younger than 15 years from 1968 to 1977. The ratio between boys and girls was 2:1. The incidence had no clear seasonal variation and it peaked at 3 to 4 years of age at onset. The rate of survival after 5 years was 17.4%, equal for boys and girls, but highest in the older age groups. Tumor growth accounted for most deaths with lack of local control in 88% of 112 children autopsied. Metastases outside of the central nervous system occurred in only 6 % of the children. Thus, intensification of the treatment should be directed towards the central nervous system.
Medulloblastoma in Nordic Children. II Neurologic and Social Prognosis in Long Term Survivors
Authors: YSSING M. , GARWICZ S. , GLOMSTEIN A. , JONMUNDSSON G. , KRUUS S.
Published: January 2008

ABSTRACT. Forty‐four Nordic children surviving from histologically documented medulloblastoma diagnosed before the age of 15 years from 1968 to 1977 were studied by clinical investigations 5–15 years after the time of diagnosis. Thirty percent of the children suffered from major neurologic and/or intellectual late effects impairing their social life. The remaining 70 % of the children had mild or no motor, sensory, or mental dysfunction. In 30–35% of the patients the quality of life and the level of function in school and in professional life were impaired to a higher degree than was expected from the clinical observations. A favourable long term prognosis with regard to neurologic and intellectual function and to quality of life was significantly correlated to age > 10 years at diagnosis, and it was insignificantly associated with biopsy as the only primary surgical intervention and with peri‐operative treatment with corticosteroids. Sex, length of diagnostic delay, and different treatment modalities did not influence the long term prognosis.
Symbiotic Interactions between Colonic Microflora and Protein Metabolism in Infants
Authors: HEINE W. , MOHR C. , WUTZKE K. D. , RADKE M.
Published: January 2008

ABSTRACT. The utilization of 15N nitrogen from 15N‐labelled bifidobacteria for whole body protein synthesis was studied in 4 infants by oral single‐pulse labellings and in 3 other infants, who had colostomies, by colonic pulse labellings. The bifidobacteria were harvested from a modified Petuely culture medium containing 15N ammonium chloride and 15N cystine as the only sources of nitrogen. The tracer dose chosen for the balance studies was 3 mg 15N/kg. 15N concentrations in urine and feces collected over 48 hours after the pulse labellings were determined by emission spectrometry. Oral administration of 15N‐labelled bifidobacteria resulted in absorption of approximately 90%, renal excretion of 15%, and fecal excretion of 12% of the tracer dose, respectively. Retention in the protein pool averaged 73%. After colonic single pulse labelling with 15N‐labelled bifidobacteria, the corresponding values were 85.5%, 2.2%, 14.5%, and 83.0%, respectively. Absorption and incorporation of the heavy nitrogen into body proteins were directly demonstrated by increased 15N atom percent excess values within the trichloroacetic acid (TCA) supernatants and the proteins of the plasma, 0.25 and 0.04 atom %, respectively, at 24 hours after oral pulse labellings. One half of the total 15N excreted in urine consisted of urea and approximately 8% was eliminated as ammonia.
Somatostatin Levels in Plasma in Nonsmoking and Smoking Breast‐Feeding Women
Authors: WIDSTRÖM A.‐M. , WERNER S. , MATTHIESEN A.‐S. , SVENSSON K. , UVNÄAS‐MOBERG K.
Published: January 2008

ABSTRACT. The objective of the study was to record how somatostatin levels in plasma are altered in response to breast‐feeding during the lactation period and to relate somatostatin levels to the success of the lactational performance and to smoking habits. Fifty‐two women were investigated 4 days post partum and 3‐4 months later. Blood samples were collected and the levels of somatostatin‐like immunoreactivity (below referred to as SLI) were measured with radioimmunoassay. The periods of exclusive breast‐feeding and of mixed feeding were assessed as well as the milk yield. Smoking habits were noted. SLI levels were found to be significantly lower on day 4 after delivery, compared to 3‐4 months later. Also the type of response to breast‐feeding was different. Thus, a significant fall of SLI was seen during breast‐feeding at the maternity unit, but not 3–4 months later. Smoking women breast‐fed fully for a significantly shorter time than nonsmokers and had significantly higher SLI levels at onset of breast‐feeding day 4 post partum. Whether the high somatostatin levels recorded in connection with breast‐feeding in smokers are related to the shorter period of breast‐feeding seen in this group remains to be established. In addition, the highest levels of somatostatin were seen the day after the very last breast‐feeding and a possible role for somatostatin in the weaning process should be explored.
Acute Effects of Acetazolamide on Cerebral Blood Flow Velocity and pCO2 in the Newborn Infant
Authors: COWAN F. , WHITELAW A.
Published: January 2008

ABSTRACT. Twelve infants with hydrocephalus were treated with acetazolamide. In those treated intravenously middle cerebral artery blood flow velocity increased by a median of 86% (range 54‐150%). Maximum increase was reached within 2‐20 min. The effect lasted as long as 3.5 hours. Intracranial pressure rose by a median of 4 mmHg (range 0–10 mmHg). There was no significant alteration in blood pressure or heart rate. Respiratory rate increased by approximately 10 breaths/min and the tcpCO2, rose by a median 0.2 kPa in infants with normal lungs. In infants treated orally, blood velocity rose by 35‐40% at 60‐80 min with no increase in intracranial pressure. In four infants with lung disease pCO2, rose by a median of 2.0 kPa (range 0.6 to 3.4 kPa). Acetazolamide was well tolerated in infants with normal lungs but should be used with caution in the presence of lung disease.
The Relation between Perinatal Conditions and Developmental Outcome in Low Birthweight Infants: Comparison of Two Cohorts
Authors: ELLISON P. H. , GREISEN G. , FOSTER M. , PETERSEN M. BLOCH , FRIIS‐HANSEN B.
Published: January 2008

ABSTRACT. We have compared the relations between perinatal conditions and developmental outcomes at age four years for two cohorts of children with birthweights 2 300 g or less, who did not develop cerebral plasy—one from Southeastern Wisconsin (children born 1975‐76) and the other from Copenhagen (children born 1980‐82). We examined the general effects of parental education and socioeconomic status, the use of Cesarean section, the degree of prematurity and neonatal complications on outcome. The methods of latent path structural analysis were used to form two models among 15 latent variables: one for children from Copenhagen and a similar model for children from Wisconsin. The impact of parental education and socioeconomic status was somewhat greater in Wisconsin. Several neonatal complications were related to outcome in Wisconsin: the early condition of the infant, use of a respirator, pneumothorax, and anemia/apnea. The only neonatal complication with a significant relation to outcome in Copenhagen was pneumothorax and to a much lesser degree major germinal layer haemorrhage. The degree of prematurity per se had a greater impact in Copenhagen. The use of Cesarean section and mechanical ventilation in the smallest infants was much more frequent in Denmark, but no association could be shown between this increased use and improved developmental outcome.
Sleep and Breathing Abnormalities in a Case of Prader‐Willi Syndrome: The Effects of Acute Continuous Positive Airway Pressure Treatment
Authors: SFORZA E. , KRIEGER J. , GEISERT J. , KURTZ D.
Published: January 2008

ABSTRACT. This report describes the polysomnographic findings and the respiratory alterations during sleep in a 20‐year‐old patient with the Prader‐Willi syndrome. Nocturnal recordings and a variant of the multiple sleep latency test showed excessive daytime sleepiness, sleep onset rapid eye movement episodes, snoring and sleep apnea. Treatment with nasal continuous positive airway pressure normalized the respiratory pattern and the sleep structure, except for rapid eye movement sleep onset. Whereas upper airway obstruction and obesity may explain the respiratory disorders, as shown by their resolution with continuous positive airway pressure treatment, hypothalamic dysfunction could play a role in the disruption of the normal nonrapid eye movement/rapid eye movement sleep periodicity.
A Case of Neonatal McCune‐Albright Syndrome with Cushing Syndrome and Hyperthyroidism
Authors: YOSHIMOTO M. , NAKAYAMA M. , BABA T. , UEHARA Y. , NIIKAWA N. , ITO M. , TSUJI Y.
Published: January 2008

ABSTRACT. We describe a female newborn infant with McCune‐Albright syndrome. In addition to the cutaneous pigmentation, she had apparent manifestations of hyperthyroidism and Cushing syndrome since birth. X‐ray examinations showed many scattered lucencies in multiple bones. Endocrinological findings were as follows: serum T4 276 nmol/l; free T4 125 pmol/l; TSH <1 mU/l; serum cortisol >2210 nmol/l; plasma ACTH < 10 pg/ml; urinary free cortisol 865 nmol/day; estradiol 0.36 nmol/l. Regardless of treatment with antithyroid drugs and an inhibitor of 3β‐hydroxysteroid dehydrogenase, the patient died of cardiac failure at the age of 4 months. Autopsy findings included a follicle cyst in the right ovary and multinodular hyperplasia in the thyroid and both adrenals. To our knowledge such a severe neonatal form of McCune‐Albright syndrome has not been described in the literature.
Renal Involvement in Patients with Congenital Cyanotic Heart Disease
Authors: KRULL F. , EHRICH J. H. H. , WURSTER U. , TOEL U. , ROTHGÄNGER S. , LUHMER I.
Published: January 2008

ABSTRACT. Patients with congenital cyanotic heart disease may develop a glomerulopathy with proteinuria and impaired renal function. In order to investigate this problem we conducted a study on 27 patients with uncorrected cyanotic heart disease who were between 1 day and 25 years old. As a consequence of hypoxaemia haematocrit was elevated to 57%. Proteinuria was above 150 mg/day/1.73 m2 body surface in 12 patients. Only one of 9 children under 10 years of age had pathological proteinuria presenting as isolated albuminuria. Seven out of 10 patients between 11 and 20 years had an elevated proteinuria with a glomerular pattern. Creatinine clearance was normal in these patients. All four patients above 20 years of age had a considerable glomerular proteinuria with a mean excretion of 5.7 g/24 h/1.73 m2 body surface. These patients suffered additionally from chronic cardiac failure and creatinine clearance was below the normal range. There was a clear relationship between pathological proteinuria and age of the patients and thus duration of hypoxaemia. Patients with pathological proteinuria had a significant higher erythrocyte count (7.3±2 1.3 vs. 5.6±1.4 1012/l p<0.01) and a lower mean corpuscular haemoglobin. In summary, children with persistent congenital cyanotic heart disease have substantial risk of developing a glomerulopathy if the cyanosis remains unchanged for more than ten years.
Tay or IBIDS Syndrome: A Case with Growth and Mental Retardation, Congenital Ichthyosis and Brittle Hair
Authors: BLOMQUIST H. K:SON , BÄCK O. , FAGERLUND M. , HOLMGREN G. , STECKSÉN‐BLICKS C.
Published: January 2008

ABSTRACT. A new, Swedish case with Tay or IBIDS syndrome is presented. The boy had growth and mental retardation, congenital ichthyosis and brittle hair. He was the only child in an uncle–niece marriage. The boy suffered recurrent infections and died at the age of 3 years from pneumonia. Clinical data on 15 cases are presented from a study of the literature.
Breast Feeding Modifies Production of SlgA Cow's Milk‐Antibodies in Infants
Authors: RENZ H. , BREHLER C. , PETZOLDT S. , PRINZ H. , RIEGER C. H. L.
Published: January 2008

ABSTRACT. One hundred and fifty‐eight healthy mature newborns were divided into 3 groups according to their risk of allergy: Group A, no risk (n=62), group B, low risk (n= 30) and Group C, high risk (n= 66). Saliva was collected at birth and after 3 and 6 months. SIgA anti‐casein and anti‐β‐lactoglobulin were determined by direct ELISA technique. The highest concentrations of secretory antibodies were measured after birth. After 3 months, breast fed infants had lower salivary SIgA anti‐casein concentrations than the group receiving cow's milk (p≤0.01). The effect of breast‐feeding was seen even after nursing period of only 3 weeks. Infants without risk of allergy fed cow's milk exclusively had higher SIgA anti‐casein (p < 0.03) and anti‐β‐lactoglobulin concentrations than low risk infants at the age of 6 months. These data show modifying effect of breast feeding on salivary SIgA production against cow's milk protein.
The Effect of Glucocorticosteroids on the Neonatal Blood Count
Authors: WINTER J. P. DE , BEL F. VAN
Published: January 2008

ABSTRACT. To investigate the influence of glucocorticosteroid therapy on the neonatal blood count, the haematologic data of 68 preterm and term infants, who had received single dose of 1 mg dexamethasone i.v., were reviewed. White blood cell (WBC) count and platelet count increased after steroid therapy. The increase in WBCs was associated with an increase in the number of neutrophilic granulocytes, whereas the number of eosinophils decreased. We conclude that glucocorticosteroids alter the neonatal blood count and influence its value as diagnostic marker for bacterial infections.
Iron Deficiency in Coeliac Disease Is Mild and It Is Detected and Corrected by Gluten‐free Diet
Authors: STÅHLBERG M.‐R. , SAVILAHTI E. , SIIMES M. A.
Published: January 2008

ABSTRACT. In 54 children with coeliac disease, mild iron deficiency anaemia or evidence of iron deficiency without anaemia were common at the time of diagnosis. Treatment with gluten‐free diet without iron medication eliminated all evidence of iron deficiency and completely normalized laboratory values. Subsequent challenge with gluten resulted in the rapid reappearance of suboptimal iron balance as evidenced by decrease in serum ferritin concentration.
A Follow‐up Study of Children with Neonatal Herpes Simplex Virus Infections with Particular Regard to Late Nervous Disturbances
Authors: MALM GUNILLA , FORSGREN MARIANNE , AZAZI MILDRED el , PERSSON ANDERS
Published: January 2008

Forty‐five children with neonatal herpes simplex virus (HSV) infection, representing all known cases in the diagnostic records of four virological laboratories within a 15‐year period, were followed up. Twelve children had died. Sixteen of the 33 survivors were healthy. Thirteen children had severe disabilities: all of them showed severe mental retardation; moreover, 11 were tetraplegic, one was hemiplegic with hydrocephalus and one had a pronounced behavioural abnormality. Four children had slight to moderate disabilities: one child was mildly mentally retarded and three, although mentally normal, had hemiplegia and delayed speech development, one of them having a learning disorder as well. Of these 17 neurologically impaired children 16 had ophthalmological abnormalities. EEG recordings were made in 29 patients in the neonatal period. They were markedly abnormal in 24 patients, 14 of whom had localized periodic complexes. An abnormal EEG was a bad prognostic sign. The neurological outcome was better in the HSV‐l‐infected children (10 cases) than in the HSV‐2‐infected ones (35 cases). Progressive or recurrent encephalitis was strongly suspected in two preterm children.
Epidemiology of Absence Epilepsy: II. Typical Absences in Children with Encephalopathies
Authors: OLSSON I. , HEDSTRÖM A.
Published: January 2008

ABSTRACT. A population‐based study of absence epilepsy in Swedish children, aged 0–15 years, comprised cases selected on the basis of EEG criteria. Absence epilepsy was found in 119 of the 134 children with 3 Hz spike‐and‐wave discharges, and 12 of these 119 (10.1%) had typical absences in addition to other generalized seizures and slow irregular spike‐and‐wave activity on the EEG. The mean annual incidence of this type of absence epilepsy was 0.7/100000. The median age at onset of absences was 6 years. Eight of the 12 patients had neurological abnormalities and/or severe mental retardation. The patients constitute a heterogeneous group of encephalopathies. They may have a genetic predisposition for absence epilepsy, causing it to appear during the course of a more severe, encephalopathy related, type of epilepsy.
Low Field Magnetic Resonance Imaging of the Central Nervous System in 15 Children with Autistic Disorder
Authors: EKMAN G. , CHATEAU P. DE , MARIONS O. , SELLDEN H. , WAHLUND L.‐O. , WETTERBERG L.
Published: January 2008

ABSTRACT. Fifteen children, 10 boys and 5 girls, with autistic disorder, were studied with low field magnetic resonance imaging (MRI). The age ranged from 2.7–13.1 years, with a mean of 8.3 years. All patients but one (who refused) had a normal CT scan of the C.N.S. The MRI investigation was performed during anaesthesia with a low field magnetic resonance imager. The cerebrum, cerebellum, and brain stem were examined. No pathological changes were found in any of the patients studied.
Acute Confusion—an Unusual Form of Cerebral Herpes Simplex Virus Infection?
Authors: NORMAN MIKAEL , LUNDBERG BJÖRN , FORSGREN MARIANNE , RYDELIUS P.‐A.
Published: January 2008

ABSTRACT. Herpes simplex virus (HSV) infections are common and, if afflicting the central nervous system, may cause death or severe sequelae. Since specific therapy is now available there is an increasing demand for the recognition of the various ways in which cerebral HSV‐infections may be manifested. This report describes a 15‐year‐old boy who presented acutely with mental symptoms of confusion, hallucinations and sleep disturbance. Simultaneously, but without any increase in cerebrospinal fluid cell or protein content, an intrathecal production of HSV‐antibodies of transient nature was detected. These findings suggest that the patient may have suffered from an unusual form of herpes infection in the central nervous system. We suggest that similar cases should be thoroughly explored for possible herpes etiology as early as possible during the acute phase of the disease.
Sudden Infant Death Syndrome Related to Climate
Authors: BEAL SUSAN , PORTER CHRISTINE
Published: January 2008

ABSTRACT. In Australia the single most important factor influencing the incidence of SIDS is the climate. The incidence in mid summer in South Australia is 0.7 per 1000 live births, in mid winter in Tasmania it is 6.3 per 1000 live births. It is predicted that if infants under 6 months of age in cold weather were tied into swaddling and placed supine to sleep as in Asia and Czechoslovakia, and older infants who may object to restriction of movement, had the cot made up with the infants' feet touching the lower end, warm clothing and no more than a single thin porous cover, the incidence of SIDS as in Asia and Czechoslovakia could be reduced to < 1.0 per 1000.
Intraparturn and Neonatal Mortality in a Traditional Indigenous Community in Rural Guatemala
Authors: BARTLETT A. V. , BOCALETTI M. E. PAZ DE
Published: January 2008

ABSTRACT. We identified high rates of intrapartum and neonatal mortality among children born in a traditional indigenous comm. unity in rural Guatemala. To examine the potential association of maternal characteristics and obstetric and newborn care practices with this mortality, we conducted a retrospective case–control study. Cases were infants born in 1986 and 1987 who died during birth or in the first month of life, as identified by civil records; for each case, the next child born who survived the first month of life was selected as control. In interviews with mothers of cases and controls standardized data were collected on demographic and socioeconomic characteristics of the mother, her general obstetric history, history of the pregnancy, labor, and delivery, condition and care of the infant at birth, and morbidity and treatments of the infant after birth. Sixty‐one cases and their controls were included in the study. Based on clinical condition at birth, we subcategorized cases into infants stillborn or dying in the first 24 hours of life (intrapartum cases) and those dying in the first month after day 1 (neonatal cases). Factors significantly associated with both subcategories of cases were maternal illiteracy, primagravity, failure to use “modern” prenatal care, and inter‐birth interval < 14 months. Intramuscular injection of oxytocin by the midwife during labor, and performance of ≥ 3 vaginal examinations by the midwife were each significantly associated only with the intrapartum subcategory of cases. Mother's estimate of infant size as “smaller than normal” was associated with neonatal, but not with intrapartum, cases. Reported clinical features of cases suggested birth asphyxia and/or trauma to be predominant among intrapartum cases, and sepsis to be the most common cause of neonatal mortality after day 1.
Studies on Perceived Breast Milk Insufficiency: III. Consequences for Breast Milk Consumption and Growth
Authors: HILLERVIK‐LINDQUIST CHARLOTTE , HOFVANDER YNGVE , SJÖLIN STIG
Published: January 2008

ABSTRACT. Fifty‐one mother–infant pairs were followed prospectively during the period 3 days to 18 months after delivery. In total 54.9% of the mothers experienced transient lactation crises, emanating mostly from a perception of breast milk insufficiency. Within the crisis group no significant difference in the infants' intake of breast milk during the crises compared with control measurements 1 week later was found. Nor had the crises any immediate impact on growth of the infants. A comparison between the crisis and the non‐crisis group, revealed that the breast milk consumption in the crisis group was throughout lower with significant differences at 3 and 5 months. The infants in the crisis group also had a significantly lower weight at 2, 3, 4 and 9 months and were significantly thinner for their height at 1–6 months and at 9 months, although both groups were above the NCHS mean. We conclude that even if the infants in the crisis group had a lower consumption and a slower growth development, the differences were comparatively small. Furthermore, evidence was provided that the breast milk insufficiency occasionally perceived as acute by the mothers was in most cases not real.
Liver Disease and Heterozygous Alpha‐1‐Antitrypsin Deficiency
Authors: PITTSCHIELER KLAUS
Published: January 2008

ABSTRACT. From 1985 to 1988 14938 newborns were screened during the first days of life to determine their protease inhibitor phenotype (Pi) and 467 PiMZ and 456 PiMS were identified. Of these 101 PiMZ and 135 PiMS were followed‐up and their clinical, biochemical and, in selected cases, histological data were recorded at two, five and twelve months of age. Nineteen out of 101 PiMZ infants showed hepatic dysfunction at two months, eight at five, and one at twelve months of age, respectively. In 20 of 135 PiMS infants, liver function tests were abnormal at two months, in ten at five and in none at twelve months. It appears that PiMZ and PiMS phenotypes can be associated with hepatic dysfunction during the first six months of life. The marked variability of serum levels of alpha‐1‐antitrypsin in heterozygotes, make Pi‐typing in all cases of neonatal hepatitis advisable. This should also be done for screening purpose.
Estrogen Treatment of Tall Girls: Dose Dependency of Effects on Subsequent Growth and IGF‐I Levels in Blood
Authors: SVAN H. , RITZÉN E. M. , HALL K. , JOHANSSON L.
Published: January 2008

ABSTRACT. Three groups of girls with mean final height predictions of 185.3 cm were treated in 3‐week cycles with 0.25, 0.5, or 1.0 mg of ethinylestradiol. The three groups were comparable with regard to chronological age, bone age, height prediction, and duration of treatment. Reduction in final height of 5.5–5.9 cm/group in comparison with the initial prediction was observed in all treated groups. This reduction appeared independent of estrogen dose. Subsequent height increases were limited to about 50% of the predicted gain in all cases. In a comparable group of untreated tall girls the prediction method overestimated the final height by 1.2 cm. In the groups receiving 1.0 and 0.5 mg EtE2 a significant reduction in ICF‐I levels was achieved within three to six months after the start of estrogen therapy.
Incidence in Sweden and Clinical Features of Familial Hemophagocytic Lymphohistiocytosis
Authors: HENTER JAN‐INGE , ELINDER GÖRAN , SÖDER OLLE , ÖST ÅKE
Published: January 2008

ABSTRACT. We retrospectively studied the incidence of familial hemophagocytic lymphohistiocytosis (FHL) in children during the 16‐year period 1971–86. First, all departments of pediatrics, pathology, and infectious diseases were enquired for children with FHL or disorders resembling FHL. Secondly, the causes of death of all children who died during the study period in Sweden (n= 19 542) were also investigated. Files and histological specimens were further studied in selected children. By using a set of inclusion/exclusion criteria, we found 32 children with FHL. The incidence was 1.2/1000000 children per year. One child per 50 000 live borns developed FHL during this period. The sex ratio was close to 1:1. Prominent early clinical signs were fever (91%), splenomegaly (84%), hepatomegaly (90%), rash (43%), and lymph node enlargement (42%). Neurological symptoms, which developed in 47%, could totally dominate the clinical picture and develop prior to other symptoms and signs. Common laboratory findings were pancytopenia, hypertriglyceridemia, hypofibrinogenemia, elevated serum transaminases, hyperbilirubinemia, hyponatremia, hypoalbuminemia, and a moderate spinal fluid pleocytosis. Chest X‐ray often revealed mostly discrete pulmonary infiltrates. FHL is an underdiagnosed disease and in only 11/32 children was diagnosis made during their lifetime. It is important to be aware of the disorder as potential therapy now exists.
Parental Reactions to a Child's First Febrile Convulsion:A Follow‐up Investigation
Authors: BALSLEV T.
Published: January 2008

ABSTRACT. The severe psychological reactions of most parents to the first febrile convulsions of their child contrast with the doctors' consideration of febrile convulsions as a simple and benign phenomenon. Fifty‐two parents whose child had been admitted with febrile convulsions were interviewed about their immediate and long‐term reactions. Most of the parents knew little about febrile convulsions before the fit. Parents with previous knowledge of febrile convulsions took more appropriate measures during the fit than parents without such knowledge. Sixty per cent of the parents slept restlessly for some time after the fit, 13% watched their child at night, and 29% had dyspeptic symptoms. Parents of young children should as a routine be offered general information by the family doctor about fever and febrile convulsions. Parents who have watched their child during a fit need specific information in order to avoid long‐term reactions.
Intestinal Colonization with Enterobacteriaceae in Pakistani and Swedish Hospital‐Delivered Infants
Authors: ADLERBERTH INGEGERD , CARLSSON BARBRO , MAN PETER DE , JALIL FEHMIDA , KHAN SHAUKAT R. , LARSSON PETER , MELLANDER LOTTA , SVANBORG CATHARINA , WOLD AGNES E. , HANSON LARS Å.
Published: January 2008

ABSTRACT. Rectal cultures from Swedish and Pakistani hospital‐delivered newborn infants were analysed regarding the early aquisition of enterobacteria. Swedish infants were delivered vaginally, Pakistani infants were delivered either vaginally or by caesarean section. The Swedish infants were all breast‐fed, whereas breastfeeding was incomplete and often started late among the Pakistani infants. Both groups of Pakistani infants were more rapidly colonized with enterobacteria than were the Swedish infants. Cultures from Swedish infants seldom yielded more than one kind of enterobacteria; E. coli and Klebsiefla were most frequently isolated. E. coli dominated in both Pakistani groups, but especially caesarean section delivered infants were in addition often colonized with Proteus, Klebsiella, Enterobacter or Citrobacterspecies. Breastfeeding from the first day of life reduced colonization with Klebsiella/Enterobacter/Citrobucter.The results suggest that environmental exposure, delivery mode and early feeding habits all influence the early intestinal colonization with enterobacteria.
Hyaline Membrane Disease and Early Neonatal Aldosterone Metabolism in Infants of Less than 33 Weeks Gestation
Authors: LESLIE GARTH I. , GALLERY EILEEN D. M. , ARNOLD JOHN D. , NICHOLSON ELLEN
Published: January 2008

ABSTRACT. We studied urine excretion of free and conjugated aldosterone by 12 control infants and 14 infants with hyaline membrane disease (HMD) on the first and seventh days after birth. Both groups had a mean gestational age of 29 weeks. Total urine aldosterone excretion (UAE) and percent excreted as conjugate were similar for both groups on both study days, and did not relate to the severity of respiratory failure in infants with HMD. Sodium intake was higher for infants with HMD on both study days (p<0.02), but their urine sodium excretion was only significantly (p<0.01) higher on day 7. For total UAE values >3 nmol/kg/d, there was no significant difference between estimated sodium‐potassium exchange by control (22 ± 5 %, n=8) and HMD (31 ± 5 %, n=10) groups. These data suggest that neither the magnitude of excretion of aldosterone in the urine, the ability to conjugate aldosterone nor the degree of relative distal tubular unresponsiveness to aldosterone are related to the severity of pulmonary immaturity in preterm infants.
Respiratory Tract Infections in Children with Recurrent Episodes as Preschoolers
Authors: SÖDERSTRÖM M. , HOVELIUS B. , PRELLNER K.
Published: January 2008

ABSTRACT. The occurrence of respiratory tract infections (RTI) in 41 school‐age children, who had recurrent RTIs treated with antibiotics as preschoolers, was followed prospectively for two years through diary reports by parents and medical consultations, and compared with that in 29 children of the same age and socio‐economic background, who had few or no such infections as preschoolers. During the two‐year follow‐up, a greater number of episodes of RTI and a longer mean duration of such episodes were reported in the diaries concerning the children with recurrent bacterial RTIs as preschoolers compared with the controls (p<0.01). The annual incidence of bacterial RTI from birth onwards decreased with age among the children with recurrent episodes as preschoolers, unlike in the control group, where the incidence remained consistently low, the difference in incidence being significant up to the age of eight years (p<0.01). Acute otitis media was the predominant bacterial RTI in preschoolers, and acute tonsillitis in school‐age children. There was a tendency toward a greater incidence of other types of disease and complication/sequelae of infections among the RTI‐afflicted group than among the controls, both as preschoolers and as school children. Our findings suggest that certain children constitute a group with high morbidity, susceptible to RTIs and other illnesses over a rather long period of years.
Repetitive Monomorphic Ventricular Tachycardia in a 4‐Year‐Old Boy with Toxic Multinodular Goiter
Authors: MINEGISHI Y. , KUMADA S. , SUZUKI H. , KUSAKA H , SHIMOZAWA K. , OKANIWA M.
Published: January 2008

ABSTRACT. A case of toxic multinodular goiter associated with repetitive monomorphic ventricular tachycardia (VT) is reported. A 4‐year‐old boy was found to have asymptomatic VT. When treatment with antiarrhythmic agents turned out to be ineffective, thyrotoxicosis was suspected due to the rapid enlargement of the left thyroid gland and associated thyroid function studies. A diagnosis of toxic multinodular goiter was made on the basis of subsequent scintigraphy and ultrasonography. Treatment with antithyroid drugs and inorganic iodine restored the thyroid function to normal, and was accompanied by the disappearance of VT. A left thyroid lobectomy was performed, and the pathological findings were compatible with toxic multinodular goiter. After the operation, the patient was transiently hypothyroid and had no VT without medication. A review of the literature revealed no previously documented cases of VT with toxic multinodular goiter.
Does VLDL‐LDL‐Cholesterol in Cord Serum Predict Future Level of Lipoproteins?
Authors: FØNNEBØ V. , DAHL L. B. , MOE P. J. , INGEBRETSEN O. C.
Published: January 2008

ABSTRACT. Lipoproteins were measured in 618 healthy, full‐term newborns. Seventy‐four with a VLD‐LDL‐cholesterol above 1.3 mmol/l (50 mg/dl) at birth and 25 randomly chosen controls with VLDL‐LDL‐cholesterol l.3 mmol/l or below at birth were followed up at age 2. Seventy‐two (52 in the high VLDL‐LDL‐cholesterol group and 20 in the low VLDL‐LDL‐cholesterol group) were followed up at age 13. At age 2 mean total cholesterol was 5.48 mmol/l (SEM 0.10) in the children with a high VLDL‐LDL‐cholesterol at birth, compared to 4.69 mmol/l (SEM 0.17) in the children with a low VLDL‐LDL‐cholesterol at birth (p < 0.001). A difference was still present at age 13 (4.74 mmol/l; SEM 0.11 versus 4.20 mmol/l; SEM 0.14; p < 0.01). At age 13 apolipoprotein B was 0.74 g/l (SEM 0.02) in the children with a high VLDL‐LDL‐cholesterol at birth, compared to 0.65 g/l (SEM 0.02) in the children with a low VLDL‐LDL‐cholesterol at birth (p < 0.01). Children with high VLDL‐LDL‐cholesterol at birth might be more liable to high lipoprotein serum levels later in life.
Clinical Manifestations in Selective IgA Deficiency in Childhood; A Follow‐up Report
Authors: LAAT P. C. J. DE , WEEMAES C. M. R. , GONERA R. , MUNSTER P. J. J. VAN , BAKKEREN J. A. J. M. , STOELINGA G. B. A.
Published: January 2008

ABSTRACT. Clinical manifestations in 40 children with selective IgA deficiency were studied during a follow‐up period of 2‐10 years. The patients were divided into two groups: group I consisted of 25 children with “sporadic” IgA deficiency and group II of 15 children with “familial” IgA deficiency. Respiratory tract infections including otitis media were frequent in both groups. Concomitant IgG,‐IgG, deficiency was found in two patients in group I. Longitudinal serum IgG levels were elevated significantly in both groups. Atopic complaints were observed in 10 children of the “sporadic” group, but only in two of the “familial” group. However, elevated serum IgE levels were more often found in group II. Two children of group I were mentally retarded and chromosomal examination showed abnormalities in both. Anti‐IgA antibodies were detected in one child in group I and three children in group II. These three patients had an IgA deficient mother with class‐specific anti‐IgA antibodies. Concomitant IgG4‐IgE deficiency was found in all four.
Mass Screening for Neuroblastoma and Estimation of Costs
Authors: NISHI M. , MIYAKE H. , TAKEDA T. , TAKASUGI N. , HANAI J. , KAWAI T.
Published: January 2008

ABSTRACT. On the basis of epidemiological data and medical costs for patients with neuroblastoma, we have calculated the cost of mass screening for neuroblastoma with high performance liquid chromatography (HPLC) compared to the cost when it is not performed. If the sensitivity of the mass screening is 80 % and 22 000 infants are screened annually the cost will be 27809000 yen ($191800). If mass screening is not performed, the cost will be 28 446 000 yen ($196 200). The difference in cost (637 000 yen or $4 400) is fairly small. If the sensitivity is 75 % and 16 500 infants are screened, the difference is also small (174000 yen or $1 200). Therefore, mass screening with the HPLC method will not be an undue financial burden. But re‐screening at an older age will be done with less financially favorable results, considering that the sensitivity may not be as high as that of the first screening and that mothers are somewhat reluctant about re‐screening. The balance of the cost of mass screening by qualitative methods may also be less favorable, since the detection rate is low.
Immobilization Related to Early Linear Growth
Authors: KARLBERG J. , HÄGGLUND G. , STRÖMQVIST B.
Published: January 2008

ABSTRACT. This study shows that children with late‐diagnosed congenital dislocation of the hip (CDH) have close to normal height development during the initial 6.0 years of life. The treatment consisted of immobilization for 0.5 to 1.3 years starting between 0.2 and 0.7 years of age. The present work addresses one specific issue that is related to the age at onset of the childhood component of the ICP growth model. The onset normally appears between 0.5 and 1.0 year of age, and is recognized as an increase in length/height velocity. The onset is thus found during a period of increasing motor activity. The normal successive change from sitting to walking position may have some influence on the onset of this tempo change in early linear growth. The present documentation implies that there is no such influence. In all 14 children with CDH, the onset manifested during the period of immobilization, and the average age at onset was found to be Virtually equivalent with that of the controls. Our conclusion is that immobilization has no significant influence on the age at onset of the childhood phase of growth. The onset is accomplished independent of body position, be it lying down or normal for the age.
Control of Protein Synthesis and Its Relationship to the Bioenergetics of Growth
Authors: FJELD CARLA R.
Published: January 2008

A key goal of nutritional therapies designed to prevent or treat protein energy malnutrition (PEM) is achievement of adequate growth rates and thus positive balances of protein and energy. Positive protein balance is the net result of protein turnover, i.e., of recycling protein through energy‐requiring anabolic and catabolic pathways. Thus, protein turnover is inexorably linked to energy metabolism and to energy balance. A long‐standing hypothesis is that new tissue synthesis is predominantly a postprandial event. Thus, postprandial rates of protein turnover may regulate the bioenergetics of growth. Traditional methods to evaluate nutritional therapies for diarrhea and PEM, i.e., metabolic balance, 15N‐labeled amino acids, fecal output, illness duration, or change in body weight, are not specific to studies of the regulation of acute protein turnover and growth by nutritional intervention. Newer techniques based on 13C‐labeled amino acids should be applied to evaluate nutritional interventions such as dietary substrate ratios, protein quality, feeding frequencies, or ratios of dietary protein to energy in terms of their abilities to optimize postprandial protein turnover and thus nutritional management of diarrhea or PEM.
Digestion and Hydrolysis of Carbohydrates in Malnutrition
Authors: LENTZE MICHAEL J.
Published: January 2008

Digestion and hydrolysis of macro‐ and micronutrients by the gastrointestinal tract are essential prerequisites for long term survival of mammals including man. Carbohydrates in particular are digested and hydrolysed by a variety of potent excretory glands products and by the brush border enzymes of the small intestine as well as by bacterial breakdown within the large intestine. A cascade of hydrolytic events leads finally to the presence of monosaccharides within the lumen of the gastrointestinal tract in order to get transported across the microvillus membrane by highly specialized transporters. Carbohydrates with high molecular weight in the form of amylose and amylopectin are hydrolysed by α‐amylase of the saliva and the pancreas. α‐Amylase can hydrolase 1‐4‐α‐glycosidic bonds which are present in amylose and partially in amylopectins. The branches of 1‐6‐α‐glycosidic side chains in amylopectins remain after the action of amylase as α‐limit‐dextrins and are further hydrolysed by sucrase‐isomaltase (1). Hydrolysis of starches is dependent upon the age of the infant. In the first 6 months of life, activity of α‐amylase is low and reaches full activity at the end of the first year of life (2). Defects in sugar digestion are possible by disturbances within the combined action of pancreatic α‐amylase and that of intestinal brush border enzymes. Decreased digestion and hydrolysis of carbohydrates will induce either osmotic diarrhea and/or bacterial overgrowth within the small intestine as well as bacterial breakdown of carbohydrates within the colon. Malnutrition is very often combined with chronic diarrhea and damage of the gastrointestinal mucosa as a consequence of lack of protein and energy. Key factors in this devastating cascade are the brush border membrane of the small intestine and its hydrolyzing and absorptive capacity. Important observations with regard to the hydrolytic capacity of intestinal disaccharidases which are responsible for sugar hydrolysis have come through the study of their intracellular pathways and processing of enzyme molecules in normal and altered human as well as animal mucosa using various techniques of molecular biology. The knowledge of these investigations has considerably increased our understanding as to how primary and more important secondary deficiencies of brush border membrane (BBM) hydrolases are defined on a molecular level. In order to understand the pathology of intracellular misarrangements of BBM‐hydrolases the normal biosynthesis and processing of these microvillar proteins will be discussed briefly.
Ethnic Minorities, Migration and Risk of Undernutrition in Children
Authors: PELTO G. H.
Published: January 2008

Well‐controlled, comparative studies suggest that in “food rich” or “resource rich” environments, ethnic minority status is not an independent risk factor for malnutrition. In England, Australia, and the United States, nutrient intake, nutritional status measures and growth are comparable between children of ethnic minorities and non‐minority children of similar economic status. Children of recent immigrants are an exception to this generalization, suggesting that recent arrival in a new environment is a risk factor for malnutrition. Family‐level studies of intraethnic diversity are required to develop more sensitive and specific profiles of risk factors for malnutrition.
Malnutrition in the Premature Infant
Authors: ZIEGLER EKHARD E.
Published: January 2008

During the first few days of life, the ill premature infant is usually subjected to acute semistarvation because the provision of nutritional support is considered cumbersome and unnecessary. However, the absence of readily recognizable adverse effects of semistarvation does not rule out the existence of significant short‐term adverse effects, nor does it rule out possible adverse sequelae in the long run. Similar concerns pertain to the later neonatal period, during which nutritional deprivation is less severe but of longer duration. Evidence is presented that qualitative malnutrition, characterized by inadequate intake of protein and relatively excessive intake of energy, is common with current feeding regimens and is responsible for increased body fat deposition in growing small premature infants.