bluuebunny/crossref_metadata_2025 huggingface dataset:
Weight and Length of the Newborn during the Years 1918–1922 and 1938–1942
Authors: ABOLINS J. A.
Published: January 2008

In several countries it has been possible to prove a decrease in the weight and length of the newborn during the two World Wars and the post‐war years.

My investigation of the birthweight and the length of newborn infants in Sweden comprises 13,697 live‐born infants with a birthweight of more than 2,500 g. The infants were born during the periods 1918–1922 and 1938–1942. Multiple births and infants born by mothers suffering from diseases such as diabetes or toxicosis which might have affected the weight and size of the infants were excluded from the material.

The investigation did not prove any statistically significant change in the weight and length of infants, at birth, during the periods studied.
Reflexes of Spinal Automatism in Internal Hydrocephalus
Authors: ASK‐UPMARK ERIK
Published: January 2008

1. A brief review is given of the diagnostic approach in neurology as practised in this department. 2. A case is described where pronounced spinal automatic reflexes were to be recorded. The disorder in this case was a hydrocephalus internus. When this condition was neurosurgically adjusted the reflexes returned to normal. The word “spinal” should accordingly only be used cum granu salis when these reflexes are concerned. 3. Attention is called to the possibility that an originally partial obliteration of the aqueduct may be turned into a complete obliteration by means of the pressure exerted upon midline‐structures by the distended cerebral hemispheres. 4. The strikingly short distance between inion and the processus spinosus of the epistropheus may be suggestive of the aqueduct as the seat of the obliteration.
Factors Influencing the Serum Haptoglobin Level in Infancy
Authors: BERGSTRAND C. G. , CZAR B. , TARUKOSKI P. H. , FÜRST P.
Published: January 2008

Some factors which possibly influence the serum haptoglobin levels in infancy were investigated with Jayle's activation method which measures the peroxidase activity of the haemoglobin‐haptoglobin complex. The following results were obtained. Sampling haemolysis, not visually detectable, has little influence on the determinations. Bilirubin added in a moderate concentration to serum samples decreases the peroxidase activity but the influence is of little practical importance except possibly at very low haptoglobin levels. Cord serum added to a normal serum does not decrease the peroxidase activity of the latter. It is concluded that cord serum does not contain to an appreciable amount substances inhibiting the formation of the haemoglobin‐haptoglobin complex or its peroxidase activity.

The possible existence in cord serum of haptoglobins forming a complex with haemoglobin without peroxidase activity could not be demonstrated with a specially devised starch gel electrophoretic method.

It was shown that infants 2 to 5 months old on an average have a higher serum haptoglobin level during acute infections than healthy infants of the same age. It is possible that the same holds true also for infants younger than 2 months. This was, however, not proved.
Infections and Staphylococci on the Maternity Ward
Authors: CEDERGREN B. , LAGERCRANTZ R. , NORÉN L.
Published: January 2008

The incidence of infections which might have been caused by hospital spread among mothers and infants in the maternity clinic of Karolinska Sjukhuset was studied. The infections were of the type which were caused by S. aureus, and generally developed within three weeks after delivery. During a second period, 56% of the patients and 54% of the personnel were carriers of S. aureus. Mode of infection was studied by determination of phage and resistance patterns. The infections' epidemiology was discussed. It was probable that the infection spread occurred primarily from personnel to infants. The relatively high incidence of infections and high carrier‐rate requires prophylactic measures.
The Cerebrospinal Fluid in Immature Infants
Authors: GYLLENSWÄRD ÅKE , MALMSTRÖM STIG
Published: January 2008

The cerebrospinal fluid (CSF) of 36 healthy immature infants was examined with regard to cell count and content of protein and bilirubin. The cell count was high, falling with increasing age. The individual variations were very great, and counts as high as 112/3 mm3 were recorded during the first days of life. There was no difference in this respect between small and large immature infants. The CSF protein level was highest in the smallest infants (maximum 292 mg/100 ml). No correlation could be demonstrated between the bilirubin levels in serum and CSF, or between the general condition of the infant and the serum bilirubin level. A high CSF redcell count was frequently recorded, although there were no signs of (cerebral) haemorrhage.
Peripheral Nerve Changes in the Diagnosis of Metachromatic Leucodystrophy
Authors: HAGBERG BENGT , SOURANDER PATRICK , THORÉN LARS
Published: January 2008

A histopathological study was made on peripheral nerves and spinal roots from biopsies and autopsies of 8 cases with metachromatic leucodystrophy (sulphatide lipidosis), 4 cases of globoid cell leucodystrophy (Krabbe's disease), 9 cases with other progressive neurological disorders of infancy and childhood and 4 controls. The sural nerve was found to give satisfactory biopsy specimens and a suitable biopsy technique is described. At histological examination, brown metachromatic deposits were revealed by the cresyl violet‐acetic acid method of v. Hirsch & Peiffer in all cases with metachromatic leucodystrophy, but were never observed in any of the other cases examined. The granular metachromatic material was localized mainly to Schwann cells and histiocytes which were accumulated in clusters around capillaries of the endo‐ and perineurium. For diagnostic purposes the most reliable and so far pathognomonic change in peripheral nerve biopsy specimens was considered to be the demonstration of brown metachromatic granular deposits in perivascular phagocytes. It is concluded that histological examination of peripheral nerves can serve as a useful supplementary tool in the diagnosis of this genetically transmitted degenerative disorder during life.
Growth in a Series of Diabetic Children on Identical Treatment with “Free” Diet and Insulin 1944–1960: A Contribution to the Aetiology of Diabetic Dwarfism
Authors: HAMNE BERTIL
Published: January 2008

An account is given of growth in a series of 195 cases of juvenile diabetes treated on a standard regimen (“free” diet + insulin) and collected during the period 1944–1960. Scrutiny of the series shows that growth was as a rule normal. Undersized children usually had small parents. A few cases of retarded growth may however be explained by excessive feeding with relative insulin deficiency. Two cases showing the classical picture of diabetes dwarfism with hepatomegaly and infantile mentality are described. Both were inadequately controlled, owing to ignorance on the part of the parents. These cases support the view that the condition is caused by relative insulin deficiency. The theory is further borne out by the improvement that took place in one of these children when she was finally brought under adequate control.
Congenital Analgia: A Case Report
Authors: HELLSTRÖM BO
Published: January 2008

A case of congenital analgia in a 21/2 year old girl is described. In addition to the typical findings of a generalized indifference to potentially painful stimuli without any other significant neurological findings an EEG discloses a marked, bilateral epileptogenic abnormality. The girl has so far not had any signs of clinical epilepsy. The findings are discussed and a short review of the syndrome is presented.
Age Variations in the Concentration of Non‐Protein Nitrogen, Creatinine and Urea in Blood of Infants and Children
Authors: JOSEPHSON BERTIL , FÜRST PETER , JÄRNMARK OLLE
Published: January 2008

Non‐protein nitrogen as well as serum urea and creatinine concentrations were determined in 113 healthy children ranging in age from newborn to six years. The results were compared with those from corresponding analyses of adult blood.

NPN and creatinine levels at birth were found to approximate those in adults but to decrease within five or six days to values significantly lower than the adult ones. This confirmed reports published much earlier. It was found, in addition, that at an age between about five and seven months the NPN concentration rose rather abruptly to the adult range. The question as to whether this phenomenon resulted from the child's natural development or whether it stemmed from the increased protein intake, is discussed.

The serum concentration of urea at birth was found to be significantly lower than the adult value. That this was attributable to a low urea concentration in the maternal blood is a possibility which cannot be ruled out. As with NPN and creatinine, the concentration of urea increased to the adult range at an age of five to seven months.

The normal means and concentration ranges for NPN, creatinine, and urea‐N in blood from children of different age groups are tabulated.
Idiopathic Hypercalcemia. A Disorder of the Calcium Homeostatic Mechanism1
Authors: LINDQUIST BERTIL
Published: January 2008

A review on pathogenetic factors related to idiopathic hypercalcemia is presented. Certain discrepancies between the clinical picture of hypervitaminosis D and that of idiopathic hypercalcemia are pointed out. On the basis of metabolic and clinical data, obtained in cases of idiopathic hypercalcemia on one side and present knowledge of fundamentals of calcium metabolism on the other, the hypothesis is advanced that idiopathic hypercalcemia is caused by a disturbance of the homeostatic mechanism controlling the intestinal absorption of calcium rather than by a state of vitamin D overactivity.
Effect of Glucagon on the Plasma Level of Free Fatty Acids
Authors: PERSSON BENGT , LARSSON YNGVE , STERKY GÖRAN
Published: January 2008

The effect of intravenously administrated glucagon on the plasma level of free fatty acids (FFA) and on blood sugar was studied in 24 girls, 11–19 years old, of which 10 had diabetes, 8 had obesity and 6 were controls. Fasting levels of FFA were significantly higher in the diabetes and obesity groups than in the control group. After glucagon there was a decrease of FFA which was more evident in the control and obesity groups than in the diabetes group. The blood sugar response was moderate and identical among the obese and the controls, but more marked and more prolonged among the diabetics. The mechanism behind the effect of glucagon on plasma FFA is discussed.
Anomalies of Midgut Rotation and Fixation Clinical Aspects Based on Sixty‐ Two Cases in Childhood
Authors: SÖDERLUND SIGRID
Published: January 2008

The series reported here was collected at the Pediatric Surgery Department of Kronprinsessan Lovisas Barnsjukhus, Stockholm, during the period 1946–1960. It comprises 62 children ranging in age from newborn to 13 years. Five different stages of anomalies of rotation and fixation are described. The series is grouped accordingly and each group reported. The anomalies were almost twice as common in boys as in girls. Of the numerous concomitant malformations detected, omphalocele was most frequent, being present in 13 cases. In 29 cases the clinical symptoms were attributable to the anomalies of rotation and fixation; in the remainder the anomalies were incidental findings. The disease usually gave rise to symptoms in the neonatal period, 18 infants being hospitalized during the first few weeks of life. Of the various types observed, mixed rotation was the most common. The symptoms were produced by obstruction of the duodenum or by intestinal volvulus, these conditions giving rise to signs of high obstruction. The onset was generally acute in children less than one year of age but insidious in older children, whose disease assumed a chronic course, particularly when associated with intermittent volvulus. Vomiting and abdominal pain were the commonest symptoms, though in rare cases they were absent. In the infants less than one year of age suspected pylorospasm was the most frequent cause of delayed surgical intervention. Only in one case were vomiting and abdominal pain found to have been due to mobile cecum. This patient had a cecal volvulus. Diagnostically, barium enema was the examination of choice; barium meal examination yielded no definite information and was sometimes misleading. The prognosis was poor in the younger age groups and when coincident malformations or volvulus occurred. Eighteen patients died, all being under 4 months of age. There were 5 children without associated malformations who died: four of them had volvulus. Early diagnosis and early operation are of prognostic significance. When vomiting occurs in children, particularly during the neonatal period, plain radiograms of the abdomen plus barium enema examination should be performed in order to establish the correct diagnosis.
Study of Systolic Blood Pressure, Heart Rate, Body Temperature of Normal Newborn Infants through the First Week of Life
Authors: CONTIS GEORGE , LIND JOHN
Published: January 2008

Forty fullterm newborn infants, after uncomplicated pregnancies, uneventful labor and spontaneous delivery, were studied. The heart rate, systolic blood pressure (according to Ashworth & Neli‐gan) and body temperature were recorded 15 and 30 min and 1, 2 and 3 hours after birth, as well as on the morning of each of the following six days.

A significant decrease in systolic blood pressure, heart rate and body temperature occurs during the first hours of life. The drop in body temperature and heart rate is most rapid during the first period of observation between 15 and 30 min of life, but the main drop in blood pressure occurs between 30 and 60 min after delivery. The decrease in blood pressure cannot be prevented by maintaining a stable body temperature in the infant.

From the second to the seventh day, the temperature is relatively level, while the blood pressure and heart rate rise significantly.
Vitamin E to Premature Infants
Authors: DYGGVE HOLGER V. , PROBST JAN H.
Published: January 2008

Lower average bilirubin levels, higher hemoglobin and more normal serum alkaline phosphatase values were found in 67 premature infants who received 100 mg a‐tocopherol intramuscularly at birth, than in a control group. The results are discussed, and the routine administration of ‐vitamin E to premature infants is suggested.
Normal Conduction Velocity of Ulnar, Median and Peroneal Nerves in Infancy, Childhood and Adolescence
Authors: GAMSTORP INGRID
Published: January 2008

The conduction velocity of the ulnar, median and peroneal nerves was studied from birth to adult age in normal infants, children and adolescents. The method allowed measurements of the conduction velocity of the thickest and fastest conducting motor nerve fibres. In newborns the conduction velocity of all 3 nerves was roughly half of that of adults. The value for the ulnar nerve increased rapidly during the first 3 years. After that age it rose only slightly and between adolescence and adult age there was a significant reduction. The value for the median nerve increased slowly before one year of age. At this time a rapid acceleration started and continued through adolescence. The value for the peroneal nerve increased rapidly through infancy, while small changes were noted after one year of age. The curve for the ulnar nerve was in all age‐groups above the other 2. The curve for the median nerve started at the same level as that for the peroneal, ran below it through infancy and early childhood, crossed it in the age‐group 3–8 years and approached that of the ulnar nerve in adolescence and adult age. These results are discussed in relation to the anatomical maturation of the peripheral nervous system and to the motor performance of the child.

An H reflex of the ulnar nerve, suggesting immaturity of the pyramidal tract, was present in some of the infants below 7 months of age.
Mongolism and Other Abnormalities in a Family with Trisomy 21–22 Tendency
Authors: HALL BERTIL
Published: January 2008

An example of trisomy 21–22, idiocy and some malformations is described and compared with the other non‐mongoloid trisomies 21–22. The difficulty is discussed of making a correct morphological identification of the chromosome in the smallest chromosome group. The present patient resembles two previously reported non‐twin sisters, both having an extra chromosome similar to, although not quite identical with, the chromosomes in group 21–22. In the present patient's family, there has also been more than one instance of trisomy. A hypothetical explanation of a non‐disjunction tendency in families with supposed translocations and in families with normal karoytypes is given.
A New Method of Pituitary Exploration and Its Use with Children: The Metopirone Test
Authors: LELONG MARCEL , CANLORBE PIERRE
Published: January 2008

From these observations, it emerges that the Metopirone test is a valuable method of establishing, given an endocrine or metabolic syndrome, the existence of a hypophyseal insufficiency. This appears to us to be of particular interest in pediatrics for a diagnosis of cases of arrested development and dwarfism where a hypophyseal cause often appears possible, but not certain. A negative or diminished response to Metopirone establishes almost invariably the hypothalamo‐hypophyseal origin of retarded growth, though a normal response does not exclude this etiology, for the Metopirone test confirms and specifies the existence in the child of dissociated anterior hypophyseal insufficiencies.
Intestinal Transport of Monosaccharides in Generalized and Selective Malabsorption
Authors: LINDQUIST BERTIL , MEEUWISSE GUNNAR W.
Published: January 2008

Intestinal intubation studies were performed in one normal child, two cases of glucose‐galactose malabsorption and one case of celiac disease. The absorption of glucose and fructose in the upper intestinal tract was measured simultaneously after administration of a liquid test meal containing PEG as a reference substance.

In glucose‐galactose malabsorption the fructose absorption was of the same order as in the normal subject, and was considerably higher than the glucose absorption, resulting in an inverse relationship between the absorption rates of fructose and glucose.

In celiac disease the absorption of fructose as well as glucose was decreased. The ratio of the absorption rate of fructose to that of glucose was not disturbed.
Thermovision — Heat‐Picture Camera
Authors: BLOMQUIST B. , MÖLLER T.
Published: January 2008

AGA's Thermovision has been used in of thermatic pictures during different paediatric material for the reproduction kinds of disease.
Kidney Glycolipids in Late Infantile Metachromatic Leucodystrophy
Authors: MÅRTENSSON ERIK , PERCY ALAN , SVENNERHOLM LARS
Published: January 2008

1. Micropreparations from kidneys of children with metachromatic leucodystrophy were analyzed for neutral glyco‐lipid and sulfatide patterns.

2. Sulfatides, neutral glycolipids, and He has supported his hypothesis by the isolation of a sulfatide sulfatase from kidney and by the demonstration of sulfatase activity in many other organs, including brain [7]. He has also presented evidence for a decreased activity of this enzyme in metachromatic leucodystrophy. The available data can then be interpreted according to the following scheme: gangliosides were isolated from a single case of metachromatic leucodystrophy and from a pooled sample of normal kidneys.

3. The sulfatide content of the pathological material was increased 70‐fold over normal. The concentration of total neutral glycolipids and gangliosides were similar in normal and pathological material, but definite variations in the ganglioside TLC pattern were detected.

4. Sulfatide fatty acid analyses differed from those of normal material. There was an increase of the hydroxy acids and of the normal C‐acids, and a decreased degree of unsaturation. Possible mechanisms for these changes are discussed.

5. Metabolic pathways of sulfatides and cerebrosides are discussed in relation to the disease mechanism.
The Circulatory and Respiratory Adaptation to Early and Late Cord Clamping in Newborn Infants
Authors: OH WILLIAM , LIND JOHN , GESSNER IRA H.
Published: January 2008

Time of first breath and frist cry, systolic blood pressure, pulse rate and respiratory rate of 32 newborn infants whose umbilical cords were clamped early and 30 whose cords were tied late after birth were studied. The early clamped infants breathed significantly sooner after birth than the late clamped infants, probably a result of anoxia due to severance of placental blood supply by immediate cord clamping. The indirectly measured sys‐ tolic blood pressure was significantly higher in the late clamped group of infants during the first 24 hours of life perhaps due to the difference in blood volumes between the two groups. This is further supported by a positive correlation found between systolic blood pressure and venous hematocrit during the first 4 hours of life. Pulse rate in the two groups of infants studied revealed no significant difference. The respiratory rate in the early clamped group of infants was significantly slower during the 1st to the 3rd hour of life when compared with the late clamped infants. No satisfactory explanation could be offered for this observation.
Nitrogen and Fat Retention in Premature Infants Fed Breast Milk, “Humanized” Cow's Milk or Half Skimmed Cow's Milk
Authors: ZOULA J. , MELICHAR V. , NOVÁK M. , HAHN P. , KOLDOVSKÝ O.
Published: January 2008

Three types of milk diet were tested in balance experiments using premature infants. They were: breast milk, “humanized” milk and half skimmed cow's milk containing more protein and less fat than the other' two milks.

It was ascertained that protein retention was about the same on all three diets, while nitrogen elimination via the urine was much higher on the half skimmed milk. Fat retention was lowest on half skimmed milk, but this may have been due to the low fat content of that milk.
Adolescent Colloid Goitre
Authors: NILSSON LARS R.
Published: January 2008

Adolescent goitre is generally conceived as a special form of goitre induced by an increased hormone requirement during puberty. But goitre a t puberty is not a uniform condition. Some causes of goitre are known with certainty, such as iodine deficiency and auto‐immune thyroiditis. The present material of “idiopathic” adolescent goitre consisted of 39 patients (37 females, and 2 males) aged 9 to 17 years. The patients were euthyroid and largely without symptoms except goitre. The enlargement of the glands was usually small to moderate. The glands were homogeneous, of elastic consistence and had a smooth, even surface. Cytologically the goitres were characterized by active secretory follicular epithelium and abundant colloid. The uptake of radioiodine by the thyroid varied, but was usually near or just above the upper normal limit of the normal range. The amount of radioactive hormone iodine.in the plasma was, however, not increased, but, if anything, decreased.

Follow‐up was not long enough t o allow any valid conclusions about the definitive prognosis of the condition. So far, half of the goitres have decreased or disappeared but several show no signs of spontaneous regression.

A familial predisposition to thyroid disease was noted. The idiopathic adolescent goitre was not conceived in the present investigation as an enlargement of the gland due to compensatory hyperplasia but as a colloid goitre on the basis of unknown structural defect or functional disorder of the gland and under the influence of some obscure factor or factors released or activated a t puberty.
Chromatographic Pattern of Serum and Urinary Amino Acids in Cretinism
Authors: GABR MAMDOOH , EL‐GAWAD ZENAB ABD , EL‐BEHAIRY FOUAD
Published: January 2008

Summary and Conclusion

The amino‐acid chromatographic patterns of the serum and urine in 23 cases of congenital hypothyroidism were studied semi‐quantitatively. An increase in serum amino‐acids was present in all cases affecting mainly glutamic acid, glycine, alanine and valine. The increase was less evident in milder cases. Urinary amino‐acids were within the normal range. Adequate thyroid replacement therapy in 13 cases resulted in lowering of the hyperamino‐acidemia to the normal range in 11 cases. The possible actions of thyroid hormone on the plasma amino‐acid level are presented and the results are discussed.
Congenital Pulmonary Lymphangiectasis
Authors: EKELUND H. , PALMSTIERNA S. , ÖSTBERG G.
Published: January 2008

A typical case of congenital pulmonary lymphangiectasia is described and compared with earlier published cases. It is a rare malformation, only about 25 cases hitherto being described, The clinical picture in liveborn infants is characterized by severe respiratory distress appearing a short time after a delivery.
Zytologische Untersuchungen zur Frage des Zellmosaiks bei heterozygoten Frauen mit Glukose‐6‐Phosphatdehydrogenase‐Mangel
Authors: BETKE Von K. , KLEIHAUER E. , KNOTEK Z.
Published: January 2008

Bei der Durchführung des Brewer‐Testes mit Blut von heterozygoten Frauen mit G‐6‐PD‐Mangel nahm im zytologischen Praparat die Zahl der methamoglobin‐haltigen Zellen im Ablauf der Zeit konti‐nuierlich ab, die der oxyhämoglobinhaltigen Zellen zu. Die Befunde sind nicht geeignet, die Existenz zweier definierter Zellpopulationen zu beweisen. In gleicher Weise verhielten sich jedoch künstliche Mischungen von Normalblut mit Blut hemizygoter Trager der Anomalie. Offen‐sichtlich leisten die normalen Erythro‐zyten den defekten Erythrozyten eine metabolische Hilfe. Diese wird wahrschein‐lich durch Leukomethylenblau vermittelt. Wenn Nilblausulfat statt Methylenblau verwendet wurde, war eine Existenz zweier Zellpopulationen im Blut heterozygoter Erauen und in kiinstlichen Mischungen besser erkennbar. Unter standardisierten Bedingungen ist die Kombination des Brewer‐Testes (mit Nilblausulfat durch‐geführt) mit der zytologischen Auswertung eines nach 4 Stunden hergestellten Aus‐striches eine wertvolle Hilfe fur die Er‐kennung von Heterozygoten.

During the course of the Brewer test in blood of heterozygous females with G‐6‐PD‐deficiency the count of methaemoglobin containing cells decreased with time, while the count of oxyhaemoglobin containing cells increased. There was, therefore, no clear‐cut evidence for the existence of two definite cell populations as postulated by the Lyon‐hypothesis [9]. However, the same findings were seen in artificial mixtures of normal blood with blood of hemizygous males. Apparently the normal cells give metabolic aid to the deficient cells. This is probably mediated by leukomethylene blue. With Nile blue sulfate instead of methylene blue the evidence for the existence of two cell populations was somewhat better. Under standardized conditions the combination of the Brewer test (done with Nile blue sulfate) with the cytological evaluation of a smear prepared after 4 hours is a valuable diagnostic aid for the detection of heterozygotes.
Body Temperature of the Newborn Infant in Relation to Placental Transfusion1
Authors: OH WILLIAM , LIND JOHN
Published: January 2008

Temperatures of the rectum, skin areas of the heels, palms, earlobes and epigastrium were serially measured in 36 normal full‐term newborn infants from birth through the 5th day of life. The umbilical cords of 14 infants were clamped immediately after birth, while in 22 infants the cords were tied after arterial pulsation had stopped. At birth the mean skin temperatures of heels and palms dropped precipitously from 35.4oC to 29.8oC during the first 10 minutes of life. The heel and palm temperatures showed a further decline during the first 4 hours of age, while the earlobe and epigastrium temperatures gradually increased at the same time.

The early clamped infants had a significantly lower skin temperature at the palms, heels and to a lesser extent at the earlobes. This is considered as indirect evidence of lower peripheral skin blood flow in this group of infants. No difference was observed in the cutaneous epigastric and rectal temperatures between the two groups.
Biochemical Genetics in Medicine1
Authors: MOTULSKY ARNO G.
Published: January 2008

1. The history of human biochemical genetics is reviewed with special references to Garrod's and Pauling's contributions.

2. Recent knowledge on the correlation of gene structure and protein structure is reviewed.

3. The influence of various mutations on the hemoglobin molecule is discussed. Heterozygote structural mutations affecting the critical active site of the hemoglobin molecule may lead to hemolytic anemia, drug sensitivity, polycythemia and methemoglobinemia. Other mutants are usually harmless. Another class of hemoglobin mutants–the thalassemias–cause diminished hemoglobin chain production and may lead to severe anemia in the ho‐mozygote state.

4. Many different glucose‐6‐phosphate dehydrogenase mutations have been discovered. Some are rare mutants, others are common and, as the sickle trait, owe their frequency to a selective advantage vis‐a‐vis falciparum malaria. The African type of G6PD mutant is caused by a structural mutation which causes more rapid degradation of the enzyme during red cell ageing. The chemically most unstable mutants are associated with chronic hemolytic disease.

5. Mutations of controlling elements as contrasted to structural mutations have not yet been proven in man, but hereditary persistence of fetal hemoglobin and oroticaciduria can best be explained by control mutations.

6. Differences in drug response and drug reactions may be caused by genetically determined biochemical variability. G6PD deficiency may cause drug hemolytic anemia. Pseudocholinesterase alterations may cause sensitivity or resistance to succinylcholine and differences in the acetylation of some drugs are genetically controlled.

7. Biochemical screening is feasible for a variety of disorders and is strongly indicated since certain forms of mental retardation may be prevented by early treatment, e.g., phenylketonuria.

8. Carrier detection of many heterozygote traits is possible. Overlap in enzyme activity between heterozygotes and normals may be caused by the existence of isoalleles in the normal population. Screening of heterozygotes of X‐linked diseases (muscular dystrophy and hemophilia) is practically more important in family counseling than screening for heterozygotes of autosomal diseases.
Familial Leukaemia — Three Cases of Acute Leukaemia in Four Siblings
Authors: LUNDMARK K. M. , THILÉN ASTRID , VAHLQUIST B.
Published: January 2008

An instance of familial leukaemia is described where 3 children out of 4 died of acute lymphocytic leukaemia at the ages of 2–4 years. Two of the siblings were twins, probably binovular. Genetic mechanisms in the aetiology of acute leukaemia are discussed and the pertinent literature is briefly reviewed.
Hemangioma of the Spleen with Thrombocytopenia and Fibrinogen Deficiency
Authors: ZERVOS N. , VLACHOS J. , KARPATHIOS T. , MANTAS J.
Published: January 2008

A case of giant splenic hemangioma associated with thrombocytopenia and fibrinogen deficiency is described. The case presented clinically with severe bleeding and splenomegaly. Treatment with prednisone and transfusions of fresh blood and fibrinogen, was unsuccessful in controlling the hemorrhage. Following splenectomy, the patient improved considerably, the bleeding tendency disappeared and the blood findings became normal. The relationship between giant heman‐giomata, either visceral or cutaneous, and the disturbed mechanisms of blood clotting is discussed.
TIME STUDIES ON FREE AMINO ACID LEVELS OF VENOUS PLASMA DURING THE NEONATAL PERIOD
Authors: LINDBLAD B. S. , BALDESTEN A.
Published: January 2008

A simplified method of ion‐exchange chromatography of essential amino acids on small amounts of human plasma is described. The precision of the instrument and the reproducibility was to be compared with those achieved with automatic amino acid analyzers. The method has been applied in a survey of venous plasma free amino acid levels and urinary concentrations during the neonatal period.

A considerable general decrease of the free amino acid levels was seen already during the first postnatal day. This was most marked in the branch‐chained amino acids, where levels that were low for infants were seen already after the first hours of starvation. The well known high urinary excretion of amino acid nitrogen was found to be due to a high excretion of a limited number of amino acids.
MEASLES VACCINATION
Authors: NORRBY ERLING , LAGERCRANTZ RUTGER , GARD SVEN
Published: January 2008

Children immunized with 4 doses of formalin‐killed (FK) whole virus vaccine containing adjuvant and aqueous, purified hemagglutinin prepared from Tween‐ether (TE) treated material in different combinations have been followed serologically and clinically during a period of 3 to 4 years after the last dose of vaccine. The average decline in HI antibody titers was about 10‐fold during this period of time, except in children who had received 4 doses of TE vaccine. The latter group displayed about a 40‐fold decrease in titers. Four out of 10 children exposed to cases of regular measles displayed clinical symptoms of varying degrees of severity. Two more children responded with a rise in HI antibody titer only. All these reactions occurred in children with a pre‐exposure HI serum titer of 160 or higher. A case of pneumonia was encountered in connection with exposure to wild virus in a child who had received only 2 doses of FK vaccine.
CHRONIC AUTOIMMUNE HEMOLYTIC ANEMIA IN CHILDHOOD WITH COLD ANTIBODIES, APLASTIC CRISES, AND FAMILIAL OCCURRENCE
Authors: SEIP M. , HARBOE M. , CYVIN K.
Published: January 2008

A report is given of two sisters who developed chronic autoimmune hemolytic anemia in their fourth and twelfth years of age, respectively. The serological findings were different in the two cases. In patient 1, a typical cold agglutinin with a titer of 256 at 4oC was found in the serum, and only complement on the red cells by the direct Coombs' test. The serum hemolysed trypsinized red cells at 37oC. Her sister had warm type hemolytic anemia with a γG antibody on her red cells. In case 1, the clinical course has been severe, with several exacerbations of the hemolytic process accompanied by erythroid aplasia in the bone marrow.

Whereas acute autoimmune hemolytic anemia with cold antibodies is not rare in children, and may be elicited by mycoplasma or viral infections, chronic autoimmune hemolytic anemia is rarely seen before adulthood. The typical, chronic cold hemagglutinin disease with hemoglobinuria and Raynaud's phenomena has not been described in childhood, and was not present in our case. It is suggested that the disease in the case presented may have started as the result of an acute infection, and that for one reason or another (genetic predisposition?) the process has not shown the usual self‐limited course.

Although a familial–probably genetic– predisposition exists for several autoimmune diseases, familial occurrence of autoimmune hemolytic anemia has rarely been observed. The authors have found nine convincing reports in the literature, and in only one of the reported families did the hemolytic anemia develop in childhood. In families predisposed to autoimmune diseases, a variety of γ‐globulin disturbances and autoimmune disorders are often found. The presence of a more fundamental, genetic aberration of the immune apparatus, preventing a normal immune homeostasis, may therefore be postulated.

A brief comment is made on the possible mechanism of the repeated periods of erythroid aplasia in the patient with cold antibodies. It is suggested that a second antibody, causing hemolysis of trypsinized red cells at 37oC may be of importance by damaging the red cell precursors. The presence of this antibody may also possibly explain why the severity of the clinical picture of the patient did not seem to be related to exposure to cold.
THE EFFECT OF ANTIMICROBIAL AGENTS ON THE BINDING OF BILIRUBIN BY ALBUMIN
Authors: MALAKA‐ZAFIRIU KETI , STRATES BASIL S.
Published: January 2008

The effect of antibiotics, on the albumin binding capacity was studied by adding various concentrations of each drug in a standard solution of crystalline human serum albumin 4 g/ 100 ml, according to method of Porter & Waters (8). We found significant depression (above 25 %) of binding capacity of the albumin after addition of the following drugs in concentrations reaching therapeutical blood levels: Novobiocin and Sulfonamides (Elkosin, Sulfexin, Gantrisin).

Moderate depression (11‐25 %) was observed with the following drugs: Oxacillin, Ce‐phalothin and Rifamycin.

Little or no decrease of the albumin binding capacity was found after the addition of the following drugs: Penicillin, Penetracin, Methi‐cillin (Staphcillin, Celbenin) Ampicillin, (Pen‐trexyl, Penbritin), Cloxacillin, Lincomycin, Cephaloridin, Streptomycin, Colimycin, Chloramphenicol succinate, Erythromycin, Tetra‐cyclin hydrochloride, Pyrolidynomethyl‐tetra‐cyclin and Oxytetracyclin.
BALANCE OF NET ACID IN GROWING INFANTS
Authors: KILDEBERG P. , ENGEL K. , WINTERS R. W.
Published: January 2008

A method for the quantitative assessment of the balance of net acid (NAB) in growing infants with a changing body composition is described. Results of seventy measurements of the daily NAB in healthy growing premature infants ingesting modified cow's milk formulae are reported. The relative contributions of the various determinants of the daily net acid input in the infant differ from those in the normal adult, and growth appears to be associated with negative net acid balances due to base deposition in skeleton and new body water. The daily load of “undetermined anion” (UA) in the diet was found to be the largest single component of the NAB. Moreover, a strong correlation between the rates of dietary UA intake and fecal UA excretion suggested an active regulation of the transintestinal acid‐base balance. A model for such regulation, based upon available evidence concerning the transport of acid and base across the gastrointestinal membrane, is proposed and briefly discussed.
IDENTICAL SYNDROMES OF CEREBRAL PALSY IN THE SAME FAMILY
Authors: GUSTAVSON K.‐H. , BERG B. HAG , SANNER G.
Published: January 2008

Familial cases of cerebral palsy were traced all over Sweden. Fortythree families were collected, in 30 of which the patients were siblings. The families were divided into three groups: (1) 16 families with cases of identical syndromes and a history of normal pregnancy, delivery and perinatal period; (2) 3 families with cases of identical syndromes but an abnormal perinatal period; (3) 24 families with non‐identical syndromes.

Within the first group, which is of main genetic importance, 10 families were found with 2‐3 siblings affected with congenital non‐progressive ataxia and mental retardation, the mode of inheritance with all probability being autosomal recessive. Three families showed ataxic diplegia, two of them only in siblings, the third with affected members of both sexes represented in three generations. Surprisingly enough, pure spastic diplegia was only revealed in one family, viz. a grandfather and his grandson. Spastic tetraplegia was found in two mentally retarded siblings in an otherwise healthy sibship of 11 members. True microcephaly combined with a dystonic tetraplegic cerebral palsy was seen in one family and was thought to have an autosomal recessive inheritance as in similar cases reported in the literature.

Chromosome studies and laboratory screening tests revealed no abnormalities indicating particular aetiological mechanisms.
ENZYMATIC STUDIES IN A CASE OF HEREDITARY TYROSINEMIA WITH HEPATOMA
Authors: GENTZ J. , HEINRICH J. , LINDBLAD B. , LINDSTEDT S. , ZETTERSTRÖM R.
Published: January 2008

The activity of δ‐aminolevulinic acid synthetase has been measured in liver and hepatoma tissue from a case of hereditary tyrosinemia with an increased excretion of δ‐aminolevulinic acid in the urine. The enzyme activity in the hepatoma tissue was seven times higher than in the surrounding liver, where the activity was not increased above the reported normal range. The activity of p‐hydroxyphe‐nylpyruvate hydroxylase (EC 1.14.2.2.) was about 25 % of the normal in the liver tissue but not measurable in the hepatoma. Tyrosine aminotransferase (EC 2.6.1.5.) activity was normal in the liver tissue but was not detectable in the hepatoma, whereas the activity of phenylpy‐ruvate keto‐enol isomerase (EC 5.3.2.1.) was the same in liver and hepatoma tissue.
BICYCLE ERGOMETER TESTS ON BUILDING APPRENTICES AGED 15–19
Authors: ÅSTRAND IRMA , KILBOM ÅSA
Published: January 2008

A work test on a bicycle ergometer was carried out on 82 Swedish boys aged 15 to 19. The heart rates were recorded, both at the submaximal loads of 600 and 900 kpm/min and at the maximal work load. Maximal aerobic power was predicted from submaximal work loads and maximal heart rate. The heart rates obtained show decreasing values with increasing age at a given work load. This is partly ascribed to physical growth, and partly to some training effect noticeable in the older boys who have completed one year of building apprenticeship.
INTRAVENOUS GLUCOSE TOLERANCE, PLASMA INSULIN, FREE FATTY ACIDS AND β‐HYDROXYBUTYRATE IN UNDERWEIGHT NEWBORN INFANTS
Authors: GENTZ JOHAN C. H. , WARRNER RICHARD , PERSSON BENGT E. H. , CORNBLATH MARVIN
Published: January 2008

Blood glucose, plasma insulin, FFA and β‐hydroxybutyrate values during intravenous glucose tolerance were reported in 20 small for gestational age (SGA) and 15 appropriate for gestational age (AGA) low birthweight infants. The babies were divided into three groups according to their age when tested; <24 hours, 24–48 hours and >48 hours.

Both the SGA and AGA infants cleared glucose more rapidly with increasing age. The change was more marked in the SGA babies. The clearance rates were similar to those reported in normal full‐sized infants.

The insulin values before the glucose load were similar in all groups and comparable to those reported in normal newborn infants.

The insulin response to glucose was variable. There were no significant differences with increasing age or between the two groups of infants. The insulin curve of the individual infant followed one of three patterns. Most commonly seen was a double‐peak curve. The infants who showed a single‐peak insulin response had a better but not significantly different glucose tolerance than that of the other babies. Infants with no appreciable insulin response still removed glucose from plasma at a rate similar to those with a double‐peak insulin curve. It is concluded that insulin as measured in peripheral plasma could not explain the rate of removal of glucose from the plasma of the newborn low birthweight infant.

Infants of low birthweight had higher plasma FFA values as compared to that reported in normal full term infants. The FFA values in SGA infants were higher than those in AGA babies. In both groups of infants, the jS‐hydroxybutyrate values were comparable to those reported in normal full‐term babies. Thus there was an unexpected discrepancy between the high FFA and relatively low β‐hydroxybutyrate levels in plasma. The fall in plasma FFA and β‐hydroxybutyrate after glucose was minimal but similar in both groups of infants.

The findings are compatible with a decreased sensitivity to insulin in the infants studied.
IMMUNOGLOBULIN LEVELS DURING CHILDHOOD, WITH SPECIAL REGARD TO IgE
Authors: BERG T. , JOHANSSON S. G. O.
Published: January 2008

In a series comprising 215 children of ages 2–15 years, representative of the population in a medium‐sized Swedish town, determinations were made of the 5 immunoglobulins IgG, IgA, IgM, IgD and IgE. The children were divided with regard to the occurrence of allergic symptoms and to their general state of health. The results were subjected to extensive statistical calculations including regression analyses with regard to age and sex for the different immunoglobulins. A longitudinal study of the IgE development during the first year of life was also carried out.

Mean values and normal variations for IgE at different ages are presented. The IgE development during childhood was found to be essentially similar to that of IgA.

Adult IgG levels were reached, on the whole, at the age of 11–12 years, and a further IgG increase was noted in children during puberty. The girls had higher IgG concentrations throughout than the boys; this sex difference is not large but is statistically significant.

The IgA levels rose gradually throughout childhood and had still not reached an adult level during puberty. No sex difference was found for IgA.

Adult IgM levels were attained by the age of 4 years. The girls showed higher IgM concentrations throughout than the boys, and this difference is statistically significant.

A statistically significant relationship was found between age and IgD level. IgD was lacking from the age of 7–8 years in 12–16 per cent of the children, a somewhat lower figure than has been reported previously for adults. No statistically significant sex difference was found. Children with atopic conditions had higher IgD levels than completely healthy children; this difference was statistically significant.
Influences of Holocene climate and water levels on vegetation dynamics of a lakeside wetland
Authors: Yu Zicheng , McAndrews J. H. , Siddiqi D.
Published: January 2008

Sediment lithology, pollen, and plant-macrofossil data from the Paynter Site, southern Ontario, revealed three wetland developmental stages during the past 11 000 years: (i) a Carex, Eupatorium, and Eleocharis dominated marsh with some Larix, Abies, and Picea (ca. 11 000–8300 cal years BP); (ii) a Verbena hastata and Mentha arvensis marsh (ca. 8300–7460 cal years BP); and (iii) a white cedar (Thuja occidentalis) swamp (7460 cal years BP to present). There were no significant successional changes in the initial diverse marsh (stage 1) for about 2700 years; its high taxon richness was maintained by fluctuating water levels. The succession from marsh to swamp at 7460 cal years BP was caused by mid-Holocene warm and dry climate, which corresponded with cedar expansion elsewhere in Ontario. The swamp peat record was interrupted at ca. 6400 cal years BP by declining water levels, culminating in a dry period, as indicated by a sandy layer, rare macrofossils, and a low sediment-accumulation rate (0.012 cm/year). The reappearance of cedar swamp macrofossils since 3200 cal years BP corresponded with the recovery of water levels owing to a more humid late Holocene climate and flooding from isostatic tilt. Human disturbance such as damming and logging caused the development of historical cedar and thicket swamps with abundant Alnus rugosa and weedy taxa. This sequence of wetland development did not match the present vegetational gradients from Carex–Typha herb marsh, through Myrica–Decodon shrub marsh and Alnus–Fraxinus thicket, to Thuja swamp. At this site, the wetland development was mainly influenced by allogenic factors such as water levels and climate rather than autogenic factors. Keywords: wetland succession, climate change, hydrological change, Holocene, paleoecology, disturbance, space-for-time substitution, plant macrofossils, Thuja expansion, Ontario.