Iréne Sjögren

PRIMARY HYPERPARATHYROIDISM IN CHILDREN: Brief Review of the Literature and a Case Report

Primary hyperparathyroidism is a rare disease in children, and only a few reports concerning its clinical manifestations have been published in the paediatric literature. In 1960 Nolan et al. l(31) reviewed briefly the 23 previously reported cases. Wilkins (49) described the symptoms under four main headings: 1) those due to hypercalcaemia, 2) those resulting from the increased excretion of calcium and phosphorus in the urine, 3) those related to changes in the skeleton and 4) metastatic calcification in soft tissues, but he pointed out that “under certain circumstances the typical symptoms and chemical changes of primary hyperparathyroidism may be altered”. On the basis of the clinical picture in a 13year-old boy and an analysis of 42 paediatric patients with hyperparathyroidism reported in the literature, we aim in this paper to elucidate the difficulties of differential diagnosis in this disease in children. We would point out, in particular, that primary hyperparathyroidism should be considered in patients with diffuse symptoms such as general fatigue and weakness, dizziness, anorexia, gastrointestinal bleeding, cardiac arrhythmia or renal colic. When

BRAIN GROWTH IN CHILDREN WITH KWASHIORKOR A Study using Head Circumference Measurement, Transillumination und Ultrasonic Echo Ventriculography

ABSTRACT. Engsner, G., Habte, D., Sjögren, 1. and Vahlquist, B. (Ethiopian Nutrition Institute and the Ethio‐Swedish Pediatric Clinic, Addis Ababa, Ethiopia, and the Department of Paediatrics, University Hospital, Uppsala, Sweden). Brain growth in children with kwashiorkor. A study using head circumference measurement, transillumination and ultrasonic echo ventriculography. Acta Paediatr Scand, 63, 1974.—Brain growth was studied in 53 children with kwashiorkor and marasmic kwashiorkor by the simultaneous use of head circumference measurement, transillumination and ultrasonic echo ventriculography. The results of examinations on admission showed that the head circumference was reduced but probably slightly less so than in marasmic children. The transillumination findings were distinctly abnormal: bearing the age difference in mind, the abnormality was more pronounced than in the cases of marasmus. The lateral ventricle index, calculated from echo ventriculograms, was increased, which was not the case in marasmus. A systematic follow‐up study for 6 months of 10 patients showed a gradual and complete normalization of the transillumination and echo ventriculographic findings. The interpretation of the results, taking into consideration also the possible sources of error, is discussed.

Malnutrition and size of the cerebral ventricles. Echoencephalographic studies in infants and young children. Preliminary communication.

Examination of the size of the cerebral ventricles by means of echoencephalography in Ethiopian infants and young children with severe malnutrition showed a moderate but significant increase in children with kwashiorkor, examined up to 3–4 weeks after admission, whereas children with marasmus showed no deviation from the normal.

THE INCIDENCE OF INFANTILE HYDROCEPHALUS IN SWEDEN.

The incidence of infantile hydrocephalus in Sweden was investigated in two separate studies; the number of cases present at birth was determined in a material from an obstetric clinic; the number of cases with an onset during the first year of life was penetrated in a field study.

Brain growth in children with marasmus. A study using head circumference measurement, transillumination and ultrasonic echo ventriculography.

Brain growth was studied by making simultaneous measurements of head circumference, transillumination and lateral ventricle indices in 102 children aged 2–24 months suffering from marasmus. The head circumference was significantly reduced, transillumination showed a slight-to-moderate increase in the children 6–24 months of age, and echo encephalography showed a normal lateral ventricle index. The results indicate a reduction of brain size which (particularly after the first 6 months of age) goes slightly beyond what may be inferred from the head circumference per se. The interpretation of the results, especially the relation between head circumference and brain size, is discussed.

CHONDRO-ECTODERMAL DYSPLASIA. (ELLIS-VAN CREVELD'S SYNDROME). TWO CERTAIN AND TWO PROBABLE CASES IN THE SAME FAMILY.

In 1940 R. W. B. Ellis and S. van Creveld described a new syndrome of multiple deformities with dysplasia of tissues from ectoderm and mesoderm [a]. The syndrome is characterized by (1) ectodermal dysplasia, (2) polydactyly, (3) chondrodysplasia and (4) congenital morbus cordis. The syndrome is now called Ellis-van Creveld’s syndrome; a t least three of the four criteria are required for the diagnosis. A total of 39 cases have been described [l, 4, 7, 81. In 1960, Dayer [4] made an exhaustive study of the syndrome with a list of all the cases previously published. No case from Scandinavia appeared among these. Here will be briefly described a brother and sister who suffered from chondro-ectodermal dysplasia. In a later pregnancy, abortion was performed on the mother. The twin foetuses showed the same skeletal changes as the living brother and sister.

TRANSILLUMINATION OF THE SKULL IN INFANTS AND CHILDREN Recording with a New Point Scale

Richard Bright (2). Since this time, several reports have appeared about transillumination as a useful diagnostic aid in neuropaediatric disorders. The method of transillumination has undergone a more scientific approach so as to systematize its main usefulness and limitations. Matthes (8) points out that transillumination is worthwhile as a routine examination in all neurological disorders, and that no special instruments are required. The only necessary items are a staff shaped torch with a rubber rim-so that the lamp can be held against the baby’s head-and a well dark-adapted examiner in a completely darkened room. In 1961 Cambern & Shurtleff (3) presented their “Photography of transilluminated intracranial lesions in infants” and in 1962 Pliquett et al. (10) gave an idea of how complicated it may be to get rid of dangerous heat and ultraviolet rays when using light sources of 2 000 watt for transillumination purposes in order to photograph the findings; their presentation of such dangers and of an expensive and space-consuming apparatus hardly made the method of transillumination more popular. According to Matthes (8) the method of transillumination gives qualitative information and may differentiate between simple hydrocephalus (when the thickness of the cerebral cortex is 1.5 cm or less) and cerebral malformations such as hydranencephaly, Dandy-Walker syndrome, some forms of porencephalic cysts, and other cerebral malformations; not least it gives possibilities of diagnosing and localizing subdural effusions (when the layer of fluid measures 0.5 cm or more) and sometimes also of diagnosing cerebral atrophies (9). Newborn babies and infants may normally transilluminate, especially over the frontal regions of their heads (4, 8, 11, 12, 14). Translucency usually disappears after the age of one year, or when the skull bone is more than 2.5 mm thick (4). The anatomical condition permitting transillumination is the presence of cledr fluid within one cm of the inner surface of the skull (1, 11, 13). Translucency is prevented by dark hair and by fresh blood in the fluid; a subdural haematoma will not transilluminate until after about three weeks, when the blood has haemolysed

Urinary Excretion of I131-Diodrast Injected Intraventricularly in Communicating Hydrocephalus and Aqueduct Stenosis

Nine cases of expansive infantile hydro‐cephalus were investigated by injecting Small amounts of I131‐Diodrast intra‐ventricularly. Urinary excretion of the tracer was determined during the first 4‐5 hours after injection. The cumulative excretion of Diodrast from the C.S.F. differs in cases with communicating hydrocephalus from those with stenosis of the aqueduct. The investigation is rapid and easily performed and has not produced any side effects.

Nils Rosén von Rosenstein - The Father of Paediatrics

One of the pioneers in medical sciences, in the modern sense of the word, was a young doctor in Uppsala, Nils Rosén – or Nils Rosén von Rosenstein (1706-1773) as he was later called as a nobleman. Already in the 1730s he devoted himself in successful empirical research, a strong interest in medical education and many important clinical experiences. This made him one of the foremost physicians of his time, Nordens Store Arkiater, also called The Hippocrates of the Modern Times. He has also, by right been acknowledged as The Father of the Swedish Medical Training. Today his name is especially well known by specialists in children´s diseases. Paediatricians all over the world honour Nils Rosén von Rosenstein as the Father of Paediatrics (Wallgren 1964). The leading question is why a scientifically learned academician as Nils Rosén in the eighteenth century started with research works concerning children and their needs and their possibilities to survive. At this time, physicians as well as laymen seemed to have thought that it was just as inevitable that 50 per cent of all the living born children died without reaching an adult age, as that we all finally have to die. The challenging question is: Can we here an now within our work learn something for the future by this very pioneer in medical sciences concerning the medical problems in our own country as well as in the developing countries.

77. MALNUTRITION AND BRAIN DEVELOPMENT — NEURO‐PHYSIOLOGICAL OBSERVATIONS

Severe malnutrition at an early age can lead to permanent stunting of physical growth. The brain manifests a very rapid growth and development at an early age. It is not surprising therefore, that also this organ may suffer. The results of animal experiments as well as of clinical observations prove that this is in fact the case. The effects of malnutrition on brain growth and development have so far been investigated mainly with histological and biochemical methods. The authors will present preliminary results of investigations with neuro-physiological methods (echo-encephalography, conduction velocity of peripheral motor nerves, transillumination). With these methods it is possible to perform without any untoward reactions for the children cross-sectional as well as longitudinal studies The results to be presented originate from investigations on Ethiopian children with different degrees of malnutrition. They are part of the research activities at the SIDAsupported Ethiopian Nutrition Institute.