HAEMORRHAGIC INFARCTION OF THE MYOCARDIUM IN A NEWBORN WITH HAEMOGLOBIN H DISEASE AND ERYTHROBLASTOSIS

ABSTRACT. Extensive haemorrhagic myocardial infarction developed in a newborn, apparently as a result of anoxia due to erythroblastosis fetalis, associated with haemoglobin H disease. Acute massive myocardial infarction in the neonatal period is rare and usually is associated with congenital malformation of the heart or its blood supply. Neonatal myocardial infarction in the anatomically normal heart with normal coronary vessels, has been described in only 8 patients (1). This communication describes a newborn with acute haemorrhagic myocardial infarction due to anoxia believed to be caused by the combined effect of erythroblastosis fetalis and haemoglobin H disease.     

AN UNUSUAL CASE OF CROHN'S DISEASE IN A WEST INDIAN CHILD

ABSTRACT. Although perianal ulceration is common in Crohn's disease, extensive perineal ulceration particularly in children is rare. The case history is presented of a child who developed progressive hypertrophy of the vulva and ulceration of the groins at the age of 11 years but diagnosis of Crohn's disease was delayed for 21 months. Treatment with steroids and azathioprine has induced a successful remission but difficulty has been experienced in reducing the steroid dosage.     

OBSESSIVE-COMPULSIVE NEUROSIS WITH CONDUCT DISORDER IN ADOLESCENCE: A REPORT OF TWO CASES

Two adolescents are described presenting with obsessive-compulsive neurosis and conduct disorder. The presence of conduct disorder is in marked contrast to the compliance and over-control characteristic of obsessionality, and was found to give rise to great difficulties in instigating and carrying through a programme of response-prevention for the obsessive-compulsive symptoms.     

IgE AND ATOPIC ALLERGY IN NEWBORNS AND INFANTS WITH A FAMILY HISTORY OF ATOPIC DISEASE

Abstract. Serum IgE levels were studied in 2 groups of children with a family history of atopic disease, 30 in whom the mother only and 38 in whom both parents had atopic disease. IgE antibodies were determined with Phadebas RAST® Test and serum IgE with Phadebas® IgE Test and Phadebas PRIST® at 0, 3, 9, 12 and 18 months of age. There was no correlation between the serum IgE levels in mothers and their newborns. RAST tests were frequently positive in maternal sera but no positive RAST test was found in the newborns. Obvious and probable atopic disease developed during the observation period in 42.1% of the children with a double family history of atopic disease. In 75% of these the serum IgE level was above the upper limit of normal on an average 6 months before the onset of atopic symptoms. An elevated IgE level without atopic symptoms during the observation period occurred in only one child. It is concluded that the serum IgE in newborns seems to be of foetal origin and that the determination of serum IgE in infants is of value in predicting atopic allergy.     

POSTINITIAL REMISSION IN DIABETIC CHILDREN– AN ANALYSIS OF 178 CASES

ABSTRACT. We studied 178 diabetic children and adolescents diagnosed during the period 1962-79 to find out the occurrence and duration of the postinitial remission, factors favoring a remission and the prognostic value of the remission. A postinitial remission occurred in 113 children (64 %) being complete in only three boys (2 %). The duration ranged from one month to 4.8 years, the mean being 8.4 months. The boys had a remission more often and of longer duration than the girls. The duration of diabetes was longer in the children without remission. The children with remission had lower blood glucose, milder hyperketonemia and ketonuria, higher pH and P_co2 at onset than those without remission. Hemoglobin A₁ (HbA₁) during 1979 were lower in the children with a positive remission history. The children with a remission lasting more than one year had a subsequently higher glucosuria index, lower HbA₁ and higher C-peptide when compared to those without remission or to those with a short remission. The remission frequency increased from 1962 to 1979. Male sex and mild metabolic derangement at onset favor a postinitial remission, which results in a persisting residual beta-cell function and better metabolic control beyond the remission.     

WILSON''S DISEASE IN AN ADOLESCENT DISPLAYING AN ADJUSTMENT REACTION TO A SERIES OF LIFE STRESSORS: A CASE STUDY

The case is presented of a 13 year old boy suffering from Wilson's disease. Initially, his medical condition was, to a large degree, masked by a range of behavioural and emotional problems which followed directly in the wake of a series of major life stresses.     

PRESUMED DISSEMINATED BCG IN A BOY WITH CHRONIC GRANULOMATOUS DISEASE OF CHILDHOOD

ABSTRACT. Verronen, P. (Department of Paediatdcs, Central Hospital, Tampere, Finland). Presumed disseminated BCG in a boy with chronic granulomatous disease of childhood. Acta Paediatr Scand 63: 627, 1974.—A male. child suffering from chronic granulomatous disease of childhood, confirmed by a Irucocyte bactericidal function test and a nitrobluc tetrazolium test, was given a BCG inoaulatlon in the neonatal pcrfod. He had draining lesions at the sites of vaccination and later miliary infiltration of the lungs, which responded favourably to antituberoulous therapy. Cultures for tb-bacilli were negative. An older sister is suspected to be a carrier of the trait responsible for chronlc granulomatous disease. Her sole clinlcal manifestation was miliary infiltration of the lungs at the age of 3 ⁹/₁₂ years.     

EFFECT OF FEEDING AN INFANT FORMULA WITH HIGH ENERGY DENSITY ON GASTRIC EMPTYING IN INFANTS WITH CONGENITAL HEART DISEASE

ABSTRACT. Cavell, B. (Department of Paediatrics, University Hospital, Lund, Sweden). Effect of feeding an infant formula with high energy density on gastric emptying in infants with congenital heart disease. Acta Paediatr Scand, 70:513,.–Gastric emptying of meals of standard infant formula and formula fortified with a glucose polymer (Caloreen) were compared in 7 infants with severe congenital heart disease aged 9 days to 5 months. A marker dilution technique was used in estimating gastric emptying. Gastric half-emptying times were significantly longer for meals containing Caloreen than for meals of standard infant formula. Although significantly smaller amount of meal containing Caloreen had left the stomach after 2 hours, the net transfer of energy to the intestine was larger than after feeding a standard meal.     

ADDISON'S DISEASE ASSOCIATED WITH PRECOCIOUS SEXUAL DEVELOPMENT IN A BOY

ABSTRACT. Marilus, R., Dickerman, Z., Kaufman, H., Varsano, I. and Laron, Z. (Institute of Pediatric and Adolescent Endocrinology, Steroid Laboratory and Pediatric Department B, Beilinson Medical Center, Petah Tikva, Sackler School of Medicine, Tel Aviv University, Israel). Addison's disease associated with precocious sexual development in a boy. Acta Paediatr Scand, 70:587,.–An eleven-year-old boy was diagnosed to have Addison's disease with an ACTH level of over 4 500 pg/ml after a short episode of fever and dehydration. From the age of 6 years his skin and buccal mucosa had been hyperpigmented but there were no other subjective complaints. The clinical examination also revealed signs of advanced puberty for his age (3rd-4th stage according to Tanner's criteria), with advanced bone age (13 years). The elevated levels of plasma testosterone and gonadotropins and their response to LH-RH stimulation were appropriate for his clinical stage of puberty and supported the diagnosis of true precocious puberty. It is proposed that the concomitant appearance of Addison's disease and precocious puberty is due to a "drift" phenomenon of LH-RH and/or gonadotropins following the prolonged elevation of ACTH secretion.     

CHRONIC PULMONARY DISEASE IN A CHILD WITH THE EHLERS-DANLOS SYNDROME

ABSTRACT. The presence of chronic pulmonary disease is an uncommon finding in the Ehlers-Danlos syndrome. The patient discussed herein presented with severe irreversible obstructive pulmonary disease characterized by marked hyperinflation, an increase in static lung compliance, and tracheal dilatation. A largely normal lung biopsy ruled out chronic pulmonary emphysema. Despite the similarity of this patient to those with the Mounier-Kuhn syndrome, the degree of tracheobronchial enlargement was not as great as is usually seen in this disorder although these anatomic changes may develop with time. The findings in this case demonstrate that any site within the tracheobronchial tree may be involved in the Ehlers-Danlos syndrome and further emphasize the need to determine the degree of reversibility of obstructive lung disease in children.     

CHRONIC PULMONARY DISEASE IN A CHILD WITH THE EHLERS-DANLOS SYNDROME

Abstract. The presence of chronic pulmonary disease is an uncommon finding in the Ehlers-Danlos syndrome. The patient discussed herein presented with severe irreversible obstructive pulmonary disease characterized by marked hyperinflation, an increase in static lung compliance, and tracheal dilatation. A largely normal lung biopsy ruled out chronic pulmonary emphysema. Despite the similarity of this patient to those with the Mounier-Kuhn syndrome, the degree of tracheobronchial enlargement was not as great as is usually seen in this disorder although these anatomic changes may develop with time. The findings in this case demonstrate that any site within the tracheobronchial tree may be involved in the Ehlers-Danlos syndrome and further emphasize the need to determine the degree of reversibility of obstructive lung disease in children.     

MVPP CHEMOTHERAPY COMBINED WITH RADIOTHERAPY IN THE TREATMENT OF HODGKIN'S DISEASE IN CHILDREN

ABSTRACT. Thirty-four children with Hodgkin's disease were treated during the years 1969–75. After radiotherapy, 7–15 cycles of MVPP were given within 24–53 months. In order to avoid chronic leukopenia, leukocyte counts were made frequently during chemotherapy, and the drug doses adjusted accordingly. A complete remission was obtained in 32 of the 34 children. Two patients died because of progressive disease. Twelve of the 32 survivors have been followed for at least 5 years, and a further 12 for at least 3 years. Three children are still on chemotherapy, whereas the remaining 29 being followed are in continued complete remission.     

V. EXERCISE STUDIES IN CHILDREN WITH CONGENITAL HEART DISEASE. AN EARPIECE DENSITOMETRIC INVESTIGATION

Abstract: Twenty-three patients have been studied by exercise testing during right heart catheterization. Cardiac output was measured by the earpiece densitometric technique before, during and after the exercise period. It is concluded that the earpiece densitometric technique in conjunction with exercise testing offers an attractive alternative for the study of pressure and flow characteristics in children with congenital heart disease.     

NEOPLASTIC DISEASE AND DELETION 22q11.2: A MULTICENTRIC STUDY AND REPORT OF TWO CASES

Deletion 22q11.2 is a chromosomal abnormality detected in young patients with clinical manifestations of the DiGeorge/velocardiofacial syndrome. Conotruncal heart defects are also associated with del22q11.2. An association of these cardiac malformations with neoplasias has been observed. Our series includes two cases of malignancies, a hepatoblastoma and a renal-cell carcinoma, arising in children with complex cardiac malformations. The aim of the study was to determine if the deletion at 22q11.2 was present and could be responsible for both pathological processes. Del22q11.2 was identified in both cases. Comparative genomic hybridization revealed terminal gains on chromosomes 1q and Xq and terminal loss on 1p in the hepatoblastoma, and gains in 1p, 12q, 16p, 20q, 22q, and whole chromosome 19 and loss of Xq in the renal-cell carcinoma. Our results confirm a common genetic basis for cardiac malformations, and del22q11.2 presents a risk factor for the development of pediatric tumours.     

LEGIONNAIRES' DISEASE COMBINED WITH ERYTHEMA MULTIFORME IN A 3-YEAR-OLD BOY

ABSTRACT. Andersen, R., Bergan, T., Halvorsen, K., Kallings, I. and ørstavik, I. (Departments of Anaesthesia, Microbiology, and Paediatrics, Aker Hospital; Department of Microbiology, Ullevål, Oslo, Norway; and the State Bacteriological Laboratory, Stockholm, Sweden). Acta Paediatr Scand, 70:427, 1981.–A case of Legionnaires' disease (LD) is described in a 3-year-old boy. He had fulminant disease with typical signs like bilateral pneumonia, gastrointestinal symptoms, and somnolence indicating involvement of the central nervous system. There was no premorbidity. An outstanding development was erythema multiforme, which has never previously been described in LD. The basic disease was caused by Legionella pneumophila . This is evidenced by specific serum IgM at admission and a subsequent significant rise in titers against L. pneumophila . At an early stage respiratory syncytial virus (RSV) was isolated from the patient's throat, although there was no antiviral serological response at the outset of erythema multiforme or 3 weeks after onset of disease; anti-RSV appeared later. The prolonged course of the disease can be explained by the successive occurrence of two infections. The possibility that the virus could have contributed to the development of erythema multiforme cannot be ruled out. The role of concomitant medication cannot be separated out, but on the basis of general knowledge of their immuno-genicity and the fact that immunosuppressive cortisone was given at the time it is less likely that the antibiotics contributed significantly.     

EFFECT OF POSITIVE-END-EXPIRATORY-PRESSURE ON RIGHT VENTRICULAR OUTPUT IN LAMBS WITH HYALINE MEMBRANE DISEASE

Abstract. Cotton, R. B., Lindstrom, D. P., Kanarek, K. S., Sundell, H. and Stahlman, M. T. (Department of Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee, USA). Effect of positive-end-expiratory-pressure on right ventricular output in Iambs with hyaline membrane disease. Acta Paediatr Scand, 69:603, 1980.—Right and left ventricular outputs, pulmonary and systemic blood flows, and blood flows in both directions through the ductus arteriosus were measured before and during positive-end-expiratory-pressure in 5 premature lambs with induced hyaline membrane disease. During positive-end-expiratory-pressure, right ventricular output increased in all Iambs without any significant change in left ventricular output or pulmonary vascular resistance. Left-to-right ductus flow decreased in lambs which initially had large left-to-right ductus shunts. Significant right-toleft ductus flow did not occur before or during positive-end-expiratory-pressure.     

THE GASTROINTESTINAL ABSORPTION OF PENICILLIN V IN CHILDREN WITH SUSPECTED COELIAC DISEASE

Abstract. The gastrointestinal absorption of penicillin V (pc-V) was investigated in 6 children, 6–12 months old, with suspected coeliac disease. The diagnosis was set after small bowel biopsy and absorption tests of vitamin A and d -xylose. As control groups served 7 children with diarrhoea but with normal small bowel biopsy and/or absorption tests and a group of 9 children with upper respiratory tract infection of the same ages as the children in the test group. The absorption of calcium pc-V in oil suspension (Penicals®) was impaired in the patients with suspected coeliac disease compared to that of the control groups. There was no significantly different absorption of pc-V between the control children with diarrhoea and those with upper respiratory tract infection. After 6–8 months of gluten free diet in the children with suspected coeliac disease their absorptive ability of oral calcium pc-V in suspension form was equal with that of a control group.