Gunilla Berglund

WISKOTT‐ALDRICH SYNDROME A Study of 6 Cases with Determination of the Immunoglobulins A, D, G, M and ND

Six cases with Aldrich syndrome are presented, one of them splenectomized at the age of 3, died at the age of 6 years of adrenal haemorrhage. Another patient died at the age of 6 years of pneumonia with empyema and meningitis. The other 4 patients (1/2–6 years) are alive in a fairly good condition. Bleeding tendency dominates the symptoms in 2 cases, in the other 2, susceptibility to infections and/or eczema are the main symptoms. The bleeding tendency does not seem to be correlated to the number of platelets.

A LONG‐TERM FOLLOW‐UP INVESTIGATION OF PATIENTS WITH HYPERTROPHIC PYLORIC STENOSIS—WITH SPECIAL REFERENCE TO THE PHYSICAL AND MENTAL DEVELOPMENT

202 of 203 patients medically treated for hyper‐trophic pyloric stenosis at the Childrens Hospital in Gothenburg 1922 to 1942 were classified according to the severity of their inanition during their disease. In the 180 registered for military service, height and the intelligence test results were investigated, and 176 of these were recently interviewed.

A LONG-TERM FOLLOW-UP INVESTIGATION OF PATIENTS WITH HYPERTROPHIC PYLORIC STENOSIS—WITH SPECIAL REFERENCE TO HEREDITY AND LATER MORBIDITY

Of 203 boys treated for hypertrophic pyloric stenosis at Childrens Hospital, Gothenburg, during the years 1922–1942 a total of 195 could be traced by a follow‐up investigation in 1970. All had been medically treated. Seven died during their first year of life, none of the disease directly. This number is the approximate figure for infant mortality in Sweden at that time.

Hashimoto's Disease in Childhood A Clinical and Immunological Study of Five Cases

In 1913 Hashimoto (10) described a goitre characterized by the formation of lymph follicles in the thyroid, an increase in lymphocytes, and subsequent marked fibrosis and destruction of the thyroid follicles. Almost ‘70 years elapsed before any new cases were reported, since then, however, a great number have been communicated. At present the disease is not considered to be exceptionally uncommon; in the material collected by Statland, Wasserman & Vichery (19), Hashimoto’x disease was found to be the cause of 1.4 9 ; of all thyroidectomies. The etiology, which earlier was a matter of controversery, is still obscure, although the demonstration of cytotoxic antibodies against thyroid tissue in serum from patients with Hashimoto’s disease gives strong evidence for the participation of an auto-immunisation mechanism in the pathogenesis of the disease (5, 13, 15, 16, 17, 18, 23). The course of the disease is a slow one. Usually an enlargement of the thyroid will appear, in some cases it may be rather insignificant, in others so pronounced that i t will cause compression. Simultaneously the gland becomes firmer. 44 C0173105 Aeta Pxdiatrica Vol. 49 There is no pain. As shown by various tests, thyroid function is already impaired in the early stages of the disease, but hypothyroid symptoms appear only gradually. Hashimoto’s disease affects mainly elderly women, but it may also appear in younger age groups and in men. It is said to be rare in childhood (23). In a study of 603 cases 3 were belou the age of 10 years (23). All were girls, 8 or 8 years old. In other studies which included children only girls have been reported as being affected (9, 19). In this communication the clinical and other findings in Hashimoto’s disease as it affects children will be reported. Five patients have been studied, two of them are boys.

Plasma Transfusion Treatment of Six Children with Idiopathic Throinbocytopenic Purpura

The treatment of idiopathic thrombocytopenic purpura (ITP) must be based on clinical experience as long as the etiology is obscure. The present conception that ITP is a condition caused by an immunological reaction does not solve the problem of treatment as long as the cause of the antibody production against the platelets is unknown. The commonly used treatment in ITP, cortisone and splenectomy, is beneficial in about 80-85 % of the patients. Both of these treatments have some side effects and risks. In 1959 Schulmaim et al. [2] demonstrated that plasma transfusions could be efficacious in the treatment of an 8-year-old girl with ITP. Though no effect was obtained after treatment with cortisone or by splenectomy, remissions of a month’s duration were achieved after each of many transfusions of fresh frozen plasma. They credited this effect to a plasma factor increasing the number of platelets. On the basis of this report plasma transfusions were introduced as the initial treatment of all ITP cases, Case E. H . (Fig. 1). A 14-month-old girl without any known heredity for bleeding disorders was admitted because of a bleeding tendency with mucosal bleedings, hematomas and petechiae since 2 months of age. On admission the girl was in good condition except for hematomas of different ages scattered over the body and a fair amount of petechiae on the upper part of the body. The tourniquet test was positive. The laboratory investigations showed ESR 4 mni/hour, Hgb 12.4 g%, WBC 8,100/mm3, platelets 3,000/ mnl3, bleeding time 16 minutes, clotting time 6 minutes, prothrombin index 120. The bone marrow smear showed a normal picture. During the first 12 days in the hospital, when no treatment was given, the platelets varied between 3,000 and 15,000/mm3. Thereafter a transfusion of 20 ml fresh plasma/kg body weight was given. The platelets increased and reached a maximum value of 354,000/mm3 on the 8th day. The bleeding time was 1 minute 30 sec. Within the next few days the platelet count had diminished to around 150,000/mm3. During the subsequent observation time of about 18 months the platelets remained unchanged between 150,000 and 200,000/mms. The bleeding time was normal and the patient had no bleeding manifestations. which have been hospitalized a t the Chil-