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Among twelve patients with homozygous alpha1-antitrypsin deficiency (Pi-type Z), five cases of infantile liver disease were diagnosed. The course of the disease was extremely variable; only one patient died of liver cirrhosis at the age of fourteen. In four cases the clinical, biochemical and histological (2 cases) findings became normal over a follow-up period of one to fifteen years. The results of these observations demonstrate that in alpha1-antitrypsin deficiency even when associated with proven liver disease the prognosis need not be unfavorable.

Bending primarily inoperable omphalocele can be avoided by simply using Stülpa tube-dressing together with a spongering. Epithelising under the employment of tannin or Betaisodona becomes much easier if this method is used.

High doses of dexamethasone (Fortecortin) for the prophylaxis of cerebral oedema were administered to 19 children aged 3 to 14 years because of head injury of varying degrees of severity. In order to assess possible suppression of the hypothalamic-pituitary-adrenocortical activity an insulin tolerance test and an intravenous tetracosactid (Synacthen) test was performed 1 to 26 weeks after the last dexamethasone dose. There was no evidence from the insulin tolerance test of inadequate cortisol excretion. After intravenous Synacthen injection satisfactory serum cortisol increase was found. However, from this test there was evidence of a lesser increase of serum cortisol depending on the time elapsed since cessation of dexamethasone. Investigation of circadian cortisol values showed 7 cases with subnormal serum cortisol at 18 o'clock independent of the time elapsed. The hypothalamic-pituitary-adrenocortical axis was functioning again during induced stress 7 days after withdrawal of dexamethasone. Subsequent stress situations do not require treatment with corticoids.

Using the paper-radioimmunosorbens test (PRIST) normal values were obtained from 200 non-allergic children. The IgE level was aged-dependent, in part with significant differences between various age groups. These normal values were compared with those in children with seborrhoeic dermitis, constitutional neurodermatitis, parasitoses, urticaria, Quincke oedema, Schönlein-Henoch purpura, pityriasis rosea, multiform exudative erythema, erythema nodosum and infantile papular acrodermatitis. Half-life of IgE in neonates was 16.2 hours.

In 56 children with acute lymphoblastic leukemia (ALL) T and B lymphocytes and immunoglobulins (IgG, IgM, and IgA) were studied before, during, and after therapy. At the time of diagnosis T lymphocytes were normal, the number of B lymphocytes was increased, and immunoglobulins usually were normal. Only two of 30 children had abnormally low immunoglobulin levels. Under therapy a progressive lymphopenia was observed. B cells were depressed most extensively. IgG and IgA levels showed a nadir during early treatment, i.e. after remission induction for IgG and 4-8 months after diagnosis for IgA. IgM was markedly elevated after meningosis prophylaxis. After cessation of therapy lymphocytes increased with an intense rebound of B cells. The recovery was not fully completed after one year free of therapy.

A 12-year-old girl was admitted to our hospital with signs of an acute abdomen with paralytic ileus. The previous and family history were without abnormalities. Abdominal pain and vomiting had started two days earlier. On palpation the swollen abdomen was painful and there was an increased tension in the left upper part. The clinical diagnosis of acute pancreatitis was confirmed by an increased serum level of lipase (4480 U/l). Clinical chemical investigations further revealed a permanent hypercalcemia in the range of 6.4 to 8.3 mval/l. This, together with concomitantly reduced levels of serum phosphate and a threefold increased level of parathyroid hormone (343 pg/ml, upper limit of reference = 100 pg/ml) were consistent with a hyperparathyroidism. In fact, sonography of the cervical organs revealed a solitary adenoma of the parathyroid glands. After surgery serum levels of calcium returned to normal. Hypercalcemia as a consequence of primary hyperparathyroidism has to be included in the differential diagnosis of acute pancreatitis in childhood.

Levels of albumin and IgG in the cerebrospinal fluid were determined in 69 children with infectious diseases of the central of the central nervous system in the years 1975 to 1978. The cerebrospinal fluid in 63 children without infectious diseases of the central nervous system, serving as a control, showed a close correlation between albumin and IgG levels. In the acute stages of serous and purulent meningitis (meningococci, streptococci, and hemophilus influenzae) however, IgG levels in the cerebrospinal fluid were overproportionately high. Very high yet correlating levels of IgG and albumin were found in purulent meningitis due to Escherichia coli. The determination of cerebrospinal fluid levels of albumin and IgG renders information about the function of the blood cerebrospinal fluid barrier, as well as about any significant production of IgG at these sites. In case of repeated determinations information about the course of inflammation and the efficacy of antibiotic therapy can be obtained. This technique is simple and inexpensive. For one determination only 50 microliter of cerebrospinal fluid is required.

Of 28 newborns with prolonged jaundice and lowered or subnormal values of alpha-1-antitrypsin in the serum six patients were found to have a Pi-type MZ, three a Pi-type MS and one patient a Pi-type MP. One of the newborns had Pi-type SZ (double heterozygous), one infant was a homozygous carrier of the Pi-type S. It seems to the possible that one of the reasons for the not yet explained cases of prolonged jaundice of the newborns period can be found in the high frequency of homozygous or heterozygous genotype carriers of Pi-system.

Various lung volumes and flows were measured in five children (aged 9-11 years), a male adolescent and one adult, all carriers of the homozygotic form of alpha 1-antitrypsin deficiency (Pi-Z type). In five patients lung function tests demonstrated overdistention, in three decreased CO diffusion capacity, as an expression of pulmonary emphysema at an early stage. Airway obstruction was not demonstrable in any of the patients (normal values for FEV 1, Tiffeneau test, peak flow and V25, V50 and V75). There was a correlation between the level of alpha 2-macroglobulin and the CO diffusion capacity, as well as between alpha 1-antitrypsin level and residual volumes. In one child there was evidence of minor liver involvement; in the other six patients, all liver-dependent serum enzymes were within normal limits. The findings suggest that the lung changes typical of adult homozygotic carriers of alpha 1-antitrypsin deficiency have their beginning in childhood.

The early diagnosis of septicaemia in children is difficult because the clinical and laboratory findings can be inconclusive. Results of blood cultures are usually available after two days only. Especially in the neonate, delayed or uncritical use of wide spectrum antibiotics can have negative consequences. We tested whether inorganic serum phosphate determinations could be helpful for diagnosing septicaemia in children. As in adults hypophosphataemia is a frequently occurring symptom in children with septicaemia. Hypophosphataemic values below 4.3 mg/100 ml in the neonate and below 3.2 mg/100 ml in an older child support the diagnosis of septicaemia. The possiblity of quick evaluation has some diagnostic importance.

Generalised reactions after an insect bite call for an exact diagnosis: nonspecific general reactions must be distinguished from true allergic reactions. 12 children with allergic reactions to bee and/or wasp stings were investigated. The results of the skin tests and the allergen-specific IgE determinations were compared and found to vary distinctly in some cases. In addition to immediate therapy (emergency pharmacy), specific hyposensitization over several years is indicated. Observation of the course of IgE antibodies is presented.

The long term follow up of 55 children with allergy treated by specific hyposensitization therapy is reported. Hyposensitization therapy had been terminated from 2--7 years previously. The success of treatment was subjectively judged to be satisfactory of good in 62% of the cases. In the group of asthmatic children, asthma attacks had decreased or ceased in 74% of the cases: in children with hay fever only 24% continued to have as many or more symptoms as before treatment. In children with asthma and pollen allergy, the success rate was slightly higher with aqueous extracts (68%) than with pyridine-extracted and alum-precipitated extract administration (43%). Also in children with hay fever symptoms alone the use of aqueous extracts seemed to bring somewhat better results (57% with good or satisfactory success) in comparison with pyridine-extracted and alum-precipitated extracts (45%). Side effects of hyposensitization therapy occured in 29 children (53%).