Circulating thyroid antibodies and thyroid function studies in children and adolescents with insulin-dependent diabetes mellitus

Significant high titres (1400–125,600) of circulating thoroid microsomal antibodies (MCHA) were found in the sera of 5 out of 59 non-ketoacidotic, insulin-dependent diabetic (IDDM) patients (mean age 14.5 years). Among these five patients (four females, one male), all of whom were over 11 years, two also had thyroglobulin antibodies. Increased thyrotropin (TSH) response to TRH was found in 3/5 MCHA positive patients and in 3/54 without circulating MCHA. Serum thyroxine (T₄) and free T₄ (FT₄) average values were significantly lower (P<0.01 and P<0.001) in diabetics (7.1±1.8g/dl and 10.2±3.1 pg/ml, x±SD) as compared to normal sex and age matched controls (8.9±1.9 g/dl and 12.2±2.2 pg/ml, respectively). T₄ and FT₄ values were inversely related to the duration of the disease. Subnormal T₄ values were found in six (five females and one male) patients, four of whom had subnormal FT₄ values. No patient had low triiodothyronine (T₃) and high reverse T₃ (rT₃) values, i.e. none displayed the biochemical pattern of the low T₃ syndrome described with ketoacidotic status. This indicates also a satisfactory compensation of IDDM in all the patients. At the time of study no patient (including also those with circulating MCHA and TGHA and with TSH hyper-response to TRH) showed either thyroid size enlargement or clinical features of thyroid dysfunction including impaired growth and bone age retardation.Abbreviations MCHA thyroid microsomal antibodies - IDDM insulin-dependent diabetes mellitus - TSH thyrotropin - T₄ serum thyroxine - FT₄ free T₄ - T₃ triiodo thyronine - FT₃ free T₃ - rT₃ reverse T₃ - TGHA thyroglobulin antibodies - TRH thyrotrophin releasing hormone     

3,3′,5′-Triiodothyronine (reverse T3) in amniotic fluid and cord serum

3,3,5-triiodothyronine (reverse T₃, rT₃) was measured in 122 samples of amniotic fluid obtained between the 29th–40th weeks of gestation, and in the blood of 37 newborn and their mothers. The mean rT₃ concentration in amniotic fluid was 0.769±0.47 ng/ml, with a slight decrease from the 29th–40th weeks of gestation which was not statistically significant. Because of the great scatter of rT₃ concentrations in amniotic fluid, its estimation does not seem to be useful in prenatal diagnosis of hypothyroidism. The mean rT₃ concentration in cord blood (2.62 ng/ml; range 1.4–4.9 ng/ml) was greatly elevated in comparison to the mean maternal level at delivery (0.34 ng/ml; range 0.08–0.69 ng/ml). The possible use of rT₃ measurements in cord blood as a screening test for congenital hypothyroidism is discussed.

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Zusammenfassung 3,3,5-Trijodthyronin (Reverse T₃, rT₃) wurde in 122 Frucht-wasserproben aus der 29.–40. Schwangerschaftswoche und im Blut von 37 Neugeborenen sowie deren Müttern bestimmt. Der mittlere rT₃-Gehalt der Fruchtwasser lag bei 0,769±0,47 ng/ml; ein leichter Abfall der rT₃-Konzentrationen bei steigendem Gestationsalter war statistisch nicht zu sichern. Aufgrund der großen Streuungen scheint das rT₃ zur praenatalen Hypothyreosediagnostik ungeeignet. Die mittlere rT₃-Konzentration im Nabelschnurblut von 2,62 ng/ml war gegenüber dem durchschnittlichen rT₃-Spiegel der Mutter unter der Geburt von 0,34 ng/ml stark erhöht. Die mögliche Verwendung der rT₃-Messung im Nabelschnurblut zum Hypothyreose-Screening wird diskutiert.

     

Elevated CSF cyclic AMP concentrations in patients with inflammatory diseases of cerebral and extracerebral origin

Cyclic 3–5 adenosine monophosphate (c-AMP) concentrations were measured in cerebrospinal fluid (CSF) from children admitted to the hospital because of suspected meningitis. c-AMP levels were found to be markedly elevated (P<0.001) during the acute phase of most of the purulent meningitis patients, as well as in patients with acute aseptic meningitis. In convalescent patients after purulent meningitis mean c-AMP concentrations remained elevated (P<0.01) beyond the normalization of the routine parameters in CSF. In addition, a variety of febrile inflammatory conditions of extracerebral origin produced elevations (P<0.001) of c-AMP although CSF by routine criteria was normal. The results suggest that c-AMP might serve as a sensitive indicator of transient cellular metabolic disturbance in the brain.Abbreviations CSF cerebrospinal fluid - c-AMP cyclic 3–5 adenosine monophosphate - WCC white cell count     

Decreased bone density in severely handicapped children and adults,with reference to the influence of limited mobility and anticonvulsant medication

Bone density and related biochemical parameters were investigatd in institutionalised children and adults with severe handicaps, who were classified according to the degree of limited mobility (group 1, bed-ridden; group 2, capable of crawling; group 3, capable of walking) and according to whether or not they were receiving anticonvulsants. As determined by microdensitometric analysis of radiograms of the second metacarpal bone, bone width (D), bone pattern area (GS) and bone salt density (GS/D) were decreased in the patients, the decreases being most prominent in group 1, followed by groups 2 and 3, in that order. Significant decreases of GS and GS/D, but not of D, were found in patients on anticonvulsant treatment in comparison to patients without therapy. Serum alkaline phosphatase (Al-p) and parathyroid hormone (iPTH) as well as urinary calcium and cyclic adenosine-3,5-monophosphate (cAMP) excretion were significantly increased in group 1. In comparison to patients without therapy, anticonvulsant-treated children showed significantly decreased levels of serum calcium (Ca), ionised Ca (Ca^2-), 25-hydroxy vitamin D₃ and urinary phosphate (PO₄) excretion, and elevated levels of Al-p, iPTH and calcitonin (iCT). It is suggested that limited physical activity results in a mild hyperparathyroid state, which is aggravated in patients on anticonvulsant treatment.Abbreviations Al-p alkaline phosphatase - iPTH immunoreactive parathyroid hormone - cAMP cyclic adenosine-3,5-monophosphate - Ca calcium - Ca²⁺ ionised calcium - PO₄ inorganic phosphate - iCT immunoreactive calcitonin - TmPO₄/GFR ratio of the maximum rate of renal tubular reabsorption of phosphate to the glomerular filtration rate - D bone width - GS bone pattern area - GS/D bone salt density     

Changes in thyroid function tests induced by 2 month carbamazepine treatment in l-thyroxine-substituted hypothyroid children

In five l-thyroxine-substituted hypothyroid children with partial epilepsy serum total thyroxine (T₄) and free T₄ (FT₄) significantly (P<0.01) decreased following 2 months of carbamazepine (CBZ) administration (20 mg/kg per BW per day) from mean (±SD) values of 12.7±1.1 g/dl and 15.5±1.8 pg/ml to mean values of 7.5±2.3 and 10.1±1.7, respectively. In all but one patient important changes in both serum total and free triiodothyronine (T₃, FT₃) were not observed; consequently T₃T₄ and FT₃FT₄ ratios significantly (P<0.05) increased in the whole series. Three subjects had post-treatment serum TSH that rose to hypothyroid levels parallel to a T₄ decrease. The negligible thyroid hormone secretion and the unmodified T₃-uptake (T₃U) or T₄-binding globulin (TBG) exclude direct effects of CBZ on thyroid gland and on carrier serum proteins, respectively. The findings observed, instead, might be due to accelerated T₄ metabolic clearance together with augmented T₄ to T₃ conversion rate, as previously demonstrated for diphenylydantoin. The sharp reduction in T₄ and FT₃ concentrations is the peripheral display of this event, which is associated with a decompensation of the metabolic status, as indicated by serum TSH enhancement. In all cases a supplement of l-thyroxine by itself was able to restore euthyroid TSH serum concentrations, suggesting that hypothyroidism in patients with partial epilepsy to whom CBZ had been administered requires a higher l-T₄ substitutive regimen.Abbreviations CBZ carbamazepine - DPH phentoin - T₄ thyroxine - T₃ triiodothyronine - TSH thyrotropin - FT₄ free T₄ - FT₃ free T₃ - rT₃ reverse T₃ - TBG thyroxine binding globulin - T₃U T₃ uptake - RIA radioimmunoassay Presented in part at the 23rd Annual Meeting of the European Society for Pediatric Endocrinology (Heidelberg, September 2–5, 1984) [5]     

Längsschnittuntersuchungen der physikalischen Kreislaufanalyse bei infektionskranken bzw. rekonvaleszenten Kindern

Zusammenfassung Bei insgesamt 364 Kindern im Alter von 4–14 Jahren wurden im Verlauf einer akuten Infektionskrankheit und in der Rekonvaleszenz Längsschnittuntersuchungen der physikalischen Kreislaufanalyse nach Wezler u. Böger durchgeführt. In den ersten Krankheitstagen ließ sich fast durchweg eine vasoconstrictorische Kreislaufdrosselung im Sinne einer Tendenz zur Zentralisation des Kreislaufs (Duesberg u. Schroeder) nachweisen. Dementsprechend waren die Werte von V_s, V_m, HJ, E/W, H_A, H_L, p vorzugsweise und die von PWG, E, W, p_s, p_d, p_m vorwiegend erhöht. Mit fortschreitender Genesung konnten wir ein Nachlassen dieser vasoconstrictorischen Grundeinstellung beobachten. Die Normalisierung der einzelnen Parameter der physikalischen Kreislaufanalyse setzte unterschiedlich ein. Richtzahlen werden gegeben.

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Longitudinal section test of a physical circulatory analysis in infectious and convalescent children

Summary Longitudinal section tests were carried out during the acute stage of the infectious disease in a total of 364 children aged from 4 to 14 and during convalescence; these were based on a physical analysis of the circulation as suggested by Wezler and Böger. During the initial days of illness, a vasoconstrictive circulatory sparing effect with near central circulatory action was observed, as reported by Duesberg and Schroeder. The values of V_s, V_m, HJ, E/W, H_A, H_L, p were chiefly, and those of PWG, E, W, p_s, p_d, predominantly increased. The basic vasoconstriction remitted with progressive recovery and normalisation of the various parameters of the physical circulatory analysis occurred at different intervals. Figures are given as guidance.

     

Adrenoceptors and the lung: their role in health and disease

- and -Adrenoceptors have each been divided into two subgroups (₁, ₂, ₁ and ₂). The basic mechanisms underlying the adrenoceptor/effector coupling are complex and vary for the -, but not for the -subpopulations. Adrenoceptors of the bronchi and the lung show a special pattern of distribution and response, ensuring that the airway system works as a functionary unit. Dysfunctions of adrenoceptormediated effects have been suggested to contribute to some important paediatric disorders such as hyaline membrane syndrome, wet lung, bronchial asthma, cystic fibrosis, and pertussis. Drugs which act on the adrenergic system influence some of these disorders directly. Further studies applying modern techniques to receptor research are needed in order to clarify the basic mechanisms involved in receptor-mediated lung disorders and the activity of drugs in lung tissue.Abbreviations AC adenylate cyclase - ADP adenosine diphosphate - -R -adrenoceptor - cAMP cyclic adenosine monophosphate - CF cystic fibrosis - GDP guanosine diphosphate - GTP guanosine triphosphate - IAP islet activating protein     

Alpha-1-antitrypsin in umbilical cord serum: Pi phenotypes and relationships with idiopathic respiratory distress syndrome

The concentrations and phenotypes of serum alpha-1-antitrypsin (_IAT) were determined in 650 newborn infants.The distribution of these 650 subjects among the various Pi phenotypes confirms the higher frequency reported for the PiS allele in Latin populations.Serum _IAT levels vary between one phenotype and the other. Besides, at birth, infants weighing more than 2,500 g have _IAT levels significantly higher (P<0.001) than infants weighing less than 2,500 g; this difference in serum _IAT concentrations is due to the low _IAT levels found in preterm infants. The significantly lower _IAT concentrations found in preterms is associated with a higher risk of developing IRDS and with a mean birth weight under 2,000 g. Infants who develop IRDS frequently have lower _IAT levels than those who do not develop the syndrome, independently from body weight.On the basis of serum _IAT quantitation, newborn infants may be separated into two groups, characterized respectively by concentrations above or below 150 mg%. From our data, it appears that if the group with an _IAT concentration lower than 150 mg% is phenotyped, it is possible to differentiate infants with a high risk of fatal IRDS from individuals with a pathological phenotype.Research supported in part by a grant from Consiglio Nazionale delle Ricerche     

Fetal bile acids in congenital biliary atresia —production in the liver and clinical significance

The 1-hydroxy bile acids have been said to be fetal bile acids. These bile acids were evaluated in eight patients with congenital biliary atresia (CBA) using gas chromatography-mass spectrometry. At the time of operation 1,3,7,12-tetrahydroxy-5-cholan-24-oic acid (CA-1-ol) and 1,3,7-trihydroxy-5-cholan-24-oic acid (CDCA-1-ol) were 0.46 ± 34 g/ml and 0.72 ± 0.45 g/ml, respectively, which was significantly higher than in the control group. The percentage CA-1-ol of total bile acids showed a tendency to decrease as age advanced. The grade of hepatic fibrosis ranged from F₂ to F₃ and the values and percentages of CA-1-ol and CDCA-1-ol were relatively higher in F₂ than F₃ patients. The percentage of total bile acids gradually increased in patients without sufficient bile flow but fell sharply after Kasai's procedure in the patients with sufficient bile flow. It appears that fetal bile acids are produced in the livers of CBA patients in the same way as in fetal liver, and that production continues in patients without good bile secretion even after Kasai's procedure. These results suggest that these hydroxylases are reactivated in CBA patients.     

α-aminoadipic aciduria and persistence of fetal haemoglobin in an oligophrenic child

The case of a mentally retarded girl with a number of dysmorphic features, Raynaud's phenomenon, hypotonia and petit mal seizures is presented. Laboratory investigations showed -aminoadipic aciduria and a high level of fetal haemoglobin. Oral l-lysine loading resulted in a marked increase of -aminoadipic acid in blood and urine. After 3 months of pyridoxine medication the increase of -aminoadipic acid in blood and urine during the oral l-lysine loading test was less than in the test before treatment. A normal degradation rate of dl--amino [1-¹⁴C] adipic acid in fibroblasts of the patient, as measured by ¹⁴CO₂ production, did not indicate a primary enzyme defect in the -aminoadipic acid transamination or decarboxylation steps.The persistent HbF could be the result of stress on the erythropoiesis by a secondary induced defect in an early stage of haemoglobin synthesis in which -amino--ketoadipic acid, a structural analogue of -aminoadipic acid, is an intermediate.     

Correlations between age-dependent protein and lipid concentrations in plasma and platelet functions in children

The correlations between the levels of various plasma proteins and lipids and platelet function on glass and platelet factor 3 (PF 3)-availability in children of different age-groups were investigated. Several statistically significant positive and some significant negative correlations were found. Although conclusions based solely on such correlations should be considered with reservation, in our opinion the following factors should stimulate platelet function: prealbumin (adhesion and PF 3-availability in all age-groups, aggregation—specifically for children in puberty); ₁-antitrypsin (PF 3-availability); ₂-macroglobulin (platelet spreading capacity, PF 3-availability); plasminogen (platelet adhesion and aggregation—specifically for boys in puberty); caeruloplasmin (number of free adhering platelets spreading capacity); lysolecithin and lecithin (time-dependent increase of spontaneous platelet adhesion and aggregation, PF 3-availability); and free fatty acids (FFA) (PF 3-availability).Plasminogen and complement component C3 show a negative relationship to the time-dependent increase of spontaneous platelet adhesiveness and aggregability in platelet-rich plasma.     

Hypocalcaemia induced by glucocorticoids in a child with hypoparathyroidism treated with 1-α-hydroxyvitamin D3

Glucocorticoids tend to lower the intestinal absorption of calcium, leading to a negative calcium balance. A 7-year-old girl with hypoparathyroidism was maintained as normocalcaemic on 1--hydroxyvitamin D₃ (1D₃) and calcium gluconate lactate. During an episode of aplastic anaemia she was treated with prednisolone, with a subsequent dramatic fall of serum calcium despite 1D₃ treatment and serum 1,25-dihydroxyvitamin D₃ (1,25(OH)₂D₃) in the high-normal range. Glucocorticoids seem to have interfered directly with cellular events reponsible for intestinal absorption of calcium.Abbreviations 1D₃ 1--hydroxyvitamin D₃ - 1,25(OH)₂D₃ 1,25-dihydroxyvitamin D₃ - PTH parathyroid hormone - PTE parathyroid extract - RIA radioimmunoassay - TRP tubular reabsorption of phosphorus     

In vivo recording of the glucose- and disaccharide-evoked potentials from the human jejunum in infancy

Using a pair of Ag–AgCl electrodes, the sugarevoked potentials (PD) were recorded from the jejunum of human infants in vivo by infusion of Ringer's solution containing glucose or a disaccharide through an intestinal tube. One of Ag–AgCl electrodes was fixed inside the tip of the intestinal tube and the other was placed in the hypodermis. The sugars tested were d-glucose, lactose, maltose and d-xylose. In the case of d-glucose, a simple Michaelis-Menten relation was seen between the amplitude of PD and concentration, and the values of Kt and PD _max for the control infants were 14.4±2.8 mM and 11.4±2.3 mV, respectively (mean±SD, n=8). Disaccharides gave a similar relationship over a wide range of concentrations except for very high concentrations, where Lineweaver-Burk plots of the data deviated slightly upward from the linear plot obtained at lower concentrations. Extrapolation of the linear segment of the plots for the disaccharide-evoked potential crossed the ordinate at the same point as the line for glucose-induced potentials. The mean magnitude of PD _max for glucose in infants with intractable diarrhea was significantly lower (about 56%) than that in the control infants, but the Kt values were not significantly different. The ratios of PD induced by 100 mM maltose to PD induced by 100 mM glucose in the patients with intractable diarrhea were not different from those in controls, while the ratios of PD induced by 100 mM lactose to PD induced by 100 mM glucose were decreased. At the recovery stage of intractable diarrhea, the magnitude of PD _max for glucose and the ratio of PD induced by 100 mM lactose to PD induced by 100 mM glucose were increased to the control level.No problems were encountered in the present study and the method may be useful in examination of digestion and absorption of sugars in infants.     

Hypertension in a neonate with 11 β-hydroxylase deficiency

A female newborn infant with ambiguous genitalia was found to have hypertension (121/82 mm Hg) immediately after birth. The plasma testosterone (T) (0.73 nmol/l), 4-androstenedione (4-A) (5.9 nmol/l), dehydroepiandrosterone (DHEA) (8.9 nmol/l), as well as 17 OH-hydroxyprogesterone (17 OHP) (152 nmol/l) were elevated. The diagnosis of 11-hydroxylase deficiency was finally established on the basis of elevated plasma eleven-deoxycortisol (compound S) (>0.6 mol/l) and confirmed by the normalisation of the blood pressure during hydrocortisone therapy. Our case is probably the youngest patient with 11-hydroxylase deficiency in whom the hypertension was found at birth.Abbreviations T testosterone - OHCS 11 hydroxycorticosteroids - DOC deoxycorticosterone - THDOC tetrahydrodeoxycorticosterone - THS tetrahydro S - 17 OHP 17 OH-hydroxyprogesterone - DHEA dihydroepiandrosterone - DO 11-deoxycorticosterone - ₄A androstenedione     

Sisters with α-mannosidosis and systemic lupus erythematosus

Alpha-mannosidosis is an autosomal recessive disorder caused by deficiency of lysosomal -mannosidase (LAMAN). Here, we report two sisters with -mannosidosis who developed systemic lupus erythematosus (SLE). The sisters were both homozygous for a one bp deletion within the LAMAN gene resulting in a truncated gene product. The coincidence of -mannosidosis and SLE are discussed with regard to both clinical and molecular findings. Conclusion:-mannnosidosis may contribute to the onset of systemic lupus erythematosus in predisposed patients.Abbreviations ACR American College of Rheumatology - LAMAN lysosomal -mannosidase - SLE systemic lupus erythematosus     

Mild adrenal 3β-hydroxysteroid dehydrogenase deficiency in children with accelerated growth,premature pubarche and/or hirsutism

We studied eight children (seven girls and one boy, of whom three are siblings) with accelerated growth, premature pubarche and/or hirsutism between the ages of 7 and 9.5 years. These patients were studied for the presence of adrenal steroidogenic defect by adrenocortico tropic hormone (ACTH) stimulation testing (Cortrosyn, 0.25 mg iv bolus dose). Baseline and stimulated 17-hydroxypregnenolone (⁵-17P), 17-hydroxyprogesterone (17-OHP), dehydroepiandrosterone (DHEA), androstenedione (⁴-A), testosterone, and cortisol levels were measured. Six children, including three siblings, were diagnosed with a mild form of 3-hydroxysteroid dehydrogenase deficiency based upon the elevated response of serum ⁵-17P and the elevated ratio of ⁵-17P/17-OHP and of DHEA/⁴-A to ACTH stimulation. Thus, a mild form of 3-hydroxysteroid dehydrogenase deficiency may not be uncommon in children with accelerated growth, premature pubarche and/or hirsutism and routine assay of ⁵-steroid metabolites should be generally available.     

The clinical phenotype of β and δβ thalassemias in Greece

Based on precise evaluation of hematological findings and clinical manifestations, the relationship between genotype and clinical phenotype was studied in 475 Greek patients with and thalassemias. Almost all known genotypes are included in this series, but the most frequent was homozygous ^th high A₂ (71.6%), ^th/^th silent (7.4%), ^th/^oth high F (6.3%) and ^th/^th Dutch (6.3%).In general, the phenotype was related to the genotype, though clinical heterogeneity was detected among patients with the same genotype. The severe type of thalassemia major was most commonly found in homozygous ^th patients mainly of ^o/^o and ^o/⁺ genotypes while homozygous ⁺ patients had milder clinical manifestation. Furthermore a small group of patients, characterized as homozygous ⁺⁺ (HbF<30%) had mild thalassemia intermedia. In addition mild thalassemia intermedia was principally related with homozygous ^oth, and compound heterozygous ^th/^th silent I, and less frequently with other genotypes such as compound heterozygous with ^th/^th Dutch, ^th/^th silent II, ^th/^th high F or Lepore.It was shown that precise genetic characterization and clinical evaluation is of primary importance in predicting the prognosis and formulating the proper treatment for the individual patient with thalassemia.     

Lethal osteogenesis imperfecta: abnormal collagen metabolism and biochemical characteristics of hypophosphatasia

We have examined collagen from a patient with the rare type IIC form of perinatally lethal osteogenesis imperfecta, in whom biochemical characteristics of hypophosphatasia were also apparent. In addition to normal 1(I) and 2(I) chains, there were chains overmodified along their lengths. Unexpectedly, the thermal stability of molecules containing these chains was normal. This suggests the existence of a structural mutation causing delayed triple helix formation, situated in either the 1(I) or 2(I) C-terminal propeptide. Since collagen synthesised by fibroblasts from each of the patient's parents was normal, the mutation was probably newly arising and dominant. In contrast to other reported cases of lethal osteogenesis imperfecta, not only was the secretion of collagen by cultured fibroblasts considerably retarded, but that of non-collagenous proteins was also severely impaired.Abbreviations OI osteogenesis imperfecta - EMEM Eagle's minimum essential medium - SDS-PAGE sodium dodecyl sulphate-polyacrylamide gel electrophoresis - T_m thermal denaturation temperature     

Severe combined immunodeficiency caused by a splicing abnormality of the <Emphasis Type="Italic"> CD3δ </Emphasis>gene

CD3 deficiency is a recently identified rare form of severe combined immunodeficiency. We analysed the CD3 gene in a Japanese family with severe combined immunodeficiency. The patients lacked T-cells with normal numbers of B-cells and natural killer cells in peripheral blood. We found a novel homozygous mutation in the splicing acceptor site of intron 2 (IVS2–2AG) in these patients. Analysis of patients mononuclear cells revealed the CD3 splicing abnormality. Chest X-ray films and computed tomography revealed small sized thymuses in these patients. Conclusion:The CD3 gene should be analysed in patients with severe combined immunodeficiency lacking T-cells with normal B- and natural killer cells irrespective of the thymus size.     

Changes of the protein composition of the cerebrospinal fluid in childhood acute leukemia

In cerebrospinal fluid from 26 children suffering from acute lymphocytic leukemia or lymphosarcoma, prealbumin, albumin, ₁-antitrypsin, ₂-macroglobulin, transferrin, ₁C/₁A-globulin, IgG, and IgA were estimated in correlation with cell count and total protein determinations. IgM was demonstrated by means of double diffusion. The cerebrospinal fluid (CSF) from 9 children with a first complete remission of bone marrow were compared with 10 patients suffering from central nervous system leukemia, and 10 more children who had achieved a further hematological as well as central nervous system remission. The results showed no significant correlation between cell count and protein content, nor between cell count and the increase of individual proteins. The increase of ₂-macroglobulin, as well as the qualitative demonstration of IgM in the CSF, reflected an enhanced permeability of the blood-CSF barrier. We discuss the problems in determining a so-called normal CSF cell count due to a simultaneous increase in a single or all protein components, which was frequently the case.

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Zusammenfassung Bei kindern mit akuter lymphatischer Leukämie wurden parallel zur Bestimmung von Zellzahl und Gesamteiweiß Präalbumin, Albumin, ₁-Antitrypsin, ₂-Makroglobulin, Transferrin, ₁C/₁A-Globulin, IgG und IgA im Liquor quantitativ erfaßt. IgM wurde qualitativ nachgewiesen. Die Untersuchung erstreckte sich auf Kinder, die noch keine Zeichen einer Meningosis leucaemica geboten hatten, an einer Meningosis erkrankt waren oder sich in einem erscheinungsfreien Intervall befanden. Eine signifikante Korrelation zwischen der Zellzahl und dem Gesamteiweiß ergab sich ebensowenig wie zwischen der Zellzahl und der Vermehrung einzelner Proteine. Eine meßbare Vermehrung ₂-Makroglobulin und der qualitative Nachweis von IgM im Liquor deuteten auf eine erhöhte Permeabilität der Blut-Liquor-Schranke hin. Auf die Problematik der sogenannten normalen Liquorzellzahl wird an Hand der häufig gleichzeitig vorhandenen Erhöhung einzelner oder aller Eiweißkomponenten hingewiesen.