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Biomedical subjects

K Kruse

Publications and source records attributed to K Kruse.

At least 91 records · Page 5Linked to original sources
PubMed1989

[Free and sulfoconjugated plasma catecholamines in premature infants and mature newborn infants after birth].

Sulfate conjugation represents a major pathway for the inactivation of free catecholamines. We investigated the ability of newborns to protect the body against an overflow of free catecholamines by sulfoconjugation. No difference of free catecholamines in the umbilical artery was found in preterm and term newborns. Furthermore, preterm and term newborns were able to form sulfoconjugated catecholamines. In term newborns, but not in preterm newborns, there was a significant relationship between free catecholamines and their respective sulfoconjugated forms (p less than 0.001). In comparison to term infants sulfoconjugated dopamine and norepinephrine were significantly decreased in preterm newborns, although the placental extraction rates of these catecholamines were markedly lower in preterm infants. This favors the conclusion, that synthesis rather than increased degradation may be responsible for the low levels of sulfoconjugated catecholamines in preterm infants. Thus, preterm newborns might be less able to inactivate free catecholamines by sulfoconjugation. The clinical importance of these results concerning treatment of preterm newborns with dopamine and noradrenaline has yet to be established.

Catecholamines↗
PubMed1988

[Intact serum parathormone (PTH 1-84). A suitable parameter for the diagnosis of calcium metabolism disorders].

Intact parathormone (PTH 1-84) was measured with a new immunoradiometric method in serum from 83 children and adults with various abnormalities of calcium metabolism. The results were compared with those of an assay of midregional PTH fraction (44-68). Both measurements discriminated well between normal controls and patients with primary or secondary hyperparathyroidism. In patients in chronic renal failure intact PTH measurement was best for demonstrating parathyroid secretion. An important advantage of the new method is in the diagnosis of PTH hyposecretion in hypoparathyroidism and of tumour hypercalcaemia, which is not possible by mid-regional PTH determination. Intravenous injection of calcium (2 mg/kg over 5 min) and of synthetic PTH fragment (6 U/kg 1-38 hPTH over 2 min) caused a reduction in intact serum-PTH to about half the initial value after five minutes. Measuring intact PTH is thus a suitable method for determining both raised and decreased parathyroid secretion in disease and in the course of function tests. It is simple to perform, subject to only minor interference, and thus suitable also as a routine laboratory test.

Adolescent↗
PubMed1988

Phaeochromocytoma without symptoms: desensitization of the alpha- and beta-adrenoceptors.

A 16-year-old boy is described who had a relapse of a phaeochromocytoma 6 years after an initially successful tumour resection. The relapse was suspected after routine testing of urinary catecholamine excretion and was confirmed by scintigraphy with 123I-meta-iodobenzylguanidine, computed tomography and magnetic resonance imaging. The plasma norepinephrine level was 3082 pg/ml (normal less than 500 pg/ml); the plasma epinephrine level was in the normal range. Surprisingly, our patient had no symptoms, including hypertension. The density of the alpha- and beta-adrenoceptors on circulating blood cells was decreased. Postoperatively the plasma catecholamine levels were in the normal range. Three months after surgery the adrenoceptor density was almost normal. We conclude that the absence of clinical symptoms was probably due to desensitization of the adrenoceptors. After a successful operation to treat phaeochromocytoma, long-term monitoring of catecholamines is necessary to rule out an asymptomatic relapse.

Adolescent↗
PubMed1988

[Disorders of calcium and bone metabolism in glucocorticoid treatment].

The effects of glucocorticoids on calcium and bone metabolism were investigated in 11 children (aged 6 months to 13 years) who were treated with dexamethasone, prednisolone and depot-ACTH because of different disorders. Alkaline phosphatase activity and osteocalcin in serum, representing indices of osteoblastic bone synthesis, and urinary hydroxyproline in relation to creatinine in morning fasting urine specimens, an index of osteoclastic bone degradation, decreased by 53-61% from baseline (P less than 0.01), with a highly significant relationship of all 3 indices to each other. Additional influences of glucocorticoids were hyperphosphaturia due to decreased renal phosphate reabsorption not mediated by secondary hyperparathyroidism, as well as marked hypercalciuria. As the consequence of the present study the following prophylactic or therapeutic recommendations are given during steroid-treatment: 1. Approvement of the negative balance of calcium and phosphate by correcting the hypercalciuria with hydrochlorothiazide, and the hypophosphatemia with oral phosphate and 2. in elder children with osteoporosis, stimulation of the decreased osteoblastic bone formation by sodium fluoride.

Adolescent↗
PubMed1988

[Hypoparathyroidism and pseudohypoparathyroidism. New aspects of the pathogenesis, diagnosis and therapy].

Recent elucidations of normal and disturbed parathyroid secretion and action have provided a basis for the understanding of general principles of endocrinology. Advanced laboratory methods and simplified study protocols have improved the diagnosis of hypoparathyroidism and pseudohypoparathyroidism, the most important feature of which is chronic hypocalcemia. The knowledge of these disorders is of great importance to the pediatrician since they occur in childhood, and the consequences of prolonged misdiagnosis and inadequate treatment may be serious.

Amino Acid Sequence↗
PubMed1988

[Intervertebral disk prolapse in childhood].

Four girls and one boy aged 11 to 16 years presented with lumbar disc disease. The main aspects of the disorder in children and its differences to adults are emphasized. The prognosis following surgical treatment is favourable.

Adolescent↗
PubMed1987

Endocrine control and disturbances of calcium and phosphate metabolism in children.

Most disorders of extracellular calcium and phosphate metabolism in childhood can be attributed to primary increased or decreased secretion/action of 1,25-dihydroxyvitamin D3 and parathyroid hormone or primary increased or decreased urinary excretion of phosphate and calcium. Based on this pathogenetic classification the most important diseases related to calcium and phosphate metabolism will be discussed.

Calcitriol↗
PubMed1987

A simplified diagnostic test in hypoparathyroidism and pseudohypoparathyroidism type I with synthetic 1-38 fragment of human parathyroid hormone.

Bovine parathyroid extract of E. Lilly (Indianapolis), which has been used to differentiate pseudohypoparathyroidism (PHP) and hypoparathyroidism (HP) is no longer available. We therefore evaluated the usefulness of the synthetic 1-38 fragment of human parathyroid hormone (1-38 hPTH), the biologically active part of the intact hormone, in a simplified modification of the traditional Ellsworth-Howard test. 1-38 hPTH was slowly injected over 2 min at a dose of 0.5 micrograms/kg body weight in healthy children and adults (n = 7), as well as in children with HP (n = 4) and PHP (n = 4). In the controls PTH administration induced a significant rise of plasma cAMP (at least 146 nmol/l), urine cAMP and serum prolactin, as well as a decrease of the tubular phosphate reabsorption. The respective responses were similar in the children with HP but markedly impaired in the patients with PHP. In four healthy adults a second PTH administration at an interval of 3 h induced a comparable response of cAMP and prolactin, indicating that these PTH effects are reproducible and not impaired by the first injection. The two PTH administrations caused a significant but variable rise of serum 1,25-dihydroxy-vitamin D in all four adults. In conclusion 1-38 hPTH has effects on the kidney and anterior pituitary similar to purified parathyroid extracts but has the advantage of being chemically and biologically defined and well tolerated. Since timed urine collections may be unreliable in young children, the plasma cAMP measurement at 5 and 10 min after the PTH injection is a good substitute for the traditional Ellsworth-Howard test, peak values over 100 nmol/l being regarded as a normal response.

Adolescent↗
PubMed1987

Infantile glycerol kinase deficiency--a condition requiring prompt identification. Clinical, biochemical, and morphological findings in two cases.

Infantile glycerol kinase deficiency (GKD) is an X-linked genetic disease characterized clinically by adrenal insufficiency and muscular dystrophy. The enzyme defect leads to increased levels of glycerol in blood and urine, which can be used for diagnosis. Without recognition of this condition, the chances for life-saving steroid treatment and for genetic counselling are missed. We report clinical, endocrinological, biochemical, and morphological findings in two non-related boys. One of them died in early infancy. The other is thriving at the age of 2 years although he is suffering from a myopathy not distinguishable from Duchenne muscular dystrophy. We discuss when to suspect and how to confirm the diagnosis of infantile GKD, and under what precautions the condition is detectable by commonly used screening procedures for inborn errors of metabolism.

Adrenal Insufficiency↗
PubMed1987

Monomeric serum calcitonin and bone turnover during anticonvulsant treatment and in congenital hypothyroidism.

Decreased basal and calcium-stimulated calcitonin serum levels have been found in children with congenital hypothyroidism and in those receiving anticonvulsant drugs. The purpose of our investigation was to confirm these results using a new technique for calcitonin measurement and to study the effect on bone turnover. Calcitonin serum levels were measured with two different antibodies before and after a low-dose Ca infusion in patients receiving phenytoin, primidone, carbamazepine, or valproate and in patients with congenital hypothyroidism receiving L-thyroxine. In comparison with control values, basal and Ca-stimulated extractable calcitonin, representing the monomeric and biologically active form of the hormone, were moderately decreased in patients with epilepsy receiving phenytoin and primidone, and severely decreased in patients with hypothyroidism. Ca and bone metabolism were normal, except for an elevated renal threshold for phosphate (indicating phosphate conservation) in patients receiving phenytoin and primidone, and increased fasting urinary excretion of Ca and hydroxyproline (indicating increased bone resorption) in patients with hypothyroidism. The secretory capacity of the C cells for monomeric calcitonin is decreased in children receiving treatment with some, but not all, anticonvulsant drugs, and lacking in patients with hypothyroidism. Patients with calcitonin deficiency may be prone to osteopenia if the tendency to increased osteoclastic activity is aggravated by secondary hyperparathyroidism in patients with epilepsy receiving phenytoin and primidone or by inappropriate thyroid replacement therapy in patients with hypothyroidism.

Adolescent↗
PubMed1987

Evidence for transient peripheral resistance to parathyroid hormone in premature infants.

Serum immunoreactive parathyroid hormone (iPTH), ionized calcium, the urinary cyclic AMP/creatinine ratio (cAMP/Cr) and some indices of bone turnover (alkaline phosphatase (AP), serum osteocalcin, and the urinary total hydroxyproline/creatinine ratio (OH-P/Cr)) were measured in 26 preterm infants during the first 4 weeks of life. Despite of stimulated parathyroid gland activity cAMP/Cr, AP, osteocalcin and OH-P/Cr were low during the first week. Thereafter iPTH decreased, whereas cAMP/Cr, and the indices of bone turnover increased, reaching high-normal values (in comparison to full-term infants) during the second and third week of life. Serum iPTH was negatively correlated to cAMP/Cr in the first week (r = -0.61, p less than 0.01), whereas the relationship became positive during the second (r = 0.47, p less than 0.05) and third (r = 0.54, p less than 0.05) week of life indicating maturation of the renal response to PTH. The study supports the concept that in premature infants a transient pseudohypoparathyroid-like state is present during the first week of life reflecting an immaturity of renal and possibly bone response to PTH. This may be an etiological factor in hypocalcemia of prematurity.

Birth Weight↗
PubMed1987

Increased concentrations of HbAlab in hereditary fructose intolerance and galactosemia.

In patients with diabetes mellitus nonenzymatic glycosylation of hemo-globin is a result of increased blood glucose concentrations. In analogy glycosylated hemo-globin fractions were determined in 23 patients with hereditary fructose intolerance (HFI) and 8 patients with galactosemia (G) by means of hemoglobin chromatography on a column packed with Bio-Rex 70 resin. The concentrations were compared to those of 14 control patients and 43 patients with type 1 diabetes mellitus. Compared to controls, in HFI- and G-patients HbAlab was significantly increased. In contrast diabetic patients presented with a marked and significant increase of the HbAlc fraction. When purified hemoglobin was incubated with different monosaccharides respectively monosaccharide phosphates, an increase of HbAlab resulted mainly after galactose and fructose-1-phosphate. The determination of HbAlab in patients with HFI and G is considered a possible means of metabolic control.

Carbohydrate Metabolism, Inborn Errors↗
PubMed1987

[Significance of serum fructosamine in the metabolic control of children and adolescents with type I diabetes mellitus].

In 1982 Johnson et al. described a simple colorimetric assay for measuring glycated proteins, termed fructosamine, in the serum of adults with diabetes mellitus and demonstrated this to be a useful index of intermediate glucose control (1-3 weeks). Our study was designed to show this as well in children and adolescents with type I diabetes mellitus. Serum fructosamine was determined in 76 children and adolescents with diabetes mellitus, and 111 age-matched controls. In the controls an age-dependency but not sex-dependency could be demonstrated. In the diabetic patients we found a significant correlation between serum fructosamine and HbA1 values (r = 0.87, p less than 0.001). In 6 patients with newly diagnosed diabetes mellitus serum fructosamine concentrations decreased at a faster rate than HbA1 values. The fructosamine assay is rapid, technically simple and inexpensive, and is at least a useful addition or perhaps an alternative to HbA1 estimation.

Adolescent↗
PubMed1986

Evaluation of serum osteocalcin as an index of altered bone metabolism.

Recent evidence suggests that the protein osteocalcin is like the bone alkaline phosphatase produced by osteoblasts and circulates in human blood. With the introduction of a radioimmunoassay for serum osteocalcin it was hoped that this test would provide a useful index of altered bone metabolism. Therefore serum osteocalcin was measured in 88 controls and 112 patients with disorders of calcium and phosphate metabolism, isolated elevation of alkaline serum phosphatase in the absence of disease (isolated hyperphosphatasaemia) and children prone to osteopenia. In the controls serum osteocalcin was higher in children less than 15 years (median and range: 11.9, 7.7-15.3 ng/ml) than in adults (3.7, 2.6-5.2 ng/ml) and was highly correlated to alkaline serum phosphatase activity (r = 0.87, n = 88, P less than 0.01). Osteocalcin was elevated in primary hypoparathyroidism, low in untreated hypoparathyroidism but normal in hypoparathyroidism (including pseudohypoparathyroidism) during vitamin D treatment. The bone protein was low-normal and increased to high-normal levels during vitamin D therapy in vitamin D deficiency rickets and familial hypophosphataemic rickets, but remained low in patients with end organ resistance to 1,25-dihydroxyvitamin D. Osteocalcin (and urinary hydroxyproline) were not elevated in isolated hyperphosphatasaemia, indicating that mechanisms other than increased bone turnover may account for the markedly elevated serum alkaline phosphatase activity in these subjects. Osteocalcin was decreased in children with diabetes mellitus type I and in patients on glucocorticoid treatment, indicating decreased bone formation. It is concluded that the measurement of serum osteocalcin seems to be a reliable index of bone formation provided that the vitamin D status and renal function are normal.(ABSTRACT TRUNCATED AT 250 WORDS)

1-Carboxyglutamic Acid↗
PubMed1986

Exclusion of close linkage between the parathyroid hormone gene and a mutant gene locus causing idiopathic hypoparathyroidism.

A family is presented in which the mother has transmitted primary hypoparathyroidism with early onset and serum PTH (44-68) and C terminal deficiency to her two sons. Restriction enzyme analysis of allelic variation at the PTH gene locus revealed that the disease and the PTH alleles segregate independently. It is therefore concluded that the primary molecular defect leading to this form of hypoparathyroidism is not located within the PTH gene itself.

Adolescent↗
PubMed1986

[Congenital piriformo-thyroid fistula as a cause of recurrent suppurative thyroiditis].

A previously healthy five year old boy presented with suppurative, febrile thyroiditis of the left lobe with pronounced general and local signs of inflammation, normal thyroid function and lack of thyroid antibodies. Intravenous antibiotic therapy improved the condition quickly. Six and nine months later however recurrent left lobe thyroiditis occurred. Endoscopic examination showed an internal fistula from the left piriform sinus to the left thyroidal lobe which was cut out. The case report confirms previous communications, that recurrent suppurative thyroiditis is nearly always caused by a piriform sinus fistula, probably a fourth pharyngeal pouch remnant which can be cured surgically.

Child, Preschool↗