Search

Degn, J. K., Wamberg, S., Engel, K. and Kildeberg, P. (Departments of Paediatrics, Clinical Chemistry, and Physiology, Odense University, Odense, Denmark). Metabolic alkabsis in obstructive vomiting. Volume depletion and balance of net acid. Acta Paediatr Scand, 63: 537, 1974.—Pre-operative balances of sodium, potassium, calcium, magnesium, chloride, and phosphorus were determined in eleven infants with hypertrophic pyloric stenosis. Additional measurements included acid-base status of blood and urine and concentrations of electrolytes in serum. From the balance data, the rate of retention of net acid (NAB), the pre-operative change in chloride space (AECW), and the effect of extracellular volume expansion on blood “base excess” (BE) were derived. The observed pre-operative change in blood BE was partitioned in retentional, dilutional, and distributional components by comparing estimates of such components based on known physiological constants with the results of multiple oorrelation analysis involving five predictor variables. The results indicate that the fall in blood BE to the normal range was almost entirely accounted for by volume expansion, and that retained net acid was probably neutralized by skeletal base.

Since lesions characteristic of Duchenne muscular dystrophy (DMD) are reproduced in animals by vascular ischemia from aortic ligation plus serotonin (5-HT) injections, 5-HT levels and serotonin 14C cellular transport mechanisms were studied in 14 boys with DMD. Plasma 5-HT levels were no different from those of 23 controls, but platelet 5-HT content was moderately reduced. No abnormalities were found in serotonin 14 C efflux or monoamine oxidase activity. These studies suggest that a simple increase in circulating 5-HT or decrease in its enzymatic degradation is not present in DMD. The major finding was a markedly reduced initial rate of accumulation of serotonin 14 C into platelets from DMD patients. This finding may be of significance to the proposed vascular pathogenesis for DMD in raising the possibility of an alteration in biogenic amine deactivation at the microvascular level.

Morphological and ecological studies as well as investigations of essential oils reveal thatValeriana panciciiHal. &Bald., an endemic of southern Montenegro, must be considered a subspecies ofValeriana celtica. ThusV. celtica includes 3 subspecies: subsp.celtica (subsp.penninaVierh.) endemic in the western, subsp.norica in the eastern Alpes and subsp.pancicii in Montenegro (Abb. 2). While the two alpine subspecies are polyploids, subsp.pancicii proved to be diploid on x=12, and may be supposed to stand closest to the ancestor of theV. celtica-complex. The occurence of subsp.pancicii in Montenegro seems to indicate an East Mediterranean-montane descent of the complex.

A method for direct determination of hemoglobin oxygen saturation at predetermined values for pH, PCO2 , PO2, and temperature is described, and its applicability to studies of the oxygen dissociation curve for whole blood under various controlled conditions is discussed and illustrated.

The construction and transient operation of a differential resistance‐bridge calorimeter which has successfully operated up to 700°C are described. Heat capacity and latent heat of transformation are measured in the calorimeter by absolute methods. The heat capacity of nickel and the heats of fusion of bismuth and cadmium are presented and agree well with previously published data.

Summary Proteins coupled to polystyrene by azo linkages were used as solid adsorbents for the determination of antibodies to soluble proteins. Studies were carried out quantitatively using I131 labeled bovine serum albumin and ovalbumin and their antibodies. Antibodies specifically combined with antigen complexes could be eluted efficiently at pH 3 (citrate buffer) whereas antigens and nonspecifically adsorbed proteins remained firmly bound to the resin. Elution of antibodies took place slightly in 10% sodium salicylate and at 60°C, pH 8, but not in 17% NaCl. Eluted antibodies showed normal precipitin curves. By diluting anti-BSA with normal globulin, it was found that 0.2 μg antibody in 1 mg globulin could be detected. Five-tenths to two micrograms of antibody/mg globulin could be determined within 10% accuracy. The nature of the effective antigen in the polystyrene-protein complex is not well defined. The original aminopolystyrene or the diazopolystyrene quenched with β-naphthol bound antigen proteins very strongly but to a lesser extent than did the diazopolystyrene itself. β-Naphthol-quenched diazopolystyrene treated with BSA was tested and found to be almost as effective as the chemically coupled BSA in purifying antibodies.

The skeletal muscle-binding site of antistriated muscle antibody (ASMAb) from individuals with myasthenia gravis (MG) was established ultrastructurally by means of an indirect immunohistochemical technique with purified sheep antihuman IgG (PSAHG) conjugated to horseradish peroxidase (HRP). Penetration of the HRP-antibody conjugate was greatly enhanced by preparing fragments, the Fab or F(ab′)2 of the PSAHG before HRP conjugation. ASMAb from nine patients with MG localized to the sarcoplasmic reticulum surrounding the I-band. No binding with the myofilaments could be demonstrated.

Metabolic studies in two patients with Refsum's syndrome provide further evidence that the phytanic acid stored has a strictly exogenous origin. On a highly restricted diet the phytanic acid levels in blood and in adipose tissue fell dramatically over a period of months. This was accompanied by a number of manifestations of clinical improvement, including increase in ulnar nerve conduction velocity, increase in strength of several muscle groups, return of some previously unobtainable reflexes, improvement in pain, light touch and position sense, and improvement in objective tests of coordination. There was no improvement in vision or hearing. The results support the hypothesis that accumulation of phytanic acid plays a pathogenetic role in Refsum's disease and justify further therapeutic trials.

Monochromatic light microscopy at 435 mµ shows in Euglena gracilis, the distribution of chlorophyll and the general orientation and geometry of chloroplasts in vivo. In addition it discloses, in swelling chloroplasts, a lamellar pigmented structure. Changes in this structure are observed in extruded swollen chloroplasts treated with lipolytic or proteolytic enzymes. Lipolytic enzymes produce an increase in the number of visible lamellae while proteolytic enzymes disrupt the lamellar array. Correlation of chloroplast swelling behavior and the effects of enzymatic degradation with current electron microscope observations support the following: (1) the pigment lamellae observed in vivo consist of component laminae; (2) the lamellae are separated by sites of swelling; and (3) the integrity of the lamellar structure is primarily dependent upon the intact state of the protein.

Aus den Abstanden der in langsamen Elektronenstrahlen auftretenden Knoten wird die Grose der positiven Kaumladung im Innern des Strahles zahlenmasig abgeschatzt.

By a direct immunofluorescent technic granular deposits of IgG, IgM and C3, alone or in combination, were found in skeletal-muscle blood-vessel walls of 17 of 39 patients with idiopathic inflammatory myopathy. There was an especially high rate (nine of 11) in childhood dermatomyositis. In 142 other patients comprising the control group, only nine, most with disorders known to involve the immune system, had intramuscular vascular deposits of IgG or IgM. Deposition of immunoglobulin and C3 in structurally abnormal muscle fibers was found in a wide variety of diseases. A vascular abnormality mediated through deposits of immunoglobulin and C3, possibly in the form of immune complexes, is hypothesized as one of the mechanisms of muscle injury in certain cases of idiopathic inflammatory myopathy, particularly in dermatomyositis of children.

Summary Simple calculations of the effect of volume depletion on the concentration of titratable base of blood show this effect to be largely adequate to account for commonly observed degrees of metabolic alkalosis in patients with mild or extreme dehydration due to obstructive vomiting. This suggests that hydrochloric acid depletion per se may not be a major determinant of the blood acid-base status in such conditions. Clearly, part of the base equivalents missing in the extracellular fluid may be sought in the cell water; but currently available evidence concerning gastrointestinal transport of acid and base invites a conception of gastrointestinal acid-base homeostasis–and the hypothesis that in obstructive vomiting changes in the composition of stool may appreciably modify the effect of gastric hydrochloric acid depletion. In studies of intracellular buffering and net base balance in gastric alkalosis, it must be realized that the primary extracellular disturbance may be largely contractional and that stool losses (or luminal pooling) of base may be an important variable.

THE influence of various electrolytes on the position (haemoglobin–oxygen affinity) and on the slope (haem–haem interaction) of the oxygen dissociation curve (ODC) of pure haemoglobin solutions has been known for several years1–3. Recent investigations4–6 have revealed the unique role of organic phosphates, such as 2,3-di-phosphoglycerate (2,3-DPG), as determinants of the haemoglobin–oxygen affinity. Thus, with pure haemoglobin solutions, an increase in the concentration of 2,3-DPG in the medium decreases the affinity by competitive reaction with oxygen6, while a decreased concentration of 2,3-DPG is associated with an increased affinity for oxygen. The concentrations of 2,3-DPG used in these experiments are in the range which occurs in normal or abnormal physiological conditions in the intact erythrocyte. Because variations in concentrations of 2,3-DPG do not appear to influence the sigmoid shape of the ODC but only its position, these compounds presumably do not affect the haem–haem interaction6.

n/a

Fresh frozen sections of mature skeletal muscle fibers from patients with genetically determined "absence" of skeletal muscle phosphorylase (McArdle's disease) have no histochemical phosphorylase activity. That regenerating muscle fibers, in vitro and in vivo, from such patients do have histochemical phosphorylase activity present suggests a loss of enzyme activity with fiber maturity.

Hydrocortisone, despite its lack of effect when used alone in a rat nervemuscle preparation, abolished the increase in muscle single-twitch tension produced by the anticholinesterase drugs neostigmine and pyridostigmine. Once the steroid had abolished the effect of these drugs, equivalent doses of the cholinesterase inhibitors were less effective than they had been before steroid was given. The results of these experiments indicate that a corticosteroid hormone may have an adverse pharmacologic interaction with anticholinesterase drugs in a mammalian nerve-muscle preparation. This significant adverse interaction might be the reason some patients with myasthenia gravis do not respond as well with corticosteroid plus anticholinesterase as with corticosteroid alone.

(1960). A New Acid-Base Nomogram an Improved Method for the Calculation of the Relevant Blood Acid-Base Data. Scandinavian Journal of Clinical and Laboratory Investigation: Vol. 12, No. 2, pp. 177-186.

MAMMALIAN SKELETAL MUSCLE has been studied with histochemical techniques after various experimental procedures such as nerve section,’-4Jo neuronal regeneration,5.6 “foreign” and cross innervation,T-@ and tenotomy.294 The effects of these methods upon the muscle cell were produced by direct involvement of the lower motor neuron innervation or the length of the muscle or both. In the present study, acute complete transection of the high thoracic spinal cord was performed to determine the indirect effects on the gastrocnemius and soleus muscles of adult and newborn guinea pigs. Histochemical and cytochemical changes in the muscles after cordotomy were compared with the effects of peripheral nerve section and skeletal fixation to elaborate on various aspects of the complex nerve-muscle interrelationship.

The influence of 2,3-diphosphoglycerate concentration (DPG) on hemoglobin-oxygen affinity (P50) in whole blood from adults and newborns has been studied. In blood from adults a decrease in DPG of 0.61 mol DPG/mol Hb is associated with a decrease in P50 of 13 mm Hg. In samples from newborns a decrease in DPG of 0.95 mol/mol Hb is associated with a decrease in P50 of 4.7 mm Hg. This difference may be due to a lesser affinity of fetal hemoglobin for DPG, and/or to the effect of DPG on the adult hemoglobin present in newborn blood.

THE neuronal trophic influence on developing skeletal muscle fibers has been the subject of speculation in attempts to understand the pathogenesis of certain congenital neuromuscular disorders, such as infantile spinal muscular atrophy 1 and central core disease. 2 Because direct evidence on the dynamics of the nerve-muscle relationship is impossible to obtain in the human, experimental animals must be used. The sequence of histochemical changes from a mixed to a uniform pattern in the guinea pig soleus extrafusal muscle fibers from the 50th gestational day to 6 weeks postnatal age and prevention of that change by neonatal denervation are described in this study. The developing soleus muscle of the rat and cat was also studied. Methods On fresh, frozen, cryostat sections of 10μ thickness, a battery of ten histochemical reactions was used as detailed elsewhere. 3,4 Our preferred technique for histochemical fiber typing is the myofibrillar adenosine triphosphatase (ATPase)

The ultrastructural development of normal explanted human skeletal muscle in tissue culture has been described in this report. New myoblasts, thought to be derived from satellite cells of explanted muscle, fused to form multinucleated cells which differentiated into mature myofibers. The ultrastructural characteristics were similar to those described in embryonic chick skeletal muscle except for the relatively poor development of the T-system and sarcoplasmic reticulum.

Experiments carried out in anesthetized, ventilated dogs to describe the short-term blood acid-base responses to induction of hypocapnia. In all experiments, a fall in plasma pCO2 to a level of 10 to 15 mm Hg was associated with a prompt rise in blood base excess (BE), reaching maximum in 10–15 mins followed by a secondary slower fall. These changes in blood base excess were evaluated in terms of the fractional contributions by (a) redistribution of bicarbonate within the body fluids (b) accumulation of lactate; (c) residual (non-renal) contribution. By examining the time course, it was found that the observed δ log pCO2/δ pH did not reach a steady value within the experimental period, thus explaining the inconsistency of data previously reported in the literature.

Summary A method for the quantitative assessment of the balance of net acid (NAB) in growing infants with a changing body composition is described. Results of seventy measurements of the daily NAB in healthy growing premature infants ingesting modified cow's milk formulae are reported. The relative contributions of the various determinants of the daily net acid input in the infant differ from those in the normal adult, and growth appears to be associated with negative net acid balances due to base deposition in skeleton and new body water. The daily load of “undetermined anion” (UA) in the diet was found to be the largest single component of the NAB. Moreover, a strong correlation between the rates of dietary UA intake and fecal UA excretion suggested an active regulation of the transintestinal acid-base balance. A model for such regulation, based upon available evidence concerning the transport of acid and base across the gastrointestinal membrane, is proposed and briefly discussed.

SUMMARYA patient has been described with Moebius syndrome in whom there was clinical paralysis of the muscles supplied by the third, fourth, sixth, and seventh cranial nerves. Limb muscle biopsy studies and electromyography of the limb muscle suggest denervation of an “anterior horn cell” type of abnormality in the spinal cord. Moebius was also of the opinion that the syndrome was secondary to lower motor neuron abnormality and that the cord could be involved. The hypogonadism of our patient was secondary to a deficiency of gonadotropic hormone.