Combination macrophage-colony stimulating factor and interferon-gamma administration ameliorates the osteopetrotic condition in microphthalmic (mi/mi) mice.

Abstract:

:Malignant osteopetrosis is a fatal congenital bone disorder characterized by defective osteoclastic function. Death frequently occurs within the first decade of life. The precise molecular defect(s) that causes osteopetrosis is not known. The possibility that osteoclasts, like macrophages, are controlled by interactions with cytokines suggests that these agents may provide a means of increasing osteoclastic function. Macrophage-colony stimulating factor (M-CSF), a cytokine known to enhance macrophage and osteoclast generation, and recombinant human interferon-gamma (rIFN), a cytokine known to stimulate superoxide generation by white cells, were administered to microphthalmic (mi/mi) mice in an attempt to improve the osteopetrotic condition. Each cytokine was administered separately and in combination to neonatal mi/mi mice for 7 consecutive d. Bone turnover, osteoclast numbers, superoxide generation by white cells, and hematocrit were assessed. rIFN, M-CSF, and a combination of the cytokines stimulates oxygen-derived free radical production by white cells and increased bone resorption. rIFN resulted in a reduction in the number of osteoclasts. This reduction in number was ameliorated by M-CSF. M-CSF alone and in combination with rIFN resulted in improved hematopoietic function, increased weight gain, and increased physical activity of the affected mutants.

journal_name

Pediatr Res

journal_title

Pediatric research

authors

Rodriguiz RM,Key LL Jr,Ries WL

doi

10.1203/00006450-199304000-00014

subject

Has Abstract

pub_date

1993-04-01 00:00:00

pages

384-9

issue

4 Pt 1

eissn

0031-3998

issn

1530-0447

journal_volume

33

pub_type

杂志文章
  • Dairy product intake decreases bone resorption following a 12-week diet and exercise intervention in overweight and obese adolescent girls.

    abstract:BACKGROUND:We examined whether increased dairy intake was associated with changes in the levels of bone-related biochemical markers in overweight/obese adolescent girls undergoing a 12-week diet and exercise intervention. METHODS:Thirty-five girls were assigned to a low dairy group (LDa; 0-2 servings/day; n = 16) or a...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1038/s41390-020-0834-5

    authors: Josse AR,Ludwa IA,Kouvelioti R,Calleja M,Falk B,Ward WE,Klentrou P

    更新日期:2020-12-01 00:00:00

  • Newborn polymorphonuclear leukocyte aggregation: a study of physical properties and ultrastructure using chemotactic peptides.

    abstract::N-formyl-L-methionyl-L-leucyl-L-phenylalanine (FMLP) and N-formyl-L-methionyl-L-phenylalanine (FMP) were used to investigate neutrophil (PMN) aggregation. Neutrophils were isolated from healthy adult volunteers and term newborn cord blood. Neutrophil aggregation was measured after the addition of FMLP and FMP. Adult P...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-198312000-00013

    authors: Olson TA,Ruymann FB,Cook BA,Burgess DP,Henson SA,Thomas PJ

    更新日期:1983-12-01 00:00:00

  • Amniotic lamellar body count and congenital diaphragmatic hernia in humans and in a rat model.

    abstract:BACKGROUND:We examined the extent of fetal lung hypoplasia and lung maturation using the amniotic lamellar body count (LBC) in congenital diaphragmatic hernia (CDH). METHODS:We obtained 30 amniotic fluid samples from human CDH cases during cesarean section. We assessed LBC, magnetic resonance imaging (MRI), and ultras...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1038/pr.2012.187

    authors: Watanabe Y,Tsuda H,Kotani T,Sumigama S,Mano Y,Hayakawa M,Sato Y,Kikkawa F

    更新日期:2013-03-01 00:00:00

  • Severe congenital hypothyroidism due to a homozygous mutation of the betaTSH gene.

    abstract::Isolated TSH deficiency leading to hypothyroidism seems to be a rare condition, escaping the diagnosis by neonatal screening programs, which are based on the primary determination of TSH. This is the first report of a case with an autosomal recessive TSH defect caused by a homozygous mutation of the betaTSH gene that ...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-199908000-00007

    authors: Biebermann H,Liesenkötter KP,Emeis M,Oblanden M,Grüters A

    更新日期:1999-08-01 00:00:00

  • Excitation-contraction coupling in the day 15 embryonic chick heart with persistent truncus arteriosus.

    abstract::Ca2+ transients were examined in embryonic chick hearts with an experimentally induced cardiac neural crest-related outflow tract defect known as persistent truncus arteriosus (PTA). In all of the animal models of neural crest-related heart defects, prenatal mortality is too high to be attributed to structural defects...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-199712000-00002

    authors: Creazzo TL,Brotto MA,Burch J

    更新日期:1997-12-01 00:00:00

  • Heart rate turbulence in children--age and heart rate relationships.

    abstract::Heart rate turbulence (HRT) is a chronotropic response of the sinus heart rhythm to ventricular premature beat (VPB). Children show decreasing heart rate together with the maturation of the autonomic nervous system. The aim of the research was to assess the relationship between HRT parameters, age, and heart rate and ...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/PDR.0b013e3181598836

    authors: Kowalewski M,Alifier M,Bochen D,Urban M

    更新日期:2007-12-01 00:00:00

  • Nyctohemeral rhythm of plasma renin activity and plasma aldosterone in children.

    abstract::Circadian rhythms of plasma renin activity (PRA) and plasma aldosterone (PA) were studied in eight healthy children, 7 to 15 years old. Blood samples were obtained at 7:00 AM, 10:00 AM, 1:00 PM, 4:00 PM, 5:00 PM, 8.00 PM, and midnight on day 1 and 4:00 AM and 7:00 AM on day 2. Children had normal activity during this ...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-198205000-00007

    authors: Dechaux M,Broyer M,Lenoir G,Limal JM,Sachs C

    更新日期:1982-05-01 00:00:00

  • Response of bifidobacterium species to growth promoters in human and cow milk.

    abstract::We used an in vitro assay to study and compare the growth-promotional activity of protein and nonprotein components in human milk (HM) and cow milk (CM) samples for infant strains of Bifidobacterium species. HM samples varied considerably in growth-promotion activity for Bifidobacterium bifidum var pennsylvanicus, Bif...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-199102000-00021

    authors: Petschow BW,Talbott RD

    更新日期:1991-02-01 00:00:00

  • Cannabidiol reduces brain damage and improves functional recovery after acute hypoxia-ischemia in newborn pigs.

    abstract::Newborn piglets exposed to acute hypoxia-ischemia (HI) received i.v. cannabidiol (HI + CBD) or vehicle (HI + VEH). In HI + VEH, 72 h post-HI brain activity as assessed by amplitude-integrated EEG (aEEG) had only recovered to 42 ± 9% of baseline, near-infrared spectroscopy (NIRS) parameters remained lower than normal, ...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/PDR.0b013e3182276b11

    authors: Lafuente H,Alvarez FJ,Pazos MR,Alvarez A,Rey-Santano MC,Mielgo V,Murgia-Esteve X,Hilario E,Martinez-Orgado J

    更新日期:2011-09-01 00:00:00

  • Glutaric aciduria type II: in vitro studies on substrate oxidation, acyl-CoA dehydrogenases, and electron-transferring flavoprotein in cultured skin fibroblasts.

    abstract::The previous biochemical evidence had suggested that glutaric aciduria type II (GA II) is due to deficient dehydrogenation of multiple short-chain acyl coenzyme A's (CoA's), bu the precise biochemical mechanism underlying this disease was unknown. We investigated substrate oxidation and in vitro activities of isovaler...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-198012000-00013

    authors: Rhead W,Mantagos S,Tanaka K

    更新日期:1980-12-01 00:00:00

  • Influence of age on the selenium status in Belgium and The Netherlands.

    abstract::Plasma selenium concentration and glutathione peroxidase activity in red blood cells were determined in subjects from different age groups. The selenium level (mean +/- SD) found in infancy (0 to 6 months) was 2 +/- 0.6 micrograms/dl, with the lowest value of 1 microgram/dl observed in a 4-month-old infant. These leve...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-198606000-00021

    authors: van Caillie-Bertrand M,Degenhart HJ,Fernandes J

    更新日期:1986-06-01 00:00:00

  • Adhesion of human neuroblasts to HIV-1 tat.

    abstract::Several neuropathologic findings in infants and children with human immunodeficiency virus type-1 (HIV-1) infection are different from those observed in adults, probably related to the fact that the retroviral infection occurs in the setting of neuro-development. This report describes the interaction and biologic acti...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-199511000-00025

    authors: Cornaglia-Ferraris P,De Maria A,Cirillo C,Cara A,Alessandri G

    更新日期:1995-11-01 00:00:00

  • Age- and dietary-related distributions of hepatic arachidonic and docosahexaenoic acid in early infancy.

    abstract::A dietary-related deficiency of docosahexaenoic acid [C22:6(n-3)] in infant cerebral cortex has been identified. Absence or very low rates of hepatic synthesis from the essential fatty acid precursor, alpha-linolenic acid [C18:3(n-3)], in early life may have been a contributory factor. We have analyzed liver total lip...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-199509000-00015

    authors: Farquharson J,Jamieson EC,Logan RW,Patrick WJ,Howatson AG,Cockburn F

    更新日期:1995-09-01 00:00:00

  • Longitudinal study of umbilical and portal venous blood flow to the fetal liver: low pregnancy weight gain is associated with preferential supply to the fetal left liver lobe.

    abstract::Recent data suggest that umbilical venous perfusion of the fetal liver has an important influence on fetal growth and postnatal liver function, and that maternal factors in late pregnancy modify this circulation. In a longitudinal study of 160 low-risk pregnancies, we determined how umbilical and portal venous blood f...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/pdr.0b013e318163a1de

    authors: Kessler J,Rasmussen S,Godfrey K,Hanson M,Kiserud T

    更新日期:2008-03-01 00:00:00

  • Effects of hypothermia on hypoxia-induced apoptosis in cultured neurons from developing rat forebrain: comparison with preconditioning.

    abstract::In neuronal cultures from the forebrain of 14-d-old rat embryos, transient hypoxia (95% N2/5% CO2, 37 degrees C) for 6 h has been shown to trigger delayed apoptotic death through sequential changes in protein synthesis, whereas preconditioning by a brief episode of hypoxia can rescue neurons. Because hypothermia has b...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-200003000-00017

    authors: Bossenmeyer-Pourié C,Koziel V,Daval JL

    更新日期:2000-03-01 00:00:00

  • Development of Na+-K+-ATPase in neonatal rat brain synaptosomes after perinatal protein malnutrition.

    abstract::Four protein dietary groups of rats were used in this study, namely: 27% casein diet fed throughout gestation and lactation (group C-C); 27% gestation, 10% lactation (group C-D); 10% gestation, 27% lactation (group D-C); 10% throughout gestation and lactation (group D-D). Mean body weight of group D-D at warning was 1...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-197503000-00008

    authors: Kissane JQ,Hawrylewicz EJ

    更新日期:1975-03-01 00:00:00

  • Aminophylline for renal protection in neonatal hypoxic-ischemic encephalopathy in the era of therapeutic hypothermia.

    abstract:BACKGROUND:Neonates with hypoxic-ischemic encephalopathy (HIE) frequently develop acute kidney injury (AKI). Aminophylline has been shown to reduce severe renal dysfunction in neonates after perinatal asphyxia. However, the effect of aminophylline on renal function in neonates undergoing hypothermia has not been studie...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1038/s41390-020-0999-y

    authors: Chock VY,Cho SH,Frymoyer A

    更新日期:2020-06-05 00:00:00

  • Glycogenosis due to liver and muscle phosphorylase kinase deficiency.

    abstract::A four-year-old Israeli Arab boy was found to have glycogen accumulation in both liver and muscle without clinical symptoms. Liver phosphorylase kinase (PK) activity was 20% of normal, resulting in undetectable activity of phosphorylase a. Muscle PK activity was about 25% of normal, resulting in a marked decrease of p...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-198104000-00002

    authors: Bashan N,Iancu TC,Lerner A,Fraser D,Potashnik R,Moses SW

    更新日期:1981-04-01 00:00:00

  • Evidence of exercise-induced arterial hypoxemia in prepubescent trained children.

    abstract::Exercise-induced arterial hypoxemia (EIAH) is a recognized phenomenon in highly trained adults. Like adult athletes, prepubescent trained children may develop high-level metabolic demand but with a limited lung capacity in comparison with adults. The purpose of this investigation was to search for evidence of EIAH in ...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/01.PDR.0000114481.58902.FB

    authors: Nourry C,Fabre C,Bart F,Grosbois JM,Berthoin S,Mucci P

    更新日期:2004-04-01 00:00:00

  • The immediate ventilatory response to added inspiratory elastic and resistive loads in preterm infants.

    abstract::We measured the changes in tidal volume, duration of the various phases of the respiratory cycle, and peak nasal pressure during elastic and resistive loading in preterm infants. Values were calculated during the first loaded breath, when chemical drive was unchanged. Tidal volume decreased by equivalent percentages w...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-197704000-00002

    authors: Boychuk RB,Seshia MM,Rigatto H

    更新日期:1977-04-01 00:00:00

  • Energy balance and nitrogen balance in growing low birthweight infants fed human milk or formula.

    abstract::Energy and nitrogen balances were measured in growing low birthweight infants fed either mother's expressed breast milk or a 20 kcal per ounce formula to determine whether or not there were differences between the two dietary groups in (1) the partition of energy among excretion, expenditure, and storage and (2) the r...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-198311000-00011

    authors: Whyte RK,Haslam R,Vlainic C,Shannon S,Samulski K,Campbell D,Bayley HS,Sinclair JC

    更新日期:1983-11-01 00:00:00

  • Purification of fibroblast-derived celiac disease autoantigen molecules.

    abstract::We have recently purified autoantigen polypeptides reacting with celiac disease patient sera IgA from the extracellular noncollagenous matrix compartment of fetal lung tissue. These molecules trigger the production of different tissue antibodies, the so-called antireticulin and antiendomysium antibodies in celiac dise...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-199310000-00007

    authors: Marttinen A,Mäki M

    更新日期:1993-10-01 00:00:00

  • Decreased gene expression of endothelial nitric oxide synthase in newborns with persistent pulmonary hypertension.

    abstract::Previous studies in adults have shown that chronic pulmonary hypertension is associated with decreased endothelial nitric oxide synthase (eNOS) expression in pulmonary arteries. However, the role of decreased eNOS expression in persistent pulmonary hypertension of the newborn (PPHN) is unknown. We investigated the hyp...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-199809000-00012

    authors: Villanueva ME,Zaher FM,Svinarich DM,Konduri GG

    更新日期:1998-09-01 00:00:00

  • Role of pili in Haemophilus influenzae adherence to, and internalization by, respiratory cells.

    abstract::To determine the role of pili in mediating adherence of type b and nontypeable Haemophilus influenzae to several respiratory cell types, piliated bacteria, nonpiliated phase variants, and nonpiliated mutants possessing insertionally inactivated pilin genes were tested. Both piliated and nonpiliated strains adhered to ...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-199602000-00025

    authors: Gilsdorf JR,Tucci M,Marrs CF

    更新日期:1996-02-01 00:00:00

  • Determination and characterization of ciliary ATPase in the presence of serum from cystic fibrosis patients.

    abstract::The purpose of this investigation was twofold: (1) to identify and characterize the enzymatic ATP hydrolysis system of epithelial cilia, and (2) to develop a quantitative, biochemical test for the ciliotoxic cystic fibrosis (CF) factor based on inhibition of ATP utilization by ciliary preparations. Our rationale for s...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-197602000-00012

    authors: Farrell PM,Fox GN,Spicer SS

    更新日期:1976-02-01 00:00:00

  • Prolonged hypoxemia enhances and acute hypoxemia attenuates laryngeal reflex apnea in young lambs.

    abstract::To determine the influence of an altered carotid body function on the laryngeal chemoreflex (LCR) response, reflex apnea was induced by laryngeal water stimulation during normoxia or acute hypoxia in unanesthetized awake lambs in which the ventilatory response to acute hypoxia was attenuated by prolonged postnatal hyp...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-199312000-00024

    authors: Sladek M,Grogaard JB,Parker RA,Sundell HW

    更新日期:1993-12-01 00:00:00

  • Temperature-sensitive mutants of influenza A virus: response of children to the influenza A/Hong Kong/68-ts-1(E) (H3N2) and influenza A/Udorn/72-ts-1(E) (H3N2) candidate vaccine viruses and significance of immunity to neuraminidase antigen.

    abstract::One of two slightly different influenza A/ts-1[E] recombinant candidate live vaccines was given intranasally to each of 23 young children. Twelve of 15 children who had no serum HI antibody but who did have serum ANAB at the time of administration became infected and 1 had mild rhinitis. All eight who lacked both type...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-197604000-00008

    authors: Kim HW,Arrobio JO,Brandt CD,Parrott RH,Murphy BR,Richman DD,Chanock RM

    更新日期:1976-04-01 00:00:00

  • Effect of sodium restriction and angiotensin II infusion in Bartter's syndrome.

    abstract::Five patients with Bartter's syndrome were investigated. Sodium restriction (less than 10 mEq/day for at least 5 days) showed a renal sodium wastage in only two patients (I and II) in spite of increased aldosterone secretion rate (from 151-427 to 680-842 mug/day). The effect of angiotensin II (A II) 80ng/kg/min for 30...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-197612000-00005

    authors: Sann L,David M,Richard P,Floret D,Sassard J,Bizollon CA,Francois R

    更新日期:1976-12-01 00:00:00

  • Hypophosphatasia: a developmental anomaly of alkaline phosphatase?

    abstract::This report deals with quantitative and qualitative investigations of alkaline phosphatase in two unrelated infants with the severe infantile form of hypophosphatasia. Both affected infants had no detectable leukocyte alkaline phosphatase activities and both sets of parents and one sibling tended to have low but varia...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-197607000-00004

    authors: Gorodischer R,Davidson RG,Mosovich LL,Yaffe SJ

    更新日期:1976-07-01 00:00:00

  • Molecular and genetic analyses of two patients with Pearson's marrow-pancreas syndrome.

    abstract::Pearson's syndrome, a rare and fatal disorder characterized by refractory sideroblastic anemia and pancreatic insufficiency in infancy, is classified into mitochondrial cytopathies. To understand the molecular and genetic bases of this disorder, we have investigated the mitochondrial respiratory chain enzymes and the ...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-199307000-00024

    authors: Sano T,Ban K,Ichiki T,Kobayashi M,Tanaka M,Ohno K,Ozawa T

    更新日期:1993-07-01 00:00:00