Changes in red cell phosphate metabolism of preterm and fullterm infants with perinatal problems during their first month of life.

The effects of perinatal problems on red cell phosphate metabolism were studied in two groups of infants (preterms B and fullterms D) during the first month of life. All infants started milk feeding from day three after birth. The results were compared to those of healthy preterms (A) and fullterms (C), respectively. Comparisons were also made between the preterm and fullterm groups B and D. The preterms with perinatal problems (B) showed a significant delay in catching up with the plasma and red cell inorganic phosphate (Pi) levels of controls (A) throughout the first month of life (p < 0.05). In parallel, the erythrocyte 2,3 diphosphoglycerate (2,3-DPG) concentrations of the sick preterms lagged significantly behind those of controls (p < 0.001); but the ATP levels were comparable between the two groups. The fullterms behaved slightly differently. No significant differences in plasma Pi (Pl Pi) and red cell 2,3-DPG were seen between the sick and healthy neonates during the month of study, while red cell Pi (RBC Pi) and ATP were found to be lower in the sick ones (p < 0.05). The fullterms with perinatal problems (D) had significantly higher Pl Pi (p < 0.05) and RBC Pi (p < 0.01) than preterms with problems (B) from the first week of life and continued in a similar pattern until the end of the month. Red cell 2,3-DPG concentrations were found to be significantly correlated with Pl Pi and RBC Pi in both preterm groups (p < 0.01) and in the sick fullterms (p < 0.001) during the time of the study. In the healthy fullterms 2,3-DPG was found to correlate only with red cell Pi (p < 0.05). Perinatal problems seem to affect Pi metabolism to a different degree in preterm and fullterm neonates in the first month of life.

Biochemical markers of bone metabolism in infants and children under intravenous corticosteroid therapy.

The short-term effects of corticosteroids (CS) administered intravenously (IV) on biochemical parameters of bone metabolism were followed in infants and children. Forty-nine patients from 2 months to 10 years of age, admitted to Pediatrics Department for bronchiolitis, viral-associated wheezing and croup, were treated with IV hydrocortisone or methylprednisolone (10 or 2 mg/Kg/day, respectively) for 3 days. Blood and fasting urine were collected on admission (day 1), 2 days later (day 3) and 12 days after the end of therapy (day 15). Fifty-one children of similar age and gender without respiratory problems or bone diseases were used as controls. On day 3, suppression of the bone formation markers osteocalcin (OC) (P < 0.001) and total alkaline phosphatase (ALP) (P < 0.05) was observed, but not of the bone resorption markers of hydroxyproline, pyridinoline and calcium excretion (UHyp/UCr, UPYD/UCr and UDPD/UCr, UCa/UCr). Significant decreases were indicated in serum phosphate (Pi) and the maximum renal tubular Pi reabsorption (TmP/GFR) compared to basal (P < 0.001). No significant changes were noticed in the circulating levels of calcium (Ca), parathyroid hormone (iPTH), 25OHD, 24,25(OH)2D, 1,25(OH)2D, the insulin-like growth factor-I (IGF-I) and its binding protein-3 (IGFBP-3). Two weeks after therapy, the increase of OC to higher than basal (P < 0.01) indicated a probable activation of the osteoblasts. Serum Pi and the TmP/GFR index values that had significantly decreased by day 3 returned to pretreatment levels by day 15. When assessing the effects of the CS in relation to age, no changes were detected in the levels of OC and total ALP in the <12-month-old children, but a fall of OC was observed in the >1-year-old group (P < 0.001). In contrast to the OC, the effects on serum and renal tubular reabsorption of phosphate were similar for both groups. In conclusion, short-term IV administered CS led to significant but reversible inhibition of bone formation markers, especially detectable in the >1-year-old children, without affecting the bone resorption ones. The adverse effects on phosphate metabolism were also significant, but temporal and irrespective of age.

Effects of intranasal salmon calcitonin in juvenile idiopathic arthritis: an observational study.

The aim of this study was to follow the changes in bone mineral density (BMD) and biochemical markers of bone turnover in 10 children (7.5-17.5 years of age) with severe juvenile idiopathic arthritis (JIA), during a 3-year therapy with salmon calcitonin (100 IU/day 2 months on and 2 off for a year and 200 IU/day for 2 years) and calcium (500 mg/day). All patients were functional classes III and IV and were measured at yearly intervals with a dual photon absorptiometer at the lumbar spine. The changes observed were 7.2-9.5% per year for BMD and 2.0-6.0% for volumetric bone mineral density (BMDvol). The bone resorption markers showed significant decreases after a year's treatment (Pyr/Cr from 175+/-15 to 108+/-15 nm/mm, P < 0.001, Pyr-D/Cr from 24.3+/-3.5 to 13.3+/-1.9 nm/mm, P < 0.05, and OHPr/Cr from 57.4+/-11 to 35.1+/-8.4 microg/mg) and smaller changes thereafter. No significant changes were observed in the bone formation markers of osteocalcin and alkaline phosphatase. Serum iPTH, the vitamin D metabolites, and calcium concentrations fluctuated within normal, while calcium excretion increased from 0.3+/-0.1 to 1.9+/-0.4 mg/kg/24 hours, P < 0.001. In conclusion, the present study, despite its limitations of not being placebo controlled, shows possible beneficial effects of intranasal calcitonin on bone resorption and pain relief in JIA patients.

Interferon-alpha treatment of children with chronic hepatitis D virus infection: the Greek experience.

BACKGROUND/AIMS: The therapeutic experience of interferon-alpha therapy against hepatitis D virus infection in affected children is rather limited. For this reason, we conducted a retrospective study (duration: 1991-1995) in order to evaluate the efficacy and the safety of interferon-alpha in children suffering from chronic hepatitis D in Northwestern Greece. METHODOLOGY: Seven children who were found to be infected with HDV in a total of 324 children seropositive for hepatitis B virus infection during the 5-year period of the study were treated with interferon-alpha, 3 x 10(6) U/m2 body surface area, intramuscularly or subcutaneously, 3 times weekly for 1 year (after an informed consent obtained from their parents). Patients were assessed monthly by hematological serological and biochemical tests. Clinical progress, levels of serum alanine aminotransferase, hepatitis D ribonucleic acid (HDV-RNA) and hepatitis B deoxyribonucleic acid (HBV-DNA), seroconversion of hepatitis B surface antigen (HBsAg) and Hepatitis Be Antigen (HBeAg) and liver histology were used as response criteria. RESULTS: Posttreatment alanine transferase levels were significantly reduced (P < 0.05) but Immunoglobulin M and total anti-hepatitis D virus (anti-HDV) antibodies remained positive in all, while hepatitis D ribonucleic acid persisted positive in 4 cases. In addition, no seroconversion of HBsAg or HBeAg was noted and the liver histology progress was disappointing. Side effects including mild fever, arthralgias and malaise and reversible neutropenia and thrombocytopenia were common, but not particularly disturbing. Nevertheless, the children remained fully active on treatment, felt well and attended school. Initially 4 children had been below the 10th percentile for weight and height. All thrived during treatment and two crossed above the 10th percentile indicating height velocity and body mass index increase. CONCLUSIONS: The administration of regular interferon-alpha doses for treating children with chronic hepatitis D was safe as attested by the mild side effects and the objective clinical criteria regarding their growth, but relatively ineffective. Although the prevalence of hepatitis D virus infection is now generally decreased, this study indirectly indicates that more effective agents and new approaches at the molecular level of the hepatitis D virus genome are urgently warranted for its control in individuals already infected with the virus. Finally, the poor therapeutic results in the present study further enhance the necessity of the expanded vaccination against Hepatitis B virus according to the World Health Organization's recommendations.

Vitamin D metabolites (25-hydroxyvitamin D, 24,25-dihydroxyvitamin D and 1,25-dihydroxyvitamin D) and osteocalcin in beta-thalassaemia.

Serum levels of the vitamin D metabolites 25-hydroxyvitamin D, 24,25-dihydroxyvitamin D, and 1,25-dihydroxyvitamin D, and of osteocalcin, C-terminal parathyroid hormone and other biochemical indices related to bone metabolism, were determined in two groups of patients with beta-thalassaemia aged 5-10 years (summer 7.8 +/- 0.4 years, mean +/- SEM, and winter 7.7 +/- 0.4 years, group A, n = 15) and 11-23 years (16.6 +/- 0.9 and 15.7 +/- 0.9 years in summer and winter, respectively, group B, n = 22). Emphasis was given to populations of school and adolescent ages and to the seasons of summer and winter when vitamin D status demonstrates the widest extremes. The mean serum levels of 25-hydroxyvitamin D in patients aged 5-10 years did not differ from those of controls during both seasons studied. In contrast, in the age group 11-23 years these levels were found to be lower in patients than in controls both in winter (10.6 +/- 0.9 ng/ml vs 15.0 +/- 2.0 ng/ml, p < 0.05) and summer (20.2 +/- 2.1 ng/ml vs 27.1 +/- 2.0 ng/ml, p < 0.05). The serum concentrations of 24,25-dihydroxyvitamin D were lower in the thalassaemic patients than in controls in both age groups and both seasons. In the patients under 10 years of age the mean values of this metabolite in winter were 1.06 +/- 0.17 ng/ml vs 1.68 +/- 0.20 ng/ml in the respective controls (p < 0.05), and in summer 1.44 +/- 0.11 ng/ml vs 2.35 +/- 0.36 ng/ml in controls (p < 0.05). In the group of patients aged 11-23 years, the mean levels of 24,25-dihydroxyvitamin D were in winter 0.65 +/- 0.12 ng/ml vs 1.12 +/- 0.19 ng/ml (p < 0.05) in controls and in summer 1.34 +/- 0.12 ng/ml vs 1.84 +/- 0.20 ng/ml (p < 0.05). The 1,25-dihydroxyvitamin D concentrations in both thalassaemic patient groups were significantly no different from those in the respective control groups. Serum osteocalcin, C-terminal parathyroid hormone, calcium, inorganic phosphate and alkaline phosphatase levels in the patients studied were not significantly different from those in controls, except for calcium and phosphate in the older group. In the older group of thalassaemic patients, serum calcium was lower than in the controls (2.26 +/- 0.03 vs 2.37 +/- 0.03 mmol/l, p < 0.05) in summer and serum phosphate higher than in the controls in winter (1.47 +/- 0.05 mmol/l vs 1.27 +/- 0.06 mmol/l, p < 0.05).

Childhood brucellosis in north-western Greece: a retrospective analysis.

UNLABELLED: Fifty-two cases of childhood brucellosis which occurred in north-western Greece during the 15-year period 1979-1993, are reviewed. It is believed that they represent very closely the total incidence of the disease in the region which has a population of 100,000 children aged 0-14 years old. Brucellosis-affected children were almost exclusively from goat- or shepherd families and of both sexes and all age groups. A broad spectrum of clinical manifestations ranging from malaise only to brain abscess was observed. Fever and arthralgia were the most common manifestations followed by malaise, myalgia, sweating, rash, cough, and gastro-intestinal, cardiac and CNS involvement. Splenomegaly was found more often than hepatomegaly and lymphadenopathy. Laboratory findings included anaemia, leukopenia, neutropenia, lymphocytopenia, monocytosis, eosinophilia, thrombocytopenia and pancytopenia. Leukocytosis and lymphocytosis were extremely rare and ESR and serum C-reactive protein levels were mildly elevated. All patients had positive Rose Bengal slide agglutination tests and standard tube agglutination titres of 1:160 or more. When performed, blood culture was often diagnostic. The children were treated with streptomycin for 2 weeks plus either tetracyclines or trimethoprim-sulphamethoxazole for 3 weeks. Treatment was well tolerated. Relapse was observed in one case. CONCLUSION: Brucellosis nowadays affects children in an occupational pattern. As symptoms, signs and first-line laboratory findings are not characteristic, agglutination tests and blood culture should be performed in any child with prolonged fever. Treatment is effective, but prevention of the disease by animal testing and education of high risk families is indicated.

Giant hemangioma in infant. A case presentation and literature review.

Giant hemangioma of infancy is a rare and extensive variant of the hemangiomas, that occurs in newborns and infants and is often complicated by life-threatening events. We describe herein an unusual case of a giant hemangioma in a female infant. At the age of 45 days she was admitted to the hospital because of increasing respiratory distress and distented abdomen. The response to supportive therapy was poor and the infant died from cardiac arrest on its 75th day of life. Autopsy revealed a giant tumor occupying extensive areas of the thoracic and abdominal cavity. The histologic diagnosis was compatible with a giant capillary hemangioma. A review of the literature was also attempted.

Clinical significance of creatine kinase isoenzymes for fetal asphyxia in women at labor.

Creatine kinase-brain isoenzyme activity (CK-BB) was measured longitudinally in the serum of 31 pregnant women in the first stage of labor (early and advanced), at delivery, and 1, 6 and 24 h after delivery, in the umbilical cord and in the serum of their neonates on the first day of life. There was no increase in serum CK-BB values of mothers that delivered normally (n = 15) or had an elective cesarean section (n = 5). Pregnant women with signs of fetal distress had an increase in CK-BB levels in the first stage of labor (mean +/- SD 4.5 +/- 4.9 U/l, p < 0.03) and 6 h after delivery (12 +/- 4 U/l, p < 0.0001). Neonates with intrauterine stress also had an increase in their CK-BB to 144 +/- 116 U/l at 6 h of life, in comparison with babies born without signs of stress. It appears that CK-BB during labor and in the first hours of life may be indicative of intrauterine stress.

Changes of mineral metabolism in juvenile chronic arthritis.

Thirty five children with ambulant JCA were studied to assess the biochemical parameters of bone metabolism. The mean age of the study group was 8.8 +/- 4.1 years and the mean duration of active disease 3.8 +/- 1.3 years. According to the onset of the disease the children belonged to the systemic (7), polyarticular (12) and pauciarticular type (16). All the patients were treated with NSAIDs. In addition the polyarticular group received either gold injections or D-penicillamine and the systemic group, steroids for at least 3 months. Two groups of controls were studied. The first one included fifteen children without chronic arthritis or bone disease and the second, four children who were treated with corticosteroids for a variety of reasons. In the group with systemic JCA Se Pi (1.28 +/- 0.29 mmol/l) and renal phosphate reabsorption (TmP/GFR = 1.07 +/- 0.18) were significantly lower than in the control groups (1.50 +/- 0.19; 1.54 +/- 0.25 mmol/l, p < 0.01 and 1.35 +/- 0.18; 1.29 +/- 0.23 mmol/l GF, p < 0.05). Also lower were serum alkaline phosphatase (58 +/- 16.4 versus 83 /- 24.2 and 80 +/- 15.6 IU/l, p < 0.05), osteocalcin (5.5 +/- 4.7 versus 11.0 +/- 4.5 and 10.0 +/- 5.7 ng/ml, p < 0.05), 25OHD (15.6 +/- 4.9 versus 27.3 +/- 6.2 and 20.6 +/- 9.8 ng/ml, p < 0.001) and 1,25(OH)2D (12.1 +/- 6.0 versus 20.9 +/- 11.0 and 27.6 +/- 3.2 pg/ml, p < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Cortisol secretion in stressed babies during the neonatal period.

The present study investigates the developmental pattern of serum cortisol secretion in sick fullterm and preterm neonates in comparison to that of normal babies over the first 30 days of life. Four groups of babies (15 in each group) were studied sequentially during the first 4 weeks of life. Serial venous blood samples were drawn at 08.00, 14.00, 20.00 and 02.00 h from each baby. The higher cortisol levels (p < 0.001) observed in sick preterm and full-term neonates, when compared to their respective controls, suggest an appropriate response to stress. Fullterm and preterm babies with no problem were found to have a free running rhythm in serum cortisol levels during the first 4 weeks of life.

Red cell phosphate metabolism in preterm infants with idiopathic respiratory distress syndrome.

Phosphate metabolism was studied in twenty-one preterm infants with idiopathic respiratory distress syndrome during and after oxygen (O2) therapy using a hood. Plasma, red cell inorganic phosphate (Pi) and the red cell concentrations of organic phosphate metabolites ATP and 2,3-diphosphoglycerate were significantly lower in the sick infants when compared to controls of similar age and birthweight, and remained low even 24 h after cessation of therapy. Plasma cortisol levels were elevated at the onset of the disease and decreased to almost control levels by the end of O2 therapy while the values of plasma calcitonin did not show any difference from controls. Plasma creatinine phosphokinase and blood lactic acid levels followed the pattern of the control group with a small increase at the beginning of the study and decreasing thereafter. Several factors may be implicated in the cause of hypophosphatemia in these infants such as inadequate feeding, acidosis and hypercortisolaemia due to stress leading to phosphaturia.

Cellular phosphate in renal tubular acidosis.

In two infants with distal renal tubular acidosis phosphate depletion was observed in the extracellular and intracellular compartments of the erythrocytes. Treatment corrected this disturbance over a period of several months. Cell phosphate deficiency may contribute to the adverse effects of renal tubular acidosis on bone.

The management of siblings with familial hypophosphatemic rickets.

Two siblings (boy and girl) born to a mother with familial hypophosphatemic rickets had abnormal values of serum phosphorus and serum alkaline phosphatase at the age of six weeks. At this age therapy with 1 alpha-hydroxycholecalciferol (1 alpha OHD3) and phosphate was started resulting in both siblings having normal growth of body length and radiological healing of the bone lesions but persistently low values of fasting serum phosphorus during the time of observation up to 60 and 26 months of age, respectively. Phosphate and 1 alpha OHD3 have a positive influence on serum phosphorus through their effect on the intestine. Neither of the patients developed hypercalcaemia during treatment. It seems, therefore, that the early administration of 1 alpha OHD3 with phosphate in infants with familial hypophosphatemic rickets prevents dwarfism and has a positive effect on intestinal absorption of phosphorus but not on fasting hypophosphatemia.