Effects of a multidisciplinary weight loss intervention on body composition in obese adolescents.

OBJECTIVE: To investigate if a multidisciplinary weight loss program in adolescents suffering severe obesity allows adequate growth and development and avoid lean mass loss. DESIGN: A total of 55 adolescents (33 girls and 22 boys) suffering severe obesity were enrolled in an interdisciplinary weight reduction program lasting 6-12 months. Progressive submaximal physical activity was performed and national dietary allowances for adolescents with low levels of physical activity energy were provided. MEASUREMENTS: Total and segmental body composition was assessed by means of dual-energy X-ray absorptiometry. RESULTS: The mean height significantly increased (P<0.001). The mean body mass index (BMI) dropped in boys from 34.5+/-3.2 to 25.5+/-2.3 kg/m(2) and in girls from 38.4+/-4.1 to 28.4+/-4.1 kg/m(2). Height increased according to the expected pattern (P<0.001). Total lean mass (LM) did not vary and was positively correlated to pubertal development in both sexes before and after weight loss. Steepest drop in fat mass (FM) was observed in the trunk (-63.2+/-10.1% in boys and -51.5+/-11.4% in girls). Decrease in BMI and FM was tightly correlated in both sexes. However, slopes significantly differed (P<0.0005) so that a decrease of 1 kg/m(2) in BMI corresponded to a decrease of 3.92 kg in FM in girls and of 5.44 kg in boys. In each sex, FM at baseline and duration of the treatment were the main determinants of the decrease in FM. CONCLUSION: During adolescence, despite a major weight loss, adequate growth and preservation of LM can be achieved. Weight loss kinetics markedly differs between boys and girls. Low-calorie diets are unnecessary to achieve a marked reduction of severe obesity during puberty.

[Normal phosphorus and calcium metabolism during pregnancy and breast feeding]. / Métabolisme phosphocalcique normal pendant la grossesse et l'allaitement.

Calcium homeostasis is stressed considerably during pregnancy and lactation. Important regulatory mechanisms are needed both for meeting the fetal requirement for calcium and for protecting the maternal skeleton from excessive resorption. For the past 10 years, more and more publications have deal with the involved mechanisms of regulation. The authors have reviewed these publications and tried to answer important questions: is there some irreversible bone loss related to pregnancy and/or lactation? How can such bone loss could be prevented?

[Pathological phosphorus and calcium metabolism during pregnancy and breast feeding]. / Métabolisme phosphocalcique pathologique pendant la grossesse et l'allaitement.

The occurrence of calcium and vitamin D deficiencies is enhanced during pregnancy and lactation. The presentation of parathyroid diseases is altered during gestation. A peculiar form of osteoporosis, pregnancy-associated osteoporosis, may be observed. Fetus and newborns suffer the consequences of all these disorders. The authors have reviewed the early detection and treatment of these diseases and emphasized their prevention.

Tibioperoneal diaphyseal toxopachyosteosis or Weismann-Netter-Stuhl syndrome: difficulties encountered in classifying this syndrome and differentiation from rickets.

Using two new cases and 70 case reports in the literature as a starting point, the authors focus on the Weismann-Netter-Stuhl syndrome. Weismann-Netter and Stuhl reported the first cases of tibioperoneal diaphyseal toxopachyosteosis in 1954. This syndrome is defined as an anomaly of the diaphyseal part of both tibiae and fibulae with posterior cortical thickening and anterior-posterior bowing. This anomaly is usually bilateral and symmetrical, and patients are therefore short in stature. The thickening of the fibula is true "tibialisation" and "is the main feature and the only feature confirming diagnosis". Routine laboratory investigations showed no abnormalities in the two new cases. The authors specify the limits encountered in classifying this anomaly and discuss the degree to which this anomaly is an entity unto itself when compared with rickets sequelae.

Identification and classification of tibioperoneal diaphyseal toxopachyosteosis (Weismann-Netter-Stuhl syndrome): based on two new cases and a review of the literature.

Using two new cases and 70 case reports in the literature as a starting point, the authors focus on the Weismann-Netter-Stuhl syndrome. Weismann-Netter and Stuhl reported the first cases of tibioperoneal diaphyseal toxopachyosteosis in 1954. This syndrome is defined as an anomaly of the diaphyseal part of both tibiae and fibulae with posterior cortical thickening and anterior-posterior bowing. This anomaly is usually bilateral and symmetrical and patients are short. The thickening of the fibula is true tibialisation and is the main feature and the only feature confirming diagnosis. Routine laboratory investigations showed no abnormalities. The authors specify the limits encountered in classifying this anomaly and discuss the degree to which this anomaly is an entity unto itself when compared with rickets sequelae.

Successful cord blood stem cell transplantation for congenital erythropoietic porphyria (Gunther's disease).

Congenital erythropoietic porphyria (Gunther's disease, GD) is a rare autosomal recessive disease. It results from the deficiency of uroporphyrinogen III synthase, the fourth enzyme on the metabolic pathway of heme synthesis. GD leads to severe scarring of the face and hands as a result of photosensitivity and fragility of the skin due to uroporphyrin I and coproporphyrin I accumulation. It also causes erythrocyte fragility leading to haemolytic anaemia. The other clinical features include hirsutism, red discolouration of teeth, finger-nails and urine and stunted growth. The outcome is poor, and the disfiguring nature of GD may partly explain the legend of the werewolf. No curative treatment was known until 1991, when the first case of BMT in GD was reported. The clinical and biological outcome after transplantation was encouraging, with an important regression of the symptoms of the disease, but the child died of CMV-infection 11 months after BMT. We report the second case of GD treated successfully by stem cell transplantation using umbilical cord blood from an HLA-identical brother in a 4-year-old girl suffering from severe GD. Our patient is very well 10 months after transplantation. We confirm that stem cell transplantation is curative for GD.

Age-related effect of a single oral dose of calcium on parathyroid function: relationship with vitamin D status.

The parathyroid response to the administration of a single oral dose of 0.5 g Cal was studied in 71 normal subjects of both sexes (34 males, 37 females) aged 20-88 y. Serum 25-hydroxyvitamin D [25(OH)D] and 1,25-dihydroxyvitamin D [1,25(OH)2D] were measured before the intake of calcium. Serum ionized calcium (Ca++) and intact parathormone (PTH1-84) were measured before and 1, 2, and 3 h after the intake of calcium. The Ca++ maximal response (delta Ca++max), which significantly decreased with age (P = 0.0001), was correlated with both 25(OH)D (r = 0.375, P < 0.01) and 1,25(OH)2D (r = 0.284, P < 0.02). The maximal PTH1-84 suppressive response expressed as a function of basal values (delta PTH%) decreased with age. Basal PTH1-84 values were significantly increased with age (P = 0.0002) and were negatively correlated with 25(OH)D concentrations (r = 0.414, P < 0.002) which were low in elderly people. These results suggest that correction of vitamin D deficiency should improve delta Ca++max, lower basal PTH1-84, and consequently make the oral intake of calcium more efficient to decrease PTH1-84 concentrations.

[Neurologic complications of osteoporotic spinal collapses. Apropos of 8 cases]. / Complications neurologiques des tassements vertébraux ostéoporotiques. A propos de huit observations.

The prevalence of neurological complications in patients with osteoporotic vertebral collapse and the risk factors for neurological compromise were studied retrospectively in 138 inpatients. Six per cent of patients had objective neurological loss. Patients with neurological loss were more likely to have changes suggestive of malignant disease on roentgenograms, computed tomography, and magnetic resonance imaging. Diagnosis was based on magnetic resonance imaging and examination of vertebral biopsy specimens. Comparison of patients with and without neurological loss suggested that an intravertebral vacuum phenomenon and/or a cortical osteoporosis may cause fracture of the posterior wall and therefore compression of neurological structures.

[Exploration of the parathyroid gland function by intake or oral calcium and phosphate (only oral intake and sequential treatment with calcium-phosphate)]. / Exploration de la fonction parathyroïdienne par prise orale de calcium et de phosphate (prise orale unique et traitement séquentiel calcium-phosphate).

The parathyroid hormone response to the oral intake of either calcium or phosphate was explored in 10 young adults (23-28 years). First, the subjects were investigated during free running diet. They ingested a single oral dose of 500 mg of calcium (as a bag of Sandocal) and 10 days later a single oral dose of phosphate (750 mg of phosphorus as a tablet of Phosphore Sandoz Forte). Samples of blood and urine were collected before and during the 4 hours following the ingestion of either calcium or phosphate. After intake of calcium an acute response was obtained with a 58% decrease in PTH 1-84 at 1 hr (p less than 0.001) and a 33% decrease in nephrogenous cAMP (p less than 0.001). After ingestion of phosphate the response was delayed and less constant with a 25% increase in PTH 1-84 at 3 hr (p less than 0.01) and a 27% increase in nephrogenous cAMP (p less than 0.001). Then, the effects of a calcium therapy (3 daily doses of 500 mg each for 20 days) and of a subsequent phosphate therapy (2 daily doses of 750 mg each for 10 days) on the parathyroid hormone response to the administration of a single dose of phosphate were studied. On days 10, 21 and 31 baseline blood and urine samples were obtained prior to calcium and phosphate administration for measuring PTH 1-84 and nephrogenous cAMP. No significant variation was found. On days 21 (after calcium therapy) and 31 (after phosphate therapy) an oral load of phosphate was administered according to the procedure described above.(ABSTRACT TRUNCATED AT 250 WORDS)

[Elevation of atrial natriuretic factor in alveolo-interstitial lesions of connective tissue diseases]. / Elévation du facteur atrial natriurétique au cours des atteintes alvéolo-interstitielles des connectivites.

Plasma levels of the atrial natriuretic factor were prospectively measured in 15 patients suffering from connective tissue disease with alveolar-interstitial lung lesions. The mean level was significantly higher in these patients than in 12 patients without known lung lesions (74.8 +/- 20.7 pg/ml versus 30.8 +/- 12 pg/ml; P less than 0.01) and in 18 healthy subjects (25.4 +/- 12.6 pg/ml). There was no correlation between these levels and lung function tests. Three patients with isolated lymphocytosis in the alveolar lavage fluid had a high level of atrial natriuretic factor.

[Metacarpal bone chondrosarcoma. Apropos of a case]. / Chondrosarcome métacarpien. A propos d'un cas.

On the basis of a personal case of non-symptomatic chondrosarcoma of the metacarpal bone and a review of the literature, the authors advise the biopsy of any lesion which resembles a chondroma of the hand. Only histological examination can provide evidence for diagnosis and guide effective treatment.

[Primary hyperparathyroidism: value of "intact parathormone" assay (PTH 1-84)]. / Hyperparathyroïdie primaire: apports du dosage de la parathormone "intacte" (PTH 1-84).

In a prospective study of 20 patients with primary hyperparathyroidism the diagnostic value of the intact parathormone assay was compared with that of intermediate fragment measurement and that of nephrogenic cyclic AMP determination. In the basal state, measuring the intact parathormone is the best way of separating hyperparathyroid patients form normal subjects. During calcium infusion tests, there is good concordance between changes in intact parathormone and in nephrogenic cyclic AMP. In addition, the intact parathormone assay enables a thorough study of the relationship between ionized calcaemia and parathyroid secretion to be performed and confirms the existence, in hyperparathyroid patients, of two types of secretory response to a rise in calcaemia. Being more reliable than the measurement of a C-terminal or intermediate fragment and easier to perform than nephrogenic cyclic AMP determination, the intact parathormone assay seems to be particularly suitable for the study of primary hyperparathyroidism.