Bernard J. Reilly

Rickets due to calcium deficiency

The importance of an adequate intake of calcium for normal skeletal growth and mineralization is well known. However, the dietary requirement for calcium is hard to define,1 2 3 4 5 6 and whether a...

Craniosynostosis in the rachitic spectrum

Premature closure of the cranial sutures can occur as an inherent mesenchymal defect.In addition, however, it can be secondary to metabolic bone disease. It is known to occur in hypophosphatasia and has been occasionally reported in vitamin D—refractory rickets. In order to ascertain the true incidence of craniosynostosis in all forms of rickets, a study was carried out on 59 children under 9 years of age, who were then or had previously been actively rachitic. Approximately one third of the children showed craniosynostosis and of these, 3 required craniectomies for decompression. The radiologic and biochemical findings have been examined in an attempt to explain why this metabolic type of craniosynostosis should occur so frequently in rachitic children. The only feature common to all cases was the presence at some time of inadequately mineralized osteoid.

Radionuclide skeletal survey for pediatric neoplasms

A prospective study comparing the radionuclide skeletal survey with the radiographic survey was performed for 159 children with known primary neoplasm. Forty-four showed radionuclide evidence of metastases, while only 14 had radiographic evidence. Thus, 68% of metastases were discovered by bone imaging alone. No false negative images for metastases were obtained, though there was one false negative image for primary tumor. Radionuclide skeletal survey is recommended as the primary evaluation for bony metastases in all children with neoplasms. Suspect areas should then receive complete radiographic examination.

Prognostic indicators of joint destruction in systemic-onset juvenile rheumatoid arthritis

We retrospectively reviewed the charts and radiographs of 38 patients with systemic-onset juvenile rheumatoid arthritis, attempting to identify early in the disease course the clinical and laboratory observations most predictive of the later development of destructive arthritis. In 12 of the patients, destructive arthritis developed within 2 years of disease onset. When first examined, these patients could not readily be differentiated from those in whom joint destruction did not develop, but they more commonly had hepatosplenomegaly (p less than 0.04), serositis (p less than 0.01), and a lower mean serum albumin concentration (26.7 vs 31.3 gm/L; p less than 0.02). However, by 6 months after onset, patients with destructive arthritis more frequently had persistent systemic symptoms (92% vs 12%; p less than 0.0001), polyarthritis (67% vs 19%; p less than 0.0005), a lower mean hemoglobin level (95 vs 114 gm/L; p less than 0.001), a higher mean leukocyte count (21.2 vs 10 x 10(9)/L; p less than 0.0003), a higher mean platelet count (794 vs 400 x 10(9)/L; p less than 0.0001), and a higher mean erythrocyte sedimentation rate (43 vs 24 mm/hr; p less than 0.05). Multivariate analysis of the results at 6 months revealed that persistent systemic symptoms and a platelet count greater than or equal to 600 x 10(9)/L were the variables most highly predictive of the later development of joint destruction. We conclude that patients at high risk for the development of destructive arthritis may be identified within 6 months of disease onset, thereby indicating the need for more aggressive early therapy.

Iatrogenic rickets in low-birth-weight infants

This report describes 4 premature infants, all with very low birth weights, whodeveloped nutritional rickets in a neonatal unit of a pediatric hospital. All infants were fed a proprietary milk formula containing vitamin D but no vitamin supplement. Because of their small size, the amount of formula ingested was small, resulting in a low daily vitamin D intake. A comparable group of infants who received a daily vitamin D supplement of 400 I.U. in addition to the enriched formula did not develop rickets. It is concluded that the rickets developed because the vitamin D intake was inadequate.

Pathogenesis of rickets in chronic hepatobiliary disease in children

To investigate whether hepatobiliary rickets is caused by defective intestinal absorption of vitamin D or by impaired hepatic hydroxylation of the vitamin, we studied three children who developed severe rickets, hypocalcemia, and hypophosphatemia, two despite having received 400 to 800 IU vitamin D per day by mouth, and one despite prolonged treatment with 10,000 IU daily. On oral vitamin D therapy, plasma vitamin D and 25-hydroxyvitamin D levels were low. When two children were treated with weekly intravenous doses of 3,000 IU vitamin D to approximate the recommended prophylactic allowance, their plasma calcium and phosphate values improved promptly, the radiographic lesions healed, and the plasma concentrations of vitamin D and 25-hydroxyvitamin D became normal. Our studies indicate that the primary cause of hepatobiliary rickets is intestinal malabsorption of vitamin D, not impairment of the hepatic metabolism of the vitamin.

The Correlation of Radiological Changes with Pulmonary Function in Cystic Fibrosis

Abstract To determine airway resistance and degree of obstruction in cystic fibrosis, the authors measured vital capacity, maximum midexpiratory flow rate, diffusing capacity, and hypoxemia. Dead space to tidal volume ratios were used as an index of imbalance between ventilation and perfusion. Chest films were assessed independently for air trapping, bronchial wall thickening, cyst formation, retained secretions within the cysts, and overall extent of disease. One hundred and ten scores were obtained in 69 patients, and an excel ent correlation was found between abnormality in pulmonary function studies and roentgeno ogically detected pulmonary disease.

Computed Tomography of Abdominal Mass Lesions in Children: Initial Experience

Use of a Delta scanner has resulted in delineation of neoplastic, obstructive and inflammatory renal diseases, hepatic tumors, and other abdominopelvic mass lesions. Of 47 patients examined, 32 had proved mass lesions. Computed tomography provided unique information about the complete delineation and nature of lesions and detected unrecognized lesions. Technical problems and solutions are discussed.

Renal osteodystrophy in children undergoing continuous ambulatory peritoneal dialysis.

Fifteen children undergoing continuous ambulatory peritoneal dialysis for 0.3 to 2.4 years were evaluated longitudinally for renal osteodystrophy. Immunoreactive parathyroid hormone, 25-OHD, total and ionized calcium, inorganic phosphate, and alkaline phosphatase levels were measured regularly. Skeletal radiographic studies were performed at the onset and conclusion of CAPD and at six-month intervals during therapy. All children received 1,25(OH)2D3 and aluminum hydroxide, and nine received supplemental calcium. Plasma 25-OHD concentrations were normal to elevated, and calcium increased steadily to high normal levels despite a trend to persistent hyperphosphatemia. The increased calcium levels suppressed parathyroid hormone overactivity in only one patient. At the onset of CAPD, nine patients had hyperparathyroid bone disease seen radiographically, three of whom also had rachitic lesions. At the end of CAPD, the hyperparathyroid lesions had improved in four patients, completely resolved in three, and deteriorated in two. Rachitic lesions had completely healed in two patients and improved in the third. However, among the six children without radiographically evident lesions at onset of CAPD, hyperparathyroid bone lesions developed in two and rachitic lesions in two others during CAPD. Although CAPD and appropriate therapy benefited most patients with renal osteodystrophy, the benefits were not uniform, and bone lesions deteriorated in some.

CARDIOMEGALY IN ASSOCIATION WITH NEONATAL HYPOGLYCAEMIA

Fifty‐two infants with hypoglycaemia were seen over a 12 month period. Six of these infants had cardiac enlargement with signs of failure and pulmonary oedema in association with isolated hypoglycaemia. Signs and symptoms suggestive of cardiac failure corresponded to the period of hypoglycaemia and disappeared when blood sugar levels were raised by appropriate treatment.