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Biomedical subjects

W A Cumming

Publications and source records attributed to W A Cumming.

At least 37 records · Page 2Linked to original sources

Bronchopulmonary dysplasia.

Neonatal pulmonary diseases may require mechanical ventilation and supplemental oxygen therapy. These supportive measures can damage the immature lung or distort the normal maturation processes of the developing lung. The formation of hyaline membranes occurs acutely, often complicated by left-to-right cardiac shunts. Pulmonary edema occurs next, followed by interstitial fibrosis and finally by parenchymal emphysema. This process is termed bronchopulmonary dysplasia. Significant morbidity and mortality are associated with this developmental pulmonary dysplasia, particularly during the first two years of life. A delay in normal growth and development is often present, acute episodes of pulmonary insufficiency are seen, and multiple episodes of pulmonary infection occur. Subsequently, the radiographic appearance and the clinical symptoms improve. Therapy is mainly supportive throughout the course of the disease and the bronchopulmonary dysplasia tends to resolve slowly. Residual pulmonary dysfunction is usually mild or absent.

Bronchopulmonary Dysplasia↗

Carbonic anhydrase II deficiency syndrome: recessive osteopetrosis with renal tubular acidosis and cerebral calcification.

Four new Saudi Arabian cases of the carbonic anhydrase II deficiency syndrome from two families are described. This autosomal recessive syndrome includes osteopetrosis with renal tubular acidosis and cerebral calcification. Additional features are mental retardation, growth failure, typical facial appearance, and abnormal teeth. Two patients showed evidence of restrictive lung disease, a finding not previously described. One of the patients reported represents the first neonate reported to be affected with this syndrome. Intrauterine growth was normal, but metabolic acidosis was already evident in the neonatal period. Radiographic evidence of osteopetrosis was probably absent at birth but appeared during the late neonatal period. Carbonic anhydrase II deficiency was demonstrated in erythrocyte hemolysates from the older two siblings of this neonate, and a 50% normal level of carbonic anhydrase II was demonstrated in the erythrocyte hemolysate from their father.

Acidosis, Renal Tubular↗

Neonatal aspiration syndrome due to vernix caseosa.

Fetal aspiration of meconium in amniotic fluid is a well-known cause of respiratory distress in newborn infants. It causes an irregular, coarse, nodular pattern on chest radiographs. Less known is that aspiration of vernix caseosa causes a similar syndrome. We present a post-mature infant in whom aspiration of vernix caseosa caused respiratory distress, ventilatory difficulty, and radiographic changes essentially the same as in aspiration of meconium.

Humans↗

Intracranial calcification in children with osteopetrosis caused by carbonic anhydrase II deficiency.

Radiographs of 18 children thought to have carbonic anhydrase II deficiency were reviewed to assess intracranial calcification. These children have an autosomal recessive syndrome consisting of osteopetrosis, renal tubular acidosis, intracranial calcification, and developmental retardation. This calcification is in the gray matter of cortex and basal ganglia and is similar, if not identical, to that seen in hypoparathyroidism and pseudohypoparathyroidism. It is not present at birth but appears at approximately 2-5 years of age and increases in density and extent through childhood.

Acidosis, Renal Tubular↗

Plastic bronchitis: large, branching, mucoid bronchial casts in children.

Expectoration of large, branching, bronchial casts, termed plastic bronchitis, is an uncommon condition in children. Asthma or allergy often is the cause of cast production, but in some instances no etiology is found. Five children produced large, obstructing bronchial casts that either were expectorated or were extracted at bronchoscopy. Four of the children had asthma or allergies and the fifth had congenital tricuspid atresia and chronic pericardial and pleural effusions. In two patients, expectorated casts initially were thought to be aspirated food material. Radiographic findings during periods of cast formation included atelectasis, obstructive emphysema, bronchiectasis, pleural effusion, and pneumomediastinum. Cast formation may be self-limited but generally ceases with appropriate treatment of the causative disorder. Bronchoscopic extraction of casts may benefit some patients.

Asthma↗

Large unilateral chest tumors in children.

When large unilateral chest tumors are hidden by pleural fluid collections, or when there is a completely radiopaque hemithorax, ultrasonography is an efficient means of identifying and characterizing a mass. Narrowing of the carinal angle in patients with mediastinal shift is helpful in identifying large intrathoracic tumors that could be mistaken for, or hidden by, large pleural effusions. Of 11 large tumors in children, seven were Ewing's sarcomas, two were metastatic sarcomas, one was a lymphoma, and one was an undifferentiated small cell tumor of unknown origin. The rib lesions of Ewing's sarcoma were seen clearly on chest radiographs in all patients with that disease.

Adolescent↗

Nephrocalcinosis in Bartter's syndrome. Demonstration by ultrasonography.

Nephrocalcinosis in Bartter's syndrome has been recognized since 1962, but has not previously been shown in an infant with the syndrome. Ultrasonography shows striking echogenicity of the renal pyramids which appears to be a specific finding in medullary nephrocalcinosis. We suspect nephrocalcinosis will be found in many infants with Bartter's syndrome if ultrasonography is used to examine their kidneys.

Bartter Syndrome↗

Renal osteodystrophy in children with chronic renal failure: an unexpectedly common and incapactating complication.

The incidence, age at onset, and progression of the biochemical, radiographic, and histologic characteristics of renal osteodystrophy were studied in 50 children in whom chronic renal failure had been recently diagnosed. During a ten-year observation period, 19 patients progressed to end-stage renal failure and radiographic signs of renal osteodystrophy developed in 15 of these (79%). Renal osteodystrophy developed in all nine patients whose chronic renal failure was diagnosed before 3 years of age and in six of the ten children with later onset of failure. The mean interval from diagnosis of renal failure to development of osteodystrophy was 1.4 years. Radiographically, growth zone lesions predominated in the younger children, whereas cortical erosions were more prevalent in the older children. Histologic examination, performed in 38 patients, showed both defective mineralization and excessive resorption and was a more sensitive diagnostic index than radiography. Noticeable deformities developed in one third of the patients with osteodystrophy, despite medical treatment including vitamin D2 therapy. Deformities were particularly frequent and severe in patients whose renal failure developed in infancy. In all 13 patients whose growth patterns were studied before and after osteodystrophy developed, the onset of bone lesions was associated with a deterioration of growth, indicating that osteodystrophy plays a major role in causing the growth retardation commonly observed in children with chronic renal failure.

Adolescent↗

The case of the missing vault: a "cold" bone lesion following electrical burn.

Osteonecrosis of the skull produced by an electrical burn presented as a dramatic "cold" bone lesion on an MDP bone scan, despite normal skull radiographs. Four months later the skull radiograph showed marked bone resorption, and the three-phase bone scan confirmed healing and new osteoblastic activity. The pathophysiology of high-voltage injuries is outlined.

Burns, Electric↗

Neonatal skeletal fractures. Birth trauma or child abuse?

When a fracture is discovered in a newborn infant, it is important to decide whether it occurred at birth or after birth. Calcification around the fracture site gives a useful estimate of the age of the fracture. We reviewed films of 23 patients with fractures resulting from delivery. The fractures occurred at three different sites: the clavicle, the humerus, and the femur. Calcification could be seen as early as seven days after birth and was absent for as long as 11 days after birth. Six of seven femoral fractures occurred in infants with neuromuscular problems. Fracture at an unusual site or absence of calcification after 11 days should alert the radiologist to the possibility of abuse.

Birth Injuries↗

Increased bone mineral content in young adults with familial hypophosphatemic vitamin D refractory rickets.

Seven adults with familial hypophosphatemia have been investigated by histologic and radiographic examination of bone, and estimates of bone mineral status by in vivo neutron activation analysis (IVNAA). Histological examination showed severe osteomalacia and osteosclerosis in all cases. Radiography showed skeletal deformities and other sequelae of severe rickets of childhood in five of the seven cases, with, in addition, thickened well-mineralized bones; the other two showed normal radiographs. IVNAA measurements showed that the first five had greater than normal bone calcium and that the other two had normal values. Thus, in all cases there is a greater than normal total bone tissue (osteoid and mineralized bone together). The quantitative body calcium measurements show clearly that osteosclerosis occurs in familial hypophosphatemia, confirming the opinions based on histological and radiological data. Although there has been occasional reference to this sclerosis in the literature, up to the present it has received little attention.

Adolescent↗

Lower esophageal sphincter function in children with and without gastroesophageal reflux.

Resting lower esophageal sphincter (LES) pressure was assessed in infants and children 2 weeks to 12 years of age. There were 62 control subjects and 35 patients with reproducible gastroesophageal reflux (GER) determined radiologically. In control subjects without GER: (1) LES pressure was well developed by 2 weeks of age; (2) in children less than 1 year of age, mean LES pressure (43.3 +/- 2.4 mm Hg) was significantly greater than mean LES pressure (30.6 +/- 2.3 mm Hg) children older than 1 year of age; (3) LES sphincter length increased with age; and (4) bethanechol 0.1 mg per kg subcutaneously caused a rise in LES pressure that increased in magnitude as LES resting pressures increased. In patients with GER: (1) only 16 or 35 children had LES pressures below the normal range for their appropriate age group; (2) LES length was shorter than control values in children beyond 6 months of age; (3) GER usually occurred in the absence of hiatus hernia; (4) clinical improvement was common and in patients with low LES pressure was associated with a rise in LES pressures to normal, even in the presenece of hiatus hernia; and (5) bethanechol caused a change and an absolute rise in LES pressure that were not significantly different from those observed in controls. These results indicate that in infants and children low LES pressure is not the sole determinant of GER, and that pharmacological stimulation of the Les could prove to be a useful adjunct to the medical management of GER.

Child↗