Edward B. Singleton

Pseudohypoparathyroidism with bone changes simulating hyperparathyroidism. Report of a case.

From the extensive investigations of Dr. Fuller Albright has emerged our present-day understanding of the pathologic physiology of the clinical syndromes related to the functional states of the parathyroid glands (1). The clinical and biochemical manifestations of hyper- and hypoparathyroidism have been well documented in the medical literature, and the perplexities regarding these two conditions have for the most part been resolved. The nomenclature in connection with hypoparathyroidism has, however, become more complicated and confusing following the introduction of such neologic terms as pseudohypoparathyroidism in 1942 (2) and pseudo-pseudohypoparathyroidism in 1952 (3) by Albright and his co-workers. The classical picture of hypoparathyroidism is that of a clinical state of tetany due to disturbances of calcium and phosphorus metabolism. The biochemical changes may be depicted as follows: (a) increased renal tubular re-absorption of phosphorus; (b) decreased urinary excretion of phosphorus; (c) eleva...

Liver diseases and osteoporosis in children

Summary 1. Of 7 children with glycogen storage disease, 9 with primary hepatic tumors, and 10 with hepatic cirrhosis, approximately two thirds of them had osteoporosis, and many also had muscle atrophy and growth retardation. 2. One child with benign hepatomashowed dramatic improvement after the tumor was removed. 3. These observations suggest that a systemicdeficit of protein may be an important etiological factor of osteoporosis in liver diseases. 4. The pathogenesis of protein deficit ineach type of liver disease has been considered.

Bowel Stenosis as a Late Complication of Acute Necrotizing Enterocolitis

Bowel stricture as a late complication of acute necrotizing enterocolitis in 10 neonates is discussed with respect to time interval to development, number of strictures, location, sexual and racial distribution and possible contributing factors. This is compared with previous reports in the literature. Differences in the distribution of acute and late sites of involvement are discussed.

Chest radiographic data acquisition and quality assurance in multicenter studies

Background. Multicenter studies rely on data derived from different institutions. Forms can be designed to standardize the reporting process allowing reliable comparison of data. Objective. The purpose of the report is to provide a standardized method, developed as a part of a multicenter study of vertically transmitted HIV, for assessing chest radiographic results. Materials and methods. Eight hundred and five infants and children were studied at five centers; 3057 chest radiographs were scored. Data were entered using a forced-choice, graded response for 12 findings. Quality assurance measures and inter-rater agreement statistics are reported. Results. The form used for reporting chest radiographic results is presented. Inter-rater agreement was moderate to high for most findings, with the best correlation reported for the presence of bronchovascular markings and/or reticular densities addressed as a composite question (kappa = 0.71). The presence of nodular densities (kappa = 0.56) and parenchymal consolidation (kappa = 0.57) had moderate agreement. Agreement for lung volume was low. Conclusion. The current tool, developed for use in the pediatric population, is applicable to any study involving the assessment of pediatric chest radiographs for a large population, whether at one or many centers.

Radiologic Considerations of the Perinatal Distress Syndrome1

During the past decade many articles have appeared in the medical literature describing the causes, symptomatology, and radiologic findings of perinatal distress. Many of these publications include under this terminology any condition responsible for respiratory difficulty in the newborn infant. Consequently, there has developed some degree of confusion regarding the nomenclature of both neonatal and perinatal distress. In the newborn there are many abnormalities, including intracranial lesions, numerous primary pulmonary anomalies, cardiovascular defects, and defects of the diaphragm, which may be responsible for the clinical manifestations of respiratory difficulty. Each of these conditions interferes either mechanically or physiologically with the normal process of respiratory exchange; the cause is usually some anatomical defect which can be recognized by standard clinical, laboratory, or radiographic procedures. Examples include tracheoesophageal fistula, diaphragmatic hernia, congenital heart diseas...

A short story

ago, I received a call from a woman who said, “Dr. Singleton, I have been told that you are interested in dwarfs.” I was momentarily taken aback, but recognizing the seriousness of the woman’s voice, I replied that as a pediatric radiologist I had a professional interest in children with bone dysplasias. She continued by saying, “My son has been diagnosed as having achondroplasia, but I believe he is a metatrophic dwarf and would like your opinion.” I invited her to bring her child to the X-ray department, and after hanging up hurriedly reviewed the available literature in my office to determine what a metatrophic dwarf [1] looked like, both physically and radiographically. Luckily, I found the reference describing the condition which had been published only a few months before and was the first description of metatrophic dwarfism. Soon the mother arrived with her child, who obviously did not bear any close physical resemblance to achondroplasia. A photograph of the youngster was made (Fig. 1), as well as radiographs of his skeleton. The mother was almost correct; the clinical and radiographic features were indeed similar to those of pseudometatrophic dwarfism or Kniest dysplasia [2]. The mother obviously had taken a great interest in her child and was well educated on the general subject of abnormally small people. Recently, over 20 years later, I was told by the pediatric resident that a 2 /2-year-old male achondroplastic dwarf had fallen down a flight of steps and needed radiographic studies for evaluation of injuries. The X-ray examination revealed no evidence of fracture but showed skeletal changes which reminded me of pseudometatrophic dwarfism or Kniest dysplasia, this being only the second case I had Edward B.Singleton A short story Pediatr Radiol (1997) 27: 452–453  Springer-Verlag 1997

Radiology of aids in the pediatric patient

The Acquired Immunodeficiency Syndrome (AIDS) has involved the pediatric age group and is especially prevalent in babies born of mothers who are intravenous drug abusers or prostitutes. Approximately 30% of children born to mothers who are seropositive for the human immunodeficiency virus (HIV) will develop HIV infection. There are several important differences in children and adults with AIDS. The incubation period of the disease is shorter, and initial clinical manifestations occur earlier in children. In addition, certain infections are more common in children, and the different types of malignancy, especially Kaposis sarcoma, are unusual in the pediatric age group. The altered immune system involves both T cells and humoral immunity and increases susceptibility to a variety of infections, particularly opportunistic organisms. In this publication the complications of pediatric AIDS involving the lungs, cardiovascular system, gastrointestinal tract, genitourinary system, and neurological system are described. The most common pulmonary complications in our experience are Pneumocystis carinii pneumonia and pulmonary lymphoid hyperplasia. The spectrum of cardiovascular involvement in pediatric AIDS includes myocarditis, pericarditis, and infectious endocarditis. Gastrointestinal tract involvement is usually due to opportunistic organisms that produce esophagitis, gastritis, and colitis. Abdominal lymphadenopathy is a common finding either due to disseminating Mycobacterium avium-intracellulare infection or nonspecific lymphadenopathy. Although cholangitis is more commonly seen in adults, it may occur in children with AIDS and, in most cases, is due to related opportunistic infections. Genitourinary infections may be the first evidence of HIV disease. Cystitis, pyelonephritis, renal abscesses, and nephropathy with renal insufficiency are complications of pediatric AIDS. A variety of neurological abnormalities may occur in pediatric AIDS. The most common cause of neurological dysfunction in children with AIDS is HIV neuropathy. We present the many complications of AIDS in children demonstrated by a variety of imaging modalities, emphasizing the importance of diagnostic imaging in children with this disease.

Computerized tomography demonstration of an intrarenal teratoma

The computerized tomographic appearance of an intrarenal teratoma is described. The differential diagnoses, especially from Wilms tumor, are discussed.

Radiological evaluation of isolated ventricular septal defects before and after surgical closure.

The remarkable and often spectacular accomplishments in the field of cardiac surgery are familiar to each of us who has been in a position to follow the radiographic course of these patients. Whereas a short time ago only those radiologists associated with large teaching hospitals had this opportunity, many institutions now have their own “cardiac team” and are successfully diagnosing and correcting surgical defects of the heart. Consequently whether we are associated with a large teaching institution or a smaller hospital, or are even following the postoperative cardiac patient in office practice, it behooves us as radiologists to be familiar with the radiologic aspects of congenital heart disease and the changes to be expected following surgery. With the development and improvement of the pump-oxygenator, the surgical correction of many forms of congenital heart disease has become a common procedure. Although an isolated ventricular septal defect reportedly represents only about 8 per cent of all forms ...

Retrograde aortography in the diagnosis of congenitalheart disease in infants

Summary The diagnosis of congenital cardiacmalformations in infants is frequently difficult. Accurate and prompt diagnosis is often essential in order to determine if the abnormality is surgically correctable. Retrograde aortography is a useful procedure in establishing or excluding the diagnosis of several types of malformation, particularly patent ductus arteriosus, aortic septal defect, coarctation of the aorta, and truncus communis from other lesions such as interventricular septal defect and congenital aortic stenosis, where the clinical findings may be similar. Five cases are presented, illustrating the usefulness of this method.