Arthur J. Newman

Late-onset pulmonary fibrosis and chest deformity in two children treated with cyclophosphamide

2. Kopits SR, Per0vic MN, McKusick VA, Robinson RA, and Bailey J~. Congenital atlantoaxial dislocations in various forms of dwarfism, J Bone Joint Surg 54A:1349, 1972. 3. Kelly TE: The mucopolysaccharidoses and mucolipodoses, Clin Orthop 116:116, 1976. 4. Kopits SE: Orthopedic complications of dwarfism, Clin Orthop 116:153, 1976. 5. Blaw ME, Langer LO: Spinal cord compression in Morquio-Brailsfords disease, J PEDIATR 74:593, 1969.

Liver biopsy findings in patients with juvenile rheumatoid arthritis receiving long-term, weekly methotrexate therapy

We performed percutaneous liver biopsy in nine children who had received a weekly dose of methotrexate, 10 mg/m2 per week, for at least 3 years to address the concern about subclinical liver toxicity from single, weekly, low-dose methotrexate therapy for juvenile rheumatoid arthritis. No patient had clinical or biochemical evidence of liver injury. All biopsy results were interpreted as normal. These results suggest that the recommendations of the American College of Rheumatology for adults receiving single weekly methotrexate therapy for rheumatoid arthritis can be extended to children.

Sarcoidosis associated with nephrocalcinosis in young children

Three patients with nonpulmonary sarcoidosis had chronic erythema nodosum within the first 2 years of life. Each subsequently had renal sarcoidosis and nephrocalcinosis; hypercalcemia was documented in each patient and hypercalciuria in two patients. Treatment with prednisone was not uniformly successful in normalizing creatinine clearance. Nephrocalcinosis may be more common than previously reported in patients with sarcoidosis.

Pulmonary, Pleural, and Thoracic Changes Complicating Chemotherapy

Chronic respiratory failure slowly developed in two pediatric patients following long-term cyclophosphamide therapy for lymphocytic malignancy. One patient survived 12 years after acute lymphocytic leukemia was diagnosed but died of respiratory failure at age 16 still in initial remission; the other, a 12-year survivor of Hodgkin disease, has progressive deterioration of pulmonary function. Each patient received cyclophosphamide (less than 70 g) during the initial years of the disease. Autopsy of the first patient and lung biopsy in the second revealed severe pulmonary fibrosis. There was loss of compliance and a dramatic change in the shape of the thorax which produced a markedly reduced anteroposterior diameter in both patients, and recurrent pneumothoraces in one.

Enuresis and nocturia in sickle cell disease

I~ National Center for Health Statistics, Seasonal Variation of Births United States 19331963, Series 21, No. 9, p. 24. 2. U. S. Department of Public Health, Education, and Welfare, United States Public Health Service National Office of Vital Statistic, Vital Statistics of the United States vol. 1, 1958-1963. 3. Feggetter, S.: A review of 1,018 cases of congenital pyloric stenosis treated by Ramstedt operations, J. Internat. Coll. Surgeons 30: 696, 1958. 4. Carter, C.: The incidence of congenital hypertrophic pyloric stenosis, Brit. M. Bull. 17: 251, 1961. 5. Longino, L., Hendren, W., and Owing, R.: Congenital hypertrophic pyloric stenosis, Am. J. Surg. 10l: 605, 1961. 6. Ravitch, M. M.: Story of pyloric stenosis, Surgery 48: t l t 7 , 1962. 7. Donovan, E., and Stanley-Brown, E.: Congenital hypertrophic pyloric stenosis, Surg., Gynec. & Obst. 115: 403, 1962. 8. Singleton, A., and Fish, J.: Obstruction of the stomach and duodenum in infancy and childhood, Am. J. Surg. 106: 596, 1963. 9. tIanell, D.: Congenital hypertrophic pyloric stenosis, J. Abdom. Surg. 5: 51, 1963. 10. Beson, G., and Lloyd, J.: Infantile pyloric stenosis, Am. J. Surg. 107: 429, 1964. 11. Gharib, R.: The incidence of infantile hypertrophic pyloric stenosis, J. PEDIAT. 65: 622, 1964.

Moyamoya, dystonia during hyperventilation, and antiphospholipid antibodies.

A 5-year-old Asian male presented with episodes of dystonia involving the right upper extremity during vigorous crying. He was diagnosed with moyamoya disease. Initial laboratory evaluation revealed positive anticardiolipin and antinuclear antibodies.

Erythrocyte lipids and vitamin E in Type II congenital dyserythropoietic anemia

An 8-year-old girl with Type II congenital dyserythropoietic anemia, characterized by morphologic abnormalities of erythroid precursors, immunologic alterations, hyperbilirubinemia, and chronic anemia, was found to be vitamin E deficient. Nutritional history and vitamin E absorption studies indicated that neither dietary lack nor intenstinal malabsorption was the cause of the deficiency. Striking changes in the patients hematologic status following administration of vitamin E included rise in hemoglobin, decrease in bilirubin and reticulocyte count, and a marked increase in red blood cell survival. Erythrocyte phospholipids, altered while the patient was vitamin E deficient, returned to normal levesl during therapy. However, hematologic improvement was not complete, immunolotic abnormalities persisted, and morphologic aberrations in erythrocyte precursors actually increased during vitamin E therapy. It is therefore concluded that vitamin E deficiency played a secondary role in the production of the childs hematologic disorder and may have been a result of the increased utilization of the vitamin in stabilization of defective cellular membranes.

The kidney in childhood leukemia.

Involvement of the kidney at some stage of childhood leukemia is a frequent if not invariable occurrence, varying from microscopic infiltration to gross enlargement with subsequent aberration of function (5). Clinically the involvement may not be recognized until enlargement and palpable nodularity take place in the kidney. Merrill and Jackson (7) reported 10 cases of impaired renal function prior to death in an autopsy series of 18 leukemic patients. All of these were adults. Gilbert, Rice, and Lechaux (3) studied renal function in 35 children with leukemia and found no laboratory evidence of impairment. Pierce (9), on the other hand, made note of signs of impending uremia caused by leukemic infiltrates and found that enlargement of the kidneys from this condition was an ominous sign, rendering control by antileukemic drugs more difficult of achievement. Gowdey and Neuhauser (4) were the first to report (1948) a roentgenographic appearance of the kidneys which seemed to be characteristic for leukemia. Th...

Psychogenic Purpura in Adolescent Patients

The occurrence of psychogenic purpura has been documented in adults. The authors present their experience with nine adolescent and young adult patients observed since 1981. The age of eight of these patients ranged from 13 to 18 years. One patient with cystic fibrosis was 23-years- old. All patients presented with painful inflammatory lesions followed by bruising. There was no evidence of an infectious process or a hematologic, immunologic, or renal abnormality. Integral to the diagnosis was the existence of an underlying emotional disorder. In all patients, bruising decreased after diagnosis was explained to the patient and family. Four patients required psycho therapy. Results of 2-year follow-up suggest that early intervention may produce a favorable outcome in this age group.

Diagnostic x-irradiation as a possible etiologic agent in thyroid neoplasms of childhood

THE ASSOCIATION BETWEEN thyroid carcinoma in children and prior irradiation of the thymus Was first noted by Duffy and Fitzgerald j in 1950. Since then, there have been many reports of both benign and malignant neoplasms after head and neck irradiation in infancy and childhood, with an estimated 700-fold increase in the risk of thYroid neoplasia? 5 In all instances, the radiation was administered therapeutically for benign conditions such as thymic and tonsillar hypertrophy, tinea capitis, acne, and hemangiomas, a practice which has rightly fallen into disrepute. We report two children who had been exposed to radiation through extensive diagnostic radiographs that included the head and neck region, and who developed thyroid carcinoma. Neither had been irradiated for therapeutic purposes.