Stickler, Gunnar B.

Primary lymphedema, a disorder causing persistent swelling in an extremity, is rare in children and adolescents; it affects 1.15/100,000 persons less than age 20 years. It primarily affects girls near menarche. The records of 125 children and adolescents, aged 0 to 20 years, who were examined at the Mayo Clinic were analyzed; 99 of these patients were contacted to obtain follow-up data. The influences of estrogen and inflammation are thought to be important etiologic factors in primary lymphedema. The diagnosis can be made on the basis of a thorough history and physical examination. Lymphangiograms, venograms, and biopsies add nothing to the diagnosis because of the low incidence of tumor in children and adolescents. Conservative treatment is recommended: a Jobst-type stocking, elevation, and proper foot care. Diuretics are not recommended. Careful psychologic counseling, especially in adolescents, is highly recommended.

In small children with nutritional vitamin D deficiency, the serum concentration of 25-hydroxyvitamin D (25-OH-D), the major circulating metabolite of vitamin D, was correlated with the stage of clinical disease. It was low (16 to 20 ng/ml) but within the normal range in the earliest (hypocalcemic) stage of the deficiency syndrome and decreased (< 15 ng/ml) in the more advanced stages. In patients with familial hypophosphatemia (X-linked dominant), mean serum 25-OH-D concentration was the same as in age-matched normal controls. Evidence is presented that endogenous parathyroid hormone may have a role in the depletion of serum 25-OH-D in deficiency states.

The study of a patient with hypophosphatemic rickets, hyperglycinuria, glycosuria, and glycyl-prolinuria by Dr. Charles R. Scriver and co-workers (Pediatrics, 34:357) was of great interest to me. However, I must take issue with certain interpretations of data presented by these authors. It is stated, "The fecal excretion of phosphorus was slightly more than the urinary excretion. On the assumption that fecal phosphorus has the relation: 0.5 X fecal calcium/calcium intake X phosphorus intake, the fecal phosphorus was an appropriate amount.

In Freeman and Borkowf's article on "Craniostenosis" in the July issue of Pediatrics(29:159, 1962), he authors quote Hemple "et al" frequently and correctly (their reference 1). It is unfortunate that the name of Dr. Lloyd E. Harris, a co-author of the Hemple paper, was not mentioned once in Freeman and Borkowf's paper except insofar as it was understood to be a part of "et al." As much as I have admired Dr. Hemple's effort and accomplishment, I feel obliged to point out that the revision of our thinking in regard to the surgical treatment of synostosis of the sagittal sutures was based on the clinical observations of my esteemed teacher, colleague, and friend, Lloyd E. Harris.

Familial metaphysial dysostosis has been described as it occurred among 18 members of a family in seven generations. The roentgenologic changes were very similar to those seen in vitamin D resistant rickets. No biochemical defect could be detected. Studies of renal function with special reference to tubular reabsorption of phosphorus gave normal values. The treatment of choice is osteotomy, after epiphysial closure has occurred, to correct the pronounced bowing of the legs. Administration of vitamin D in high doses has no effect on the course of this disease.

The term "polypharmacy" is used to describe the excessive use of drug therapy. In 1980, I reviewed the evidence of a continuing epidemic of polypharmacy.1 In 1975, there were 1.7 billion visits per year to physicians in private practice.2 Such visits to a family physician resulted in the prescription of medications to 73% of the patient contacts; in 63% of the patient visits, medications were suggested by a pediatrician. Do two thirds of our patients require medication when seen in an ambulatory setting? Pediatric hospitals did not fare much better. Of 993 patients admitted to a children's hospital, 36.7% received antibiotics.3 Using strict criteria, these authors concluded that 38% of medical patients received antibiotics inappropriately and 78% of the surgical patients did not require antibiotics.

The clinical course of 41 patients who had systemic lupus erythematosus complicated by nephritis with symptoms beginning before they were 15 years old was reviewed. The retrospective analysis showed a definite trend toward prolonged survival among the patients receiving high doses of steroids, compared to those receiving no or low doses. We conclude that it is no longer justified to withhold high-dose steroid therapy from children with lupus nephritis, but it should be emphasized equally that this does not apply to children with discoid lupus erythematosus, rheumatoid arthritis with a positive LE clot test, or drug-induced lupus erythematosus. Our present plan of management is outlined.

We continue to agree with Dr. Nellhaus1 concerning the clinical value of the measurement of head circumference in children. Since publication of the paper by O'Connell and colleagues2 the opportunity has presented at the Mayo Clinic to study in detail a group of 15 children with idiopathic hypopituitary dwarfism. It was generally agreed that the children with this type of growth failure and normal intelligence do not have head circumferences of less than -2 standard deviations (S.D.).

A survey of the case records of children seen at the Mayo Clinic from 1935 to 1965 revealed that 77 had nephro-ureterolithiasis and 6 had nephrocalcinosis. Calculous disease occurred twice as frequently in boys as in girls; the incidence did not change with age between the ages of 1 and 15 years. Urologic lesions, urinary infections, or both were present in 49 children and cystinuria was present in 5. In 8 children, renal stones were considered causally related to hypercalciuria, whereas in 14 children no etiologic associations could be found. Chemical analysis of calculi, which was performed in 36 cases, revealed that most stones consisted of calcium oxalate or phosphate; no uric acid stones were found.

The method of collection of urine for bacteriologic investigation is of utmost importance. Catheterization and midstream collection* are both acceptable and widely employed methods of obtaining urine from the female child for colony counts. Nonetheless, neither is practical nor possible in many instances. Often urine must be collected by having the child void into a sterile container after thorough cleansing of the area. Frequently, in such instances, evidence of bacteriuria is found which may be puzzling in the absence of symptoms or other evidence of urologic disease. Contamination in such cases is a likely source and we wish to point out what is considered a significant observation that may have important influence on the method of collection of urine.