Raynaud's features in childhood. Clinical, immunological and capillaroscopic study.

Raynaud's phenomenon, uncommon in childhood, often heralds connective tissue disorder. Since microvascular abnormalities can be detected at an early stage of the connective tissue disease, especially in scleroderma, a specific diagnosis can be made in patients presenting with Raynaud's phenomenon alone or Raynaud's phenomenon associated with symptoms suggestive of connective tissue disease. Raynaud's phenomenon was studied in 11 consecutive children, 10 girls and 1 boy, ages 6 to 15. One child had a definite diagnosis of cutaneous polyarteritis nodosa. In 6 others connective tissue disease was suspected: 4 had arthritis, 2 has telangiectasia, leg ulcers and antinuclear antibodies. Of the remaining 4, one had hemiplegia and 3 Raynaud's phenomenon only. Oscillometry of the radial artery was reduced in 7 of 9. Decreased capillary resistance was found in 2 of 6, while abrupt thinning in conjunctival vessels was seen in 3 of 7. On nailfold capillaroscopy, reduced vascularity was noted in 5 of 11, dilated capillaries in 4 of 11, tortuousity in 2 of 11, capillary thinning in 1 of 11, capillary spasm in 1 of 11 and normal pattern in 3 of 11. Two patients presenting with Raynaud's phenomenon were found to have "scleroderma-like pattern" on nailfold capillaroscopy. One of them died 2 years later of cardiopulmonary sclerosis, and another developed esophageal stricture and Barrett's esophagus. Neither has sclerodermatous skin. In childhood Raynaud's phenomenon, nailfold capillaroscopy is a non-invasive examination enabling early diagnosis of "systemic scleroderma sine scleroderma".

Infantile multisystem inflammatory disease: a specific syndrome?

We report two patients with infantile onset of evanescent rash, fever, arthropathy with severe deformities, periosteal changes, chronic meningitis, hydrocephalus, convulsions, developmental delay, papilledema, unusual uveitis, and lymphadenopathy. A few patients with similar findings have been previously reported. Although some similarity exists between findings in these patients and in others with systemic juvenile rheumatoid arthritis, they appear to differ both in regard to the nature and severity of the clinical and pathologic features. We suggest that this group of patients has a separate rheumatic disorder not yet included in the standard classifications of the childhood rheumatic diseases.

Juvenile gouty arthritis. Two cases associated with mild renal insufficiency.

Two patients had onset of juvenile gouty arthritis at ages 16 and 1 1/2 years, respectively. Both had mild renal insufficiency, with creatinine clearances of 46 and 54 mL/min/1.73 sq m, respectively. Their presenting hyperuricemia (13.8 and 11 mg/dL, respectively) was out of proportion to the degree of renal insufficiency. Clinical and laboratory studies did not suggest an inborn error of purine metabolism, glycogen storage disease type I, or any myeloproliferative disorder. Neither patient had a family history of gout or inherited renal disease. Although juvenile gouty arthritis is rare, it must be considered in the differential diagnosis of episodic arthritis in children, especially if renal impairment, even mild, is present.

In vitro study of cytotoxic factors against endothelium in childhood dermatomyositis.

In a previous report we showed that leukocytes from a group of patients with childhood dermatomyositis (CDM) were not cytotoxic toward cultured normal human skeletal muscle cells. Blood products from 11 patients with CDM and 12 age- and sex-matched controls were tested for cytotoxicity toward human endothelium using a chromium 51 assay. Mixed lymphocytes, monocytes, and serum alone or in combination did not produce endothelial cell death. The combination of serum and leukocytes, however, did produce some cytotoxic effects in three of 11 patients with CDM. We conclude that those factors tested in vitro are not responsible for the endothelial cell death but together may produce cytotoxic changes in some patients.

In vivo atrial excitability and anti-arrhythmic drugs.

The threshold of excitability of the atrial muscle was studied in the in vivo beating canine heart. Unipolar cathodal and anodal strength-interval curves were constructed and found to be dissimilar in shape. It was found that at any interval within the relative refractory period of the atrium, as in the ventricle, there is a wide range of current levels delineated by an upper (TU) and lower (TL) limit of threshold which can stimulate the atrial myocardium. Within these limits the threshold varies spontaneously and can be reduced to TL level by a run of extrasystoles. Such TU and TL curves were repeatedly determined following administration of therapeutic doses of quinidine, procaine amide or lidocaine. It was observed that all three drugs prolonged the refractory period. The TU values increased following each of the drugs, and mostly after quinidine, while the TL curve was less affected by quinidine. It is suggested that the exit block thus produced is the principal mechanism whereby quinidine depresses atrial disrhythmias.