Evaluation of febrile infants under 3 months of age: is routine lumbar puncture warranted?

Fever may be the sole clinically evident presentation of serious bacterial infection (SBI) in a very young infant, and therefore lumbar puncture is still widely regarded as a mandatory procedure in the sepsis workup of febrile infants under 2 months of age. In this retrospective study, we evaluated the frequency and the diagnostic value of cerebrospinal fluid examination in 492 infants aged less than 3 months who were hospitalized because of fever during 1988-1994. The patients were categorized as being at "high risk" or "low risk" for SBI according to current clinical and laboratory criteria. Among the 492 infants, 196 (40%) were identified as "high-risk" for SBI, and 296 (60%) were at low risk. Among the overall series of infants, 60 babies (12%) were subsequently proven with bacterial infection. Among the 196 "high-risk" babies, 26% had bacterial infection, compared to only 3% of the 296 infants at low risk (p < 0.0001), denoting a sensitivity of 85% and a specificity of 65% of the clinical classification criteria. Lumbar puncture was done to 186 (46%) infants upon hospital admission; 176 punctures yielded satisfactory samples of cerebrospinal fluid (CSF). Sixteen (3%) patients had abnormal CSF findings: 2 of them had positive bacterial cultures and 14 were compatible with aseptic meningitis. The 2 patients with purulent meningitis were clinically very ill and were immediately recognized as deserving a lumbar puncture. Of the 14 patients with aseptic meningitis, 13 were initially screened as being at high risk for serious infection, and therefore underwent a lumbar puncture. Over the years of this survey, a declining trend for performing lumbar puncture in "low-risk" young febrile infants became evident: during 1988-1992, evaluation of sepsis included a lumbar puncture in 45% of the infants, compared to 27% during the following 2 years (p < 0.0001). Not one instance of purulent meningitis evolved among the infants in whom lumbar puncture was not performed. Our observations suggest that hospitalized young febrile infants may safely be spared a lumbar puncture when they do not meet the proposed criteria for being at high risk, or when their clinical and laboratory picture suggests being at low risk for SBI.

[Diabetic hand syndrome in juvenile diabetes].

Patients with Type I diabetes may develop a scleroderma-like syndrome, including limitation of joint mobility. This syndrome, cherioarthropathy, is considered a complication of diabetes, but its cause is unknown. We examined 30 Jewish and 13 Arab patients in our juvenile diabetes clinic for skin and joint involvement. Signs of cherioarthropathy in both hands were found in 13 children (30.2%); all had skin changes and 6 (13.5%) also had articular involvement of the hands. There was no correlation between the presence of cherioarthropathy and the patient's age or the duration of diabetes. The syndrome was significantly more frequent among Arabs (8/15, 61.5%) than Jewish children (5/30, 16.6%), p less than 0.01. There was a indirect correlation between incidence of cherioarthropathy and adequacy of glycemic control, but no difference in glycemic control between Arab and Jewish children. This may indicate a genetic factor in the development of cherioarthropathy in juvenile diabetes.

Familial homozygous hypercholesterolemia: clinical and cardiovascular features in 18 patients.

Homozygous familial hypercholesterolemia (HFH) is a very rare autosomal dominant disease characterized by accelerated severe atherosclerosis. We examined 18 patients from 9 families with HFH. The age range was 6-30 years (mean = 16 years). Male to female ratio was equal. All patients had huge, multiple tuberous xanthomas on the skin and tendons. Mean +/- standard deviation of plasma cholesterol, triglycerides, low-density lipoproteins (LDL), and high-density lipoproteins (HDL) cholesterol levels were 608 +/- 89, 122 +/- 39, 550 +/- 88, and 26 +/- 8 mg/dl, respectively. Five patients (28%) had angina pectoris, two sustained a myocardial infarction, and one died at the age of 15 years. Two-dimensional echocardiography demonstrated supravalvular aortic stenosis in 3 of the 13 patients (23%). Coronary arteriography performed in 11 patients demonstrated significant obstruction in 6 patients, 2 each with single-, double-, and triple-vessel disease. Left main stenosis was present in 3 patients (27%). Supravalvular aortic narrowing was demonstrated in 6 patients (54%) and was associated with a gradient in 2 (25 and 35 mmHg, respectively). Segmental contraction abnormalities were detected in 2 of the 11 patients (18%). It is concluded that coronary artery disease is prevalent in patients with HFH and, based on the data presented, we recommend the performance of noninvasive technique, coronary arteriography and supravalvular aortography at an early age to detect and to follow the progression of the disease.