Airway in the oculo-auriculo-vertebral spectrum: two cases and a review of the literature.

Two patients with oculo-auriculo-vertebral syndrome and multiple airway anomalies are presented. Both patients had esophageal atresia with a distal tracheoesophageal fistula and tracheomalacia due to innominate artery compression. Pulmonary hypoplasia, obstructive sleep apnea, and a laryngeal anomaly were also noted. The literature of airway anomalies and obstructive sleep apnea described in association with the oculo-auriculo-vertebral spectrum is reviewed.

Necrotizing pneumococcal pneumonia in childhood.

We describe the rare complication of necrotizing pneumonia and invasive pneumococcal infection in 3 previously healthy pediatric patients. Lobar consolidation and pleural effusions appeared initially, followed within several days by the appearance of multiple small lucencies in the area of consolidation. In one case, necrosis progressed to a large abscess cavity. Surgical intervention was limited to treatment of pleural space complications. There were no deaths. Pulmonary parenchymal residual was limited to a thin-walled cavity in one case.

Spontaneous resolution of a plasma cell granuloma in a 9-year-old.

A previously healthy 9-year-old white boy presented with a 13-lb weight loss over a period of 4 weeks and a 4.5-cm mass in the right lung. Histology was compatible with a plasma cell granuloma, which is the most common benign childhood lung tumor. Surgical management with segmental or wedge resection is the usual standard of care in this situation. However, it has been suggested that with a confirmed histologic diagnosis surgical resection is not warranted. This patient was managed conservatively. Repeat computed tomography scan 6 weeks later revealed significant resolution of the lesion, and at 7 months the lesion had totally resolved. Spontaneous resolution of this lesion has been rarely described in pediatric populations.

Hyperleukocytosis with pertussis.

A 9-month-old nonimmunized white female patient presented with a paroxysmal cough and a white blood cell count of 114,000/mm3. A nasopharyngeal culture was positive for Bordetella pertussis. Hyperleukocytosis is a rare complication of pertussis and is attributed to lymphocytosis-promoting factor. Hyperleukocytosis with pulmonary leukostasis can result in significant hypoxemia, although it did not occur in the case presented.

Central nervous system manifestations of parainfluenza virus type 3 infections in childhood.

Three children were admitted within an 8-week period with parainfluenza virus type 3 infection accompanied by encephalitis. All 3 patients had electroencephalograms characterized by slowing, disorganization of background activity, and multifocal sharp-wave activity with temporal predominance. Apnea and periodic breathing were observed in 1 patient, opsoclonus-myoclonus in another, and disease mimicking herpes encephalitis in the third. All 3 patients recovered without neurologic residua.

Pseudomonas aeruginosa flagellar antibodies in patients with cystic fibrosis.

An enzyme-linked immunosorbent assay specific for flagellum type (a or b) of Pseudomonas aeruginosa was used to detect serum immunoglobulin antibodies in 98 random outpatients and 14 colonized cystic fibrosis patients. Antibodies were detected to both types of flagella in addition to M-2 lipopolysaccharide. Titers to both flagellar antigens (FlAg) were 10 to 100 times higher in cystic fibrosis patients than in random outpatients of a comparable age group. Mean antibody titers against b-type FlAg were 454 for outpatients (ages newborn to 21 years), whereas the mean titer for cystic fibrosis patients (ages 6 to 21 years) was 51,520. Titers against a-type FlAg were generally lower, with mean outpatient titers of 68 and mean cystic fibrosis patient titers of 34,323. Differences were also seen in antibody titer against M-2 lipopolysaccharide, but these differences did not correspond to M-2 FlAg titers. In 98 random outpatients (ages newborn to 86 years), FlAg titers generally increased with age. To demonstrate further specificity of the enzyme-linked immunosorbent assay for flagellum antibody, Western blots were performed with selected high-titer cystic fibrosis patient sera. Sera that had a high titer (greater than 25,600) for b- or a-type FlAg showed a corresponding reactive band. These results demonstrate that flagellum antibodies are produced in humans in response to P. aeruginosa infection.

Severe giardiasis in a patient with cystic fibrosis.

A 20-year-old male patient with cystic fibrosis (CF) is described, who acutely developed hypoalbuminemia concurrently with giardiasis. Hypoalbuminemia could not be explained by the usual causes seen in patients with CF, but resolved with quinacrine therapy. Subsequently, asymptomatic giardiasis was sought but not found by either the string test or stool exam in any of 15 patients with pancreatic insufficiency who were examined in a prospective manner. Although pancreatic insufficiency is felt to be able to act synergistically with giardiasis to accentuate malabsorption, patients with pancreatic insufficiency per se are not necessarily at greater risk for colonization with Giardia.

Appendiceal abscess in cystic fibrosis. A diagnostic challenge.

A patient with cystic fibrosis who developed appendicitis, rupture, and a periappendiceal abscess is presented. A retrospective chart review revealed 5 other cases that demonstrate a spectrum of clinical presentation of periappendiceal abscess in patients with cystic fibrosis. Three patients were symptomatic for less than 5 days, but the remaining 3 patients were symptomatic for 8, 12, and 30 days before diagnosis. There were two deaths due to respiratory failure. Other complications included a perirectal fistula and 2 cases of recurrent abscess. This demonstrates the difficulty with which this diagnosis is reached in this patient population and the relatively high incidence of abscess formation compared with normal populations. A retrospective autopsy review of 51 cystic fibrosis patients showed that in 49 of 51 instances, the mucosa of the appendix was hyperplastic, and the mucosal glands were distended with eosinophilic secretions. In 12 cases (24%), the appendix itself was grossly firm, dilated, and distended, although the mucosal wall was free of inflammation. This lends credence to the suggestion that these inspissated secretions may be protective against the occurrence of appendicitis, the incidence of which may be as low as 1%-2% among cystic fibrosis patients.

Klebsiella ozaenae in a patient with cystic fibrosis.

For a 3 1/2-year period, a mucoid strain of Klebsiella ozaenae supplanted the growth of Pseudomonas aeruginosa in the respiratory tract of a patient with cystic fibrosis. He showed no clinical signs of ozena. While the patient was colonized with K ozaenae, his pulmonary status essentially remained unchanged. However, his clinical condition deteriorated rapidly when P aeruginosa colonization again became predominant.

Role of antiviral antibodies in resistance against coxsackievirus B3 infection: interaction between preexisting antibodies and an interferon inducer.

An experimental model of coxsackievirus B3 infection in newborn mice was utilized to examine the protective role of antiviral antibodies and an interferon inducer, polyinosinic acid-polycytidylic acid [poly(I:C)]. Subcutaneous administration to the infected mice of specific antiviral antibodies resulted in significant protection against coxsackievirus B3 infection. Antibody-treated animals had shortened viremia, early clearance of virus from tissues, and a reduced mortality rate. Dose response to antibodies was clearly demonstrated. However, the time of antibody administration in relation to the infection cycle was important. The protection was observed if antibodies were given before infection (24 h) or shortly after (2 h) infection. Administration of antibodies 24 h after infection resulted in no protection. The interferon inducer poly(I:C) prolonged the survival time of the infected mice, but this protective effect was incomplete and could only be demonstrated in animals treated before infection (24 h) or shortly after (2 h) infection. Enhanced protection against lethal coxsackievirus B3 infection was achieved in animals treated with a combination of antiviral antibodies and poly(I:C). These data confirm that antibody-mediated immunity plays a significant role in resistance against coxsackievirus B3 infection and suggest that antiviral antibodies may interact with poly(I:C) or work independently to produce an enhanced protective effect.

Bacterial endocarditis due to penicillin-resistant Streptococcus viridans.

Bacterial endocarditis remains a formidable diagnostic and therapeutic problem for clinicians. Streptococcus viridans still accounts for 45 to 50 per cent of all cases and between 5 to 10 per cent of all clinical isolates of Streptococcus viridans from patients with bacterial endocarditis may be relatively resistant to penicillin. The case of a 9-year-old child with Tetralogy of Fallot and a Waterston shunt who subsequently developed bacterial endocarditis due to penicillin-resistant Streptococcus viridans following failure of oral penicillin dental prophylaxis is presented. In the face of penicillin resistance, additional considerations for workup, including microbiological assays for antimicrobial synergism become necessary in the selection of a therapeutic regimen.

Endometritis and neonatal sepsis due to Streptococcus pneumoniae.

Attention is called to the rarely described clinical entity of pneumococcal infection involving both mother and neonate. A case is described in which neonatal sepsis and puerperal endometritis were documented by isolating Streptococcus pneumoniae type 3 from both mother and child. Clinical implications and a review of relevant literature are briefly discussed.