Volume 95 Number 6

W i l l e b r a n d disease reported, t r e a t m e n t o f the associated m a l i g n a n t disorder resulted in the resolution o f b l e e d i n g manifestations in our patient. It would thus seem reasonable to speculate that the t u m o r itself m a y have p r o d u c e d or released a substance t h a t selectively i n h i b i t e d a p l a s m a factor resulting in a defect similar to that observed in genetic von W i l l e b r a n d disease. The authors thank Leon Hoyer, M.D., Farmington, Connecticut, for measurements of VIIIR:Ag; Charles Abildgaard, M.D., Davis, California, for assay of the Ristocetin co-factor; William Morris, M.D., for referring to and assisting us in the care of this patient; and Victoria Villaseca and Nancy Anderson for their excellent technical assistance.

Brief clinical and laboratory observations

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REFERENCES

1. Chun-Yet Lian E, and Deykin D: Diagnosis of von Willebrand's disease-a comparative study of diagnostic tests on nine families with von Willebrand's disease and its differential diagnosis from hemophilia and thrombocytopathy, Am J Med 60:344, 1976. 2. Handin RI, Martin V, and Maloney WC: Antibody-induced von Willebrand's disease: A newly defined inhibitor syndrome, Blood 48:393, 1976. 3. Joist JH, Cowan JF, and Zimmerman TS: Acquired von

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Willebrand's disease: Evidence for a quantitative and qualitative factor VIII disorder, N Engl J Med 298:988, 1978. Rosborough TK, and Swain WR: Acquired yon Willebrand's disease, platelet release defect and angiodysplasia, Am J Med 65:96, 1978. Owen CA, Bowie EJW, and Thompson JH: The diagnosis of bleeding disorders, ed 2, Boston, 1975, Little Brown & Company, pp 85-154. Hruby MA, and Schulman 1: Failure of combined factor VIII and cyclophosphamide to suppress antibody to factor VIII in hemophilia, Blood 42:919, 1973. Hoyer LW: Immunologic studies of antihemophilic factor (AHF, Factor VIII). IV. Radioimmunoassay of AHF antigen, J Lab Clin Med 80:822, 1972. MacFarlane DE, Stibbe J, Kirby EP, Zucker MB, Grant RA, and McPherson J: A method of assaying von Willebrand factor (Ristocetin co-factor), Thromb Diath Haemorrh 34:306, 1975. Olson JD, Brockway W J, Fass DN, Magnuson MA, and Bowie EJW: Evaluation of Ristocetin-Willebrand factor assay and ristocetin-induced platelet aggregation, J Lab Clin Pathol 63:210, 1975. Montgomery RR, Hays T, and Tubergen DG: Spontaneous remission of acquired von Willebrand's disease (VWD) following resection of Wilms' tumor (abstract) Pediatr Res 12:469, 1978,

Severe Mycoplasmapneumoniae infection in otherwise health): siblings Jonathan I. Singer, M.D.,* and William M. DeVoe, M.D., Cincinnati, Ohio

m Y c o P L A S M A L D I S E A S E a m o n g children is c o m m o n l y subclinical or clinically benign, a n d medical a t t e n t i o n may not even be sought. 1 T h e respiratory m a n i f e s t a t i o n s of disease p r o d u c e d by Mycoplasma pneumoniae in children are generally mild a n d brief, but occasionally m a y b e prolonged.-' C h i l d r e n with lower respiratory illnes s typically have m i n i m a l physical findings,:' A characteristic r o e n t g e n o g r a p h i c feature in Mycoplasma pneumoniae p n e u m o n i a is a lack of significant pleural effusion. ~ Severe or life-threatening m y c o p l a s m a l infection, as described in the following case presentations, is distinctly unusual?

From the University of Cincinnati College of Medicine, Division of Ambulatory Services; Cincinnati Children's Hospital Medical Center. *Reprint address: Children's Hospital Research Foundation, Elland and Bethesda A yes. Cincinnati, OH 45229.

0022-3476/79/120999+03500.30/0 9 1979 The C. V. Mosby Co.

CASE REPORTS Patient 1. A previously well 5-year-old Korean-American boy was admitted with a ten-day history of anorexia, cough, coryza, fever to 40.0~ and an evanescent papular truncai exanthem. He had received a four-day course of ampicillin prescribed for otitis media at another health facility. When symptoms persisted, evaluation at our institution revealed a vigorous child with physical findings and roentgenographic confirmation of right middle-lobe pneumonia. A tuberculin skin test and blood culture were performed and subsequently found to be negative. He was treated on an outpatient basis with penicillin, given intramuscularly and orally for six days, without clinical response. Admission was prompted by abrupt temperature elevation, onset of respiratory distress, and pleuritic chest pain. Examination revealed an acutely ill-appearing child with a temperature of 39.7~ pulse 156, respiratory rate 56, and blood pressure 80/60. He had an erythematous, maculopapular rash on his trunk. There were tales in the right lung base with decreased breath sounds. His right chest cage was splinted during paroxysms of coughing.

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Brief clinical and laboratory observations

Figure.

Patient 1. Chest radiograph reveals right lower lobe consolidation with massive pleura[ effusion.

Admission WBC count was 14,000 with 82% polymorphonuclear leukocytes and 6% bands. The chest radiograph revealed multilobe involvement on the right with a small amount of pleural fluid. Diagnostic thoracentesis yielded several milliliters of serous fluid that failed to yield growth of any bacterial or fungal organisms, and routine virus cultures on Rhesus monkey, Cynomolgus monkey, embryonic lung (WI-38), and HeP-2 tissue cultures were negative. The patient was treated with ampicillin intravenously and remained febrile up to 41.1~ By the fourteenth day of the illness, increasing respiratory embarrassment and development of right pleural effusion mandated pleural drainage for an eight-day period (Figure). Treatment was changed to intravenous chloramphenicol on the sixteenth day, and his condition slowly improved over a 12-day period. Intermittent fever to 38.9~ persistent leukocytosis, and macular or papular exanthems continued until the twenty-eighth day of the illness, at Which time the second sibling was admitted. The acute myeoplasma complement fixation titer on admission was 1:512 and the convalescent titer drawn at the time of discharge was 1:256. Patient 2. The previously well 3-year-old sibling of Patient 1 was admitted with fever, tachypnea, and pleurtic chest pain following a three-day illness manifested by anorexia, malaise, coryza, cough, and unresponsiveness to ampicillin administered orally by the parents without medical supervision. Examination revealed an ill-appearing child with a temperature of 39.4~ pulse 150, respiratory rate 40, and blood pressure 108/62. He had bilateral bulging tympanic membranes and rhonchi but no tales or alterations of breath sounds. ~ '~ Admission WBC count was 6,830 with a normal differential. A radiograph revealed diffuse parenchymal disease involving tire right upper lobe and lingular segments of the left lower lobe without pleural effusion. The patient was treated with ampicillin intravenously but

The Journal of Pediatrics December 1979

within two days, when the patient's course deteriorated clinically and radiographical!y, therapy was changed to chloramphenicol. On the sixth day of the illness, treatment was changed to erythromyein when mycoplasma complement fixation.titers on patient number one were returned. This patient had a slow recovery with intermittent temperature elevation and transiently elevated serum values of glutamic oxalacetic transaminase and glutamic pyruvic transaminase, and microscopic hematuria. He was discharged after a week's hospitalization without any apparent sequelae. Acute mycoplasma complement fixation titer was I: 16 and the convalescent titer drawn prior to discharge was > 1:128. Patient 3. The previously wel! 7-year-old sibling was admitted on the same day as Patient 2 with pneumonia unresponsive to two days of orally administered ampicillin. He had been ill in the preceding week with anorexia, malaise, nonproductive cough, and !ow-grade fever. On the day of admissiom, he complained of chills and epigastric discomfort. On examination, he was in mild distress with a temperature of 38.3~ pulse 120, respiratory rate 40, and blood pressure 104/60. He had decreased breath sounds in the lung base. A WBC count was 6,500 with a normal differential. A chest radiograph revealed left lower-lobe pneumonia with pleural effusiorf. Thoracentesis revealed serosanguineous fluid. He was treated with chloramphenicol intravenously but appeared refractory to therapy and treatment was subsequently changed to erythromycin. He underwent an open pleural and lung biopsy a t the time of insertion of a chest tube two days after admission. Pleural drainage was maintained for five days. He was discharged after nine days of hospitalization and has subsequently shared the good health of his two brothers. The acute mycoptasma complement fixation titer was 1:16 and convalescent tiler drawn at the time of discharge was > l: 128. For all siblings, sputum and blood cultures were sterile. Pleural fluid and pleural tissue were sterile on culture. Blood and pleural fluid for pneumococcal and Haemophilus capsular antigens were negative. No virus was isolated from nasopharyngeal and rectal swabs or pleural fluid. Cold agglutinins, histoplasma complement fixation titers, and culture and complement fixation titers for Legionnaire disease were negative. Fungal immunodiffusion screen for histot~smosis, blastomycosis, coccidioidomycosis, and aspergillosis was negative. Hemoglobin electrophoresis, complement profile, serum immunog!obins, and isohemagglutinin titer were normal. Tuberculin skin tests were negative; candida skin tests were reactive. Lymphocytic stimulation with phytohemagglutinin was normal. The morphology and numbers of circulating neutrophils were normal. eDISCUSSION "An illness characterized by toxicity, fever, infiltrates with effusion, leukocytosis, a n d lack o f response to low oraI doses o f antibiotics is most characteristic o f the bacterial p a t h o g e n s that m a y cause p n e u m o n i a . However, k n o w n p u l m o n a r y p a t h o g e n s were sought in the blood, pleural fluid, and other body fluids in these three patients, a n d were not identified.

Volume 95 Number 6

Brief clinical and laboratory observations

The clinical characteristic serving as the first clue to the etiologic diagnosis was the lack of response to the various antibiotic regimens utilized. The diagnosis of Mycoplasma gneumoniae infection in these children was m a d e on the basis of elevated mycoplasma complement fixation titers, exclusion of other known respiratory pathogens, and on epidemiologic grounds. The father, who had been symptomatic with respiratory complaints the week before the first child's illness, was found to have a stable, high mycoplasma complement fixation titer. Serious mycoplasmal infections manifested by respiratory distress, multilobular pneumonitis, pleural effusion, prolonged fever, and leukocytosis have been described previously in patients with hemoglobinopathies ~~ or dysgammagl0bulinemias.~ There have been no reports of mycoplasmal pulmonary disease of comparable severity in otherwise healthy children. Though severe mycoplasmal disease has been uncommon in children, our experience with these three siblings suggests that the disease spectrum is wider than previously thought, and that severe pulmonary involvement can occur in otherwise healthy children.

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REFERENCES

1. Glezen WP, and Denny FW: Epidemiology of acute lower respiratory disease in children, N Engl J Med 288:498, 1973 2. Glezen WP, Th0rnburg G, Chin TDY, and Herbert AW: Significance of mycoplasma infections in children with respiratory disease, Pediatrics 39:516, 1967. 3. Clyde WA and Denny FW: Mycoplasma infections in childhood, Pediatrics 40:669, 1967. 4. Major DHH: The roentgen features of Eaton agent pneumonia, Am J Roentgenol Radium Ther Nucl Med 98:300, 1966. 5. Shulman ST, Bartlett J, Clyde WA, and Ayoub EM: The unusual severity of myc9piasmal pneumonia in children with sickle-cell disease, N Engl J Med 287:164, 1972. 6. Chusid M J, Lachman BS, and Lazerson J~ Severe mycoplasma pneumonia and vesicular eruption in SC hemoglobinopathy, J PEDIATR93:449, 1978. 7. Foy HM, Ochs H, Davis SD, Kenny GE, and Luce RR: Mycop!asma pneumoniae infections in patients with immunodeficiency syndromes: Report of four cases, J lnfect Dis 127:388, 1973.

Endophthalmitis due to Salmonella enteritidis Larry I. Corman, M.D., Robert H. P0irier, M.D., Christine A. Littlefield, M.D., and Ciro V. Sumaya, M.D.,* San Antonio, Texas

WE HAVE RECENTLY SEEN an infant who had unilateral inflammation of the intraocular contents, endophthalmitts, caused bY Salmonella enteritidis. CASE REPORT

Patient R.B. was a 7-week-old white boy who presented to the hospital with a n inflamed right eye with leukocoria. He was the product of a normal first pregnancy of a 16-year-old mother. Approximately 'four days prior to hospital admission the infant developed yellow, watery diarrhea which responded to administration of clear fluids. On the day prior to admission the diarrhea became more severe and the infant became febrile; he was examined at the Walk-In Clinic of the Robert B. Green Hospital and was noted to have injection of the conjunctiva and slight From WilJbrd Hall USAF Medical Center and the Departments of Ophthalmology, Pediatrics, and Pathology, University of Texas Health-Science Center. *Reprint address: Dr. Sumaya, Department of Pediatrics, University of Texas Health Science Center, 7703 Floyd Curl Drive, San Antonio, TX 78284.

periorbital swelling of the right eye. Sodium sulfacetamide ophthalmic ointment was prescribed. He was admitted to the hpspita[ the following day because of persistent high fever and the development of marked periorbital swelling, proptosis, mild corneal edema, and a yellow-white "mass" behind the pupil. Laboratory results included the following: hemoglobin 9 gm/dl, WBC count 19,700, 56% neutrophils !7% stabs, 27% lymphocytes. Roentgenograms of the skull, facial bones, and sinuses showed no abnormalities other than soft tissue swelling around the right eye. A chest roentgenogram revealed a right upper lobe pneumonia. Computerized tomography of the orbits demonstrated a large irregular right globe with extensive soft tissue swelling. No definite tumor was identified. Paracentesis of the anterior chamber of the right eye yielded a purulent exudate which contained no malignant appearing cells. Salmonella enteritidis group D was cultured from the exudate. No organisms were cultured from the blood, cerebrospinal fluid, or stools. The patient initially received ampicillin 200 mg/kg/day, gentamicin 5 mg/kg/day, and methicillin 200 mg/kg/day, all by the intravenous route. As the eye isolate was found to be sensitive to ampicillin, methici!lin and gentamici n were discontinued. The

Severe Mycoplasma pneumoniae infection in otherwise healthy siblings.

Volume 95 Number 6 W i l l e b r a n d disease reported, t r e a t m e n t o f the associated m a l i g n a n t disorder resulted in the resolution o...
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