Erythrocyte sedimentation rate and C-reactive protein discrepancy and high prevalence of coronary artery abnormalities in Kawasaki disease.

BACKGROUND: An outbreak of Kawasaki disease (KD) in Colorado between November, 1997, and June, 1998, provided the opportunity to study inflammatory indices and coronary artery abnormalities. METHODS: Medical records of the 33 patients diagnosed with KD at The Children's Hospital during the outbreak were reviewed. Demographic and clinical information, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR) and echocardiogram results were recorded. Traditional abnormalities (dilatation, aneurysm, ectasia), as well as "prominence" of the coronary arteries were noted. RESULTS: Twenty-five patients had CRP and ESR performed on the day of admission; 11 of 25 (44%) had a discrepancy between the height of the ESR and CRP values (high ESR and low CRP or low ESR and high CRP). The mean CRP was higher in patients who presented in <10 days than in patients who presented in > or =10 days: 13.9 mg/dl vs. 5.2 mg/dl (P = 0.01). The ESR value did not correlate with the day of illness. Age, gender or presence of coronary artery abnormalities did not correlate with the height of CRP or ESR elevation. Thirty percent of patients had at least one abnormality on their initial echocardiogram (dilatation, aneurysm, ectasia). An additional 24% of patients displayed prominence as the only finding on their initial echocardiogram. Of the 33 patients 7 (21.2%) had coronary artery aneurysms. CONCLUSIONS: Many patients with KD have discrepancies in the degree of elevation of CRP and ESR. Physicians should consider obtaining both tests in patients with KD. This outbreak was associated with a high degree of coronary artery abnormalities. The finding of coronary artery prominence is an observation that deserves further study.

Fort Chaffee revisited: the epidemiology of tick-borne rickettsial and ehrlichial diseases at a natural focus.

A retrospective cohort study was conducted among troops training at Fort Chaffee, Arkansas, from May through June 1997, to identify infections caused by tick-borne pathogens. Serum samples were tested by IFAs for antibodies to selected Rickettsia and Ehrlichia species and by an investigational EIA for spotted fever group Rickettsia lipopolysaccharide antigens. Of 1,067 guardsmen tested, 162 (15.2%) had antibodies to one or more pathogens. Of 93 guardsmen with paired serum samples, 33 seroconverted to Rickettsia rickettsii or spotted fever group rickettsiae (SFGR) and five to Ehrlichia species. Most (84.8%) of the personnel who seroconverted to SFGR were detected only by EIA, and seropositivity was significantly associated with an illness compatible with a tick-borne disease. In addition, 34 (27%) of 126 subjects with detectable antibody titers reported a compatible illness. The primary risk factor for confirmed or probable disease was finding > 10 ticks on the body. Doxycycline use and rolling up of long sleeves were protective against seropositivity. The risk of transmission of tick-borne pathogens at Fort Chaffee remains high, and use of the broadly reactive EIA suggests that previous investigations may have underestimated the risk for infection by SFGR. Measures to prevent tick bite and associated disease may require reevaluation.

Rocky Mountain spotted fever in the United States, 1993-1996.

During 1993 through 1996, 2,313 cases of Rocky Mountain spotted fever (RMSF) were reported to the Centers for Disease Control and Prevention (CDC) by 42 states and the District of Columbia through the National Electronic Telecommunications System for Surveillance (NETSS). During this same interval, 1,752 case report forms (CRFs) were submitted to CDC and 1,253 (70%) of the cases were categorized as confirmed RMSF by laboratory testing. On the basis of analyses performed with NETSS data, the average annual RMSF incidence during 1993-1996 was 2.2 cases per million persons; the incidence rose from 1.8 in 1993 to 3.3 per million persons in 1996. Incidence for confirmed cases was highest among children 5-9 years of age (3.7 per million) and lowest among individuals older than 70 years of age (1.4 per million). The south Atlantic region accounted for the largest proportion of confirmed cases (52%). The case-fatality rate was highest among persons 70 years of age and older (9.0%) and lowest among adults 40-49 years of age (0.6%).

An investigation into the possibility of transmission of tick-borne pathogens via blood transfusion. Transfusion-Associated Tick-Borne Illness Task Force.

BACKGROUND: Tick-borne illnesses were diagnosed in a group of National Guard members, including some who had donated blood a few days before the onset of symptoms. A voluntary recall of those blood components was issued and a multistate investigation was conducted to determine if transfusion-transmitted illness had occurred. STUDY DESIGN AND METHODS: Donors and recipients were asked to complete questionnaires regarding symptoms and risk factors for infection and to provide blood samples for laboratory analysis. RESULTS: Among National Guard personnel who donated blood, 12 individuals were found to have a confirmed or probable case of Rocky Mountain spotted fever or ehrlichiosis. A total of 320 units (platelets or packed red cells) from 377 donors were transfused into 129 recipients. Although 10 recipients received units from National Guard personnel with confirmed or probable infection, none became ill. CONCLUSION: Transfusion-transmitted illness did not occur. Despite the awareness of the risk for tick-borne diseases and the use of tick-preventive measures, many National Guard personnel reported exposure to ticks. In addition to augmenting current tick-preventive measures, scheduling blood drives before rather than after field exercises could further reduce the potential for transmission of tick-borne pathogens.

Hidden mortality attributable to Rocky Mountain spotted fever: immunohistochemical detection of fatal, serologically unconfirmed disease.

Rocky Mountain spotted fever (RMSF) is the most severe tickborne infection in the United States and is a nationally notifiable disease. Since 1981, the annual case-fatality ratio for RMSF has been determined from laboratory-confirmed cases reported to the Centers for Disease Control and Prevention (CDC). Herein, a description is given of patients with fatal, serologically unconfirmed RMSF for whom a diagnosis of RMSF was established by immunohistochemical (IHC) staining of tissues obtained at autopsy. During 1996-1997, acute-phase serum and tissue samples from patients with fatal disease compatible with RMSF were tested at the CDC. As determined by indirect immunofluorescence assay, no patient serum demonstrated IgG or IgM antibodies reactive with Rickettsia rickettsii at a diagnostic titer (i.e., >/=64); however, IHC staining confirmed diagnosis of RMSF in all patients. Polymerase chain reaction validated the IHC findings for 2 patients for whom appropriate samples were available for testing. These findings suggest that dependence on serologic assays and limited use of IHC staining for confirmation of fatal RMSF results in underestimates of mortality and of case-fatality ratios for this disease.

Carcinoma of the ampulla of Vater.

Adenocarcinoma of the ampulla of Vater represents about 10% of periampullary neoplasms. This tumor is slow-growing, often amenable to radical surgical resection, and associated with a better prognosis than the other neoplasms which arise in the periampullary area. Correct identification of ampullary carcinoma may be difficult but is essential because of its better prognosis. A review of 31 patients with adenocarcinoma of the ampulla of Vater treated at our institution reveals that the classical history of fluctuating jaundice and the well known findings of a palpable gallbladder and occult blood in the stool may not be present. The treatment of choice for ampullary carcinoma in the absence of hepatic or distant metastases is pancreaticoduodenectomy. Our operative mortality for pancreaticoduodenectomy is 16%, and the five-year survival rate is 32%. The role of palliative procedures, the operative complications, and the survival rates are discussed.

Primary carcinoma of the gallbladder. The role of adjunctive therapy in its treatment.

Of forty-three patients with carcinoma of the gallbladder discovered ih a twenty-five year period (during which 10,349 patients were diagnosed as having cholelithiasis), eighteen patients (42 per cent) had no obvious tumor outside of the gallbladder at the time of operation, nineteen patients (44 per cent) had local spread of the disease, and six patients (14 per cent) had abdominal carcinomatosis or distant metastasis. In the twenty-one patients who underwent surgical therapy for cure of their disease, the five year survival rate was 33 per cent. The more extensive surgical procedures (other than cholecystectomy alone) did not significantly increase survival. Neither the duration of the symptoms nor the pathologic type of the tumor altered the eventual outcome. No patients with tumor outside the gallbladder at the time of operation survived longer than two years. Compared to those who did not receive it, the fifteen patients treated postoperatively with adjunctive therapy (radiation therapy or chemotherapy or both) lived longer and also were significantly better palliated when tumor outside of the gallbladder was found at the time of operation. From these findings, the routine use of adjunctive therapy is recommended in all patients with disease outside of the gallbladder and serious consideration should be given to its use in all patients found to have carcinoma of the gallbladder.

Gallbladder disease in children and adolescents.

Seventy-nine patients, twenty years old or younger, with cholecystitis underwent cholecystectomy during a five year period at Santa Rosa Medical Center. There was a considerable delay in diagnosis in many cases. Etiologic factors differed with race and age; however, the disease appears to be quite similar in adolescents and adults. Hemolytic disease was present in all five blacks but in none of the remaining seventy-four patients. Patients younger than ten years of age are more likely to have congenital anomalies or infectious etiologies for the gallbladder disease. Cholecystectomy was associated with minimal morbidity and no mortality in this series. Cholecystitis should be considered early in the child or adolescent with unexplained abdominal pain, and oral cholecystograms proved to be a safe and reliable method of diagnosis.

Primary tumors of the small bowel.

Primary tumors of the small bowel are uncommon, representing less than 6 per cent of all gastrointestinal tumors and less than 2 per cent of all malignant gastrointestinal tumors. This report concerns a twenty-five year survey of our clinical records from 1946 to 1971 which revealed 140 primary small bowel tumors, excluding periampullary tumors. Fifty-two of the neoplasms (37 per cent) were benign; eighty-eight (63 per cent) were malignant and included twenty-eight adenocarcinomas (31.8 per cent), twenty-four lymphosarcomas (27.3 per cent), nineteen carcinoids (21.6 per cent), and ten leiomyosarcomas (11.4 per cent). The average age at the time of diagnosis was 56.9 years for patients with benign tumors and 55.9 years for those with malignant tumors. The illusive and obscure nature of small bowel tumors is illustrated by the fact that 63.3 per cent of patients with benign lesions and 47.6 per cent of those with malignant lesions had symptoms for more than six months before the diagnosis was made. Bleeding was the most common present complaint in patients with benign neoplasms (52.9 per cent) whereas patients with malignant lesions more often had symptoms of obstruction (50.6 per cent). Most of the benign lesions were located proximally in the small bowel (duodenum, 34.6 per cent; ileum, 11.5 per cent), and most of the malignant lesions were located distally (duodenum, 17.0 per cent; ileum, 61.4 per cent). Treatment of patients with malignant lesions was radical excision whenever possible. Adjunctive radiation therapy was used for those with lymphoma. A second benign or malignant tumor occurred in 42.9 per cent of the patients with primary small bowel tumors. The average period of survival after diagnosis of a malignant small bowel tumor was 5.03 years: for patients with adenocarcinoma, 3.6 years; lymphosarcoma, 1.3 years; carcinoid, 6.8 years; and leiomyosarcoma, 8.3 years.