Paul E. Hermans

Dysgammaglobulinemia associated with nodular lymphoid hyperplasia of the small intestine

Abstract A syndrome, noted in eight patients, is described. It is characterized by (1) dysgammaglobulinemia, consisting of virtual absence of the IgA and IgM immunoglobulins and a moderately decreased level of IgG immunoglobulin in the serum; (2) an unusual susceptibility to infections; (3) diarrhea, sometimes with steatorrheal features; (4) the presence of Giardia lamblia in the stools; and (5) nodular lymphoid hyperplasia of the small intestine. This combination of features allowed differentiation from gluten-sensitive idiopathic sprue and from acquired idiopathic hypogammaglobulinemia. The screening of patients with sprue-like syndromes also resulted in the detection of two patients with isolated IgA immunoglobulin deficiency. The nodular lymphocytic hyperplasia which may occur in association with dysgammaglobulinemia may be related to functional abnormalities of the so-called central lymphoid tissues. Of practical clinical importance are (1) the possibility of a presumptive diagnosis by roentgenographic studies which may show the nodules in the small intestine in most cases and (2) the suggestion that, in at least some of the patients, remission of the diarrhea may be achieved by the use of tetracycline. Also of clinical significance and of theoretical interest is an increased incidence of carcinoma of the gastrointestinal tract in patients with dysgammaglobulinemia and nodular lymphoid hyperplasia of the small intestine.

Incidence, distribution, and outcome of episodes of infection in 100 orthotopic liver transplantations

Of 83 patients who underwent 100 orthotopic liver transplantations, 53 had a single transplant procedure and at least 6 months of follow-up. In this main study group of 53 patients, major infections developed in 28 (53%) (a mean of 1.8 major episodes per infected patient). Of 51 major infections, 27 were bacterial, 19 were viral, 3 were protozoan, and 2 were fungal. Of the 27 bacterial infections, 22 (81%) occurred in the first 2 months after transplantation. Of the 40 bacterial isolates in the 27 bacterial infections, gram-positive aerobic bacteria were isolated in 26 (65%), anaerobic bacteria in 8 (20%), and aerobic gram-negative bacteria in 6 (15%). Only 1 of 16 bacteremic episodes was due to a gram-negative aerobic bacterium. Cytomegalovirus (CMV) infection occurred in 30 of the 53 patients (57%) and was symptomatic and invasive in 18. CMV infection was diagnosed a mean of 26 days after transplantation. Infections due to Pneumocystis carinii occurred later (2 to 3 months after transplantation). Death from infection occurred in 4 of the 53 patients (8%). In the group of 16 patients with two or more liver transplantations, fungal infection occurred in 2 and CMV infection in 13. In all 16 patients who underwent more than one liver transplantation, a major infection developed. The observations made in the main study group were consistent with findings in 13 patients with one liver transplantation but less than 6 months of follow-up. Infection is a major complication after liver transplantation, generally occurring in the first 2 months. Our observations suggest that the use of selective bowel decontamination may be associated with a relatively lower incidence of gram-negative aerobic bacterial infections.

Chronic mucocutaneous candidiasis as a surface expression of deep-seated abnormalities: Report of a syndrome of superficial candidiasis, absence of delayed hyper sensitivity and aminoaciduria

Abstract Three patients with chronic mucocutaneous candidiasis have been studied. In the first patient, who also had an extensive skin infection due to Trichophyton rubrum, aminoaciduria and absence of delayed hypersensitivity to test antigens including oidiomycin were found. The coexistence of aminoaciduria and an immunologie abnormality has not been reported previously in a patient with chronic superficial candidiasis. The second patient had the following abnormalities: candidiasis, primary Addisons disease, enlarged thymus, miniscule spleen devoid of lymphoid tissue and lymphocytic infiltration of the thyroid gland. The third patient represents an example of the rare syndrome of familial juvenile keratoconjunctivitis, primary hypoparathyroidism, primary Addisons disease and superficial candidiasis. The patient who was lymphopenic lacked the addisonian component of the syndrome but had a sister who had the combination of hypoparathyroidism. Addisons disease and ovarian failure without associated candidiasis. Conditions favoring the development and persistence of chronic mucocutaneous candidiasis are discussed, with a review of the pertinent literature.

Infections due to group C streptococci in man

Although a common cause of infection in animals, group C streptococci are rarely noted to be pathogenic in man. A total of 150,000 blood cultures obtained at the Mayo Clinic from 1968 to 1977 revealed group C streptococci in only eight patients. Acute bacterial endocarditis, meningitis, pheumonia, cellulitis and bacteremia due to group C streptococci are described in a host who had undergone immunosuppression (immunosuppressed host), and the relatively few cases previously reported are reviewed. Although severe, these infections may respond favorably to penicillin therapy. Endocarditis caused by group D streptococci is acute and destructive, and associated with early cardiac decompensation. The manifestations of cellulitis and pneumonia are similar to those when group A streptococci are causative organisms. Meningitis due to group C streptococci is acute and severe, and responds slowly to antimicrobial therapy. Colonization also occurs.

Rapid Shell Vial Culture and Tissue Histology Compared With Serology for the Rapid Diagnosis of Cytomegalovirus Infection in Liver Transplantation

Sixty-six patients who had undergone 78 liver transplantations, with no detectable cytomegalovirus (CMV) infection before transplantation, were studied to assess the value of CMV serology compared with the rapid detection of the virus in shell vial cell cultures or histology for the diagnosis of (1) the first evidence of infection, (2) symptomatic disease, and (3) asymptomatic infection. Of 28 evaluable patients, shell vial cell culture assay and histologic findings provided the first evidence of CMV infection earlier in 22 (79%) or at the same time as the serologic response in 5 (18%). Serologic results yielded the first indication of CMV infection in only 1 of these 28 patients (3%). Of 17 evaluable transplantations in 15 patients in whom symptomatic invasive disease developed, shell vial culture or histology provided a laboratory diagnosis of CMV infection earlier or at the same time as serologic responses in 16 (94%). Similarly, shell vial culture or histology provided evidence of CMV infection in 10 of 11 patients (91%) who had asymptomatic CMV infection and remained asymptomatic. Urine surveillance cultures yielded the first evidence of CMV infection in 14 of 19 patients who initially had an asymptomatic infection, of which 6 became symptomatic. Culturing of the blood with use of the rapid shell vial technique showed that viremia preceded CMV organ involvement in 7 of 10 patients. We recommend culture by the shell vial assay as the most rapid and sensitive method of determining CMV infection in liver transplant patients.

Association of gastric carcinoma with idiopathic late-onset immunoglobulin deficiency.

Abstract Among 39 patients with idiopathic late-onset immunoglobulin deficiency (LID) seen at the Mayo Clinic in the past 10 years, 3 had a concomitant carcinomatous gastric lesion (incidence, 7.7%...

Prevalence of Cholelithiasis in Idiopathic Late-Onset Immunoglobulin Deficiency

Twelve of 50 consecutive patients with idiopathic late-onset immunoglobulin deficiency had cholelithiasis. This represents a prevalence rate of 24%, which is significantly above the rate in a control group of 500 patients from this medical center matched 10:1 for age and sex (P less than 0.005). This finding adds a new gastrointestinal abnormality to those known to occur in idiopathic late-onset immunoglobulin deficiency.

Empiric Therapy With Moxalactam Alone in Patients With Bacteremia

Moxalactam was administered (20 mg/kg intravenously every 8 hours) as single-drug empiric antimicrobial therapy to 63 patients with bacteremia who were neither neutropenic nor immunosuppressed. Six patients (10%) had microorganisms that were susceptible to moxalactam and resistant to all other antimicrobial agents tested; two patients (3%) had microorganisms that were resistant to moxalactam and other agents tested. Of these 63 patients, 47 (75%) were cured with moxalactam therapy. Nine patients (14%) had breakthrough bacteremia while receiving other antimicrobial therapy and were cured subsequently with moxalactam therapy alone. The two major risk factors for failure of moxalactam therapy were polymicrobial bacteremia and an extrahepatic intra-abdominal source of infection; these two conditions frequently coexisted. Six of nine patients with polymicrobial bacteremia died. Superinfection (one pseudomonal, five enterococcal) was responsible for 6 of the 16 treatment failures. Enterococcal superinfection occurred exclusively among patients who had received relatively prolonged therapy with moxalactam for extrahepatic intra-abdominal infection, especially intraabdominal abscess. These five patients died, and postmortem examination showed that enterococcal superinfection was the major cause of death in all. Mild, reversible adverse reactions associated with use of moxalactam occurred in 14 of the 63 patients (22%). None had clinically overt bleeding. The use of moxalactam alone seems to be safe and effective and a cost-effective alternative empiric antimicrobial therapy for most patients with bacteremia who are not immunosuppressed or neutropenic and who are not at high risk of having Pseudomonas or polymicrobial bacteremia.