Burton J. Grossman

Social factors in relation to participation in follow-up care of rheumatic fever

One hundred twenty-three patients with rheumatic fever were studied as to the medical and social factors which influenced participation in a program of follow-up care. Three general factors were associated with the quality of cooperation: (1) social circumstances, (2) illness of others in the family which interferes markedly with good participation, and (3) quality of interpersonal relations which showed that good interpersonal relations within the family tend to predict good cooperation and that problems and interpersonal conflict tend to predict the reverse. Identification of these factors helps focus attention on the problem early in the contact with the patient.

Bone and Joint Changes following Burn Injury

Received for publication April, I~

Congenital afibrinogenemia; report on a newborn infant without fibrinogen.

l; accepted June, 1981. ’ Edators’Nate; This article reports an important and serious complication of burns in children. One editorial reviewer of the article writes, &dquo;These rather catastrophic complications of what should have been an easily managed burn in a I-year-old child point out the need for team management in an active burn center. Although there is no proof, the article suggests and I personally believe that these complications might have been avoided by early mobilization and active move-

RESPIRATORY TRACT INFECTIONS: Chronic Polymicrobial Bacteremia

Summary 1. A case of congenital afibrinogenemia in a newborn infant is presented. 2. The finding of a possible reduction in fibrinogen in the father and two brothers of the patient is reported. 3. The use of clotting techniques as a sensitive means of detecting fibrinogen deficits is discussed.

Independence of Protamine Titration and Platelet Level in Certain Hemorrhagic Diseases.

A 13-year-old girl had chronic polymicrobial bacteremia with Enterobacter hafnia and Enterobacter agglomerans in the absence of any demonstrable underlying illness, use of immunosuppressive drugs, or discovery of portal of entry of the bacteria. She was treated successfully with a prolonged course of carbenicillin and aminoglycoside antibiotics which were tolerated well. The only side effect of the therapy was a transient episode of vestibular dysfunction which was reversible following cessation of gentamicin. The principles of management of polymicrobial bacteremia are presented.

Tutoring and Ventilation: A Pilot Study of Reactions of Hospitalized Children

Summary 1. The platelet concentration influences the sensitivity of bloods and plasmas to the effect of added heparin. The anticoagulant potency of heparin is also enhanced by prothrombin deficiency. The potentiated effect of heparin in thrombocytopenic bloods may be due to reduced thromboplastin (cephalin) activity. 2. The protamine titration may be increased when the platelet count is normal and it may be normal in the presence of thrombocytopenia. It is influenced by heparin, heparinoid substances, prothrombin deficiency, hemophilia, and possibly other factors. 3. Increased protamine titrations may occur in bleeding patients who have no other apparent clotting defects, including the whole blood clotting time. Many of these patients cease bleeding when given intravenous toluidine blue. The protamine titration under these conditions can be returned to or toward normal regardless of the platelet level.

Rheumatology and Immunology

them recommend procedures that may be adopted to reduce the probability of psychologic trauma. Among these procedures, communication with a child patient at an appropriate level about his illness and about hospital procedures is generally included. &dquo;Communication&dquo; in this context is often taken to mean both the process of imparting information to the child and the process of facilitating the expression, by the child, of his principal concerns. Indeed, it is difficult to conceive of doing the former appropriately without doing the latter either previously or simultaneously. The process of imparting information, or simply tutoring the child about his illness and its implications, and the process of permitting the child to express himself, or to ventilate,

SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) AND DERMATITIS HERPETIFORMIS (OH) IN A GIRL WITH MARFAN'S SYNDROME

The list of contributors to this handsome 8 1/2×11-in volume reads like a Whos Who of the leading authorities in rheumatology and immunology. The text is well supplied with tables, charts, photomicrographs, roentgenograms, and schematic drawings complementing well-written, well-researched presentations on a wide range of subjects in the fields of rheumatology and immunology. The rheumatology section provides a systematic approach to the basic subject matter with major headings on regional structure and function, diagnostic procedures, differential approach to major rheumatic syndromes, and specific articular and connective-tissue diseases. The immunology section is divided into the following categories: concepts and diagnostic procedures, differential approach to major immunologic syndromes, and specific immunologic diseases. Each subsection, within the major sections, is written by an authority or authorities in the specific field and provides a small number of the basic references on the subject. A fine example of the clarity and excellent organization of material

ANTICONVULSANT-INDUCED SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) AND NEPHROTIC SYNDROME (MS)

The concurrence of DH and SLE has previously been reported in 2 patients. Our patient is a 15 year old black girl with Marfans Syndrome who developed a bultous eruption which was clinically, histologically, and immunopathologically characteristic of DH. Sulfapyridine was given. Two months later she developed fever, pleural effusion, renal insufficiency, nephrotic syndrome, hypertension, hypocomplementemia, positive ANIF and positive LE preparation. Immunofluorescence of uninvolved skin demonstrated IgG, IgM and C3 at dermal-epidermal junction and around papillary blood vessels. Kidney biopsy showed Type III (proliferation with deposits) lupus glomerulonephritis. Prednisone treatment resulted in improvement of clinical manifestations. She subsequently expired when cystic medial necrosis of the ascending aorta resulted in cardiac tamponade. Since both DH and SLE are diseases mediated by the deposition of immune complexes, our patient clearly exhibited a rare predisposition to formation of immune complexes. The presence of two immune-mediated diseases in the same patient suggests a common underlying immune defect.

RENAL HISTOPATHOLGY AND CLINICAL COURSE OF 53 CHILDREN WITH SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

A 3 year old boy was treated with diphenylhydantoin (DPH), ethosuximide (E), and phenobarbital for convulsions following herpes encephalitis. After 3 months of anticonvulsant treatment he developed NS (24 hour uriae protein-9.1 grams, serum albumin-0.76 gms.%, cholesterol-356 mg.%), positive ANIF (1:800), “suspiciously” positive LE preparation, positive skin biopsy (dermalepidermal IgG), and high total eosinophil count (985 - nl 50-250)., After withdrawal of DPH and E there was gradual resolution of NS and normalization of urine, serum protein, cholesterol, ANIF and eosinophilla. Renal function and serum complement levels remained normal. Corticosteroids were not given.Although anticonvulsants have been associated with SLE-like syndromes, serologic abnormalities, nephritis or NS, no previously reported patient developed SLE and NS concurrently. The urgency in discontinuing DPH and E prevented our identifying the offending drug. It is likely that this patient sustained a syndrome of drug-induced SLE with immune-mediated reversible glomerulonephritis and NS.