Cytomegalovirus (CMV) infection poses a serious threat to immunocompromised patients in early life. Transmission of CMV to newborn via breastfeeding is a recognized mode. In our case, the affected child presented with the aftermaths of CMV infection affecting gastrointestinal tract (GIT) and central nervous system (CNS) with over-whelming viral replication and via ingestion of infected breast milk many times a day. Worsening clinical manifestations prompted the investigations for a possible primary immune deficiency (PID) disorder, and rising CMV copies in blood despite institution of anti-CMV therapy led to check other body fluids of the infant. There were no viral copies detected in mother’s blood sample; so breast milk was tested to identify the source of CMV being transmitted to him. Here we describe an unusual case of RAG2 gene mutation induced PID presenting clinically as severe combined immunodeficiency (SCID) phenotype rather than Ommen’s syndrome, and acquired CMV infection via breast milk.

Case report 3

Pathology and genetics of tumors of soft tissue and bone

This study of 195 patients with giant-cell tumor reemphasizes the female predominance, the predilection for the region of the knee, and the extreme rarity of the tumor in patients with immature skeletons. One of the tumors occurred in a patient with Paget's disease of bone. There were 2 patients with 2 giant-cell tumors each. Surgical treatment short of complete resection or amputation was followed by recurrence of benign giant-cell tumor in 44.6% of cases. Adjunctive radiation or cautery did not decrease this rate. Primary en bloc resection or amputation was uniformly curative. Primary radiation therapy had very limited value. Sarcomas developed in 17 patients (8.7%) of the total; these lesions we have called malignant giant-cell tumors. In 4 instances, zones of anaplastic sarcoma were found in otherwise typical giant-cell tumors. The remaining 13 sarcomas were found in recurrent tumors, and in 11 of these, the primary treatment had included radiation. The evidence indicates that surgical removal is the best treatment and that radiation should be employed only for tumors that are unresectable because of their location.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/1097-0142%28197005%2925%3A5%3C1061%3A%3AAID-CNCR2820250509%3E3.0.CO%3B2-E

Giant-cell tumor: a study of 195 cases.

Sixty-six cases of aneurysmal bone cyst were reviewed clinically, pathologically and radiologically. Different therapies, including curettage, block excision, amputation and radiation therapy, and a new treatment, cryosurgery, were employed. Cryosurgery, which avoids the growth and neoplastic complications of radiation therapy and the loss of bone with block excision, had an extremely low rate of recurrence when compared to simple curettage. In manometric studies of 6 cysts, 3 had elevated vascular pressures as high as arteriolar levels. Thirty-two percent of the aneurysmal bone cysts had an accompanying benign primary lesion of bone. Based on these findings, a new hypothesis for the etiology and pathogenesis of aneurysmal bone cysts was proposed: a primary lesion of bone initiates an osseous, arteriovenous fistula and thereby creates, via its hemodynamic forces, the secondary reactive lesion of bone, which we term aneurysmal bone cyst.

https://acsjournals.onlinelibrary.wiley.com/doi/epdf/10.1002/1097-0142%28197009%2926%3A3%3C615%3A%3AAID-CNCR2820260319%3E3.0.CO%3B2-I

Aneurysmal bone cysts. A clinicopathologic study of 66 cases

collected in the literature. Most of the descriptions of sarcomas in fibrous dysplasia that are in the literature are single case reports or reports of a few case^.^-'^ The largest series, which includes 15 cases, is that from Memorial Sloan-Kettering Cancer Center, New York.2,20 In 1988, Yabut and coworkers' reported a new case and found 83 cases in a careful review of the literature. The most common of the malignancies was osteosarcoma, followed by fibrosarcoma and chondrosarcoma. The possibility of sarcomatous change in fibrous dysplasia without prior irradiation seems well established, as verified by several reports of documented case~.~~~~'' The role of radiation therapy in the occurrence of sarcoma in fibrous dysplasia is still c~ntroversial.'~~~~' In Huvos' series," only 1 of 15 patients with sarcomas in fibrous dysplasia had received radiation therapy. In the review of the literature by Yabut and coworker^,^ 23 patients had received radiation therapy, 46 had not had prior irradiation, and 14 did not have information available. The purpose of the current study was to review and report our experience with malignancies in fibrous dysplasia. Materials and Methods

Malignancies in fibrous dysplasia.

Tumors and tumorous conditions of the bones and joints

Early detection and diagnosis of cancer.

Smoking and your life.

The administration of alcohol did not appear to have .any adverseeffect on the clinical condition of the patients over the period of the observations,which was ten to thirty-two days. All the subjectsshowedmarkedanorexia at the beginning of the observation period, but after a few days with alcohol the appetite.improved greatly, the patients felt better, and some gained weight. There was no evidence that alcohol consumptionhad any significant effect on nitrogen balance. Liver function tests showed no deteriorationduring the period of the experiment. In most of the patients the hepatic histological condition, as seen in biopsy specimens,was better at the end than at the beginning of the experiment. Alcohol did not impede the cure of peripheralneuritis where this was present.

The solitary pulmonary nodule

diseases, it has chapters on other venereally acquired infections, the non-venereal treponematoses and other nonvenereal diseases of the genitals. An appendix gives details for the routine investigation of new patients, together with descriptions of the most commonly used diagnostic techniques. The numerous plates, in colour and monochrome, have been well chosen and reproduced. There are no end-ofchapter references for further reading. Only two typographical errors were noticed: "Wassermann" is misspelt in the historical introduction to syphilis, and the chapter "Public Health Control" is incorrectly indexed. Practitioners in this country will find that some details in the recommendations for treatment of syphilis and gonorrhcea are somewhat different from current local practice, and one of the recommended penicillin preparations, P.A.M., has been unobtainable in Australia for several years. Although glandular fever is mentioned as one of the diseases likely to give a false-positive serological reaction, this disease is not listed in the differential diagnosis of secondary syphilis. Induratio penis plastica (Peyronie's disease) is uncommon, but not rare, and could have been included in the chapter on other lesions of the genitalia. The technique of urethro-vesical irrigations is given in the appendix, but their use as adjuvant therapy is not mentioned in the treatment of non-gonococcal urethritis. Like other countries, Australia has in recent years experienced an increased incidence of gonorrhoea and syphilis. General practitioners are seeing more and more patients suffering from these diseases, and this textbook is warmly recommended as an excellent guide to their management. It is even more particularly recommended to medical students, whose tuition in venereal diseases is almost completely neglected at some Australian teaching hospitals.

L. Henry Garland

Papers

Tumors and tumorous conditions of the bones and joints

Early detection and diagnosis of cancer.

Smoking and your life.

The solitary pulmonary nodule

THE FUNDAMENTAL IDEAS OF MEDICINE: A Brief History of Medicine