Liver transplant for giant cell hepatitis with autoimmune haemolytic anaemia

Giant cell hepatitis (CGH) with autoimmune haemolytic anaemia (AHA) is a distinct entity with an aggressive course. Immunosuppression may help early disease. A case is reported of a child with GCH and AHA with early disease recurrence after liver transplantation for end stage liver disease.     

Liver transplantation for homozygous familial hypercholesterolaemia

Homozygous familial hypercholesterolaemia is a rare inherited condition with an incidence of approximately one in a million. It is associated with severe premature atherosclerosis and early death from cardiovascular complications. The results of liver transplantation reported to date have suggested only partially effective reduction of the hypercholesterolaemia. Three boys with familial hypercholesterolaemia, aged 10.0 to 15.1 years, received liver grafts at Addenbrooke's Hospital. Their untreated fasting lipid concentrations were grossly raised. All three had angiographic evidence of coronary atheroma and two had exertional angina. One child had such severe atheroma that coronary artery bypass surgery was considered necessary before liver transplantation. All three had straightforward operative and postoperative courses and their lipid concentrations returned rapidly to normal. One boy developed chronic rejection requiring retransplantation. Currently all three boys are well, on normal diets, and with normal liver function. It is concluded that (1) liver transplantation offers highly effective treatment for this lethal condition, (2) timing the operation is difficult but it should be undertaken before coronary artery disease has progressed too far (when combined liver and heart transplantation may be the only possibility), and (3) in well grown children with no previous abdominal surgery the immediate risks of liver transplantation are low but chronic rejection remains a danger.     

Stapedectomy and vertigo

The relationship between vestibular and cochlear function in Stapedectomy was investigated in 722 patients (925 ears). There were no differences in hearing between patients with and without pre-operative complaints of vertigo. Post-operatively patients with long lasting vertigo obtained equally good hearing results as the others. The direction of spontaneous nystagmus post-operatively in relation to the operated ear was of no prognostic significance regarding short term hearing results. However, at follow-up an average of 15 years after the operation, hearing was somewhat poorer in those having spontaneous nystagmus towards the operated ear. At follow-up 17% had an abnormal caloric test.     

Spontaneous osteonecrosis of the knee and medial meniscal tears

Several factors may play a role in the etiology of "spontaneous" osteonecrosis of the medial femoral condyle. Corticosteroids are known to induce osteonecrosis, and 45% of the patients in this study received steroids parenterally or by intra-articular injection. Another factor, heretofore given little attention, is the association of medial meniscal tears and "spontaneous" osteonecrosis. Twenty-one (78%) of 27 knees examined by arthrography demonstrated meniscal tears. Stress concentration over the edge of the meniscal fragment may result in ischemic necrosis of the femoral condyle. Early detection of a medial meniscal tear by arthrography in older patients and prompt treatment may be important in avoiding the late changes of "spontaneous" osteonecrosis.     

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In summary, the naturopathic approach would treat this patient with dietary modification, nutritional and herbal supplements, regular preventive nasal irrigation, and adjunctive therapies such as homeopathy and osteopathic manipulation as indicated. Hopefully, this preventive approach will reduce or eliminate the need for the use of antibiotics to treat the acute exacerbations of what is really a chronic, lifestyle-related condition. As illustrated here, the Naturopathic Therapeutic Order can both guide us in treating a specific condition and assist us in assessing our patients on an individual, prevention-oriented basis.     

Site-directed mutagenesis of the human D antigen: definition of D epitopes on the sixth external domain of the D protein expressed on K562 cells

BACKGROUND: The antigens of the human Rh system are of great clinical significance in transfusion medicine and pregnancy. Of the Rh system antigens, D is clinically the most important, being one of the most immunogenic structures arising from human cells. The human D antigen represents a collection of epitopes expressed on a red cell membrane protein that is predicted to have 12 membrane-spanning segments giving rise to six exofacial domains. STUDY DESIGN AND METHODS: By site-directed mutagenesis using the method of inverse polymerase chain reaction, cE and D cDNA mutant constructs were generated with changes to the RHD-specific residues 350, 353, and 354 in the predicted sixth exofacial loop. Each mutant cDNA was subcloned into the pBabe puromycin retroviral vector, and supernatants were used to transduce K562 cells. Puromycin-resistant K562 clones were screened by flow cytometric analysis using a panel of monoclonal antibodies with specificities to ep (epitope) D1 through epD9. RESULTS: De novo expression of epD3 and epD9 was generated in the K562 cell lines expressing the mutated cE polypeptide (cE-Asp350His, Gly353Trp, Ala354Asn). Expression of c and E was unaffected. Conversely, the cells expressing the mutated D polypeptide demonstrated loss of expression of epD1, epD2, epD3, epD4, and epD9. CONCLUSION: The data provide strong evidence for the critical involvement of three amino acids, Asp₃₅₀, Gly₃₅₃, and Ala₃₅₄, in the expression of epD3 and epD9 on the predicted sixth external domain of the D protein. This domain also appears to be essential for the expression of epD1, epD2, and epD4, as a loss of expression of these epitopes was observed in K562 cells transduced with the D_mut construct (encoding His₃₅₀, Trp₃₅₃, and Asn₃₅₄). The K562/D_mut, cell line has an identical molecular and serologic profile as the red cell D^IVb pheno-type, which confirms that retroviral gene transfer of Rh cDNA into K562 cells provides us with a powerful means by which to further map epitopes of D.     

Nasopharyngeal carcinoma: correlation of histopathology with radiation response

Forty-nine cases of NPC were evaluated histopathologically and the radiation responses of the tumor types were assessed. The overall radiation response were as follows: CR 40.8%, PR 14.3%, NC 42.9%, PD 2.0%. The distribution of tumor types were: Squamous cell carcinoma (WHO type I) 4.08%; Non-keratinizing carcinoma (WHO type II) 4.08%; Undifferentiated carcinoma (WHO type III) 91.84% var. Lympho-epitheliomatous (LE), Anaplastic (A), Spindle cell (SC), Clear cell (CC). 94.38% of the cases were classified as clinical stage II, III and IV (tumor found beyond the nasopharyngeal space). The radiation response rate of the tumor in the lymphnode was reversed to the clinical stage and extent of node involvement, namely the higher stage and node involvement, the less rate of radiation response. Twelve cases received radiation dose of less than 4000 cGy, which comprised 11 NC cases and 1 PD case. Of the 37 cases who got 4000 cGy and over, the response rate was 72.97%. Of these WHO type I was not evaluated because of insufficient radiation dose; WHO type II showed 50% response rate; WHO type III showed 74.29% response rate. Radiation response (as assessed from the cervical lymphnode involvement) of NPC was influenced by tumor burden and histologic type of the tumor, namely the less differentiated tumor responded better than the more differentiated one. No definite conclusion can be drawn yet of the different behaviour (if any) of the variants of undifferentiated carcinoma (WHO type III) toward irradiation.     

Some experience with in vivo staining of early laryngeal cancer in Jakarta, Indonesia

Laryngeal cancer, clinically as well as pathologically, usually does not pose a difficult problem in diagnosis. The problem lies in the clinical diagnosis of early laryngeal cancer. We began to study and develop the in vivo staining technique for laryngeal lesions in our clinic, using toluidine blue, an acidophilic metachromatic dye. This preliminary study comprised eight cases of laryngeal lesions, five of which were carcinoma. Among these latter cases, besides carcinoma, we were able to detect dysplasia in two cases, carcinoma in situ in one case, and a false-positive area in one case. Noncarcinoma cases consisted of dysplasia, dysplasia in postradiation for carcinoma, and carcinoma in situ. In two cases of carcinoma, the tumor margin was accurately defined by positive staining area. This experience suggests that in vivo staining may be a useful method for early detection of laryngeal cancer used as an adjunct to microlaryngoscopy.