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PubMed1994

Leiomyosarcoma with prominent osteoclast-like giant cells. Analysis of eight cases closely mimicking the so-called giant cell variant of malignant fibrous histiocytoma.

Eight cases of leiomyosarcoma with osteoclast-like giant cells, arising in deep soft tissue, and that mimicked closely the "giant cell variant of malignant fibrous histiocytoma (MFH)," have been studied morphologically and immunohistochemically. The age of the patients ranged from 7 to 88 years (mean, 66.2 years; median, 74 years); five were female patients. Three lesions arose in the lower limbs, two in the buttock, and one each in the shoulder, chest wall, and the floor of the mouth. Follow-up in one case revealed a local recurrence and in two cases systemic metastases. All cases showed, at least focally, interwoven spindle cell fascicles, with the cytologic features of smooth muscle cells, as well as strong positivity for alpha-smooth-muscle actin, muscle actin, and desmin. The morphologically benign osteoclast-like giant cells expressed CD68 but failed to stain with myogenic markers. The association of leiomyosarcoma with prominent osteoclast-like giant cells is not as uncommon as generally believed, being evident in 8.7% of the deep-seated nonvisceral leiomyosarcomas that we have studied. These results provide good evidence for myogenic differentiation in at least a subset of those tumors with morphologic features currently classified as the giant cell variant of MFH. Considering that at least some other reported cases of giant cell MFH appear to be a variant of extraskeletal osteosarcoma, we would suggest that lesions with this distinctive pattern should be more carefully classified according to their apparent line of differentiation.

Aged↗
PubMed1977

[Clinical picture and course of early recurrent schizophrenia at a late stage of the disease].

The clinico-follow-up study of 90 patients with recurrent schizophrenia with an early onset (up to 20 years) permitted to depict peculiarities of the clinical picture and the course at a remote period of the disease depending upon the clinical manifestations of the previous stage and the degree of the progressiveness. An analysis of the clinical material has demonstrated that periodic schizophrenic with an early onset contains, as well as in adults, various variants of the course that differ in the degree of progressiveness (mild cyclothimuc, reduced, "cliche" and complicated states) which most likely condition a different level of "outcome": from the mild ones on the level of psychic asthenia up to more expressed in the form of personality autization of a different degree. The stated negative symptomatology was determined on the background of asthenical remission of a combined character (affective and asthenical disorders).

Affective Symptoms↗
PubMed1991

Antithrombin Glasgow II: alanine 382 to threonine mutation in the serpin P12 position, resulting in a substrate reaction with thrombin.

A female with recurrent thrombosis was found to have a functional abnormality of antithrombin, with a ratio of functional to immunological activity in plasma of approximately 50%. Crossed immunoelectrophoresis in the presence of heparin was normal, indicating an abnormality of the reactive site, rather than the heparin binding domain. Accordingly, the antithrombin was isolated by heparin-Sepharose chromatography: this produced a mixture of normal and variant antithrombin, as the patient was heterozygous for the abnormality. To remove the normal component, the antithrombin was passed through a column of thrombin-Sepharose. On sodium dodecyl sulphate polyacrylamide gel electrophoresis (SDS-PAGE), prior to its application to thrombin-Sepharose, the antithrombin migrated as a single band with identical mobility to that of normal antithrombin. After thrombin-Sepharose, the purified variant component was proteolysed, and migrated as two components, one with a reduced and one with enhanced mobility under non-reducing conditions. This demonstrated that the variant was unable to form stable inhibitor-thrombin complexes and was cleaved in a substrate reaction with thrombin. One site of cleavage was unambiguously ascertained to be the Arg 393-Ser 394 reactive site bond, by NH2 terminal sequencing of the cleaved variant antithrombin: 10 steps beginning at the P1' position, Ser-Leu-Asn-Pro-Asn-Arg,..., were clearly identified. The mutation responsible for this defect was studied by polymerase chain reaction (PCR) amplification of exon 6 of the antithrombin gene and direct sequencing of the amplified product. The presence of both a G and A in the first position of codon 382, identified the mutation GCA to ACA, which results in the substitution of Ala 382 to Thr. This is identical to that reported for antithrombin Hamilton (Devraj-Kizuk et al, 1988), although antithrombin gene polymorphism analysis suggests that the antithrombin Glasgow II mutation has arisen independently. We have recently shown (Caso et al, 1991) that mutation at a nearby position, Ala 384 to Pro, also transforms another variant, antithrombin Vicenza/Charleville, into a substrate for thrombin. The present results with antithrombin Glasgow II suggest that all the alanine residues at the base of the reactive site loop in positions P12-10 may be important for the formation of a stabilized inhibitor-thrombin complex.

Adult↗
PubMed1992

Survey of 1120 Japanese women with a history of recurrent spontaneous abortions.

Our recent 7-year clinical survey showed that among the 1120 women with repeated spontaneous abortions registered in this clinic, 2898 out of a total of 3216 pregnancies (90.1%) had terminated in spontaneous abortion. Among these wastages, 84.2% occurred before 12 weeks of gestation, and 11.1 percent occurred between 12 and 15 weeks. Through routine examination of reproductive wastage, 82 (9.9%) of the 825 Japanese couples examined were shown to have either a chromosomal abnormality or normal variants in the wife and/or husband, thus demonstrating no racial difference in the incidence of chromosomal abnormalities in infertile patients in comparison with studies performed in other countries. One hundred and forty-seven congenital uterine anomalies (14.7%) were found in 1000 hysterosalpingographies, and 12 of 148 examined females were positive for anti-cardiolipin antibody. 393 other females with no major abnormalities likely to induce spontaneous abortions were indicated for immunotherapy. Ample time spent on genetic counseling prevented further reproductive wastage, and ideal metroplasty resulted in a successful post-operative pregnancy course in more than 85% of cases. Immunosuppressant and anticoagulant therapy decreased the serum titer of anti-cardiolipin antibody, enabling pregnancies to be maintained to term. Immunotherapy utilizing the husband's lymphocytes also brought more than 80% of pregnancies to successful completion, with 200 deliveries achieved with this therapy. In contrast, 64.1% of pregnancies again terminated spontaneously in patients who were indicated for immunotherapy but did not receive treatment. The findings of the present study suggest that the causes of reproductive wastage, especially the etiology of early recurrent spontaneous abortion, are complex.(ABSTRACT TRUNCATED AT 250 WORDS)

Abortion, Habitual↗
PubMed2004

Decreased pain threshold in children with growing pains.

OBJECTIVE: To investigate whether children with recurrent musculoskeletal pain termed growing pains (GP) have lower pain thresholds than children without GP. METHODS: We measured the pain threshold of 44 children with GP and 46 controls. Pain thresholds were measured by use of a Fisher type dolorimeter with pressure applied to areas associated with increased tenderness in fibromyalgia (FM), control points, and anterior tibia, the usual region of pain in children with GP. Unpaired Student's t test and chi-square tests were used to compare the pain threshold and number of tender points in patients and controls. RESULTS: The pain threshold in characteristic tender points of FM, control points, and anterior tibia in the children with GP was significantly lower in children with GP (3.5 +/- 0.6 kg/cm2 in GP versus 4.0 +/- 0.7 in controls, p < 0.001, 3.8 +/- 0.7 versus 4.4 +/- 0.8, p = 0.005; 5.1 +/- 1.1 versus 5.9 +/- 1.5, p = 0.004). Children with GP had a significantly greater number of tender points in response to an applied pressure of 4 kg/cm2. CONCLUSION: Children with GP have more tender points and lower pain thresholds than children without GP indicating that GP may represent a variant of a noninflammatory pain syndrome in younger children.

Child↗
PubMed2003

The variant form of hairy-cell leukaemia.

Hairy-cell leukaemia-variant (HCL-variant) is a rare B-cell disorder which accounts for 10% of HCL cases. It affects elderly or middle-aged males. The main features are splenomegaly, lymphocytosis and cytopenias without monocytopenia. The circulating cells have a morphology intermediate between prolymphocytes and hairy cells. The immunophenotype shows a mature B-cell phenotype with expression of the B-cell antigens CD11c and CD103-but unlike typical HCL the cells are CD25- and HC2-negative. The histology of bone marrow and spleen shows a pattern of infiltration similar to that in HCL. There is no recurrent chromosomal abnormality but complex karyotypes and monoallelic p53 deletion by fluorescence in situ hybridization are common. Patients are resistant to alkylating agents and interferon-alpha (IFN-alpha) and only half achieve partial responses to pentostatin and/or cladribine. Splenectomy results in long-lasting partial responses in over two-thirds of the patients and is a good palliative treatment. Despite the lack of response to most therapies, the clinical course of HCL-variant is chronic. The median survival is 9 years and 42% of patients die of unrelated causes. Transformation to large cell is seen in 6% of patients. The inferior survival in HCL-variant compared with typical HCL cases may reflect the chemotherapy resistance.

Antineoplastic Agents↗
PubMed2004

Mannan-binding lectin insufficiency in children with recurrent infections of the respiratory system.

Blood samples were collected over a 4-year period from 335 children (aged 1-16 years) suffering from recurrent respiratory infections and 78 controls. The patients were subdivided into four groups: I, children with no immune system defects detected (n = 101); II, children with allergies (n = 94); III, children with humoral response defects (n = 93); and IV, children with disturbances of cellular immunity (n = 66). Nineteen patients had both humoral and cellular abnormalities. All patients and controls were investigated to determine the exon 1 and promoter region variants of the mbl-2 gene. MBL serum concentrations were also determined in samples from 291 patients and 75 controls. The proportion of O (B, D or C) alleles was significantly higher in the patient group compared to controls, and this association was strongest for subgroup III. The promoter LX variant frequency was also commoner in the patients as a whole, and significantly so in subgroups II and IV. Genotypes markedly influenced MBL concentrations in all groups, and correlated with ability to activate the lectin pathway of complement activation. The strongest and most significant inverse correlations between serum MBL and respiratory disease were found in patient group III and in 17 patients with multiple humoral and/or cellular abnormalities. Among nine patients with unexpectedly low LP activity in view of their MBL concentrations, one person was found to be MASP-2 deficient. Our results indicate that mannan-binding lectin insufficiency, with or without a coexisting immune defect, is associated with the occurrence of recurrent respiratory infections in childhood, and this relationship is particularly strong and statistically significant in children with concomitant impairments of humoral immunity.

Case-Control Studies↗
PubMed1996

Pericytosis and edema generation: a unique clinicopathological variant of meningioma.

OBJECTIVE: We report a group of eight patients with a distinctive histological variant of meningioma that is associated with severe peritumoral edema. The clinical presentation, radiographic findings, and histology of this type of tumor may lead to misdiagnosis as an aggressive or malignant process. METHODS: We reviewed the histology from patients who had removal of meningiomas performed in our institution between 1978 and 1992. Tumors having abnormal proliferation of cells in the intramural vascular spaces were selected for study; case histories and radiographs were reviewed. Tumor material was subjected to special stains, immunocytochemical examination, and election microscopy. RESULTS: Several lesions were misread radiographically as being malignant. Patients underwent craniotomy with complete excision of the tumor. All lesions were small (< or = 3 cm), and no brain invasion, unusual tumor vascularity, or dural sinus involvement was noted in any case. Histologically, the meningioma pattern in each case was meningothelial and benign in appearance. The immunocytochemical and electron microscopic features of the unusual cells in the blood vessel walls are most consistent with their being of pericytic origin. All patients have remained asymptomatic and without evidence of tumor recurrence with follow-up from 3 to 12 years. CONCLUSION: These tumors showed proliferation of pericytes in blood vessel walls and represent a new subtype of meningothelial meningioma. The apparently benign nature of these lesions necessitates their recognition. Characteristic findings of pericytic proliferation associated with edema generation have led us to descriptively term this the PEG variant of meningioma.

Aged↗
PubMedDate not supplied

[The incidence and characteristics of the clinical picture of recurrent myocardial infarct in the rural population].

As many as 3033 patients with myocardial infarction residing in rural areas were kept under observation. In this population, myocardial reinfarction (MRI) was diagnosed in 411 subjects, with 78.8% having had it for 4 years. Those MRI patients ranging between 51 to 60 years showed the greatest prevalence (44.3%). The ratio of micro- to macrofocal (through-and-through) MRI was 1:3, that of males to females 9:1. Microfocal MRI was commonly associated with a pain-free variant of the disease (23.5%) with low frequency of thromboembolic complications (3.1%). Every fifth patient with macrofocal (through-and-through) renecrosis presented with aneurysm of the heart. In a 10-year and longer follow-up, mortality from MRI was 43.6 percent among patients with macrofocal MRI, while deaths attributable to microfocal MRI were estimated to be 28.9 percent. High mortality rates suggest great severity of illness and low efficacy of the drug therapy adopted.

Adult↗
PubMed1999

Hürthle cell carcinoma: A malignancy of low-grade potential.

BACKGROUND: Hürthle cell carcinoma of the thyroid is a variant of follicular carcinoma, which has been considered by many as a more aggressive disease than the usual well-differentiated carcinoma of the thyroid. Others, however, have found Hürthle cell carcinoma to be a low-grade malignancy with little morbidity or mortality. METHODS: This is a retrospective report on all patients with Hürthle cell carcinoma diagnosed during the years 1951-1997 by the authors. The behavior of the disease and results of treatment were analyzed biostatistically and the outcome was compared with that of patients with pure follicular carcinoma treated during the same period. RESULTS: Forty-two patients were diagnosed with Hürthle cell carcinoma and 153 with follicular carcinoma during this period (2.8% and 10.3% of all differentiated carcinomas of the thyroid, respectively). The rate of local recurrence and disease-related mortality were both 9.5%, compared with 3.2% & 15.7%, respectively, for the follicular cancers. There was a trend for better outcome in patients younger than 55 years of age, in patients with tumors under 4 cm in size, and in patients treated by total thyroidectomy. Distant metastases occurred in four patients (9.5%) and were the cause of disease-related mortality in three. CONCLUSIONS: When treated assertively, Hürthle cell carcinoma of the thyroid, an oncocytic variant of follicular carcinoma, has a favorable outcome, similar to that of pure follicular carcinoma. Uniting those two entities in a future classification and reporting should be considered.

Adenocarcinoma↗
PubMed2003

Investigation of the role of ANKH in ankylosing spondylitis.

OBJECTIVE: The ank/ank mouse develops a phenotype similar to ankylosing spondylitis (AS) in humans. ANKH, the human homolog of the mutated gene in the ank/ank mouse, has been implicated in familial autosomal-dominant chondrocalcinosis and autosomal-dominant craniometaphyseal dysplasia. This study was undertaken to investigate the role of ANKH in susceptibility to and clinical manifestations of AS. METHODS: Sequence variants were identified by genomic sequencing of the 12 ANKH exons and their flanking splice sites in 48 AS patients; variants were then screened in 233 patients and 478 controls. Linkage to the ANKH locus was assessed in 185 affected-sibling-pair families. RESULTS: Five single-nucleotide polymorphisms were identified within the coding region and flanking splice sites. No association between either susceptibility to AS or its clinical manifestations and these novel polymorphisms, or between disease susceptibility and 3 known promoter variants, was seen. No linkage between the ANKH locus and AS was observed. Multipoint exclusion mapping rejected the hypothesis of a locus of a magnitude lambda>/=1.4 (logarithm of odds score <-2) (equivalent to a genetic contribution of >10% to the AS sibling recurrence risk ratio) within this area contributing to AS. CONCLUSION: These findings indicate that ANKH is not significantly involved in susceptibility to or clinical manifestations of AS.

Adult↗
PubMed2002

Maxillary odontogenic myxoma: a diagnostic pitfall on aspiration cytology.

A painless, slow-growing cheek swelling in a young male clinically considered a salivary gland mass was aspirated. Cytology smears were hypocellular. The striking feature was abundant myxoid material with a few monomorphic oval cells, interpreted as myxoid variant of pleomorphic adenoma. Subsequent CT scan was suggestive of a malignant tumor but biopsy confirmed it as myxoma. Myxoma of the jaw is a rare benign tumor that has a tendency for bone destruction, invasion into surrounding structures, and a relatively high recurrence rate. Maxillary myxoma is less frequent but behaves more aggressively than in the mandible, as it spreads through the maxillary sinus. Cytologically, it should be differentiated from other tumors showing predominant myxoid change. Awareness of potential diagnostic pitfalls and careful evaluation of clinical and radiological data is necessary to narrow the differential diagnosis.

Adenoma, Pleomorphic↗
PubMed1995

Mutational analysis of patients with X-linked adrenoleukodystrophy.

Adrenoleukodystrophy (ALD) is an X-linked neurodegenerative disorder characterized by elevated very long chain fatty acid (VLCFA) levels, reduced activity of peroxisomal VLCFA-CoA ligase, and variable phenotypic expression. A putative gene for ALD was recently identified and surprisingly encodes a protein (ALDP) that belongs to a family of transmembrane transporters regulated or activated by ATP (the ABC proteins). We have examined genomic DNA from ALD probands for mutations in the putative ALD gene. We detected large deletions of the carboxyl-terminal portion of the gene in 4 of 112 probands. Twenty-five of the ALD probands whose ALD genes appeared normal by Southern blot analysis were surveyed for mutations by Single Strand Conformation Polymorphism (SSCP) procedures and DNA sequence analysis. SSCP variants were detected in 22 probands and none in 60 X-chromosomes from normal individuals. Mutations were detected in all of the ALD probands. The mutations were distributed throughout the gene and did not correlate with phenotype. Approximately half were non-recurrent missense mutations of which 64% occurred in CpG dinucleotides. There was a cluster of frameshift mutations in a small region of exon 5, including an identical AG deletion in 7 unrelated probands. These data strongly support the supposition that mutations in the putative ALD gene result in ALD.

ATP Binding Cassette Transporter, Subfamily D, Mem↗
PubMedDate not supplied

Differentiated thyroid carcinoma in children and adolescents.

From 1936 to 1990, 89 children and adolescents (72 girls and 17 boys) were treated for differentiated thyroid carcinoma at the University of Michigan Medical Center; 58 of these patients were first reported in 1971. Thirty (34%) patients had previously received external irradiation to the head and neck, although only 1 (3%) patient of the last 33 patients seen had this history. Patients first presenting from 1971 to 1990 had less advanced disease than those seen earlier. Also, during this time period, the rate of initial palpable cervical adenopathy fell from 63% to 36%, local infiltration of primary cancer from 31% to 6%, and initial pulmonary metastases from 19% to 6%. The incidence of cervical nodal metastases has remained 88% for 54 years. Papillary or the follicular variant of papillary carcinoma was found in 93% of all patients. Seventy-nine (89%) patients had total or completion total thyroidectomy. Surgical management of lymphatic metastases varied from regional excision of nodes to radical neck dissection. The overall rate of permanent accidental recurrent laryngeal nerve palsy and hypoparathyroidism was 4.5%, although neither has occurred in a child or adolescent undergoing surgery at the center in the past 25 years. The most recent 33 patients had a low rate of local/regional persistence or recurrence. Cervical nodal persistence occurred in 21%; there were no thyroid recurrences. Eighty-two percent of patients received 131I. The long-term mortality rate was 2.2%. We continue to advocate total thyroidectomy, cervical lymph node dissection, and postoperative 131I therapy as the most conservative treatment regimen for children with differentiated thyroid carcinoma.

Adenocarcinoma↗
PubMed2004

Bullous mastocytosis treated with oral betamethasone therapy.

Bullous mastocytosis is a very rare variant of cutaneous mastocytosis. The condition is characterized by a diffuse infiltration of the skin by mast cells manifesting as yellowish, thickened doughy skin with appearance of large blisters. The authors report herewith a 7-month-old female infant with history of recurrent episodes of vesiculobullous lesions on the face, trunk and the extremities and excessive tendency to rub and scratch the skin for 3 months. She also had recurrent episodes of facial flushing. On cutaneous examination there were multiple flaccid bullae, urticarial wheals and crusted erosions on her scalp, face, neck, trunk and extremities. She had generalised yellowish thick and rough skin, giving doughy feel and 'peau d' orange' appearance of the skin at places. Systemic examination was within normal limits. Skin biopsy from a lesion showed subepidermal bulla and an upper dermal inflammatory infiltrate comprising of lymphocytes and many mast cells. Toluidine blue staining of the cells showed presence of metachromatic granules in these cells. A diagnosis of bullous mastocytosis was made and the patient was treated with oral antihistamines to which there was no satisfactory response. Betamethasone in a dose of 0.1 mg/kg/day given orally caused complete remission of the disease in 4 weeks. The drug was gradually tapered and stopped over the next 6 weeks. There were no side effects of the therapy.

Administration, Oral↗
PubMed2002

The pagetoid variant of bladder urothelial carcinoma in situ A clinicopathological study of 11 cases.

Pagetoid urothelial carcinoma in situ (CIS) is a rare variant of bladder cancer that is characterized by an intraepithelial proliferation of large cells arranged singly or in clusters and randomly distributed. These neoplasms deserve recognition and attention, chiefly because they may be overlooked or misdiagnosed as urothelial dysplasia, then causing unsuspected tumor recurrence after surgery. We report on the clinicopathological features and immunohistochemical findings of 11 (14.86%) cases of pagetoid CIS in a retrospective study of 74 cases of conventional carcinoma in situ. Most patients were male ( n=10). Their ages ranged from 31 years to 78 years. The lesion can be present with primary ( n=2) or secondary ( n=9) CIS. Pagetoid CIS is usually a focal lesion occurring in a clinical and histological setting of conventional CIS, and these patients essentially have the same progression and survival rates as patients without pagetoid changes and are treated in the same way. In cases with extensive urothelial denudation, pagetoid CIS may be focally present in otherwise normal-looking urothelium, thus alerting the pathologist to search for additional CIS elsewhere in the bladder. Given that primary extramammary Paget disease of the external genitalia and of the anal canal may extend to the bladder and, conversely, some bladder cases of pagetoid CIS may extend to the urethra, ureter, and beyond to the external genitalia, the differential diagnoses between these two entities represent an important therapeutic consideration. Our data suggest that a panel of immunostains including CK7+/CK20+/TM+ may assist in differentiating urothelial pagetoid CIS from extramammary Paget disease which is known to be CK7+/CK20-.

Adult↗
PubMed2006

Management of a sporadic malignant subfrontal peripheral nerve sheath tumor.

Malignant subfrontal (olfactory) peripheral nerve sheath tumors (MPNSTs) are exceedingly rare. Although meningiomas are the most common subfrontal extra-axial lesions, it is important to recognize that MPNSTs, which are radiographically similar to meningiomas, can also be present in this location. MPNSTs require more aggressive surgical and postoperative management than meningiomas. In this paper, we describe a patient with a subfrontal MPNST with unusual histological characteristics and present a review of the literature. A 49-year-old woman presented with chronic sinusitis and progressive headaches. A neurological examination revealed left-sided anosmia. Brain-imaging studies revealed a large left subfrontal mass with extension into the frontal and ethmoid sinuses and the nasal cavity. The patient underwent both a bifrontal transbasal craniotomy and a transnasal approach for an attempt at total resection of both the intradural and extradural components of the MPNST. The patient was treated postoperatively with radiation therapy, and had no evidence of recurrence at her follow-up examination 1-year after treatment. Subfrontal PNSTs are extremely rare and usually benign. The specific cell and nerve of origin for these tumors remains unknown. Our case shows that these rare lesions can present as a malignant variant and thus require aggressive surgical and postoperative management to provide long-term tumor control.

Adult↗
PubMedDate not supplied

Arrhythmogenic right ventricular cardiomyopathy/dysplasia: is there a role for viruses?

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a primary heart muscle disease characterized structurally by progressive fibrofatty replacement of the right ventricle and clinically by life-threatening ventricular arrhythmias with left bundle branch block morphology. Recently, there has been a great deal of interest on ARVC/D as a cause of sudden death in young people, and it has been reported as the most common cause of exercise-related sudden death among competitive athletes in Italy. An autosomic dominant familial occurrence has been recognized, and four disease-causing genes have been recently identified in the dominant forms: ryanodinic cardiac receptor 2, desmoplakin, plakophilin 2, and transforming growth factor (TGF)-beta3. Furthermore, plakoglobin has been identified as the first gene responsible for the recessive variant of ARVC/D associated with palmoplantar keratosis and woolly hair (Naxos disease). However, although much progress has been made in molecular genetics, up to today, the pathogenesis of the disease is still unclear. The occurrence of myocyte apoptosis has been documented, suggesting that recurrent bouts of apoptosis may account for progressive atrophy of the myocardium, which is then replaced by fibrofatty tissue. Considering the frequent finding of myocarditis at histology, an inflammatory theory has been advanced, and infective mechanisms have been postulated to contribute to the onset and the progression of the disease. Cardiotropic viruses have been detected in some ARVC/D cases, and they have been proposed as possible etiologic agents. Several etiopathogenetic theories are herein presented in detail with particular attention to the inflammatory/infective one and its possible links between this and the genetic/dystrophic theories are discussed.

Animals↗