1/11. A family with IgA nephropathy and hereditary lymphoedema praecox.immunoglobulin a (IgA) nephropathy is the most common primary glomerulonephritis worldwide. The pathogenesis is still unknown and treatment has not yet been established. Rarely it can be associated with other disorders. Its association with hereditary lymphoedema is not reported before. We report four patients, a 60-year-old father, his two sons and his daughter, with hereditary lymphoedema. The family had nine members and in four of them lymphoedema was evident. The other members had neither lymphoedema nor IgA nephropathy. This is the first report of IgA nephropathy in association with hereditary lymphoedema.- - - - - - - - - - ranking = 1keywords = praecox (Clic here for more details about this article) |
2/11. Computed tomography in unilateral extremity swelling of unusual cause.Computed tomography (CT) were used in the evaluation of unilateral soft tissue swelling of unusual cause. Three patients with unilateral myositis are described and the diagnostic information provided by the CT scan is discussed. A fourth patient with lymphedema praecox and unilateral extremity swelling is presented.- - - - - - - - - - ranking = 0.25keywords = praecox (Clic here for more details about this article) |
3/11. Meige disease (familial lymphedema praecox) and cleft palate: report of a family and review of the literature.This is a report of a family with lymphedema praecox and cleft palate. The mother had only lymphedema of the lower extremities; she gave birth to five sons, 3 of whom had both lymphedema of the lower extremities and cleft palate. Others have reported similar associations in the literature without calling attention to their relationship. In familial cases, an autosomal dominant mode of transmission with variable expression has been suggested. A common pathogenetic mechanism that might account for the localized lymphedema and associated cleft palate can only be speculated at this stage of our understanding of the condition.- - - - - - - - - - ranking = 1.25keywords = praecox (Clic here for more details about this article) |
4/11. yellow nail syndrome associated with mental retardation in two siblings.Two siblings with yellow nail syndrome and mental retardation are described. In addition to nail changes, the younger brother was found to have congenital lymphoedema and idiopathic pleural effusion, and the elder brother, lymphoedema praecox. They had in common a history of respiratory tract infections. Their intelligence quotient as measured by the Suzuki-Binet tests was 30% and 70%, respectively.- - - - - - - - - - ranking = 0.25keywords = praecox (Clic here for more details about this article) |
5/11. Familial lymphedema praecox: Meige's disease.Familial lymphedema should be classified in two categories depending on onset. Milroy's disease, or congenital hereditary lymphedema, is present from birth, painless, without tendency to ulcerate, and may have cholestasis or intestinal lymphangiectasia associated with it. Meige's disease, hereditary lymphedema praecox, is lymphedema with onset in the first or second decade, often presenting with inflammation, and may have a number of associated related anomalies including distichiasis, extradural cysts, vertebral anomalies, cerebrovascular malformation, yellow nails, and sensorineural hearing loss. Both types follow an autosomal dominant pattern. This paper presents a family of 39 persons in 5 generations with 13 affected persons having Meige's disease. The importance of recognizing this type of familial lymphedema is discussed.- - - - - - - - - - ranking = 1.25keywords = praecox (Clic here for more details about this article) |
6/11. Transformation of the common wart into squamous cell carcinoma in a patient with primary lymphedema.A patient is reported in whom treatment-resistant periungual and subungual warts underwent malignant transformation into squamous cell carcinoma. Similar changes were observed in multiple warts of the vulvar, perianal, and intravaginal as well as cervical area. The tumors that develop were in many instances microinvasive but did not metastasize. The remarkable conversion of a common wart into a squamous cell carcinoma further implicates the human papovavirus in oncogenesis. The specific factors accountable for the malignant change remain completely unknown, but the patient's lymphedema praecox, as well as her severe reaction to vaccinia, and her development of a disseminate systemic mycobacterium scrofulaceum infection suggest that the critical factor may be one of immunity.- - - - - - - - - - ranking = 0.25keywords = praecox (Clic here for more details about this article) |
7/11. Relief of lymph obstruction by use of a bridge of mesentery and ileum.The background to the use of a bridge of ileum with its associated mesentery in the relief of lymphatic obstruction is described. A case report of the successful use of this method in a 22-year-old patient with lymphoedema praecox is presented. Following experience with further cases, it is suggested that, provided changes in the lymph channels and in the tissues of the affected limb are not irreversibly damaged, effective drainage through small gut lymphatics may be achieved.- - - - - - - - - - ranking = 0.25keywords = praecox (Clic here for more details about this article) |
8/11. Cutaneous secondary follicular centre cell lymphoma in association with lymphoedema praecox.We report a patient who developed cutaneous metastases in a lymphoedematous leg, from a follicular centre cell lymphoma, in a setting of lymphoedema praecox and recurrent deep-vein thromboses. To our knowledge, this is the first report of such a case. We discuss the possible causes for this localization.- - - - - - - - - - ranking = 1.25keywords = praecox (Clic here for more details about this article) |
9/11. lymphedema praecox seen as isolated unilateral arm involvement: case report and review of the literature.lymphedema praecox, a type of primary lymphedema, becomes evident at puberty, occurs mostly in girls, and is usually confined to the lower extremities. Arms tend to be involved in either congenital or secondary lymphedema. We describe a unique localization in an 11-year-old girl who had primary lymphedema of the right hand and arm. The pertinent literature is reviewed.- - - - - - - - - - ranking = 1.25keywords = praecox (Clic here for more details about this article) |
10/11. lymphedema as a cause of unilateral leg swelling: a case report with 6-yr follow-up.OBJECTIVE: To discuss a case of unilateral lower extremity swelling caused by an uncommon condition, lymphedema praecox. An emphasis is placed on diagnostic imaging. CLINICAL FEATURES: A 48-yr-old man suffered from low back and left leg pain. The left leg was swollen with evidence of cellulitis. Orthopedic evaluation separated the low back pain from the leg pain, and a diagnosis of sacroiliac syndrome concurrent with lymphedema was made. Further evaluation led to a diagnosis of congenital lymphedema, specifically lymphedema praecox, because of the age of onset. INTERVENTION AND OUTCOME: Because of active cellulitis, direct treatment of the lymphedema was contraindicated. The patient was advised in techniques to keep the leg clean and to help decrease pooling of lymph by placing the limb in an elevated position. The patient was also referred to a medical facility for antibiotics. The low back pain was addressed using chiropractic. CONCLUSION: Congenital lymphedema is an uncommon condition that causes unilateral limb swelling. The differential diagnosis includes other uncommon entities that should be ruled out so that proper treatment may be administered. Congenital lymphedema is best treated conservatively and only after any cellulitis has been controlled.- - - - - - - - - - ranking = 0.5keywords = praecox (Clic here for more details about this article) |
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