Anhidrotic ectodermal dysplasia.
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Biomedical subjects
Publications and source records attributed to P K Guha.
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Some aspects of clinical and epidemiological presentation of 172 non-lepromatous leprosy patients, belonging to the Armed Forces of India, have been presented. Tuberculoid type (TT) was the commonest form of the disease. Majority of the patients (59.8%) had a single lesion at the time of diagnosis. Age of onset of 62.2% of the patients was between 20 and 29 years. Incidence of gross neurological deficit or deformity was rather low in these patients. Ulnar nerve involvement showed evidence of clinical symptom most often.
Examination of intrafamilial contacts among the first degree relatives of 400 hanseniasis patients, revealed an additional 101 cases. Distribution pattern of disease types detected in the contacts, in relation to that found in the index cases, are analysed. An attempt has been made to evaluate the role of genetic factor in determining the type of hanseniasis in a patient.
Penile involvement was seen in 19.5% of 261 patients, aged 11 to 30 years, with tinea cruris. It was more common in patients under the age of 20 years (p less than 0.05). The increased incidence is probably related to the use of langota, a semiocclusive undergarment that may favor the growth of dermatophytes.
Age of onset of leprosy is merely a subjective information based upon the memory, intelligent appreciation and awareness of the patient and his relatives, in absence of a more reliable method. In the present study, a modification of the conventional method which we named "Complemented recall" method was adopted to determine the age of onset of 400 leprosy patients, in an attempt to collect a better approximate data.
Innate incapacity to elaborate a protective cellular immunity has been thought to underlie the evaluation of the disease process of lepromatous leprosy. Although both family and twin studies were suggestive of the involvement of some genetic factors, they have so far not been evaluated fully. Keeping this in view, the present study, conducted at the family level, was designed to evaluate the role of genetic factors in determining the type of leprosy in a patient.
One case of embryonal paratesticular sarcoma and 2 cases of leiomyosarcomas are reported. The 13-year-old boy with embryonal sarcoma is well after an orchiectomy and high ligation of the spermatic cord followed by radiotherapy and chemotherapy. A 66-year-old man has been doing well after orchiectomy and hemiscrotectomy for a paratesticular leiomyosarcoma. The third patient had a highly pleomorphic leiomyosarcoma and died 2 months postoperatively. The clinical and histological diagnosis of paratesticular rhabdomyosarcomas and leiomyosarcomas is reviewed and the various therapeutic approaches to these neoplasms are discussed. Retroperitoneal lymph node dissection, radiotherapy and chemotherapy are important adjuncts to orchiectomy in the management of rhabdomyosarcomas. On the contrary, retroperitoneal lymph node dissection and radiotherapy are not indicated in leiomyosarcomas, since these neoplasms tend to metastasize by the hematogenous route and are radioresistant. At the present time we are unable to evaluate chemotherapy in the management of paratesticular leiomyosarcomas.
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Unusual presentation of a BT leprosy patient with multiple nerve abscess and an approach to diagnosis and management of the problem have been described. The article will refamiliarize the reader with unusual nature of presentation, diagnosis and management of nerve abscess associated with leprosy.
Twenty freshly diagnosed, previously untreated, adult male patients with paucibacillary leprosy were treated with Multi drug therapy (MDT) according to WHO regimen (1982). The patients remained in the hospital throughout the course of the study. At the end of six months treatment, they were evaluated clinicobacteriologically and histologically for evidence of the disease activity. Active disease was present in 55% of the patients at the end of six months treatment. DDS had to be discontinued in one patient who developed hepatitis.