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Dysmorphic features in three sibs with congenital dyserythropoietic anaemia type 1 are described. These findings include growth retardation/short stature, congenital ptosis, abnormal tarsal bones, metatarsal duplication/hypoplasia, nail/phalangeal hypoplasia of fingers and toes, Madelung deformity, syndactyly of toes, and hallux valgus. The patients also showed a very low mitotic index of their peripheral blood lymphocyte cultures. Phenotypic heterogeneity was elicited amongst the three Bedouin sibs. The present report confirms the association between a subset of congenital dyserythropoietic anaemia type 1 and a specific form of distal limb anomalies and suggests that other traits, congenital ptosis and low mitotic index, could represent part of the syndrome profile.
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PMID:Non-haematological traits associated with congenital dyserythropoietic anaemia type 1: a new entity emerging. 922 Jan 89

Prolonged penile prolapse in horses has been reported in association with administration of phenothiazine tranquilizers, trauma, neuropathies, severe general debilitation or exhaustion, starvation, rabies, herpes myeloencephalitis, equine infectious anemia, and purpura hemorrhagica. A 5-year-old gelding was admitted for treatment of prolonged penile prolapse of 12 days' duration that developed after acepromazine maleate was administered to allow examination of a laceration that had resulted in severe blood loss. The horse was sedated, and the penis was replaced in the preputial cavity by use of a combination of massage and bandaging. Treatment was successful, and recovery was complete.
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PMID:Persistent penile prolapse associated with acute blood loss and acepromazine maleate administration in a horse. 929 Aug 25

The authors report their experience in the treatment of hemorrhoids by rubber band ligation according to Barron's modified technique which foresees that the ligation is performed thanks to the suction of the hemorrhoidal node through the shaft of the band applicator connected with an aspirator. Eighty-four patients underwent consecutively this treatment over a 18-month period; all were performed with a minimum follow-up of 6 months. Forty male patients (mean age 46.6 years) showed symptoms lasting since 9 years. Forty-four female patients (mean age 42.6 years) showed such pathology since 8.9 years. 70.2% of the patients were classified as III stage of disease, 19% II stage and 10.7% I stage. Thirty-four patients had anal pain, 49 bleeding, 5 anemia, 21 thrombosis of the hemorrhoidal plexus, 54 prolapse of the ano-rectal mucosa. These symptoms and signs were present in most of the patients contemporaneously. Three patients had previously received rubber band ligations, 4 hemorrhoidectomy, 1 sclerotherapy and 1 rubber band ligation and sclerotherapy. In the whole we performed 285 sessions and 304 rubber band ligations. Each treatment consisted of 3.4 sessions and 3.6 rubber band ligations. Recovery was achieved with only one session in 9 patients; 66.7% of them showed 1st degree disease. Multiple sessions were necessary in patients with advanced disease degree; 100% at stage II and 94.9% at stage III. Sixty-five patients did not refer immediate and long-term significant complications. The remaining patients complained during the first hours about heavy feeling and/or tenesmus and two, 2 weeks after the end of treatment, showed bleeding episodes, which cleared up spontaneously. In 5 cases it was necessary, during follow-up, to carry out a completion rubber band ligation and in 3 we performed trimming surgery at the out-patients' department by resection of the exceeding skin and anal mucosa. The technique enables to achieve results just as valid as those of traditional methods in the treatment of hemorrhoidal pathology with the advantage that it can be performed in an out-patient's department, it does not need local anesthesia, it enables the patient to immediately return to his normal working activity and, restricted to the observation period (6- and 12-month follow-up) it allows a satisfactory control of the disease.
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PMID:[Ambulatory treatment of hemorrhoidal pathology with elastic bands according to a modified Barron technique]. 957 46

A case of pure red cell aplasia (PRCA), myasthenia gravis (MG) and thymoma is reported. A 70-year-old woman presented with severe anemia. She had been diagnosed as having MG 8 years earlier and her symptoms were adequately controlled with ambenonium chloride. When she visited our hospital, her hematocrit was 13.7% with a hemoglobin concentration of 4.7 g/dl and her reticulocyte counts were persistently abnormal at 0.1%. Although both direct and indirect Coombs' tests were positive, there was no evidence of hemolysis. Routine screening tests for other etiologies of anemia were negative. Serological tests for anti-DNA and anti-acetylcholine receptor antibodies gave positive results. A bone marrow examination revealed severe erythroid hypoplasia. PRCA was diagnosed and the patient was treated with periodic transfusions. A lateral view chest roentgenogram and a computed tomography scan of the thorax showed the presence of an anterior mediastinal mass which was suspected to be thymoma. The patient underwent thymothymectomy and the tumor was diagnosed as a thymoma. Although the patient received no treatment for MG and PRCA after surgery, her hematological test results rapidly improved and she was discharged from the hospital on the 29th postoperative day. At that time, her hematocrit was 33.2%, her hemoglobin concentration was 10.0 g/dl, her peripheral reticulocyte level was 1.8% and her left partial ptosis had improved. She is doing well, 9 months after surgery. For a patient to remain in remission without treatment for PRCA and MG after thymothymectomy is extremely rare.
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PMID:Pure red cell aplasia and myasthenia gravis with thymoma: a case report and review of the literature. 986 Dec 37

An interdisciplinary group of researchers developed a conceptual and methodological framework to determine the extent of the problem of reproductive morbidity at the community level in Middle Eastern society and then, in turn, to improve reproductive health conditions in women. It conducted an exploratory study in a family planning clinic in Cairo, Egypt; a medical workshop on clarification of the symptomatology of reproductive morbidity; and a focus group in a village in Giza, Egypt, to ensure that the reproductive morbidity questions of interview questionnaires were appropriate and complete. The group tested the accuracy of the questionnaires in 2 villages in rural Giza (509 women). Field workers went to the women's homes to administer the questionnaire on characteristics of the household during the first visit. During the second visit, they administered the questionnaire on reproductive morbidity, and then the social researcher went with the women to the health center so the women could undergo a gynecological examination. 50% of the women had reproductive tract infections, especially vaginitis. 56% had prolapse, and prevalence increased significantly with age. 63% had anemia, especially 14-19 year olds (76%). Just 24 women (5%) had no reproductive morbidity. About 50% had at least 3 reproductive conditions. The leading problems reported by the women were vaginal discharge (77%), dysmenorrhea (71%), perceived delay in conception (48%), stress incontinence (37%), and pain during intercourse (36%). Feeling of heaviness below, probably indicating prolapse, was the only symptom which increased with age (p = .03). 41% had been pregnant in the last 2 years. 77% delivered at home. Leading conditions during pregnancy were headache (59%) and discharge (45%), and those after delivery were fever and discharge/inflammation (30% for both). The interdisciplinary group proposed 3 mechanisms which are of utmost importance to policy: conducting similar research in other communities, expansion of reproductive health services at the community level, and implementing changes in the education and training programs of health professionals and social scientists.
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PMID:Concepts and measures of reproductive morbidity. 1014 96

This is the case of a 27 years-old woman with signs and symptoms of severe untreatable congestive heart failure, anemia, gingival mucosa ulcers, photosensitivity and alopecia. The electrocardiographic, echocardiographic, angiographic and hemodynamic data oriented the diagnosis of restrictive cardiomyopathy, mitral insufficiency secondary to mitral prolapse and bi-atrial dilation. The histologic study of the endomyocardial biopsy, performed during catheterization, showed signs of endomyocardial fibrosis, and immunological analysis was compatible with systemic lupus erythematosus. As far as we know, this is the first case of endomyocardial fibrosis (Davies disease) associated with systemic lupus erythematosus published in the medical literature. The etiology of Davies disease remains unrevealed and its association with systemic lupus erythematosus suggest a probable autoimmune origin.
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PMID:Endomyocardial fibrosis (Davies disease) coincidental with systemic lupus erythematosus. 1085 12

A May 1991 workshop on reproductive morbidity attended by 60 participants in Cairo included presentation of results of a study of 509 nonpregnant women aged 20-60 years. The study was conducted by an anthropologist, a biostatistician, 2 obstetrician-gynecologists, and a microbiologist in 2 rural villages of Gizeh. The majority of the women studied had married before age 19, and 80% were illiterate. Despite initial reticence, over 90% of the village women ultimately took part in the study. The team observed the frequency with which the women accepted illness, weakness, and pain as a normal part of life. Physical examinations revealed that 44% had vaginitis, 9% were anemic, 17% had severe anemia, 56% had prolapse, 18% were hypertensive, and 42% were obese. Survey questionnaires revealed that 36% experienced pain during intercourse, 18% had pain in the lower abdomen, 71% had menstrual pain, 15% had pruritus, and 48% feared they were sterile. The team observed that the women were apparently reluctant to use the local health services. The villages had their own health centers staffed by female physicians, but only 1/3 of the women giving birth in the preceding 2 years had sought prenatal care, and 75% chose to deliver at home. Relations between the health workers and the village women must be strengthened if the situation is to be improved. The seminar recommended that the health and social workers make greater efforts to encourage use of the health services by local women.
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PMID:[In Egypt, researchers assess the health of village women]. 1228 55

In 1998, a multidisciplinary group of researchers investigated women's reproductive health in two rural villages in Giza, Egypt using data from clinical examinations and laboratory tests. In addition, a questionnaire supplemented with in-depth interviews illuminated women's perceptions of illness and the sociocultural context of the community. Examining a random sample of 509 married, nonpregnant women, the researchers found that the vast majority of these women suffer from a spectrum of gynecological and related conditions. Beyond reproductive tract infections, which affect roughly half of the women, genital prolapse afflicts 56% and anemia 63%. Suspicious cervical cell changes (11%) and cervical erosion (22%) in women were noted. Moreover, related conditions were also highly prevalent: women suffered from urinary tract infections (14%), obesity (43%), and hypertension (18%). The team also discovered that two-thirds of women with symptoms had not sought care. Factors other than the physical accessibility, inadequacy in the quality and nature of available health services, and low priority that women place on their health have all contributed to women's lack of seeking treatment. This study suggests that a multidisciplinary approach to medical provision, taking women's perceptions into account is the most effective way to address reproductive health and ill health in communities of the developing world.
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PMID:Rural Egyptian women bear heavy disease burden. 1229 56

Few peoples of the world have completely avoided the practice of ritual mutilation, which at present appears particularly widespread in Africa. This article discusses several forms of mutilation practiced in Africa, most of which are currently rare and disappearing. Cranial deformation due to mechanical pressure in the early years of life was common in Africa and elsewhere, but its origins are difficult to trace. Collars were worn by females in some parts of Africa to elongate the neck, sometimes so weakening the skeletal structure that the head could not be held upright without the device. Many women subjected to their use died at young ages of cerebral vascular accidents. The shortening of the uvula is regarded as a benign mutilation, but could result in infection and death due to the unsanitary conditions in which it was practiced. Perforations of the nose, lips, or ears to permit use of personal adornments were common. Filing of teeth and scarring were other techniques of uncertain origin. Among genital mutilations, circumcision was the most widely known and commonly practiced. It was usually performed on boys of 7-12 years and was invested with ritual significance. Amputation of the gland by unskilled operators or later development of pseudoelephantiasis, nodules, penile fistulas, or stenosis were not rare complications. The custom of excising the clitoris and the labia minora was imported from the Near East and spread west and south with the penetration of Islam. It is still carried out at puberty. Although life-threatening complications are rare, local sequelae including urinary problems, vaginal obstruction, and scleroses leading to vesicovaginal fistulas during childbirth are common. The motive appears to be to prove the subordination of women and the prohibition of all sexual pleasure. Infibulation involves excision of the external genitalia and the creation of an orifice the size of a pencil to allow escape of menstrual fluid and urine. The operation is performed on little girls of 7 or 8. The husband creates a small opening at the time of marriage, and the woman is cut open and sewn up at each delivery. The complications are variable and may include local infection, hemorrhage, anemia, clitoral cysts, cystitis and acute urinary retention, vesical lithiasis, and psychic problems. Disinfibulation is complicated by sectioning of the anal sphincter, bladder damage, and uterine prolapse. The custom is only seen among Islamic peoples.
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PMID:[Problems related to African customs and ritual mutilations]. 1231 26

A critical evaluation of the maternal deaths that occurred in the performance of 745 caesarean sections performed in the rural environment of India over the 1965-1973 period was conducted. During this period there were 20 maternal deaths, giving an incidence of 2.7%. In the series there were 11 moribund cases of placenta previa with history of internal examination done outside in 9 cases. Out of 5 deaths in obstructed labor, 4 were in group 2 (obstructed labor with pronounced effect on mother but with a living fetus) and 1 in group 3 (obstructed labor with dead fetus). Out of 4 deaths in secondary cervical dystocia, 3 were associated with prolapse and 1 with carcinoma cervix. The clinical condition at the time of section was severe anemia with shock and bleeding in 8 cases, features of exhaustion with or without evidence of sepsis in 10 cases and apparently normal in 2 cases. While there was no death in elective section, in emergency cases the mortality was 4.1%. With increasing duration of labor the risk was found increased from nil to as high as 6.8% where caesarean section was performed beyond 48 hours of labor. Shock, sepsis and embolism accounted for 75% of deaths. 7 of 20 deaths were within 6 hours of operation and as many as 9 deaths occurred after 72 hours. There were 13 stillbirths and 2 neonatal deaths out of 20 maternal deaths.
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PMID:A critical evaluation of maternal deaths in caesarean section met in rural obstetric practice. 1232 30


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