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Z O Amarin

Publications and source records attributed to Z O Amarin.

11 recordsLinked to original sources

Acute splenic sequestration in female children with sickle cell disease in the North of Jordan.

The objective of this study was to evaluate the rate of acute splenic sequestration (ASSC) in patients with sickle beta-thalassaemia and sickle cell anaemia, the risk of recurrence in those who survive the first episode, and the relationship between ASSC episodes and subsequent hypersplenism. All patients with confirmed diagnosis of sickle cell disease at a tertiary referral teaching hospital, between January 1994 and December 2002 were interviewed and had their medical records reviewed. Seventy-seven patients with sickle cell disease were identified. Their ages ranged between 2 and 18 years (mean, 10.1 years). There were 35 females and 38 males. Thirty-seven (50.6%) had sickle beta-thalassaemia, and 36 (49.4%) had homozygous sickle cell anaemia. Of these, 26 had high level of Hb F and 11 had normal level of fetal haemoglobin (Hb F). Twenty-one patients (28%) had 63 episodes of acute splenic sequestration. Thirty-seven episodes were experienced by 12 patients with sickle beta-thalassaemia; of these 11 were major attacks with one fatality. Twenty-six episodes were experienced by nine patients with sickle cell anaemia. Splenomegaly and hypersplenism were greater in the acute splenic sequestration group than in the rest of the sickle cell anaemia patients, and the differences were extremely significant. ASSC was found in nine siblings of sickle beta-thalassaemia group, while none were found in the sickle cell anaemia group. The mean age of the first episode was significantly higher in sickle beta-thalassaemia, with significant differences in the levels of Hb F, Hb S, size of spleen and severity of crisis between both groups. In the sickle cell anaemia group the only significant difference between patients with and these without acute splenic sequestration was the difference in the size of spleen. In this study, the rate of ASSC in the sickle beta-thalassaemia patients was 32%, in contrast to 25% in the sickle cell anaemia patients. The risk of recurrence was about 70% in those who survived their first episodes. There was a close relationship between ASSC and subsequent hypersplenism. Important predictable factors for ASSC in sickle beta-thalassaemia patients were the presence of splenomegaly of more than 5 cm below the costal margin, history of acute splenic sequestration in siblings and high Hb F. Most of first episodes in sickle cell anaemia occur under the age of 2 years, while in sickle beta-thalassaemia the majority of patients have their first crisis at the age of > or =3.5 years.

Acute Disease↗

Hypothalamic-pituitary-gonadal function in adolescent females with beta-thalassemia major.

OBJECTIVE: To evaluate the function of the hypothalamic-pituitary-gonadal axis in adolescent female patients with beta-thalassemia major. MATERIALS AND METHODS: A prospective study of the function of the hypothalamic-pituitary-gonadal axis function of 31 beta-thalassemia major females aged between 13 and 22 years and in 12 control females aged between 12 and 22 years. All were treated at Princess Rahma Teaching Hospital, Irbid, Northern Jordan between April 2001 and April 2003. RESULTS: Of the 31 beta-thalassemia major females, 13 (41.9%) had delayed puberty. Hypothalamic-pituitary-ovarian axis dysfunction was found in 15 patients (48.4%). Twelve patients (38.7%) had hypogonadotropic hypogonadism and 5 (16.1%) had ovarian failure. High levels of serum ferritin were significantly higher in patients with delayed puberty. CONCLUSION: Pituitary and ovarian dysfunction are common problems in beta-thalassemia major patients. The main possible cause is iron overload. This stresses the need for intensive and regular use of chelation therapy to prevent damage to the hypothalamic-pituitary-ovarian axis.

Adolescent↗

Risk of malignancy index in the preoperative evaluation of pelvic masses.

OBJECTIVES: To assess the ability of a risk malignancy index (RMI) based on serum levels of CA 125, ultrasound findings, and menopausal status to discriminate between benign and malignant pelvic masses in a particular population. METHODS: A retrospective study was conducted of 100 women with pelvic masses admitted for laparotomy. The sensitivity and specificity of serum levels of CA 125, ultrasound findings, and menopausal status were calculated both separately and combined into a RMI to diagnose malignancy. RESULTS: The RMI was more accurate than any single criterion in diagnosing malignancy. Using a cut-off level of 200 to indicate malignancy, the RMI gave a sensitivity of 90%, specificity of 89%, positive predictive value of 96%, and negative predictive value of 78%. CONCLUSION: The RMI is able to correctly discriminate between malignant and benign pelvic masses. It is a simple scoring system that can be introduced easily into clinical practice to facilitate the selection of patients who would benefit from primary surgery.

Adenocarcinoma↗

Bilateral partial oophorectomy in the management of severe ovarian hyperstimulation syndrome. An aggressive, but perhaps life-saving procedure.

Two case histories are described, in which protracted courses of severe ovarian hyperstimulation syndrome (OHSS) responded poorly to conservative treatment. Each patient underwent bilateral partial oophorectomy at 14 and 16 days respectively, post oocyte retrieval. Serum albumin levels returned to normal within three days of the operation in each case and the patients, one pregnant with twins, made a rapid recovery. This seemingly 'aggressive' procedure is proposed as a potentially useful treatment when faced with patients who are severely or critically affected with OHSS.

Adult↗

Does consanguinity affect the severity of pre-eclampsia?

To determine whether consanguinity is more likely to be associated with severe forms of pre-eclampsia/eclampsia. Presuming a pure genetic contribution, we speculated that consanguineous marriages would increase the occurrence of severe forms of pre-eclampsia/eclampsia, through an expected increased chance for homozygosity to the putative gene. The study is a clinical case series on pre-eclamptic/eclamptic primiparae delivered at Princess Badea Teaching Hospital, which is a tertiary referral center. The internationally accepted definitions for hypertension, proteinuria, mild and severe pre-eclampsia were adopted. The study included 77 primiparae. The incidence of consanguinity in the studied sample was 38%. Of them, 28 (36.4%) had mild pre-eclampsia, 45 (58.4%) had severe pre-eclampsia and four (5.2%) had eclampsia. There was no statistically significant difference in the occurrence of severe pre-eclampsia/eclampsia between primiparae married to a first cousin or a relative other than a first cousin and primiparae married to a non-relative, odds ratio 1.1 (95% CI 0.33-3.87), P value 0.94, odds ratio 2.6 (95% CI 0.45-27.6), P value 0.30, respectively. Also, there was no statistically significant difference in occurrence of severe pre-eclampsia/eclampsia between primiparae whose parents are first cousins or relatives other than a first cousin and primiparae whose parents are non-relatives, odds ratio 1.3 (95% CI 0.36-4.72), P value 0.81, odds ratio 1.61 (95% CI 0.23-18.4), P value 0.70, respectively. Our study did not support a causal relationship between consanguinity and the occurrence of severe pre-eclampsia/eclampsia. The role of more complex genetic, immunologic, metabolic, hemostatic or, possibly yet, other unknown factors have to be explored.

Adolescent↗

The use of intrapartum defibulation in women with female genital mutilation.

OBJECTIVE: To assess the use of intrapartum defibulation for women who have had female genital mutilation. DESIGN: A retrospective case analysis. SETTING: King Abdulaziz University Hospital, a teaching hospital in Jeddah, Saudi Arabia. SAMPLE: Two hundred and thirty-three Sudanese and 92 Somali women who were delivered at the hospital between January 1996 and December 1999. METHODS: The outcome of labour of women with female genital mutilation who needed intrapartum defibulation were compared with the outcome of labour of women without female genital mutilation who did not need intrapartum defibulation. RESULTS: One hundred and fifty-eight (48.6%) women had infibulation and needed intrapartum defibulation to deliver vaginally, 116 women (35.7%) did not have infibulation and gave birth vaginally without defibulation, and 51 (15.7%) women were delivered by caesarean section. There were no statistically significant differences, between women who underwent intrapartum defibulation and those who did not, in the duration of labour, rates of episiotomy and vaginal laceration, APGAR scores, blood loss and maternal stay in hospital. The surgical technique of intrapartum defibulation was easy and no intraoperative complications occurred. CONCLUSIONS: Intrapartum defibulation is simple and safe, but sensitivity to the cultural issues involved is essential. In the longer term, continuing efforts should be directed towards abandoning female genital mutilation altogether.

Adult↗

Round spermatid separation and in-vitro maturation.

OBJECTIVE: To combine 2 methods; spermatid isolation and in-vitro maturation to improve their quality and enhance their ability of fertilization. METHODS: A discontinuous Percoll gradient was used to separate immature germ cells. Co-culture with Vero cells was attempted to convert round spermatids into more mature forms. In total 87 spermatids were studied. RESULTS: Of a final number of 77 round spermatids only 12 (15.5%) showed a certain degree of maturation in 3 out of 7 patients (42%). Of those 12 maturing spermatids, only 4 developed to an early elongated spermatid stage Sc1, but without flagella. CONCLUSION: Considering the limited in-vitro round spermatid maturation achieved in this study, the low fertilization and pregnancy rate with spermatids in general and round spermatids in particular, further refinements of this technology have to be achieved before its regular implementation in routine clinical practice is justifiable.

Animals↗

Hypogammaglobulinaemia during pregnancy in identical twin sisters.

The clinical histories and reaction to pregnancy is described in identical twin sisters with acquired hypogammaglobulinemia. Additional evidence for the genetic basis of this disorder is advanced. A possible association with myasthenia gravis is noted and the occurrence of fetal growth retardation is described. Replacement therapy should be given when the diagnosis is made, and high doses might be useful during pregnancy especially if fetal growth retardation seems to be present.

Adult↗