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Biomedical subjects

M Asfour

Publications and source records attributed to M Asfour.

At least 19 recordsLinked to original sources
PubMed2004

Transfusion of RhD-incompatible blood components in RhD-negative blood marrow transplant recipients.

BACKGROUND: Transfusion-dependent bone marrow transplant recipients are routinely transfused with ABO group and RhD-compatible blood components. However, because of the scarcity of RhD-negative blood components, particularly platelets, a policy was developed to transfuse RhD-positive blood components to RhD-negative patients during periods of shortage. METHODS: We reviewed the records of 78 RhD-negative patients with hematologic malignancies who received RhD-negative bone marrow and/or peripheral blood stem cells, from June 1995 to August 2000. The patients transfused with RhD-incompatible blood components were screened periodically for evidence of the development of red blood cell (RBC) alloimmunization. RESULTS: Three of 78 patients (4%) developed anti-D antibodies after receiving RhD-incompatible platelet transfusions. One of the patients developed evidence of anti-RhD antibodies after receiving 42 units of RhD-positive random donor platelets; the second patient developed such evidence after receiving 6 apheresis platelets and 2 infusions of intravenous immunoglobulin G (positive for anti-RhD). The third patient received 206 RhD-positive random donor platelets and 5 apheresis units. All patients were discharged from the hospital. The overall immunization rate was 4%. Six patients received Rh-incompatible packed RBCs and showed no evidence of neither anti-RhD nor any other anti-RBC antibodies. All 78 patients had received RhD-incompatible platelets throughout their engraftment period. CONCLUSION: Transfusion of RhD-positive blood components to Rh-negative patients with hematologic cancers, who have received RhD-negative bone marrow and/or peripheral blood stem cells, are at low risk of developing RhD antibodies. These findings allow for a flexible strategy of blood component therapy support for this special patient population during periods of shortage.

Adult↗
PubMed1997

Congenital adrenal hyperplasia complicated by central precocious puberty: linear growth during infancy and treatment with gonadotropin-releasing hormone analog.

Some children with congenital adrenal hyperplasia (CAH) develop true precocious puberty with early maturation of the hypothalamic-pituitary-gonadal axis. We have seen six such children who had the diagnosis of CAH with late initiation of corticosteroid treatment and/or poor compliance who developed central precocious puberty (CPP). These patients were treated with standard-dose hydrocortisone and fludrocortisone. Administration of depot leuprorelin (3.75 mg subcutaneously every 28 days) for 2 years or longer was effective in arresting the manifestations of puberty, decelerating the pretreatment growth velocity ([GV] 10.8 +/- 1.5 v3.65 +/- 0.95 cm/yr), increasing the predicted adult height ([PAHT] 147.5 +/- 7.8 v 153.4 +/- 8.3 cm), and decreasing the bone age to statural age ratio (1.26 +/- 0.13 v 1.16 +/- 0.09). Analysis of auxanological data during the first 2 years of life showed that linear growth was significantly accelerated and bone age was advanced in patients who developed CPP compared with 11 age-matched patients. It appears that proper glucocorticoid replacement to achieve adequate control of hyperandrogenemia during early life might prevent development of CPP in these patients. Gonadotropin-releasing hormone agonist (GnRHa) therapy can improve the final adult height, bringing it closer to that expected from the genetic potential.

Adrenal Hyperplasia, Congenital↗
PubMed1997

Growth hormone secretion and circulating insulin-like growth factor-I (IGF-I) and IGF binding protein-3 concentrations in children with sickle cell disease.

Impaired growth involving both height and weight accompanying sickle cell disease (SCD) poses diagnostic and therapeutic problems. We undertook this study to test the hypothesis that this impaired growth is associated with abnormalities of the growth hormone (GH)/insulin-like growth factor-I (IGF-I)/IGF binding protein-3 (IGFBP-3) axis in 21 children with SCD and that SCD is associated with GH resistance. Nine of 21 children with SCD had a defective GH response to both clonidine and glucagon provocation (peak < 10 micrograms/L); these children differed from the 12 others in having slower linear growth velocity (GV and GVSDS), lower circulating concentrations of IGF-I and IGFBP-3, and either partial or complete empty sellae in computed tomographic scans of the hypothalamic-pituitary area. In this group of patients with SCD, it appears that defective GH secretion and consequent low IGF-I production are the major etiological factors causing the slow growth. The two groups with SCD did not differ significantly in dietary intake, body mass index (BMI), midarm circumferences, skinfold thickness, serum albumin concentration, or intestinal absorption of D-xylose. A single injection of GH produced a smaller increase in circulating IGF-I in children with SCD with or without defective GH secretion versus 10 age-matched children with idiopathic short stature (ISS) and 11 children with isolated GH deficiency (GHD), suggesting partial GH resistance in the SCD group. The presence of defective GH secretion, decreased IGF-I synthesis, and partial resistance to GH in short children with SCD suggests that treatment with IGF-I may be superior to GH therapy for improving growth.

Adrenergic alpha-Agonists↗
PubMed1997

Mode of presentation and progress of childhood diabetes mellitus in the Sultanate of Oman.

We surveyed the clinical presentation, initial management and subsequent course of a prospectively registered cohort of 60 children with insulin-dependent diabetes mellitus (IDDM) diagnosed before age 15 years in the Sultanate of Oman between January 1990 and December 1993. Clinical details from the time of diagnosis were available on all the children. At diagnosis 9 (15 per cent) presented with severe ketoacidosis (DKA) with pH less than 7.1 or plasma bicarbonate less than 10 mmol/l, and 16 (27 per cent) had mild to moderate ketoacidosis with pH 7.1-7.35 or plasma bicarbonate 10-18 mmol/l. During DKA electrolyte disturbances included: hypokalemia (K < 3.5 mmol/l) 25 per cent), hyperkalemia (K > 5.5 mmol/l) (18 per cent) and hyponatremia (Na < 130 mmol/l) (40 per cent). Serum creatinine concentrations were high in 25 per cent of children with DKA. Within the first year of diagnosis, 17 of the 60 children (28 per cent) experienced symptomatic hypoglycaemia, which in six (10 per cent) led to one or more admissions. Re-admission for unstable glycaemic control, excluding acute hypoglycaemia occurred at least once in six children (10 per cent) within 1 year of diagnosis and in 10 (17 per cent) within 2 years. Statural growth velocity (GV) and GVSDS (6.9 +/- 0.85 cm/year and 0.75, respectively) were significantly higher in the group of children with good glycaemic control (HbA1C = 7.9 +/- 0.4 per cent) compared to those children (3.7 +/- 0.44 cm/ year and -1.6, respectively) with bad glycaemic control (HbA1C = 12.5 +/- 1.5 per cent). Insulin-like growth factor-I (IGF-I) concentrations were significantly higher (260 +/- 21 ng/ml) in the group with good glycemic control v. the group with bad control (149 +/- 15 ng/ml). In summary, greater public and medical awareness of the presenting features of diabetes in young children is needed to reduce the frequency of DKA at presentation, and improvement of patient and family education is necessary to reduce the incidence of DKA and hypoglycaemia in children with IDDM.

Adolescent↗
PubMed1996

Insulin and glucagon responses to provocation with glucose and arginine in prepubertal children with thalassemia major before and after long-term blood transfusion.

Hypertransfusion therapy has dramatically increased the duration and quality of life in patients with B-thalassemia major; however, it leads to chronic iron overload, and is frequently complicated by the development of diabetes mellitus or impaired glucose tolerance. To determine the early effect of iron overload on the endocrine pancreatic function, we studied glucose, insulin, and glucagon responses to oral load of glucose and to arginine provocation in 15 children with B-thalassemia major, before and after (3.1 +/- 0.6 years) high-transfusion and iron chelation and compared them with 15 age matched normal controls. In addition, we evaluated growth hormone (GH) responses to oral clonidine and measured the circulating insulin-like growth factor-I concentration in thalassemic children on long-term transfusion and controls. After long-term high-transfusion, thalassemic children had significantly decreased serum insulin concentrations and low insulin/glucose ratios at 60 and 120 min after an oral glucose load (1.75 g/kg) in comparison with values before therapy and those for controls. None of the thalassemic children had glucose intolerance after this period of frequent blood transfusion; however, their serum glucose levels at 60 and 120 min after the oral glucose load were significantly higher compared to control children. Thirty minutes after starting arginine infusion, serum insulin concentration was significantly lower in thalassemic children compared to before therapy. Basal and arginine-stimulated glucagon secretions were significantly elevated in thalassemic children on long-term blood transfusion with significantly low serum insulin/glucagon ratios. In addition, the high basal serum glucagon concentrations were not suppressed after the oral glucose load. Despite hyperglucagonaemia in all thalassemic children, their blood glucose dropped appropriately below 50 per cent of the fasting glucose level after an intravenous insulin dose (0.1 U/kg) ruling out any significant insulin-resistance. GH responses to clonidine provocation were subnormal in thalassemic children after long-term blood transfusion compared to controls. In summary, thalassemic children on long-term blood transfusion and iron chelation have progressive and early loss of B-cell mass, manifested by decreased insulin release in response to secretagogues, before the development of significant insulin resistance or impairment of glucose tolerance.

Arginine↗
PubMed1996

Hypoinsulinaemia has an important role in the development of oedema and hepatomegaly during malnutrition.

Various alterations in hormonal levels have been suggested to contribute to the development of nutritional oedema and fatty liver in children with kwashiorkor. We present an infant who underwent near-total pancreatectomy at the age of 4 weeks and developed kwashiorkor after 11 weeks. The sequence of events following surgery can be divided into two phases. The first phase was characterized by hyperinsulinaemia and hypoglycaemia before feeds. During this phase, although the weight gain was slow (10 g/day) serum albumin (32 g/I) and prealbumin (0.23 g/I) concentrations were maintained with no oedema or hepatomegaly. In the second phase, insulin deficiency prevailed and he was receiving the same amount of milk (protein)/day (enriched with starch). During that phase he rapidly developed hypoalbuminaemia (18 g/l), hypoprealbuminaemia (0.1 g/l), oedema, hepatomegaly, and dermatosis. This case demonstrates clearly the important role of defective insulin secretion in the development of nutritional oedema and hepatomegaly.

Edema↗
PubMed1996

Defective growth hormone secretion in children with pycnodysostosis and improved linear growth after growth hormone treatment.

Short stature is a characteristic feature of pycnodysostosis. We report defective growth hormone secretion in response to provocation and low insulin-like growth factor-I (IGF-I) concentration in five out of six patients with pycnodysostosis. Physiological replacement with growth hormone increased IGF-I concentration and improved linear growth in these children.

Adult↗
PubMed1995

Testosterone treatment in adolescent boys with constitutional delay of growth and development.

Administration of androgens to adolescent boys with constitutional delay in growth has been highly controversial. One hundred forty-eight adolescent boys with constitutional delay of growth and puberty with a mean age of 14.3 +/- 0.7 years were treated with testosterone enanthate 100 mg intramuscularly each month for 6 months. Growth parameters, sexual maturation, and circulating concentrations of testosterone and insulin-like growth factor-I (IGF-I) were compared with those for 50 age-matched adolescent boys with constitutional delay of growth and puberty with a mean age of 14.1 +/- 0.9 years who did not receive any treatment. The mean height growth velocity, height standard deviation score, weight gain, and IGF-I concentration were significantly greater in the treatment group after 1 year of follow-up evaluation. The advancement in bone age equaled that in chronologic age in the treatment group, with no significant change in the bone age to chronologic age ratio (BA/CA) before versus after therapy. All subjects in the treatment group had clearly entered puberty by the end of 1 year. Testicular size increased significantly in the treatment group and they had significantly higher serum testosterone concentrations 6 months after the end of testosterone therapy as compared with the control group, denoting activation of the hypothalamic-pituitary testicular axis. All subjects in the treatment group were psychologically satisfied with the enhanced growth and increased muscle mass, versus only 40% of those in the control group. In conclusion, our regimen appears to be efficacious and safe for treatment of boys with constitutional delay of growth and puberty and has no deleterious effect on skeletal age.

Adolescent↗
PubMed1995

Circulating growth hormone (GH), insulin-like growth factor-I (IGF-I) and free thyroxine, GH response to clonidine provocation and CT scanning of the hypothalamic-pituitary area in children with sickle cell disease.

Serum growth hormone (GH), cortisol, free thyroxine (FT4), thyroid-stimulating hormone (TSH), and insulin like growth factor I (IGF-I) concentrations were measured in 15 children with sickle cell disease (SCD) together with their heights < 5th percentile for age and gender, and in 15 healthy age-matched children who had normal variant short stature (NVSS). GH response to an oral dose of clonidine (0.15 mg/m2) and cortisol response to ACTH stimulation were determined in the two groups. Children with SCD had significantly lower serum concentrations of IGF-I and decreased GH response to stimulation. Eight out of the 15 children with SCD did not mount an appropriate GH response to clonidine provocation (> 10 micrograms/l). CT scanning of the hypothalamic-pituitary area in those eight children with SCD revealed a partial or complete empty sella in all of them. It appears that defective GH release, and consequently low IGF-I production and slow growth velocity in children with SCD might be secondary to hypoxic-vascular insults to their hypothalamic-pituitary axis during one or more of the sickling episodes.

Case-Control Studies↗
PubMed1995

Nocturnal growth hormone (GH) secretion and GH response to clonidine provocation in children before and after long-term prednisone therapy.

Glucocorticoids reduce growth hormone (GH) response to the majority of exogenously administered stimuli and can variably inhibit growth in man and animals. Recently, however, glucocorticoids have been shown to have varying effects on GH secretion depending on the time of administration and, furthermore, to be potent secretagogues. We evaluated growth parameters, GH response to high-dose clonidine and integrated (12-h) nocturnal and mean (12-h) nocturnal GH secretion in 10 prepubertal children before and after long-term alternate-day prednisone therapy (LTPT). Height standard deviation scores (HtSDS) and growth velocity standard deviation scores (GVSDS) decreased significantly after LTPT. GH response to clonidine as well as integrated and mean nocturnal GH secretion were significantly after LTPT. GH response to clonidine as well as integrated and mean nocturnal GH secretion were significantly depressed after LTPT v. before treatment. We compared growth parameters and GH data of two groups of children before and after LTPT. Group 1 had low GH peak response to clonidine after LTPT, and group 2 had normal GH response to clonidine. Group 1 children had significantly more impairment of their statural growth and nocturnal GH secretion. Growth parameters (HtSDS and GVSDS) during LTPT correlated significantly with the peak GH response to clonidine (r=0.69 and 0.78, respectively) as well as to the growth parameters before therapy (r > 0.9). It appears that LTPT impairs both physiologic and pharmacologically provoked GH secretion and consequently retards growth in children. However, this effect is variable and is affected by the attained statural growth before therapy.

Adrenergic alpha-Agonists↗
PubMed1995

Growth hormone deficiency and empty sella in DIDMOAD syndrome: an endocrine study.

Two girls with DIDMOAD syndrome are presented. One also had severe megaloblastic-sideroblastic anaemia and the other several neurological manifestations. Both were short with defective growth hormone secretion. Computed tomography revealed empty sella in both girls; one had widespread atrophic cortical and cerebellar changes. High doses of thiamine improved the anaemia in the first case, increased C peptide secretion in both, but had no effect on the neurological abnormalities.

Anemia, Sideroblastic↗
PubMed1979

Serum and seminal gonadotropins in normal and infertile men: correlations with sperm count, prolactinemia, and seminal prolactin.

Serum and seminal gonadotropins were evaluated in 30 normozoospermic and 30 oligozoospermic patients. Mean values of basal serum gonadotropins were higher in the oligozoospermic group than in the normozoospermic group: 3.8 versus 1.8 ng/ml and 3.3 versus 2.2 ng/ml for FSH and LH, respectively. Seminal FSH levels were comparable in the normozoospermic and oligozoospermic groups (0.8 versus 0.8 ng/ml, respectively) and were constantly lower than serum FSH levels in both groups. Seminal LH values were constantly higher than serum LH values in the normozoospermic group, whereas in the oligozoospermic group seminal LH values were higher, similar, or lower than serum LH values. However, no significant difference was noted between the two groups for seminal LH values: 3.6 (normozoospermic group) versus 4.1 ng/ml (oligozoospermic group).

Adult↗
PubMed1977

[The sella turcica and pituitary function in congenital hypothyroidism due to thyroid ectopia or hypogenesis (author's transl)].

Nine patients suffering from hypothyroidism due to thyroid ectopia or hypogenesis, with a large sella turcica, were examined at adolescence or during adult life. TSH, prolactin and growth hormone function were studied. Basal plasma TSH, evaluated in 5 cases, was raised and responded explosively to stimulation with TRH. The administration of L-dopa was not associated with any notable changes in TSH. In one case, the chronic administration of l-T3 resulted in a fall followed by normalisation in plasma TSH levels. Basal plasma prolactin, explored in 6 patients, was high in 4 females and normal in 2 males. TRH stimulation resulted in a marked increased in prolactin in the female cases and a moderate increase in the male cases. L-dopa caused a fall only in high prolactin values. Stimulation of GH by argininehydrochloride, insulin hypoglycaemia and L-dopa gave variable responses. In one case, substitutive thyroid hormone therapy restored the GH response to L-dopa and arginine hydrochloride to normal. Paradoxically, GH increase following stimulation with TRH.

Adolescent↗
PubMed1977

Hypogonadism, galactorrhoea and hyper-prolactinaemia: Evaluation of pituitary gonadotrophins reserve before and under bromocriptine.

Twenty patients with hypogonadism (19 women with amenorrhoea and 1 man with impotence and infertility), galactorrhoea and hyper-prolactinaemia (range: 36 to 344 ng/ml) were studied. The radiological study of the sella turcica, including in all cases hypocycloidal tomograms, allowed classification of the patients into 3 groups: group I (n = 4) had a grossly enlarged sella turcica, group II (n = 12) had localized alterations indicating the probable existence of a prolactin-secreting microadenoma ("microdeformation") while group III patients presented no radiological abnormality. Before treatment, all the patients were submitted to a complete evaluation of the function of their anterior pituitary, including the LH and FSH responses to iv administration of Gn-RH. All the group I patients had low basal LH levels and a blunted response to Gn-RH. The basal LH and in response to Gn-RH were normal in most of the group II patients and in all of the group III patients. An exaggerated FSH response to Gn-RH was observed in 6/12 patients with microdeformation (group II) but not in groups I and III patients. A low LH and a blunted LH response to Gn-RH is highly suggestive of the existence of a pituitary prolactin-secreting adenoma in case of amenorrhoea and hyper-prolactinaemia patients; a normal response does not however rule out such a diagnosis. The reasons for a exaggerated FSH response to Gn-RH in patients with suspected prolactin-secreting microadenoma remain to be investigated though this pattern can also occur in other cases of amenorrhoea. Hence the Gn-RH test might contribute to the assessment of the hypothalamo-pituitary axis of patients with hyper-prolactinaemia. Six patients treated for 4 months with bromocriptine (CB-154) were submitted to re-evaluation of their pituitary gonadotrophins reserve. All the women experienced restoration of menses with 39 days of treatment and the male patient regained potency. It was observed that bromocriptine treatment and subsequent normalized prolactin levels in the 4 group II women tested were associated with normalization of their previously exaggerated FSH response to Gn-RH; LH responses were also diminished in these cases. These data are compatible with the hypothesis that hyper-prolactinaemia per se could interfere with the endogenous secretion of Gn-RH at the hypothalamic level. In one patient with grossly enlarged sella turcica and a previous lack of an LH and FSH response to Gn-RH, bromocriptine treatment restored a normal gonadotrophins response, confirming that, in this case, the alteration of this response was indeed due to a prolonged lack of endogenous Gn-RH secretion.

Adolescent↗