Priapism in a sickle cell prepuberal child.

Between 1985 and 1995, six sickle cell children listed SS were hospitalized in our department for priapism persisting after 24 hours of medical treatment. Two cases regressed after 6 hours of treatment follow-up. Four children were treated by a corpus cavernosum puncture, through the gland, leading to priapism detumescence in 3 cases. In one of our cases, the persistence of priapism induced us to perform a spongiocavernous anastomosis at the 72nd hour. One of the children developed a cerebrovascular accident 10 days after the beginning of the priapism. The other children have been re-examined. They have not presented any reccurence and have normal erection after puberty. Starting from a literature review, we explain the principles we adopted. The physiopathology of priapism in sickle cell patients remains unclear, for both high and low flows do exist. In addition to the risks related to blood products, the treatment by transfusional exchange involves neurological risks and must not delay any surgery. Analgesic treatment is often required. Corpus cavernosum puncture is efficient, with no fibrosis risk. In case of failure, a cavernospongious shunt, with a biopsy needle, can be performed at the same time. Surgical anastomoses are then proposed in case of priapism persistence. General evolution is satisfactory. But the sickle cell patient's priapism needs a long-term follow-up in order to recognise any minor recurrences. The latter could be the principal cause of fibrosis and impotency.     

Hepatic abscess in sickle cell anaemia: a rare manifestation.

A child with sickle cell anaemia developed a hepatic abscess, which was managed successfully by percutaneous drainage under ultrasound control. A history of attacks of pain dissimilar to usual vaso-occlusive crises should be treated with suspicion and investigated appropriately.     

Priapism complicating sickle cell disease in Nigerian children

Forty-two out of a total of 45 children who presented with priapism in the University College Hospital Ibadan during an eight-year period had associated sickle cell disease (SCD). Conservative management was satisfactory in 35 out of 40 patients, but the outcome in all the five cases subjected to surgery was poor. Our findings suggest that associated SCD should be suspected in children presenting with priapism in areas of the world where SCD is endemic, and that conservative management is preferable to surgery.     

Bone marrow transplantation in sickle cell anaemia.

Sickle cell anaemia is still responsible for severe crippling and death in young patients living in developing countries. Apart from prophylaxis and treatment of infections, no active treatment can be safely proposed in such areas of the world. Therefore a bone marrow transplantation was performed in 12 patients staying in Belgium and planning to return to Africa. Twelve patients, aged between 11 months and 23 years (median 4 years), underwent a HLA identical bone marrow transplantation. The conditioning regimen included oral busulphan for four consecutive days (4 mg/kg) followed by four days of intravenous cyclophosphamide (50 mg/kg). In 10 patients the engraftment was rapid and sustained. A further patient suffered transient red cell hypoplasia and another underwent a second bone marrow transplantation from the same donor at day 62 because of graft rejection. All patients are alive and well with a follow up ranging from 9-51 months (median 27 months). In all cases a complete cessation of vaso-occlusive episodes and haemolysis was observed as was a change in the haemoglobin pattern in accordance with the donor''s electrophoretic pattern.     

Endocrine functions in sickle cell anaemia patients.

In this study, 80 male and female sickle cell patients, aged 4-50 years, with mild (severity index, SI < 6) and severe (SI > or = 6) forms of the disease were investigated). The levels of luteinizing hormone (LH), follicle stimulating hormone (FSH), testosterone, cortisol, growth hormone (GH), free thyroxine (T4), and free triiodothyronine (T3) were determined. The results were evaluated and the mean +/- 2 SD values were compared with those obtained in age- and sex-matched normal controls. The findings indicated gonadal hypofunction in the sickle cell patients, but with varied deviations from the mean results. Patients with the severe form of the sickle cell disease showed more frequent abnormalities of LH, FSH, cortisol and testosterone in comparison with the patients with a mild disease. The LH, FSH, cortisol and testosterone levels were lower, while T3 and T4 did not show significant differences between patients and the controls. The results suggest that the sickle cell gene abnormality has an adverse effect on endocrine functions. Follow-up and appropriate management of endocrine dysfunctions are advocated in such patients.     

Hepatic abscess in sickle cell anaemia: a rare manifestation

A child with sickle cell anaemia developed a hepatic abscess, which was managed successfully by percutaneous drainage under ultrasound control. A history of attacks of pain dissimilar to usual vaso-occlusive crises should be treated with suspicion and investigated appropriately.     

小儿慢性粒细胞性白血病合并阴茎异常勃起一例

患儿男,11岁,住院号594203。因“阴茎异常勃起15h”于2004年5月3日凌晨零点八分急诊入院。患儿于入院前15h洗澡时无明显诱因出现阴茎勃起,勃起呈持续性,不能疲软,并感阴茎疼痛,可排尿,尿线较粗。在当地医院行镇静、冰盐水冷敷、冰盐水灌肠等处理不见好转,急来我院小儿外科就诊并收入院。患儿既往健康,但最近有乏力、消瘦的病史,无肝炎、结核等传染病史及接触史,无外伤及手术史,无药物食物过敏史。     

Ocular manifestations of sickle cell anaemia in Arab children.

A complete ophthalmic examination was carried out of 46 Arab children with various forms of sickle cell disease. Mild and infrequent signs in anterior and posterior segments were found, but no cases of sickle cell retinopathy of any kind were detected. These results were compared with the reported findings in black American children of African origin with the same disease. The probable explanations were the high prevalence of fetal haemoglobin in Arab sicklers, the rarity of sickle cell disease among the Arabs and the possible existence of a different gene.     

Priapism and Fabry disease: A case report

A 10-year-old boy presented with priapism of 10h duration which after unsuccessful conservative measures, was relieved by a saphenocorporeal shunt. A4-year history of intermittent vague aching of fingers and toes accompanied by lowgrade fever was reported. Fabry disease was confirmed by the lack -galactosidase acitivity in serum and leucocytes. Other characteristic features of Fabry disease were not present. The condition was also diagnosed in his 14-year-old brother and suspected in his maternal grandfather who died at the age of 49 from renal failure of unknown aetiology.     

Value of serum ferritin estimation in sickle cell anaemia.

In a group of 35 children with sickle cell anaemia serum ferritin concentration ranged from 70 to 2460 microgram/l (mean 367, median 180 microgram/l). This was significantly higher than the ferritin levels (range 8-101, mean 34, median 30 microgram/l) in a group of 63 normal control children of the same age group. 30 (86%) of the sickle cell children showed serum ferritin levels greater than 101 microgram/l, and 2 (6%) levels greater than 1000 microgram/l. 7 of the patients had not been transfused before this study. Their serum ferritin levels were all raised and showed a significant correlation with age but not with haemoglobin level. In the remainder of the patients the serum ferritin bore no significant correlation with age, haemoglobin level, or number of units of blood transfused. 2 children with HbSC disease had levels within the control range. Since patients with sickle cell anaemia have an increasing chance of long survival, we suggest that serial estimations of their iron status be made by means of serum ferritin assay in order to determine which patients are accumulating excessive iron.     

Malarial parasitaemia in febrile children with sickle cell anaemia.

One-hundred-and-sixty-six episodes of fever in 162 children with sickle cell anaemia (SCA) aged 6 months to 16 years, presenting to the children emergency room of the University of Benin Teaching Hospital, Nigeria with rectal temperature greater than or equal to 38.3 degrees C were studied for malarial parasitaemia. Non-sicklers of similar ages and with similar temperatures were also studied as controls. Malarial parasitaemia was documented in 9 per cent of children with sickle cell anaemia, and 29 per cent of controls (P less than 0.0001). Bacteraemia occurred in 33 per cent of children with SCA and 26 per cent of controls (P greater than 0.10). Bacteraemia was, therefore, the commonest cause of pyrexia in febrile children with sickle cell anaemia on antimalarial prophylaxis. It is suggested that children with SCA on regular anti-malarial prophylaxis who present with significant pyrexia should be carefully screened for bacterial infection and appropriate anti-microbial therapy instituted.     

A clinico-epidemiological study of sickle cell anaemia in Saudi Arabia.

Ninety-nine Saudi Arabian children aged between 3 months and 15 years attending the sickle cell anaemia (SCA) clinic were studied using a review of case notes and an interview at clinic visits. All the patients come from the southwest and west area of Saudi Arabia. Despite the lack of infant screening for SCA, 73 per cent were diagnosed before the age of 3 years, reflecting severe early symptoms of the disease. The serious complications of SCA were two times higher, the number of admissions were three times higher and the attack rate for pneumococcal meningitis was nine times higher in our study population than previously reported from the Eastern Province of the country. Hand-foot syndrome was the commonest complication affecting 58 per cent of the children. It has been stated previously that there is no need to give penicillin prophylaxis to SCA children in Saudi Arabia, based on the observations in the Eastern Province only. Our study shows that SCA in western and southwestern population of Saudi Arabia is as severe as is described in American blacks. Therefore, we recommend penicillin prophylaxis to be given to the SCA children in the southwest and west regions of the country.     

Myocardial perfusion in children with sickle cell anaemia

Background. Myocardial ischaemia is an unexpected complication with potentially serious clinical damages in patients with sickle cell anaemia (SCA). Conventional techniques, such as exercise testing and echocardiography, have low sensitivity and specificity for the detection of myocardial ischaemia in patients with SCA. Objective. To assess myocardial perfusion using thallium-201 (²⁰¹Tl) single-photon emission computed tomography (SPECT) in children with SCA. Materials and methods. Eight patients (11.5 ± 5.0 years, mean ± SD) who were free of cardiac symptoms were studied. Myocardial perfusion was assessed by ²⁰¹Tl-SPECT after stress and 3 h later after a further injection. Left ventricular ejection fraction (LVEF) was assessed by equilibrium radionuclide angiography. Results. Myocardial perfusion was abnormal in three of eight patients: two had reversible defects and one had a fixed defect. The mean LVEF was 53 ± 8 %. There was no relationship between myocardial perfusion and LVEF. Conclusions. Treatment of asymptomatic myocardial ischaemia remains unclear, and more aggressive therapy of the haematological disease should be considered. Received: 17 October 1999 Accepted: 15 November 1999     

Bone marrow in sickle cell anaemia at time of anaemic crisis.

Samples of bone marrow from 33 Ghanaian children with homozygous sickle cell anaemia who presented with profound anaemia (haemoglobin less than 5 g/dl) were studied. The principal finding was depression of erythropoiesis (aplastic crisis) in 14 children and erythroid hyperplasia in 17. A splenic sequestration crisis was clinically diagnosed in the remaining two children. Stainable iron was absent in the marrow of 14 children and reduced in another five. Megaloblastic changes compatible with folate deficiency were present in 8 children. It is suggested that iron and folate deficiencies may complicate sickle cell anaemia in children living in geographical areas where nutritional deficiencies are prevalent.