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1.
International journal of surgery ; 7(1): 70-73, Feb 2009.
Article in English | MedCarib | ID: med-17723

ABSTRACT

OBJECTIVES: To report the experience of performing laparoscopic cholecystectomy (LC) in patients suffering from sickle cell disease (SCD), and to assess if their postoperative complications can be minimized by shortening the operating time. METHODS: Strict measures were taken to minimize the operating times and duration of pneumoperitoneum in SCD patients undergoing LC. Data collected included demographics, preoperative haemoglobin, the surgical technique used, operating times, insufflation pressures, perioperative complications and hospital length of stay. RESULTS: In the 5-year period from July 2003 to June 2008, 19 patients with SCD underwent elective LC. Of these, 84 per cent were female. The mean age was 21.5 years. The most common indication for surgery was symptomatic cholelithiasis (60 per cent). The mean preoperative haemoglobin was 8.2g/dL. No preoperative blood transfusions were given. Four patients had preoperative endoscopic retrograde cholangiopancreatography (ERCP). Mean operating time was 27.9 min (range 20-45 min) which is 2.5-6 times quicker than most reports in the literature. There was no conversion to open surgery. Mean hospital length of stay was 2.5 days. Postoperative complications were noted in four patients with painful crises accounting for 50 per cent. There was no mortality. CONCLUSION: The study suggests that if stringent measures are taken to shorten the operating time, LC can be safely and effectively performed in SCD patients with minimal perioperative complications.


Subject(s)
Humans , Cholecystectomy, Laparoscopic , Anemia, Sickle Cell , Surgical Procedures, Operative , Trinidad and Tobago
2.
Clinical laboratory hematology ; 28(5): 299-302, May 2006. graf
Article in English | MedCarib | ID: med-17640

ABSTRACT

We observed consecutive hospital admissions for acute painful crisis (APC) among adults with Sickle Cell Disease (SCD) over a 6-month period in Trinidad and Tobago. Episodes (111) of APC resulted in 82 admissions of 59 patients. The most common site for pain was the trunk. Patients ranged in age from 17 to 53 years (median: 25). Median length of hospital stay was 4 days. Total dose of Pethidine given per admission ranged from 100 to 1650 mg (median: 525). The mean dose of morphine was 70 mg. Six (7%) of patients were readmitted within 10 days of discharge. Twenty-five (30%) of patients had chest pain at presentation of whom 10 (12%) had consolidation on chest X-ray, defining the acute chest syndrome (ACS). There was one death caused by biliary sepsis. The study revealed seemingly low opiate usage for in-hospital treatment of APC with acceptable rates of readmission. The BCSH 2003 guidelines seemed applicable apart for the choice and route of fluid for rehydration and opiate analgesia.


Subject(s)
Adolescent , Adult , Middle Aged , Aged , Humans , Male , Female , Pain Clinics , Pain , Anemia, Sickle Cell , Trinidad and Tobago
3.
Arch Dis Child ; 85(5): 375-8, Nov. 2001. tab, gra
Article in English | MedCarib | ID: med-48

ABSTRACT

AIMS: (1) To investigate the distribution of age at menarche in a representative sample of 99 patients with homozygous sickle cell disease (ss), 69 with sickle cell haemoglobin C (sc) disease, and 100 controls with a normal haemoglobin (AA) genotype followed in a cohort study from birth. (2) To explore the determinants of the age menarche. METHODS: Children ascertained in a new-born screening programme were followed prospectively from birth to age 18-26.5 years with regular assessments of height, weight, puberal stage, and haematological indices at the Sickle Cell Clinic of the University Hospital of the West Indies. RESULTS: All subjects have now reached menarche and the mean age in normal controls (13.0 years) was significantly earlier than in SC disease (13.5 years) or SS disease (15.4 years). Greater weight and earlier age at menarche was the only association significant across all genotypes although additional contributions occured from fetal haemoglobin and red cell count in SS disease. Alpha thalassaemia, which ameliorates many of the effects of SS disease, had no discernible effect on menarche. CONCLUSIONS: Mean age at menarche is delayed by 0.5 years in SC disease and by 2.4 years in SS disease. Weight appears to be the principle determinant of age at menarche. (AU)


Subject(s)
Child , Female , Humans , Infant, Newborn , Adolescent , Anemia, Sickle Cell/complications , Menarche , Puberty, Delayed/etiology , Anemia, Sickle Cell/blood , Anemia, Sickle Cell/physiopathology , Puberty, Delayed/blood , Puberty, Delayed/physiopathology , Jamaica/ethnology , Follow-Up Studies , Prospective Studies , Age Distribution , Body Weight , Growth , Hemoglobin SC Disease/blood , Hemoglobin SC Disease/complications , Hemoglobin SC Disease/physiopathology , Linear Models
4.
West Indian med. j ; 50(Suppl 5): 12, Nov. 2001.
Article in English | MedCarib | ID: med-268

ABSTRACT

OBJECTIVE: Universal neonatal screening for sickle cell disease using cord blood has taken place in the University Hospital of the West Indies (UHWI) since 1997. Comprehensive quality control of the screening programme has not been implemented. Our aim was to determine how many deliveries were left unscreened, and to ascertain factors contributing to failure to screen for sickle cell disease. METHODS: All live births in 1999 entered in the delivery book of the Labour Ward, UHWI, were scrutinized against the sample book of the Neonatal Screening Laboratory, Sickle Cell Unit. Identified unscreened infants were matched with a randomly chosen control, born on the same day. Time and mode of delivery, persons assisting the delivery, birth weight, level of urgency, location of delivery and Apgar scores at 1 and 5 minutes were collected for unscreened infants and screened controls. Secular variation within the year was assessed by comparing the frequency of unscreened births in each month to the previous month. Frequencies of potential determinants of failure to screen were tabulated. The independent predictive power of these potential determinants was assessed using conditional logistic regression, to account for correlations between predictors, and for the case-control design of the study. RESULTS: Of 2,763 live births in 1999, 139 (5 percent) infants were not screened for sickle cell disease. There was no statistically significant secular variation within the year (relative risk 1.02, 95 percent confidence interval 0.97, 1.07, x2 = 0.77, p = 0.38). Independently significant predictors of failure to screen were: emergency delivery (odds ratio 4.8, 95 percent confidence interval 2.3, 10.0), nigttime delivery (OR2.5, 0.9) CONCLUSIONS: This study identified that 5 percent of infants born in UHWI in 1999 were discharged from hospital without having been screened for sickle cell disease. Statistically significant associations with failure to screen were found to be emergency delivery, night-time delivery, and low 5 minute Apgar score. The risk of being left unscreened increased 5-fold for emergency deliveries. Increased awareness of these risk factors may reduce failure to screen. Of course, such effort does not obviate the need for regular quality control, including timely identification and tracing of unscreened infants. (AU)


Subject(s)
Humans , Infant, Newborn , Anemia, Sickle Cell/prevention & control , Neonatal Screening , Jamaica , Data Collection , Hospitals, University
5.
Br J Haematol ; 113(3): 661-5, Jun. 2001. tab, gra
Article in English | MedCarib | ID: med-109

ABSTRACT

The transfusion history and frequency of red cell antibodies in patients with homozygous sickle cell (SS) disease have been compared in 190 subjects from the Jamaican cohort study and 37 patients attending a sickle cell clinic in Manchester, England. The proportion of patients transfused did not differ between the groups although the number of units transfused and the frequency of red cell antibodies were significantly greater in the Manchester group. Immune antibodies occurred in three Jamaicans (2.6 percent of those transfused) and 16 UK subjects (76 percent of those transfused). Multiple antibodies occurred in 10 (63 percent) UK subjects but in no Jamaicans. Indications for transfusion also differed between the groups, Jamaican patients typically receiving 1-2 units for acute anaemia or acute chest syndrome, whereas UK patients frequently had multiple transfusions in preoperative exchange or prophylaxis programmes. The greater red cell alloimmunization among UK patients probably reflects both the greater use of transfusion and the disparity between donor and recipient populations in the UK. (AU)


Subject(s)
Adult , Female , Humans , Male , Comparative Study , Adolescent , Anemia, Sickle Cell/ethnology , Anemia, Sickle Cell/immunology , Blood Group Antigens/immunology , Blood Transfusion/adverse effects , Isoantibodies/blood , Anemia, Sickle Cell/therapy , Blood Transfusion/statistics & numerical data , Chi-Square Distribution , Cohort Studies , Jamaica , United Kingdom , Statistics, Nonparametric
6.
Arch dis child ; 84(2): 156-9, Feb. 2001. tab
Article in English | MedCarib | ID: med-116

ABSTRACT

OBJECTIVE: To investigate the cause and outcome of high fever in Jamaican children with homozygous sickle cell disease. DESIGN: Retrospective review of febrile episodes in a three year period (1 September 1993 to 31 August 1996). SETTING: Sickle Cell Clinic, an outpatient clinic in Kingston run by the Medical Research Council Laboratories (Jamaica). PATIENTS: Patients with homozygous sickle cell disease under 17 years of age presenting with an anxillary temperature o 39.0§c (102.4§F). MAIN OUTCOME MEASURES: Diagnosis, death. RESULTS: There were 165 event in 144 patients (66 (45.8 percent) boys) with a median age of 6.1 years. Bacteraemia was found in 10 (6.1 percent) events (three Streptococcus pneumoniae, two Haemophilus influenzae type b, two Salmonella sp, one Escherichia coli, one Enterobactor sp, and one Acinetobacter sp), and urinary tract infections in four (2.4 percent). All cultures of cerebrospinal fluid were sterile. Acute chest syndrome occured in 36 (21.8 percent) events. A painful crisis was associated with 45 (27.3 percent) events and was the only pathology identified in 20 events (12.1 percent). Hospital admission was necessary in 66 cases including all those with bacteraemia and 31 with acute chest syndrome. There were two deaths: a 5 year old boy with septic shock associated with H influenzae septicaemia, and a 3 year old boy with the acute chest syndrome. CONCLUSIONS: Painful crisis and acute chest syndrome were the most common complications associated with high fever, but other important associated features included bacteraemia and urinary tract infection. Enteric Gram negative organisms accounted for 50 percent of positive blood cultures. (AU)


Subject(s)
Child , Child, Preschool , Female , Humans , Male , Adolescent , Infant , Anemia, Sickle Cell/complications , Fever/etiology , Anemia, Sickle Cell/genetics , Bacteremia/etiology , Homozygote , Length of Stay , Lung Diseases/etiology , Pain/etiology , Regression Analysis , Retrospective Studies , Syndrome , Urinary Tract Infections/etiology
7.
J Pediatr ; 138(1): 65-70, Jan. 2001. tab
Article in English | MedCarib | ID: med-117

ABSTRACT

OBJECTIVES: To examine the clinical features and epidemiology of invasive pneumococcal disease in homozygous sickle cell (SS) disease and the efficacy of pneumococcal prophylaxis. STUDY DESIGN: A retrospective study of 80 episodes in 68 patients in the Jamaican Sickle Cell Clinic in a 25-year period (1973-1997). RESULTS: Clinical features included a history of fever (94 percent), vomitting (70 percent), an ill appearance (80 percent), fever (89 percent), abnormal chest signs (43 percent), and meningismus (39 percent). There were 14 deaths - 13 among 68 initial episodes (6 of which were deaths on arrival) and one death during a recurrence. Thirteen episodes occurred in patients who should have been receiving antibiotic prophylaxis. Ten were due to failure to adhere to protocols, and 3 occurred during prophylaxis; one patient was receiving oral erythromycin, and two had received injections of benzathine penicillin 4 and 24 days before the episode. All but one of the pneumococcal isolates were susceptible to penicillin. The 32 patients who received pneumococcal vaccine had more mild clinical courses as indicated by a greater chance of being treated as outpatients or surviving after admission (Mann-Whitney U test, P = .03). CONCLUSIONS: Penicillin remains the mainstay of prophylaxis, although breakthroughs occur and will become more common with the increasing frequency of penicillin-resistant organisms. Pneumococcal immunization appears to ameliorate the course of invasive disease. (AU)


Subject(s)
Child , Child, Preschool , Female , Humans , Male , Infant , Adolescent , Anemia, Sickle Cell/complications , Pneumococcal Infections/etiology , Pneumococcal Infections/prevention & control , Jamaica/epidemiology , Pneumococcal Infections/epidemiology , Antibiotic Prophylaxis , Anti-Bacterial Agents/therapeutic use , Outpatient Clinics, Hospital , Retrospective Studies , Treatment Outcome , Vaccination , Survival Analysis , Recurrence , Hospitals, University
8.
Trop Doct ; 30(4): 214-6, Oct. 2000. tab
Article in English | MedCarib | ID: med-129

ABSTRACT

Twenty-seven children aged 18 years and under with homozygous sickle-cell disease had open cholecystectomy for symptomatic gallstones over the 12 year period 1985-1997. Emergency procedures (done during period of acute exacerbation of symptoms) were performed on 16 patients. Four with haemoglobin levels greater than 1g/dl below their steady state received a simple blood transfusion preoperatively designed to raise haemoglobin levels to 10g/dl. All had acute or acute on chronic cholecystitis based on histological examination of gallbladder specimens. Twelve had common bile duct stones. In two patients calculi were missed intraoperatively but these subsequently passed into the duodenum after a period saline irrigation via an in-situ t-tube. Six developed the acute chest syndrome (aetiology not determined) and this progressed to multi system failure and death in one. This high level of postoperative mobility and mortality may in part be due to the high proportion of emergency procedures. (AU)


Subject(s)
Child , Female , Humans , Male , Adolescent , Anemia, Sickle Cell/complications , Child Health/statistics & numerical data , Cholecystostomy/statistics & numerical data , Cholelithiasis/epidemiology , Cholelithiasis/surgery , Cholecystostomy/mortality , Cholelithiasis/etiology , Emergency Treatment , Jamaica/epidemiology , Prevalence
9.
West Indian med. j ; 50(3): 28, July, 2001.
Article in English | MedCarib | ID: med-214

ABSTRACT

Sickle cell disease is the commonest haemo-globinopathy. The gene is present in about 8 percent of Afro-American and 10 percent of Afro-Jamaican. Severe ocular complications of this condition include proliferative retinopathy, vitreous haemorrhages, and retinal detachment ultimately leading to blindness in a number of cases. There are not many reports of vitreo-retinal surgery in the literature dealing with cases. This is a report of 28 vitero-retinal surgeries in 20 eyes of 19 patients with sickle cell eye disease. Four eyes had surgery for viterous haemorrage, 16 for retinal detachment. All four eyes with vitreous haemorrage had successful pars plana vitrectomy (PPV) with full restoration of full potential vision. Eight of 16 eyes had successful retinal detachment surgery. Four eyes had pneumatic retinopexy with one unsuccessful result. Five eyes had scleral buckles with four having successful results, and six had bad results and one was unchanged. Six of ten eyes that had pars plana virectomy for retinal detachment had bad results. The unsuccessful cases were difficult ones with extensive proliferative vitero-retinopathy and long-standing retinal detachment. (AU)


Subject(s)
Humans , Anemia, Sickle Cell/complications , Vitreoretinopathy, Proliferative/surgery , Retina/surgery , Jamaica , Vitreous Hemorrhage/complications , Retinal Detachment/complications , Vitrectomy
10.
West Indian med. j ; 49(suppl. 2): 61, Apr. 2000.
Article in English | MedCarib | ID: med-884

ABSTRACT

OBJECTIVE: To determine whether pain severity scores (visual analogue scale (VAS) from 0-10) and/or grades (none, mild, moderate or severe) for bone pain in children with sickle cell disease, agreed when performed by patients, doctors and nurses, and to determine whether the pain scores and/or grade assigned predicted the use of narcotics or a return visit for the same pain episode. DESIGN AND METHODS: All children aged 5-18 years known to have homozgous SS disease who attended the Sickle Cell Clinic at the University Hospital of the West Indies, and were admitted to the day care center with painful crisis, from 25/04/1999 to 01/06/1999 were invited to participate. Informed consent was obtained. All patients/guardians consented, leading to observation of 30 episodes in 22 children (four children had 2 episodes, two children had 3 episodes). On admission and discharge, pain severity ws confidentially and independently assigned a VAS score and grade by patient, doctor and nurse. Medicatons used for pain were recorded. Patients with return visits within 7 days were noted. Scores/grades by each individual at admission and discharge were compared to the other 2 scores/grades yielding kappa values. The distribution of scores across grades was determined. VAS scores and grades at patient admission and discharge were compared across patient groups defined by subsequent return visit to clinic, using the Mann-Whitney two-sample statistic. RESULTS: Comparison of VAS scores between rates yielded kappas ranging from 0.40 to 0.69, demonstrating fair to substantial agreement, wheras with grades, kappas ranged from 0.52 to 0.90, demonstrating moderate to near perfect agreement. The minimum score associated with norcotic use was 5. The scores/grades at the initial visit did not predict those who would have return visits (po0.4). CONCLUSIONS: The visual analogue scale and grading system can successfully be used in this clinical setting. In this pilot study, doctors and nurses correlated well with patients in scoring the pain. There is a minimum threshold of a score of 5 below which narcotics are not prescribed. Return visits are determined by factors other than the score at initial visit. Further work using this type of tool is to be encouraged.(AU)


Subject(s)
Child , Child, Preschool , Humans , Adolescent , Anemia, Sickle Cell/complications , Pain Measurement/methods , Jamaica/epidemiology , Receptors, Opioid, kappa , Patient Compliance
11.
West Indian med. j ; 49(Supp 2): 39, Apr. 2000.
Article in English | MedCarib | ID: med-948

ABSTRACT

We determined optimal folate, vitamin B12 and vitamin B6 dosages in 21 sickle cell disease (SCD) patients (11 HbSS, 10 HbSC; mean 7 years, range 7-16), using plasma homocysteine (Hcy) as functional marker. They received daily 400 g (0-3 weeks), 700 g (3-6) and 1000 g (6-70) folate; 1 (0-21), 3 (21-45 and 5 RDA (45-70) vitamin B12; and 1 RDA vitamin B6 (0-70). Blood was taken at baseline (P0) and after 3 (PI), 6 (P2), 9 (P3), 21 (P4), 33 (P5), 45 (P6), 57 (P7) and 70 (P8) weeks for measurement of erythrocyte (RBC), serum folate, plasma vitamin B12, whole blood vitamin B6 and plasma Hcy. Vitamin B6 increased from P0 to P1 and P1 to P2; vitamin B12 from P4 to P8; serum folate from P0 to P1 and P1 to P2; RBC folate from P0 to P1, P1 to P2 and P2 to P3. Hcy decreased from P1 to P2 and P4 to P6. Most pronounced Hcy decreases occurred from P0 to P1 (43 percent of patients), P1 to P2 (14 percent) and P4 to P5 (24 percent). Haematological indices did not change. Patients with HbSS had higher RBC folate at P1, P2 and P8. The entire group exhibited inverse relations between RBC folate and haemoglobin on P1, P2, P3, P6, P7 and P8. We conclude that RBC folate is less valuable for folate status assessment in SCD patients. The optimal daily supplement is 700 g folate (3.5-7 RDA vitamin B12 (4.2-6.0 g) and 1 RDA vitamin B6 (1.4-2.0 mg). This combination causes Hcy levels that do not decrease further upon higher dosages and may reduce by simple and relatively inexpensive means their inherently high risk of endothelial damage.(Au)


Subject(s)
Child , Humans , Anemia, Sickle Cell/blood , Vitamin B 12 Deficiency/diet therapy , Vitamin B 6 Deficiency/diet therapy , Pteroylpolyglutamic Acids/deficiency , Data Collection
12.
Arch Dis Child ; 82(3): 204-8, Mar. 2000.
Article in English | MedCarib | ID: med-866

ABSTRACT

Objective: To derive height and weight growth reference curves for children with homozygous sickle cell disease. STUDY DESIGN: Subjects (n = 315) were participants in a population based, longitudinal cohort study of sickle cell disease in Kingston, Jamaica. Regular measurements of height and weight were made from enrolment into the study at birth up to 22 years of age. RESULTS: Sex specific growth reference curve for height for age and weight foir age covering the age range 0-18 years are presented. CONCLUSIONS: These growth reference curves are suitable for identifying coincidental growth problems in children with homozygous sickle cell disease.(AU)


Subject(s)
Adult , Child , Child, Preschool , Infant , Female , Humans , Male , Infant, Newborn , Adolescent , Anemia, Sickle Cell/physiopathology , Body Height/physiology , Body Weight/physiology , Jamaica , Follow-Up Studies , Reference Values , Sex Factors
13.
West Indian med. j ; 49(1): 52-4, Mar. 2000. tab
Article in English | MedCarib | ID: med-1075

ABSTRACT

The presence of a chronically ill family member may adversely affect the psychological health of siblings. This study used the General Health Questionnaire and the Modified Social Adjustment Scale to assess psychological distress in 20 younger siblings (4 AA, 16 AS genotypes), aged 16-19 years, of patients with homozygous sickle cell (SS) disease. The results were compared with those previously obtained in the 20 older siblings with SS disease and in 89 controls with a normal haemoglobin (AA) genotype. High levels of psychological distress occurred among all three groups. Greater psychological distress and poorer social adjustment occurred among siblings compared to AA controls but these differences disappeared after adjusting for the reduced age of sibings. The two measures were similar in SS patients and AA controls. The level of psychological distress among siblings of SS patients did not differ from that in SS patients or AA controls.(Au)


Subject(s)
Humans , Adolescent , Adult , Female , Male , Stress, Psychological , Anemia, Sickle Cell/psychology , Nuclear Family/psychology , Jamaica/epidemiology , Cohort Studies , Age Factors , Genotype , Hemoglobins , Homozygote , Sibling Relations
14.
J Pedriatr ; 136(1): 80-5, Jan. 2000.
Article in English | MedCarib | ID: med-749

ABSTRACT

The prevalence, incidence, risk factors, clinical associations, and morbidity of gallstones were studied in 311 patients with homozygous sickle cell disease and 167 patients with sickle cell-hemoglobin C disease in a cohort study from birth. Gallstones developed in 96 patients with homozygous sickle cell disease and 18 patients with sickle cell-hemoglobin C disease; specific symptoms necessitating cholecystectomy occurred in only 7 patients with homozygous sickle cell disease.(AU)


Subject(s)
Adult , Child , Humans , Male , Female , Adolescent , Anemia, Sickle Cell/epidemiology , Cholelithiasis/epidemiology , Hemoglobin SC Disease/epidemiology , Anemia, Sickle Cell/genetics , Anemia, Sickle Cell/mortality , Cholecystectomy , Cholelithiasis/complications , Cholelithiasis/surgery , Cohort Studies , Follow-Up Studies , Hemoglobin SC Disease/mortality , Homozygote , Incidence , Jamaica/epidemiology , Population Surveillance , Prevalence , Risk Factors , Survival Rate
15.
Kingston; s.n; 2000. 55 p. tab, gra.
Thesis in English | MedCarib | ID: med-486

ABSTRACT

Sickle cell disease is a global health problem. It is an inherited blood disorder affecting the red blood cells. There are different types of sickle cell disease. Homozygous sickle cell (SS) disease, results when the infant inherits two abnormal sickle genes, one from each parent. In Jamaica SS disease affects approximately 1 in 300 births and some form of sickle cell disease, 1 in 150 births. Complications of sickle cell disease include acute splenic sequestration. The cause and triggers of acute splenic sequestration (ASS) are largely unknown. The spleen becomes acutely enlarged, trapping a proportion of the red cell mass and leading to acute anaemia, circulatory failure and sometimes death. Emergency blood tranfusion is extremely important in the management of acute splenic sequestration. Parental education may allow prevention of death from ASS by early transfusion. Parents need to be taught how to diagnose splenic enlargement, and to recognize the signs and symptoms of increasing anaemia characterized by pallor. It is important for mothers to examine splenic size on a regular basis and when the child looks ill. At the Jamaican Sickle Cell Unit, parents are instructed to examine the spleen at least once a day. The aim was to identify the parents' and guardians' knowledge of ASS, their attitudes towards the examination of the abdomen and practices in detecting an enlarging spleen. The objectives were to determine the percentage of parents and guardian who regularly examined the abdomen to determine spleen size; who had detected an enlarging spleen and the outcome; who were aware of the proper technique for examining the spleen; who were aware of what to do if an enlarging spleen was found; and identify the barriers that prevent regular examination for splenic enlargement. The study was carried out on the parents and guardians of children four years and younger, with homozygous sickle cell disease, attending the sickle cell unit. At the time of the study, the population of children four years and younger with SS disease was 23.7. A questionnaire was designed based on the objectives of the study, to solicit data from the partcipants. The researcher administered the questionnaire to 60 parents and guardians who attended the clinic over a two-month period- March and April 2000. The participants were also asked to demonstrated their competence in examining the spleen.(Au)


Subject(s)
Adult , Child , Child, Preschool , Middle Aged , Aged , Humans , Infant , Adolescent , Splenic Diseases/diagnosis , Anemia, Sickle Cell/complications , Health Knowledge, Attitudes, Practice , Jamaica , Spleen/abnormalities , Parents/education , Hypersplenism , Splenic Diseases/prevention & control , Cohort Studies
16.
Ned Tijdschr Geneeskd ; 143(41): 2049-53, Oct. 9, 1999.
Article in English | MedCarib | ID: med-759

ABSTRACT

OBJECTIVE: To determine the differences in quality of life between children with sickle cell disease and healthy immigrant children. DESIGN: Descriptive, comparative. METHOD: The quality of life of children with sickle cell disease between 5 and 15 years old being treated in the Emma Children's Hospital AMC in Amsterdam, the Netherlands, was assessed by using a questionnaire for parents (TNO-AZL Children's Quality of Life Questionnaire (TACQOL) parent form) if the child was between 8 and 15 years old. The study period was April-October 1998. The questionnaires were completed by 45 (parents of) patients. The results were compared with a healthy reference group of immigrant children. Statistical analysis was performed using the Student t-Test. RESULTS: Children with sickle cell disease as well as their parents scored signifcantly lower on the items general physical, motor and independent daily functioning and on occurrence of negative emotions. No significance was observed for the items cognitive functioning and school performance nor for social functioning or occurrence of positive emotions. CONCLUSIONS: In children, sickle cell disease leads to compromised physical and possibly also psychological wellbeing, as well as the experience of decreased independence in daily functioning, but not to compromised cognitive or social aspects of the quality of life. (AU)


Subject(s)
Child , Comparative Study , Female , Humans , Male , Adolescent , beta-Thalassemia/psychology , Anemia, Sickle Cell/psychology , Quality of Life/psychology , Africa , Caribbean Region/ethnology , Case-Control Studies , Hemoglobin SC Disease/psychology , Netherlands/epidemiology , Parents/psychology , Surveys and Questionnaires , Suriname/ethnology
17.
West Indian med. j ; 48(3): 132-6, Sept. 1999. tab, gra
Article in English | MedCarib | ID: med-1498

ABSTRACT

The electrocardiogram remains the most commonly used method of cardiac assessment in developing countries. To determine the prevalence of electrocardiographic left ventricular hypertrophy (LVH) and the clinical significance of Sokolow-Lyon voltage criteria in sickle cell patients, echocardiographic and ECG findings were studied in 112 patients (71 with haemoglobin SS disease and 41 with haemoglobin SC disease). Electrocardiographic left ventricular hypertrophy (ECGLVH) defined as Sokolow-Lyon voltage greater than or equal to 35 mm was detected in 39 (55 percent) SS patients and 11 (27 percent) SC patients. This prevalence was higher in men than in women. There were statistically significant trends for increasing prevalence of ECGLVH with height (p<0.007 in SS, and p<0.01 in SC patients). But no significant trend was found with increasing posterior wall (PWT) or interventricular septal thickness (IVST). Sensitivity of Sokolow-Lyon criteria for detection of echocardiographic left ventricular hypertrophy was 63 percent and 33 percent in SS and SC patients, respectively, and specificity was 51 percent and 74 percent, respectively. Sokolow-Lyon voltage correlated with left ventricular mass in SS and SC patients (r = 0.44, p < 0.01 and r = 0.32, p < 0.05) and with left ventricular internal dimension (r = 0.2, p < 0.01 and r = 0.32, p < 0.05) but not significantly with PWT and IVST. We conclude that, in sickle cell patients, the electrocardiographic LVH mainly indicates the existence of an eccentric echocardiographic LVH with increase of left ventricular internal dimension.(AU)


Subject(s)
Adult , Female , Humans , Male , Patch-Clamp Techniques/statistics & numerical data , Anemia, Sickle Cell/therapy , Electrocardiography , Data Interpretation, Statistical , Echocardiography , Guadeloupe
18.
J Pediatr ; 134(3): 304-9, Mar. 1999.
Article in English | MedCarib | ID: med-1405

ABSTRACT

OBJECTIVE: To determine whether children with homozygous sickle cell (SS) disease and splenectomy are at greater risk of death, overwhelming septicemia, or other complications. METHODS: A total of 130 patients with SS treated by splenectomy (46 recurrent acute splenic sequestration, 84 chronic hypersplenism) over a 22.5-year period at the Sickle Cell Clinic of the University Hospital of the West Indies, Kingston, Jamaica, were compared with a control group matched for sex, age, and duration of follow-up in a retrospective review. Deaths and bacteremias were examined over the whole study period. Painful crises, acute chest syndromes, and febrile episodes were compared in the 90 patients completing 5 years of postsplenectomy follow-up. FINDINGS: Mortality and bacteremic episodes did not differ between the splenectomy and control groups. Painful crises were more common in the splenectomy group than in the control group (P = .01) but did not differ between splenectomy indications. Acute chest syndrome was more common in the splenectomy group than in the control group (P < .01) and was more common in the acute splenic sequestration group than in the hypersplenism group (P = .01). Febrile events did not differ between the groups or between the indications for splenectomy. CONCLUSION: Splenectomy does not increase the risk of death or bacteremic illness in patients with SS disease and, if otherwise indicated, should not be deferred for these reasons (Au)


Subject(s)
Adult , Adolescent , Child , Child, Preschool , Female , Humans , Male , Infant , Comparative Study , Anemia, Sickle Cell/complications , Homozygote , Splenectomy/standards , Antibiotic Prophylaxis , Bacteremia/epidemiology , Bacteremia/prevention & control , Case-Control Studies , Cause of Death , Chi-Square Distribution , Follow-Up Studies , Jamaica/epidemiology , Logistic Models , Penicillins/therapeutic use , Retrospective Studies , Risk Factors , Anemia, Sickle Cell/mortality , Anemia, Sickle Cell/surgery
19.
Br J Haematol ; 104(1): 93-6, Jan. 1999.
Article in English | MedCarib | ID: med-1406

ABSTRACT

In the U.K. and the U.S.A., painful crises account for 80-90 percent of sickle-related hospital admissions, with average durations of 5-11 d. In Jamaica, many severe painful crises are managed in a day-care centre. Patients (n = 1160) with homozygous sickle cell (SS) disease aged 18 years and over were registered with the clinic during a 1-year study period. Of these, 216 patients with 476 painful crises attended the day-care facility for a total of 688 d. Most patients (119 or 55.1 percent) had single crises and for most crises (338 or 71 percent), patients attended for only 1 d, when they were given bed rest, assurance, rehydration and analgesia. Patients with complicated painful crises were usually referred for admission after initial pain relief and the rest were monitored during the day. In the evening they were given the option of hospital admission or allowed home with oral analgesia. Hospital admission for complicated painful crises or inadequate pain relief occurred in 42 (8.8 percent) crises and home management in 434 (91.2 percent) crises. Of 186 patients initially selecting home management, 20 percent returned for further day-care and five (2.7 percent) died during subsequent admission for that painful crisis, one without other known complications, two with acute chest syndrome (one associated with Salmonella septicaemia), another with Salmonella septicaemia, and one with dengue haemorrhagic fever. With suitable oral analgesia, adequate education and support, the majority of severe painful crises in SS disease in Jamaica have been managed on an outpatient basis. This model of patient care may merit assessment in other communities where painful crises are a common clinical problem.(Au)


Subject(s)
Adult , Female , Humans , Male , Anemia, Sickle Cell/therapy , Day Care, Medical/organization & administration , Home Care Services/statistics & numerical data , Jamaica , Pain/etiology , Pain/prevention & control , Day Care, Medical/statistics & numerical data
20.
Br Dent J ; 185(2): 90-2, July 25, 1998.
Article in English | MedCarib | ID: med-1652

ABSTRACT

AIM: the aim of this study was to investigate the prevalence of orofacial pain in a large group of sickle cell disorder (SCD) sufferers. METHODS: In 1996, 51 Jamaican adults and teenagers with SCD and 51 matched subjects (non-SCD) without any sickle cell problems were questioned about their experience of pian in the maxilla or mandible, and pain in their teeth. The subjects, aged 13 to 45 years, had oral examinations and the state of the teeth was recorded together with the presence or absence of deep periodontal pockets, vertical or horizontal mobility, sepsis, trauma and servere erosion. RESULTS: Mandibular or maxillary pain during the previous 12 months was reported by 49 percent of those with SCD and only 8 percent of the non-SCD subjects. Among the SCD group reporting this pain, 68 percent were found to have no dental problems which could have caused the pain. Dental disease accounted for the facial pain in all non-SCD subjects. Headaches were experienced by 77 percent of the SCD group and only 47 percent of the non-SCD group. Experience of toothache during the previous year was reported by 67 percent of the SCD group and 57 percent of the non-SCD. In half on these SCD subjects there was no obvious dental pathology to account for the dental pain but none of the controls had dental pin in the absence of dental pathology. CONCLUSION: People who suffer from sickle cell disorders are likely to suffer orofacial and dental pain in the absence of dental pathology and this is probably because of sickling crises within the microcirculation of the facial bones and dental pulps and small areas of necrosis in the bone marrow(AU)


Subject(s)
Adolescent , Adult , Humans , Middle Aged , Anemia, Sickle Cell/complications , Facial Pain/etiology , Toothache/etiology , Anemia, Sickle Cell/epidemiology , Case-Control Studies , Dental Care for Chronically Ill , Headache/etiology , Jamaica/epidemiology , Prevalence , Surveys and Questionnaires
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