الفهرس 
Sickle Cell AnaemiaOnly 14 pages are availabe for public view
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Abstract
Sickle cell anemia is an inherited disease characterized by the presence of an abnormal hemoglobin called hemoglobin S (HbS). During deoxygenation, the red blood cell (RBC) shape changes from the biconcave shape to the sickle shape due to the abnormal hemoglobin.
The clinical presentation of SCD is variable, with some patients having a normal life; however, some patients show increased morbidity and mortality due to severe thrombotic, aplastic, and sequestration crises. The vaso-occlusive crises are the commonest. Their etiology is usually attributed to low oxygen tension as in high altitude, water loss, and infection. Vaso-occlusion leads to severe pain especially in bones (hips, shoulders, and vertebrae) Infarcts of the small bones lead to painful dactylitis (hand-foot syndrome). It is usually the first presentation of the disease and may lead to digits of varying lengths.
Copeptin is secreted stoichiometrically from the neurohypophysis. It is produced in equimolar ratio to vasopressin. Copeptin has been shown to be more stable than vasopressin and its level is easy to determine. Copeptin has been shown to be elevated consequent upon major stress.
Complications of sickle cell disease varies from anemia to infections, vaso-occlusive crisis, acute chest syndrome, painful crisis, sequestration crisis, renal, cardiac, and hepatobiliary major complications. Infectious complications of sickle cell disease are a major cause of morbidity and mortality. In literature, there are limited date about the assessment of serum copeptin in sickle cell anemia and impact of complications on its level.
Therefore, the aim of this study was to assess serum copeptin level in pediatric patients with sickle cell anemia and the influence of complications on it. This cross-sectional control study was conducted out on sixty-two children in Pediatric Department, Menoufia University hospital from April 2021 to September 2022, and they were classified into two groups: group I (case group):
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thirty-one of pediatric patients diagnosed with sickle cell anemia by clinical and laboratory criteria as case group and group II (control group): thirty-one children who are apparently healthy with hemoglobin genotype (HbAA) (matched for age and gender) as control group.
All cases were subjected to the following:
Detailed history
Demographic data including (Age and Sex).
Duration of illness.
Frequency of blood transfusion.
Type of chelation therapy.
History of splenectomy.
Clinical examination
General examination.
Vital signs.
Central nervous system examination.
Cardiovascular system examination.
Abdominal examination.
Anthropometric Measurements:
Weight.
Height.
Body mass index.
Laboratory evaluation in the form of:
Complete blood count
Serum ferritin
Hb electrophoresis
liver function tests
renal function tests
Serum Copeptin level.
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Radiological investigations:
Echocardiographic examination was performed.
Summary of the results of this study:
There were no significant differences among the studied group regarding age, sex, history of splenectomy and age of onset (P>0.05)
There was a significant decrease among case than control group regarding weight, height, and body mass index (P<0.05)
Haemoglobin, haemoglobin A and haematocrit test were significantly decreased among case group than control group (P<0.001). While, fatal haemoglobin, haemoglobin S, white blood cells, serum ferritin and copeptin level were significantly increased among case group than control group (P<0.05). On the other hand, no significant differences among the studied groups regarding haemoglobin A2 and platelets (P>0.05).
Urea and creatinine were significantly increased among case group than control group (P<0.05). While no significant differences found among the studied groups regarding aspartate transaminase and alanine transaminase (P>0.05).
Urinary tract infection, bossing of the forehead, hepatomegaly, painful crisis, and hand foot syndrome were the most common complications among case the studied cases by 90%, 90%, 77%, 74%, and 74%) respectively (P<0.001). Also, copeptin level was significantly increased among patients with infections, painful crisis, acute chest syndrome, neurological complications, renal complications, bone complications and dermatological complications (P<0.05).