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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Multiple complications in sickle cell anaemia
Taimoor Khalid Janjua1, Syeda Amna Haider1, Naila Raza2
1Liaquat National Medical College.
Insights
Sickle Cell Disease (SCD) in a male adolescent led to severe malnourishment, causing hemolytic anemia, ascites, hepatomegaly, and fractures. Early intervention and proper management are crucial to prevent life-threatening complications in homozygous SCD.
Area of Science:
- Hematology
- Genetics
- Pediatrics
Background:
- Sickle Cell Disease (SCD) is a genetic blood disorder characterized by abnormal hemoglobin.
- It presents with diverse clinical manifestations and varying severity.
- The homozygous form (HbSS) can lead to severe, life-threatening complications if not managed properly.
Observation:
- A male adolescent with homozygous SCD presented with significant clinical issues.
- Key observations included hemolytic anemia, massive ascites, and hepatomegaly.
- Multiple fractures were also noted, indicative of severe underlying pathology.
Findings:
- The patient's symptoms were directly linked to severe malnourishment.
- Malnourishment exacerbated the effects of sickle cell disease, leading to organ damage and skeletal issues.
- This case highlights the critical role of nutrition in managing SCD complications.
Implications:
- This case underscores the importance of comprehensive care for homozygous SCD patients.
- Aggressive nutritional support is vital to mitigate severe manifestations like organomegaly and fractures.
- Improved management strategies are needed to prevent avoidable, life-threatening conditions in adolescents with SCD.
Abstract:
Sickle Cell Disease (SCD) is a structural haemoglobinopathy which is extremely diverse in its presentation regarding disease severity and organ involved. The homozygous form if poorly managed gives rise to numerous life threatening conditions which are otherwise avoidable. Here we report the case of a male adolescent with homozygous SCD who presented with haemolytic anaemia, massive ascites, hepatomegaly and multiple fractures secondary to severe malnourishment associated with the disease.
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