Related Experiment Video

Updated: Mar 14, 2026

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
05:23

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload

Published on: March 14, 2017

20.6K

Clinical Course of Two Children with Unstable Hemoglobins: The Effect of Hydroxyurea Therapy

Harriët M Loovers1, Nienke Tamminga2, André B Mulder1

  • 1a Department of Laboratory Medicine, Department of Hematology , University Medical Center Groningen (UMCG) , the Netherlands.

Hemoglobin
|October 1, 2016
PubMed

Case reports on the effect of hydroxyurea (HU) therapy for unstable hemoglobins (Hbs) are sparse; only three adult cases have been reported. We report for the first time on the effect of HU therapy in children carrying unstable Hbs. The first case concerns a female child with a familial history of chronic hemolytic anemia. She was diagnosed with Hb Volga (HBB: c.83C>A) at the age of 7 months. At age 6, treatment options were reconsidered due to increasing fatigue and decreasing Hb concentration. The second case also concerns a female child with chronic hemolytic anemia and icterus since the age of 5. She was diagnosed with Hb Köln (HBB: c.295G>A) at the age of 9. At age 10, treatment options were reconsidered due to decreased general condition and poor school performance. Both children were started on HU therapy. The child with Hb Volga showed reduced clinical symptoms and increased average Hb concentrations. She has been on HU therapy for over 7 years at preparation of this manuscript. The child with Hb Köln showed decreasing Hb concentrations upon start of therapy; clinical symptoms did not improve. Therapy was discontinued after 3½ months. The Hb Volga case report suggests that HU therapy could improve clinical symptoms in some patients with unstable Hbs. Based on these and previously published cases, it was speculated that response can be predicted by the percentage of Hb F and reticulocyte counts.

Keywords:
Hb KölnHb VolgaHemoglobinopathyhydroxyurea (HU)pediatricunstable hemoglobin

More Related Videos

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy07:24

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy

2.0K
Modeling Posthemorrhagic Hydrocephalus of Prematurity in Rats04:12

Modeling Posthemorrhagic Hydrocephalus of Prematurity in Rats

Published on: March 28, 2025

658

Related Concept Videos

Disorders of Erythrocytes01:27

Disorders of Erythrocytes

Disorders of erythrocytes, or red blood cells (RBCs), include a range of conditions affecting their number, shape, or function.
Erythrocyte disorders can be broadly categorized into two main types: anemic and polycythemic conditions.
A low oxygen-carrying capacity of the blood due to the loss, lower production, or destruction of erythrocytes is termed anemia. Hemorrhagic anemia, for example, occurs when bleeding from an external wound or internal ulcer reduces erythrocyte counts.
On the other...
2.7K
Lifecycle of Erythrocytes01:22

Lifecycle of Erythrocytes

Erythrocytes, also known as red blood cells, constantly move through blood capillaries. As a result, they damage their plasma membrane due to the continuous friction. Typically, after 100 to 120 days, erythrocytes become rigid and fragile as they wear out. As they pass through small vessels in the spleen and liver, they can get trapped and break apart into fragments.
The resident phagocytic macrophages deal with these damaged cells by engulfing them and separating their globin and heme groups....
5.8K
Pharmacokinetics in Pediatric Patients: Drug Excretion01:26

Pharmacokinetics in Pediatric Patients: Drug Excretion

In pediatric medicine, understanding the renal function and drug elimination nuances is crucial for administering safe and effective treatments. Newborns, in particular, display markedly slower renal functions than adults, profoundly affecting how drugs are cleared from their bodies. This slower drug clearance requires clinicians to extend the dosing intervals for many medications to prevent drug accumulation and toxicity while ensuring therapeutic efficacy.One key area where these adjustments...
349
Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

Pharmacokinetics in Pediatric Patients: Drug Metabolism

In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses...
312
Hemodialysis III: Nursing Management01:25

Hemodialysis III: Nursing Management

The nursing management of a patient undergoing hemodialysis includes several critical steps, starting with a thorough assessment before the procedure.Before the Hemodialysis ProcedureFirst, record the patient's vital signs—blood pressure, heart rate, respiratory rate, and temperature—to establish a baseline. This baseline is essential for detecting conditions such as hypotension that could impact the patient's response to dialysis. Document the patient's pre-dialysis weight, as this...
1.3K
Hemodialysis II: Procedure and Complications01:24

Hemodialysis II: Procedure and Complications

DialyzersA hemodialysis (HD) dialyzer is a plastic cartridge containing thousands of parallel hollow fibers, which serve as semipermeable membranes. These fibers are typically made from cellulose-based or other synthetic materials. During HD, blood is pumped into the top of the cartridge and distributed among these fibers. Simultaneously, dialysis fluid, known as dialysate, is introduced into the bottom of the cartridge, bathing the outside of the fibers. Across the semipermeable membrane,...
1.5K