A couple comes into the clinic for genetic counseling. Both parents are heterozygous for sickle cell trait. What is the chance each of their children will have sickle cell anemia?

Choice A reason:

Adequate oxygenation is important in managing vaso-occlusive sickle cell crisis, but the replacement of factor V is not relevant to this condition. Factor V is involved in the blood clotting process, and its replacement is typically associated with bleeding disorders such as hemophilia. Therefore, this choice is not appropriate for managing a vaso-occlusive crisis.

Choice B reason:

Adequate hydration and pain management are critical components of care for a child in vaso-occlusive sickle cell crisis. Hydration helps to reduce the viscosity of the blood, which can prevent further sickling of red blood cells and improve blood flow. Pain management is essential because vaso-occlusive crises are extremely painful and require prompt and effective pain relief, often with opioid analgesics. These measures help to alleviate symptoms and prevent complications.

Choice C reason:

Pain management is indeed a crucial aspect of care for vaso-occlusive sickle cell crisis, but the administration of heparin is not typically part of the treatment. Heparin is an anticoagulant used to prevent blood clots, and it is not indicated for managing vaso-occlusive crises. The focus should be on hydration and pain relief rather than anticoagulation.

Choice D reason:

Correction of acidosis may be necessary in some cases, but it is not the primary focus of care for vaso-occlusive sickle cell crisis. The main goals are to manage pain and ensure adequate hydration to improve blood flow and reduce the risk of further sickling of red blood cells. While addressing acidosis can be part of the overall management, it is not the primary intervention.

Choice A reason:

Bilious vomiting and constipation are not typical manifestations of hypertrophic pyloric stenosis. Bilious vomiting, which is green or yellow, indicates that the vomit contains bile and is usually associated with intestinal obstruction beyond the stomach. Hypertrophic pyloric stenosis typically causes non-bilious, projectile vomiting because the obstruction is at the pylorus, before the bile duct.

Choice B reason:

Abdominal distention and currant jelly-like stools are not indicative of hypertrophic pyloric stenosis. Currant jelly-like stools are a classic sign of intussusception, a different condition where part of the intestine telescopes into itself. While abdominal distention can occur in pyloric stenosis, the presence of currant jelly-like stools points to a different diagnosis.

Choice C reason:

A rounded abdomen and hypoactive bowel sounds can be seen in various gastrointestinal conditions but are not specific to hypertrophic pyloric stenosis. While a rounded abdomen may be present due to gastric distention, hypoactive bowel sounds are not a hallmark of this condition. The primary symptom of pyloric stenosis is projectile vomiting.

Choice D reason:

Ravenously hungry after vomiting is a classic manifestation of hypertrophic pyloric stenosis. Infants with this condition often vomit forcefully after feeding and then appear hungry again because the food does not pass through the pylorus into the intestines. This symptom, along with projectile vomiting, is a key indicator of pyloric stenosis.