BACKGROUND: Few cases of pregnancy in women with systemic mastocytosis have been reported. The effects of this disease on pregnancy have not been well documented, nor have the benefits of doula services for labor been...BACKGROUND: Few cases of pregnancy in women with systemic mastocytosis have been reported. The effects of this disease on pregnancy have not been well documented, nor have the benefits of doula services for labor been reported for mastocytosis. CASE: A 35-year-old woman with indolent systemic mastocytosis sought preconception counseling regarding the effects of her disease on pregnancy and the effects of drugs and anesthesia on her disease. She then had an uncomplicated pregnancy and delivery,with multidisciplinary coordination and assistance by doulas to reduce the need for medication in labor. CONCLUSION: Mastocytosis patients can achieve normal pregnancy outcomes. Predelivery planning can help prepare staff and patients for complications. Doulas can assist with labor preparation and delivery using nonmedical approaches to relief of pain and anxiety.展开更多
Mastocytosis refers to a rare collection of disorders, both cutaneous and systemic, that are characterized by increased numbersof mast cells. Depending on the extent of the disease, these disorders may present with sy...Mastocytosis refers to a rare collection of disorders, both cutaneous and systemic, that are characterized by increased numbersof mast cells. Depending on the extent of the disease, these disorders may present with symptoms resulting from mast cell degranulation including flushing, diarrhea, vomiting, cramping,syncope, or anaphylaxis. In pediatric patients, cutaneous involvement is most prevalent in the form of urticaria pigmentosa ,which is typically asymptomatic or minimally so with resolution by adolescence. In this case report and review of literature, we review a case of a 3-year-old child with uritcaria pigmentosa displaying recurrent syncope and anaphylaxis as the first presentation of systemic mastocytosis. We found data to be limited on this topic, and concluded that pediatric patients with prior diagnoses of cutaneous mastocytosis could benefit from either more aggressive screening for systemic disease or prophylactic treatment with antihistamines and rescue subcutaneous epinephrine.展开更多
文摘BACKGROUND: Few cases of pregnancy in women with systemic mastocytosis have been reported. The effects of this disease on pregnancy have not been well documented, nor have the benefits of doula services for labor been reported for mastocytosis. CASE: A 35-year-old woman with indolent systemic mastocytosis sought preconception counseling regarding the effects of her disease on pregnancy and the effects of drugs and anesthesia on her disease. She then had an uncomplicated pregnancy and delivery,with multidisciplinary coordination and assistance by doulas to reduce the need for medication in labor. CONCLUSION: Mastocytosis patients can achieve normal pregnancy outcomes. Predelivery planning can help prepare staff and patients for complications. Doulas can assist with labor preparation and delivery using nonmedical approaches to relief of pain and anxiety.
文摘Mastocytosis refers to a rare collection of disorders, both cutaneous and systemic, that are characterized by increased numbersof mast cells. Depending on the extent of the disease, these disorders may present with symptoms resulting from mast cell degranulation including flushing, diarrhea, vomiting, cramping,syncope, or anaphylaxis. In pediatric patients, cutaneous involvement is most prevalent in the form of urticaria pigmentosa ,which is typically asymptomatic or minimally so with resolution by adolescence. In this case report and review of literature, we review a case of a 3-year-old child with uritcaria pigmentosa displaying recurrent syncope and anaphylaxis as the first presentation of systemic mastocytosis. We found data to be limited on this topic, and concluded that pediatric patients with prior diagnoses of cutaneous mastocytosis could benefit from either more aggressive screening for systemic disease or prophylactic treatment with antihistamines and rescue subcutaneous epinephrine.
