Supportive treatment includes rest, normothermia and transfusion when indicated. Intensive care for the development of acute kidney injury should be offered. Plasmapheresis is proposed to be an adjunctive measure to facilitate recovery. Steroids and other immunosuppressants are sometimes administered and the beneficial effect is uncertain. Immunotherapy is considered in refractory to corticosteroids and immunosuppression. Monoclonal antibodies e.g. rituximab (anti-CD20) and eculizumab (anti-C5) have been used but the therapeutic benefits are controversial. Antibiotic therapy should be given if syphilitic cause is confirmed, while investigations and management for hematological malignancies should be pursued in adult patients with unexplained PCH. Epidemiology
Estimated incidence of PCH is 0.4 per 100000 population, and the prevalence ranges from 1.6% to 40% in patients with autoimmune hemolytic anemia. The prevalence depends on the sensitivity of the immunologic methods applied. The age of onset is often <5 years in the pediatric population, with male predominance ranging from 2.5:1 to 5:1 in male-to-female ratio.The majority of PCH were accounted by congenital or tertiary syphilis in the early 1900s. Since the application of antibiotic therapy and prenatal screening, syphilitic PCH has become a rare entity. It is now increasingly recognized in pediatric patients with preceding viral-like illness. See also
Cold agglutinin disease
Cold sensitive antibody
Hemolytic anemia
List of hematologic conditions

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Multisystemic smooth muscle dysfunction syndrome (MSMDS) is a genetic disorder caused by R179 missense mutations in the ACTA2 gene. Initially described as a case report in 1999, it was characterized in 2010 as a syndrome of congenital mydriasis, patent ductus arteriosus, and aneurysmal arterial disease—in particular aortic and thoracic aneurysms. The disorder has variable penetrance, ranging from severely symptomatic and fatal in early neonatal period to a more benign and manageable course with surgical intervention. Signs and symptoms
Signs and symptoms are usually detectable prenatally or shortly after birth. In its severe manifestations, MSMDS has been associated with prune belly sequence. In less severe forms, the earliest signs of MSMDS are congenital fixed mydriasis (can be misdiagnosed as partial aniridia), and a PDA requiring surgical intervention. Most carriers of the mutation will eventually develop thoracic arterial disease between the ages of 10–25. Pathogenesis
The most commonly associated mutation with MSMDS is R179H. Management
Management of MSMDS requires lifelong surveillance and surgical intervention when required. The first comprehensive guidelines were published in 2018 by the same group that first categorized the syndrome in 2010. == References ==