Surgery in patients with congenital or acquired coagulation defects has always been challenging and requires special care with a multidisciplinary approach. Percutaneous nephrolithotomy (PCNL) is a standard procedure performed in patients with kidney stones. Although prone to bleeding more than most of the widely performed surgical procedures, there are not much data regarding PCNL in patients ...

Background: Congenital hepatic fibrosis (CHF) is an autosomal hereditary disorder affecting the porto-biliary system. It is a rare hereditary disorder often presenting in childhood or adolescence with hepatomegaly, splenomegaly, and gastrointestinal bleeding. A timely diagnosis of organomegalies by sonography can prevent esophageal varices. Liver transplantation is now the only...

IN CONTRAST to the hereditary disorders of red cells and white cells, inherited disorders of the platelets appear to be rare. Among the few reports of hereditary thrombocytopenia,1'4 some lack essential history or supporting laboratory data. Those which present more convincing evidence display a remarkable variety cf clinical features and genetic patterns. Only the so-called Aldrich syndrome7 e...

severe thrombocytopenia with bleeding is rarely reported in children with brucellosis, and recurrent epistaxis is extremely rare. brucellosis with hemorrhage should be differentiated from viral hemorrhagic fever, malignancy, and other blood disorders. bone marrow aspiration (bma) is mandatory to differentiate from other blood diseases. an 8-year-old boy was admitted with recurrent epistaxis, pe...

Menorrhagia is a common gynecological symptom. In adolescents, the cause is generally dysfunctional uterine bleeding. Menorrhagia may also be due to undiagnosed coagulation defects, endocrine disorders, gynecological abnormalities of the uterus, or other systemic disorders. Menorrhagia may be the only clinical manifestation of an inherited bleeding disorder. We report a case of inherited hypopr...

BACKGROUND AND PURPOSE The treatment of epistaxis in patients with hereditary hemorrhagic telangiectasia can be very challenging. The purpose of our study was to evaluate our experience with endovascular epistaxis embolization in patients with hemorrhagic hereditary telangiectasia and to compare this with our experience in patients treated for idiopathic epistaxis. MATERIALS AND METHODS Over ...

Hereditary benign telangiectasia is an uncommon idiopathic skin disorder characterized by generalized telangiectases and angiomatous lesions of the skin. The diagnosis should be suspected in patients with such cutaneous lesions, positive family history, no associated bleeding problems, and no mucosal involvement. We present six cases of hereditary benign telangiectasia in three generations of o...

Bleeding symptoms are common in healthy children but occasionally may indicate an underlying congenital or acquired bleeding diathesis. The rare bleeding disorders (RBDs) comprise inherited deficiencies of coagulation factors I (congenital fibrinogen deficiencies), II, V, VII, X, XI, and XIII and combined factor deficiencies, most notably of factors V and VIII and of vitamin K–dependent factors...

Bleeding symptoms are common in healthy children but occasionally may indicate an underlying congenital or acquired bleeding diathesis. The rare bleeding disorders (RBDs) comprise inherited deficiencies of coagulation factors I (congenital fibrinogen deficiencies), II, V, VII, X, XI, and XIII and combined factor deficiencies, most notably of factors V and VIII and of vitamin K–dependent factors...