Primary sclerosing cholangitis in a child.

Primary sclerosing cholangitis (PSC) is a rare disease in Taiwan and has not been described in Taiwanese children previously. We report a 4-year-old girl who presented with prolonged fever, eosinophilia (11%), hepatomegaly, and markedly elevated serum levels of alkaline phosphatase (3,318 IU/L) and gamma-glutamyl transpeptidase (475 IU/L). Subsequent investigations including endoscopic retrograde cholangiopancreatography and liver histology confirmed the diagnosis fo PSC. Treatment with a low dose of prednisolone for 2 months and ursodeoxycholic acid during 32 months of follow-up resulted in clinical remission and halted disease progression. A high index of suspicion is necessary for physicians to diagnose this disorder in children with chronic liver disease. Our experience in this case indicates that therapy with prednisolone and ursodeoxycholic acid may be helpful for the treatment of PSC in children, and suggests the need for more trials of combined therapy.     

Ascites and elevated androgen level in a pregnant patient with an ovarian sclerosing stromal tumor.

We report the case of a 31-year-old woman with an androgen-producing sclerosing stromal tumor found during the eighth week of gestation in association with ascites and elevated serum androgen and cancer antigen (CA)-125 levels. The combined features of ascites, elevated serum androgen and elevated CA-125 in association with this rare type of ovarian tumor is unusual. Surgical removal of the tumor resulted in relief of symptoms, resolution of ascites, and decreases in serum androgen and CA-125 levels. The pregnancy was uneventful.     

Obstetric complications in a patient with Bernard-Soulier syndrome.

We describe the obstetric complications and management of a patient with Bernard-Soulier syndrome. Severe bleeding at the time of delivery and delayed postpartum hemorrhage were prominent features of her pregnancies. Further complicating this woman's pregnancies was the development of antibodies to platelet glycoprotein IB/IX, leading to neonatal alloimmune thrombocytopenia.     

Pulmonary-renal syndrome in a patient with bacterial endocarditis.

Pulmonary-renal syndrome is defined as a combination of pulmonary hemorrhage and glomerulonephritis. We report an unusual case of bacterial endocarditis presenting with pulmonary hemorrhage and rapidly progressive glomerulonephritis as the initial manifestations of the disease. A 37-year-old man was admitted with fever, hemoptysis, and dyspnea. Admission examinations revealed severe renal failure requiring dialysis therapy. Chest radiograph showed extensive pulmonary reticulonodular infiltrates. Echocardiography revealed ventricular septal defect. Furthermore, blood cultures grew viridians group streptococci. The kidney and lung biopsies demonstrated diffuse cresentic glomerulonephritis and alveolar hemorrhage, respectively. Bacterial endocarditis was diagnosed according to the Duke criteria and the patient was treated with intravenous antibiotic therapy. The pulmonary infiltrates disappeared gradually. However, renal function did not improve, even after trial of a course of immunosuppressive therapy. The patient survived and remained on regular hemodialysis. We conclude that bacterial endocarditis should be included in the differential diagnosis of pulmonary-renal syndrome.     

Acute pancreatitis and cholecystitis associated with postpartum HELLP syndrome: a case and review.

We report a case of preeclampsia associated with hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome and concomitant nonbiliary acute pancreatitis and cholecystitis in the first postpartum day. A thorough investigation ruled out known etiologies of both pancreatitis and cholecystitis. Following conservative treatment, the patient's HELLP syndrome, pancreatitis, and cholecystitis resolved on the third postpartum day. Preeclampsia is associated with microvascular abnormalities that may involve the splanchnic circulation. These abnormalities may cause not only HELLP syndrome but also pancreatitis and cholecystitis. Recognizing that ischemia can damage not only the liver but also the pancreas and gallbladder, could result in improvements in the diagnosis and management of pancreatitis in patients with preeclampsia.     

Coexisting sclerosing angiomatoid nodular transformation of the spleen with multiple calcifying fibrous pseudotumors in a patient.

Primary tumor or tumor-like lesions of the spleen are rare. Among them, vascular lesions are the most common. Vascular tumor of the spleen is different from the usual hemangioma of soft tissue because the vascular structure of the spleen is unique. Sclerosing angiomatoid nodular transformation (SANT) is a recently described vascular lesion of the spleen. Grossly, it is a multinodular, well-circumscribed tumor containing a hypervascular core. Microscopically, it comprises three types of vessels, and each type recapitulates the immunohistochemical characteristics of the normal vascular elements of the splenic red pulp, i.e. capillaries, sinusoids, and small veins, respectively. Because of the rarity of this entity, its actual pathogenesis is still unknown. In this study, we report a case of SANT occurring in a 43-year-old woman, in whom there were also multiple calcifying fibrous pseudotumors (CFPTs) in the abdominal cavity. Both SANT and CFPT are thought to be variants of inflammatory pseudotumor. Coexistence of these two rare entities in a patient has never been reported, and this fact suggests that there might be a common mechanism contributing to the formation of these two types of lesions.     

Endometriosis in a patient with Rokitansky-Kuster-Hauser syndrome

This case with Rokitansky-Kuster-Hauser syndrome and right ovarian endometriosis was previously presented as a proof against the transplant theory of Sampson. New operative findings showed a functioning endometrium in a right occult rudimentary horn and retrograde menstruation through the Fallopian tube with hematosalpinx, tubal endometriosis, right ovarian and appendicular endometriosis.     

Pregnancy in a patient with Turner syndrome

Fertility in patients with a diagnosis of Turner's syndrome confirmed by karyotype examination is a very rare phenomenon. Only in 2% of all cases the pregnancy is a result of a spontaneous ovulation and intrauterine fertilization. Due to high proportion of complications occurring in pregnancy, in labour and in puerperium, only minority of patients can expect delivering a healthy infant. Literature data indicate that 40% of patients with Turner's syndrome give birth to a healthy child. Other pregnancies are terminated either by delivering a child with congenital defect or by a spontaneous abortion. We report a case of a 26-year-old patient with Turner's syndrome (46XX/45XO) who menstruated regularly after hormonal replacement therapy. She had regular menstrual bleeding through period of two years, after withdrawal of hormones administration. In march diagnosis of pregnancy was made, as a result of a spontaneous ovulation. The healthy, full-term fetus was born in 40th week of pregnancy.     

Bilateral granulosa cell tumor in a patient with blepharophimosis syndrome.

Blepharophimosis syndrome is a rare, autosominal, dominant ocular disorder and has been reported to be associated with ovarian dysfunction and premature menopause. We report a case of bilateral granulosa cell tumor associated with blepharophimosis syndrome. The combination of the long-term hypergonadotrophism and oocyte depletion associated with blepharophimosis syndrome may have contributed to the pathogenesis of the granulosa cell tumors. In female patients with blepharophimosis syndrome, close gynecologic surveillance should be instituted.     

Early severe pre-eclamptic findings in a patient with Cushing's syndrome.

Cushing's syndrome occurs rarely in pregnancy because of ovulatory disturbances including anovulation which is caused by hypercortisolism, but it can cause maternal complications such as hypertension, gestational diabetes, spontaneous abortion, premature birth, pre-eclampsia and stillbirth. Herein we present the case of a 22-year-old patient in the 11th week of pregnancy who was admitted to our hospital with Cushing's syndrome complicated by early pre-eclampsia. Severe pre-eclampsia has high maternal and perinatal morbidities, and therefore the possibility of this complication requires that Cushing's syndrome, although rare in pregnancy, be given a high clinical suspicion. Medical therapy and/or surgical therapy should be considered promptly to influence outcome favorably.     

Acute gangrenous cholecystitis with suppurative acute cholangitis in the 1st days of the puerperium]

A 25-year-old secundipara developed acute gangrenous cholecystitis coupled with suppurative cholangitis on the fourth pureperal day. The patient recovered after cholecystectomy and choledochotomy. It is pointed out that cholelithiasis is not rare in pregnancy but acute cholecystitis is very rare, especially in the puerperium. For this reason the diagnosis is difficult, yet the final issue depends on its early establishment and also on the early surgical intervention.     

Sick sinus syndrome in a patient with single coronary artery anomaly.

Single coronary artery anomaly is very rare. The reported manifestations include angina pectoris and congestive heart failure. Here we describe a case of single coronary artery anomaly presenting as sick sinus syndrome, which has no literature precedence. A 47-year-old woman had complained of intermittent dizziness for years. A Holter electrocardiogram showed sinus bradycardia and junctional or ventricular rhythm with a maximal ventricular pause of up to 3.2 seconds. Electrophysiologic study revealed prolonged corrected sinus nodal recovery time. Coronary angiography showed that the left anterior descending artery had a long course with a side branch originating from the proximal part and coursing anteriorly to the territory of the proximal portion of the right coronary artery. The sinus node is usually supplied by the sinoatrial branch via the right coronary artery. Aortography showed that the right coronary artery ostium was absent. A permanent pacemaker was implanted and the patient was discharged in good condition. The present case suggests that coronary artery anomaly may lead to compromised blood supply to the sinus node, and hence sick sinus syndrome.     

Twin pregnancy following gonadotrophin therapy in a patient with Sheehan's syndrome.

A case of Sheehan's syndrome presented with secondary amenorrhea and was put on L-thyroxine, prednisolone and cyclical estrogen and progestin. Ovulation induction with gonadotrophins and intrauterine insemination with husband's semen resulted in a twin pregnancy. Antepartum course was complicated by bronchial asthma, gestational diabetes and pregnancy-induced hypertension. Cesarian section was done at 34 weeks gestation for preterm rupture of membranes and breech presentation. Both babies and their mother were doing well at 6 months of follow-up.