A Case of Recurrent Rosai-Dorfman Disease Successfully Treated with 2-Chlorodeoxyadenosine (Cladribine) / 임상소아혈액종양

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare histiocytic disorder of unknown etiology. Most patients with RDD have spontaneous remission, but in some patients, the disease recurs after complete remission and may not respond to general treatment. Some patients with RDD involving the extranodal system can have serious symptoms such as vital organ dysfunction due to mass effects, neurological symptoms caused by intracranial involvement, and respiratory distress with airway involvement. We report the case of a 7-year-old girl with severe dyspnea due to refractory extranodal RDD that caused progressive upper airway obstruction. She was admitted because of nasal congestion and persistent cervical lymphadenopathy, and diagnosed as having RDD by cervical lymph node incisional biopsy. The initial prednisone treatment did not improve her symptoms. The following contrast-enhanced neck computed tomography revealed a newly developed airway mass protruding in the upper trachea. After 8 weeks of chemotherapy with vinblastine, methotrexate, and prednisone, complete remission was attained. Seven months after chemotherapy cessation, the disease recurred, and chemotherapy with vincristine, cytarabine, and prednisone was resumed. Despite the chemotherapy and emergency radiotherapy, no improvement was observed in the cervical lymph node enlargement and airway obstructive symptom due to the upper tracheal mass. 2-Chlorodeoxyadenosine (cladribine) therapy was initiated, and the patient got complete remission after 6 cycles of the cladribine treatment and maintained no evidence of disease for 2 years. We suggest that cladribine is an effective treatment option for recurrent/refractory RDD.

Sudden Unexpected Death caused by Olfactory Groove Meningioma: A Case Report / 대한법의학회지

Meningiomas, one of the most common neoplasms of the central nervous system, may be encountered incidentally during autopsy. Most of these tumors, however, are benign and hence, are not considered as the chief cause of death. Further, sudden unexpected death caused by meningioma is very unusual. Moreover, the diagnosis of an incidental meningioma as the cause of sudden death may sometimes be difficult. In the present report, we describe an autopsy case of a sudden, unexpected death due to a large olfactory groove meningioma accompanied by severe cerebral edema and tonsillar herniation.

Myoplasmic Ca2+, Crossbridge Phosphorylation and Latch in Rabbit Bladder Smooth Muscle

Tonic smooth muscle exhibit the latch phenomenon: high force at low myosin regulatory light chains (MRLC) phosphorylation, shortening velocity (Vo), and energy consumption. However, the kinetics of MRLC phosphorylation and cellular activation in phasic smooth muscle are unknown. The present study was to determine whether Ca(2+)-stimulated MRLC phosphorylation could suffice to explain the agonist- or high K(+)-induced contraction in a fast, phasic smooth muscle. We measured myoplasmic [Ca2+], MRLC phosphorylation, half-time after step-shortening (a measure of Vo) and contractile stress in rabbit urinary bladder strips. High K(+)-induced contractions were phasic at both 22degrees C and 37degrees C: myoplasmic [Ca2+], MRLC phosphorylation, 1/half-time, and contractile stress increased transiently and then all decreased to intermediate values. Carbachol (CCh)-induced contractions exhibited latch at 37degrees C: stress was maintained at high levels despite decreasing myoplasmic [Ca2+], MRLC phosphorylation, and 1/half-time. At 22degrees C CCh induced sustained elevations in all parameters. 1/half-time depended on both myoplasmic [Ca2+] and MRLC phosphorylation. The steady-state dependence of stress on MRLC phosphorylation was very steep at 37degrees C in the CCh- or K(+)-depolarized tissue and reduced temperature flattend the dependence of stress on MRLC phosphorylation compared to 37degrees C. These data suggest that phasic smooth muscle also exhibits latch behavior and latch is less prominent at lower temperature.

A case of small bowel obstruction during pregnancy / 대한주산의학회잡지

Small bowel obstruction is an unusual complication of pregnancy, but it is associated with high fetal and maternal mortality. Diagnosis is often delayed due to confusion over symptoms by both the patient and physicians, because symptoms such as colicky abdominal pain, vomiting and constipation are all common at various stages of pregnancy. If is small bowel obstruction of gravid patient suspected, prompt abdominal radiological evaluation and surgical intervention to reduce both maternal and fetal mortality are warranted. We report a recently report a recently experienced case of small bowel obstruction with brief review of literatures.

A case of lemierre syndrome poorly responsive to medical treatment / 대한내과학회지

Lemierre syndrome is a rare clinical condition that is characterized by anaerobic oropharyngeal infections leading to septic thrombophlebitis of the internal jugular vein and frequent secondary metastatic infections. The accurate diagnosis and treatment, for early stage disease, is important because it may be associated with a high mortality rate if untreated. We present the case of a 34-year-old man who presented with a history of typical for the diagnosis of Lemierre syndrome. Supportive care with antibiotics did not improve the clinical condition. The clinical course improved after treatment with IV anticoagulant and surgical thrombectomy. In addition, he had the antiphospholipid syndrome, which is known to be a common cause of acquired arterial or venous thrombosis. Therefore, in this patient the associated antiphospholipid syndrome might precipitate an internal jugular venous thrombophlebitis after an oropharyngeal infection or might account for the poor response to medical treatment.

A case of ovarian endometriosis after laparoscopically assisted vaginal hysterectomy / 대한산부인과학회지

Endometriosis is defined as the presence of endometrial tissue outside the uterus and located usually within the pelvis. It may be detected as a rare complication associated with surgery. Endometriosis following obstetric and gynecologic procedure, which is localized mostly in the surgical scar including Cesarean section scar, hysterotomy scar, trocar scar, Bartholin cyst exision scar, or episiotomy scar, may caused by implantation of endometrial gland and stroma. Endometriosis developing after hysterectomy, especially, is a quite rare condition except a recurrent disease. We have experienced a case of a 47-year-old woman who had diagnosed ovarian endometriosis two years after laparoscopically assisted vaginal hysterectomy for a large leiomyomatous uterus with no evidence of endometriosis, so we report this case with a brief review of literature.