A Case of Adult Type Colonic Hypoganglionosis / 대한소화기내시경학회지

Hypoganglionosis is a rare form of intestinal neuronal malformation, which is characterized by scarce ganglia and a reduced number of parasympathetic nerves in the intestinal wall. The pathogenesis of intestinal neuronal malformation is mainly attributed to developmental disorders of the enteric nervous system (ENS). Furthermore, the ENS can be damaged during the postnatal period by ischemic, inflammatory, autoimmunological process or neurotoxic agents. Subsequently abnormalties of the ENS may cause chronic constipation or ileus. Intestinal neuronal malformation should be suspected in patients with a history of chronic stool retention and appropriate radiographic findings. Hypoganglionosis is extremely difficult to establish the diagnosis histochemically unless full-thickness biopsies are performed. Recently we experienced a case of adult type hypoganglionosis, which is assumed to be an acquired type. It was treated with right hemicolectomy.

Azathioprine-associated Acute Pancreatitis in Rheumatoid Arthritis / 대한내과학회지

Drug-induced pancreatitis is now recognized as a distinct, although uncommon, entity. Azathioprine has been incriminated as a causative agent of acute pancreatitis. We report a 36-year-old female who developed acute pancreatitis in the course of treatment with azathioprine for rheumatoid arthritis. She was admitted due to epigastric pain, radiating to the back, for four days. Serum amylase and lipase levels were 226U/L (normal : 30-110U/L) and 1300U/L(normal : 23-300U/L), respectively. The abdominal ultrasonogram and CT scan revealed diffuse enlargement of the pancreas. We diagnosed it as acute pancreatitis. There was no history of alcohol use or gallbladder disease. We considered drugs, especially azathioprine, as the cause of acute pancreatitis. The patient was treated conservatively with intravenous fluid and analgesics. The symptoms subsided within three days. Thirteen days later, azathioprine was resumed. After six hours, the patient complained of epigastric pain. The elevated level of serum lipase(542U/L) confirmed the recurrence of pancreatitis. Her symptoms subsided when azathioprine intake stopped, and the serum lipase level returned to normal within four days.

Functional Dyspepsia and Subgroups in Korea and Short Term Outcome of Therapeutic Trial of Cisapride: Multicenter Study / 대한소화관운동학회지

BACKGROUND/AIMS: The aims of this study were to determine subgoups of functional dyspesia and to evaluate the short-term effect of cisapride in patients with functional dyspepsia in Korea. METHODS: 1025 patients, with a mean age of 42.6 years, with symptoms of functional dyspepsia, were recruited consecutively and upper gastrointestinal symptoms were investigated by interview in 41 hospitals in Korea. In an open, multicenter trial, 1025 patients received Smg of cisapride three times a day (TID) for at least .2 weeks for the treatment of symptoms of functional dyspepsia. When necessary, the dose of cisapride was increased to 10mg TID and the duration of therapy was extended to 4 weeks. RESULTS: The most frequently reported symptoms of functional dyspepsia were epigastric discomfort or fullness (85%), bloating (70%), belching (53%), early satiety (52%) and epigastric pain (46%) retrospectively. Subgroups of functional dyspepsia were as follows; dysmotility-like 73.5%, ulcer-like 39.7%, reflux-like 13.0%, and unspecified dyspepsia 14.0%. However, 33.2% of subjects with functional dyspepsia could be classified into more than one subgroup. Upper gastrointestinal symptoms were decreased to average 50.3% (range; 42.2 to 59.2%) after 2 weeks of cisapride treatment and to 25% (19.2 to 29.9%) after 4 weeks. cisapride therapy resulted in good or excellent improvement in 59.0% of the patients after two weeks, in 75% of patients after 4 weeks. Adverse events were occurred in 52 patients (5.8% of all patients), most commonly, loose stools or diarrhea (3.5%), abdominal pain (1.1%), and dizziness (0.3%). The majority of adverse events was mild and transient in nature and led to premature discontinuation of treatment in 4 patients. CONCLUSIONS: Although the majorities of patients with functional dyspepsia have dysmotility like symptoms in Korea, there is such overlap among the dyspepsia subgroups. Most patients responded well to a short therapeutic trial with cisapride without significant side effects.

Acquired Idiopathic Sideroblastic Anemia: A clinical study of 15 patients / 대한혈액학회지

BACKGROUND: Acquired idiopathic sideroblastic anemia (AISA) is a heterogeneous condition. Most instances, involving only the erythroid line, are benign disease with a longer survival and a low propensity for evolution into acute leukemia. A subset of patients have severe clinical course and evidence of other cell line involvement at presentation, may develop the emergence of blast cells and evolution into acute leukemia. In an attempt to identify the natural history and the risk factors for the development of acute leukemia, the clinical, hematological and outcome data were studied in the patients with AISA. METHODS: We reviewed retrospectively the medical records of 15 patients of AISA treated at the Catholic University of Taegu-Hyosung and Kyungpook National University Hospital from March 1989 to December 1995. RESULTS: The median age at diagnosis was 41 years and the male to female ratio was 8 : 7. On bone marrow examination, erythroid abnormalities were prominent in all cases, 5 patients also showed involvement of the granulocytic and/or megakaryocytic cell lines (AISA with myelodysplastic features, AISA-M). The median follow-up duration was 32 months. Transfusion dependence occurred in 11 of 16 cases. Progression towards refractory anemia with excess of blasts or acute leukemia (M2) was observed in two patients with AISA-M after follow-up period of 16 months and 24 months, respectively. Infections and hemorrhages were causes of death in 3 patients with AISA-M but not in patients with dyserythropoiesis only (AISA-erythroid, AISA-E). CONCLUSIONS: Most patients with AISA have a relatively benign course with prolonged survival after the onset of anemia. Patients with features of dysgranulopoiesis and/or dysmegakaryopoiesis in addition to dyserythropoiesis at presentation were increased risk of transformation to refractory anemia with excess of blasts or acute leukemia and shorter surtival. But further study of larger numbers of patients and longer follow-up may be warranted.

Influence of Enalapril on Blood Pressure in Spontaneously Hypertensive Rats

We aimed to evaluate the long term trantment of enalapril on the vascular response in the isolated aorta, and in anesthetized or pithed spontaneously hypertensive rats(SHR). In the isolated aorta, the increase in tension provoked by addition of KC1 16.7mM was attenuated by enalapril treatment(3mg/kg/day for 6 weeks), whereas the increment by addition of NE 0.1uM tension was not influenced. The frequency-dependent vasoconstricution induced by electrical field stimulation of aorta was also attenuated by enalapril treatment. In pithed SHR, the frequency-related hypertension provoked by electrical stimulation(10sec, 1ms with 40V) of sympathetic pregnglionic nerve was also attenuated by enalapril treatment. Neither dose-related vasorelaxation by acetylcholine addition in the aorta nor decrease of DBP by intravenously(i.v.)-injected aetylcholine was altered by enalapril treatment. However, beta2-agonist, salbutamol-induced vasorelaxation in enalapril-treated group, was more remarkable than that in control group. The hypotension by i.v.-but not by intracerebroventricularly-injected salbutamol strengthened by enalapril treatment. These results suggest that the suppression of development of hypertension by enalapril treatment may result from the reduction of adrenergic neurotransmission and activity to voltage dependent calcium channel by acting on vascular smooth muscle itself.