Taiwan yi xue hui za zhi. Journal of the Formosan Medical Association最新文献

Four cases of severe preeclamptic patients with the findings of hemolysis (H), elevated liver enzymes (EL) and a low platelet count (LP) were presented. Maternal mortality occurred in two of the four cases with one experiencing liver rupture. Intrauterine fetal death occurred in 3 cases. The entity has been termed the HELLP syndrome. The high maternal and perinatal mortality of our cases was mainly due to the severe coagulopathy associated with the HELLP syndrome. The practicing obstetrician must be knowledgeable about this severe consequence of toxemia in pregnancy and aggressive management is essential to improve maternal and perinatal outcome.

We present the first report of neonatal lupus erythematosus from Taiwan. A female baby, born to a mother with documented systemic lupus erythematosus (SLE), developed cutaneous lupus lesions after phototherapy for hyperbilirubinemia. She had additional clinical features of hemolytic anemia and thrombocytopenia. Detailed serological and immunogenetic studies were performed. Transplacental passage of both anti-SSA/Ro and anti-SSB/La antibodies were demonstrated and their disappearance at the age of 6 months correlated with regression of clinical symptoms. This patient inherited human leukocyte antigen (HLA) A11, Bw60 Cw3, DR2 and Aw33, Bw57, Cw7, DRw6 from her father and mother, respectively. A long-term follow-up is required for observing whether she will develop SLE in the future.

To evaluate the diagnostic value of radionuclide inferior veno-cavagraphy (RIVC) for Budd-Chiari Syndrome, RIVC using Tc99m was performed on 106 patients with massive ascites. A positive RIVC result was defined as having at least two of the three following criteria: (1) a delay of more than 4 seconds in visualizing the heart; (2) sharply truncated inferior vena cava with marked hang-up of isotope activity; and (3) extensive collateral circulation. Of the 106 patients, 18 were RIVC positive and were later confirmed by operation or contrast venography to have Budd-Chiari Syndrome with IVC obstruction. Of the remaining 88 RIVC negative patients, 3 were shown by operation, computerized tomography and cardiac echo, respectively, to be Budd-Chiari Syndrome with IVC obstruction. Thus, the diagnostic sensitivity and specificity of RIVC for this syndrome was 85.7% and 100% respectively. If RIVC is combined with hepatic scintigraphy, it will help to elucidate the anatomic and functional change of IVC, as well as, liver parenchymal disease, such as liver cirrhosis, hepatic tumor or hepatic vein obstruction. RIVC is a simple safe, accurate, noninvasive and reproducible procedure. This study confirms the high diagnostic specificity and sensitivity of RIVC. We therefore recommend RIVC as the first-line study for IVC patency. Contrast venography may be used as a confirmatory study in preparation for surgical intervention.

Congenital mesoblastic nephroma (CMN) is a rare renal tumor with a distinct pathology and a unique clinical, therapeutic, and prognostic pattern. The characteristic pathological feature is uniform mesenchymal spindle-shaped cells arranged in interlacing bundles. The prognosis of typical CMN is believed to be excellent by nephrectomy alone. We report a typical case of congenital mesoblastic nephroma which was prenatally detected, and was associated with maternal polyhydramnios.

The antiangiogenic activity of several steroid eye drops was evaluated in a PGE-induced corneal neovascularization. Pred-Forte (1% prednisolone acetate) and Maxidex (0.1% dexamethasone) were very effective in preventing neovascularization. Medroxyprogesterone acetate at 0.1% in Tears Naturale was moderately effective and fluorometholone was less effective in blocking neovascularization. Cortisone alone was not very effective; however, the addition of heparin greatly enhanced its inhibitory effect. PGE1 induced corneal neovascularization and increased the leukocytes infiltration. The application of Maxidex right after the implantation of PGE1 pellet not only inhibited the leukocytes infiltration, but also blocked the neovascularization. These results suggested that the leukocytes infiltration might be one of the factors contributing to the development of neovascularization. The effect of steroid eye drops on the growth of preformed capillaries was also evaluated. The result demonstrated that Pred-Forte also suppressed the growth of well-developed capillaries.

Determined with automated cell counters, mean corpuscular volume (MCV) of erythrocytes was screened in 4,100 consecutive gravidas who delivered at the National Taiwan University Hospital during the period from July 1986 through August 1988. In total, 248 (6%) women had MCV less than 80 fl and were recruited for further study, including Hb A2 quantitation using microcolumn chromatography, cord blood Hb electrophoresis on cellulose acetate membrane, and alpha-globin gene mapping in some cases. In the first control group of 51 cases of obligate carriers of heterozygous beta-thalassemia, the mean MCV +/- standard deviation (SD) was 67 +/- 4.5fl (range 59 to 76fl) and the mean Hb A2 +/- SD was 6.53 +/- 1.31% (range 4.3 to 8.0%). In the second control group of 40 cases of obligate carriers of heterozygous alpha-thalassemia 1, the mean MCV +/- SD was 70 +/- 3.7fl (range 63 to 78fl) and the mean Hb A2 +/- SD was 2.28 +/- 0.54% (range 1.5 to 3.8). Forty-five microcytic women (1.1%) had Hb A2 over 4%, a cut-off level for heterozygous beta-thalassemia in our laboratory. In this study group, the mean MCV +/- SD was 68 +/- 5.4 and the mean Hb A2 +/- SD was 6.03 +/- 1.41. None of the newborns delivered by these women had Hb Bart's in cord blood. alpha-Globin gene mapping using DNA hybridization was done in 14 women, and the results were all compatible with the exclusion of deletional alpha-thalassemia. It is concluded that approximately 1% of people in northern Taiwan are beta-thalassemia heterozygotes.

Although part of pancreatic endocrine tumors may be multihormonal by immunohistochemical study, the clinical manifestations are often related to hypersecretion of only one type of peptide hormone. Only a few cases have shown two or more syndromes simultaneously or transition of one type of syndrome to another with the passage of time. The case reported here is an islet cell carcinoma with liver metastases. The clinical manifestations changed from Zollinger-Ellison syndrome to hypoglycemic syndrome with the secretion of gastrin, insulin and serotonin. The immunohistochemical study confirmed multihormonal production. The elevation of growth hormone with acromegaly was also noted in this case. It remains to be seen if the excessive production of growth hormone was due to growth hormone-releasing hormone secreted by the endocrine pancreatic tumor or to the possible presence of a pituitary microadenoma as a component of the multiple endocrine neoplasia type I syndrome.

The retrofascial space is a concealed one located behind the transversalis fascia in the posterior peritoneal cavity. It contains mainly muscles and fasiae and extends from the mediastinum to the pelvis. Clinical manifestations of an abscess are usually obscure except for hip joint problems when the abscess developed. Computed tomography is done immediately if an abscess is suspected, and there have been good results under incision with drainage and antibiotics. It is different from retroperitoneal abscess anatomically, pathologically, bacteriologically and clinically, so we report a case for reference. The patient, a 43-year-old female worker, had suffered from disability of her left lower limb for more than 5 months before admission. She even visited her local medical doctor, but in vain. In the week prior to admission, left hip swelling with awkwardness in walking was felt, and she called on an orthopedist. After brief examination, impaired hepatic and renal function was noted, so she was referred to our hospital. The chief complaint on admission was left hip swelling and pain with radiation to the inguinal and knee area for one week. The past history was insignificant except for a cesarean section and an appendectomy done more than 10 years before. Upon physical examination, the patient was found to be pale, emaciated, mildly icteric and had a flexion contracture of the left thigh. Laboratory data revealed anemia, leukocytosis, impaired hepatic and renal function. The chest X-ray was normal, and KUB demonstrated left hip joint flexion and external rotation. A sonogram showed a localized hypoechoic lesion in the left posterior peritoneal cavity 2 days later.(ABSTRACT TRUNCATED AT 250 WORDS)

Hypopituitarism can occur after cranial irradiation for tumors distant from the pituitary gland. Recent studies have suggested that this is hypothalamic in origin. Hypothalamic and pituitary functions were studied in 11 patients, 4 men and 7 women, 4.5 years or more after radiotherapy for nasopharyngeal carcinomas. The estimated average total dose was 5000 cGys for the hypothalamus and pituitary gland. Except for 2 women with amenorrhea and 4 men with impotency, the patients did not have evident endocrine deficiency. Baseline hormone profiles revealed normal T4, T3 and cortisol levels, 6 with elevated prolactin, 3 with reduced testosterone and 3 with slightly elevated basal TSH. The four menopausal women had impaired gonadotropin response to LHRH (100 micrograms, i.v.). Four (1 menstruating, 1 amenorrheic, 2 menopausal) women did not reach peak FSH response 4 hours after LHRH injection. The other amenorrheic woman had minimal FSH and LH response to LHRH which persisted even after 8 days of pulsatile infusion of LHRH (1 microgram/90min). TSH response to TRH (400 micrograms, i.v.) was delayed in 7 patients. GH response to human GRH (1 microgram/kg, i.v.) was impaired in 6 patients (maximal GH less than 5 mU/l). ACTH response to ovine CRH (1 microgram/kg, i.v.) was impaired in 3 patients (less than 50% elevation from baseline). Three patients who had normal GRH tests had impaired GH response to insulin hypoglycemia. Six patients had an empty sella on CT scan. From this study the following conclusions are drawn: (1) Among the four axes, GH is the most vulnerable. (2) The insulin tolerance test is still the best single test for evaluation of hypothalamic function.(ABSTRACT TRUNCATED AT 250 WORDS)

To investigate the response pattern of patients with chronic airway obstruction (CAO) to bronchodilators, 128 subjects consisting of 80 men and 48 women with a mean age of 52.6 years were randomly tested with three inhalant bronchodilators: a non-selective beta-agonist (isoproterenol), a beta 2-selective agonist (terbutaline) and an anticholinergics (ipratropium) plus placebo. All patients had a baseline FEV/FVC of less than 65% and their clinical diagnosis were classified as either COPD or bronchial asthma according to the criteria of the ACCP-ATS Joint Committee on Pulmonary Nomenclature. The drugs were delivered to the patient via a metered dose inhaler (MDI) using the conventional inhalation technique. Spirometry was measured at regular intervals with a computerized pulmonary function analyzer CHESTAC-65(Japan). A more than 15% increase in FEV1 and/or FVC after treatment was regarded as responsive. While 108 patients were tested with a single drug, the remaining 20 patients were tested sequentially with terbutaline, ipratropium and placebo in a randomized cross-over manner for studying additivity. Our results showed that the response rates of asthmatic patients to isoproterenol, terbutaline and ipratropium were 50%, 87% and 67%, respectively. Only 27% of COPD patients responded to isoproterenol and 60% to terbutaline. On the other hand, ipratropium possessed a 61% response rate in the treatment of COPD and showed a bronchodilator effect independent to that of terbutaline. Combined use of both drugs resulted in additional improvement. Once patients responded to the drugs, apparent changes in FEV1 and/or FVC occurred within 30 minutes and reached the maximum usually at 60 minutes post-inhalation.(ABSTRACT TRUNCATED AT 250 WORDS)