Propylthiouracil-induced acute interstitial nephritis with acute renal failure requiring haemodialysis: successful therapy with steroids.
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Acute Kidney Injury↗
Biomedical subjects
C C Huang
Publications and source records attributed to C C Huang.
Cyclosporin inhibits nitric oxide production in medullary ascending limb cultured cells.
BACKGROUND: Nitric oxide (NO) has been shown to play a role in cyclosporin (CsA) nephrotoxicity, but its mechanism of action is still unclear. As inducible NO synthase (iNOS) mRNA has been found to be expressed in rat medullary thick ascending limb (mTAL) cells, we investigated the effects of CsA on NO production in a model of mouse cultured mTAL cells. MATERIALS AND METHODS: The experiments were carried out on sub-cultured cells derived from isolated mTAL microdissected from the kidney of C57BL/6 mice. The identification of the iNOS mRNA in mTAL microdissected segment and cultured cell was confirmed by RT PCR and RsaI digestion. Nitrite (NO2) released by mTAL cells was determined using the modified Griess reagent method and taken as an index of nitric oxide production. The cultured cells were treated with various concentrations of CsA and different signal transduction regulators to assess the effect and possible pathway(s) of action of CsA on NO production in mTAL cells. RESULTS: The basal production of NO by mTAL cells increased by 1.8-fold following incubation with bacterial lipopolysaccaride (LPS). Both aminoguanidine and L-NAME inhibited NO production. CsA (10-300 ng/ml) also inhibited NO production in a dose-dependent manner and prevented its increase induced by LPS. Phorbol 12-myristate 13-acetate (PMA), a PKC stimulator, enhanced slightly the production of NO under basal conditions and prevented the inhibitory action of CsA on NO production. These results suggest that the NO secreted by mouse cultured mTAL cells is dependent on the PKC pathway. CONCLUSION: These results show that CsA may down-regulate the production of NO by cultured mTAL cells expressing iNOS mRNA and that the PKC pathway is involved in this process.
Animals↗
A plasmid isolated from phytopathogenic onion yellows phytoplasma and its heterogeneity in the pathogenic phytoplasma mutant.
A 3.6-kbp DNA fragment was cloned from the extrachromosomal DNA of a pathogenic plant mollicute, onion yellows phytoplasma (OY-W). Sequence analysis of the fragment revealed an open reading frame (ORF) encoding the replication (Rep) protein of rolling-circle replication (RCR)-type plasmids. This result suggests the existence of a plasmid (pOYW1) in OY-W that uses the RCR mechanism. This assumption was confirmed by detecting the single-stranded DNA (ssDNA) of a replication intermediate that is specifically produced by the RCR mechanism. This is the first report on the identification of the replication system of this plasmid and the genes encoded in it. With a DNA fragment including the Rep gene region of pOYW1 used as a probe, Southern and Northern (RNA) blot hybridizations were employed to examine the heterogeneity between the plasmids found in OY-W and a pathogenic mutant (OY-M) isolated from OY-W. Multiple bands were detected in the DNA and RNA extracted from both OY-W and OY-M infected plants, although the banding patterns were different. Moreover, the copy number of plasmids from OY-W was about 4.2 times greater than that from OY-M. These results indicate constructive heterogeneity between OY-W and OY-M plasmids, and the possibility of a relationship between the plasmid-encoded genes and the pathogenicity of the phytoplasma was suggested.
Amino Acid Sequence↗
Aganglionosis of the appendix: is it reliable for diagnosis of total colonic aganglionosis?
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Appendix↗
Isolated intracranial Rosai-Dorfman disease: case report and literature review.
Rosai-Dorfman disease, first described in 1969, is a rare idiopathic histioproliferative disease affecting the lymph nodes. Typical clinical features include bilateral painless lymphadenopathy, fever and polyclonal hypergammaglobulinemia. In approximately 43% of cases, extranodal sites may be involved and occasionally represent the initial or sole manifestation of the disease. Central nervous system manifestations are exceedingly rare, and only 17 cases have been recorded, among which there are merely seven isolated intracranial cases without concurrent nodal or other extranodal involvement. Herein, we report a 38-year-old male presenting with generalized tonic-clonic seizure and radiological findings indicative of meningioma. Complete physical examinations and laboratory surveys demonstrate the absence of involvement at other body sites. Microscopically, the lesion consists of proliferative histiocytes exhibiting emperipolesis coupled with the characteristic cytoplasmic staining against S-100 protein. The differential diagnosis of polymorphic inflammatory meningioma-mimicking masses is discussed, and a review of previously reported intracranial Rosai-Dorfman disease is presented.
Adult↗
Acute symptomatic seizure disorders in young children--a population study in southern Taiwan.
PURPOSE: To determine the incidence, etiology, and prognosis of acute symptomatic seizures in children by age 3 years. METHODS: In a population-based birth cohort study of all 15,209 neonatal survivors born in Tainan City between October 1989 and December 1991, parents or caretakers of 13,493 children aged 3 years were surveyed by telephone regarding any provoked convulsive disorder, particularly acute symptomatic seizure, in the children; medical records were reviewed. RESULTS: Sixty-three children (39 boys, 24 girls) had acute symptomatic seizures (incidence 0.46 in 100). The leading causes of acute symptomatic seizures were acute gastroenteritis, encephalitis/encephalopathy, and bacterial meningitis. Age-specific incidence was highest in the group aged 1-12 months. Intracranial hemorrhage, bacterial meningitis, and metabolic disturbance were the major causes of acute symptomatic seizures in children aged 1-12 months. Acute gastroenteritis, encephalitis/encephalopathy, and bacterial meningitis accounted for 85% of the causes in children aged 13-24 months, and gastroenteritis and encephalitis/encephalopathy were the predominant causes in those aged 25-36 months. By age 5 years, subsequent unprovoked seizures developed in 14% of the survivors of acute symptomatic seizures. CONCLUSIONS: Many acute symptomatic seizures are preventable. The risk of subsequent unprovoked seizures is determined by underlying precipitating factors. Public education regarding the danger of shaken-baby syndrome and excessive water supplement, as well as and nationwide vaccination against bacterial meningitis in young children, is necessary.
Acute Disease↗
Chronic inorganic mercury induced peripheral neuropathy.
We report the clinical features, electrophysiological studies, and morphometric analysis of sural nerve pathology in a patient with polyneuropathy due to inorganic mercury intoxication. He developed slowly progressive generalized paralysis of all limbs after 3 months ingestion of herb drugs which contained mercuric sulfate. Electrophysiologic studies revealed axonal polyneuropathy involving both motor and sensory fibers. Sural nerve biopsy demonstrated axonal degeneration with demyelination and a predominant loss of large myelinated fibers. His muscle strength showed only mild improvement after 2 years' follow-up. We concluded that inorganic mercury exposure may induce severe axonal sensorimotor polyneuropathy in humans and that neurological deficits may persist in severe cases.
Biopsy↗
Subdural empyema in 10 infants: US characteristics and clinical correlates.
PURPOSE: To investigate the ultrasonographic (US) characteristics of subdural empyema (SDE) and its differentiation from reactive subdural effusion (RSE) in infants with meningitis. MATERIALS AND METHODS: Images in 10 infants with SDE with or without RSE complicating meningitis were retrospectively reviewed and correlated with clinical findings and compared with US and magnetic resonance (MR) images in four infants with meningitis and RSE. RESULTS: At US, 15 of 16 SDEs in the 10 infants were seen as heterogeneous to hyperechoic convexity collections. Hyperechoic strands were seen in 12 SDEs. Other findings included a thick, echogenic inner membrane (n = 9), increased echogenicity of pia-arachnoid and exudates in the subarachnoid space (n = 16), mass effect (n = 16), and loculated extraaxial collections (n = 2). MR imaging findings correlated well with US and surgical results. At computed tomography, SDE was misinterpreted as RSE in one patient, due to the absence of inner membrane contrast material enhancement. Clinical outcome was related to the degree of brain damage consequent to meningitis and the chronicity of SDE. RSEs in seven infants (three with concurrent SDE) were anechoic; an inner membrane was seen in six, and increased pia-arachnoid echogenicity was seen in four. CONCLUSION: SDE has complex US features that are helpful for differentiation from anechoic RSE in infants with meningitis.
Diagnosis, Differential↗
Central nervous system candidiasis in very low-birth-weight premature neonates and infants: US characteristics and histopathologic and MR imaging correlates in five patients.
PURPOSE: To analyze the high-resolution (i.e., 5.0- or 7.5-MHz) ultrasonographic (US) imaging characteristics of central nervous system (CNS) candidiasis and their histopathologic and magnetic resonance (MR) imaging correlates in very low-birth-weight (VLBW) premature neonates and infants with systemic candidiasis. MATERIALS AND METHODS: The US images obtained in one VLBW neonate (age, 24 days; weight, 846 g) and four VLBW infants (age range, 36-161 days; weight range, 800-1, 360 g) with proved CNS candidiasis complicating systemic candidiasis and the clinical (n = 5), histopathologic (n = 3), and MR imaging (n = 2) correlates were retrospectively reviewed. RESULTS: The cranial US findings consisted of parenchymal abnormalities in four and ventricular lesions in three patients. The most common findings were multiple echogenic, rim-like microabscesses (four patients) scattered in the subcortical, periventricular, and basal ganglial areas. Confluent macroabscess formation, seen as echogenic mass-like lesions with hypoechoic centers in the periventricular regions and/or thalami, was noted on the US images obtained in two patients. The ventricles showed dilatation in two, increased ependymal echogenicity in three, the presence of intraventricular echogenic septa in two, and a lumpy choroid plexus or the presence of debris in two patients. US findings correlated well with MR imaging and autopsy findings. The histopathologic changes in the posterior fossa were well demonstrated on MR images but not demonstrated on 5.0- or 7.5-MHz US images. CONCLUSION: CNS candidiasis in VLBW neonates and infants has characteristic US features that may help in the early diagnosis of CNS involvement in systemic candidiasis.
Autopsy↗
Acute dystonia with thalamic and brainstem lesions after initial penicillamine treatment in Wilson's disease.
Dystonia is a common manifestation in Wilson's disease (WD). The striatum, especially the putamen, has been considered to be responsible for dystonia. We reported 3 patients who developed acute generalized dystonia and akinetic rigid syndrome following an initial therapy with d-penicillamine 125-500 mg daily. Brain MRI revealed lesions in the thalamus and the brainstem, particularly the tegmentum, and the basis pontis in addition to the basal ganglion lesions. After the episode, 1 patient continued to receive d-penicillamine therapy and 2 changed to zinc sulfate treatment. The generalized dystonia improved in the following 3 months and 3 years respectively in 2 patients. Follow-up brain MRI of these 2 patients revealed that the lesions in the thalamus and brainstem disappeared or resolved almost completely. From these data, acute generalized dystonia with brainstem and thalamic lesions may occur in WD patients after an initial d-penicillamine therapy. Furthermore, the dystonia may resolve following the disappearance of the brainstem and thalamic lesions.
Adolescent↗
Long-term progression in chronic manganism: ten years of follow-up.
We studied the long-term clinical course of five patients with chronic manganese intoxication. The mean scores of the King's College Hospital Rating Scale for Parkinson's disease increased from 15.0 +/- 4.2 in 1987 to 28.3 +/- 6.70 in 1991 and then to 38.1 +/- 12.9 in 1995. The deterioration was most prominent in gait, rigidity, speed of foot tapping, and writing. Tissue concentrations of manganese in blood, urine, scalp hair, and pubic hair returned to normal. Follow-up MRIs did not show paramagnetic high-signal intensity on T1-weighted images. The data indicate that clinical progression in patients with manganese parkinsonism continues even 10 years after cessation of exposure.
Chronic Disease↗
Early dexamethasone therapy in preterm infants: a follow-up study.
OBJECTIVES: To study the outcome at 2-year corrected age of infants who participated in a double-blind controlled trial of early (<12 hours) dexamethasone therapy for the prevention of chronic lung disease (CLD). METHODS AND MATERIALS: A total of 133 children (70 in the control group, 63 in the dexamethasone-treated group) who survived the initial study period and lived to 2 years of age were studied. All infants had birth weights of 500 to 1999 g and had severe respiratory distress syndrome requiring mechanical ventilation within 6 hours after birth. For infants in the treatment group, dexamethasone was started at a mean age of 8.1 hours and given 0.25 mg/kg every 12 hours for 1 week and then tapered off gradually over a 3-week period. The following variables were evaluated: interim medical history, socioeconomic background, physical growth, neurologic examinations, mental and psychomotor development index score (MDI and PDI), pulmonary function, electroencephalogram, and auditory and visual evoked potential. RESULTS: Infants in the control group tended to have a higher incidence of upper respiratory infection and rehospitalization than did the dexamethasone-treated group because of respiratory problems. Although there was no difference between the groups in somatic growth in girls, the dexamethasone-treated boys had significantly lower body weight and shorter height than the control boys (10.7 +/- 3.0 vs 11.9 +/- 2.0 kg; 84.9 +/- 5.7 vs 87.5 +/- 4.8 cm). The dexamethasone-treated group had a significantly higher incidence of neuromotor dysfunction (25/63 vs 12/70) than did the control group. The dexamethasone-treated infants also had a lower PDI score (79 +/- 26) than did the control group (87 +/- 23), but the difference was not statistically significant. Both groups were comparable in MDI, incidence of vision impairment, and auditory and visual evoked potential. Significant handicap, defined as severe neurologic defect and/or intellectual defect (MDI and/or PDI </= 69), was seen in 22 children (31.4%) in the control group and 26 (41.2%) in the dexamethasone-treated group. CONCLUSIONS: Although early postnatal dexamethasone therapy for 4 weeks significantly reduces the incidence of CLD, this therapeutic regimen cannot be recommended at present because of its adverse effects on neuromotor function and somatic growth in male infants, detected at 2 years of age. A longer follow-up is needed. If early dexamethasone therapy is to be used for the prevention of CLD, the therapeutic regimen should be modified. The proper route of administration, the critical time to initiate the therapy, and the dosage and duration of therapy remain to be defined further.
Child Development↗
Distribution patterns of primary and permanent dentition in children with unilateral complete cleft lip and palate.
OBJECTIVE: To investigate the distribution patterns of primary and permanent teeth in the cleft area and the numerical variation in teeth in unilateral complete cleft lip and palate (UCLP) patients. DESIGN: A survey of the dentition in UCLP patients. SETTING: Craniofacial Center, Chang Gung Memorial Hospital, Taipei, Taiwan. PATIENTS: 137 UCLP patients who met the following criteria: (1) have had at least one panoramic film taken, (2) the first panoramic film illustrates either primary or early mixed dentition. Evaluation of both permanent and primary dentition was available in 91 cases. MAIN OUTCOME MEASURES: Two evaluators performed independent evaluations of number and distribution of teeth in UCLP patients. The hypothesis that there are two odontogenic origins for maxillary lateral incisors was proposed to explain the occurrence of distribution patterns of dentition in the cleft area and to explain differences between primary and permanent dentition in UCLP patients. RESULTS: Four distribution patterns in the cleft area were identified in both the primary and the permanent dentition. In the primary dentition, placement of the lateral incisor distal to the alveolar cleft was the predominant pattern (pattern y, 82.4%), followed by absence of the cleft side maxillary lateral incisor (pattern ab, 9.9%), presence of one tooth on each side of the alveolar cleft (pattern xy, 5.5%), and placement of the lateral incisor mesial to the alveolar cleft (pattern x, 2.2%). In the permanent dentition, the most common pattern was the absence of the maxillary lateral incisor on the cleft side (pattern AB, 51.8%), followed by lateral incisor placement distal to the alveolar cleft (pattern Y, 46%), lateral incisor placement mesial to the alveolar cleft (pattern X, 1.5%) and the presence of one tooth on each side of the alveolar cleft (pattern XY, 0.7%). The discrepancy between the distribution patterns of primary dentition and permanent dentition successors is 57.1%. Variations in tooth number in both primary and permanent dentition of UCLP patients occurred most often in the cleft area. Abnormalities in the number of teeth (hypodontia or hyperdontia) outside the cleft area were more common in the permanent dentition than in the primary dentition (24.1% versus 4.4%). CONCLUSIONS: Four distribution patterns in the cleft area were identified in both sets of dentition. Our findings of distribution patterns in UCLP patients support the hypothesis that there may be two odontogenic origins for maxillary lateral incisors. Clinicians involved in managing the dentition of UCLP patients should consider the high frequency of numerical variation both in and outside the cleft area before starting dental treatment.
Anodontia↗
Toxicokinetic parameters in the management of poisoning: an example of podophyllotoxin intoxication.
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Animals↗
Screening for positive transfected clones with coexpressed green fluorescent protein.
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Genetic Vectors↗
Watermelon stomach--an unusual cause of recurrent upper gastrointestinal bleeding in a uremic patient receiving estrogen-progesterone therapy: case report.
A 50-year-old woman who had been on maintenance hemodialysis for 5 years developed severe anemia resistant to treatment with iron supplements and erythropoietin 4 months prior to hospital admission. Her stool occult blood test was positive, and an initial panendoscopy revealed evidence of possible antral gastritis. However, repeated administration of sucralfate, H2 blockers and a proton pump inhibitor was not effective in preventing further gastrointestinal tract blood loss and subsequent refractory anemia. She required multiple blood transfusions and hospital admissions during this period. There was no obvious coagulopathy or thrombocytopenia. After her third admission, a second panendoscopy demonstrated the typical picture of watermelon stomach. A trial of hormone therapy with estrogen and progesterone increased the hemoglobin level within a month without further evidence of active gastrointestinal bleeding. From our experience with this case, we found that the diagnosis of antral vascular ectasia (watermelon stomach) with bleeding requires a high degree of clinical alertness and careful endoscopic examination. Estrogen and progesterone therapy may provide a good option for treating the disease in uremic patients without an obvious complication. To the best of our knowledge, this is the first report demonstrating the use of maintenance hormone therapy in a female uremic patient to successfully treat watermelon gastric bleeding.
Estrogens↗
Lipoblastomatosis combined with intestinal neuronal dysplasia.
We report the case of a male infant with multifocal lipoblastomatosis and intestinal pseudo-obstruction for whom the pathologic findings of rectal biopsy were consistent with intestinal neuronal dysplasia. Lipoblastomatosis is a benign neoplasm of human fetal white adipose tissue that mainly occurs in infants and children. Intestinal neuronal dysplasia is a disorder that clinically mimics Hirschsprung's disease and that is pathologically characterized by parasympathetic hyperganglionosis of the enteric nervous system. To our knowledge, the combination of lipoblastomatosis and intestinal neuronal dysplasia has never been documented, and the gene mutations involved are completely different. In this case, therefore, we postulate that intestinal neuronal dysplasia may be secondary to either compression of the nerve tract or inhibition of bowel movement due to lipoblastomatosis.
Diagnosis, Differential↗
Arterial oxygenation is unchanged during hemodialysis in patients mechanically ventilated in assist-control mode.
Dialysis-induced hypoxemia can occur in spontaneously breathing renal failure patients but whether it occurs during bicarbonate hemodialysis in critically ill patients receiving mechanical ventilation in assist-control mode is not clear. Twenty-one patients admitted to the medical intensive care unit who required mechanical ventilation and hemodialysis with the use of a cuprammonium dialyzer were enrolled and 25 sessions of hemodialysis were performed. Arterial blood gas, white blood cell count, minute ventilation, respiratory rate, and blood pressure were measured before dialysis (time 0) and at 15, 30, 60, 120, 180, and 240 minutes thereafter. The white blood cell count dropped immediately and reached the nadir 15 minutes after hemodialysis began. Thereafter, it recovered and overshot the predialysis value at the end of dialysis. The serum HCO3- concentration increased progressively after dialysis began and resulted in significant metabolic alkalosis. The P (A-a)O2 was not aggravated and minute ventilation was not depressed by rapid metabolic alkalosis under mechanical ventilatory support. The PaO2 remained stable throughout hemodialysis. No significant hypoxemia occurred in groups of varying predialysis cardiopulmonary dysfunction. These findings suggest that in renal failure patients ventilated in assist-control mode, l) hypoventilation and accompanying hypoxemia did not occur during bicarbonate (35 mEq/L) dialysis, despite significant metabolic alkalosis; and 2) patients with higher Acute Physiologic and Chronic Health Evaluation (APACHE) III scores and P(A-a)O2 levels were not more prone to dialysis-induced hypoxemia.
Adult↗