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Biomedical subjects

C Y Lin

Publications and source records attributed to C Y Lin.

At least 91 records · Page 5Linked to original sources

Risk of advanced proximal neoplasms in asymptomatic adults according to the distal colorectal findings.

BACKGROUND AND METHODS: The clinical significance of a distal colorectal polyp is uncertain. We determined the risk of advanced proximal neoplasia, defined as a polyp with villous features, a polyp with high-grade dysplasia, or cancer, among persons with distal hyperplastic or neoplastic polyps as compared with the risk among persons with no distal polyps. We analyzed data from 1994 consecutive asymptomatic adults (age, 50 years or older) who underwent colonoscopic screening for the first time between September 1995 and December 1998 as part of a program sponsored by an employer. The location and histologic features of all polyps were recorded. Colonoscopy to the level of the cecum was completed in 97.0 percent of the patients. RESULTS: Sixty-one patients (3.1 percent) had advanced lesions in the distal colon, including 5 with cancer, and 50 (2.5 percent) had advanced proximal lesions, including 7 with cancer. Twenty-three patients with advanced proximal neoplasms (46 percent) had no distal polyps. The prevalence of advanced proximal neoplasia among patients with no distal polyps was 1.5 percent (23 cases among 1564 persons; 95 percent confidence interval, 0.9 to 2.1 percent). Among patients with distal hyperplastic polyps, those with distal tubular adenomas, and those with advanced distal polyps, the prevalence of advanced proximal neoplasia was 4.0 percent (8 cases among 201 patients), 7.1 percent (12 cases among 168 patients), and 11.5 percent (7 cases among 61 patients), respectively. The relative risk of advanced proximal neoplasia, adjusted for age and sex, was 2.6 for patients with distal hyperplastic polyps, 4.0 for those with distal tubular adenomas, and 6.7 for those with advanced distal polyps, as compared with patients who had no distal polyps. Older age and male sex were associated with an increased risk of advanced proximal neoplasia (relative risk, 1.3 for every five years of age and 3.3 for male sex). CONCLUSIONS: Asymptomatic persons 50 years of age or older who have polyps in the distal colon are more likely to have advanced proximal neoplasia than are persons without distal polyps. However, if colonoscopic screening is performed only in persons with distal polyps, about half the cases of advanced proximal neoplasia will not be detected.

Adenoma↗

Pulmonary function changes and increased Th-2 cytokine expression and nuclear factor kB activation in the lung after sensitization and allergen challenge in brown Norway rats.

BACKGROUND: Our purpose was to evaluate the expression of Th-1 and Th-2 related cytokine mRNA and nuclear factor (NF) kB in the lung tissue of ovalbumin (OA) sensitized brown Norway rats (BNR). We also evaluated the correlation between bronchial hyperreactivity (BHR) and eosinophils with cytokine mRNA expression. METHODS: Eight BNR (weight range 250-350 g) were sensitized by inhaled OA (group I) with a 1-week interval between and then provoked with OA 1 week later. Pulmonary function tests (PFT) were performed at baseline and 24 h after acetylcholine challenge. Eight weight-matched normal BNR served as controls (group II). All animals were anesthetized, paralyzed with gallamine, and ventilated via tracheostomy. They were given varying doses of acetylcholine (25, 50, 75, 100 microg/kg) injected through a jugular venous catheter. Five seconds after acetylcholine injections, PFTs were performed, including a maximal forced expiratory maneuver (MFEM), airway opening pressure (P(ao)) at tidal breathing and total dynamic lung compliance (C(dyn)). Bronchoalveolar lavage (BAL) was then performed with 20 ml normal saline divided into two doses. Thereafter, the lungs were removed and examined histologically. Total RNA was extracted from lung tissue samples and reverse-transcriptase polymerase chain reaction was performed using primers for mRNA of IL-4, IL-5, IL-10, interferon-gamma (IFNr) and beta-actine. RESULTS: Group I OA treated rats had typical airway obstruction on PFTs and airway inflammation on histological examination. Ratios of IL-4, IL-5, IL-10 and inducible nitric oxide synthase (iNOS) mRNA levels to beta-actine as measured by densitometry were significantly lower in controls than in OA-sensitized rats. The IFNr mRNA to beta-actin ratio was significantly reduced in OA-sensitized rats. Group I demonstrated a band shift when compared with group II in electromobility shift assay (EMSA) for NF-kB indicating increased activation of this transcription factor. CONCLUSION: Th-2 related cytokine mRNA was increased but Th-1 related cytokine mRNA was decreased in OA-sensitized BNR. An increased level of Th-2 related cytokine mRNA correlated with decreased airflow and inflammatory changes. These results demonstrate the value of the BNR model for studying allergic asthma at the molecular level.

Allergens↗

Expression of the c-kit protein is associated with certain subtypes of salivary gland carcinoma.

The c-Kit protein, a receptor type tyrosine kinase that plays an important role in the development of hematopoietic cells, melanocytes, and germ cells, is expressed in mastocytosis, gastrointestinal stromal cell tumors (GISTs), germ cell tumors, and several other tumors. Gain-of-function mutations in exon 11 and exon 17 have been shown as a mechanism of c-kit activation in some tumors. To study the role of c-kit in salivary gland carcinomas, we analyzed the c-kit protein expression in 79 carcinomas of major and minor salivary glands by immunohistochemistry. Although varying in intensity of staining, c-kit expression was identified very often in adenoid cystic carcinomas (20/25), lymphoepithelioma-like carcinomas (6/6) and myoepithelial carcinomas (2/2), but not in other types of salivary gland carcinoma (0/46), P<0.00001. By DNA sequencing, genetic alteration of c-kit juxtamembrane domain (exon 11) and tyrosine kinase domain (exon 17) was not found in all the three types of salivary carcinoma that had c-kit protein expression. In conclusion, c-kit protein overexpression is involved in the pathogenesis of certain types of salivary gland carcinoma, but mutation of the gene is not the mechanism of c-kit activation.

Carcinoma, Acinar Cell↗

A novel high-performance fourier transform ion cyclotron resonance cell for improved biopolymer characterization.

A new trapped ion cell design for use with Fourier transform ion cyclotron resonance mass spectrometry is described. The design employs 15 cylindrical ring electrodes to generate trapping potential wells and 32 separately assignable rod electrodes for excitation and detection. The rod electrodes are positioned internal to the ring electrodes and provide excitation fields that are thereby linearized along the magnetic field over the entire trapped ion volume. The new design also affords flexibility in the shaping of the trapping field using the 15 ring electrodes. Many different trapping well shapes can be generated by applying different voltages to the individual ring electrodes, ranging from quadratic to linearly ramped along the magnetic field axis, to a shape that is nearly flat over the entire trap volume, but rises very steeply near the ends of the trap. This feature should be useful for trapping larger ion populations and extension of the useful range of ion manipulation and dissociation experiments since the number of stages of ion manipulation or dissociation is limited in practice by the initial trapped ion population size. Predicted trapping well shapes for two different ring electrode configurations are presented, and these and several other possible configurations are discussed, as are the predicted excitation fields based on the use of rod electrodes internal to the trapping ring electrodes. Initial results are presented from an implementation of the design using a 3.5 T superconducting magnet. It was found that ions can be successfully trapped and detected with this cell design and that selected ion accumulation can be performed with the utilization of four rods for quadrupolar excitation. The initial results presented here illustrate the feasibility of this cell design and demonstrate differences in observed performance based upon different trapping well shapes.

Biopolymers↗

Selective enhancement of resonance Raman spectra of separate bacteriopheophytins in Rb. sphaeroides reaction centers.

Tunable dye laser excitation of carefully prepared samples of Rb. sphaeroides reaction centers provides richly detailed resonance Raman (RR) spectra of the bacteriopheophytins, H, and the accessory bacteriochlorophylls, B. These spectra demonstrate selective enhancement of the separate bacteriopheophytins on the active (H(L)) and inactive (H(M)) sides of the reaction centers. The spectra are assigned with the aid of normal coordinate analyses using force fields previously developed for porphyrins and reduced porphyrins. Comparison of the H(L) and H(M) vibrational mode frequencies reveals evidence for greater polarization of the acetyl substituent in H(L) than H(M). This polarization is expected to make H(L) easier to reduce, thereby contributing to the directionality of electron transfer from the special pair, P. In addition, the acetyl polarization of H(L) is increased at low temperature (100 K), helping to account for the increase in electron transfer rate. The polarizing field is suggested to arise from the Mg(2+) of the neighboring accessory bacteriochlorophyll, which is 4.9 A from the acetyl O atom. The 100 K spectra show sharpening and intensification of a number of RR bands, suggesting a narrowing of the conformational distribution of chromophores, which is consistent with the reported narrowing of the distribution in electron transfer rates. Excitation at 800 nm produces high-quality RR spectra of the accessory bacteriochlorophylls, and the spectral pattern is unaltered on tuning the excitation to 810 nm in resonance with the upper exciton transition of P. Either the resonance enhancement of P is weak, or the bacteriochlorophyll RR spectra are indistinguishable for P and B.

Bacterial Proteins↗

Preparation of betaTCP/HAP biphasic ceramics with natural bone structure by heating bovine cancellous bone with the addition of (NH(4))(2)HPO(4).

In this study, the calcined bovine bone (CBB)-removing the organic substance by a burning process-with addition of different quantities of ammonium phosphate [(NH(4))(2)HPO(4)] (AP) was heated to a high temperature to transform its crystalline phase constitution from hydroxyapatite (HAP) into a tricalcium phosphate (TCP)/HAP biphasic structure. Results revealed that the CBB without AP appeared to be mainly composed of an HAP type pattern when heated to 1300 degrees C. After adding doped AP to CBB, the HPO(4)(2-) ions of AP condensed into P(2)O(7)(4-) ions at temperatures of 400-600 degrees C. P(2)O(7)(4-) ions reacted with the OH(-) ions of HAP to form betaTCP at temperatures up to 600 degrees C. The conversion reaction of HAP to betaTCP finished at around 900 degrees C. With increasing AP in the CBB, HAP gradually converted into different phase compositions of TCP/HAP or TCP at high temperature. Mechanical testing results showed that there was no significant difference in sintered CBB with different quantities of AP. By heating calcined bovine cancellous bone with different quantities of AP, we obtained different crystalline phase compositions of bioceramics with a natural porous structure.

Animals↗

Heat shock protein (hsp 70)-related epitopes are common allergenic determinants for barley and corn antigens.

IgE reactive components of barley and corn were compared using sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) and immunoblotted with sera from workers exposed to complex bioaerosols. The antibody made against Arabdopsis heat shock protein (hsp 70) was used to identify those components equivalent to hsp 70 in molecular size. Components with a molecular mass of 69 kDa and 33 kDa were positively reacted, and immunoblots of two-dimensional polyacrylamide gel electrophoresis were compared.

Allergens↗

The prevalence of heavy proteinuria and progression risk factors in children undergoing urinary screening.

To evaluate the prevalence and incidence density of heavy proteinuria and chronic renal insufficiency (CRI) and the factors related to disease progression, 10,288,620 urinary screenings of elementary and junior high-school students from 1992 to 1996 were studied retrospectively. Urinary screening included pH, protein, occult blood, and glucose measurements. Blood sample analysis included total protein, albumin, A/G ratio, blood urea nitrogen, creatinine (Cr), antistreptolysin O titer, C3, cholesterol, hepatitis B virus surface antigen, IgA, and fasting blood sugar. The results showed that the 4-year prevalence of proteinuria was higher in girls than in boys (6.87x10(-4) vs. 4.83x10(-4) respectively). There were 189 cases with disease progression into CRI among the 10,288,620 students screened and followed continuously, with the prevalence of disease progression into CRI higher in boys than in girls (2.24x10(-5) vs. 1.41x10(-5) respectively). Of the 119 cases (63%) presenting with CRI since the first urine screening and blood sampling, only 14 had serum Cr levels higher than 6.0 mg/dl. There were 1,289 patients (10.5%) with proteinuria in 1992 and 705 patients (7.1%) in 1996. The absolute number of patients with heavy proteinuria decreased. The percentage of underlying glomerulonephritis in children on dialysis also decreased from 63.2% in 1992 to 47.0% in 1996. Logistic regression analysis showed that a persistent serum cholesterol level higher than 220 mg/dl, an albumin level lower than 3.5 g/dl, total protein less than 6 g/dl, and diastolic pressure higher than 90 mmHg were the significant risk factors for disease progression to CRI. We conclude that early detection of students with heavy proteinuria by mass urinary screening, early appropriate treatment, and monitoring of significant risk factors may help to decrease or delay the progression of renal disease, delay the introduction of dialysis in these predialysis CRI patients and maintain their growth and development.

Adolescent↗

Lipid solubility of vasodilatory vanilloid-type beta-blockers on the functional and binding activities of beta-adrenoceptor subtypes.

Various vanilloid-type beta-adrenoceptor blockers were studied on guinea pig right atrium and trachea and rat colon. In addition, we also investigated their beta(1)-, beta(2)-, and beta(3)-adrenoceptor binding affinities. All these beta-adrenergic antagonists inhibited (-)isoproterenol-induced positive chronotropic effects of the right atrium and tracheal relaxation responses in a concentration-dependent manner. Some of these agents prevented the inhibition of rat colon spontaneous motility by (-)isoproterenol. Of the agents tested, we found that ferulidilol, eugenodilol, eugenolol, isoeugenolol, and ferulinolol, as well as propranolol and metoprolol, possessed beta(3)-adrenoceptor blocking activities, others were nearly without effectiveness. Furthermore, the binding characteristics of vanilloid-type beta-adrenergic antagonists were evaluated in [3H]CGP-12177, a beta(1)/beta(2)-adrenoceptor blocker and a beta(3)-adrenoceptor agonist, binding to beta(1)-, beta(2)-, and beta(3)-adrenoceptor sites in rat ventricle, lung, and interscapular brown adipose tissue (IBAT) membranes, respectively. Eugenodilol, eugenolol, metoprolol, isoeugenolol, and ferulinolol were less potent than both propranolol and ferulidilol in competing for the beta(3)-adrenoceptor binding sites. From the results of in vitro functional and binding studies, we suggested that propranolol, ferulidilol, eugenodilol, eugenolol, metoprolol, isoeugenolol, and ferulinolol all possessed beta(3)-adrenoceptor blocking activities. On the other hand, we also found that eugenodilol, eugenolol, metoprolol, isoeugenolol, and ferulinolol had a low lipid solubility in comparison with propranolol and ferulidilol. In conclusion, we proposed that beta(3)-adrenoceptor antagonistic actions of these vanilloid-type beta-blockers were positively correlated with their lipid solubility.

Adrenergic beta-Agonists↗

A unique xanthine derivative KMCP-98 with activation of adenosine receptor subtypes.

KMCP-98 is a newly synthesized adenosine receptor agonist by alkylation at the 7-position of the xanthines nucleus. We first investigated the pharmacological activities of KMCP-98 under in vivo and in vitro conditions. Acute intravenous injection of KMCP-98 (1.0, 2.0 and 3.0 mg/kg) produced a temporary fall in blood pressure and heart rate, followed by a sustained fall in heart rate in pentobarbital-anesthetized Wistar rats. The hypotensive and bradycardiac responses were inhibited by pretreatment with an A(1) adenosine receptor antagonist 8-phenyltheophylline (8-PT, 0.5 mg/kg). Both KMCP-98 and adenosine (0.3-100 microM) produced negative inotropic activity in isolated guinea pig left atria. The negative inotropic activity of KMCP-98 was significantly blocked by pretreatment with A(1) receptor antagonists 8-PT (10 microM) and xanthine amine congener (XAC, 10 microM), a nonselective adenosine antagonist theophylline (10 microM), a K(+) channel blocker tetraethylammonium (TEA, 10 mM) and a K(ATP) channel blocker glibenclamide (1 microM). KMCP-98 (0.03-30 microM) produced concentration-dependent relaxations in carbachol (1 microM) precontracted guinea pig tracheal smooth muscle. The trachea relaxant response of KMCP-98 was markedly inhibited by A(2), A(2a) and A(2b) adenosine receptor antagonists 3,7-dimethyl-1-propargylxanthine (DMPX, 10 microM), 8-(3-chlorostyryl)caffeine (CSC, 10 microM) and alloxazine (10 microM), respectively, the nitric oxide synthase (NOS) inhibitor L-NAME (100 microM) and also by TEA and glibenclamide. In addition, KMCP-98 (0.03-30 microM) elicited relaxant response in norepinephrine (3 microM) precontracted rat thoracic aorta in a concentration-dependent manner. The thoracic aorta relaxant response of KMCP-98 was also significantly inhibited by DMPX, CSC, alloxazine, L-NAME, TEA and glibenclamide. Furthermore, the binding characteristics of KMCP-98, adenosine and 5'-N-ethylcarboxaminoadenosine (NECA) were evaluated in [(3)H]DPCPX and [(3)H]CGS 21680 binding to rat cortex and striatum, respectively. The K(i) values of KMCP-98 for predominate A(1) and A(2) adenosine receptor sites were 3908+/-952 and 158+/-10 nM, respectively. In conclusion, KMCP-98 was found to be a xanthine-based adenosine receptor agonist associated cardiac depression, tracheal and aortic smooth muscle relaxations.

Animals↗

Lack of correlation between serum levels of E- and P-cadherin fragments and the presence of breast cancer.

Breast cancers often show reduced expression of the transmembrane cell-cell adhesion protein, E-cadherin. In addition, approximately half of breast carcinomas express P-cadherin, which correlates with poor survival. A large fragment of the E-cadherin extracellular domain can be detected in serum, and it has been proposed that an increase in serum E-cadherin can denote the presence of a tumor. In this study, we tested the possibility that serum E- or P-cadherin levels might be useful diagnostic or prognostic indicators in breast cancer. However, we found no indication that the level of serum E-cadherin correlated with the presence of breast cancer. In addition, although we successfully detected a fragment of P-cadherin in serum, we found that its level was considerably lower than that of E-cadherin and did not correlate with the presence of P-cadherin-positive breast cancer.

Breast↗

Pressure-time curve predicts minimally injurious ventilatory strategy in an isolated rat lung model.

BACKGROUND: We tested the hypothesis that the pressure-time (P-t) curve during constant flow ventilation can be used to set a noninjurious ventilatory strategy. METHODS: In an isolated, nonperfused, lavaged model of acute lung injury, tidal volume and positive end-expiratory pressure were set to obtain: (1) a straight P-t curve (constant compliance, minimal stress); (2) a downward concavity in the P-t curve (increasing compliance, low volume stress); and (3) an upward concavity in the P-t curve (decreasing compliance, high volume stress). The P-t curve was fitted to: P = a. tb +c, where b describes the shape of the curve, b = 1 describes a straight P-t curve, b < 1 describes a downward concavity, and b > 1 describes an upward concavity. After 3 h, lungs were analyzed for histologic evidence of pulmonary damage and lavage concentration of inflammatory mediators. Ventilator-induced lung injury occurred when injury score and cytokine concentrations in the ventilated lungs were higher than those in 10 isolated lavaged rats kept statically inflated for 3 h with an airway pressure of 4 cm H2O. RESULTS: The threshold value for coefficient b that discriminated best between lungs with and without histologic and inflammatory evidence of ventilator-induced lung injury (receiver-operating characteristic curve) ranged between 0.90-1.10. For such threshold values, the sensitivity of coefficient b to identify noninjurious ventilatory strategy was 1.00. A significant relation (P < 0.001) between values of coefficient b and injury score, interleukin-6, and macrophage inflammatory protein-2 was found. CONCLUSIONS: The predictive power of coefficient b to predict noninjurious ventilatory strategy in a model of acute lung injury is high.

Animals↗

Choledochal cyst associated with acute acalculous cholecystitis.

A 7-year-old girl underwent laparotomy for suspected acute appendicitis. Instead, bile peritonitis with hydrops of gallbladder and normal appendix were noted. Dilatation of the CBD was observed, and choledochal cyst was documented by intraoperative cholangiography. Cholecystectomy and T-tube placement were performed. Postoperative follow-up by ultrasound lasted for 9 months. The CBD remained the same size without clinical manifestation.

Acute Disease↗

Low frequency of rearrangement of TRK protooncogene in Chinese thyroid tumors.

The TRK protooncogene (NTRK1) encodes a cell-surface transmembrane tyrosine kinase (TK) acting as a receptor for nerve growth factor. Oncogenic potential in thyrocytes results from replacing the 5' portion by regulatory parts of other genes, leading to constitutive TK expression. In Italy, human papillary thyroid carcinoma (PTC) shows a frequent activation (50%) of the TK receptor genes NTRK1 and RET. Both genes undergo oncogenic rearrangements by the same mechanism. We previously reported high frequency (6/11) of rearrangement of the RET protooncogene in Chinese PTCs. Wide differences in the frequency (0-10.9%) of the NTRK1 rearrangement in PTCs have been reported in different populations. To investigate the frequency of TRK protooncogene rearrangement in Chinese thyroid tumors, we performed reverse transcriptase polymerase chain reaction to amplify specific TRK rearrangement transcripts. We examined thyroid tumors of 40 patients, including 14 papillary carcinomas, 4 follicular carcinomas, 1 Hurthle cell carcinoma, 1 insular carcinoma, and 20 nodular goiters. NF874 NIH3T3, NF723 NIH3T3, NF861 NIH3T3, and NF881 NIH3T3 were used as controls for TRK-T3, TRK-T2, TRK-T1, and TRK, respectively. No known TRK protooncogene rearrangements were detected among the 40 thyroid tumors in our studies. We suggest that the TK receptor NTRK1 activation seems less important than RET activation in PTCs in the Chinese population.

Adenocarcinoma↗

In vivo and in vitro natural frequency analysis of periodontal conditions: an innovative method.

BACKGROUND: When measuring periodontal disease, various types of equipment for making objective measurements of tooth mobility have been proposed. However, these devices and methodology are insufficient in terms of reliability. An innovative method using vibrational theories to assess the periodontal attachment level of natural teeth is presented in this study. METHODS: Modal testing technique, a non-destructive and time-saving method, was used for non-invasive and quantitative measurement of the natural frequencies of the upper central incisor in vivo and in vitro. A finite element model was established, and modal testing experiments were simulated to assess the relationship between bone level and teeth. RESULTS: The first dominant natural frequency of healthy human upper central incisors ranges from 710 Hz to 3,360 Hz, with an average of 1,701+/-679 Hz. Both in vitro experiment and finite element simulation showed that lowering of the attachment level causes a significant decrease in the natural frequencies, and a strong relationship (r = -0.99, P <0.01) was observed between the frequency and the bone level height. CONCLUSIONS: Our results suggested that natural frequency is an important parameter for assessing the periodontal condition and that the modal testing technique is a useful supplemental method for assessing the attachment conditions of the periodontium.

Acceleration↗

Impact of hepatitis B and C virus infection on the outcome of kidney transplantation in Chinese patients.

BACKGROUND: There has been improvement in kidney transplantation over the years; however, the impact of hepatitis B and C virus (HBV, HCV) infection on the long-term outcome of kidney transplant is still controversial. METHODS: A total of 113 patients who received renal allografts from 1986 to 1998 were analyzed. Nine were positive for both hepatitis B surface antigen (HBsAg) and antibody to HCV (anti-HCV) (Group 1), 20 were HBsAg-positive and anti-HCV-negative (Group 2), 30 were HBsAg-negative and anti-HCV-positive (Group 3) and 54 were negative for both markers (Group 4). The outcome and survival were compared among the four groups of patients. RESULTS: The mean follow-up period was 5.1 +/- 3.2 years (range, 0.5-13 years) for all patients. Group 2 patients had significantly higher liver-related complications (35% vs 0%, p < 0.0001) and liver-related deaths (20% vs 0%, p = 0.004) than did Group 4 patients. Among all, four HBsAg-positive patients had fulminant hepatitis and died within two years of transplantation. Three (Group 2) of the patients who died were seropositive for hepatitis B e antigen and/or HBV DNA and none had a history of or positive serologic marker to indicate hepatitis of other etiologies. The remaining patient (Group 1) had evidence of superinfection of HCV. Liver cirrhosis occurred in one, two and one patient in Groups 1, 2 and 3, respectively, and hepatocellular carcinoma occurred in two and one patient in Groups 2 and 3, respectively. Despite high liver-related mortality in HBV-infected patients, paradoxically, no significant differences among the four groups in the long-term graft and patient survivals were demonstrated. The presence of HBsAg or anti-HCV was not associated with a poor prognosis of survival as determined by Cox regression analysis. CONCLUSIONS: HBV or HCV infection is not a contraindication to kidney transplantation in Chinese patients. However, it should be noted that serious liver-related complications may occur and limit survival in HBV- and/or HCV-infected patients after kidney transplantation.

Adolescent↗

Chronic granulomatous disease: a case report.

Chronic granulomatous disease (CGD) is a rare inherited disorder caused by defects in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex of phagocytic leukocytes. The leukocytes of the CGD patients cannot produce adequate amount of superoxide and other oxygen metabolites which are toxic to microorganisms. As a result, the phagocytes fail to kill the ingested microorganisms, especially those with catalase activity. Typically, CGD patients suffer from recurrent pyogenic infections starting from the first year of life. We report a young boy who had experienced recurrent perianal abscess, osteomyelitis and bacterial enterocolitis. Flow cytometric analysis revealed defects in the neutrophil respiratory burst pathway and defined the carrier state of his mother and younger sister. He received antimicrobial prophylaxis at our out-patient clinics and remained well at present. We try to make clinical physician keep in mind the diagnosis of CGD by presenting this typical case. In the meantime, we review the recent literature regarding the advances in diagnosis and management of CGD.

Animals↗