MEDICAL JOURNAL OF TABRIZ UNIVERSITY OF MEDICAL SCIENCES
Year:2005 | Volume:27 | Issue:2|Papers Count:19

Background and Objectives: Burn wound is one of the most common causes, which not only damages skin, but also associated with different bacterial infections. Because of increased bacterial resistance to antibiotics, treatment of burn wounds becomes much more difficult. The aim of this study was to identify the causative agents of burn wound infections, determination of sensitivity pattern of isolates and investigation of different factors such as sex, age, mortality in relation to burns.Materials and Methods: During one year (1999-2000), samples were taken from the burn wound infections on 126 selected and hospitalized patients at burn wards of Sina Medical Center, and were examined by microscopic and, cultural methods. For epidemiological study, data were collected from the patients' files. Results: A total of 126 samples were processed, and 73 bacteria were identified, 47 (64.38%) out of them were P. aeruginosa and 25 (35.62%) constituted by other bacterial species. Ninety point nine percent of patients who died had positive culture results. Pseudomonas aeruginosa was the most prevalent bacterium (64.38%) followed by S. aureus (13.69%) among burn infection isolates. Despite susceptibility of P. aeruginosa to the third generation cephalosporins such as cefoprazon (76.9%), ceftazidim (71%) and cefotaxim (59.3%), its increased resistance to different antibiotics must be taken into consideration. The most common cause of burns after boiling water (46%) was kerosene accidents (23%). The most common cause of burns in children was also boiling water. Conclusion: Recognition of the clinical importance of P. aeruginosa and other bacteria in burn wound infections and their resistance to antibiotics shows the need to take appropriate measures to prevent and minimize transmission and infection in the hospitalized patients. Boiling water takes the first most important cause of burns.

Background and Objectives: Onychomycosis is a prevalent fungal nail infection caused by some species of dermatophytes, yeast, yeast like and saprophyte fungi including 15 - 35% of total nail disorders and 1/3 of total fungal infections. The aim of this study was to document the descriptive epidemiological features of fungal nail infection in the patients referred to medical mycology lab.Material and Methods: This study was carried out on 2150 patients and continued for three years. 235 cases (10.9%) had disorders of toenails or finger nails. Direct exam of scales of nails was performed with 10-20%KOH and then cultured on Saboured dextrose agar and Mycobiotic agar medium.Results: Eighty eight (37.4%) out of235 patients who had nail disorders were infected with fungal agents .About 57 cases (35.0%) were female and 31 cases (40.8%) were male. Twenty one of patients were under 10 years old. The agents of infections were 60 cases (68.2%) species of candida, 17 cases (19.3%) dermatophyte and 11 cases (12.5%) saprophyte fungi. Candida albicans, Trichophyton mentagrophytes and Scopolariopsis were the most common agents. Conclusion: Results of this study showed that onychomycosis is an important fungal infection in patients with nail disorders.Yeast and yeast like fungi are the most prevalent agents that causes onychomycosis. Females were more infected as they are at more exposure because of many contacts to detergents and more contacts of their hands with water. Sucking the hand fingers and scratching of anal are the main reasons for nail candidal infections in children. This study revealed that Candida albicans, Trichophyton mentagrophytes and Scopolariopsis are the most prevalent agents for development of onychomycosis in this area.

Background and Objectives: Imaging studies have failures to evaluate patients with cervical radiculopathy, for example, false positive, low sensitivity, ... and routine electrodiagnosis investigate distal portions, and definite evidence of denervation in EMG and abnormal F-waves are low in frequency while slowing or block of conduction may be the only abnormality in proximal segment. This survey have been planned for actual experience of root stimulation to determinate efficacy diagnostic rate in cervical radiculopathy, possible complication and patient uncompliance.Matriels and Methods: Patients were tested in two major clinical groups including: without sign or maybe only with radicular symptom and radiculopathy with sign (probable with symptom and one radicular sign i.e., sensory, motor or reflex involvement-definite with symptom and two or three radicular sign). Two monopolar needle inserted into paraspinal muscles, one cathod immediately adjust and below spinous process of C5, C6,C7 vertebral body and other anode inserted in contralateral side and also two disc electrode recorded compound muscle action potential that active was on motor point of biceps, triceps and abductor digiti minimi and reference electrode was on tendon of same muscle in distal area, respectively for evaluation of C5-6,C7,C8-T1 radiculopathy and then if latency difference in affected side was at least 1 ms greater than unaffected side, test result was positive. In addition, routine electrodiagnosis was performed before this process.Results: Pneumothorax complication was not being noted. All patients (33) undergone complete performing of test. Thirsty patients with unilateral radiculopathy were included in diagnostic component of study. Twenty four cases (80%) were positive and six (20%) had negative results. All the patients in definite and probable radiculopathy (radiculopathy with sign, 16 cases) had positive results and in possible group six cases had negative results (42.8%).  Conclusion: This survey is assumed that root stimulation is safe, acceptable by patients and sensitive in evaluation of cervical radiculopathy. This method has benefits as a complementary test in evaluation of proximal nerve pathway lesions, especially in conduction disorder.

Background and Objectives: We carried out a prospective study on insulin resistance and its relationship with anovulation from November 2000 to January 2002 in Montaseria Infertility Center.Materials and Methods: We had two groups of infertile women including insulin resistance and without insulin resistance to study. They were similar in age, menarch age, marriage age and duration of infertility. They had no important history of medical or surgical diseases. Results: Oligomenorrhea was more common in study group than control group (80% vs 26.7%), whereas an ovulation was common in study group (80% vs 23%). The relationship between two groups was significant. Obesity was more common in study group than control group (80% vs 55.4%), and the average BMI was 32.13 Kg/m2 vs 28.9 Kg/m2, but there was no relationship between them. The mean waist to hip ratio (WHR) in the insulin resistant patients was greater than control group and the relationship between them was significant. Hisutism was 6.5 times more common in the study group than the other group. Study group patiens had gerater amount of fasting serum insulin level (27.06µu/ml), greater LH/FSH ratio (2.48 vs 1.48) and lesser amount of serum progestron (2.58 vs 5.9 mg/dl) than control group. The fasting blood sugar was similar in two groups. Conclusion: It is proper to monitor glucose tolerance with periodic glucose tolerance testing in infertile patients with oligomenorrhea, android obesity and hisutism. In the treatment process it is necessary to get weight loss and exercise as well as adding anti insulin drugs to ovulation induction agents.

Background and Objectives: Endoproteases play a key role in cell physiology and also in incidence of diseases. Considering their role in the cell in one hand, and the number of the known ndoproteases in the other hand, they clearly imply the importance of the development of a new repeatable and easy strategy to identify cytoplasmic site specific endoproteases. The aim of this study was to develop a new strategy for identification of novel site-specific endoproteases that are localized to the cytoplasm.To develop this system, two GAL4-VP16 and HSV.tk-Zeo recombinant fusion proteins have been exploited.Materials and Methods: Molecular biology methods were used to construct plasmids. Constructed plasmids were amplified using competent bacterial cells and their functional activity was studied using HeLa cells. Different recombinant HeLa cells were developed using constructed vectors and their sensitivity to drugs was studied using MTT assay.Results: HeLa cells were transfected with the pcDBHZm plasmid containing inducible HSV.tk-Zeo cassette and then selected with G418. The transfected cells were retransfected with pcG4VPl6 plasmid, which was being constructed during this study. Sensitivity of the transfected cells to GCV was assayed in comparison to the untransfected (served as negative control) and pCC1 transfected HeLa cells (used as positive control). Data showed that, pcDBHZm transfected cells were 30-fold more resistant to GCV compared to pcDBHZM+pcG4VPI6 cells. These results confirmed that the expression of HSV.tk-Zeo was under the control of GAL4-VPI6 and in the presence of this protein, HSV.tk-Zeo was expressed and the cells became sensitive to GCV and could be killed in the presence of this drug (negative selection). Having confirmed the feasibility of the selection of the cells with GCV, another plasmid called pcG4VPl6 was constructed. In this plasmid some restriction sites were created between GAL4 and VPl6 coding sequences in order to incorporate oligonucleotide library or known sequences between GAL4 and VP16 fragments. Inclusion of oligonucleotide library or known endoprotease coding sequences allows us to use this system to identify novel site specific endoprotease or to investigate the distribution of putative endoprotease in different cell types.Conclusion: Results confirmed the feasibility of the developed system for selection of the cells in which the expression of the HSV.tk-Zeo is placed under the control of GAL4-VP16 and in the presence of this protein, HSV.tk-Zeo cassette is expressed. Expression of HSV.tk-Zeo induces 30-fold higher GCV sensitivity cells resulting to cell death in the in the cells containing active GAL4-VP16 (negative selection). This provides possibility to select the cells containing inactive GAL4-VP16 (due to its proteolytic cleavage) from the cells containing active GAL4- VP 16 (due to absence of proteolytic cleavage) in the presence of GCV.

Background and Objectives: The fungal infection of the nail (Onychomycosis) is one of the most significant and most prevalent causes of nail involvement. Any misdiagnosis or late treatment of these infections will lead to extensive disorders in the nails. The objective of this survey was to determine different types of nail fungal infections and to discover the predisposing factors of this disease.Material and Methods: In this descriptive study, 100 patients with nail disorders were selected and introduced for laboratory studies during one year. The removed nail samples were evaluated by direct microscopy and culture.Results: Forty eight cases out of 100 patients with nail lesions were positive from fungal contamination point of view. Direct examination of nail samples included 30 cases (62.5%) yeast fungi, 13 cases (27%) dermatophytosis and 5 cases (10.5%) saprophytic fungi. In culture examination of the samples among the yeasts, Candida albicans and among the dermatophytes, Trichophyton mentagrophytes and among the saprophytic fungi, Aspergillus fumigatus were the most prevalent ones.Conclusion: The findings of this survey indicate that early diagnosis and correct treatment of nail fungal infections (Onychomycosis) due to its high rate of prevalence is very important and any misdiagnosis will lead to chronicity and severe destruction of the nails.

Background and Objectives: Mean platelet volume (MPV) is considered as a parameter in differential diagnosis of common platelet disorders which is related to platelet production. This study was done to evaluate MPV changes in common disorders of platelet such as leukemia, ITP and aplastic anemia.Materials and Methods: One hundred patients admitted with platelet disorders at Tabriz Pediatric Medical Center were compared with 20 patients without hematological problem. The factors studied included platelet, WBC, Hgb and MPV which were done by H1 Coulter. Sixty six cases studied had leukemia, 27 patients had ITP and 7 out of them had aplastic anemia. The final diagnosis was done by BMA.Results: In patients having leukemia with average platelet count of 54 154/mm3 and MPV of 7.29±1.19 there was not meaningful relationship between disease and MPV (p>0.067). Patients with ITP had average platelet count of 22359/mm3 and MPV of 10.40±1.280 which did not show a meaningful relationship between disease and MPV (p>0.05), but in patients with aplastic anemia average platelet count 25142/mm3 and MPV 4.48±0.91 showed a meaningful relationship between aplastic anemia and MPV (p<0.001).Conclusion: Although MPV is an indicator of platelet production and it decreases in bone marrow failure and increases in bone marrow production activity, this study shows that MPV is only helpful in diagnosis of aplastic anemia and has no diagnostic value in leukemia or ITP.

Background and Objectives: In addition to EMG and muscle biopsy, measurement of muscle enzymes is helpful in diagnosing of neuromuscular disorders. Therefore, possible effects of needle EMG on serum levels of muscle enzyme are of importance. Although several studies addressed the question whether needle EMG causes elevation of muscle enzymes (creatine phosphokinase [CKP] and lactatedehydrogenas, [LDH]) the results were conflicting. However, these studies used srerilizable needle - electrodes and different protocols and methods to record EMG and determine muscle enzymes. We investigated prospectively whether muscle enzymes are elevated after sterilizable concentric needle EMG.Materials and Methods: In 70 subjects who attended the electrodiagnosis unit of physical medicine and rehabilitation department of Imam Khomeini hospital in Tabriz for an EMG investigation were studied between June 2003 and February 2004. Blood sampling and muscle enzymes were determined immediately following EMG with sterilizable concentric needle electrodes. Subjects with hypercratinephosphokinemia as a result of other neuromuscular disorders were excluded.Results: Seventy subjects, 28 women and 42 men, aged 17-72 years (mean 36.1 year) were studied and the number of investigated muscle was between 2-17 (mean 5.5) per subject. Wilcoxon signed ranks test was applied due to the abnormal distribution. We obtaited the following results:Serum LDH value was increased before and after EMG, but the difference was not statistically significant.There was no difference in serum CPK value before and after EMG.Difference of serum CPK value would be significant if more than 5 muscles were investigated.Conclusion: EMG with sterilizable concentric needle electrodes does not cause false – positive results for muscle enzyme serum levels.

Background and Objectives: Urinary tract infections (UTIs) are the most prevalent infections after respiratory infections, particularly in females. Escherichia coli are the most common causative bacterium in UTI. There are different methods to study these bacteria based on phenotypic and genotypic characteristics. Phenotypic characteristics tend to vary, but genotypic characteristics are more stable. Plasmid profile analysis provides useful information in tracing the outbreak origin and number of different clones which exist in the outbreak. This study was performed to show plasmid profiles of Ecoli strains isolated from UTI of in- and out-patients and their restriction endonuc1eases digestion patterns to survey epidemiologic relationships between isolated strains. Materials and Methods: For plasmid DNA extraction, alkaline lysis procedure was used. Extracted plasmid DNA was subjected to electrophoresis in 0.8% agarose gel and stained with ethidium bromide. Then plasmid bands were visualized under DV. For detection of open circular bands from supercoiled ones, two dimentional electrophoresis and enzyme digestion methods were used. For enzymatic digestion, Eco. R1, Hind III, Bam1 and SmaI restriction enzymes were used. Results: Antibiotic susceptibility and plasmid profiles of 100 Ecoli strains isolated from urine of in- and out-patient samples with UTIs were analysed. Ninety nine (99%) of the strains were resistant to ampicillin, but all were sensitive to amikacin. Antibiotic susceptibilities were recorded as 96%, 64%, 57% and 27% against nitrofurantoin, ciprofloxacin, nalidixic acid and co-trimoxazole, respectively. Ten strains (10%) lacked any plasmid and in 90 strains (90%), 1-7 plasmids were detected. Although some strains contained plasmids of >21kb molecular mass, but mainly our isolated plasmids ranged from 0.9 kb to 21 kb. Ecoli strains isolated from inpatients revealed to contain more number and larger molecular weight plasmids compared to strains from out-patients. Eighty three (83%) resistant strains to ampicillin had 21 kb plasmid. Forty four plasmid profiles were obtained from total of 90 plasmid containing strains. When the isolated plasmids were digested using restriction endonucleases, in some cases similar digestion profiles were obtained, and in other cases although the digestion profiles were different, but similar  bands were detected.Conclusion: Our findings suggest plasmid presence in Ecoli strains isolated from UTIs in out-patients and in-patients, and there were great similarities in their plasmid profiles. Since these strains had been isolated from various wards in a short period of time, our results raise the possibility of unique source for some strains and high prevalence of genetic exchange among hospital isolates.

Background and Objectives: The clinical presentation of obsessive - compulsive disorder (OCD) is highly heterogeneous. Fluoxetine has been known as an effective drug in the treatment of OCD, but there is no convincing information about the effect of the agent on the subtypes of the illness. This study was conducted to investigate the effectiveness of fluoxetine in treating three subtypes of OCD (washing, obsessive thoughts, and checking).Materials and Methods: One hundred OCD patients referred to Razi hospital and two private clinics in Tabriz, were selected randomly. They were examined by DSM-IV -TR based interview. Physical and substance related disorders were excluded. They were assigned to three groups: washing, obsessive thoughts and checking. All groups completed the Yale – Brown scale before and two months after receiving fluoxetine with average doses between 40-80 mg/day. Two scores (before and after treatment) were compared and analyzed with statistical paired t - test.Results: The results showed that all subjects (regardless of their subtypes) significantly responded to fluoxetine (p<0.001). Washer subjects and the patients with obsessive thoughts showed better response to the drug (p-values were 0.000 and 0.001, respectively).Conclusion: There was a significant difference between checkers and the other groups of OCD patients in response to fluoxetine therapy and the checker group showed no significant response. This finding can help physicians in treatment planning of OCD patients.

Background and Objectives: Upper gastrointestinal bleeding (UGIB) is a life threatening disease. This study was conducted to identify the etiology of UGIB and preventable deaths.Materials and Methods: In this retrospective study, files of UGIB patients who had been hospitalized at two university hospitals of Sina and Imam Khomeini in Tabriz in 2001 were reviewed.Results: Among 578 patients admitted due to UGIB, 64 individuals (11%) died. UGIB consisted 12% of admissions and 13% of mortality of internal medicine ward. UGIB was the cause of 16.2% out of the mortality of surgical ICU. Peptic ulcer was the most common cause of bleeding. None of the etiologies had significant effect on mortality. In mortality group, 36.4% were operated and 63.6% were treated conservatively. Seventy percent of mortality in operated patients and 17% of the conservatively treated patients were potentially preventable.Conclusion: The most common cause of preventable mortality in operated patients was delayed surgery and technical faults. The most common cause of mortality in the conservatively treated patients was restraining of operation. Return to early operative indications in UGIB patients could potentially decrease mortality.

Background and Objective: Tannase (tannin acyl hydrolase) is an industrially important enzyme produced by a large number of fungi, which hydrolyzes the ester and depside bonds of gallotannins and gallic acid esters. In the present work, a partial gene of tannase from Aspergillus niger as an important bioreactor has been cloned and sequenced.Materials and Methods: Genomic DNA was extracted from Aspergillus niger using a modified version of the method described by Mennium (1977). Degenerated primers were designed against highly conserved amino acid sequences of tannase from Aspergillus awamori, Aspergillus aculeatus and Aspergillus oryzae and used for PCR after determining optimum annealing temperature and magnesium concentration. Southern analysis was carried out with a number of restriction enzymes using DIG labelled tannase probe and a single band between 1.5- 6 kb were observed depending on the restricting enzymes.Results: A PCR product of the predicted size (about 990 bp) were obtained and ligated into pGEMT®- Easy vector and cloned into E. coli Top-10F' (Stratigen) using the standard cloning procedures. Following transformation of competent cells, blue/white selection performed on ampicillin agar plates containing x-gal and IPTG. Plasmid DNA was extracted from transformant cells restricted with EcoR I and digested DNA run out by gel electrophoresis. Three colonies containing the expected insert size for tannase gene(s) were sent for sequencing.Conclusion: All three nucleotide sequences were identical and shared a high degree of homology to Aspergillus awamori, Aspergillus aculeatus and Aspergillus oryzae tannase sequences (64-52% identity at the nucleotide level). Result of southern blotting indicating probability of single copy of tannase gene in the genome of A. niger.

Background and Objectives: The majority of patients suffering from a low back pain and/or radiculopathy caused by a herniated nucleus pulposus heal spontaneously without surgery. This leaves only a small percentage of patients with persistent symptoms that are amenable to surgery. One of the techniques for this surgery is standard discectomy. This may be associated with complications such as recurrent disc herniation, spinal instability, infection, epidural scar formation and arachnoiditis and epidural hematoma. Some of these complications need reoperation. In this paper we reviewed our patients with multiple lumbar spine surgeries after first standard discectomy.Materials and Methods: From January 1997 to September 2003 we performed 1136 standard discectomy in our ward. Of these, 36 out of patients had multiple surgeries, 36 individuals underwent two and 3 patients three operations. With regard to the patients' previous graphs and surgery records and new graphs we determined the causes of these patients' multiple surgeries.Results: Of these patients 52.7% were men and 47.3% were women. The mean interval between two operations was 28.8 months and between the second and third operations was 11.6 months. The most common lesions needed for resurgery were as herniated disc in adjacent space (28.2%), reherniation in the same space (23.7%) and spinal instability, arachnoiditis, epidural infection and hematoma with decreasing incidence.Conclusion: Doing procedure through suitable method and attention to some points of surgery may improve the results of standard discectomy and decrease the need for resurgery.

Background and Objectives: Rapid recovery involving early extubation and mobility, decreased duration of ICU stay, and decreased duration of hospitalization has recently become an interesting issue in cardiac surgery. The purpose of this randomized clinical trial study was to evaluate the efficacy of propofol versus midazolam on recovery characteristics such as eyeopening, response to verbal stimulation, tracheal extubation, length of ICU stay after coronary artery bypass graft surgery (CABG).Materials and Methods: One hundred patients that scheduled for elective CABG-surgery entered into the study. Fifty patients induced with propofol 1.5 mg/Kg, fentanyl 7 mcg/kg, pancuronium 0.1 mg/kg and 50 patients with midazolam 0.2 mg/Kg, fentanyl 7 mcg/kg, pancuronium 0.1 mglkg. Anesthesia was maintained with Propofol 2 mg/Kg/h versus midazolam 0.1 mg/Kg/h before cardio pulmonary bypass and pancuronium, fentanyl halothane were used in both groups with the same doses.Results: Patients receiving propofol were extubated earlier (mean 492 min vs. 696 min p=0.041) with earlier eye opening (162 min vs.215 min p=0.019), earlier response to verbal stimuli (210 min vs.325 min p=0.031) and ICU stay (33h vs.43 h p=0.021). Hemodynamic changes were similar in both groups and the use of inotrops and nitroglycerin and the incidence of myocardial ischemia and myocardial infarction were similar.Conclusion: The results of this study highlight the ability to fast-track recovery in patients receiving propofol based anesthesia in CABG surgery with earlier recovery and extubation in ICU and lower length of ICU stay than traditional method of midazolam based anesthesia without hemodynamic and ischemic adverse effects.

Background and Objectives: Prognosis of acute myelogenous leukemia (AML) is evaluated by both overall survival and response to treatment. Prognosis of AML is correlated with several factors. Of these factors, old age and high white blood cell count are known as poor prognostic factors. Other prognostic factors are cell surface markers. Expression of CD34 has been reported as a poor prognosis factor more than other cell surface markers. The aim of this study was to investigate the correlation of CD34 expression and response to induction chemotherapy in patients with AML.Materials and Methods: Patients admitted for remission induction chemotherapy (since 1999 to 2002) in Tabriz Shahid Gazi Tabatabaie Hospital were studied. Response rate to chemotherapy and the correlation of CD34 expression with response to treatment was evaluated. These markers confirmed by flowcytometry, from bone marrow aspiration or peripheral blood samples, at the time of diagnosis. Response to chemotherapy confirmed by bone marrow aspiration sample study.Results: Positive response rate to treatment is significantly lower in CD34 positive patients (25.9%) compared with CD34 negative patients (55.6%) (p=0.019).Conclusion: The results of this study indicated that CD34 is an independent poor prognostic factor in AML.

Background and Objectives: Respiratory failure has a very important role in increasing morbidity and mortality of surgical patients. The objective of this study was to report the mortality and morbidity of surgical intensive care unit patients who required mechanical ventilation and compare this with other centers.Materials and Methods: In this descriptive survey, we studied data on demographic and clinical profile of the patients who required mechanical ventilation in surgical intensive care unit of Tabriz Imam Khomeini Hospital in a two-year period from September 1995 till September 1997. One hundred and forty patients (71.4% male, 28.6% female) were admitted for mechanical ventilation during this period, 94.3% as an emergency and 5.7% following elective surgeries. The cause was trauma in 68(48.6%) patients (60 cases of blunt and 8 penetrating) and other causes in 72 (51.4%). Mean age of trauma group was 35.1±16.7 year and, 57.2±19.1 for the other patients (p<0.001).Results: Indications for mechanical ventilation were as: disorders of mechanical function of the rib cage (flail chest, multiple rib fractures, diaphragm rupture, post-thoracotomy, ...) in 14 (10%) decreased level of consciousness (head trauma, apnea after cardiopulmonary resuscitation, neurologic syndromes,...) in 22 (15.7%), and respiratory failure due to other causes in 104 patients (74.3%). Ten patients had a history of chronic obstructive pulmonary disease. Eighty six patients (61.4%) were mechanically ventilated for 1-7 days and 54 patients (38.5%) over 7 days. Tracheostomy was performed only in 8 cases (5.7%). The in-hospital mortality rate was 51.4% among trauma patients and 64.0% among non-trauma patients (p<0.05); the most common causes of death were cardiac complications, renal failure, and septic shock, respectively. Mortality was higher in non- trauma patients and those who ventilated for over 15 days (p<0.05).Conclusion: The relatively high mortality of our patients could be primarily due to the severity of their underlying disorder and earlier diagnosis and treatment of the underlying disorder would prevent this. Use of newer equipments and techniques for patients care in the intensive care units is another essential option for reducing this high mortality.

Asphyxiating thoracic dysplasia or Jeune's syndrome is a very rare osteo-chondrodysplasia that demonstrates with failure of membranous and enchondral ossification. Jeune's syndrome associated with thorax, limbs, kidney, liver, pancreas, heart, retina and brain abnormalities. The disease presentation is variable and has two forms. Major form usually leads to severe respiratory failure and multiorgan involvement resulting in early neonatal death. In minor form there are minimal respiratory manifestations, and patient may die from complication of nephritis, hepatic and pancreatic fibrosis in childhood and adolescence. The disease has autosomal recessive inheritance that maps to chromosome 15 q 13. Recent medical and surgical advances have centered to mechanical ventilation, rib expansion techniques and renal/hepatic / pancreas transplantation resulting in good survival. In this study, we report a case of 43 day- old neonate with Jeune's syndrome.