case of diaphragmatic hernia

Congenital diaphragmatic hernia is a rare condition addressed in the medical literature as a surgical problem. It is usually a disorder of the newborns, equally occurring in males and females. There are 3 basic types of congenital diaphragmatic hernia: the posterolateral Bochdalek hernia, the anterior Morgagni hernia, and the hiatus hernia. This is a presentation of a newborn with congenital diaphragmatic hernia and a favorable outcome after surgical repair

Gulf implantable cardioverter-defibrillator registry: rationale, methodology, and implementation

Background: The implantable cardioverter-defibrillator [ICD] is effective in the prevention of sudden cardiac death in high-risk patients. Little is known about ICD use in the Arabian Gulf. We designed a study to describe the characteristics and outcomes of patients receiving ICDs in the Arab Gulf region

Methods: Gulf ICD is a prospective, multi-center, multinational, and observational study. All adult patients 18 years or older, receiving a de novo ICD implant and willing to sign a consent form will be eligible. Data on baseline characteristics, ICD indication, procedure and programing, in-hospital, and 1-year outcomes will be collected. Target enrollment is 1500 patients, which will provide adequate precision across a wide range of expected event rates

Results: Fifteen centers in six countries are enrolling patients [Saudi Arabia, United Arab Emirates, Kuwait, Oman, Bahrain, and Qatar]. Two-thirds of the centers have dedicated electrophysiology laboratories, and in almost all centers ICDs are implanted exclusively by electrophysiologists. Nearly three-quarters of the centers reported annual ICD implant volumes of

Conclusions: Gulf ICD is the first prospective, observational, multi-center, and multinational study of the characteristics and, the outcomes of patients receiving ICDs in the Arab Gulf region. The study will provide valuable insights into the utilization of and outcomes related to ICD therapy in the Gulf region

Language disorders in children conceived by the assisted reproductive technologies

The new reproductive technologies have made it possible for parentless couples to become parent. Very little information is known about language and cognitive development in child conceived by ART and few studies have been done in order to evaluate the communicative disorders in these children. The objective of this study is to establish base line data about the size and distribution of language disorders among the children conceived through the ART procedures. This study was set in the Egyptian IVF center, the Jesuit Father's school and the preterm pediatric clinic in El-Minia governorate. One hundred children conceived by the ART procedures and 100 naturally conceived babies were included in this study. The children in the two groups were matched as much as possible in order to evaluate the effect of the ART procedures on the language abilities. The children of both groups were subjected to the protocols of language assessment which applied in the Phoniatric Unit of Am Shams University Hospital Revealed significant difference between both groups regarding the receptive, semantics and total language test scores. The results from this study revealed that the low birth weight, poor neonatal outcome, premature and multiple birth were important risk factors for worsening the language abilities of the children conceived by the ART more than the mode of conception by the ART procedures. In conclusion, A wider study is necessary to determine the effect of ART on the language skills of ART children

Prenatal diagnosis of aneuploidy among a sample of Egyptian high risk pregnancies

A number of studies have shown that aneuploidies of only 5 chromosomes [13, 18, 21, X and Y] account for about 65% of all chromosomal abnormalities and 95% of chromosomal aberrations cause live-born birth defects. Fluorescent in-situ Hybridization [FISH] has been found to be highly effective for rapidly determining the number of specified chromosomes in interphase cells. Prenatal diagnosis was performed on 40 high risk pregnancies chosen from mothers attending the Antenatal Clinic of Ain Shams University Medical genetics Center [ASUMGC]. Early amniocentesis for conventional karyotype analysis of cultured amniocytes and interphase FISH studies of uncultured amniocytes for rapid detection of aneuploidies of chromosomes [13, 18, 21, X and Y] was performed. Normal karyotype was detected in 35 cases [87.5%] and in 4 cases [10%] chromosomal abnormalities were detected by conventional karyotype. However, culture failed in one case [2.5%] due to culture contamination. FISH assay confirmed the cytogenetic findings, for the probes used, on interphase nuclei in all cases analysed, except three cases of structural chromosomal abnormalities: [46, XX, add 21[q22]; 46, XX, t[5; 20] mat, 46, XY inv[9] [p11:q13]] paternal. In one case of culture contamination, FISH analysis was useful in excluding the aberrations of specific chromosomes 13, 18, 21, X and Y on the uncultured/interphase nuclei. Molecular cytogenetic technique of FISH is very useful in urgent cases of prenatal diagnosis where it can be used on uncultured amniocytes for rapid and accurate detection of common aneuploidies

Percutaneous dilatation tracheostomy: does it improve the outcome in ICU?

Percutaneous dilatation tracheostomy [PDT] is increasingly being used in the intensive care unit [ICU], and has probably increased the number of procedures performed. The primary aim of this study was to compare the percutaneous dilatation tracheostomy [PDT] with the traditional surgical tracheostomy [97] as regards ICU outcome. A prospective study was performed in the ICU. 46 patients were divided to undergo surgical tracheostomy [ST] or percutaneous dilatation tracheostomy [PDT]. Perioperative complications were recorded and analyzed. 21 patients underwent [ST] and 25 patients for [PDT]. There were no difference between the two groups in terms of age, sex, BMI, APACHE II score and baseline fractional inspired oxygen [FIO2]. The duration of the procedure and intubation days were significantly decreased in [PDT] group compared with [ST] group. Perioperative complications were significantly more in the [ST] group. The overall outcome was favorable with the [PDT] group compared with the [ST] group

Intra-abdominal pressure in critically Ill patients: it is time to pay attention

Intra-abdominal pressure [IAP] measurements can be used for the early detection and management of the abdominal compartment syndrome [ACS]. Increased intra-abdominal pressure [IAP] can lead to multi-organ dysfunction and eventually death. In this study we collected and analyzed data of the clinical cases which were prone to develop intra-abdominal hypertension [IAH] and [ACS]. A prospective study was performed in the ICU over 7 months. Patients were classified according to their IAP. The follow up of those who had high IAP was concerned with the incidence of ACS and the mortality rate. 115 patients were allocated for this study; we found prevalence of IAH in total number of patients [68.7%]. Male sex and high body mass index [BMI] predominance was noticed in the group of patients who developed IAH [62.2% with P=0.02]; and [31 +/- 6.2 with P=0.03] respectively. The incidence of ACS was 55% in the group of patients with IAH and overall mortality rate among them was 59%. IAH and ACS are significant causes for multi organ failure and increased mortality among wide variety of patients. So, we should emphasize "IAH and ACS" it is time to pay attention

Reduction of hepatitis C virus load during low flux renal haemodialysis in patients with end stage renal disease infected with HCV

HCV infection remains a common problem in patients with end stage renal disease [ESRD] undergoing maintenance dialysis especially Haemodialysis [HD]. Inspite that dialysis patients usually are not immunologically competent due to uraemia, hepatitis C virus infection runs usually indolent course. A number of studies support the notion that this dynamics of HCV viral load may be related to lowering of HCV RNA titres during the HD procedure that could be related to the dialyser immaterial used. This work aimed mainly to study the change in HCV RNA titre before and after HD session using low flux dialyser. 18 HCV positive, end stage renal disease [ESRD] patients on regular HD with detectable viral RNA using qualitative polymerase chain reaction [PCR] technique were included. All patients had quantitative PCR just before and at the end of a single 4 hours HD session. Standard low flux [Cuprophan or Polysulphon] dialysis filters 1.2m[2] surface area were used for all patients included. Mean HCV RNA for all patients pre dialysis was 135, 143 +/- 16,993 copies/ml. Mean post dialysis HCV RNA was 78, 786 +/- 48,651 copies/ml. Observed reduction in HCV title post/pre dialysis ratio 13.8%-82.l% with median value of +/- 40%. There was a significant reduction in the level of HCV RNA after dialysis [p<0.001, 95% Confidence Interval 125, 332-144, 954]. Low flux haemodialysis session has a significant effect on hepatitis C viral load with marked reduction in HCV RNA that can have a positive impact on the prognosis of hepatitis C in ESRD on regular HD

Effect of haemofiltration in prevention of contrast induced nephropathy and on acid/base status in predialysis chronic kidney disease patients undergoing cardiac catheterization

High risk renal failure patients have increased risk for contrast nephropathy during coronary angiography despite the use of conventional prophylaxis with intravenous hydration and acetylcysteine. The aim of the study was assess the role of haemo filtration versus conventional prophylaxis in preservation of kidney function and prevention of contrast nephropathy in chronic kidney disease patients undergoing cardiac catheterisation. There was a highly significant reduction in BUN, creatinine, serum uric acid [p<0.001] in haemofiltration group in comparison with baseline and control group till day 4 that was not significant after one week. There was a significantly improving rise in pH and serum HCO[3] level in haemofiltration in haemofiltration group till 24 hours post contrast injection [p<0.001]. Haemofiltration can provide adequate pres ervation of kidney function in high risk renal patients under going cardiac catheterisation. It also achieved proper adjustment of acid/base status in such patients

Outcome of pediatric malignancies in intensive care units

Our study has been carried out in intensive care unit [ICU] at Nasser Institute under supervision of Pediatric oncology specialists from Jan 2000 to Oct. 2003. It has included 49 children [29 males and 20 females] with childhood malignancies with mean age 5 years to evaluate the paediatric risk of mortality [PRISM] as a scoring 10 +/- 5 systems for prediction of outcome in PICU of children with malignancies. This score and presence of organ system failure [OSFs] were done for all patients on the first day of admission. Our results owed that the duration of hospital stay was, and days among all admission, survivors and non survivors respectively. Sex and age had no effect on survival rate and the duration of hospital stay. Also our results revealed that 35 [71.43%] of study cases had haematological malignancies, 8 [16.33%] intracranial tumors and [12.24%] extracranial solid tumors. The mean PRISM score was [15.17.76, 19.425.02, 6.193.62] among admissions, non survivors and survivors respectively and the difference between survivors and non survivors was statistically highly significant [p<0.01]. As regards the causes of admission to PICU these included respiratory failure, CNS problems, septic shock and haematological and renal problems [15, 15, 13 and 6] respectively, with mortality rate 33.3%, 18.2%, 36.4% and 12,1% respectively of total mortality with higher mortalities among septic shock patients. The total mortalities were 33 [67.3%] and the survival 16 [32.7%] and it was correlated significantly with OSFs. Both CVS and RS failures had the greatest effect on MR [100%] when any one developed as a part of Organ System Failure [OSF]s. The need for mechanical ventilation or inotropic support correlated with poorer outcome it was 85% and 92.9% respectively. The PRISM score had significant correlation with OSFs particularly heart and respiratory systems failure. We can conclude that the PRISM score is highly recommended for evaluation of PICU performance with other standard one, early admission to PICU for early prediction, proper therapeutic intervention and prevention of OSFs with subsequently reduction of MR, cost and over utilized ICU resources, also infection control strategies should be strictly applied for control of infection and sepsis

Upper Limb lengthening in children and adolescents using monoiaterai fixators

Upper limb lengthening is indicated in large length discrepancies, severe angular deformities due to exostosis, trauma to the epiphyseal plate or congenital deficiency, which may result in functional and cosmetic problems. In our work, 8 upper limb segments in 8 patients were lengthened, which included 6 ulnae, 1 radius and 1 humerus. The cases included 3 patients with multiple hereditary osteochondromatosis, 2 patients with congenital short ulna and dislocated head radius and 2 post-traumatic patients with [pbyseal injuries and old fractures] and one patient with Ollier's disease. The average age at the time of surgery was 11.5 years [range 5-19]. In all cases the Orthofix Limb reconstruction system LRS [pediatric size] was used. Callotasis method was applied. The average follow up period was 19.5 months [range 6- 44]. The average lengthening achieved was 3 cm [range 2.3-5]. The percentage increase over initial length averaged 20% [range 15-31%]. The healing index [HI] averaged 45.7 days/cm. In one case callus deformity occurred and temporary decrease of the radio-carpal joint mobility occurred in another case. In conclusion, the results of our work on upper limb lengthening as regards the functional and aesthetic out come, the complication rate and the healing [HI] index were satisfactory

Role of C.T scan inearly detection of sacroiliitis and prognosis of patients with reactive arthritis [ReA]

The aim of this work was to study the role of CT in early sacroiliitis and to detect changes in the patients four months after treatment with a short course of antibiotics, thus pointed out to the prognosis of patients with ReA. Thirty patients with ReA were included in this study. Complete history, clinical examination, plain X-ray of sacroiliac [SI] joint [AP view of pelvis] and CT scan of SI joint before and after antibiotic therapy [minocycline capsules, 100 mg/twice daily for four weeks] in CT proven sacroiliitis were done. The clinical findings included inflammatory low back ache in 93% of the patients with typical characteristics such as morning back stiffness, heel tenderness in 70%, inflamed joint of knee [asymmetrical] in 76.6%, urethritis in 26.6% and eye inflammation in 26.6% mainly as conjunctivitis. CT scan of SI joint before treatment showed erosions and narrowing in 26.6% of patients with reactive arthritis. CT scan findings of SI joints after four months of treatment showed the same bone erosions and narrowing in patients with reactive arthritis who already had previous findings. CT scan is superior to radiography in the imaging of early bone changes

Blood lead level in children with behavioral and or psychiatric disorders

A case-control study was carried out compromising 60 children enrolled into two groups: Group I included 40 cases suffering from different behavioral and/or psychiatric disorders and group II included 20 clinically healthy children serving as controls. Full medical history taking, assessment of exposure to risk factors of lead poisoning as well as its symptomatology using a standardized questionnaire, thorough clinical examination, delineation of behavior problems using Arabic translated child behavior checklist, diagnosis of psychiatric disorders in the studied cases using DSM IV criteria, scaling of depression and anxiety using children depression inventory [CDI] and child manifest anxiety scale, respectively, for children above the age of eight years, cognitive function assessment, language age and language age delay estimation, complete blood count, blood smear examination, assessment of blood indices and estimation of blood lead level using the technique of flameless atomic absorption spectrophotometry were done for all children enrolled in the current study. The current study showed a significant direct link between blood lead level and inattentiveness, conduct disorder and socialized aggression in the studied sample of cases with behavior and/or psychiatric disorders as well as a significant negative correlation with the patients' cognitive abilities and some hematological variables; namely, hemoglobin and mean corpuscular hemoglobin

Serum and CSF electrolytes, zinc and glucose concentration in febrile convulsions; are they useful biochemical markers in such cases?

This study was designed to assess serum and CSF concentrations of sodium, potassium, zinc and blood glucose in infants and children presented with febrile convulsions in comparison with age matched healthy controls. On the other hand, it aimed to investigate the correlation between serum and CSF concentrations of those variables in enrolled cases in a trial to delineate their usefulness as biochemical markers in such cases. Twenty-one cases with febrile convulsions [FC] were included in the current study as group I [cases] and ten age matched clinically healthy children were also enrolled as group II [controls]. Full history taking, thorough clinical examination, CBC and ESR estimation, assessment of serum Na, K, Zn and blood glucose concentrations were done for all subjects included in the current study. Lumbar puncture with complete CSF examination was carried out for all cases, excluding bacterial or viral meningitis or encephalitis and determining the CSF concentrations of the previously mentioned biochemical variables. In conclusion, it was recommended to assess Na and Zn levels in cases with febrile convulsions to detect any possible deficiency and to conduct future studies to investigate the effect of their supplementation, whenever deficient, on the course and recurrence of convulsions

Uremic pruritus: skin ultrastructure and laboratory study

Uremic pruritus is a common cause of morbidity for both hemodialysis [HD] and peritoneal dialysis patients. The pathogenesis is still conflicting. This work was planned to study skin ultrastructure and some biochemical parameters with possible relevance to pruritus including serum urea, creatinine, calcium, phosphorus, alkaline phosphatase, parathyroid hormone [PTH], and calcium-phosphorus products. The effects of dialysis duration, frequency, adequacy, and dialysate buffer were also studied. The study was conducted on 45 persons divided into three groups. Group 1 included 15 patients with CRF on HD with severe pruritus. Group II included 15 patients without pruritus on HD. Group III included 15 normal persons as control. Skin biopsy was performed for ten patients from each of group I and group II patients for electrom microscopic [EM] examination. Uremic pruritus was neither correlated to dialysis duration, frequency, adequacy, nor to dialyste buffer. The serum levels of urea, creatinine, calcium, alkaline phosphatase, and calcium-phosphorus products showed no significant difference between both HD groups. Whereas, serum phosphorus and PTH were significantly higher in both patient groups than controls, and in the pruritic than the non-pruritic HD patients. The present EM study demonstrated increased density of mast cells as well as an increase of mast cell granule size and number. These mast cell changes were common for both patient groups. Sweat glands in the pruritic, but not the non-pruritic group, revealed non-uniform cell morphology with altered villi. The nuclear envelope was irregular with segregation of nucleoli. The skin nerve endings, sebaceous glands and blood vessels were normal in both patient groups. It was concluded that the markedly elevated serum levels of phosphorus and PTH might play a crucial role in the development of pruritus in maintenance HD patients. Also, the co-occurrence of sweat gland changes with increased mast cell number; granularity and granule size could be a marker of uremic pruritus

Hepatitis B vaccination in hemodialysis patients: protective level of HBV antibodies could be achieved by the ordinary schedule?

Hepatits B vaccine is effective in producing protection against hepatitis B virus [HBV] infection in hemodialysis [HD] patients, but the antibody response is variable, so different vaccine regimens were adopted. The aim of the present work is to assess the immune response to HBV vaccine in 67 HD patients receiving 20 micro g of recombinant HBV vaccine / dose for 3 doses, intramuscularly [IM], at 0, 1, and 6- month intervals, compared with 36 medical staff members in the same unit as control receiving the same regimen. The results showed the presence of anti-HBc antibodies in 34 patients and 11 control subjects, those were exculded from the study so as to assess the effect of the vaccine only and not the natural infection. The antibody response is defined as protective when the level of anti-HBs antibodies exceeds or equals to 10 mlU/ml. The response rate was significantly lower in HD patients [51.5%] than in control subjects [96%] [P= 0.0002]. To months after the last dose of the vaccine the difference in seroprotection rate was insigificant being 62.5% in HD patients versus 66.7% in the control [P = 0.89], while after 6 and 12 months, the difference was statistically significant being 56.3%, 33.3% in HD patients versus 100%, and 100% in the control subjects respectively [P = 0.003, 0.006]. On the whole, the levels of anti-HBs, expressed as geometric mean titres and 95% confidence intervals [GMT [95% Cl]], were non-significantly lower in HD patients 125.64 [58.37-269.99] mlU/ml, than controls 239.67 [137.88-416.63] mlU/ml respectively [P = 0.149]. The gender, age, cause of renal failure, HCV co-infection, duration of HD, HD frequency, dialyzer membrane and dialysate buffer did not show a significant influence on the response to HBV vaccine. There were significant positive correlations between the level of anti-HBs antibodies and both serum albumin and predialysis urea [P = 0.00] indicating the improtant role of malnutrition on the poor response to the vaccine in HD patients. From this study it was concluded that the ordinary three-doses regimen is not enough for protection in a good proportion of HD patients, and other regimens should be assessed

Long term follow-up of surgrically treated skeletal deformities in osteogenesis imperfecta

In this study we evaluate the long term functional results in surgically treated Osteogenesis Imperfecta [O.I.] patients. The goals of our treatment are to maximize function, minimize deformity and disability. We reviewed 128 patients with O.I. who presented to the out patients Orthopaedic Department of the Paediatric Hospital, Cairo University. In this study we present the functional results in 31 surgically treated patients who responded to our letters among 62 operated children scince 1984. Most of the children were operated upon at a delayed age [average 6.5 years]. Our follow-up period range from 4 to 14 years [average 7 years]. None of these patients attended their follow-up dates on a regular basis, and our policy was to review them every 6 months. Revision surgeries were done for a large mumber of patients [71%]. Complications following surgery were common. At the final examination, there were only 4 patients walking without aid

Clinical and radiological parameters of posture in overweight individuals suffering of low back pain

Forty overweight individuals with low back pain and 40 normal [healthy] individuals were included in this study. An evaluation of the status of the sagittal spine angles in overweight individuals with low back pain and healthy individuals by clinical and radiological parameter was done. The results showed a significant difference in the angles of lumbar lordosis and sacral inclination between overweight and normal weight individuals, but there was no difference in the angles of thoracic kyphosis between both groups. The effect of body mass index seemed to be pronounced as the lumbar lordosis and sacral inclination in overweight individuals were increased and correlated with body mass index. There was also a correlation between the radiological evaluation of lumbar lordosis and sacral inclination in overweight individuals. However, the clinical judgment regarding the status of the lumbar lordosis and sacral inclination could be erroneous