Early parenteral supplementation with glutamine dipeptide for acute myeloid leukaemia patients receiving high dose chemotherapy

Background: Glutamine dipeptide [Gln] is one of conditionally essential amino acids that have a crucial role in cancer

Objectives: The aim of this study was to investigate the effects of early Gln supplementation in patients with acute myeloid leukemia [AML] receiving intensive chemotherapy, but without bone marrow transplant and regardless of receiving parenteral nutrition

Methods: A randomized control trial was carried out and included 46 AML patients who received high dose chemotherapy. Their ages ranged between 17-65 years. AML patients were randomly allocated as intervention group [Gln group; n=23] and control group [n=23]. Gln group received intravenous supplementation with Gln [40 gm] from day 1 to day 5 of chemotherapy, while the patients in the control group received 40 gm per day of a standard amino acid mixture. Clinical end points included the body weight, body mass index, length of hospital stay, days of neutropenia, superoxide anion generation and length of neutropenic fever and serum albumin difference. White blood cells and absolute neutrophil count were done every second day till ANC reached >500microl then superoxide anion generation was measured

Results: Weight loss, length of hospital stay, the mean days of neutropenia and neutropenic fever were significantly lower in the Gln group, whereas the mean superoxide anion generation was found higher comparing to the control group [50 ng/L vs 43ng/L respectively]. Complications other than febrile neutropenia were significantly less common in Gln group. Gln supplementation has resulted in significant improvement in serum total protein and serum albumin

Conclusion: This trial suggested that early Gln supplementation regardless receiving parenteral nutrition enhances neutrophil function, maintains nutritional status and decreases hospital stay

Gene mutations in Wilson disease in Egyptian children: report on two novel mutations

Wilson disease [WD] is an autosomal recessive disorder, caused by defects in copper-transporting P-type adenosine triphosphatase [ATPase] encoded by the ATP7B gene, resulting in the deposition of copper in the liver and brain with significant disability or death if left untreated. An available regimen of treatment gives hope to those predisposed to the disease if diagnosed early. The objective of this study was to determine the frequency of the most common European mutation [p.H1069Q] in Egyptian children with WD, in addition to screening for previously reported mutations in the Egyptian patients in our selected group. Direct DNA sequencing was applied to exons [13, 14, 18, and 19] of the ATP7B gene for 19 patients previously diagnosed with WD. Then DNA sequencing and pedigree analysis were performed in the families of the patients showing variations in their results for the purpose of family screening and carrier detection. Six out of 19 patients were studied with their families [three families]. We identified five variants of which two were novel among the studied patients. One of the novel variants was synonymous substitution [p.A1074A] in 16% of patients and the other was predicted to be missense disease-causing mutations [p.T1076I] in 16% of patients, and three previously published mutations p.H1069Q were detected in 5% of patients, p.P1273Q in 10% of patients, and a silent variant p.A1003A in 26% of patients. Screening for the two exons 14 and 18 of the ATP7B gene is important in Egyptian patients especially in suspected patients without hepatic manifestations

Breast conserving therapy for early breast cancer: long term follow-up

Breast conserving surgery followed by breast irradiation for appropriately selected patients with early stage breast cancer gives the same long term survival rates as radical mastectomy. The large breast volume of female Egyptian patients allows for a wide resection margin during wide local excision without a marked alteration in the aesthetic breast contour. Between 1995 and 2004, 101 female patients with early stage breast cancer were diagnosed and treated, at the Cairo National Cancer Institute hospital, by breast conserving surgery followed by postoperative radiotherapy and adjuvant systemic therapy. Patients were regulary followed up till the end of the study. Mean age of patients was 47.1 years [SD +/- 10.2]. The median time of follow-up of patients was 34 months [range: 3-127]. Kaplan-Meier estimate of 5-year local recurrence rate was 11.5% [95% confidence interval]. There was no axillary nodal recurrence. The cumulative 5 year disease tree survival was 80%. 44.5% of patients showed a good cosmetic results while 40.6% showed a fair cosmetic result. It is concluded that, inappropriately selected patients with an early stage breast cancer, they should be offered breast conserving surgery followed by breast radiation. BCT do not affect patient's survival, it preserves the breast in an acceptable cosmetic result and avoid the mutilating result of mastectomy

Speech and click evoked auditory brainstem response in delayed language children with normal pure tone audiogram

Children with normal auditory thresholds may still suffer from auditory processing disorders, resulting in poor speech understanding. Abnormal electrophysiological responses to speech syllables at the brainstem level have been associated with a wide spectrum of diagnosed learning problems. The aim of this study was to identify the pattern of speech evoked brainstem response in normal peripheral hearing children with delayed language development and to develop an objective method to test those children. The speech and click evoked ABR results of twenty children, their age ranging from 4-6 years [of both genders], complaining from delayed language development with normal pure tone audiogram and normal IQ results were compared to the results obtained from twenty normal children matching in age and gender. There was no significant difference in the wave V latency and amplitude values of click-evoked ABR in normal and delayed language subjects. However, a statistically significant difference between the wave V latency and amplitude values of the speech-evoked ABR in normal versus delayed language subjects was detected. There was also a highly significant difference in the duration and slope of the V-to-A transition between the two groups. Results obtained from this study suggest that speech evoked ABR may be a useful objective tool in the early diagnosis of children with delayed language development. More wide scale studies on speech evoked ABR must be conducted to obtain age related norms, and to assess other abnormalities such as learning disabilities

Intratympanic injection in sudden sensorineural hearing loss

Purpose: the aim of this study was to evaluate intratympanic corticosteroid injection in cases with idiopathic sudden sensorineural hearing loss [SSNHL]

Material and Methods: seventy patients with sudden onset sensorineural hearing loss [SSNHL] were included in this study; 40 females and 30 males. Their age ranged from 14 to 67 years old. The hearing loss was graded into mild, moderate, severe and profound SNHL. The patients were classified into 3 groups according to the treatment modality; Group I did not receive corticosteroid therapy and included 10 patients, group II received systemic corticosteroid therapy and included 15 patients and group III received intratympanic corticosteroid therapy and included 45 patients

Results: in group I, 30% of patients showed improvement and in group 11, 40% of patients showed improvement and in group III, 82% showed improvement of hearing

Conclusion: intratympanic corticosteroid therapy for SSNHL has a better success rate than systemic corticosteroid therapy and control patients with no treatment. It has a low incidence of complications. Patients with mild and moderate hearing loss have a better prognosis than patients with severe and profound hearing loss

Probe microphone real ear insertion gain verification in children

The application of probe-microphone measurements to children is one of the most useful new developments in pediatric hearing aid selection. They offer a reliable method to verify hearing aid selection decisions. To assess the matching between real ear insertion gain [REIG] and prescribed target gain according to the desired sensation level [DSL] formula. This study included 40 ears of 30 children aged between 4 to 12 years with different degrees of hearing loss. They were fitted with linear hearing aids [10 binaural and 20 monaural]. Real ear unaided response [REUR], and real ear aided response [REAR] were measured. The real ear insertion gain was then calculated and compared to the prescribed target gain. There were 26 ears [of 19 children] failed to be within 10 dB of the DSL target gain at one or more frequencies from 500-3000Hz. Among these 26 ears who failed to achieve the DSL target gain, there were 16 ears [61.5%] found to have a flat audiogram and 13 ears [50%] had profound hearing loss. The difference in hearing threshold level was found to be of no clinical value in predicting the likelihood of failure. After appropriate modifications in hearing aid fitting, out of the 16 children [22 ears] who attended for hearing aid modifications, 18 ears [82 %] achieved a satisfactory gain within 10dB of the DSL target at all frequencies up to 3000Hz. No audiometric index was of clinical value in predicting those cases that required insertion gain measurements to ensure an adequate hearing aid prescription. The number of children who fail to achieve adequate gain with their initial hearing aid fitting is very high. These children have a wide range of degree and configuration of hearing loss. The routine measurement of real ear insertion gain in all first-time fittings would result in greatly improved amplification in many patients. More research in the field of pediatric hearing aid fitting is recommended to ensure delivery of adequate amplification to infants and young children

Spontaneous regression of lumbar disc herniation

We present 3 cases of spontaneous regression of lumbar herniated disc. The disc regression correlated with clinical improvement documented by MRI studies. Although the phenomenon of spontaneous disappearance of decrease in size of herniated disc fragments is well known, the exact mechanism underlying this process remains unclear. We discuss 3 possible explanations for disc regression: retraction into the vertebral space, dehydration/shrinkage, and resorption due to inflammatory reaction

Neurosurgical gossypiboma radiologically mimicking a lumbar abscess

Lumbar discectomy is the most common operative technique at neurosurgery clinics around the world. The complications of lumbar disc operation include infections, dural tear, bleeding, vascular, and intestinal injuries. Infectious complications of lumbar disc surgery are superficial and profound tissue infections, meningitides, and epidural abscess. Although retained surgical sponges [gossypiboma] are well known intraoperative complications in other surgical branches, they have not been widely reported in neurosurgery. In this report, we present a case of retained surgical sponge at the operation site and discuss with the literature

Rotary chair findings in patients with peripheral vestibular lesions

Imbalance and vertigo are among the most common symptoms causing patients to visit a physician. The rotational chair is one test modality used in the diagnosis of peripheral vestibular lesions through its ability to test higher, more physiologic frequencies and provides an adjunct to caloric and other ENG tests. The aim of this study was to assess the role of low frequency rotary chair testing in the evaluation and diagnosis of peripheral vestibular lesions. Forty subjects suffering from unilateral peripheral vestibular lesions, documented by the caloric test participated in this study. Twenty healthy adults served as control. They were subjected to: history taking, audiological evaluation, ENG and rotational chair testing in the form of Rotational Sinusoidal Harmonic Acceleration [SHA] test and Rotational Velocity Step Test [VST]. Results of the SHA test showed phase lead and reduced gain at low and mid frequencies [p<0.001] that improved at higher frequencies, together with reduced time constant [TC] in the velocity step test [p<0.001] between the study and control groups. TC was reduced in the study group in response to rotation towards the side of the lesion. Results of the VOR phase and gain between study groups according to different etiologies revealed that acoustic neuroma had the highest mean in the gain parameter [p<0.01]. In patients with unilateral peripheral vestibular lesions: gain reduction and phase lead are the main abnormalities seen with SHA test and are more pronounced at low frequencies of the test range. Rotational VST demonstrated response asymmetries in the form of reduced TC in response to rotation towards the side of the lesion. The onset and course of the peripheral vestibular lesion have an important effect on the degree of compensation mechanism

Elevated intracranial pressure management

The treatment strategy for increased intracranial pressure [ICP] management includes decisions on head and body position, hypothermia, ventilation, anesthetics, osmotic drugs and surgical procedures. We can treat sudden increases in ICP using osmotic agents, some anesthetics and short episodes of mild hyperventilation. Propofol seems to be suitable for sedation of the increased ICP patients. Surgical decompression of the cranium seems to improve the outcome of the younger patients [below 50 years old], especially children

Milk clotting enzyme from bacillus stearothermophilus

A large number of thermophilic bacilli were screened for their ability to produce extracellular milk clotting enzyme as well as protease activity. A potent strain exhibited high milk clotting activity with lower protease formation was identified and characterized as Bacillus stearothermophilus NRC 41. Optimum temperature and pH for milk clotting enzyme activity was found to be 65-70 degrees and 6.1 respectively. Maximum enzyme activity was obtained when CaCl2 was added to skim milk at a concentration of 125 mg/l. Maximum yield of enzyme was obtained after 48 hr of incubation at an initial pH of 7.0. From all carbon and nitrogen sources tested, lactose and NH4NO3 respectively were found to be the most suitable for enzyme formation. On the other hand, the presence of glucose, sucrose, fructose or raffinose as well as [NH4]2SO4, [NH4]3PO4 or NH4Cl in the medium resulted in suppressing enzyme formation

B-Glucosidase from yeasts

Thirty two yeast strains belonging to different genera were screened for their ability to produce beta-glucosidase. The qualitative analysis showed' that only seven strains [21.9%] exhibited highest productivity while quantitative assay proved that kluyveromyces fragilis NRRL Y-1137 was the most resuhful strain. In addition, its beta-glucosidase was successfully purified by fractional precipitation with acetone. On the other hand, the properties of the partially purified enzyme obtained were as follows: Optimum temperature of 50°, optimum pH value 6.2 and the rate of pNPG hydrolysis was linear up to 20 min under optimum assay conditions. Addition on cellobiosc or glucose to the reaction mixture decreased the enzyme activity. Furthermore, the enzyme was relatively more stable at pH 6.0-7.0 and rapidly inactivated at > 50°

Carbon sources influencing the production of cellulases and proteases by different cellulolytic microorganisms

Treated corn cobs cellulose [2%] was used as the only carbon source for the growth and production of extracellular cellulases and proteases by four cellulolytic microbes: Penicillium funiculosum, Phanerochaete chrysosporium, Micromonospora calcea and Trichoderma virideTViride and Pfuniculosum showed a great ability for the production of carboxy-methyl cellulase [CMCase], filter paper-ase [FPA], neutral and acid proteasesMaximum neutral protease activity was obtained with MCalcea and TvirideOptimum activities in relation to the incubation periods varied according to the enzyme studied and the organism testedThe effect of different carbon sources on cellulases and proteases production indicates that cellulose and lactose were good inducers for CMCase production by Pfuniculosum and PhChrysosporium respectivelyThe latter inducers were also the best for the production of neutral proteases by Tviride and Mcalcea respectively, while cellobiose and lactose were the best inducers for acid protease production by PFuniculosum and Phchrysosporium, respectively