Spinal Dysraphism with Accessory Male Genital Organs (A Case Report)

Clinico-Radiological and operative observations of a case of spinal dysraphism with accessory male genital organs are presented. Operative observations substantiated the presence of myelomeningocele in association with the spinal dysraphism in a case with normal sized penis, urethra and scrotum on top of a huge swelling over the back.     

Characterization of Three Isoforms of a 9kDa γD-Crystallin Fragment Isolated From Human Lenses

The purpose of this study was to determine whether a 9kDa γD-crystallin fragment, on in vivo post-translational modifications, exists as isoforms in the water soluble protein fraction of human lenses. In this study, three isoforms of the 9kDa polypeptide (named as 9kDa I, II and III) were identified and purified. In addition, the possible modified amino acids and their locations in the three isoforms were identified. The purification of the three isoforms was achieved by four steps which included separation of a mixture of crystallin fragments from the intact crystallins by a Sephadex G-50 chromatography under denaturing conditions, followed by purification of the 9kDa polypeptide isoforms by a non-denaturing gel electrophoresis, preparative SDS–PAGE and HPLC using a C-18 column. Each of the isoforms showed a single protein band and a single peak during SDS–PAGE and HPLC analyses respectively. The three isoforms on their partial N-terminal sequence analyses, exhibited sequence identical to γD-crystallin starting at residue no. 87 suggesting that the isoforms contained residues no. 87 to 173 of γD-crystallin. On comparison of the amino acid compositions of the isoforms with that of the identical 9kDa γD-crystallin fragment, the isoforms showed relatively lower amino contents of Asp, Arg, Leu and Tyr residues suggesting modifications of these residues in the isoforms. To identify the specific regions at which these amino acid residues were modified, the Western blot analysis with six site-specific polyclonal antibodies to six regions of the 9kDa γD-crystallin polypeptide was carried out. Of the six antibodies raised, one was to the N-terminal region (residue nos 87–95; named as anti-9kDaN-Ab), second to the C-terminal region (residue nos 165–173; named as anti-9kDaC-Ab) and four to the four different middle regions [named as anti-9kDa M1 (nos 94–100)-Ab, M2 (nos 114–120)-Ab, M3 (nos 137–143)-Ab and M4 (nos 149–154)-Ab] of the polypeptide. The Western blot analysis suggested that the 9kDa I and 9kDa II isoforms had modified amino acid residues in the regions of residue nos 114–120 and 165–173 whereas the 9kDa III isoform in the regions of residue nos 114–120, 137–143, 149–154 and 165–173. It was also determined whether the 9kDa isoforms exhibit an age-related appearance in human lenses. Western blot analysis as above of the WS-proteins from lenses from donors of different ages was carried out. On comparison of these results with an identical Western blot analysis of the three purified 9kDa isoforms, I, II and III, it was inferred that the 9kDa isoform III appeared earlier than other isoforms during aging in human lenses.     

Glutathione Status and Cadmium Neurotoxicity: Studies in Discrete Brain Regions of Growing Rats

Glutathione Status and Cadmium Neurotoxicity: Studies in DiscreteBrain Regions of Growing Rats. Shukla, G. S., Srivastava, R.S., and Chandra, S. V. (1988). Fundam. Appl. Toxicol. 11, 229-235.Intraperitoneal administration of cadmium (Cd2+, 0.4 mg/kg)daily for 30 days to rats was found to decrease the contentsof reduced glutathione (GSH) and increase oxidized glutathione(GSSG) in various brain regions. These changes resulted in asignificant decline in the GSH/GSSG ratio in different brainregions, except for the hippocampus and midbrain. In addition,the activities of glutathione reductase (GR) and glucose-6-phosphatedehydrogenase (GPDH) were also significantly inhibited in differentbrain regions. Measurement of regional Cd levels revealed thatCd administration significantly increased the levels in allbrain regions except for the hippocampus, which could be thereason for not finding any change in any of the biochemicalparameters studied in this region. The observed changes in theregional GSH/GSSG ratios could be the result of inhibition inGR activity, as this enzyme catalyzes an irreversible conversionof GSH to GSSG and is responsible for higher cellular GSH levels.GR uses NADPH in its reaction; therefore, the inhibition ofGPDH may further aggravate the situation because of the shortsupply of NADPH. The alterations in the regional "glutathionestatus" may affect various related metabolic processes, includingthose required for detoxification of lipid peroxides which haverecently been suggested to play a role in the mechanism of Cdneurotoxicity.     

Hand dermatitis/eczema: Current management strategy

Ever since its inception a couple of centuries ago, hand dermatitis/eczema has been in the reckoning. Idiosyncrasies continued to loom large thereafter, till it acquired its appropriate position. Dermatitis/eczema are synonymous, often used to indicate a polymorphic pattern of the inflammation of the skin, characterized by pruritus, erythema and vesiculation. A spectrum delineated into acute sub‐acute and chronic dermatitis of the hands. Pompholyx, recurrent focal palmer peeling, ring, wear and tear and fingertip eczema, apron, discoid eczema, chronic acral dermatitis, gut and patchy papulosquamous eczema are its clinical variants. Occupational dermatitis/eczema may be contributory. Etiological definitions are clinched by detailed history of exogenous and endogenous factors. However, scientific confirmation of the entity is through patch testing by using available antigens.     

Oxidative stress in non‐small cell lung cancer patients after chemotherapy: Association with treatment response

Background and objective: The aim of this study was to assess the level of oxidative stress after chemotherapy in non‐small cell lung cancer patients, and its association with treatment response and survival. Methods: Two hundred and three previously untreated non‐small cell lung cancer patients and 150 healthy subjects were selected for the study. Patients received cisplatin + etoposide, and were followed for up to six cycles, for evaluation of oxidative stress. Blood levels of lipid peroxidation products (LPO), glutathione (GSH) and nitric oxide (NO), and superoxide dismutase (SOD) activity were measured at day 0 and after the third and sixth cycles of chemotherapy. Response and survival were measured at the end of follow up. Survival was estimated by the Kaplan–Meier method using the log–rank test. Results: In the patients, pretreatment levels of LPO and NO were low, while GSH and SOD levels were high compared with those after the third and sixth cycles of chemotherapy. Among the 203 patients, there were 51 deaths, 82 non‐responders and 70 responders at the end of the sixth cycle. Overall mean survival was higher among responders than non‐responders (24.6 vs 21.2 weeks, P < 0.01). The hazard ratio was 2.4 (95% CI: 1.3–3.77). Pretreatment levels of oxidative stress were similar among responders and non‐responders (P > 0.05). After the third and sixth cycles of chemotherapy, LPO and NO levels were low (P < 0.05 or P < 0.01) and GSH levels and SOD activity were high (P < 0.05 or P < 0.01) in responders as compared with non‐responders. Conclusions: In lung cancer patients, oxidative stress increased and anti‐oxidant enzymes decreased as the disease progressed. Chemotherapy may suppress oxidative stress and decreased anti‐oxidant enzyme activity in responders as compared with non‐responders. These effects may contribute to improved survival among responders.     

A study of reported factor IX use around the world

Summary. Replacement therapy has significantly improved the life expectancy and lifestyle of people with haemophilia. The objectives of this article were to study the reported factor IX (FIX) use on a country‐by‐country basis and address the following question: Does the reported FIX use vary by national economies? We obtained data on the reported number of international units (IUs) of FIX used for 90 countries from the Marketing Research Bureau and the World Federation of Hemophilia. Results show that the reported FIX use varies considerably across national economies, even among the wealthiest of countries.Trends suggest that the reported FIX usage increases with increasing economic capacity and has been increasing over time. Trends also suggest that consumption of FIX has been increasing at a greater rate in high income countries. Given these trends, there will likely be an overall increase in the amount of FIX concentrates used in the treatment of haemophilia B. We also found that FIX use both in terms of IUs per capita and IUs per person provide a complete picture of the level of haemophilia care within a country. Such information is critical for planning efforts of national healthcare agencies to determine realistic budget priorities and pharmaceutical manufacturers to determine adequate production levels of FIX concentrates. By improving the data collection and surveillance of FIX use for the treatment of people with haemophilia B, we can identify trends and needs of patients and highlight best treatment practices among countries.     

A patient‐prioritized ability assessment in haemophilia: the Canadian Occupational Performance Measure

Summary. Assessment of musculoskeletal function in individuals with haemophilia has been attempted with clinimetric instruments, which use predetermined domains for assessing the same. This study introduces the application of an instrument, the Canadian Occupational Performance Measure (COPM), which is an open‐ended questionnaire that allows patients to prioritize their needs and rate their performance in different tasks of daily living as well as their satisfaction in performing them. To study the utility of COPM in evaluating the musculoskeletal functional status of patients with haemophilia and to assess its effectiveness in planning individualized management plans for them. COPM was administered to 67 individuals with haemophilia aged 10–55 years and the data were compared with functional deficits identified through FISH (Functional Independence Score for Haemophilia). A total of 31 performance difficulties in the areas of self‐care (62%), productivity (21%) and leisure (17%) were identified by COPM. All eight domains of FISH were identified in COPM as problems in self‐care. In addition to these, COPM identified problems in the areas of productivity and leisure. In 78% of the responses on COPM, there was concordance between the performance and satisfaction scores. However, there was discordance between the two in the remaining 22% of responses. COPM is a useful tool for assessment of musculoskeletal dysfunction in haemophilia. It provides a greater insight into the needs of each patient and helps in planning individualized intervention strategies.     

Clinical outcome of 36 male patients with primary urethral carcinoma: A single center experience

AIM: Retrospective analysis of male urethral carcinoma to assess the best therapeutic approach to the management of this tumor. METHODS: A review of 36 cases of male urethral carcinoma diagnosed and treated at our center was performed. Clinical features, treatment modality and outcomes were analysed. RESULTS: The overall median survival time was 55.16 months. The 5-year overall and disease-free survival rate for the cohort was 49% and 23%, respectively. The 5-year survival is 67% for low-stage versus 33% for high-stage tumors and is significantly different (P = 0.001). The survival was 72% for tumors of the distal urethra versus 36% for tumors of the proximal, with a P-value of 0.02. CONCLUSION: The tumor location and clinicopathological stage were the most important predictors of the disease-free and overall survival. Multimodal approach is necessary for achieving local control especially for proximal and higher stage tumors.