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R Saxena

Publications and source records attributed to R Saxena.

At least 73 records · Page 4Linked to original sources
PubMed2002

Pregnancy associated aplastic anemia--a series of 10 cases with review of literature.

INTRODUCTION: Pregnancy induced aplastic anemia is a rare entity and the association is not well explained. There are approximately 80 cases in the literature and we are presenting the largest series, so far, of 10 cases. RESULTS: Total of 10 cases had 11 pregnancies. Mean age at presentation was 25.45 years and mean gestation when symptoms first developed was 17.09 weeks. Pallor and bleeding manifestations were the most common presenting complaints. Mean Hb, TLC, ANC and platelets were 4.97 g/dl, 2.74 x 10(9)/l, 1.11 x 10(9)/l and 41 x 10(9)/l, respectively. Bone biopsy cellularity ranged from <5 to 25%. Nine out of 11 (81%) pregnancies were successful of which 7 was full term and 2 were premature. Two babies were small for dates. One spontaneous abortion and one intra uterine death (IUD) were observed. Two out of 11 mothers died due to disease after delivery. Two of the 8 surviving mothers, had spontaneous partial response (22%); 4 mothers were asymptomatic after therapy with immunosuppressives given for 6 months and 3 were lost to follow up without response. Specific therapy (cyclosporin) was tried in two mothers antenatally with partial response in one. One child whose mother was given cyclosporin antenatally had jejunal atresia at birth. CONCLUSION: Pregnancy associated aplastic anemia is a rare association. Spontaneous remission can occur in 25-30% of patients. In the first trimester patients, pregnancy can be terminated while in advanced pregnancy patients can be followed up with stringent supportive care. Cyclosporin may be a safe drug antenatally in such patients. Patients with established aplastic anemia should avoid pregnancy.

Abortion, Spontaneous↗
PubMed2001

Congenital myotonic dystrophy.

Myotonic muscular dystrophy is the most frequent autosomal muscular dystrophy affecting adults and children. It affects multiple organ systems and is probably the best example of variable expressivity in a human disease. This article presents a patient with congenital myotonic dystrophy who had facial dysmorphism, hypotonia, talipes, feeding and respiratory difficulties in the neonatal period and later presented to us with developmental delay and had percussion myotonia. His mother had clinical and electrophysiological features of myotonia. Expansion of unstable CTG trinucleotide repeat in the myotonic protein kinase gene was demonstrated in both. The identification of this molecular defect allows its specific diagnosis in relation to other neuromuscular disorders as well as accurate prenatal diagnosis.

Child, Preschool↗
PubMed2001

Postpartum acquired haemophilia: clinical recognition and management.

Postpartum acquired haemophilia is a rare but serious complication of an otherwise normal pregnancy. Patients usually present with postpartum haemorrhage (PPH) or uncontrolled bleeding following surgical interventions, which fail to respond to conservative treatment. A high index of clinical suspicion along with early laboratory diagnosis and prompt institution of appropriate therapy is essential for the management of acute bleeding episodes. Our patient, a 32-year-old female, presented with severe PPH and shock. She had undergone dilation and curettage three times, with subsequent total abdominal hysterectomy and internal iliac artery ligation, before she was diagnosed with acquired haemophilia (factor VIII autoantibodies) and an inhibitor level of 8 Bethesda units (BU). The patient underwent an abdominal laparotomy for removal of the abdominal packing used in the previous operation, and blood and blood clots, and was given FEIBA(R) therapy. The patient responded to these measure and the factor VIII inhibitor level decreased to 2 BU at the time of discharge 10 weeks later.

Adult↗
PubMed2001

Danazol therapy in factor X deficiency.

Factor X (FX) deficiency is a rare coagulation disorder that usually presents with bleeding manifestations and is treated with fresh frozen plasma or prothrombin complex concentrates. We report a case of FX deficiency in which the patient presented with bleeding as well as thrombosis. The patient responded to Danazol and relapsed when the drug was stopped. The occurrence of thrombosis in FX deficiency and the role of Danazol in coagulopathies are reviewed.

Adult↗
PubMed2001

Determinants of pancreaticoenteric anastomotic leak following pancreaticoduodenectomy.

BACKGROUND: The purpose of the present paper was to study the incidence, presentation and management of pancreaticoenteric anastomotic (PEA) leak following pancreaticoduodenectomy (PD) and to identify risk factors associated with PEA leak. METHODS: One hundred and twenty patients underwent PD for benign and malignant pancreatic and periampullary lesions from 1989 to 1998. Prospectively collected data were analysed for incidence and outcome of PEA leak. Four clinical, three laboratory parameters, preoperative biliary drainage (PBD), perioperative octreotide use, nine intraoperative parameters, site of tumour and stage of malignant tumours were analysed by univariate and multivariate logistic regression analysis to identify factors influencing PEA leak. RESULTS: Pancreatic leak developed in 15 (12.5%) patients. Nine patients (60%) had a PEA leak that manifested as controlled leak through the drain. All were managed conservatively and the leak stopped after a mean duration of 17 days (range: 6-32 days). Six (40%) patients had associated intra-abdominal complications, and three (50%) died in the postoperative period. Pancreatic fistula healed in the three remaining patients after a mean duration of 18 days (range: 15-25 days). Diabetes (P = 0.02; odds ratio (OR) = 4.60; 95% confidence interval (CI): 1.23-17.18), PBD (P = 0.03; OR = 4.82; 95% CI: 1.21-19.24), sequence of reconstruction (bilioenteric anastomosis as first anastomosis; P = 0.01; OR = 6.25; 95% CI: 1.45-26.83) and duration of surgery > 8 h (P = 0.01; OR: 5.61; 95% CI: 1.54-20.39) were associated with a significantly higher incidence of PEA leak. CONCLUSION: Pancreaticoenteric anastomotic leak occurred in 12% of patients undergoing PD for pancreatic and periampullary tumours. The majority of these were uncomplicated and healed with conservative treatment. Complicated leaks were associated with high mortality. Diabetes mellitus, PBD, prolonged surgery and the sequence of reconstruction were risk factors associated with an increased incidence of PEA leak.

Adult↗
PubMed2001

A multi-center study in order to further define the molecular basis of beta-thalassemia in Thailand, Pakistan, Sri Lanka, Mauritius, Syria, and India, and to develop a simple molecular diagnostic strategy by amplification refractory mutation system-polymerase chain reaction.

The spectrum of the beta-thalassemia mutations of Thailand, Pakistan, India, Sri Lanka, Mauritius and Syria has been further characterized by a multi-center study of 1,235 transfusion-dependent patients, and the mutations discovered used to assess the fidelity of a simple diagnostic strategy. A total of 44 beta-thalassemia mutations were identified either by allele-specific oligonucleotide hybridization, amplification with allele-specific primers, or DNA sequencing of amplified product. The results confirm and extend earlier findings for Thailand, Pakistan, India, Mauritius and Syria. This is the first detailed report of the spectrum of mutations for Sri Lanka. Two novel mutations were identified, codon 55 (-A) and IVS-I-129 (A-->C), both found in Sri Lankan patients. Two beta-thalassemia mutations were found to coexist in one beta-globin gene: Sri Lankan patients homozygous for the beta0 codon 16 (-C) frameshift were also homozygous for the beta+ codon 10 (C-->A) mutation. Studies of Sri Lankan, Pakistani, and Indian carriers suggest the codon 10 (C-->A) mutation is just a rare polymorphism on an ancestral allele, on which the beta0 codon 16 (-C) mutation has arisen. Each country was found to have only a few common mutations accounting for 70% or more of the beta-thalassemia alleles. A panel of primers to diagnose the majority of the mutations by the amplification refractory mutation system was developed, enabling a simple molecular diagnostic strategy to be introduced for each country participating in the multi-center study.

Asia↗
PubMed2001

Laparoscopic bile duct injuries: spectrum at a tertiary-care center.

BACKGROUND AND PURPOSE: Laparoscopic cholecystectomy (LC) is associated with a higher incidence of bile duct injury than is open cholecystectomy. We reviewed our experiences with the management of laparoscopic bile duct injuries. PATIENTS AND METHODS: From October 1992 through August 1998, 34 patients with bile duct injuries (BDI) following LC were seen. The presentation, type of injury (Strasberg classification), management, and outcome were analyzed in these patients. RESULTS: Of the 16 patients who sustained injury at our center (type A [N = 9], D [N = 5], and E1 [N = 2]), in 14, the injury was detected during LC, and two patients manifested with postoperative bile leak. All patients had an excellent outcome at a median follow-up of 5.5 (range 1.9-8.0) years. Of the 18 patients who sustained injury elsewhere (type C [N = 1], D [N = 2], E [N = 14; 6 with external biliary fistula (EBF) and 8 with benign biliary stricture (BBS)], and not known [1]), 9 had EBF, 1 had biliary peritonitis, and 8 had BBS at the time of presentation. Of these 18 patients, 4 underwent early repair of the BDI before referral (repair over a T-tube [N = 2] and Roux-Y hepaticojejunostomy [N = 2]). Three of them developed restricture. One patient was referred to us within 12 hours of injury and had a successful repair over a T-tube. Two patients with early repair for lateral injury had an excellent outcome. Eleven patients with BBS underwent repair with an excellent (N = 10) or fair (N = 1) outcome at a median follow-up of 5.0 (2.0-6.2) years. Three patients were lost to follow-up. CONCLUSION: The spectrum of injuries sustained at LC at a tertiary-care center is different from that in the community hospitals. Missed injuries and attempts at repair in inexperienced hands result in serious sequelae of stricture formation and long-term morbidity.

Adult↗
PubMed2001

Outcome following pancreaticoduodenectomy in patients undergoing preoperative biliary drainage.

OBJECTIVE: To assess the role of preoperative biliary drainage (PBD) in the early outcome following pancreaticoduodenectomy (PD) for periampullary tumors. DESIGN: Retrospective analysis of prospective database. PATIENTS AND METHODS: 121 PDs were performed for periampullary tumors between 1989 and 1998. 54 patients were operated following a PBD (group A) while 67 patients were operated without PBD. 50 patients underwent internal biliary drainage while 4 patients underwent external biliary drainage. Of the 67 patients without PBD, serum bilirubin was >10 mg% in 41 patients (group B) while 26 patients had bilirubin level of <10 mg% (group C). RESULT: Patients were well matched for age, sex distribution, presence of medical risk factors, duration of surgery, operative blood loss and stage of disease. Group A patients had a higher incidence of wound infection (43 vs. 24%; p = 0.03), intra-abdominal abscess (28 vs. 15%; p = 0.06), pancreaticojejunal anastomotic leak (20 vs. 5%; p = 0.01) and overall infective complications (52 vs. 29%; p = 0.01) compared to group B patients, and a higher overall infective complication rate than group C patients (52 vs. 27%; p = 0.02). Group B patients had a higher incidence of intra-abdominal bleeding compared to group A (20 vs. 6%; p = 0.01) and group C patients (20 vs. 4%; p = 0.03). Reoperation rate was significantly higher in group B compared to group A patients (27 vs. 13%; p = 0.04). The mortality rates were not significantly different in the three groups. CONCLUSION: Patients with jaundice (>10 mg%) have a higher risk of bleeding complications while those with PBD have more infective complications. PBD should be judicially employed in selected patients.

Adult↗
PubMed2001

Risk of early death and recurrent stroke and effect of heparin in 3169 patients with acute ischemic stroke and atrial fibrillation in the International Stroke Trial.

BACKGROUND AND PURPOSE: We sought to investigate the apparently high risk of early death after an ischemic stroke among patients with atrial fibrillation (AF), identify the main factors associated with early death, and assess the effect of treatment with different doses of subcutaneous unfractionated heparin (UFH) given within 48 hours. METHODS: We studied the occurrence of major clinical events within 14 days among 18 451 patients from the International Stroke Trial, first for all treatment groups combined. Then, among patients with AF, we examined the effects of treatment with subcutaneous UFH started within 48 hours and continued until 14 days after stroke onset. RESULTS: A total of 3169 patients (17%) had AF. Seven hundred eighty-four patients were allocated to UFH 12 500 IU SC BID, 773 to UFH 5000 IU SC BID, and 1612 to no heparin. Within each of these groups, half of the patients were randomly assigned to aspirin 300 mg once daily. Compared with patients without AF, patients with AF were more likely to be female (56% versus 45%), to be old (mean age, 78 versus 71 years), to have an infarct on prerandomization CT (57% versus 47%), and to have impaired consciousness (37% versus 20%). The initial ischemic stroke type was more often a large-artery infarct (36% versus 21%). A lacunar stroke syndrome was less common (13% versus 26%). Death within 14 days was more common in patients with AF (17% versus 8%) and more often attributed to neurological damage from the initial stroke (10% versus 4%). The frequency of recurrent ischemic or undefined stroke was not significantly different (3.9% versus 3.3%). The proportion of AF patients with further events within 14 days allocated to UFH 12 500 IU (n=784), UFH 5000 IU (n=773), and to no-heparin (n=1612) groups were as follows: ischemic stroke, 2.3%, 3.4%, 4.9% (P=0.001); hemorrhagic stroke, 2.8%, 1.3%, 0.4% (P<0.0001); and any stroke or death, 18.8%, 19.4% and 20.7% (P=0.3), respectively. No effect of heparin on the proportion of patients dead or dependent at 6 months was apparent. CONCLUSIONS: Acute ischemic stroke patients with AF have a higher risk of early death, which can be explained by older age and larger infarcts but not by a higher risk of early recurrent ischemic stroke, although slightly more patients with AF died from a fatal recurrent stroke of ischemic or unknown type (1.3% versus 0.9%). In patients with AF the absolute risk of early recurrent stroke is low, and there is no net advantage to treatment with heparin. These data do not support the widespread use of intensive heparin regimens in the acute phase of ischemic stroke associated with AF.

Acute Disease↗
PubMed2001

SCA12 is a rare locus for autosomal dominant cerebellar ataxia: a study of an Indian family.

Spinocerebellar ataxia 12 (SCA12) is an autosomal dominant cerebellar ataxia (ADCA) described in a single family with a CAG repeat expansion in the PPP2R2B gene. We screened 247 index cases, including 145 families with ADCA, for this expansion. An expanded repeat ranging from 55 to 61 triplets was detected in 6 affected and 3 unaffected individuals at risk in a single family from India. The association of the PPP2R2B CAG repeat expansion with disease in this new family provides additional evidence that the mutation is causative.

Adult↗
PubMed2001

Significance of haematological manifestations in patients with tuberculosis.

OBJECTIVE: Tuberculosis is a major public health problem in India. Haematological changes associated with tuberculosis have been incompletely investigated. To the best of our knowledge, there is no comprehensive study assessing the haematological abnormalities in these patients from the Indian subcontinent. In the present study, we have compared peripheral blood and bone marrow findings in patients with disseminated/miliary tuberculosis (DTB/MTB) as well as pulmonary tuberculosis (PTB). An attempt has also been made to assess the effect of antituberculosis therapy on the haematologic abnormalities. MATERIAL AND METHODS: Thirty two patients with disseminated/miliary tuberculosis and 23 patients with pulmonary tuberculosis were prospectively studied to determine the various haematological manifestations in tuberculosis and the effect of antituberculosis therapy. All patients received standard antituberculosis treatment. They were subjected to a detailed haemogram including peripheral blood examination, which was repeated on completion of antituberculosis therapy. Bone marrow aspiration and biopsy was also done in all patients before starting antituberculosis treatment. RESULTS: Normocytic normochromic anaemia was the most common abnormality observed in all the groups and subgroups (DTB/MTB 84%, PTB 86%). Other haematological abnormalities of the white blood cells include leucopenia (DTB/MTB 25%, PTB 0%; p < 0.02), neutropenia (DTB/MTB 22%, PTB 0%; p < 0.04), lymphocytopenia, monocytopenia, leukocytosis, neutrophilia, lymphocytosis and monocytosis. Pancytopenia was observed only in patients with disseminated/miliary tuberculosis (p < 0.05). Thrombocytopenia was more common in patients with disseminated/miliary tuberculosis (p < 0.007), whereas thrombocytosis was more common in patients with pulmonary tuberculosis (p < 0.04). The patients of disseminated/miliary tuberculosis with granulomas in the bone marrow had certain significant differences as compared to patients without granulomas. These patients showed severe anaemia, peripheral monocytopenia and bone marrow histiomonocytosis. The haemogram reverted to normal with antituberculosis therapy in these patients. CONCLUSIONS: In view of the varied haematological abnormalities observed in patients with tuberculosis in this part of the world, it is concluded that the differential diagnosis of tuberculosis should be entertained in patients with varied haematological disorders.

Adult↗
PubMed2000

Four DAZ genes in two clusters found in the AZFc region of the human Y chromosome.

The DAZ genes are candidate fertility factors that lie within the human Y chromosome's AZFc region, whose deletion is a common cause of spermatogenic failure. The number of DAZ genes has been difficult to determine, in part because the nucleotide sequences of the DAZ genes are nearly identical. Here, fluorescence in situ hybridization and characterization of BAC clones revealed four full-length DAZ genes on the human Y chromosome. They exist in two clusters, each comprising an inverted pair of DAZ genes (3' <-- 5'::5' --> 3'). Analysis of genomic sequences and testicular transcripts suggested that three or four DAZ genes are translated. Each gene contains at least seven tandem copies of a previously described, 2.4-kb repeat unit that encodes 24 amino acids. In addition, two DAZ genes contain tandem copies of a 10.8-kb repeat unit that encodes the RNA-binding domain, which appears to be multimerized in some DAZ proteins. Combining our present results with previous studies, we can reconstruct several steps in the evolution of the DAZ genes on the Y chromosome. In the ancestral Y-chromosomal DAZ gene, amplification of both intragenic repeats began before the human and cynomolgus (Old World) monkey lineages diverged. During subsequent evolution, an inverted duplication of this modified gene occurred. Finally, the resulting two-gene cluster was duplicated, generating the two-cluster/four-gene arrangement found on modern human Y chromosomes.

Base Sequence↗
PubMed2000

[Clinical thinking and decision making in practice. A student with sudden headache].

A previously healthy man aged 18 years suddenly developed a severe headache, followed by nausea, vomiting, fever. During the following weeks a left-sided hemiparesis developed. CT of the brain revealed a pansinusitis and a frontal epidural empyema. A few days after surgical drainage his condition deteriorated and subsequent CT showed a right-sided subdural empyema. This was surgically evacuated and followed by long-term high-dose intravenous antibiotic therapy. The patient made a complete recovery. A sudden severe headache can be caused by an intracranial infection and intracranial pus collections can occur in the subdural as well as in the epidural space. Epidural empyema is a limited disease with relatively mild symptoms and a favourable prognosis, whereas subdural empyema may rapidly spread and cause severe disease with a poor prognosis. Quick antibiotic treatment and surgical drainage are required.

Acute Disease↗
PubMed2000

Derivation of hepatocytes from bone marrow cells in mice after radiation-induced myeloablation.

Following a report of skeletal muscle regeneration from bone marrow cells, we investigated whether hepatocytes could also derive in vivo from bone marrow cells. A cohort of lethally irradiated B6D2F1 female mice received whole bone marrow transplants from age-matched male donors and were sacrificed at days 1, 3, 5, and 7 and months 2, 4, and 6 posttransplantation (n = 3 for each time point). Additionally, 2 archival female mice of the same strain who had previously been recipients of 200 male fluorescence-activated cell sorter (FACS)-sorted CD34(+)lin(-) cells were sacrificed 8 months posttransplantation under the same protocol. Fluorescence in situ hybridization (FISH) for the Y-chromosome was performed on liver tissue. Y-positive hepatocytes, up to 2.2% of total hepatocytes, were identified in 1 animal at 7 days posttransplantation and in all animals sacrificed 2 months or longer posttransplantation. Simultaneous FISH for the Y-chromosome and albumin messenger RNA (mRNA) confirmed male-derived cells were mature hepatocytes. These animals had received lethal doses of irradiation at the time of bone marrow transplantation, but this induced no overt, histologically demonstrable, acute hepatic injury, including inflammation, necrosis, oval cell proliferation, or scarring. We conclude that hepatocytes can derive from bone marrow cells after irradiation in the absence of severe acute injury. Also, the small subpopulation of CD34(+)lin(-) bone marrow cells is capable of such hepatic engraftment.

Animals↗