Publications by authors named "Samuel Oommen"

19 Publications

  • Page 1 of 1

Unusual Presentation of Adrenal Hypoplasia Congenita.

Indian J Pediatr 2020 Oct 9;87(10):865-866. Epub 2020 Jul 9.

Developmental Pediatrics Unit, Department of Child Health, Christian Medical College, Vellore, Tamil Nadu, India.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12098-020-03239-6DOI Listing
October 2020

Developmental Delay with Intermittent Twisting of Neck.

Indian Pediatr 2020 05;57(5):469-470

Developmental Pediatrics unit, Christian Medical College and Hospital, Vellore, India.

View Article and Find Full Text PDF

Download full-text PDF

Source
May 2020

Metabolic Stroke: A Novel Presentation in a Child with Succinic Semialdehyde Dehydrogenase Deficiency.

Ann Indian Acad Neurol 2020 Jan-Feb;23(1):113-117

Department of Medical Genetics, Christian Medical College, Vellore, Tamil Nadu, India.

Succinic semialdehyde dehydrogenase (SSADH) deficiency is an autosomal recessive disorder of gamma-aminobutyric acid metabolism. Children with SSADH deficiency usually manifest with developmental delay, behavioral symptoms, language dysfunction, seizures, hypotonia, extrapyramidal symptoms, and ataxia. Diagnosis of SSADH deficiency is established by an abnormal urine organic acid pattern, including increased excretion of 4-hydroxybutyric acid and the identification of biallelic pathogenic variants in aldehyde dehydrogenase 5 family, member A 1 () gene. Here, we describe a 15-month-old girl with SSADH deficiency presenting with developmental delay, language deficits, and acute-onset right hemiparesis, following recovery from a diarrheal illness. Brain magnetic resonance imaging revealed hyperintense signal changes involving the left globus pallidus in T2-weighted images with restriction of diffusion in the diffusion-weighted images. Increased excretion of 4-hydroxybutyric acid, threo-4,5-dihydroxyhexanoic acid lactone and erythro-4,5-dihydroxyhexanoic acid lactone was detected by urine organic acid analysis and a diagnosis of SSADH deficiency was confirmed by the identification of homozygous pathogenic variant in . Stroke mimic is a novel presentation in our patient with SSADH deficiency. She was initiated on treatment with vigabatrin and has shown developmental gains with the recovery of right hemiparesis. Follow-up neuroimaging shows near complete resolution of signal changes in the left globus pallidus, while there was subtle hyperintensity in the right globus pallidus. The phenotypic spectrum of SSADH deficiency is widely expanding, and this disorder should be considered in the differential diagnosis of children with metabolic stroke.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/aian.AIAN_213_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7001443PMC
February 2020

Neurodevelopmental Outcomes of Very Low Birth Weight Infants at 18-24 Months, Corrected Gestational Age in a Tertiary Health Centre: A Prospective Cohort Study.

J Trop Pediatr 2019 12;65(6):552-560

Department of Biostatistics, Christian Medical College, Vellore, Tamil Nadu, India.

Objective: To determine the prevalence and risk factors for poor neurodevelopmental outcome in a cohort of very low birth weight (VLBW) infants.

Subjects And Methods: Four hundred and twenty-two infants of a total of 643 VLBW survivors from a teaching hospital in South India were followed up to assess their neurodevelopmental outcomes.

Results: Among the 422 children who completed the assessment, results of 359 children whose assessments were done between 18 and 24 months were analysed. Thirty-seven children (10.31%) had poor neurodevelopmental outcome, six children [1.67%] had cerebral palsy, one child had visual impairment and another had hearing impairment. Poor post-natal growth was independently associated with poor neurodevelopmental outcomes in the multivariate analysis (p = 0.045). Neonatal complications were not associated with the developmental outcome.

Conclusion: Despite lower rates of neonatal complications compared with Western cohorts, significant proportion of VLBW infants had poor neurodevelopmental outcomes. Poor post-natal growth was an important determinant of the developmental outcome.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/tropej/fmz006DOI Listing
December 2019

Consolidation mFOLFOX6 Chemotherapy After Chemoradiotherapy Improves Survival in Patients With Locally Advanced Rectal Cancer: Final Results of a Multicenter Phase II Trial.

Dis Colon Rectum 2018 Oct;61(10):1146-1155

Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York.

Background: Adding modified FOLFOX6 (folinic acid, fluorouracil, and oxaliplatin) after chemoradiotherapy and lengthening the chemoradiotherapy-to-surgery interval is associated with an increase in the proportion of rectal cancer patients with a pathological complete response.

Objective: The purpose of this study was to analyze disease-free and overall survival.

Design: This was a nonrandomized phase II trial.

Settings: The study was conducted at multiple institutions.

Patients: Four sequential study groups with stage II or III rectal cancer were included.

Intervention: All of the patients received 50 Gy of radiation with concurrent continuous infusion of fluorouracil for 5 weeks. Patients in each group received 0, 2, 4, or 6 cycles of modified FOLFOX6 after chemoradiation and before total mesorectal excision. Patients were recommended to receive adjuvant chemotherapy after surgery to complete a total of 8 cycles of modified FOLFOX6.

Main Outcome Measures: The trial was powered to detect differences in pathological complete response, which was reported previously. Disease-free and overall survival are the main outcomes for the current study.

Results: Of 259 patients, 211 had a complete follow-up. Median follow-up was 59 months (range, 9-125 mo). The mean number of total chemotherapy cycles differed among the 4 groups (p = 0.002), because one third of patients in the group assigned to no preoperative FOLFOX did not receive any adjuvant chemotherapy. Disease-free survival was significantly associated with study group, ypTNM stage, and pathological complete response (p = 0.004, <0.001, and 0.001). A secondary analysis including only patients who received ≥1 cycle of FOLFOX still showed differences in survival between study groups (p = 0.03).

Limitations: The trial was not randomized and was not powered to show differences in survival. Survival data were not available for 19% of the patients.

Conclusions: Adding modified FOLFOX6 after chemoradiotherapy and before total mesorectal excision increases compliance with systemic chemotherapy and disease-free survival in patients with locally advanced rectal cancer. Neoadjuvant consolidation chemotherapy may have benefits beyond increasing pathological complete response rates. See Video Abstract at http://links.lww.com/DCR/A739.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/DCR.0000000000001207DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6130918PMC
October 2018

A Tale of Treatable Infantile Neuroregression and Diagnostic Dilemma with Glutaric Aciduria Type I.

J Pediatr Neurosci 2017 Oct-Dec;12(4):356-359

Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.

Nutritional deficiencies related neurological manifestations are not uncommon in infants and children. Here, we describe an infant with Vitamin B12 deficiency due to depleted maternal Vitamin B12 status presenting with progressive encephalopathy and extrapyramidal signs. Diagnosis of infantile tremor syndrome was established in our patient based on the clinical and biochemical parameters. Magnetic resonance imaging had shown frontotemporal atrophy with widened Sylvian fissures and prominent cerebrospinal fluid spaces. Clinical and imaging findings might create a diagnostic dilemma with glutaric aciduria type I. Knowledge and identification of infantile tremor syndrome are essential, as it is a potentially treatable disorder. Our patient had significant developmental gains with Vitamin B12 treatment and infant stimulation program. Vitamin B12 deficiency must be looked for as a cause of neuroregression in children hailing from low socioeconomic status, infants of vegetarian mother, and infants with delayed or improper weaning. Screening for Vitamin B12 deficiency is essential in all infants and children with unexplained neuroregression, as this disorder is potentially treatable. More population-based studies in India are needed to explore the prevalence of Vitamin B12 deficiency in pregnant and lactating women and also to assess the need for Vitamin B12 supplementation during pregnancy and lactation.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/jpn.JPN_35_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5890558PMC
April 2018

Dysmorphism in Non-Syndromic Autism: A Cross-Sectional Study.

Indian Pediatr 2017 Jul 2;54(7):560-562. Epub 2017 Feb 2.

Developmental Paediatrics Unit, Christian Medical College (CMC) and Hospital, Vellore, India. Correspondence to: Dr Susan Mary Zachariah, Developmental Paediatrics Unit, CMC, Vellore, India.

Objective: To determine the effect of association of dysembryogenesis (manifested by presence of dysmorphic markers) on the developmental profile of autistic children.

Methods: 26 autistic children were classified into complex autism (if they had specific dysmorphic markers) or essential autism (in the absence of dysmorphic markers) using the Miles Autism Dysmorphology Measure (ADM). The developmental abilities (Griffith's Mental Development Scales) and the clinical severity (Childhood Autism Rating Scale) of both groups were compared. The prevalence of dysmorphic markers was also determined in 140 non-autistic controls.

Results: Children with complex autism had poorer development (General Quotient 29.4 vs 34.0, P=0.06) and earlier onset of autistic symptoms (18 vs 24 mo, P=0.05). Dysmorphic markers were significantly more in autistic children compared to normal children (27% vs 10%, P=0.002).

Conclusion: Dysembryogenesis may contribute to the clinical heterogeneity of autistic children.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s13312-017-1068-4DOI Listing
July 2017

Early motor repertoire in very low birth weight infants in India is associated with motor development at one year.

Eur J Paediatr Neurol 2016 Nov 30;20(6):918-924. Epub 2016 Jul 30.

Department of Laboratory Medicine, Children's and Women's Health, Faculty of Medicine, Norwegian University of Science and Technology, P.O. Box 8905, 7491 Trondheim, Norway; Department of Pediatrics, St. Olavs Hospital, Trondheim University Hospital, P.O. Box 3250 Sluppen, 7006 Trondheim, Norway. Electronic address:

Background: Most studies on Prechtl's method of assessing General Movements (GMA) in young infants originate in Europe.

Aim: To determine if motor behavior at an age of 3 months post term is associated with motor development at 12 months post age in VLBW infants in India.

Methods: 243 VLBW infants (135 boys, 108 girls; median gestational age 31wks, range 26-39wks) were video-recorded at a median age of 11wks post term (range 9-16wks). Certified and experienced observers assessed the videos by the "Assessment of Motor Repertoire - 2-5 Months". Fidgety movements (FMs) were classified as abnormal if absent, sporadic or exaggerated, and as normal if intermittently or continually present. The motor behaviour was evaluated by repertoire of co-existent other movements (age-adequacy) and concurrent motor repertoire. In addition, videos of 215 infants were analyzed by computer and the variability of the spatial center of motion (C) was calculated. The Peabody Developmental Motor Scales was used to assess motor development at 12 months.

Results: Abnormal FMs, reduced age adequacy, and an abnormal concurrent motor repertoire were significantly associated with lower Gross Motor and Total Motor Quotient (GMQ, TMQ) scores (p < 0.05). The C was higher in children with TMQ scores <90 (-1SD) than in children with higher TMQ scores (p = 0.002).

Conclusion: Normal FMs (assessed by Gestalt perception) and a low variability of the spatial center of motion (assessed by computer-based video analysis) predicted higher Peabody scores in 12-month-old infants born in India with a very low birth weight.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ejpn.2016.07.019DOI Listing
November 2016

Organ preservation for clinical T2N0 distal rectal cancer using neoadjuvant chemoradiotherapy and local excision (ACOSOG Z6041): results of an open-label, single-arm, multi-institutional, phase 2 trial.

Lancet Oncol 2015 Nov 22;16(15):1537-1546. Epub 2015 Oct 22.

Brigham and Women's Hospital, Boston, MA, USA.

Background: Local excision is an organ-preserving treatment alternative to transabdominal resection for patients with stage I rectal cancer. However, local excision alone is associated with a high risk of local recurrence and inferior survival compared with transabdominal rectal resection. We investigated the oncological and functional outcomes of neoadjuvant chemoradiotherapy and local excision for patients with stage T2N0 rectal cancer.

Methods: We did a multi-institutional, single-arm, open-label, non-randomised, phase 2 trial of patients with clinically staged T2N0 distal rectal cancer treated with neoadjuvant chemoradiotherapy at 26 American College of Surgeons Oncology Group institutions. Patients with clinical T2N0 rectal adenocarcinoma staged by endorectal ultrasound or endorectal coil MRI, measuring less than 4 cm in greatest diameter, involving less than 40% of the circumference of the rectum, located within 8 cm of the anal verge, and with an Eastern Cooperative Oncology Group performance status of at least 2 were included in the study. Neoadjuvant chemoradiotherapy consisted of capecitabine (original dose 825 mg/m(2) twice daily on days 1-14 and 22-35), oxaliplatin (50 mg/m(2) on weeks 1, 2, 4, and 5), and radiation (5 days a week at 1·8 Gy per day for 5 weeks to a dose of 45 Gy, followed by a boost of 9 Gy, for a total dose of 54 Gy) followed by local excision. Because of adverse events during chemoradiotherapy, the dose of capecitabine was reduced to 725 mg/m(2) twice-daily, 5 days per week, for 5 weeks, and the boost of radiation was reduced to 5·4 Gy, for a total dose of 50·4 Gy. The primary endpoint was 3-year disease-free survival for all eligible patients (intention-to-treat population) and for patients who completed chemotherapy and radiation, and had ypT0, ypT1, or ypT2 tumours, and negative resection margins (per-protocol group). This study is registered with ClinicalTrials.gov, number NCT00114231.

Findings: Between May 25, 2006, and Oct 22, 2009, 79 eligible patients were recruited to the trial and started neoadjuvant chemoradiotherapy. Two patients had no surgery and one had a total mesorectal excision. Four additional patients completed protocol treatment, but one had a positive margin and three had ypT3 tumours. Thus, the per-protocol population consisted of 72 patients. Median follow-up was 56 months (IQR 46-63) for all patients. The estimated 3-year disease-free survival for the intention-to-treat group was 88·2% (95% CI 81·3-95·8), and for the per-protocol group was 86·9% (79·3-95·3). Of 79 eligible patients, 23 (29%) had grade 3 gastrointestinal adverse events, 12 (15%) had grade 3-4 pain, and 12 (15%) had grade 3-4 haematological adverse events during chemoradiation. Of the 77 patients who had surgery, six (8%) had grade 3 pain, three (4%) had grade 3-4 haemorrhage, and three (4%) had gastrointestinal adverse events.

Interpretation: Although the observed 3-year disease free survival was not as high as anticipated, our data suggest that neoadjuvant chemoradiotherapy followed by local excision might be considered as an organ-preserving alternative in carefully selected patients with clinically staged T2N0 tumours who refuse, or are not candidates for, transabdominal resection.

Funding: National Cancer Institute and Sanofi-Aventis.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/S1470-2045(15)00215-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4984260PMC
November 2015

Effect of adding mFOLFOX6 after neoadjuvant chemoradiation in locally advanced rectal cancer: a multicentre, phase 2 trial.

Lancet Oncol 2015 Aug 14;16(8):957-66. Epub 2015 Jul 14.

Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY, USA.

Background: Patients with locally advanced rectal cancer who achieve a pathological complete response to neoadjuvant chemoradiation have an improved prognosis. The need for surgery in these patients has been questioned, but the proportion of patients achieving a pathological complete response is small. We aimed to assess whether adding cycles of mFOLFOX6 between chemoradiation and surgery increased the proportion of patients achieving a pathological complete response.

Methods: We did a phase 2, non-randomised trial consisting of four sequential study groups of patients with stage II-III locally advanced rectal cancer at 17 institutions in the USA and Canada. All patients received chemoradiation (fluorouracil 225 mg/m(2) per day by continuous infusion throughout radiotherapy, and 45·0 Gy in 25 fractions, 5 days per week for 5 weeks, followed by a minimum boost of 5·4 Gy). Patients in group 1 had total mesorectal excision 6-8 weeks after chemoradiation. Patients in groups 2-4 received two, four, or six cycles of mFOLFOX6, respectively, between chemoradiation and total mesorectal excision. Each cycle of mFOLFOX6 consisted of racemic leucovorin 200 mg/m(2) or 400 mg/m(2), according to the discretion of the treating investigator, oxaliplatin 85 mg/m(2) in a 2-h infusion, bolus fluorouracil 400 mg/m(2) on day 1, and a 46-h infusion of fluorouracil 2400 mg/m(2). The primary endpoint was the proportion of patients who achieved a pathological complete response, analysed by intention to treat. This trial is registered with ClinicalTrials.gov, number NCT00335816.

Findings: Between March 24, 2004, and Nov 16, 2012, 292 patients were registered, 259 of whom (60 in group 1, 67 in group 2, 67 in group 3, and 65 in group 4) met criteria for analysis. 11 (18%, 95% CI 10-30) of 60 patients in group 1, 17 (25%, 16-37) of 67 in group 2, 20 (30%, 19-42) of 67 in group 3, and 25 (38%, 27-51) of 65 in group 4 achieved a pathological complete response (p=0·0036). Study group was independently associated with pathological complete response (group 4 compared with group 1 odds ratio 3·49, 95% CI 1·39-8·75; p=0·011). In group 2, two (3%) of 67 patients had grade 3 adverse events associated with the neoadjuvant administration of mFOLFOX6 and one (1%) had a grade 4 adverse event; in group 3, 12 (18%) of 67 patients had grade 3 adverse events; in group 4, 18 (28%) of 65 patients had grade 3 adverse events and five (8%) had grade 4 adverse events. The most common grade 3 or higher adverse events associated with the neoadjuvant administration of mFOLFOX6 across groups 2-4 were neutropenia (five in group 3 and six in group 4) and lymphopenia (three in group 3 and four in group 4). Across all study groups, 25 grade 3 or worse surgery-related complications occurred (ten in group 1, five in group 2, three in group 3, and seven in group 4); the most common were pelvic abscesses (seven patients) and anastomotic leaks (seven patients).

Interpretation: Delivery of mFOLFOX6 after chemoradiation and before total mesorectal excision has the potential to increase the proportion of patients eligible for less invasive treatment strategies; this strategy is being tested in phase 3 clinical trials.

Funding: National Institutes of Health National Cancer Institute.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/S1470-2045(15)00004-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4670237PMC
August 2015

Cockayne syndrome: characteristic neuroimaging features.

Acta Neurol Belg 2015 Sep 9;115(3):427-8. Epub 2014 Nov 9.

Department of Radiology, Christian Medical College, Vellore, 632004, India,

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s13760-014-0390-zDOI Listing
September 2015

Cri du chat syndrome: a series of five cases.

Indian J Pathol Microbiol 2012 Oct-Dec;55(4):501-5

Cytogenetics Unit, Department of Neurology, Christian Medical College, Vellore, India.

The cri du chat syndrome (CdCS) is a chromosomal deletion syndrome associated with a partial deletion of the short (p) arm of chromosome 5. We describe five children who were diagnosed to have CdCS by conventional cytogenetic analysis. The deletion was at 5p15 in four patients, whereas the fifth had a larger, more proximal deletion at 5p14. Fluorescence in situ hybridization (FISH) analysis confirmed the deletion of the CdCS critical region at 5p15.2. All five children had global developmental delay and dysmorphism with microcephaly. The other clinical features were variable. Since the clinical diagnosis of CdCS may not always be evident because of the phenotypic heterogeneity, cytogenetic analysis is necessary to establish the diagnosis and confirm that the deletion involves the CdCS critical region. This will enable early intervention which plays an important role in improving the outcome.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/0377-4929.107791DOI Listing
November 2013

Growth and development profile of Indian children with Down syndrome.

Indian Pediatr 2012 Aug;49(8):676-7

In this retrospective study, we describe the profile of 88 children with Down syndrome. The average BMI for children showed a progressive increase with age. Compared to the previously published development profile, there was a significant improvement in the language domain.
View Article and Find Full Text PDF

Download full-text PDF

Source
August 2012

An Indian boy with additional features in Pallister-Killian syndrome.

Indian J Pediatr 2012 Sep 20;79(9):1238-40. Epub 2011 Oct 20.

Department of Clinical Genetics, Christian Medical College and Hospital, Vellore, 632004, India.

Pallister-Killian syndrome (PKS; OMIM: # 601803) is a rare sporadic genetic disorder characterized by pigmentary skin changes, distinctive dysmorphology, developmental delay, and mosaicism for tetrasomy of chromosome 12p. The authors report a case of PKS in a 2-y-old boy. He had pigmentary skin changes, characteristic facial features, developmental delay and hearing loss. He had sacral and post-auricular pits in addition, which has not yet been reported. A diagnosis of PKS was suspected on the basis of the patient's clinical features. Skin fibroblast culture was done which showed mosaic tetrasomy of isochromosome 12p consistent with Pallister-Killian syndrome. This case highlights the importance of dysmorphology as a diagnostic tool for recognition and accurate genetic counseling in genetic syndromes.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12098-011-0585-8DOI Listing
September 2012

A phase II trial of neoadjuvant chemoradiation and local excision for T2N0 rectal cancer: preliminary results of the ACOSOG Z6041 trial.

Ann Surg Oncol 2012 Feb 14;19(2):384-91. Epub 2011 Jul 14.

Department of Surgery, City of Hope, Duarte, USA.

Purpose: We designed American College of Surgeons Oncology Group (ACOSOG) Z6041, a prospective, multicenter, single-arm, phase II trial to assess the efficacy and safety of neoadjuvant chemoradiation (CRT) and local excision (LE) for T2N0 rectal cancer. Here, we report tumor response, CRT-related toxicity, and perioperative complications (PCs).

Methods: Clinically staged T2N0 rectal cancer patients were treated with capecitabine and oxaliplatin during radiation followed by LE. Because of toxicity, capecitabine and radiation doses were reduced. LE was performed 6 weeks after CRT. Patients were evaluated for clinical and pathologic response. CRT-related complications and PCs were recorded.

Results: Ninety patients were accrued; 6 received nonprotocol treatment. The remaining 84 were 65% male; median age 63 years; 83% Eastern Cooperative Oncology Group performance score 0; 92% white; mean tumor size 2.9 cm; and average distance from anal verge 5.1 cm. Five patients were considered ineligible. Therapy was completed per protocol in 79 patients, but two patients did not undergo LE. Among 77 eligible patients who underwent LE, 34 patients achieved a pathologic complete response (44%) and 49 (64%) tumors were downstaged (ypT0-1), but 4 patients (5%) had ypT3 tumors. Five LE specimens contained lymph nodes; one T3 tumor had a positive node. All but one patient had negative margins. Thirty-three (39%) of 84 patients developed CRT-related grade ≥3 complications. Rectal pain was the most common PC.

Conclusions: CRT before LE for T2N0 tumors results in a high pathologic complete response rate and negative resection margins. However, complications during CRT and after LE are high. The true efficacy of this approach will ultimately be assessed by the long-term oncologic outcomes.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1245/s10434-011-1933-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5720824PMC
February 2012

Laparoscopic proctectomy after neoadjuvant therapy: safety and long-term follow-up.

Surg Endosc 2011 Jun 24;25(6):1902-6. Epub 2010 Dec 24.

Department of Surgery, Oregon Health & Science University, Portland, Oregon, USA.

Purpose: The oncologic value of laparoscopic proctectomy for rectal adenocarcinoma is uncertain. Long-term data, particularly in tumors at higher risk of recurrence, is lacking. This study evaluated short- and long-term outcomes in patients who underwent laparoscopic proctectomy for locally advanced cancer (transmural and/or node positive) after neoadjuvant chemoradiotherapy (CRT).

Methods: This is a retrospective cohort study of 50 consecutive patients with transmural and/or node-positive rectal cancer, from a single surgeon's practice, from 2001 to 2009. All patients were treated with neoadjuvant CRT. All cases were started laparoscopic or hand-assist.

Results: Of 50 patients, 58% were men, mean age was 60.9 years, and mean body mass index (BMI) was 26.3. The average distance of the tumor from the anal verge was 5.7 cm. All patients completed CRT, and the subsequent mean time to operation was 7.8 weeks. The conversion to open rate was 26%. Thirty-day mortality was 2%. Twenty-two percent had a complete response to CRT. Two patients had positive margins: one developed distant recurrence only, and the other died 2 years later without evidence of local recurrence. The average distal margin was 3.26 cm. The average lymph nodes resected was 11.9. Seven patients had an ileus that delayed discharge and one had a pelvic abscess. Median length of stay was 6 days. Three patients were readmitted within 30 days; all for dehydration. Mean follow-up was 2.72 years. According to Kaplan-Meier analysis, the 5-year local recurrence rate was 9.6%, and the distant recurrence rate was 31%. Five-year disease-specific survival was 80% and overall survival was 68%.

Conclusions: Patients with locally advanced rectal cancer treated with neoadjuvant therapy can safely undergo laparoscopic proctectomy with a low rate of complications. Oncologic outcomes, including 5-year disease-free survival and local recurrence rates, are comparable to published reports of open proctectomy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00464-010-1484-1DOI Listing
June 2011

Development and dysmorphism in Joubert syndrome--short case series from India.

J Trop Pediatr 2010 Jun 15;56(3):209-12. Epub 2009 Sep 15.

Five children with Joubert syndrome (JS), who fulfilled the criteria and had molar tooth sign (MTS) on magnetic resonance imaging were included in the study. Prominent forehead, open mouth and low set ears were consistent facial features. Severe developmental delay was seen in three children (66%). A differential developmental delay was noticed in all children and was independent of the radiological features. The children who had complications in the neonatal period were found to have more developmental delay on follow-up. The optimal control of sleep disturbances and hyperkinesis in one child resulted in a better cognitive performance. A regular neuro-developmental follow-up and interventions can optimize the potential of children with JS. In addition to the regular screening for retinal, renal and hepatic functions in JS, there is a need to monitor cognitive functions, sleep and behavior.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/tropej/fmp084DOI Listing
June 2010

Amylase-resistant starch as adjunct to oral rehydration therapy in children with diarrhea.

J Pediatr Gastroenterol Nutr 2006 Apr;42(4):362-8

Department of Child Health, Christian Medical College, Vellore, India.

Background: Oral rehydration solution (ORS) for treatment of diarrhea relies on enhancement of small intestinal sodium and fluid absorption to correct dehydration. Amylase-resistant starch added to ORS significantly reduced the duration and severity of diarrhea in adults with cholera, presumably by generation of short-chain fatty acids in the colon and enhancement of colonic sodium and fluid absorption. The present study was initiated to determine whether addition of amylase-resistant starch to standard World Health Organization glucose-ORS (G-ORS) would reduce the duration of diarrhea and fecal fluid losses in children with acute diarrhea.

Methods: One hundred eighty-three children (6 months to 3 years) with acute watery diarrhea were randomized to receive either standard treatment with G-ORS or G-ORS with additional amylase-resistant starch, HAMS (HAMS-ORS, 50g/L). Stool weight and consistency were monitored serially until development of formed stool or development of treatment failure defined as either the need for unscheduled intravenous fluid therapy or diarrhea longer than 72 hours.

Results: Five of the subjects were lost to follow up. In 178 remaining children (87 HAMS-ORS and 91 G-ORS) with evaluable data, time from enrolment to last unformed stool was significantly less in children receiving HAMS-ORS (median, 6.75 hours; 95% confidence interval, 4.27-9.22) than in children treated with G-ORS (12.80 hours, 8.69-16.91) (P = 0.0292). Time to first formed stool was also significantly shorter in children receiving HAMS-ORS (median, 18.25 hours; 95% confidence interval, 13.09-23.41) compared with children receiving G-ORS (median, 21.50 hours; 95% confidence interval, 17.26-25.74) (P = 0.0440). The total amount of ORS consumed was similar in both groups. There was a trend toward lower mean stool weight in first 24 hours (P = 0.0752) as well as total diarrheal stool weight (P = 0.0926) in patients in the HAMS group compared with the G-ORS group.

Conclusion: In children with acute diarrhea, the addition of amylase-resistant starch to glucose ORS significantly shortened duration of diarrhea compared with standard treatment.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/01.mpg.0000214163.83316.41DOI Listing
April 2006