Chronic idiopathic myelofibrosis (CIM) with myeloid metaplasia is a myeloproliferative disorder characterized by leukoerythroblastosis, tear drop erythrocytes, extra-medullary hematopoesis (EMH), and varying degree of myelofibrosis. CIM, presenting as refractory ascites secondary to peritoneal hematopoesis, is extremely rare with only six adult cases reported in literature. This is a report of a child with CIM presenting as refractory ascites as a consequence of EMH in the peritoneum.
View Article and Find Full Text PDFWe present a case of presumed amniotic fluid embolism in a 33-year-old parturient at 30 weeks of gestation, which occurred just before she was due to receive spinal anaesthesia for urgent caesarean section. While sitting, the woman suddenly lost consciousness, started having convulsions and finally suffered cardiorespiratory collapse. She was resuscitated and a live baby was delivered by emergency caesarean section.
View Article and Find Full Text PDFBackground: The diagnosis of retroperitoneal lesions is one of the most difficult areas in surgical pathology. The retroperitoneal space allows both primary and metastatic tumors to grow silently before the appearance of clinical signs and symptoms. Fine needle aspiration cytology has shown promising role in establishing the diagnosis in this region.
View Article and Find Full Text PDFJ Pediatr Endocrinol Metab
March 2009
Background: Vitamin D nutrition has a profound effect on the development of an infant. Vitamin D status of mothers and their infants are closely correlated. While hypovitaminosis D has emerged as a significant public health problem across all age groups, there is limited information of this condition in lactating mothers and their breast fed infants.
View Article and Find Full Text PDFJ Pediatr Hematol Oncol
March 2009
Rosai-Dorfman disease (RDD), originally described as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. Formerly thought to be a disease process limited to lymph nodes, RDD has now been reported in many organ systems like bone, skin and soft tissue, central nervous system, eye and orbit, and upper respiratory tract. Here we report a case of RDD with hepatic involvement, which is even more rare.
View Article and Find Full Text PDFThe neutrophil/lymphocyte ratio (NLR) has recently been described as a predictor of mortality in patients who undergo percutaneous coronary intervention. The aim of this study was to investigate the utility of admission NLRs in predicting outcomes in patients with acute coronary syndromes (ACS). A total of 2,833 patients admitted to the University of Michigan Health System with diagnoses of ACS from December 1998 to October 2004 were followed.
View Article and Find Full Text PDFObjective: To study the etiological profile of patients with acute febrile encephalopathy syndrome focusing chiefly on the viral etiology, and to correlate clinical and radiological features of patients with viral encephalitis.
Methods: A prospective hospital based study conducted on the consecutive patients admitted in a pediatric unit during the period of 1(st) February 2004 to 31st January 2005 based on the following inclusion criteria: (1) Age more than 1 month and less than 18 years and (2) A diagnoses of acute febrile encephalopathy, based on the following criteria: (i) fever (ii) acute depression of consciousness or mental deterioration for more than 12 hours with or without motor or sensory deficit and (iii) Total duration of illness at the time of admission 1 week or less.
Results: The final study group comprised of 151 patients with mean age of 3.
Objective: To study the etiological profile of childhood stroke and its relation with prothrombotic states.
Methods: Children with acute stroke with no evidence of CNS infection or head injury were studied. Stroke was confirmed by CT scan and further evaluated by MRI.
J Pediatr Endocrinol Metab
December 2006
Objective: To assess the iodine nutrition status of exclusively breast-fed infants and their mothers.
Hypothesis: In the presence of environmental and maternal iodine deficiency, an exclusively breast-fed baby is likely to receive a suboptimal iodine supply during the period of rapid brain growth.
Methods: Spot urinary iodine (UI) and serum TSH levels were measured in 175 healthy, exclusively breast-fed infants and their mothers.
An 11-month female with a poor socio-economic status presented to a tertiary care paediatric hospital with complaints of fever of 4-5 days and diarrhoea of 20 days duration. The patient didn't respond to the prescribed antimicrobials namely--Norfloxacin and metronidazole. On admission she was diagnosed as persistent diarrhea with PEM grade III with sepsis.
View Article and Find Full Text PDFBerardinelli-Seip congenital lipodystrophy (BSCL) is a very rare genetic disorder characterized by lipoatrophy, hypertriglyceridemia, hepatomegaly and acromegaloid features. On the basis of mutational and haplotype analysis, BSCL families have been classified into three types BSCL 1, BSCL2 and BSCLX. We report Berardinelli-Seip congenital lipodystrophy (BSCL2 type) in three subjects from two unrelated Indian families (family1 and family2).
View Article and Find Full Text PDFThe inter-relationship between sleep and epilepsy is well recognized. Sleep is known to activate inter-ictal epileptiform discharges. A special timing of seizure in relation to sleep wake cycle is certainly a childhood epileptic syndrome.
View Article and Find Full Text PDFAcute disseminated encephalomyelitis(ADEM) is an uncommon inflammatory demyelinating disease involving central nervous system white matter. A series of seven cases seen over a period of one year have been described. The patients presented with acute onset of focal neurological deficit (3 patients), alteration of sensorium(3) and ataxia(1).
View Article and Find Full Text PDFObjective: To study incidence of spina bifida occulta in nocturnal enuresis cases and to compare outcome of enuresis with spina bifida occulta and enuresis without spina bifida occulta.
Methods: Patients with enuresis divided into two groups based on X-ray lumbosacral spine. Outcome of these patients were compared on behavioral therapy.
This double-blind, randomized, placebo-controlled study in epileptic children, aged 3 to 12 years, evaluated the effect of add-on melatonin on the sleep behavior of these children on sodium valproate monotherapy using a parental questionnaire. Of the 31 patients, 16 randomly received add-on melatonin, whereas 15 received add-on placebo. The questionnaire showed good internal consistency in our patient population (Cronbach's alpha = .
View Article and Find Full Text PDFPurpose: Melatonin has been shown to exhibit antioxidant, antiexcitotoxic, and free radical-scavenging properties in various animal models. The study was designed to assess its effects on the blood levels of antioxidant enzymes in children with epilepsy receiving carbamazepine (CBZ).
Methods: In a double-blind, randomized, parallel-group, placebo-controlled trial, we assessed the effect of add-on melatonin (6-9 mg/day for 14 days) on the antioxidant enzymes glutathione peroxidase (GPx) and glutathione reductase (GRd) in 31 children with epilepsy receiving CBZ monotherapy, who were seizure free at least for the last 6 months.
Onset of diabetes in the first year of life is uncommon, most cases being those of transient diabetes of the newborn. We report the case of a child who presented at 10 weeks of age with diabetic keto-acidosis, and continues to be insulin dependent at 18 months.
View Article and Find Full Text PDFAims: To compare the effect of add-on melatonin with placebo on the antioxidant enzymes (glutathione peroxidase and glutathione reductase) in epileptic children on valproate monotherapy.
Methods: In a double-blind, randomized, placebo controlled trial, the effect of add-on melatonin administration on the antioxidant enzymes in epileptic children on valproate (VPA) monotherapy was assessed. A total of 31 patients met the entry criteria.
Evaluation of a child with cerebral palsy (CP) requires a multidisciplinary approach with a team of professionals comprising of a pediatrician or pediatric neurologist, occupational therapist, a physiotherapist, child psychologist, and a social worker. The assessment is necessary to confirm the diagnosis, determine the cause, assess the motor function and associated problems. The diagnosis of CP is clinical but selected investigations may be required for ascertaining the cause.
View Article and Find Full Text PDFThis randomized, double-blind, placebo-controlled study in epileptic children aged 3-12 years evaluated the effects of add-on melatonin administration on the quality of life of these children on sodium valproate (VPA) monotherapy using a parental questionnaire. Quality of Life in Childhood Epilepsy is a questionnaire designed to assess a variety of age-relevant domains such as physical function, emotional well-being, cognitive function, social function, behavior, and general health. Of the 31 patients, 16 randomly received add-on melatonin (MEL), whereas 15 received add-on placebo (P).
View Article and Find Full Text PDFProtozoal infections of the gastrointestinal tract occur worldwide and have substantial morbidity and mortality. Prevalence is higher in the economically deprived regions of the world, especially the developing countries. Infections like amoebiasis and giardiasis have a worldwide distribution, being endemic in India.
View Article and Find Full Text PDFObjective: To derive norms for the size of uterus, uterine shape (fundal-cervical ratio) and ovarian volume in girls in various Tanners stages of puberty.
Methods: Pelvic ultrasound was performed in ninety-two healthy girls in the age group of 8-15 years. These included twenty girls each in Tanner stages 1-4 and twelve in stage 5.