Effect of hypertonic saline in the management of elevated intracranial pressure in children with cerebral edema: A systematic review and meta-analysis.

OBJECTIVE: To determine the hypertonic saline efficacy in children with cerebral edema and raised intracranial pressure. METHOD: Studies assessing the efficacy and safety of hypertonic saline in children with cerebral edema and elevated intracranial pressure were identified using Medline, Web of Science, Scopus, and Google Scholar databases. Two reviewers independently assessed papers for inclusion. The primary outcome was a reduction of elevated intracranial pressure by the administration of hypertonic saline. RESULTS: We initially evaluated 1595 potentially relevant articles, and only 7 studies met the eligibility criteria for the final analysis. Out of the seven studies, three of them were randomized controlled trials. Three of the studies found that hypertonic saline significantly reduced elevated intracranial pressure compared to control. One study reported a resolution of the comatose state as a measure of reduced intracranial pressure. It also found a significantly higher resolution of coma in the hypertonic saline group rather than the control. Three studies reported that the reduction of intracranial pressure was comparable between the groups. The random-effects model using pooled estimates from four studies showed no difference in hypertonic saline and conventional therapy mortality outcomes. Hypertonic saline was administered as bolus-only therapy at a rate of 1-10 mL/kg/dose over 5 min to 2 h and or bolus followed by infusion therapy (0.5-2 mL/kg/h). One study reported a twofold faster resolution of high intracranial pressure following hypertonic saline administration compared to controls. The re-dosing schedule varied greatly in all included studies. However, three studies reported adverse events but not methodically, and there were no reports on neurological sequelae. CONCLUSION: Hypertonic saline appears to reduce intracranial pressure in children with cerebral edema. However, we cannot draw a firm conclusion regarding the safest dose regimens of hypertonic saline, including the safe and effective therapeutic hypernatremia threshold in the management of raised intracranial pressure with cerebral edema. Future clinical trials should focus on the appropriate concentration, dose, duration, mode of administration, and adverse effects of hypertonic saline to standardize the treatment.

Intraspinal Intramedullary Primitive Neuroectodermal Tumor in a Young Girl: A Case Report from BSMMU, Bangladesh.

Primitive neuroectodermal tumors are malignant tumor of pluripotent cells of neural crest. It has diverse clinical presentation and aggressive clinical behaviour. Clinical features may provide some clue but imaging studies such as MRI of brain; tissue histopathology, immune histochemistry and cytogenetic are essential to confirm the diagnosis. Here we describe a girl of 4½ years age admitted with the complaints of, low back pain for 2 months, gradually worsening weakness in both lower limbs for the same duration along with urinary incontinence and constipation for 1 month. On examination she was afebrile, mildly pale, no lymphadenopathy, having normal vital signs and anthropometrically well thriving. Back and spine examination showed angulation of spine involving T11 - L4, without paravertebral swelling. Neurological examination of lower limbs revealed decreased muscle tone, diminished muscle power (3/5), both knee and ankle jerks were also diminished and bilaterally equivocal planter response, sensory function of all modalities were impaired. MRI findings were suggestive of ependymoma. Consultation was done with department of neurosurgery and they did the relevant surgery. Tissue histopoathology and immune histochemistry confirmed the diagnosis of primitive neuroectodermal tumor. Finally the child was referred to Paediatric Haemato-oncolgy department for further management.

Poncet's disease (tubercular rheumatism) - a case report.

Poncet's disease is a rare condition in childhood. It occurs due to immunological reaction to tubercular protein resulting in reactive arthritis and manifest with polyarthritis associated with features of active tuberculosis. We are reporting a case of Poncet's disease that was initially treated as a case of Juvenile Idiopathic Arthritis (JIA) without any improvement. The diagnosis was made clinically from history and physical findings with supportive radiological findings and confirmed by granulomatous changes on FNAC. Our patient improved dramatically after treatment with anti-tubercular drugs. Though very rare, Poncet's disease should be strongly considered in the differential diagnosis of fever and polyarthritis of obscure cause, especially in tubercular endemic countries like ours.

The relationship of placental weight with birth weight.

To assess the relationship between placental weight and birth weight, two hundred forty six pregnant mothers, who were otherwise healthy, were prospectively followed in a city hospital during antenatal period until delivery and immediate post-partum period. Height of mothers was measured initially and weight measured at each visit during the antenatal check-up. Placental weight and birth weight of babies were measured by one of the authors immediately after delivery by a weighing scale. Eighty one percent of the mothers were between the age group of 20-29 years. The BMI of 92% mothers was 18.5 and above. Most of the mothers came both with primigravida (42%) or second gravida (33%) and in 25% cases 3rd or onwards. In 49% cases the placental weight was between 401-500 gm, in 30% cases >500 gm and in 21% cases 400 gm or less. There was delivery of appropriate-birth-weight babies in 85% cases and low-birth-weight babies in 15% cases. It was observed that a very strong correlation existed between placental weight and birth weight (r = 0.391, p<0.001). Even this correlation was stronger in small for gestational age babies. However, there was no correlation between placental weight and APGAR score at one minute. It is concluded that increment of birth weight occurs with increase of placental weight. If placental weight can be measured by ultrasonography in second or early third trimester of pregnancy birth weight is possible to be assessed and appropriate measure can be taken to increase the birth weight.

Chronic Caffey's disease: an uncommon entity in children.

Chronic Caffey's disease in an uncommon condition in children is characterized by an acute inflammatory reaction in the periosteum along with systemic disturbances. A 30 months old boy was reported in the pediatric unit of BSMMU, Dhaka about two and half years back with the complaints of multiple painful soft tissue swelling in different parts of the body since birth and delay in growth and development. The child was found well and alert, moderately pale, febrile with hard, tender swelling of mandible on both sides. There were multiple swellings over the right arm, forearm, both thighs and bowing of the lower limbes. Investigations revealed normal serum calcium and phosphate level with mild elevation of alkaline phosphatase. Radiological findings showed periosteal new bone formation in mandible and long bones. There was diaphyseal expansion of the long bones with expansion of the ribs anteriorly. He was diagnosed as a case of chronic caffey's disease on the basis of history, clinical examination and investigation.

Studies on congenital abnormalities and related risk factors.

Congenital malformations were studied prospectively covering 11680 consecutive deliveries. The overall incidence of malformations was 2.3%. Musculoskeletal system was the most commonly involved system. The incidence of malformation was higher in still borns, premature, low birth weight babies and those with positive heredo-familial history. It was also higher in babies, born to mothers who were more than 35 years of age and gravida four and above. Consanguinity of marriage, drugs and hormone ingestion during pregnancy, antenatal complication like hydramnios, pre eclamtic toxemia, gestational diabetes was associated with high incidence of congenital malformations.

Rat hepatic stellate cell expression of alpha2-macroglobulin is a feature of cellular activation: implications for matrix remodelling in hepatic fibrosis.

1. Hepatic stellate cells are key mediators of hepatic fibrosis. We have studied hepatic stellate cell expression of the collagenase and general protease inhibitor alpha2-macroglobulin after activation in tissue culture and in response to certain cytokines. 2. Hepatic stellate cells isolated by Pronase-collagenase digestion were activated by culture on uncoated plastic. By Northern analysis hepatic stellate cells undergoing activation (5 days) expressed alpha2-macroglobulin mRNA and alpha2-macroglobulin could be immunolocalized to hepatic stellate cells from 5 to 15 days of culture. 3. By ELISA of cell culture supernatants hepatic stellate cell secretion of alpha2-macroglobulin was found to increase from 2. 78+/-1.13 ng x ml-1 x microgram-1 DNA per 24 h at 5 days of culture (n=8) to 13.55+/-4.64 ng x ml-1 x microgram-1 DNA per 24 h at 15 days of culture (n=7). Stimulation of hepatic stellate cells with interleukin-6 at 5 days caused a significant increase in alpha2-macroglobulin expression as did exposure to Kupffer-cell conditioned medium. However, exposure of hepatic stellate cells to interleukin-1, transforming growth factor-beta1 and tumour necrosis factor-alpha had no significant effect. 4. During profibrotic liver injury plasma alpha2-macroglobulin levels were found to increase to between 850% and 250% of the control value (100%) after bile duct ligation (72 h to 13 days respectively), and to 1166% and 1106% of the control value during progressive CCl4-induced fibrosis (24 h to 4 weeks respectively). 5. These data suggest that hepatic stellate cells are a potential source of the potent protease inhibitor alpha2-macroglobulin, expression of which may inhibit matrix remodelling during progressive fibrosis.

Kupffer cell-derived 95-kd type IV collagenase/gelatinase B: characterization and expression in cultured cells.

Release of 92-kd type IV collagenase/gelatinase, also known as gelatinase B, by inflammatory and tumor cells is increasingly recognized and is believed to facilitate cellular migration across basement membranes. It has been implicated in the pathogenesis of many diseases, but little is known of its cellular origin(s) and function in liver. In this study we have demonstrated synthesis and release of gelatinase B by human and rat Kupffer cells in primary culture. Northern analysis of RNA extracted from Kupffer cells stimulated with phorbol ester demonstrated a 2.8 kb transcript for gelatinase B. Immunoblotting and zymography of serum-free Kupffer cell-conditioned media demonstrated extracellular release of immunoreactive enzyme and gelatinase activity, Mr 92,000 (95,000 from rat cells). The organomercurial 4-aminophenyl mercuric acetate (APMA) activated the enzyme in vitro, indicating secretion primarily as a proenzyme. Stimulation of Kupffer cells by phorbol ester markedly induced gelatinase B release, which was inhibited by cycloheximide. In contrast, cycloheximide had no effect on constitutive secretion in culture, suggesting that there is some intracellular storage. Kupffer cell-derived gelatinase B was also partially purified and characterized. After separation by gelatin sepharose and gel filtration chromatogrpahy, gelatin-degrading activities of 95, 88, 75, and 65 kd were detected, the three lower-molecular-weight species probably representing activated forms. Enzyme activity was inhibited by ethyl-enediaminetetra-acetic acid (EDTA), but not by serine- and thiol-protease inhibitors, and was restored by zinc. Activity was also inhibited by tissue inhibitor of metalloproteinase-1 (TIMP-1) and alpha-2 macroglobulin. The partially purified enzyme rapidly degraded denatured collagens (gelatin) as well as native types III, IV, and V collagens, but had no activity against casein, types I and VI collagens.

Prostacyclin concentrations in haemolytic uraemic syndrome after acute shigellosis in children.

The role of prostacyclin in the pathogenesis of haemolytic uraemic syndrome was evaluated in 11 children with acute shigellosis. Plasma concentrations of 6-keto prostaglandin, F1 alpha, a stable metabolite of prostacyclin, were measured by radioimmunoassay during acute illness, early convalescence, and after clinical recovery. Its concentration was low during acute illness in each patient, returning to normal concentrations or above at the time of the last sample. These results suggest that plasma prostacyclin may be involved in the development of the syndrome.