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Biomedical subjects

D K Bosman

Publications and source records attributed to D K Bosman.

At least 19 recordsLinked to original sources

[Pyomyositis of the M. quadriceps femoris in an 8-month-old boy following pneumococcal meningitis].

An 8-month-old boy developed pyomyositis of the M. quadriceps femoris following meningitis caused by Streptococcus pneumoniae. The child had no underlying immune defect. Conservative treatment with antibiotics led to complete clinical and radiological resolution of the infection. In Europe, pyomyositis is rarely seen in children. Pyomyositis should be considered in the differential diagnosis in children with fever and complaints of joint pain or myalgia. Ultrasound and MRI are helpful techniques for establishing the diagnosis and for differentiating pyomyositis from other pathological conditions. Treatment consists of adequate antibiotics and in some cases drainage.

Diagnosis, Differential↗

[Diagnostic methods for Helicobacter pylori infection in children].

A Helicobacter pylori infection is usually acquired during early childhood. Poor socioeconomic circumstances form an important risk factor for this. An untreated infection may lead to peptic ulceration and, particularly in adults, to gastric cancer and mucosa associated lymphoid tissue (MALT) lymphoma. The gold standard for the diagnosis of H. pylori infection is gastroscopy with histology and culture of biopsy specimens. Non-invasive tests are serology, 13C-urea breath test and stool antigen test. The sensitivity and specificity of serology tests are low in children, but for both the 13C-urea breath test and the stool antigen test the sensitivity and specificity are high. A 'test and treat' approach is advised with due consideration for possible symptoms and the risk for peptic ulcers and gastric cancer at a more advanced age. The treatment results must be evaluated. If necessary, young children can be treated at a later age.

Antigens, Bacterial↗

[Dientamoeba fragilis: possibly an important cause of persistent abdominal pain in children].

OBJECTIVE: To determine the effect of an antiprotozoic treatment on children with persistent abdominal pain and infection with Dientamoeba fragilis. DESIGN: Retrospective. METHOD: A total of 43 children with D. fragilis infection and persistent gastrointestinal complaints were included in the study. Of these 27 were treated with clioquinol and 16 with a nitroimidazole drug: metronidazole or tinidazole. The parasitological and clinical effects of the treatment were assessed. RESULTS: In 33 of the 43 (77%) children, no parasites were detected during follow-up with a triple faeces test: 22/27 following treatment with clioquinol and 11/16 following treatment with a nitroimidazole drug. In 27 of the 33 (82%) children with a negative follow-up result, gastrointestinal complaints were considerably less or had completely disappeared. In 2 of the 10 (20%) children in which D. fragilis had not disappeared in the follow-up period, the complaints were less or had disappeared. CONCLUSION: Effective treatment of D. fragilis infection in children with longstanding gastrointestinal complaints often resulted in a reduction or disappearance of the complaints.

Abdominal Pain↗

Hepatoblastoma in a girl with biliary atresia: coincidence or co-incidence.

Hepatoblastoma (HB) and biliary atresia (BA) are both rare conditions that occurred in the patient described. This is the second such case in the literature. An explanation for this apparent coincidence could possibly be found in the existance of pluripotent liver stem cells. In humans, small epithelial cells (SEC) behave as pluripotent stem cells. These are cells that can develop as biliary epithelial cells or as liver parenchymal cells, and are thought to be the precursor cell of HB. They are present in a much higher concentration in the livers of patients with BA, which may make them prone to develop HB.

Biliary Atresia↗

Sphenoethmoidal encephalocele: a case report.

OBJECTIVE: This article documents the characteristics and treatment of an infant patient with a sphenoethmoidal encephalocele. An extracranial, transpalatal approach was used to eliminate the encephalocele.

Cleft Lip↗

[Microvillus inclusion disease, a rare cause of severe congenital diarrhea].

To date, microvillus inclusion disease (MID) has been diagnosed in six Dutch patients. It is a rare autosomal recessive hereditary intestinal disorder mostly presenting with malabsorption and severe secretory diarrhoea from birth. The diagnosis is confirmed by electron microscopy of intestinal mucosal biopsies, which show characteristic intracytoplasmic vesicles containing clearly recognisable microvilli and irregularly distributed microvilli in the brush border. The two clinical forms of the disease that have been recognised internationally, a 'congenital' and a 'late-onset' form of MID, have also been observed in the Dutch patients. At the last follow-up five patients had died, the sixth was 17 years old and alive. The pathogenesis and genetics of MID are, as yet, unknown. Eventually, all patients die from complications of the disease, notably from the total parenteral nutrition. The only chance of survival is intestinal or combined liver-intestinal transplantation.

Adolescent↗

Choledochal cysts: age of presentation, symptoms, and late complications related to Todani's classification.

PURPOSE: The aim of this study was to compare presentation, complications, diagnosis, and treatment of choledochal cysts in pediatric and adult patients. METHODS: Forty-two patients were analyzed after subdivision into 3 groups: group A, less than 2 years (n = 10); group B, 2 to 16 years (n = 11); group C, greater than 16 years (n = 21). RESULTS: The cysts were classified as extrahepatic (n = 33), intrahepatic (n = 5), and combined (n = 4). Seventy-six percent of patients presented with abdominal pain, (20 of 21 group C), and 57% with jaundice, (10 of 10 group A). Cholangiocarcinoma occurred in 6 patients, 4 of whom had previously undergone internal drainage procedures. Excision of the extrahepatic cyst was performed in 27 of 37 patients. Five patients, of whom, 4 had cholangiocarcinoma, were beyond curative treatment at the time of diagnosis. Six patients had died at the closure of this study, 5 of them had carcinoma. CONCLUSIONS: Presenting symptoms are age dependent with jaundice prevailing in children and abdominal pain in adults. In view of the high risk of cholangiocarcinoma, early resection and not internal drainage is the appropriate treatment of extrahepatic cysts. Patients who had undergone internal drainage in the past still should undergo resection of the cyst.

Abdominal Pain↗

Helicobacter pylori Stool Antigen test: a reliable non-invasive test for the diagnosis of Helicobacter pylori infection in children.

OBJECTIVE: To evaluate the Helicobacter pylori Stool Antigen (HpSA) test for the diagnosis of H. pylori infection in children. DESIGN AND SETTING: Prospective cohort study in an academic medical centre. PATIENTS AND METHODS: A total of 106 consecutive children who underwent gastroscopy were included. Biopsy specimens were sampled from the gastric antrum and corpus for the assessment of H. pylori infection by culture and histology. A patient was defined to be H. pylori positive if the results of culture and/or histology proved to be H. pylori positive; a patient was defined to be negative if both test results were negative. All children provided a stool sample within 2 days of gastroscopy. H. pylori antigens in faeces were assessed by an enzyme immunoassay (Premier HpSA, Meridian Diagnostics, Inc., Cincinnati, OH, USA). RESULTS: The mean age of included patients was 8.5 years (range 1-18.5). Thirty patients were H. pylori positive and 76 patients were H. pylori negative. Using the recommended cut-off values of 0.140 optical density (OD) and 0.159 OD, sensitivity and specificity of 100% and 92% were found. The positive and negative predicting values were 83% (30/36) and 100% (70/70), respectively. CONCLUSION: The HpSA test is an accurate test for the diagnosis of H. pylori infection in children, and might therefore be a good alternative for diagnostic tests such as the 13C-urea breath test (UBT).

Antigens, Bacterial↗

[Fabry's disease; towards a treatment].

Fabry's disease, deficiency of the enzyme alpha-galactosidase A, is an X-linked lysosomal storage disorder. Clinical symptoms are caused by continuous deposition of specific glycolipids in endothelial cells, neural cells, skin and cornea. These depositions give rise to skin (angiokeratoma) and eye abnormalities (cornea verticillata), acroparaesthesias and anhidrosis and later in life cause renal insufficiency and cardiovascular complications. Hemizygous males suffer from Fabry's disease, whereas female carriers (heterozygotes) are usually asymptomatic. Recently, an atypical presentation of Fabry's disease was described in males who only presented with cardiac involvement. Therefore, the actual number of Fabry patients in the Netherlands could be higher than the predicted 300. Diagnosis in males can be established by measuring alpha-galactosidase enzyme activity in plasma, leukocytes or fibroblasts. Apart from kidney transplantation only symptomatic therapy is available today. Enzyme supplementation therapy (as shown in Gaucher's disease) and substrate deprivation are possible ways of treatment in the future.

Cardiomyopathy, Hypertrophic↗

[Percutaneous endoscopic gastrostomy in children with psychomotor retardation; less complaints and not as stressful].

OBJECTIVE: To evaluate the effects of percutaneous endoscopic gastrostomy (PEG) in children with psychomotor retardation. DESIGN: Prospective. METHOD: Data on symptoms, pros and cons and complications were collected by means of questionnaires from the parents of children with psychomotor retardation and severe nutritional problems in whom a PEG tube had been introduced between August 1995 and March 1998 in the department of Children's Gastroenterology and Nutrition of the Emma Children's Hospital/Academic Medical Centre in Amsterdam, the Netherlands, comparing the situations before the introduction and 6 and 18 months afterward. RESULTS: The patient group consisted of 17 boys and 23 girls with a mean age of 6 years and 3 months (range 8 months-10 years and 1 month). The frequency of vomiting and of airway infections decreased and the general nutritional condition improved. Restlessness and pain occurred less often in over half the children. The disadvantages most often reported were the logistics concerning the feeding (n = 11) and the reduced contact with the child (n = 10). Thirteen children displayed mild side effects such as skin irritation and proud flesh. Technical problems consisted of leakage (n = 11) and obstruction of the tube (n = 2). In one child, introduction of the tube was followed by a major complication. CONCLUSION: The PEG tube in this patient group reduced the frequency of complaints about nutrition and constituted a patient-friendly alternative to the nasal tube.

Child↗

[Immunology in medical practice. XXII. T-cells and strategies for tolerance induction].

Up to the present, aspecific immunosuppressive medication is still required to prevent rejection of transplanted allogeneic tissues and to treat autoimmune disorders. These drugs are associated with numerous adverse effects. Therefore, alternative forms of immunomodulation are being extensively studied. T-cells play a central role in regulating the immune system. Expanding insight into the cellular mechanisms involved in immune recognition and activation led to the development of new tolerance-inducing strategies. Potential sites for tolerance induction are situated centrally in the thymus as well as in the peripheral T-cell population. Explicitly mentioned tolerance strategies are (a) allogeneic bone marrow transplantation and intrathymic tolerance induction (central tolerance induction), (b) peripheral T-cell clonal deletion, induction of anergy and immune deviation (all three are examples of peripheral tolerance induction).

Allergy and Immunology↗

Neocortical dialysate monoamines of rats after acute, subacute, and chronic liver shunt.

Intracerebral microdialysis was applied to monitor the neocortical extracellular levels of the aromatic amino acids phenylalanine, tyrosine, and tryptophan, the neurotransmitters dopamine (DA), noradrenaline (NA), and serotonin (5-HT), and the metabolites 3,4-dihydroxyphenylacetic acid (DOPAC) and 5-hydroxyindole-3-acetic acid (5-HIAA) in rats with various forms of experimental hepatic encephalopathy (HE). The extracellular aromatic amino acid levels were clearly increased in acute, subacute, and chronic HE. No changes compared with controls in the neocortical DA release could be detected in the three experimental HE rat models investigated. The NA release showed a significant increase only in the subacute HE group. These data suggest that HE may not be associated with any major reduction of neocortical DA or NA release as previously suggested. In acute and subacute HE, decreased extracellular DOPAC but elevated 5-HIAA concentrations were seen. In chronic HE, elevations of both DOPAC and 5-HIAA were observed. Neocortical 5-HT release did not change in subacute and chronic HE, whereas it decreased in acute HE compared with control values. Significant increase in extracellular concentrations of 5-HIAA and of the 5-HIAA/5-HT ratio in the present study are in agreement with previously reported increases in 5-HT turnover in experimental HE. However, a substantially increased 5-HT turnover in experimental HE does not appear to be related to an increase in neuronal neocortical 5-HT release.

3,4-Dihydroxyphenylacetic Acid↗

The effect of extrahepatic cholestasis on liver regeneration after partial hepatectomy in the rat.

Liver regeneration after partial hepatectomy was studied in four groups of rats: control rats (n = 12), rats with 1 week of common bile duct obstruction (n = 11), rats with restoration of bile flow after 1 week of obstruction (n = 9) and a sham-operated group (n = 7). Parameters of DNA synthesis--thymidine kinase activity and in vivo bromodeoxyuridine incorporation--were measured at partial hepatectomy (T = 0), and 24 and 48 h after partial hepatectomy. During common bile duct obstruction, DNA synthesis was already stimulated at T = 0, but partial hepatectomy in common bile duct obstruction rats induced a delayed DNA synthesis. After 1 week of restoration of bile flow, normal DNA synthesis had returned at T = 0, but DNA synthesis after partial hepatectomy was still delayed. The sham-operated rats showed a normal regeneration response after partial hepatectomy assessed by bromodeoxyuridine incorporation but delayed as assessed by thymidine kinase activity, partly due to the impaired physical condition of the animals. The present data support the hypothesis that during cholestasis, regeneration promoting, and inhibitory factors accumulate in the liver, their balance determining whether regeneration after partial hepatectomy will occur in a normal, enhanced or delayed way.

Animals↗

Amino acid release from cerebral cortex in experimental acute liver failure, studied by in vivo cerebral cortex microdialysis.

Both increased gamma-aminobutyric acid (GABA)-ergic and decreased glutamatergic neurotransmission have been suggested relative to the pathophysiology of hepatic encephalopathy. This proposed disturbance in neurotransmitter balance, however, is based mainly on brain tissue analysis. Because the approach of whole tissue analysis is of limited value with regard to in vivo neurotransmission, we have studied the extracellular concentrations in the cerebral cortex of several neuroactive amino acids by application of the in vivo microdialysis technique. During acute hepatic encephalopathy induced in rats by complete liver ischemia, increased extracellular concentrations of the neuroactive amino acids glutamate, taurine, and glycine were observed, whereas extracellular concentrations of aspartate and GABA were unaltered and glutamine decreased. It is therefore suggested that hepatic encephalopathy is associated with glycine potentiated glutamate neurotoxicity rather than with a shortage of the neurotransmitter glutamate. In addition, increased extracellular concentration of taurine might contribute to the disturbed neurotransmitter balance. The observation of decreasing glutamine concentrations, after an initial increase, points to a possible astrocytic dysfunction involved in the pathophysiology of hepatic encephalopathy.

Acute Disease↗

Three different hepatocyte transplantation techniques for enzyme deficiency disease and acute hepatic failure.

The effects of three different techniques of hepatocyte transplantation were investigated: transplantation of free hepatocytes into the spleen and intraperitoneal transplantation of microcarrier-attached hepatocytes or of microencapsulated hepatocytes. The liver-supportive functions of these transplanted hepatocytes were analyzed using either the Gunn rat (hyperbilirubinemia) or rats with acute liver failure. In the Gunn rat intraperitoneal transplantation of microcarrier-attached hepatocytes resulted in a significant reduction of plasma bilirubin for 28 days whereas intraperitoneal transplantation of microencapsulated hepatocytes was ineffective notwithstanding immunosuppression by cyclosporin A. Intrasplenic hepatocyte transplantation was only effective in reducing plasma bilirubin for 14 days. During acute liver failure, liver support was achieved temporarily by hepatocyte transplantation in the spleen, by intraperitoneally transplanted microcarrier-attached hepatocytes, and by microencapsulated hepatocytes to equal extents, the microencapsulated hepatocytes being the least effective after 8 h of liver ischemia.

Animals↗