PubMed Health⌕ Search

Biomedical subjects

J M Draaisma

Publications and source records attributed to J M Draaisma.

At least 19 recordsLinked to original sources
PubMed2001

[Three infants with epidural hematoma].

Three infants, two boys aged 10 months and one girl aged 5 months, developed drowsiness and emesis within a few hours of a fall (after which they had not lost consciousness). Radiological examination revealed an epidural haematoma which was treated with emergency decompression. The children subsequently made a good recovery. An epidural haematoma is a potentially life-threatening event. More than 20% of all cases of epidural haematoma occur in childhood. In contrast with distinct symptoms in most adults and older children, an epidural haematoma in infancy can have a minimum of symptoms. The open fontanelle means compression occurs less rapidly, but consequently blood loss can be greater, leading to acute anaemia, hypovolaemic shock and consumptive coagulopathy.

Accidental Falls↗
PubMed2001

Local interferon-gamma levels during respiratory syncytial virus lower respiratory tract infection are associated with disease severity.

To investigate the role of cell-mediated immunity during respiratory syncytial virus (RSV) infection, interferon (IFN)-gamma and interleukin (IL)-10 levels in nasopharyngeal secretions were measured in infants with lower respiratory tract infection (LRTI) caused by RSV. A novel technique was used to measure in vivo cytokine levels in nasopharyngeal aspirates (NPAs). Cytokine levels in the NPAs of 17 mechanically ventilated infants and 43 nonventilated hospitalized infants were compared. As expected, mechanically ventilated infants were significantly younger than nonventilated infants (7 vs. 14 weeks). IFN-gamma levels were above the limit of detection in the NPAs of 3 (18%) mechanically ventilated infants and in the NPAs of 26 (60%) nonventilated infants. IL-10 levels in the NPAs of mechanically ventilated and nonventilated infants were comparable. It is hypothesized that maturation-related mechanisms have a key role in the development of RSV LRTI that results in mechanical ventilation.

Age Factors↗
PubMed2001

Effects of growth hormone treatment on left ventricular dimensions in children with Noonan's syndrome.

OBJECTIVE: To study the effects of long-term growth hormone (GH) treatment on left ventricular (LV) dimensions in children with Noonan's syndrome (NS). METHODS: Echocardiographic measurements of LV dimensions were performed before and during GH treatment in 27 participants (21 boys, 6 girls) in a partly controlled 3-year trial of high-dose GH treatment (0.15 IU/kg/day). Nineteen children had a congenital heart defect, 1 of them had hypertrophic obstructive cardiomyopathy. In the first 3 years, the children were assigned to 1 of 2 groups: group A with discontinuation of GH treatment in the 3rd year, or group B without GH treatment in the 1st year. After the 3rd year, 12 of the 27 children were followed up for 2 additional years to evaluate the long-term effects of GH treatment on the heart. RESULTS: At baseline, LV internal diameters were smaller, while posterior wall thickness were thicker than normal. Over the 1st year, changes in LV dimensions were comparable between the 2 groups. No significant differences were found in LV dimensions between the situation at baseline and after 4 years of GH treatment. CONCLUSION: Long-term high-dose GH treatment does not have clinically significant adverse effects on LV dimensions in children with NS.

Adolescent↗
PubMed1998

Severe mycobacterial and Salmonella infections in interleukin-12 receptor-deficient patients.

Interleukin-12 (IL-12) is a cytokine that promotes cell-mediated immunity to intracellular pathogens by inducing type 1 helper T cell (TH1) responses and interferon-gamma (IFN-gamma) production. IL-12 binds to high-affinity beta1/beta2 heterodimeric IL-12 receptor (IL-12R) complexes on T cell and natural killer cells. Three unrelated individuals with severe, idiopathic mycobacterial and Salmonella infections were found to lack IL-12Rbeta1 chain expression. Their cells were deficient in IL-12R signaling and IFN-gamma production, and their remaining T cell responses were independent of endogenous IL-12. IL-12Rbeta1 sequence analysis revealed genetic mutations that resulted in premature stop codons in the extracellular domain. The lack of IL-12Rbeta1 expression results in a human immunodeficiency and shows the essential role of IL-12 in resistance to infections due to intracellular bacteria.

Adult↗
PubMed1998

[Acute pancreatitis in children].

Acute pancreatitis is probably commoner in children than was previously thought. In children it is most commonly associated with trauma or viral infection. The presentation may be subtler than in adults, requiring a high index of suspicion in the clinician. In three children, two boys aged 4 and 10 and a girl of 15 years, acute pancreatitis was suspected because of the findings at ultrasonography and endoscopic retrograde cholangiopancreatography performed when the disease recurred (the boy aged 4), apathy and immobility without dehydration or other obvious causes (the boy aged 10), and severe abdominal pain in combination with vomiting (the girl). All three patients had severely increased (urinary) amylase levels. Most often, acute pancreatitis in children tends to be a self-limiting disease which responds well to conservative treatment.

Acute Disease↗
PubMed1998

Localization of the ICF syndrome to chromosome 20 by homozygosity mapping.

Immunodeficiency in association with centromere instability of chromosomes 1, 9, and 16 and facial anomalies (ICF syndrome) is a rare autosomal recessive disorder. ICF patients show marked hypomethylation of their DNA; undermethylation of classical satellites II and III is thought to be associated with the centromere instability. We used DNA from three consanguineous families with a total of four ICF patients and performed a total genome screen, to localize the ICF syndrome gene by homozygosity mapping. One chromosomal region (20q11-q13) was consistently found to be homozygous in ICF patients, whereas all healthy sibs showed a heterozygous pattern. Comparison of the regions of homozygosity in the four ICF patients localized the ICF locus to a 9-cM region between the markers D20S477 and D20S850. Analysis of more families will be required, to refine the map location further. Isolation of the gene associated with the ICF syndrome not only will give insight into the etiology of the ICF syndrome but will also broaden our understanding of DNA methylation processes.

Chromosome Mapping↗
PubMed1997

[Reading children's temperatures with the tympanic infrared thermometer and the rectal mercury thermometer: equally good results in the emergency room].

OBJECTIVE: To compare the results of reading body temperatures with a tympanic infrared thermometer and a rectal mercury thermometer in children in an emergency department. DESIGN: Prospective comparative study. SETTING: St. Elisabeth Hospital, Tilburg, the Netherlands. METHOD: In children up to 11 years of age seen in the emergency room between 1 January 1994 and 1 April 1994, the body temperature was measured with a rectal mercury thermometer as well as with a tympanic infrared thermometer. Data were collected on temperature read, clinical picture on arrival (not ill, ill, seriously ill) and appearance of the tympanic membrane (signs of acute otitis media, presence of cerumen). For the statistical comparison, the differences between the findings of the two methods were plotted against the means. The sensitivity and specificity of the results of tympanic measurement in relation to the values read rectally were determined. RESULTS: Data were collected on 213 children, of whom 19 were younger than 3 months, 46 between 3 and 12 months, and 148 between 1 and 11 years. The mean temperatures measured with the rectal and tympanic thermometers were 38.01 and 38.03 degrees C, respectively. The mean difference between the rectal and tympanic temperatures was -0.013 degree C. The correlation between the rectal and tympanic temperatures was high (r = 0.86; P = 0.0001). The results were the same in groups differing in age, severity of disease and appearance of the tympanic membrane. The sensitivity of the tympanic measurement for fever (rectal temperature > 38.0 degrees C) was 80.6% with a specificity of 92.5%. The sensitivity was 83.8% when a rectal temperature > 38.5 degrees C was taken as the criterion, with a specificity of 95.9%. CONCLUSION: The tympanic infrared measurement in children in an emergency department gave the same results as rectal measurement using a mercury thermometer.

Adult↗
PubMed1997

Use of random amplification of polymorphic DNA in a case of Pasteurella multocida meningitis that occurred following a cat scratch on the head.

We cultured Pasteurella multocida from the cerebrospinal fluid (CSF) of a 4-month-old infant who presented with meningitis. The patient had been scratched on the head by a cat. Culture of the cat's claws also yielded P. multocida. The isolates had identical biochemical patterns. Analysis of both strains by random amplification of polymorphic DNA and comparison of these strains with P. multocida strains isolated from other cats showed that the two strains were identical and completely different from the unrelated isolates. Our patient's meningitis most likely resulted from direct inoculation of P. multocida into the CSF.

Animals↗
PubMed1996

Myopathology in patients with a Noonan phenotype.

Two patients with a Noonan phenotype and progressive hypertrophic obstructive cardiomyopathy are described, in whom abnormal histopathological changes in striated musculature were detected. In both patients an increased density of muscle spindles was found at biopsy. The significance of an increased density of muscle spindles in patients with Noonan phenotype can only be speculated. The question is raised of whether these changes are a distinct feature within the spectrum of patients with Noonan phenotype.

Cardiomyopathy, Hypertrophic↗
PubMed1995

Autosomal recessive Melnick-Needles syndrome or ter Haar syndrome? Report of a patient and reappraisal of an earlier report.

We report on a patient with congenital glaucoma, brachycephaly with flat occiput, large anterior fontanel, hypertelorism, anteverted nostrils, thoracolumbar kyphosis, prominent coccyx with skin fold, short hands and feet, flexion deformity of fingers, and clubfeet. He had a double-outlet right ventricle with ventricular septal defect, and severe tricuspid insufficiency. Mild skeletal changes included short tubular bones, absence of distal phalanges of toes, caliber variation of ribs, and scalloping of the anterior surface of vertebrae. The patient died at age 21 months. He belongs to the same extended family as 3 similarly affected patients, previously described by ter Haar et al. [1982: Am J Med Genet 13:469-477] as representing an autosomal recessive form of Melnick-Needles syndrome. We believe this diagnosis is no longer tenable. After having reviewed the relevant literature, we conclude that most probably we are dealing with a new autosomal recessive syndrome. We propose to name this entity ter Haar syndrome.

Diagnosis, Differential↗
PubMed1994

Surgical repair of an aortic coarctation in a patient after treatment with extracorporeal membrane oxygenation.

Extracorporeal membrane oxygenation (ECMO) is a life-saving treatment for neonates who have severe respiratory failure that does not respond to maximal conventional therapy. A consequence of venoarterial ECMO is the sacrifice of the right common carotid artery. Evaluation of the impact of a single carotid artery in babies treated with ECMO concerns mostly long-term neurodevelopmental outcome. The authors encountered a peculiar problem caused by a single carotid artery in a post-ECMO patient during the surgical correction of aortic coarctation with hypoplastic distal aortic arch. For patients with a confirmed cardiac malformation that necessitates future surgical repair and for whom ECMO support is required, reconstruction of the right common carotid artery should be considered. Veno-venous ECMO is an alternative solution if this approach is not contraindicated because of the patient's clinical condition. Patients with congenital diaphragmatic hernia have a higher incidence of cardiac malformations; therefore, careful cardiological attention is required. Anomalies masked by pulmonary hypertension also must be considered.

Aortic Coarctation↗
PubMed1992

Mononeuritis multiplex in a child with cutaneous polyarteritis.

A 14-year-old boy presented with a febrile illness associated with arthritis. Shortly later he developed mononeuritis multiplex. After certain typical skin lesions had developed after two months, the diagnosis cutaneous polyarteritis could be made. The diagnostic features of this benign disease, which may involve peripheral nerves, are discussed.

Adolescent↗
PubMed1991

[Neonatal dural sinus thrombosis].

Neonatal dural sinus thrombosis is a relatively unknown disease. We recently diagnosed two newborn patients with dural sinus thrombosis. Both patients had associated conditions known to predispose to dural sinus thrombosis. One patient had severe perinatal asphyxia, and the other patient had a deficiency of antithrombin III. Both patients had severe neonatal seizures. The literature on some aspects of this entity is discussed. It is concluded that dural sinus thrombosis may represent an important and underrecognized cause of neonatal seizures.

Antithrombin III Deficiency↗
PubMed1990

Rupture of the supradiaphragmatic inferior vena cava by blunt decelerating trauma: case report.

Lacerations of the inferior vena cava resulting from blunt external trauma are relatively rare, but extremely serious. The high lethality is due to the difficulty in diagnosis and technical problems with repair, particularly if the injury is located above the renal veins. During a 12-month period seven patients with inferior vena cava laceration were seen, of whom two presented with laceration of the inferior vena cava above the diaphragm. Both had a deceleration injury while wearing seatbelts. The clinical presentation was similar. The etiology is discussed. Caval continuity should be repaired because acute sudden occlusion at the suprahepatic level is incompatible with survival. Median sternotomy is advised, moreover it provides good exposure for eventual cannulation.

Adult↗