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Biomedical subjects

C V Hulzebos

Publications and source records attributed to C V Hulzebos.

9 recordsLinked to original sources
PubMed2004

Progressive facial hemiatrophy: a complex disorder not only affecting the face. A report in a monozygotic male twin pair.

UNLABELLED: Progressive facial hemiatrophy (PFH) is a ubiquitous disease, characterized by hyperpigmentation of skin followed by unilateral craniofacial atrophy of subcutaneous tissues, including fat, muscle and bone. Hereditary factors have been postulated to be involved in the aetiology of PFH. Yet, the occurrence of PFH in one of two identical male twins reported here makes this possibility unlikely. PFH usually occurs in the first two decades of life, and the clinical presentation resembles linear scleroderma. PFH may be complicate by autoimmune, neurological, ocular and dental disorders. Management of PFH comprises a long term follow-up of somatic disorders, and prevention of psychological problems. Treatment of PFH is symptomatic and consists of plastic surgery after the disease activity has stopped. CONCLUSION: The occurrence of PFH in one of monozygotic twin pair suggests that genetic factors are not involved in its aetiology. Early diagnosis of PFH and accurate follow-up is essential to disclose the occurrence of complications.

Child↗
PubMed2001

Measurement of parameters of cholic acid kinetics in plasma using a microscale stable isotope dilution technique: application to rodents and humans.

A stable isotope dilution method is described that allows measurement of cholic acid (CA) kinetics, that is, pool size, fractional turnover rate (FTR), and synthesis rate in mice, rats, and humans. Decay of administered [2,2,4,4-2H4]CA enrichment was measured in time in 50-microl plasma samples by gas-liquid chromatography/electron capture negative chemical ionization-mass spectrometry, applying the pentafluorobenzyl-trimethylsilyl derivative. The kinetic data expressed species-dependent differences. The CA pool sizes were 16.8 +/- 2.1, 10.6 +/- 1.2, and 2.4 +/- 0.7 micromol/100 g body weight for mice, rats, and humans, respectively. The FTR values were 0.44 +/- 0.03, 0.88 +/- 0.10, and 0.46 +/- 0.14 per day for mice, rats, and humans. The corresponding synthesis rates were 7.3 +/- 1.6, 9.3 +/- 0.1, and 1.0 +/- 0.2 micromol/100 g body weight per day. The human data agreed well with literature data obtained by conventional isotope dilution techniques. For rats and mice these are the first reported isotope dilution data. The method was validated by confirmation of isotopic equilibrium between biliary CA and plasma CA in the rat. Its applicability was demonstrated by the observation of increased CA FTR and CA synthesis rate in rats fed cholestyramine, which is known to increase fecal bile acid excretion. The presented stable isotope dilution method enables the determination of CA kinetic parameters in small plasma samples. The method can be applied in unanesthetized rodents with an intact enterohepatic circulation and may also be valuable when studying the development of human neonatal bile acid kinetics.

Adult↗
PubMed1999

Vertebral osteomyelitis associated with cat-scratch disease.

We describe a patient with vertebral osteomyelitis and paravertebral soft-tissue collections associated with cat-scratch disease (CSD). Diagnosis was established on the basis of histologic examination and serological and polymerase chain reaction (PCR) tests. Treatment consisted of administration of antibiotics, and although skeletal lesions were persistently evident on radiography the patient showed complete clinical recovery. In addition, 15 cases of documented osteomyelitis associated with CSD are reviewed.

Cat-Scratch Disease↗
PubMed1998

[Accidental ingestion of cigarettes by children].

Accidental ingestion of cigarettes (and butts) is mainly seen in young children. Nicotine in tobacco products is easily absorbed by the oral mucosa and intestines; absorption depends on nicotine content and pH of tobacco. Symptoms are caused by the nicotine component and usually develop rapidly (< 4 hours). The most common symptom is vomiting. Although cigarettes are potentially toxic, their ingestion by children is generally benign. Decontamination of the mouth with water may be useful. Induction of emesis is not advised. Gastric lavage is not needed in asymptomatic patients (with an unreliable history) or after vomiting. Children who ingested cigarettes should receive medical observation for four hours after ingestion. Children with significant symptoms should be admitted and eventually treated by supportive care. In symptomatic children or children with a reliable history of ingestion of large quantities who have not vomited gastric lavage with administration of activated charcoal has to be performed. When after vomiting other symptoms persist activated charcoal can be given via a nasogastric tube.

Accidents, Home↗
PubMed1998

[Urinary antigen test as a screen for the diagnosis of beta-hemolytic streptococcal infections in newborn infants].

OBJECTIVE: To differentiate between neonates with high and low risk of infections caused by group B beta-haemolytic streptococci (GBS), by using the urinary group B streptococcal antigen test. DESIGN: Retrospective. SETTING: Medical Centre Leeuwarden and Public Health Laboratory, Friesland, the Netherlands. METHODS: In a period of two years clinical, haematological and microbiological (including urinary group B streptococcal antigen detection) data were collected in newborns and their mothers who met one or more of the following criteria: a previous affected child, prolonged (> or = 12 hrs) rupture of membranes, fever in labour, unexpected preterm delivery, unexplained perinatal asphyxia. On the basis of surveillance cultures a colonization rate was made. GBS infection was 'suspected' in an unwell infant with a 'high' colonization rate; infection with GBS was 'proved' by a positive blood culture with GBS. RESULTS: 6 of 342 neonates had an infection with GBS. Risk of invasive infection increased with higher colonization rates. Sensitivity of the antigen test to detect colonization was low, sensitivity to detect neonatal infection was high (51 versus 100%). The negative predictive value of urinary antigen testing was 100%. Prolonged rupture of membranes (1.5% risk of infection) and maternal fever (5%) were the most important risk factors. CONCLUSION: In healthy neonates with risk factors but with a negative antigen detection test the risk of GBS infection is extremely low. In children with a risk factor a positive test result can indicate heavy colonization or infection. These children should be carefully observed and examined.

Adult↗
PubMed1998

Splenic epithelial cysts and splenomegaly: diagnosis and management.

Splenomegaly is a common problem. In the absence of systemic illness or malignancy splenic cysts must be considered, especially the epithelial variety. For large cysts total splenectomy has long been recommended. Recognition of the risk of an overwhelming postsplenectomy infection (OPSI), especially in children, has led to spleen conserving surgery. We describe the use of an absorbable Vicryl net after partial splenectomy with total cystectomy in the management of splenic epithelial cysts.

Adolescent↗
PubMed1996

[Progressive partial lipodystrophy; an external problem with internal anomalies].

Progressive partial lipodystrophy (PPL) was diagnosed in two girls aged 6 and 8 years. PPL is characterized by loss of subcutaneous fat, starting in the face and progressing to trunk and arms. Diagnosis is based upon the cachectic appearance and the normal growth parameters. It is a rare disease of unknown aetiology, usually beginning in childhood and more frequent in females. An association with diabetes mellitus, hypertriglyceridaemia and glomerulonephritis has been described. Follow-up should be focused on these and on psychological effects. No causal therapy is available. The facial appearance can be restored by injection of liquid silicones. Life expectancy does not appear to be affected.

Child↗