BACKGROUND: PCT has recently drawn attention as a quite specific marker for bacterial, fungal, and parasitic origin of severe sepsis-syndrome. These specific properties could make PCT to an important tool for sepsis monitoring in severely immunocompromised children. The clinical value of PCT in comparison to CrP was investigated in children after bone marrow transplantation (BMT). METHODS: PCT was measured in the serum of 48 children (median age 12.4 years) after BMT in a prospective study. Results were correlated with the clinical findings and compared to the C-reactive protein (CrP). RESULTS: PCT showed a sensitivity for diagnosing a sepsis-syndrome of 56%, a specificity of 87%, a positive predictive value of 69%, and a negative predictive value of 80%. Regarding CrP they were 100%, 41%, 46% and 100% respectively. The relative risk to die due to sepsis-syndrome was 26.4 for PCT levels over 10 ng/ml and 4.0 for CrP levels over 200 mg/l. It could be shown furthermore that there can be a significant liberation of PCT even during hematological aplasia. CONCLUSION: (1) Measuring PCT levels in the sera of children undergoing BMT improves the possibility of diagnosing severe infection and gives an important prognostic tool. (2) Measuring PCT can be recommended if severe sepsis-syndrome is suspected and there is an additional need for differential diagnosis and prognostic evaluation.

Adolescent↗

PubMedDate not supplied

Hematopoietic stem cell transplantation for mucopolysaccharidoses and leukodystrophies.

The only effective treatment for selected metabolic diseases is a successful allogeneic hematopoietic stem cell transplantation (HCT). Best results with HCT are obtained when performed early in the course of the disease. Hence, timely identification and referral are critical. Also, early identification of affected patients during the newborn period via screening may be invaluable, particularly for the infantile onset rapidly progressive forms of diseases. Rapid availability of a donor graft is often crucial for these patients. Preliminary experience suggests that results after umbilical cord blood (UCB) transplant may be comparable to those after marrow transplants. UCB grafts have certain benefits of rapid availability and even reduced risk of GvHD. Hence, UCB transplant represents an alternative to marrow HCT. Related haploidentical HCT, possibly with very high doses of CD34+ cells, may also represent an option. However, expertise has been developed in very few transplant centers and no large reports are available of its use for patients with inherited metabolic diseases.

Adrenoleukodystrophy↗

PubMedDate not supplied

[The slit ventricle syndrome--causes, differential diagnosis and therapeutic problems].

Shunt dependent patients with clinical signs of shunt malfunction and slit ventricles on computerized tomography could suffer either from low pressure or from a slit-ventricle-syndrome with increased pressure. In such cases we recommend a lumbal punction with pressure monitoring to make the diagnosis certain. In case of increased pressure we firstly inject 0.9% NaCl into the proximal part of the ventricular catheter to remove it's obturation. Sometimes shunt revision can so be avoided in the slit-ventricle-syndrome.

Adolescent↗

PubMedDate not supplied

[The tethered cord syndrome].

The "tethered cord syndrome" as a complication of spinal dysraphism is probably more important than assumed earlier. An abnormally low position of the conus medullaris is caused by different anomalies: e.g. adhesions, lumbosacral lipoma, tight filum terminale. In some patients no skinny changes can be detected. A "tethered cord syndrome" should be considered, if neuromuscular skeletal changes as club-foot, scoliosis, muscular atrophy of disturbances of gait, sensibility or function of bladder and rectum are recognized. It is also a result of a inadequately operated meningomyelocele. For experienced examiners sonography is an interesting non-invasive diagnostic procedure during infancy. The diagnosis should be completed by spinal computerized tomography and myelography. Surgery should be performed prophylactically. The "tethered cord syndrome" is explained by case histories of the University Hospital of Children, Freiburg.

Child, Preschool↗

PubMedDate not supplied

[Heparin-induced type II thrombocytopenia within the scope of high dose chemotherapy with subsequent stem cell rescue].

We report on a 17-year-old young man with rhabdomyosarcoma in the right parotid area. Relapse therapy was performed with high dose chemotherapy and consecutive autologous stem cell rescue. During this therapy heparin-induced thrombocytopenia was diagnosed. Because of its proven antithrombotic activity Danaparoid-Sodium, a natural low molecular glycosaminoglycan preparation, was used for further antithrombotic prophylaxis. We discharged our patient from the laminar air flow unit six months ago. The alternative antithrombotic therapy was tolerated without any problems. No bleeding events occurred, thrombotic complications and veno-occlusive disease of the liver were avoided.

Adolescent↗

PubMedDate not supplied

[Conclusiveness of various diagnostic procedures for squamous cell carcinomas of the visceral cranium and the oral cavity as compared with histological evaluations].

In 60 patients with primary squamous cell carcinomas of the oral cavity, clinical examination was complemented by 5 additional diagnostic procedures prior to surgery to assess tumor extension. Comparing these finding with the histological evaluation of the excised tissue demonstrated that, while the TNM classification remains the foundation of all tumor diagnostic procedures, the use of radiography, bone scintigraphy and CT is indispensable to ascertain the amount of tissue to be excised. CT competes with B-scan sonography in cervical lymph node diagnostics. Tumor scintigraphy has been found to be an unreliable method.

Aged↗