Demand for evaluation of vaccination antibody titers in children considered for renal transplantation.

Vaccinations are recommended for achieving protection against vaccine-preventable infections in solid-organ transplant recipients. In order to evaluate the protection at the time of renal transplantation, the antibody titers against measles, mumps, rubella, varicella, hepatitis B, diphtheria, and tetanus were determined in 35 children one month prior to transplantation. Only 26% of patients on dialysis listed for transplantation showed protective antibodies against all tested pathogens. Particularly, low protection was found for hepatitis B. Children younger than four yr showed significantly lower protective antibody titers compared with older children for almost all vaccines. Children who completed vaccination in the last six months to six yr prior to renal transplantation showed higher rates of protective antibody titers against all pathogens compared with children who had vaccination more than six yr before transplantation. Preventive strategies in children with chronic renal failure include repeated measurements of serum antibodies and appropriate revaccination if titers decline. Our results underline the demand for continuous surveillance of specific antibody titers against vaccine-preventable diseases in the risk group of renal transplant recipients.

Sporadic case of X-chromosomal Alport syndrome in a consanguineous family.

Alport syndrome (AS) is a genetic disorder of basement membranes caused by mutations in type IV collagen genes that is characterized by chronic hematuria and progressive nephropathy leading to renal failure. The main extrarenal features include sensorineural hearing loss and ocular lesions. The mode of inheritance is X-linked dominant in about 80%-85% of the affected families, whereas autosomal transmission is rarely encountered. We report a male patient originating from a healthy consanguineous Lebanese family who presented with an unusual association of obstructive uropathy and AS. Hematuria and proteinuria were initially attributed to a suspected poststreptococcal glomerulonephritis (GN) and high-grade subpelvic ureteral stenosis. Persistence of symptoms after medical treatment of poststreptococcal GN and surgical correction of obstructive uropathy finally led to renal biopsy. The observed ultrastructural changes of the glomerular basement membrane were typical for AS. Molecular genetic studies revealed a previously undescribed de novo mutation in the COL4A5 gene, excluding maternal heterozygotic carrier status. This case report emphasizes the importance of hereditary nephritis in the differential diagnosis of chronic hematuria, and demonstrates the value of molecular studies for genetic counselling in AS.

Protein gene product (PGP) 9.5 in diagnostic (neuro-) oncology. An immunomorphological study.

Most likely identical to ubiquitin carboxyl-terminal hydrolase isozyme L1 (UCH-L1), protein gene product (PGP) 9.5 is one of the major constituents of cytoplasmic polypeptides in neurons. The antigen seems to be expressed almost exclusively in neuronal and neuroendocrine tissues and their neoplasms. PGP 9.5 is also present in undifferentiated embryonic neoplasms like primitive neuroectodermal tumors (PNETs). However, the significance of PGP 9.5 as a marker in diagnostic (neuro-) oncology has not been systematically evaluated yet. In the present study the sensitivity and specificity of the widely used antiserum against PGP 9.5 has been retrospectively examined on 290 formalin-fixed, paraffin-embedded tumors of the nervous system and small round blue cell tumors. The presence of the antigen in tumors of neuronal (24/24) and neuroendocrine (63/73) differentiation confirm PGP 9.5 as a sensitive marker of these tumor groups, particularly in the diagnosis of pituitary adenomas where the expression was neither related to the staining behavior of the tumors in the PAS-orange G-reaction nor to their degree of polypeptide- and glycoprotein hormone activity. The nearly constant immunostaining of medulloblastomas (18/19) and other PNETs (5/6) demonstrate the role of PGP 9.5 as an additional marker in the detection of these embryonic tumors as this peptide was not or only weakly found in glial (11/58), meningeal (5/29), and nerve sheath neoplasms (1/28). On the other hand, the significance in the differential diagnosis of small round blue cell tumors seems to be limited because of positive immunoreactions in neuroblastomas (7/7) and oat cell carcinomas of the lung (7/7) although rhabdomyosarcomas (1/6) and malignant Non-Hodgkin-lymphomas (0/13) react completely negative in our series.

Liver failure due to disseminated HSV-1 infection in a newborn twin.

Most cases of neonatal herpes simplex virus (HSV) infection result from contact with maternal genital tract secretions and are caused by infections with HSV type 2. We report on a fatal HSV-1 infection in a newborn twin presenting with liver failure. The infection was acquired by single contact with an aunt. The route of transmission was proven by PCR followed by restriction endonuclease fingerprinting and DNA sequencing. This report demonstrates that liver failure may be an early and single symptom in life-threatening neonatal HSV-1 infection.