Transmission of the L-Zagreb mumps vaccine virus, Croatia, 2005-2008.

We report on three cases of symptomatic transmission of the L-Zagreb mumps vaccine virus from three vaccinated children to five adult contacts. The five contact cases were parents of the vaccinated children and presented with parotitis and in one case also with aseptic meningitis. The etiology of the contacts' illness was determined by viral culture, genomic sequencing, serology and epidemiological linking. Two of the vaccinated children developed vaccine associated parotitis as an adverse event three weeks following immunization. Symptoms in contact cases developed five to seven weeks after the vaccination of the children. The five contact cases, as well as the three children with adverse events recovered completely. The children had been vaccinated with MMR vaccine produced by the Institute of Immunology Zagreb, each of them with a different lot. One of the possible explanations for these adverse events is that the very low levels of wild mumps virus circulation in the last decade, combined with waning immunity in those who received one dose of vaccine or suffered from mumps in childhood, resulted in susceptible young adults and that this unique epidemiological situation allows us to detect horizontal transmission of mumps vaccine virus.

Right atrial reduction for tachyarrhythmias in Ebstein's anomaly in infancy.

A 20-month-old girl with Ebstein's anomaly developed supraventricular paroxysmal tachycardia, which seemed to be a result of the wall tension of the giant right atrium. Right atrial resection reduced the wall tension and overall dimensions of the right atrium and finally resolved the tachycardia and ectopic electrical conduction. Six-year follow-up electrocardiograms confirmed continuing normal sinus rhythm without occurrence of supraventricular paroxysmal tachycardia or other ectopic electrical activity The follow-up echocardiograms showed the size of the right atrium to be unchanged from the time of operation. There are few data in the available literature about performing right atriotomy to resolve a tachyarrhythmia associated with Ebstein's anomaly and none, to the best of our knowledge, about performing right atrial resection for this purpose.

[Familial adenomatous colonic polyposis]. / Obiteljska adenomatozna polipoza crijeva.

We described two patients (brother and sister) with familial adenomatous polyposis of the colon. It is an inherited disease with autosomal dominant pattern of inheritance. The incidence is 1:8.000, with usual onset of polyps development late in the first decade of life or during adolescence, and malignant alteration up to the fourth decade of life. APC gene located on long arm of chromosome 5 is responsible for occurrence of the disease that presents with onset of multiple adenomatous polyps in the colon (from some of them to 1000). The treatment includes chemoprevention by sulindac or aspirin that prevents or reverse process of carcinogenesis. Surgical approach is preventive colectomy up to 20 (25) years of life. APC gene mutation (deletion at codon 1309-1311) was proven by DNA analysis from blood and polyp in both patients. There was no evidence of mutations of genes p53 and K-ras. Preventive colectomy is planned as soon as possible.

Partial trisomy 13q22-->qter and monosomy 18q21-->qter as a result of familial translocation.

We report on a patient with a partial trisomy of chromosome 13q22-->qter and partial monosomy of chromosome 18q21-->qter showing distinct malformations. The phenotype of this unbalanced karyotype has not been previously described. The proband had a craniofacial dysmorphism, neck pterygium, closed fists with overlapping fingers, cutaneous appendix of the left fist, equinovarus and postaxial hexadactyly of the feet, atrial septum defect, unilateral cryptorchidism and hypertrophic pyloric stenosis. Using fluorescence in situ hybridization (FISH) the father's karyotype 46,XY.ish t(13;18)(13pter-->13q22::18q21-->18qter; 18pter-->18q21::13q22-->13qter) and the child's 46,XY.ish der(18)(18pter-->18q21::13q22-->13qter)pat were established. The mother's karyotype was normal. A risk of unbalanced offspring in carriers of a balanced reciprocal translocation depends on the length and genetic constitution of the exchanged segments. Risk figures should come only from empirical data. A phenotypically normal child with a balanced or normal karyotype could be born in the case of alternate segregation. Amniocentesis should therefore be recommended in any further pregnancy.