Stipe Batinica

Increased Toll-like receptor 4 expression in infants with respiratory syncytial virus bronchiolitis

The fusion protein of the respiratory syncytial virus (RSV) binds to the pattern recognition receptors, TLR4 and CD14, and initiates innate immunity response to the virus. The aim of the study was to investigate the expression of TLR4 on peripheral blood lymphocytes and monocytes in peripheral blood of infants in both acute and convalescent phase of RSV bronchiolitis (n = 26). In addition, TNF‐α expression in lipopolysaccharide‐stimulated monocytes was also assessed. The results showed TLR4 to be expressed predominantly by monocytes in both sick infants and controls. During the acute phase of infection monocytes up‐regulated TLR4 in eight infants, which returned to the levels recorded in controls 4–6 weeks from infection. There was no difference in the percentage of TNF‐α secreting monocytes. Of the clinical parameters tested, minimal oxygen saturation was found to correlate negatively with this expression in the group of infants with increased TLR4. Additional studies are under way to correlate this finding with the outcome of the immune response to RSV.

Intestinal duplications--experience in 6 cases.

Intestinal duplications are rare congenital anomalies, and most of them are detected in the first 2 years of life or antenatally. The clinical presentation depends on location, size, and the presence of ectopic gastric mucosa. Ultrasound scans during pregnancy result in a higher rate of antenatally detected duplications which allows early treatment and avoidance of possible complications. Resection of the duplication without adjacent bowel should be the treatment of choice. En bloc resection or partial excision with mucosectomy is advised in some cases. We present our experience with 6 patients treated in our institution from February 2002 to October 2005.

Xerostomia in patients with triple A syndrome – a newly recognised finding

Abstract Triple A syndrome is characterised by achalasia, alacrima, adrenal insufficiency and progressive neurological abnormalities including impaired autonomic nervous function. We present five patients with triple A syndrome in whom we describe xerostomia for the first time, a symptom which was presumed to be practically exclusive to Sjøgren syndrome and familial dysautonomia. Conclusion We recommend the investigation of salivation in all patients with triple A syndrome and treatment of xerostomia in order to ease swallowing. Further, our results corroborate earlier doubts that some patients with Sjøgren syndrome, especially those with the so-called “achalasia sicca” syndrome and adrenocortical insufficiency, actually had triple A syndrome. Therefore, adrenocortical function should be assessed in all patients with Sjøgren syndrome, particularly in those with difficulties in swallowing, because even latent adrenocortical insufficiency could be life-threatening for these patients in stressful situations.

Percutaneous transluminal renal angioplasty (PTRA) as a method of therapy for renovascular hypertension in children

The results of PTRA in treatment of renovascular hypertension in four children aged 5, 7, 13 and 15 are presented. All patients suffered from severe hypertension. Tests showed all of them to have fibromuscular dysplasia stenosis of the main renal artery and significant lateralization of renin levels. One suffered from associated polycystosis of kidneys and in another the renal artery stenosis was bilateral. In all patients successful patency was achieved. In one patient, the arterial pressure after PTRA was normalized, while in the others it was considerably improved. Two patients, tested 8 and 12 months after PTRA, were lost to further follow-up. In one of the remaining two, stenosis and hypertension reappeared 5 years after PTRA. After autotransplantation the patient was normotensive. In the other, also 5 years later, recurrent hypertension appeared related to the associated polycystosis.

An unusual outcome in a child with hepatosplenic cat-scratch disease.

ZusammenfassungDie klassische Katzenkratzkrankheit (Infektion mit Bartonella hensalae) ist bei einem immunkompetenten Kind mit der Anamnese eines Kratzers, Bisses oder intimen Kontaktes mit einer Katze vergesellschaftet. Die meisten Patienten entwickeln nach drei bis 10 Tagen einen harten Knoten in der Kratzlinie. Dieser kann wenige Tage, aber auch zwei bis drei Wochen bestehen bleiben. Während der nächsten ein bis zwei Wochen, manchmal auch länger, schwellen die regionalen Lymphknoten, die das Gebiet drainieren, in 10% der Patienten allmählich an und wieder ab. Die Knoten entwickeln ein Erythem, manchmal eitern sie. In wenigen Fällen von Katzenkratzkrankheit ist der Verlauf atypisch: Es kommt dann zu Augen- oder neurologischen Symptomen, Befall der Leber und Milz, Osteomyelitis der Wirbel, Endocarditis und Ähnlichem. Die Infektion mit Bartonella hensalae kann bei immunkompromittierten Individuen zu einer bazillären Angiomatose, bzw. Peliosis und zu einer rezidivierenden Bakteriämie führen. Es gibt verschiedene Berichte über Granulome in der Leber oder Milz die bei immunkompetenten Kindern durch B. hensalae ausgelöst wurden. Wir berichten über einen sechsjährigen Knaben mit einer derartigen hepatosplenalen Form der Katzenkratzkrankheit. Trotz früher Diagnose und Langzeit-Antibiotika-Behandlung konnte bei ihm eine Splenektomie nicht vermieden werden.SummaryTypical cat-scratch disease (Bartonella henselae infection) in an immunocompetent child is usually associated with a history of scratch, bite or intimate contact with a cat. Most patients develop a non-tender papule in the scratch line after three to ten days. This may persist for only a few days or as long as two to three weeks. During the next two weeks or more, regional lymph nodes that drain the area gradually enlarge and then slowly resolve in more than 10% of patients. The nodes develop overlying erythema and may suppurate. Atypical forms of cat-scratch disease occur in a minority of cases and are characterized by ocular or neurological manifestations, hepatosplenic involvement, vertebral osteomyelitis, endocarditis etc. Immunocompromised individuals with B. henselae infection may develop bacillary angiomatosis, bacillary peliosis, and relapsing bacteremia. There have been several reports of hepatosplenic granulomas caused by B. henselae in immunocompetent children. We report a case of a 6-year-old boy with the hepatosplenic form of cat-scratch disease. Despite early diagnosis and long-term antimicrobial treatment, splenectomy could not be avoided.

Successful Early Pyeloplasty in Infants

BACKGROUND Early pyeloplasty for the treatment of congenital ureteropelvic junction obstruction to maximize nephron salvage is justified only if potential hazards of operating on small infants are avoided. METHODS The records were analyzed of all infants who underwent pyeloplasty over a 5-year period. Open pyeloplasty was performed if collecting systems had deteriorated or were demonstrated to be obstructed; it was also performed for severe cases of hydronephrosis. Outcome of surgery in the younger infant (patients <2 months of age) was compared with the older infant group (patients >2 months of age). Preoperative evaluation in case of mild or moderate hydronephrosis was directed toward ruling out a non-obstructed collection system and included voiding cystourethrography, and serial ultrasonography and/or dual isotope diuretic renography. Postoperative assessment consisted of serial ultrasonography and/or nuclear imaging to confirm decompression and relief of obstruction. RESULTS A total of 24 pyeloplasties were performed on 22 patients in the younger infant group (two bilateral) and 30 were performed on 27 infants in the older infant group (three bilateral). The only significant differences between the groups were as follows: patients in the younger infant group were likely to present in utero (75%, p = 2.69), whereas those in the older infant group were more likely to present with a urinary tract infection (48%, p = 4.12). During follow-up examination, 23 renal units in the younger infant group and 24 in the older infant group were judged to be stable or improved. Four kidneys were not salvaged after pyeloplasty, one in the younger infant group and three in the older infant group. CONCLUSIONS Good results of pyeloplasties performed in the infants in this series support early correction of ureteropelvic junction obstruction in infants.

PREHEPATIC PORTAL HYPERTENSION: 43 YEARS OF FOLLOW-UP IN SINGLE INSTITUTION AND 20 YEARS AFTER INTRODUCTION OF SCLEROTHERAPY: PH1-23

Introduction: Surgery was treatment of choice for children with complications of prehepatic portal hypertension until sclerotherapy and band ligation has occurred. Introduction of octerotide was another landmark in treatment. We report a 43- year (first 26 retrospective and next 17 prospective) analysis of 64 patients. Methods: The charts of all patients treated before 1987 were reviewed retrospectively and from that year on a prospective evaluation based on database and registry has been started. Results: Between 1960 and Dec 2003 64 children were followed up between 2 and 43 years with a median of 17.4 years. We have divided our patients in two groups. First consisted of all patients (No 20) treated before the introduction of sclerotherapy (1984. in our hospital), and in the second group were remaining 44 patients. First symptom in historical group was hematemesis in 17 of 20 patients (85%). Thirteen palliative surgical interventions, and 4 shunt (spleno-renal) procedures were done in 14 patients (mortality rate 35%), a 6 patients were treated medically (mortality rate 33%). In group which was treated initially with sclerotherapy hematemesis was leading symptom in 54% of patients, and overall mortality rate is 9%. In this group, 20 patients along the course of their disease, due to failure of conservative therapy or other reasons were assigned for 25 operations. Mortality rate in combination therapy group is 12.5%. Rest of the patients (24) were treated only with sclerotherapy or octreotide. Mortality rate in this subgroup is only 5%. In the last 2 years, there was any new case of prehepatic portal hypertension and we are the only hospital in our country equipped for dealing with such a problem. There is a steady decline in incidence that has been observed for years probably due to disappearance of so far unknown etiological factor and improving neonatal practices. Conclusion: There is a decline in incidence of prehepatic portal hypertension. Sclerotherapy and octreotide have improved outcomes considerably, but there is still subset of patients who have to be operated. Our data support thesis that exist subgroup of patients who should be candidates for primary surgical procedure.