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-alpha 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-alpha 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.

Sclerosing haemangioma of the lung in a 4-year-old child.

Sclerosing haemangioma of the lung (SHL) should be recognised as a distinct clinicopathological entity. It is a benign neoplasm, probably of epithelial origin. Clinically, the tumour is asymptomatic and shows a striking preponderance in middle-aged women. SHL is often detected incidentally, as a round, well-defined homogeneous mass on routine chest radiograms. The diagnosis is based on pathohistologic examination of the biopsy material, therapy is surgical, and prognosis is excellent. We report a case of a 4-year-old boy with SHL, which is extremely rare in childhood.

[Hepatic portoenterostomy and primary liver transplantation in the treatment of biliary atresia]. / Hepatoportoenterostomija ili primarna transplantacija jetre u lijecenju bilijarne atrezije.

Results show that the use of sequential surgical treatment, employing Kasai portoenterostomy in infancy, followed by selective liver transplantation for children with progressive hepatic deterioration yields improved overall survival. All children with successful Kasai portoenterostomy procedures who do not require orthotopic liver transplantation are survivors. Using newer transplant techniques, the 5-year survival rate for children who receive transplants with a primary diagnosis of biliary atresia was 82%. This yields an overall survival rate of 86% in this entire study population. Limited donor availability and increased complications after liver transplantation in infants less than 1 year of age mitigate against the use of primary liver transplantation without prior portoenterostomy for infants with biliary atresia. At present, these two operative procedures should be used as sequential and complementary modes of treatment rather than as competitive procedures. When biliary atresia is not recognized in infancy and established cirrhosis has resulted, primary transplantation should be offered as the initial surgical treatment.

Xerostomia in patients with triple A syndrome--a newly recognised finding.

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.

[Primary adrenocortical micronodular dysplasia]. / Primarna adrenokortikalna mikronodularna displazija.

Two girls (11 and 13 years old) with Cushing's syndrome due to primary adrenocortical micronodular dysplasia (PAMD) are presented. High plasma cortisol concentrations, elevated urinary free cortisol and 17-ketogenic steroids excretion, in addition to low or normal plasma adrenocorticotropic hormone (ACTH) levels pointed towards independent adrenal cortisol hypersecretion. In both girls bilateral adrenalectomy was performed, followed by replacement therapy with glucocorticoids and mineralocorticoids. Pathohistological findings of otherwise enlarged adrenal glands, showed characteristic small nodules measuring 1-2 mm, composed of cells resembling those of zona fasciculata, with abundant, clear cytoplasm. Our younger patient fulfilled the criteria of "Carney complex", because beside PAMD she has had the lentigines.

Zimmerman-Laband syndrome: An unusually early presentation in a newborn girl.

We report on a female newborn, the youngest patient with Zimmermann-Laband syndrome hitherto reported. She had gingival hyperplasia, bulbous soft nose and ears, hypoplastic toenails, and hyperextensibility of the joints, as well as deep palmar and plantar creases, a sign not previously described in literature.

[Posterior sagittal anorectoplasty (PSARP) in children with high anal atresia]. / Posteriorna sagitalna anorektoplastika (PSARP) u djece s visokom atrezijom rektuma.

Anorectal anomalies are highly significant (the incidence of 1:3000 infants). The knowledge of the factors related to fecal continence encouraged the introduction of new surgical approaches in the second half of this century. With high supralevator anorectal anomalies the percentage of incontinence in children was earlier 50-80% although in all surgical procedures efforts were made to reconstruct the normal anatomy of the small pelvis. It was only in 1982 that by the PSARP method the anatomic reconstruction was achieved. In this operation an electric stimulator is used to identify the entire muscle-sphincter complex (external sphincter, the cross-striated muscle complex and the levator musculature) and then by stimulation of muscles a mobilized and modeled rectum is inserted into the muscle-sphincter complex. During the ten-year application of the PSARP method 15 children underwent the operation, of which 14 boys and 1 girl. The girl had rectovaginal fistula and 11 boys had rectourethral fistula. More than 70% of our children showed accompanying anomalies. Control examinations of children older than 3 years show the 67% continence in our children operated by the Pena-de Vries method.

[The Johanson-Blizzard syndrome]. / Johanson-Blizzardov sindrom.

A 17 year and 10 month old boy with Johanson-Blizzard syndrome is presented as a case report for the first time. Diagnosis has been established on the basis of craniofacial abnormalities: microcephalia, parietal skin and bone defects, sparse hair with frontal up sweep, alae nasi hypoplasia, irregular dentition and nasolacrimal fistula, with mental insufficiency, partial exocrine pancreatic insufficiency and low birth-weight and length, hypotonia and failure to thrive in infancy. Congenital cataract and hiatus sacralis apertus are additional signs that have never been described in the literature concerning Johanson-Blizzard syndrome.

Vaginal yolk sac tumor in a nine-month-old female child.

A case of yolk sac tumor in a nine-month-old girl is presented. Vaginal hemorrhage was observed during the patient's check-up at the Bjelovar Children's Outpatient Clinic. The patient was referred to the Department of Pediatrics at the Zagreb University Hospital, where an endodermal tumor of the vaginal yolk sac was diagnosed. The patient underwent surgery, followed by chemotherapy. She is now 3.5 years old, in good general health and under regular control.

[Laparoscopic cholecystectomy in a child with sickle cell disease]. / Laparoskopska kolecistektomija u djecaka s drepanocitozom.

Laparoscopic cholecystectomy has become the procedure of choice for the removal of the diseased gallbladder in children. Its use in small children and infants is rare. At the Department of Pediatric Surgery of the Medical faculty in Zagreb five children underwent laparoscopic cholecystectomy. One of them was a five year old boy with sickle cell disease and cholelithiasis. Before the advent of laparoscopy, few children with sickle cell disease and cholelithiasis underwent cholecystectomy, because of the high morbidity associated with open cholecystectomy. The authors use the case to present details of the operative procedure of laparoscopic cholecystectomy in small children and infants. Laparoscopic cholecystectomy is the procedure of choice in the management of cholelithiasis in small children and infants, especially those with sickle cell disease.

Urolithiasis in childhood: surgery and lithotripsy.

From 1982 through 1993, 174 interventions in 160 infants and children with urolithiasis were performed at our department. There were 101 boys and 59 girls, mean age 8.9 years. The main causes of the interventions were congenital malformations in 62, followed by recurrent urinary tract infections in 29, previous operations in 17, and hypercalciuria in 35 patients. In 17 children, the cause of urolithiasis remained unknown. As a therapeutic option, extracorporeal shock-wave lithotripsy (ESWL) has become available since 1988. The malformations and postoperative conditions are surgically corrected, and the calculi removed in the same act. For post-infectious, idiopathic or hypercalciuric calculi, ESWL has been used in the majority of patients since 1988. Thus, a total of 129 calculi were removed by operation and 47 by ESWL, whereas 18 calculi were endoscopically extracted. There were no complications after either ESWL or open surgery. Calculi in congenital malformations or as a result of operation are still removed by open surgery. In other cases, ESWL is the method of choice.

[Cholinesterase in plasma and cerebrospinal fluid in patients with viral meningitis]. / Kolinesteraza u plazmi i cerebrospinalnom likvoru bolesnika s virusnim meningitisom.

Pseudocholinesterase activity (PChE) in human plasma and cerebrospinal fluid was determined in 30 patients; in 15 patients with acute viral meningitis and in 15 patients with meningism. Concentration of total proteins in cerebrospinal fluid was also determined. PChE activity and protein concentration in cerebrospinal fluid of patients with meningitis is significantly higher than in control group (P < 0.001). Relationship of PChE activity and protein concentrations in cerebrospinal fluid of patients with meningitis does not differ from that in the control group patients. Thus, the increase of PChE activity in the cerebrospinal fluid of patients with viral meningitis suggests an alteration in the blood-brain barrier, leading to an increase in the passage of serum components.

Our 10-year experience with embolized Wilms' tumor.

In the Department of Pediatric Surgery, Clinical Hospital Center Rebro in Zagreb, during the last 10 years a new method of invasive diagnostic and at the same time therapeutic procedure for the treatment of Wilms' tumor has been introduced. The treatment is preoperative Percutaneous Transcatheter Intraarterial Embolization (PTIE) of the renal artery. The aim of this procedure is to reduce vascularization, to decrease the mass of kidney affected by the tumor, to separate it from the surrounding tissue, to decrease intraoperative spillage of malignant cells into the blood stream and their metastasizing. As a result nephrectomy is easier to perform. It has been confirmed that it is best to perform nephrectomy 48 hours after embolization. The authors present their own experience with 33 patients, ranging from 1 to 16 years of age.

War wounds in the Sibenik area during the 1991-1992 war against Croatia.

This report presents the analysis of war casualties treated at the Department of Surgery of Sibenik Medical Center during a 5.5-month period, i.e., from the beginning of the aggression in August 1991 to the international recognition of the Republic of Croatia on January 15, 1992. A total of 321 war casualties were treated, 157 of whom were out-patients and 164 in-patients with severe multiple injuries, 39.25% of the wounded were civilians, 10 of whom were children. Fragment wounds from mortar shells, anti-personnel mines, and infantry weapons were the most frequent type of injuries. The most common were injuries of the limbs. Gunshot injuries to the brain had the worst prognosis. Early management of the wounds was of special importance. Stabilization of the fracture using external fixators was indicated in patients with severe, complicated fractures associated with extreme defects of the tissue. Comatose patients with possible intra-abdominal injury presented a specific diagnostic problem, which was best treated by exploratory laparotomy. The overall mortality rate was 2.49%.

Bilateral gonadoblastoma in a 9-month-old infant with 46,XY gonadal dysgenesis.

A 9-month-old infant with hypertrophic clitoris and separated urethral and vaginal opening was diagnosed as having 46,XY incomplete "pure gonadal dysgenesis". Examination of both gonads revealed gonadoblastoma and bilateral salpingo-gonadectomy and partial hysterectomy were performed. Patient is raised as a girl, and clitoroplasty will be done in next several months. The fact that gonadal neoplasia in our patient was found already at the age of 9 months, confirms the need for gonadectomy at the time of diagnosis in the patients with 46,XY gonadal dysgenesis.

Cloacal exstrophy: a case report.

A case of an extremely rare type of cloacal exstrophy in a male infant with a normally developed subvesical part of the urinary system and external genitalia but absent distal colon segment is presented. The patient also had omphalocele, upper urinary tract anomalies and sacrococcygeal teratoma.

Congenital pulmonary arteriovenous fistula: a rare cause of cyanosis in childhood.

Two children (both females) aged 15 months and 4 years are described as very rare cases of central cyanosis in childhood being caused by a congenital pulmonary arteriovenous fistula. The initial diagnosis was made based on cyanosis and chest radiographs, with normal physical, ECG, and radiological findings of the heart. They had no family history of the Rendu-Weber-Osler syndrome. The patients underwent cardiac catheterization and pulmonary angiography, where the diagnosis was confirmed. After the surgery, both were symptom-free, and had no evidence of the disease.

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.

[The present status of treatment of splenic injuries in children]. / Danasnji stav o lijecenju ozlijedene slezene u djece.

Small but very dangerous risk of postsplenectomic sepses gave rise to the examination of surgical attitudes of medical treatment of damaged and ruptured spleen. Some new ideas of immunology revealed the importance of the spleen in the protection of the host and serious defect of immunologic system if the spleen is removed. According to those ideas, ruptured spleen is to be retained by all means. Some minor lesions and hemorrhages that are easily controlled by conservative measures, are not to be operated. In case of serious ruptures and massive hemorrhages necessitating more than 20 ml/kg of fresh blood to replace lost volume, penetrated abdominal injuries and with polytraumatic patients, laparotomy, surgical hemostasis and preservation of the spleen are indicated if possible. In the period of the last eight years, 90 children were treated because of the possibility they had injured spleen. Thirty-eight patients (42%) were under observation. Hemodynamic indicators were normal, so we did not make any aggressive diagnostic examinations. Other 47 patients had splenic scintigraphy, computed tomography (CT) of the abdomen or ultrasound examination. The findings were positive with 32 (69%) patients. Of those patients, 14 (44%) were immediately operated since serious rupture of the spleen was diagnosed and clinical signs suggested the presence of severe abdominal bleeding. To this number of operated patients, four polytraumatic patients and one suffering from penetrated injury are to be added. They were indicated the exploration of the abdomen without previous diagnostics. There were 19 surgical treatments of ruptured spleen; three of them included splenectomy while the other patients underwent splenic preservation.(ABSTRACT TRUNCATED AT 250 WORDS)

Primary vesicoureteric reflux treated by antireflux ureterocystostomy at the vertex of the bladder. A 12-year follow-up and analysis of operative failure.

A total of 618 children with primary vesicoureteric reflux (VUR) were examined preoperatively and 6, 9 and 24 months after an antireflux ureterocystostomy of the vertex of the bladder (AUVB); 206 children were operated on bilaterally, so that 824 AUVBs were performed. Most patients had grade 3 or 4 reflux and only 5% had grade 2 reflux. Successful results were noted in 792 ureteric units; in 10 cases of post-operative stenosis and 17 cases of recurrent VUR a second AUVB was performed. The following causes of operative failure were found: obstruction of the submucosal ureter after tailoring (5 cases), stricture of the new ureteric orifice (3), necrosis of the terminal part of the ureter (2), submucosal ureter too short or too wide (12) and fibrosis of the detrusor muscle over the submucosal ureter (5); a success rate of 84% was noted after the second procedure.