Ductal plate malformation in patients with biliary atresia.

UNLABELLED: The presence of ductal plate malformation (DPM+) on liver histology in children with biliary atresia (BA) is a marker of early intrauterine disease onset and an indication of an unfavorable prognosis. We studied the prognostic value of DPM in infants with BA after hepatoportoenterostomy (HPE). We reviewed 28 BA patients who underwent HPE in a single medical center. We examined the time of jaundice onset after delivery (conjugated hyperbilirubinemia): early onset (fetal phenotype with no jaundice-free interval) vs. late onset (perinatal phenotype with jaundice-free interval) and the presence or absence of DPM (DPM+ or DPM-) histopathology. Primary outcome was jaundice clearance at 3 months after HPE and survival with native liver (SNL). Eight children had fetal and 20 had perinatal BA (8 DPM+, 12 DPM-). At 3 months after HPE, no patients with fetal BA had achieved jaundice clearance, while jaundice clearance was achieved in five patients with DPM+ perinatal disease and four patients with DPM- perinatal BA (P = 0.03, comparing all three groups; P = 0.36, comparing DPM+ vs. DPM- perinatal patients). Median SNL was 8.6 months for fetal BA patients, 148.2 months for DPM+ perinatal BA patients, and 93.2 months for DPM- perinatal BA patients (log-rank test, P < 0.001, comparing all three groups; P = 0.59, comparing DPM+ vs. DPM- perinatal patients). After adjusting for BA type, age older than 2 months at HPE was associated with worse SNL [P = 0.03; hazard ratio = 4.0 (95 % CI, 1.1-14.2)]. CONCLUSIONS: Early onset of jaundice, regardless of DPM histology, was the most ominous sign of poor outcome in infants with BA after HPE.

[Results of treatment of tibial fractures in children]. / Rezultati lijecenja prijeloma potkoljenice u djece.

Diaphyseal fractures of both lower leg bones are the most common fractures of lower extremities, and account for about 15% of all fractures of long bones in children. These fractures are usully unstabilae, difficult to reposition, and retention of the fragments, and the process of their treatment is not fully compliant. The paper analyzes the late results of treating 234 children with tibial fractures, depending on the method of treatment (surgical and conservative method). Twenty-three children had open fractures (9.8%). Nonsurgical method was used in the treatment of 194 children, and surgical in 40 children. The most frequent surgical method was closed reposition of the fragments, and percutaneous elastic stable intramedullary nailing with titanium wires. The success of the treatment was measured: residual angular deformities and difference in length between treated and healthy leg. Secondary displacement of fragments after primary conservative treatment was found in 32 children. Angular deformities of the treated tibia was seen in 80 children, 68 (35.0%) treated conservatively and 12 (30.0%) surgically. In 131 (67.5%) conservatively treated and 29 (72.5%) surgically treated children there were no differences in the length of sick and healthy leg. Results of treatment in our children confirmed that there were no statistically significant differences in late effects depending on treatment methods.

[Surgical treatment of thyroid gland diseases in childhood--our results]. / Kirursko lijecenje bolesti stitne zlijezde u djecjoj dobi--prikaz nasih bolesnika.

Thyroid gland diseases are the most common endocrinopathies in children. Vast majority of these conditions are treated with medical therapy but in individualised cases surgery is indicated. Decision about surgical treatment is made in cooperation of pediatric endocrinologist and surgeon, treatment options depend on the nature of the disease while the extent of surgical procedure is determined by the pathological changes present in the gland. In this paper we represent the results of surgical treatment of 41 children at our department from 1991 to 2009 and current trends in surgical treatement of thyroid gland diseases.

[Congenital hyperinsulinism--novel insights into etiology, diagnosis and treatment]. / Kongenitalni hiperinzulinizam- -novosti o nastanku, dijagnosticiranju i lijecenju bolesti.

Congenital hyperinsulinism (CHI) is a major cause of persistent hypoglycemia in the neonatal and early infancy periods. Althought the disease is relatively rare with incidence of about 1:25 000-50 000 live births, the importance of the disease should not be underestimated. Namely, prompt recognition and management of patients with CHI is essential, if permanent neurological impairment is to be avoided. CHI is caused by mutations in one of the 7 genes involved in the regulation of insulin secretion in pancreatic beta-cells. It is important to introduce specific medical therapy as soon as diagnosis is established. Severe, neonatal forms of CHI are often resistant to medications, thus they require surgical procedure. The preoperative genetic testing and scintigraphy are indicated to distinguish histological subtypes of the disease (focal vs. diffuse CHI). Patients with focal disease are usually cured after pancreatic resection, while diffuse disease has much worse prognosis. This manuscript offers novel insights into CHI and emphasizes the role of early diagnosis as crucial for succesful treatment that was recently enriched with novel options.

Biliary atresia: the Croatian experience 1992-2006.

The objective of this study was to determine the outcomes of Croatian children with biliary atresia. Health records of infants born in Croatia between January 1, 1992 and December 31, 2006 who were diagnosed with biliary atresia and treated at a single university center were reviewed. Survival rates were calculated with the Kaplan-Meier method. Twenty-nine patients with biliary atresia were identified. Incidence was one in 23,600 live births (95% confidence interval 1/17,400-1/27,200). The median age at Kasai operation was 66 days (range 22-192). Median follow-up was 2.65 years (range 0.2-14.3). Overall survival rates for the patients who underwent Kasai portoenterostomy, including those six (20%) who subsequently underwent liver transplantation, were 75.6%. Five and 10-year native liver survival rates were 51.7% and 38.8%, respectively (median survival time was 7.88 years). Survival rate curves in two groups of patients according to the biliary atresia phenotype (fetal or perinatal form) were divergent. Survival was 87.7% in perinatal form and 43.8% in fetal form (Breslow chi-square 8.082, p < 0.01). Overall survival rates of patients with biliary atresia in Croatia compared unfavorably with current international standards; this could be improved with earlier referral for liver transplantation. Results of Kasai operation (native liver survival rates) compared favorably with results reported elsewhere.

[Results of treatment of displaced supracondylar fractures in children]. / Rezultati lijecenja suprakondilarnih prijeloma humerusa s pomakom ulomaka u djece.

Supracondylar fractures of humerus are the most common fractures in children. The management of severely displaced, unstable fractures of the humerus in children continues to be controversial. We undertook a retrospective study of 48 children with displaced supracondylar fractures (8 were treated with plaster and 40 with cross percutaneous Kirschner wire pinning). Only three children were treated with open reduction and percutaneous cross-pin fixation. Clinical outcome was evaluated by loss of elbow motion and change of carrying angle. According to Flynn s criteria, results were good or excellent in 93.7% patients. The cubitus varus is the most frequent long-term complication (16.7%). Closed reduction with percutaneous pin fixation is believed to represent a safe, reliable, and efficient method of managing displaced supracondylar fractures.

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

Typical 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.

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.

[Ethics and organ transplantation]. / Etika i transplantacija organa.

Organ transplantation becomes an accepted method of treatment, being closely accompanied with the development of transplantation ethics. Numerous problems and dilemmas occurring in this process can be grouped as problems connected with confirming death, and ethic dilemmas connected with obtaining and distributing organs. An attempt to overcome a chronic great lack of cadaver donors is made by various creative attitudes that can be applied only if they fulfill ethical criteria. A difficult question of just organ distribution causes many ethical dilemmas. Solution should be sought in the multidisciplinary approach of bioethics with medicine, politics, legislature and sociology.

Intracardiac shrapnel in a polytraumatized child.

Penetrating cardiac trauma is a life-threatening condition and presents a therapeutic challenge for the surgeon. Additional multiple organ-system injuries, as are common in the setting of war, further complicate the management of such patients. We present the case of a 9-year-old girl who sustained multiple injuries from an unexploded artillery shell, resulting in a retained intracardiac shrapnel. Her cardiac pathology consisted of a shrapnel located in the interventricular septum accompanied by a pneumopericardium and a right-sided hemopneumothorax. The presentation and management of this patient are the subjects of this report.

The value of cholinesterase activity after Kasai operation.

Cholinesterase (ChE) is an enzyme synthesized in the liver. The aim of this study was to determine the value of ChE as an index of liver function. We measured the ChE activity as well as the values of bilirubin, alkaline phosphatase, gamma-glutamyl transpeptidase, aminotransferases and albumin before and 7 days after Kasai operation in 25 infants with biliary atresia. The increased activity of ChE in plasma after Kasai operation was accompanied by a decrease of other measured values ( P<0.0001), except for albumin. We can conclude that the increase of ChE activity together with the decrease of bilirubin, alkaline phosphatase and gamma-glutamyl transpeptidase show early improvement of liver function after Kasai operation. ChE activity can be used to assess liver function in terms of synthesis.

[Diaphragmatic eventration]. / Eventracija osita.

Eventration of the diaphragm is an abnormal elevation of an intact diaphragm. There are two distinct etiologic types of eventration, congenital and acquired. Congenital eventration is characterized by muscular aplasia, and acquired eventration is caused by injury to the phrenic nerve. The one-year-old girl had recurrent respiratory inflammations and permanent cough since she was two months old. Examinations, such as chest radiogram, chest and abdominal ultrasound, liver doppler, computed tomography of thorax and abdomen, did not distinguish eventration from possible tumorous formation in the right lower lung. The eventration of the diaphragm and atelectasis of the right lower lobe was confirmed during the surgery. Diaphragmatic plication was done, and atelectactic lobe as well as hypoplastic part of the diaphragm were removed. The eventration was confirmed pathohystologically.

Choanal stenosis, hypothelia, deafness, recurrent dacryocystitis, neck fistulas, short stature, and microcephaly: report of a case.

We report on a 11-year-old girl with bilateral choanal stenosis, hypothelia, hearing loss, recurrent dacryocystitis, neck fistulas, short stature, and microcephaly. Only three individuals with choanal atresia from a consanguineous family have been reported. One of the patients also had hypoplastic nipples, hypotonia, and delay in speech development. Similar clinical features were seen in two children reported by Greenberg [1987: Am J Med Genet 28:931-934] and Wilson et al. [1998: Am J Med Genet 75:220-222]. They were prenatally exposed to methimazole because of maternal Graves disease. Neck fistulas and microcephaly noted in our patient were not previously reported as features of the syndrome or in the patients prenataly exposed to methimazole. Our patient and those reported by Qazi et al. [1982: Am J Med Genet 13:413-416] most probably have a rare syndrome characterized by this distinctive combination of symptoms. Prenatal exposure to methimazole can cause a phenocopy of the syndrome, which was probably the case in the patients reported by Greenberg and Wilson et al.

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.