[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.

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.

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.

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.