Multicenter evaluation of the Paragon CZE 2000 capillary zone electrophoresis system for serum protein electrophoresis and monoclonal component typing.

Serum protein electrophoresis and typing of monoclonal components (MCs) are routine but time-consuming and technically demanding assays. We evaluated capillary electrophoresis (Paragon CZE 2000) for automation of the two assays. CZE and cellulose acetate electrophoresis gave similar data on 794 samples. Within-run and between-run CVs were < 2% for albumin and gamma-globulins and 4-7% for alpha 1-, alpha 2-, and beta-globulins. Bilirubin, hemoglobin, triglycerides, and fibrinogen were found not to interfere. No carryover by capillaries was detected. The detection limit for MC was < 0.5 g/L. MC assessment by immunosubtraction on 403 samples identified the monoclonal type in all samples with peak concentrations > 10 g/L; only 50% of MCs that could not be quantified by densitometric scan were typed.

Accurate placement of central venous catheters in pediatric patients using endocavitary electrocardiography: reassessment of a personal technique.

To avoid the need for radiological control in the assessment of the proper location of central venous catheters (CVC), a particular use of endocavitary electrocardiography (EC-ECG) was proposed 10 years ago. The aim of this study is to reassess our experience with this method. EC-ECG assumes that the CVC, when filled with normosaline and connected to a standard electrocardiograph, behaves like an exploring electrode. The approach of the catheter tip to the right atrium is then detected by a slightly increasing negative P wave. When the tip reaches the exact level of the sinus node, the P wave suddenly deepens. After a preliminary test of the reliability of the technique versus the standard method in 50 CVC placements verified by both EC-ECG and chest x-ray, we have placed 807 CVCs in children using EC-ECG only. There have been no false-positive and one false-negative test result (lead connector misplacement). In 17 cases in which intrinsic deflection was not detected, the catheter tip was found to be wrongly positioned; all the remainder CVCs have been successfully positioned. For 10 years this technique has proved to be a simple, safe, quick, inexpensive and highly reliable method to assess the correct positioning of the CVC.

Fortuitous diagnosis of cystic fibrosis at laparotomy for acute appendicitis.

Two children aged 11 and 9 years were operated upon for acute appendicitis and were discovered to have cystic fibrosis: the histologic pattern of the removed appendix was typical for this disorder. Appendicitis may thus be the first presenting sign of occult cystic fibrosis.

[A follow-up study in individuals subjected to ileo-cecal resection in infancy and childhood]. / Controlli a distanza di individui sottoposti in età pediatrica a resezioni intestinali interessanti anche la valvola ileo-cecale.

It is stated that the ileocecal valve delays the passage of ileal contents into the cecum and acts as a barrier against reflux and ascension of colonic bacterial flora into the small bowel: its resection may lead to bacterial colonization of the ileum and to abnormalities of intestinal motility, transit and absorption. In this study twenty individuals subjected in pediatric age (1 day to 11 years) to ileocecal resection have been evaluated from 2 to 19 years after surgery. Three patients underwent limited ileocecal resection, in four this was associated with a significant ileal resection, in five with extensive right colon resection and in eight with extensive ileal and right colon resection. Growth, stool habit, hematology and serum biochemistry were examined; all patients also underwent abdominal ultrasonography. In all body weight and height were within normal limits; seven had moderate diarrhea up to 18 months after surgery and two who required extensive intestinal resection (40 and 30 cm of small bowel left) had diarrhea until about 36 months after surgery: now all of them have daily fecal evacuation. Hematological, biochemical, urinary and fecal studies proved normal except in one treated with TPN who presented transaminases slightly increased and in three suffering from mucoviscidosis in whom steatorrhea with moderate alterations of fats and elevation of alkaline phosphatase and transaminases were present. Urinary and gall stones were not seen in anyone. In conclusion from this study it can be postulated that removal of ileocecal valve can be done safely in children.

[Follow-up study of pulmonary function in individuals subjected to lobectomy in infancy and childhood]. / Valutazione a distanza della funzionalità respiratoria in individui sottoposti a lobectomia polmonare in età pediatrica.

Experimental research in animals have demonstrated that pulmonary development continues after birth. This happens in man as well: alveolar proliferation and enlargement go on until the eighth year of age, furthermore complete pulmonary development is achieved until the 20-22 years of age. It is therefore possible to hypothesise that respiratory deficit following pulmonary exeresis in children would regress with time. In the Pediatric Surgery of IRCCS Policlinico S. Matteo of Pavia eight children, 4 days to 8 years of age, underwent lobectomy for pulmonary hypertensive emphysema (3), lung abscess (2), bronchogenic cysts (2) and hystiocytoma (1). One of them was lost to follow-up; the remainders were controlled from 3 to 25 years after surgery: all of them enjoy a normal active life. Respiratory function was evaluated through blood chemistry, blood gas analysis, basal and after strain ECG, chest X-ray, spirometry, basal and after strain pulmonary scintigraphy. Basal and after strain ECG, chest X-ray, and arterial blood gas were normal in every examined subjects; spirometry, although results are quite variable, did not show serious respiratory deficits; basal after strain pulmonary scintigraphies weren't homogeneous. These results, although they aren't univocally interpretable, positively confirmed that the residual lung after lobectomy has a satisfactory possibility of functional recovery.

Surgical treatment of necrotizing enterocolitis: when? how?

In 10 years (1981-1990) 28 out of 54 neonates (51.8%) with definite necrotizing enterocolitis (NEC) underwent surgery. Operation was performed at 13.5 +/- 8.8 (range 3-38) days of life, after 1.7 +/- 1.5 (range 1-6) days from the onset of symptoms. Aiming to perform laparotomy before the occurrence of perforation, surgery was liberally indicated in stage IIIa, according to Walsh-Kliegman. Explorative laparotomy (+peritoneal drainage in 2 cases) was performed in 4 patients with massive intestinal necrosis: all died within 3 days of surgery. In one neonate, only pneumatosis was present and resection was not considered mandatory. Intestinal resection and enterostomy was performed in 17 neonates, 5 of them with perforation; three developed an intestinal stenosis. Enterostomy was closed after 116.2 +/- 61.8 days (range 26-193); 11 patients (64.7%) are long-term survivors. Intestinal resection and primary anastomosis was performed in 6 babies, 3 of them with perforation. Postoperatively, 2 dehiscences and 1 stenosis were recorded, but all children survived. In our opinion, resection followed by primary anastomosis seems to be the most satisfactory surgical option.

Conservative surgery of bladder-prostate rhabdomyosarcoma in children: results after long-term follow-up.

Between 1968 and 1982, 10 children (7 boys and 3 girls) with a mean age of 3.2 years affected by bladder (8 cases) and prostate (2 cases) rhabdomyosarcoma (RMS)--all histologically embryonal--were seen. Three (1 with bone metastases at diagnosis and 2 with stage III tumor) underwent radical surgery; all died because of progression of the disease. Seven children were treated with conservative surgery: 3 solid type bladder tumors were removed together with a narrow strip of apparently healthy bladder wall, sparing 30% to 70% of the bladder; 3 botryoid type bladder tumors were removed bluntly and implantation sites fulgurated; in 1 prostate tumor osmotic cytolysis of the neoplastic mass was achieved with multiple injections of hypertonic saline solution (NaCl 30%). Surgery was followed by chemotherapy with a vincristine actinomycin D and cyclophosphamide protocol and radiotherapy (20 to 39.5 Gy). Six of the seven are alive and disease free with a normal functional bladder after 10 to 21 years. The validity of a primary conservative surgical approach with preservation of the bladder, followed by adjuvant therapy, is emphasized.

[Intestinal atresia: what is changed in the last 30 years]. / L'atresia dell'intestino: cosa è cambiato negli ultimi 30 anni.

The changes occurred in the management of intestinal atresia in the last 30 years are presented. On the basis of a series of 55 neonates (29 M/26 F; 14 premature; mean body weight 2640 g, range 1340-4100 g; 23 duodenal, 30 small bowel, 2 colon) four aspects of this malformation are discussed; epidemiology, diagnostics, therapy and prognosis. Epidemiology. 40% incidence reduction is detected; unchanged the relative incidence of Down neonates with duodenal atresia. Diagnostics. Prenatal US scan useful in 4 of 8 cases only; emphasized the usefulness of plain X-ray abdomen. Perioperative treatment. Dramatic impact of the artificial nutrition on the management of short bowel syndrome, with 7 cases (residual small bowel from 75 to 9 cm, mean 40 cm) successfully treated. Surgical technique. Confirmed the validity of the one-stage termino-terminal anastomosis. Prognosis. Significant improvement in the last three decades respectively with 50%, 87% and 93% of long-term survivors. In the first decade 6 over 8 deaths were related to the atresia, while in the last 10 years the only death recorded was related to an associated severe cardiac malformation.

HIV, HBV, delta-agent and Treponema pallidum infections in two rural African areas.

In order to compare the seroepidemiology of human immunodeficiency virus (HIV), hepatitis B virus, delta agent and Treponema pallidum infections in two rural populations living in north Uganda (Kitgum district) and in central Burundi (Butezi, Ruyigi region), 448 sera were tested for HBS-Ag, HBS-Ab, and anti-HIV antibodies and screened for syphilis using the T. pallidum haemagglutination (TPHA) test. HBS-Ag positive sera were also tested for anti-delta antibodies. Overall seropositivity rates in healthy subjects, outpatients and inpatients (non-AIDS) were 14.2% and 9.5% in Kitgum district and Butezi, respectively. The prevalence of HBS-Ag and HBS-Ab ranged from 10.0% to 15.6% and from 66.2% to 68.9%, respectively. In north Uganda the rates of anti-delta positivity were 3.1% in the overall population and 30.6% in the HBS-Ag positive subjects. No serum obtained in Butezi was anti-delta positive. In Ugandan people, 64.0% of anti-HIV positive and 25.8% of anti-HIV negative patients were also TPHA-positive (P less than 0.01). For Butezi the corresponding figures were 21.4% and 1.6% respectively (P less than 0.04). On the contrary, no correlation was found between either anti-HIV or TPHA positives and seropositivity for B and delta hepatitis serological markers. The study demonstrated an association between seropositivities for HIV and T. pallidum (TPHA), suggesting common patterns of transmission. On the contrary, no association seemed to exist between HBV and HIV infections.

PIP: In 1986, health workers collected 358 serum samples from 134 people living in rural Kitgum district in north Uganda and, in 1987, 90 serum samples from 30 healthy people in the Butezi and Ruyigi regions of central Burundi to compare the seroepidemiology of HIV, hepatitis B virus (HBV), delta agent hepatitis, and Treponema pallidum infections. Laboratory staff used ELISA to test for HIV and confirmed all positive samples with the Western blot test. The radioimmunoassay (RIA) test was used for HBV infection and for delta agent hepatitis infection. T. pallidum hemagglutination (TPHA) test was used to check for past syphilis infection. 10% of the people in Kitgum district tested positive for HBV surface antigens (HBS-Ag) and 66.2% for HBV antibodies (HBS-Ab) compared to 15.6% and 68.9% for those in Butezi. Further 3.1% of all people tested in northern Uganda tested positive for anti delta agent hepatitis. Moreover 30.6% of the people in northern Uganda who tested positive for HBS-Ag also tested positive for anti delta agent hepatitis. Yet no one in Butezi district who tested positive for anti delta agent hepatitis. In healthy patients, 14.2% of those in Kitgum district and 9.5% of those in Butezi district tested positive for HIV. In clinically suspected AIDS patients, these corresponding figures were 84.2% and 74.1%. 32.7% of the people in northern Uganda and 7.85 of those in Burundi tested positive for T. pallidum. Further 64% of all HIV positive cases also tested positive for TPHA compared to 25.8% of HIV negative cases (p.01). In Butezi, these figures were 21.4% and 1.6% (p.04). As for those with clinical features of AIDS and tested positive for HIV, 62.5% also tested positive for TPHA in Uganda and 25% in Burundi. In conclusion, HIV infection was strongly associated with previously preexisting patterns of sexually transmitted diseases, i.e., TPHA, but not with the HBV mode of transmission.

[Cystic lymphangioma of the greater omentum appearing as an inguinal hernia in an 18-month-old child]. / Lymphangiome kystique du grand épiploon se présentant comme une hernie inguinale chez un enfant de 18 mois.

Cystic lymphangioma of greater omentum is an exceptional pathology: a world literature review in 1978 reported on only such cases. Preoperative diagnosis is actually impossible, due to the lack of pathognomonic symptoms or signs. A child 18-months-old was recently operated upon with diagnosis of hydrocele: surprisingly, at surgery we found an inguinal hernia containing a cystic lymphangioma of the greater omentum, histologically proved. A partial resection of the cystic omentum was successfully performed. An obstruction of the omental lymphatic drainage is the most reliable pathogenetic hypothesis.

Partial nephrectomy for Wilms tumor.

We report a case of a left Wilms tumor that was treated successfully by partial nephrectomy. The advantages and disadvantages of this approach are discussed.

Anuria due to bilateral cystine urolithiasis in an infant.

A 10-month-old female presented with anuria due to bilateral cystine urolithiasis. Although bilateral obstruction of the ureters by calculi is very rare in children, it may be the presenting feature of cystinuria even in the first year of life.