Cholangiocyte apoptosis is an early event during induced metamorphosis in the sea lamprey, Petromyzon marinus L.

BACKGROUND: Research in biliary atresia has been hindered by lack of a suitable animal model. Lampreys are primitive vertebrates with distinct larval and adult life cycle stages. During metamorphosis the biliary system of the larval lamprey disappears. Lamprey metamorphosis has been proposed as a model for biliary atresia. We have begun to explore cellular events during lamprey metamorphosis by assessing for cholangiocyte apoptosis. MATERIALS AND METHODS: Sea lamprey larvae were housed under controlled environmental conditions. Premetamorphic larvae were induced to undergo metamorphosis by exposure to 0.01% KClO(4). Animals were photographed weekly, and the stage of metamorphosis was assigned based upon external features. Livers were harvested and processed for routine histology and immunohistochemistry. DNA fragmentation was detected using deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling (TUNEL) assays and cholangiocytes were identified with antibodies to cytokeratin-19. Percent TUNEL+ cholangiocytes at different stages of metamorphosis was determined. RESULTS: The percentage of TUNEL+ cholangiocytes was 10% in premetamorphic (stage 0) lamprey (n = 6), 51% at stage 1 (n = 6), 40% at stage 2 (n = 5), 18% at stage 3 (n = 5), and 9% stage 4 (n = 4). Routine hemotoxylin and eosin stained paraffin-embedded tissue sections revealed frequent apoptotic bodies at stages 3 and 4 of metamorphosis without histologic evidence of necrosis. CONCLUSIONS: DNA fragmentation is identified at the earliest stages of metamorphosis during induced metamorphosis in lampreys. Additional studies are necessary to validate this potentially valuable animal model.

Partial splenectomy prevents splenic sequestration crises in sickle cell disease.

PURPOSE: Acute splenic sequestrations (SSs) are potentially fatal complications in sickle cell disease (SCD). Total splenectomies in young patients may predispose them to a higher risk of overwhelming infections, whereas partial splenectomy may maintain immunocompetence. We present our series of partial splenectomies in patients with multiple SS episodes. METHODS: We retrospectively reviewed the records of 6 patients who underwent open partial splenectomies for SS. Data on their clinical courses were collected and analyzed. RESULTS: None of the 6 patients had SS postprocedure, down from 2.1 +/- 1.0 (P = .003) sequestrations per year and 3.5 +/- 1.4 (P = .002) total sequestrations per patient. The transfusion requirements were significantly reduced postoperatively (10.2 +/- 5.6 vs 2.0 +/- 3.1 per year; P = .002). There was no increase in the infection-related hospital admissions during the period of follow-up (1.5 +/- 1.8 vs 0.8 +/- 0.8 per year after partial splenectomy; P = .363). The upper pole was preserved in all cases with blood supply off the main splenic artery. CONCLUSIONS: Partial splenectomy decreases the risk of SS in SCD and reduces the need for blood transfusions. Infection rates did not increase after the procedure during the follow-up period. Partial splenectomy should be considered for patients who experience multiple acute SS crises or have long-term transfusion requirements.

Management and outcomes of ovarian masses in children and adolescents.

Ovarian masses in the pediatric age group are rare, and malignancies are even less common. We reviewed our large single-center experience to determine the rate of malignancy and discuss management. We retrospectively reviewed the cases of ovarian masses in children in our institution over a 10-year period. Demographic and tumor-specific data were reviewed and analyzed, and a Student's unpaired t test was used where appropriate. A total of 49 children and adolescents with ovarian masses were found. The mean age at presentation was 13.3 years. Eight masses were malignant (16%) with malignant teratoma, dysgerminoma, and germ cell tumors found. These patients responded to chemotherapy, but there were three recurrences noted that responded to further therapy. Seventy-four per cent of the benign tumors were teratomas. The most common presentation was abdominal pain in 27 patients (55%) followed by an abdominal mass. Ultrasound and CT scans were the most common imaging studies with a mean mass size of 14.7 cm. A majority of the patients underwent a laparotomy with 12 per cent having a minimally invasive procedure. Only 37 per cent of the operations were performed by the pediatric surgeons. There were no deaths in this series after a follow up of over 6 years. Most ovarian masses in childhood are benign. Malignant lesions have favorable outcomes with chemotherapy, even with recurrent disease. Consideration for laparoscopic procedures should be given for the benign lesions.

Gastric electrical stimulation for children with intractable nausea and gastroparesis.

PURPOSE: Gastric electrical stimulation (GES) has been performed in adults as a treatment of refractory nausea and vomiting in patients who have failed medical treatment, but has not been used in children. METHODS: Nine patients with chronic nausea and vomiting with a mean age of 14 years were evaluated for temporary GES. All 9 patients subsequently underwent placement of a temporary followed by permanent GES device. Symptoms were recorded at baseline, after temporary GES, and then after permanent GES using a Likert scale for gastroparesis. Statistical analysis was performed using a paired Student's t test. RESULTS: At baseline, all patients were symptomatic and most had delayed solid gastric emptying. As a group, there was a significant improvement in combined symptoms score (P = .04), nausea (P = .039), and vomiting (P = .0016). Gastric emptying and electrogastrogram values did not change significantly. Follow-up ranged from 8 to 42 months, with 7 of the 9 patients reporting sustained improvement in symptoms and improved quality of life. CONCLUSIONS: Gastric electrical stimulation can be successfully applied to adolescents with intractable nausea and gastroparesis symptoms who fail medical therapy. There is a significant improvement in symptoms over a prolonged period, and there are no adverse effects of the GES. Long-term efficacy of this therapy in children needs to be established.

Primary laparoscopic repair of high imperforate anus in neonatal males.

PURPOSE: The standard approach to males with high imperforate anus has been a staged procedure starting with a descending colostomy, then posterior sagittal anorectoplasty with colostomy closure after 3 months. Recently, a minimally invasive approach to the repair of high imperforate anus has been described in infants after colostomy. We describe 6 newborn males with high imperforate anus successfully repaired laparoscopically as a primary, single-stage procedure. METHODS: A retrospective chart review was performed on all patients with imperforate anus from October 2003 to October 2006. RESULTS: We evaluated 9 newborn males with high imperforate anus. Of these patients, 6 underwent primary laparoscopic repair on day 1 to day 2 of life. Of these 6 patients, 3 were found to have bladder neck fistulas, whereas the other 3 had prostatic urethra fistulas. All patients passed stool within the first 72 hours postoperatively. One patient has required a procedure for a mild rectal prolapse. Follow-up ranges from 2 to 30 months in the single-stage group. CONCLUSION: Our early results using primary laparoscopic repair appear encouraging. Laparoscopy allows excellent visualization and assessment of the fistula and repair of high imperforate anus without need for colostomy. Long-term follow-up will be needed to assess outcomes and continence rates.

Congenital pancreatic cyst arising from occlusion of the pancreatic duct.

Congenital pancreatic cysts, particularly solitary cysts, are rare causes of abdominal masses in newborns. The etiology of these cysts is unknown and they can vary in location within the pancreas. We present a novel case of an infant with a solitary congenital cyst of the pancreatic duct, itself, as a result of occlusion of the pancreatic duct. Solitary congenital pancreatic cysts are a rare cause of abdominal masses in infants. We recently evaluated a 7-week-old infant with progressive abdominal distension since birth. This child was found to have a large solitary pancreatic cyst. This cyst resulted from occlusion of the main pancreatic duct at the level of the ampulla resulting in cystic degeneration of the pancreatic duct. Based on our review of the literature we believe this is the first documented case of such a cyst.

Acute, subacute, and chronic cervical lymphadenitis in children.

Lymphadenopathy refers to any disease process involving lymph nodes that are abnormal in size and consistency. Lymphadenitis specifically refers to lymphadenopathies that are caused by inflammatory processes. Cervical lymphadenopathy is a common problem in the pediatric age group and is largely inflammatory and infectious in etiology. Although most patients are treated successfully by their primary care physician, surgical consultation is frequently required for patients who fail to respond to initial therapy or for those in whom there is an index of suspicion for a neoplastic process. This article addresses current approaches to the diagnosis and management of cervical lymphadenitis in children.

Nitrovasodilator responses in pulmonary arterioles from rats with nitrofen-induced congenital diaphragmatic hernia.

BACKGROUND/PURPOSE: Many infants with congenital diaphragmatic hernias (CDHs) experience persistent pulmonary hypertension that is refractory to treatment with inhaled nitric oxide (NO). We have examined the responses of isolated pulmonary arterioles from prenatal and postnatal rats with and without nitrofen (2,4-dichlorophenyl-p-nitrophenyl ether)-induced CDH to a variety of activators of the NO-cyclic guanosine monophosphate (cGMP) pathway. METHODS: Right-sided CDH was induced in fetal rats by feeding nitrofen to pregnant rats on day 12 of gestation. Control rats were fed olive oil (vehicle). Third-generation pulmonary arterioles were isolated from the right lung of prenatal rats at term and from newborn rats within 8 hours after birth. Responses to increasing concentrations of sodium nitroprusside (SNP), atrial natriuretic peptide, or 8-bromo-cGMP were measured in pulmonary arterioles from control rats and from rats with nitrofen-induced CDH. Postnatal responses to 8-bromo-cGMP were also recorded in the presence of zaprinast, a type V phosphodiesterase inhibitor. RESULTS: Pulmonary arterioles from prenatal rats did not dilate in response to SNP, atrial natriuretic peptide, or 8-bromo-cGMP. Vasodilatory responses of postnatal pulmonary arterioles from control rats to SNP and 8-bromo-cGMP were significantly greater than for arterioles from rats with CDH. Zaprinast pretreatment resulted in similar responses for postnatal CDH and control arterioles to 8-bromo-cGMP. CONCLUSIONS: Postnatal pulmonary arterioles from CDH rats exhibit altered nitrovasodilator responsiveness, which may be due to rapid degradation of cGMP.

Congenital diaphragmatic hernia: searching for answers.

BACKGROUND: Pulmonary hypoplasia and hypertension are the primary causes of morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). At present, the origin of CDH and the causes of pulmonary hypoplasia and hypertension are unknown. DATA SOURCES: This article reviews the available published data regarding the origin of CDH and the pathogenesis of the associated pulmonary hypertension and hypoplasia. These investigations have employed human tissues as well as two types of CDH animal models. CONCLUSIONS: Investigations performed to date have not yet provided definitive answers regarding the pathogenesis of CDH. However, they have yielded many new and exciting discoveries and several opportunities for intervention. Ongoing research should open new possibilities to improve the outcome for these unfortunate babies with CDH.