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Hitler's Elite Guard at War, — Operation is both for treatment and staging to determine further therapy. The direct hernia protrudes through the posterior wall of the inguinal canal, i. The cause for sub-ileus is a raised tone in the residual sphincter. In malrotation the right colon can create peritoneal attachments that include and obstruct the third portion of the duodenum Ladd's bands.

Cardiac tamponade due to protrusion into the pericardial cavity has been reported. US and CT-Scan can demonstrate the defect. Trans-abdominal subcostal approach is preferred with reduction of the defect and suturing of the diaphragm to undersurface of sternum and posterior rectus sheath.

Large defects with phrenic nerve displacement may need a thoracic approach. Results after surgery rely on associated conditions. Two types of esophageal hernia recognized are the hiatal and paraesophageal hernia. Diagnosis is made radiologically always and in a number of patients endoscopically. The hiatal hernia HH refers to herniation of the stomach to the chest through the esophageal hiatus.

The lower esophageal sphincter also moves. It can consist of a small transitory epiphrenic loculation minor up to an upside-down intrathoracic stomach major. HH generally develops due to a congenital, traumatic or iatrogenic factor. Most disappear by the age of two years, but all forms of HH can lead to peptic esophagitis from Gastroesophageal reflux. Repair of HH is determined by the pathology of its associated reflux causing failure to thrive, esophagitis, stricture, respiratory symptoms or the presence of the stomach in the thoracic cavity.

In the paraesophageal hernia PH variety the stomach migrates to the chest and the lower esophageal sphincter stays in its normal anatomic position. PH is a frequent problem after antireflux operations in patients without posterior crural repair. Small PH can be observed. With an increase in size or appearance of symptoms reflux, gastric obstruction, bleeding, infarction or perforation the PH should be repaired. The incidence of PH has increased with the advent of the laparoscopic fundoplication.

A hernia is defined as a protrusion of a portion of an organ or tissue through an abnormal opening. For groin inguinal or femoral hernias, this protrusion is into a hernial sac. Whether or not the mere presence of a hernial sac or processus vaginalis constitutes a hernia is debated. Inguinal hernias in children are almost exclusively indirect type.

Those rare instances of direct inguinal hernia are caused by previous surgery and floor disruption. An indirect inguinal hernia protrudes through the internal inguinal ring, within the cremaster fascia, extending down the spermatic cord for varying distances. The direct hernia protrudes through the posterior wall of the inguinal canal, i. The embryology of indirect inguinal hernia is as follows: During the third month of gestation, the processus vaginalis extends down toward the scrotum and follows the chorda gubernaculum that extends from the testicle or the retroperitoneum to the scrotum.

During the seventh month, the testicle descend into the scrotum, where the processus vaginalis forms a covering for the testicle and the serous sac in which it resides. At about the time of birth, the portion of the processus vaginalis between the testicle and the abdominal cavity obliterates, leaving a peritoneal cavity separate from the tunica vaginalis that surrounds the testicle.

The typical patient with an inguinal hernia has an intermittent lump or bulge in the groin, scrotum, or labia noted at times of increased intra-abdominal pressure. A communicating hydrocele is always associated with a hernia. This hydrocele fluctuates in size and is usually larger in ambulatory patients at the end of the day.

If a loop of bowel becomes entrapped incarcerated in a hernia, the patient develops pain followed by signs of intestinal obstruction. If not reduced, compromised blood supply strangulation leads to perforation and peritonitis. Most incarcerated hernias in children can be reduced. Associated to these episodes of incarceration are chances of: Symptomatic hernia can complicate the clinical course of babies at NICU ill with hyaline membrane, sepsis, NEC and other conditions needing ventilatory support.

Repair should be undertaken before hospital discharge to avoid complications. Postconceptual age sum of intra- and extrauterine life has been cited as the factor having greatest impact on post-op complications. These observation makes imperative that preemies with post conceptual age of less than 45 weeks be carefully monitored in-hospital for at least 24 hours after surgical repair of their hernias. Outpatient repair is safer for those prematures above the 60 wk.

The very low birth weight infant with symptomatic hernia can benefit from epidural anesthesia. At times, the indirect inguinal hernia will extend into the scrotum and can be reduced by external, gentle pressure. Occasionally, the hernia will present as a bulge in the soft tissue overlying the internal ring.

It is sometimes difficult to demonstrate and the physician must rely on the patient's history of an intermittent bulge in the groin seen with crying, coughing or straining.

Elective herniorrhaphy at a near convenient time is treatment of choice. Since risk of incarceration is high in children, repair should be undertaken shortly after diagnosis. Simple high ligation of the sac is all that is required.

Pediatric patients are allowed to return to full activity immediately after hernia repair. Bilateral exploration is done routinely by most experienced pediatric surgeons. Recently the use of groin laparoscopy through the hernial sac permits visualization of the contralateral side. Testicular feminization syndrome TFS is a genetic form of male pseudohermaphroditism patient who is genetically 46 XY but has deficient masculinization of external genitalia caused by complete or partial resistance of end organs to the peripheral effects of androgens.

This androgenic insensitivity is caused by a mutation of the gene for androgenic receptor inherited as an X-linked recessive trait.

In the complete form the external genitalia appear to be female with a rudimentary vagina, absent uterus and ovaries. The incomplete form may represent undervirilized infertile men. This patients will never menstruate or bear children. Early gonadectomy is advised to: Vaginal reconstruction is planned when the patient wishes to be sexually active. These children develop into very normal appearing females that are sterile since no female organs are present. A hydrocele is a collection of fluid in the space surrounding the testicle between the layers of the tunica vaginalis.

Hydroceles can be scrotal, of the cord, abdominal, or a combination of the above. A hydrocele of the cord is the fluid-filled remnant of the processus vaginalis separated from the tunica vaginalis. A communicating hydrocele is one that communicates with the peritoneal cavity by way of a narrow opening into a hernial sac. Hydroceles are common in infants. Some are associated with an inguinal hernia. They are often bilateral, and like hernias, are more common on the right than the left.

Most hydroceles will resolved spontaneously by years of age. After this time, elective repair can be performed at any time. Operation is done through the groin and search made for an associated hernia. Aspiration of a hydrocele should never be attempted. As a therapeutic measure it is ineffective, and as a diagnostic tool it is a catastrophe if a loop of bowel is entrapped. A possible exception to this is the postoperative recurrent hydrocele.

The undescended testis is a term we use to describe all instances in which the testis cannot be manually manipulated into the scrotum. The testes form from the medial portion of the urogenital ridge extending from the diaphragm into the pelvis. In arrested descent, they may be found from the kidneys to the internal inguinal ring.

Rapid descent through the internal inguinal ring commences at approximately week 28, the left testis preceding the right. Adequate amounts of male hormones are necessary for descent. The highest levels of male hormones in the maternal circulation have been demonstrated at week Thus, it appears that failure of descent may be related to inadequate male hormone levels or to failure of the end-organ to respond.

The undescended testes may be found from the hilum of the kidney to the external inguinal ring. The undescended testis found in 0. Testes that can be manually brought to the scrotum are retractile and need no further treatment. Parents should know the objectives, indications and limitations of an orchiopexy: To improve spermatogenesis producing an adequate number of spermatozoids surgery should be done before the age of two.

Electron microscopy has confirmed an arrest in spermatogenesis reduced number of spermatogonias and tubular diameter in undescended testis after the first two years of life. Other reasons to pex are: The management is surgical; hormonal Human Chorionic Gonadotropin treatment has brought conflicting results except bilateral cases.

Surgery is limited by the length of the testicular artery. Palpable testes have a better prognosis than non-palpable. Laparoscopy can be of help in non-palpable testis avoiding exploration of the absent testis. Viens, MS University of Toronto. An umbilical hernia is a small defect in the abdominal fascial wall in which fluid or abdominal contents protrude through the umbilical ring.

The presence of a bulge within the umbilicus is readily palpable and becomes more apparent when the infant cries or during defecation. The actual size of the umbilical hernia is measured by physical examination of the defect in the rectus abdominis muscle, and not by the size of the umbilical bulge.

The size of the fascial defect can vary from the width of a fingertip to several centimetres. Embryologically, the cause of an umbilical hernia is related to the incomplete contraction of the umbilical ring. The herniation of the umbilicus is a result of the growing alimentary tract that is unable to fit within the abdominal cavity. Umbilical hernias are more prevalent in females than in males and are more often seen in patients with African heritage.

The increased frequency of umbilical hernias has also been attributed to premature babies, twins and infants with long umbilical cords. There is also a frequent association with disorders of mucopolysaccharide metabolism, especially Hurler's Syndrome gargoylism. Most umbilical hernias are asymptomatic; the decision to repair the umbilical hernia in the first years of life is largely cosmetic and is often performed because of parental request, not because of pain or dysfunction.

In the past, some parents use to tape a coin over the umbilical bulge, however, manual compression does not have an effect on the fascial defect. Treatment of umbilical hernia is observation.

However, surgical repair is recommended if the hernia has not closed by the age of five. The incidence of incarceration trapped intestinal loop is rare, even in larger defects. Females should especially have their umbilical hernia corrected before pregnancy because of the associated increased intra-abdominal pressure that could lead to complications.

The procedure is simple and incidence of complication such as infection is extremely rare. The repair is usually done as outpatient surgery under general anesthetic. Inguinal and umbilical hernia repair in infants and children. Surg Clinics of North Am 73 3: Swenson's Pediatric Surgery - 5th edition. The developing human - 4th edition. Philadelphia, WB Saunders, pp. Some observations on umbilical hernias in infants.

The comparative incidence of umbilical hernias in colored and white infants. J Natl Med Assoc The three most common abdominal wall defect in newborns are umbilical hernia, gastroschisis and omphalocele. Omphalocele is a milder form of primary abdominoschisis since during the embryonic folding process the outgrowth at the umbilical ring is insufficient shortage in apoptotic cell death. Defect may have liver, spleen, stomach, and bowel in the sac while the abdominal cavity remains underdeveloped in size.

The sac is composed of chorium, Wharton's jelly and peritoneum. The defect is centrally localized and measures cm in diameter. A small defect of less than 2 cm with bowel inside is referred as a hernia of the umbilical cord.

Epigastric localized omphalocele are associated with sternal and intracardiac defects i. Cardiac, neurogenic, genitourinary, skeletal and chromosomal changes and syndromes are the cornerstones of mortality.

Cesarean section is warranted in large omphaloceles to avoid liver damage and dystocia. After initial stabilization management requires consideration of the size of defect, prematurity and associated anomalies.

Primary closure with correction of the malrotation should be attempted whenever possible. Antibiotics and nutritional support are mandatory. Manage control centers around sepsis, respiratory status, liver and bowel dysfunction from increased intraabdominal pressure. The protruding gut is foreshortened, matted, thickened and covered with a peel. The IA might be the result of pressure on the bowel from the edge of the defect pinching effect or an intrauterine vascular accident.

Rarely, the orifice may be extremely narrow leading to gangrene or complete midgut atresia. In either case the morbidity and mortality of the child is duplicated with the presence of an IA. Alternatives depend on the type of closure of the abdominal defect and the severity of the affected bowel. With primary fascial closure and good-looking bowel primary anastomosis is justified. Angry looking dilated bowel prompts for proximal diversion, but the higher the enterostomy the greater the problems of fluid losses, electrolyte imbalances, skin excoriation, sepsis and malnutrition.

Closure of the defect and resection with anastomosis two to four weeks later brings good results. Success or failure is related to the length of remaining bowel more than the specific method used.

Initially do an Apt test to determine if blood comes from fetal origin or maternal origin blood swallowed by the fetus. If this coagulation profile is normal the possibilities are either stress gastritis or ulcer disease. If the coagulation profile is abnormal then consider hematologic disease of the newborn and manage with vitamin K.

The apt test is performed by mixing 1 part of vomitus with 5 part H2O, centrifuge the mixture and remove 5 ml pink. If the coagulation profile is abnormal give Vit K for hematologic disorder of newborn. If it's normal do a rectal exam. A fissure could be the cause, if negative then consider either malrotation or Necrotizing enterocolitis.

The stress includes prematurity, sepsis, hypoxia, hypothermia, and jaundice. These babies frequently have umbilical artery, vein catheters, have received exchange transfusions or early feeds with hyperosmolar formulas.

The intestinal mucosal cells are highly sensitive to ischemia and mucosal damage leads to bacterial invasion of the intestinal wall. Gas-forming organisms produce pneumatosis intestinalis air in the bowel wall readily seen on abdominal films.

Full-thickness necrosis leads to perforation, free air and abscess formation. These usually premature infants develop increased gastric residuals, abdominal distension, bloody stools, acidosis and dropping platelet count.

The abdominal wall becomes reddened and edematous. There may be persistent masses and signs of peritonitis. Perforation leads to further hypoxia, acidosis and temperature instability.

The acid-base status is monitored for worsening acidosis and hypoxia. The white blood cell count may be high, low or normal and is not generally of help.

Serial abdominal films are obtained to look for evidence of free abdominal air, a worsening picture of pneumatosis intestinalis, or free portal air. Therapy consist initially of stopping feeds, instituting nasogastric suctioning and beginning broad-spectrum antibiotics ampicillin and gentamycin.

Persistent or worsening clinical condition and sepsis or free air on abdominal films require urgent surgical intervention. Attempts to preserve as much viable bowel as possible are mandatory to prevent resultant short gut syndrome.

Complicated NEC is the most common neonatal surgical emergency of modern times, has diverse etiologies, significant mortality and affects mostly premature babies. Consist of a right lower quadrant incision and placement of a drainage penrose or catheter under local anesthesia with subsequent irrigation performed bedside at the NICU.

Initially used as a temporizing measure before formal laparotomy, some patient went to improvement without the need for further surgery almost one-third. They either had an immature fetal type healing process or a focal perforation not associated to NEC?

Some suggestion made are: PPD should be an adjunct to preop stabilization, before placing drain be sure pt has NEC by X-rays, persistent metabolic acidosis means uncontrolled peritoneal sepsis, do not place drain in pts with inflammatory mass or rapid development of intraperitoneal fluid, the longer the drainage the higher the need for laparotomy.

In the initial evaluation a history should be obtained for bleeding disorders, skin lesions, and aspirin or steroid ingestion. The physical exam for presence of enlarged liver, spleen, masses, ascites, or evidence of trauma or portal hypertension. Labs such as bleeding studies and endoscopy, contrast studies if bleeding stops. Common causes of Upper GI bleeding are: Esophagus a Varices- usually presents as severe upper gastrointestinal bleeding in a year old who has previously been healthy except for problems in the neonatal period.

This is a result of extrahepatic portal obstruction portal vein thrombosis most commonly , with resulting varices. The bleeding may occur after a period of upper respiratory symptoms and coughing. Management is initially conservative with sedation and bedrest; surgery ir rarely needed.

Treatment consist of antacids, frequent small feeds, occasionally medications and if not rapidly improved, then surgical intervention with a fundoplication of the stomach. This was thought to be uncommon in children because it was not looked for by endoscopy.

It probably occurs more often than previously thought. Treatment initially is conservative and, if persistent, oversewing of the tear through an incision in the stomach will be successful.

They bleed when there is ectopic gastric mucosa present. Total excision is curative. Occasionally requires surgical intervention with local repair or ligation of hepatic vessels. Anal fissure is the most common cause of rectal bleeding in the first two years of life.

Outstretching of the anal mucocutaneous junction caused by passage of large hard stools during defecation produces a superficial tear of the mucosa in the posterior midline. Pain with the next bowel movement leads to constipation, hardened stools that continue to produce cyclic problems.

Large fissures with surrounding bruising should warn against child abuse. Crohn's disease and leukemic infiltration are other conditions to rule-out. The diagnosis is made after inspection of the anal canal. Chronic fissures are associated with hypertrophy of the anal papilla or a distal skin tag.

Management is directed toward the associated constipation with stool softeners and anal dilatations, warm perineal baths to relax the internal muscle spasm, and topical analgesics for pain control. If medical therapy fails excision of the fissure with lateral sphincterotomy is performed. Meckel's diverticulum MD , the pathologic structure resulting from persistence of the embryonic vitelline duct yolk stalk , is the most prevalent congenital anomaly of the GI tract. MD can be the cause of: Diagnosis depends on clinical presentation.

Rectal bleeding from MD is painless, minimal, recurrent, and can be identified using 99mTc- pertechnetate scan; contrasts studies are unreliable.

Persistent bleeding requires arteriography or laparotomy if the scan is negative. Obstruction secondary to intussusception, herniation or volvulus presents with findings of fulminant, acute small bowel obstruction, is diagnosed by clinical findings and contrast enema studies. The MD is seldom diagnosed preop.

Diverticulitis or perforation is clinically indistinguishable from appendicitis. Mucosal polyps or fecal umbilical discharge can be caused by MD. Overall, complications of Meckel's are managed by simple diverticulectomy or resection with anastomosis.

Laparoscopy can confirm the diagnosis and allow resection of symptomatic cases. Removal of asymptomatic Meckel's identified incidentally should be considered if upon palpation there is questionable heterotopic gastric or pancreatic mucosa thick and firm consistency present. Histology features a cluster of mucoid lobes surrounded by flattened mucussecreting glandular cells mucous retention polyp , no malignant potential.

Commonly seen in children age with a peak at age As a rule only one polyp is present, but occasionally there are two or three almost always confined to the rectal area within the reach of the finger. Most common complaint is bright painless rectal bleeding. Occasionally the polyp may prolapse through the rectum. Diagnosis is by barium enema, rectal exam, or endoscopy. Removal by endoscopy is the treatment of choice.

Rarely colotomy and excision are required. Wilms tumor WT is the most common intra-abdominal malignant tumor in children affecting more than children annually in the USA.

It has a peak incidence at 3. WT present as a large abdominal or flank mass with abdominal pain, asymptomatic hematuria, and occasionally fever. Other presentations include malaise, weight loss, anemia, left varicocele obstructed left renal vein , and hypertension. Initial evaluation consists of: The presence of a solid, intrarenal mass causing intrinsic distortion of the calyceal collecting system is virtually diagnostic of Wilms tumor.

Doppler sonography of the renal vein and inferior vena cava can exclude venous tumor involvement. Metastasis occurs most commonly to lungs and occasionally to liver.

Operation is both for treatment and staging to determine further therapy. Following NWTSG recommendation's primary nephrectomy is done for all but the largest unilateral tumors and further adjuvant therapy is based on the surgical and pathological findings. Important surgical caveats consist of using a generous transverse incision, performing a radical nephrectomy, exploring the contralateral kidney, avoiding tumor spillage, and sampling suspicious lymph nodes.

Nodes are biopsied to determine extent of disease. Stage I- tumor limited to kidney and completely resected. Stage II- tumor extends beyond the kidney but is completely excised. Stage III- residual non-hematogenous tumor confined to the abdomen. Stage IV- hematogenous metastasis.

Stage V- bilateral tumors. Further treatment with chemotherapy or radiotherapy depends on staging and histology favorable vs. Non-favorable histologic characteristics are: Prognosis is poor for those children with lymph nodes, lung and liver metastasis. They tend to occur in younger patients. Routine abdominal ultrasound screening every six months up to the age of eight years is recommended for children at high risk for developing WT such as the above-mentioned syndromes.

It was originally thought that WT developed after the two-hit mutational model developed for retinoblastoma: When the first mutation occurs before the union the sperm and egg constitutional or germline mutation the tumor is heritable and individuals are at risk for multiple tumors. Nonhereditary WT develops as the result of two-postzygotic mutations somatic in a single cell.

The two-event hypothesis predicts that susceptible individuals such as familial cases, those with multifocal disease and those with a congenital anomaly have a lower median age at diagnosis than sporadic cases.

It is now believed that several genes' mutations are involved in the overall WT pathogenesis. Loss of whole portions of a chromosome is called loss of heterozygosity LOH , a mechanism believed to inactivate a tumor-suppressor gene. Children with the WAGR association shows a deletion in the short arm of chromosome 11 band 13 11p13 but a normal 11p15 region. Up to a third of sporadic WT have changes in the distal part of chromosome 11, a region that includes band p The region of the deletion has been named the WT1 gene, a tumor suppressor gene that also forms a complex with another known tumor-suppressor, p WT1 gene express a regulated transcription factor of the zinc-finger family proteins restricted to the genitourinary system, spleen, dorsal mesentery of the intestines, muscles, central nervous system CNS and mesothelium.

The important association of WT1 mutation and WAGR syndrome with intralobar nephrogenic rests immediately suggest that WT1 expression be necessary for the normal differentiation of nephroblasts. Inactivation of WT1 only affects organs that express this gene such as the kidney and specific cells of the gonads Sertoli cells of the testis and granulosa cells of the ovary.

WT1 has been shown to cause the Denys-Drash syndrome. Most of the mutations described in DDS patients are dominant missense mutations. A small subset of BWS has a 11p15 duplication or deletion. The region 11p15 has been designated WT2 gene and is telomeric of WT1. This might prove that two independent loci may be involved in tumor formation.

A gene for a familial form FWT1 of the tumor has also been identified in chromosome 17q. There also might be a gene predisposing to Wilms tumor at chromosome 7p, where constitutional translocations have been described. Mutation in p53 is associated with tumor progression, anaplasia and poor prognosis. Most WT are probably caused by somatic mutations in one or more of the increasing number of WT genes identified.

A few chromosomal regions have seen identified for its role in tumor progression. LOH at chromosome 16q and chromosome 1p has been implicated in progression to a more malignant or aggressive type Wilms' tumor with adverse outcome.

These children have a relapse rate three times higher and a mortality rate twelve times higher than WT without LOH at chromosome 1p. Patients with WT and a diploid DNA content indicating low proliferation have been found to have an excellent prognosis.

Hyperdiploidy high mitotic activity is a poor prognostic feature of Wilms tumor, rhabdomyosarcoma and Osteosarcoma. Nephrogenic rests are precursor lesions of WT. Two types are recognized: Also the association between BWS and some cases of hemihypertrophy with abnormalities of more distant loci on chromosome 11p raises the possibility that the putative WT2 gene might be more closely linked to PLNR.

An advantage of genetic testing is that children with sporadic aniridia, hemihypertrophy or the above discussed syndromes known to be at high risk for developing WT can undergo screening of the germline DNA. This might identify if they harbor the mutation and need closer surveillance for tumor development.

Neuroblastoma NB is the most common extracranial solid tumor in infants. More than new cases are diagnosed annually in the United States. NB is a solid, highly vascular tumor with a friable pseudocapsule.

Most children present with an abdominal mass, and one-fourth have hypertension. Horner's syndrome, Panda's eyes, anemia, dancing eyes or vaso-intestinal syndrome.

Diagnosis is confirmed with the use of simple X-rays stipple calcifications , Ultrasound, and CT-Scan. Management of NB depends on the stage of disease at diagnosis.

Localized tumors are best managed with surgical therapy. The Evans classification for NB staging comprised: Stage I - tumor confined to an organ of origin. Stage II - tumor extending beyond an organ of origin, but not crossing the midline. Ipsilateral lymph nodes may be involved. Stage III - tumor extending beyond midline. Bilateral lymph nodes may be involved.

Stage IV - remote disease involving skeleton, bone marrow, soft tissue or distant lymph nodes. A poor outcome is characteristic of higher stages, older patients and those with bone cortex metastasis. Other prognostic variables are: Neuroblastoma is a malignant tumor of the postganglionic sympathetic system that develops from the neural crest: In vitro three cell types have been identified: These cells are responsible for producing cathecolamines and vasoactive substances which help in diagnosis and follow-up therapy.

NB can behave seemingly benignly and undergo spontaneous regression, mature into a benign ganglioneuroma or most commonly progress to kill its host. This disparate behavior is a manifestation that we are dealing with related tumors with differently genetic and biological features associated with a spectrum of clinical behaviors.

Conclusive associations with environmental factors have not been proved in NB. Hereditary factors are important in NB since a few cases exhibit predisposition following a dominant pattern of inheritance. LOH of the short arm of chromosome 1 is also associated with an unfavorable outcome, suggesting that a tumor suppression gene may be found in this region.

The common region of deletion or LOH resides at the distal end of the short arm of chromosome 1 from 1p Loss or inactivation of a gene at this site is critical for progression of neuroblastoma. A few candidate genes from this site have been mapped. Gain of chromosome 17 is associated with more aggressive tumors. N-myc protooncogene is found on chromosome 2p and its activation results in tumor formation.

The amplified N-myc sequence is found on extrachromosomal double minutes DM or on homogeneous staining regions HSR involving different chromosomes in neuroblastoma N-type cell lines.

N-myc amplification is strongly associated with advance stages of disease, rapid tumor progression and poor outcome independent of the stage of the tumor or the age of the patient. NB tumors associated with N-myc amplification needs aggressive therapy. N-myc amplification associated with deletion of 1p is correlated with a poor outcome. Deletion of the long arm of chromosome 1 1q- is also a poor prognostic sign.

Though most NB cells are diploid, a good number of them are hyperdiploid or triploid. Neuroblast cells needs nerve growth factor NGF for proper differentiation. NB tumor cells do not respond to NGF or do not express the receptor. High TRK-A levels correlate strongly with improved survival and plays a role in the propensity for tumors to regress or differentiate into a more benign nature. Alteration in the NGF receptor function or expression promotes tumorigenesis.

In conclusion, high levels of TRK expression are associated with better prognosis, earlier stage, lower patient age and lack of N-myc expression. Neuroblastomas in newborns, cystic tumors, bilateral tumors in infants, and infants less than one year of age with neuroblastoma stage IV-S can undergo neuronal cell differentiation with spontaneous regression.

It is thought that high level of TRK-A found in this cases might explain differentiation and regression as high level of this glycoprotein is associated with a favorable prognosis.

Regression might be associated with non-affected tumor cell apoptosis. Other biological markers associated with NB are the multidrug resistance-related protein MRP gene, telomerase activity and bcl-2 gene activity.

MRP shows a strong correlation with an advanced clinical stages and poor prognosis. High telomerase activity is associated with poor prognosis and high N-myc amplification. The bcl-2 gene produces a protein that prevents neuronal cell death apoptosis and promotes tumor progression. Bcl-2 expression is associated with a poor outcome. Apoptosis in NB may result in tumor progression. The RET proto-oncogene is a protein tyrosine kinase gene Ret protein expressed in the cells derived from the neural crest.

The activation of RET involves a chromosomal inversion of the long arm of chromosome 10 that juxtaposes the tyrosine kinase encoding domain of RET to the amino terminal sequences of at least three unrelated genes.

Germline mutations in the RET gene have been associated with neuroblastoma, pheochromocytoma, multiple endocrine neoplasia MEN 2, familial medullary thyroid carcinoma MTC , radiation-induced thyroid papillary carcinoma, and recently Hirschsprung's disease. RET analysis is a suitable method to detect asymptomatic children with MEN at risk to develop MTC allowing us to consider thyroidectomy at a very early stage of neoplasm development C-cell hyperplasia or prophylactically.

High levels of neuron specific enolase and serum ferritin levels are associated with a poor prognosis in NB. Nm and ganglioside GD2 are still other tumor markers associated with poor outcome, active disease and tumor progression. It has a peak incidence before the age of five years, and a second surge during early adolescence. Head, neck and pelvic malignancies are more prevalent in infancy and early childhood, while trunk, extremity and paratesticular sites are largely a disease of adolescents.

RMS arises from a primitive cell type and occurs in mesenchymal tissue at almost any body site excluding brain and bone. The predominant histologic type in infants and small children is embryonal rhabdomyosarcoma, occurring in the head and neck, genitourinary tract and retroperitoneum.

Embryonal RMS is associated with a favorable prognosis. Botryoid RMS is a subtype of the embryonal variety, which ordinarily extends into body cavities such as bladder, nasopharynx, vagina, or bile duct. The alveolar cell type, named for a superficial similarity to the pulmonary alveoli, is the most common form found on the muscle masses of the trunk and extremities, and is seen more frequently in older children and young adults.

Alveolar RMS is associated with a poor prognosis. This unfavorable prognosis is the result of early and wide dissemination, bones marrow involvement and poor response to chemotherapy. Clinical findings, diagnostic evaluation and therapy depend upon location of the primary tumor and are beyond the scope of this review. Head and neck RMS are most common and occur in the orbit, nasopharynx, paranasal sinuses, cheek, neck, middle ear, and larynx. Most are treated by simple biopsy followed by combined therapy or preoperative chemotherapy and radiation followed by conservative resection.

Operations for extremity lesions include wide local excision to remove as much of gross tumor as possible. The trend in management is more chemotherapy with conservative surgical therapy. Survival has depended on primary site, stage of disease, and treatment given. Most RMS occurs sporadically. Other risk factors in the development of RMS include maternal use of marijuana and cocaine, exposure to radiation, and maternal history of stillbirth.

Alveolar and embryonal RMS are the most genetically studied sarcomas in children. In alveolar RMS novel fusion genes encoding chimeric fusion proteins have been identified. The t 2;13 activates the oncogenic potential of PAX3 by dysregulating or exaggerating its normal function in the myogenic lineage and affecting the cellular activities of growth, differentiation and apoptosis.

PAX7-FKHR tumors tend to occur in younger patients, are more often in the extremity, are more often localized lesions and are associated with significantly longer event-free survival. Still, a small subset of alveolar RMS does not contain either fusion mutation. Identification of fusion gene status by PCR is a useful diagnostic tool in differentiating RMS from other round cell tumors.

Embryonal RMS contains frequent allelic loss on chromosome 11 11p15 , a genetic feature specific for this type of tumor. Allelic loss is manifested by the absence of one of the two signals in the tumor cells indicating a genetic event such as a chromosome loss, deletion, or mitotic recombination that eliminates one allele and the surrounding chromosomal region.

The smallest affected region has been localized to chromosomal region 11p The presence of a consistent region of allelic loss is often indicative of the presence of a tumor suppressor gene inactivated in the associated malignancy. The mechanism for inactivation of tumor suppressor genes is postulated to be a two-hit scenario in which both copies of the gene are sequentially inactivated: This leads to over expression of the insulin-like growth factor II gene known to play a role in the development of embryonal tumors.

Notably, these distinct changes predominantly involved chromosomes 2, 12, and 13 in both subtypes. Both tumors share alterations in the p53 gene at the germline level contributing to increase susceptibility to other tumors characteristics of the Li-Fraumeni syndrome. All this favors a multi-step origin of RMS tumors generating phenotypic changes of growth autonomy, abnormal differentiation and motility. More than one-half of the cancers overall and nearly one-third of the breast cancers were diagnosed before 30 years of age.

Among females, breast cancer is the most common. Germline mutations within a defined region of the p53 gene have been found in families with the Li-Fraumeni syndrome. Persistence of the mutation in the germline suggests a defect in DNA repair in the family member first affected. Asymptomatic carriers of p53 germline mutation needs closed evaluation and follow-up for early detection and treatment in case neoplasia develops. Hepatoblastoma HB is the most common primary malignant neoplasm of the liver in children mostly seen in males less than four year of age.

Diagnostic laparotomy will decide resectability. Markers associated to this tumor are: Only reliable chances of cure is surgical excision although half are unresectable at dx. Unresectable tumors can be managed with preop chemotx. Disadvantages of preop chemotx are: Tumor necrosis is more extensive in pt. Osteoid present in tumors after chemotx may represent an inherent ability of the tumor to maturate and differentiate.

Diploid tumors on DNA flow cytometry show a better prognosis. Sacrococcygeal teratoma SCT is the most common extragonadal germ cell tumor in neonates with an incidence of one in , live births. SCT present as a large, firm or more commonly cystic masses that arise from the anterior surface of the sacrum or coccyx, protruding and forming a large external mass.

Histology consist of tissue from the three germ cell layers. SCT is classified as: Prenatal sonographic diagnostic severity criteria are: Large tumors should benefit from cesarean section to avoid dystocia or tumor rupture.

Management consist of total tumor resection with coccyx recurrence is associated with leaving coccyx in place.

Every recurrence of SCT should be regarded as potentially malignant. Aggressive surgery is limited to avoid compromising reproductive capacity and endocrine function. Low incidence and need of mulitinodal therapy encourages referral to centers dealing with effective cancer therapy. The most common histology is germ cell: This is followed by the sex-cord stroma tumors with a low incidence of malignancy. They can cause feminization granulosa-theca cell and masculinization androblastoma.

Ovarian tumors present with acute abdominal symptoms pain from impending rupture or torsion. They also cause painless abdominal enlargement, or hormonal changes. Preop work-up should include: The most important prognostic factor in malignant tumors is stage of disease at time of diagnosis. Objectives of surgery are: Radiotherapy is generally not effective, except in dysgerminoma.

The need to differentiate malignant from benign thyroid nodules is the most challenging predicament in management. After reviewing our ten-year experience with twenty-four pediatric thyroid nodules we found nineteen benign and five malignant lesions. Benign nodules were soft, movable, solitary and non-tender. Malignant nodules were found during late adolecence, characterized by localized tenderness, a multigandular appearance and fixation to adjacent tissues.

US and RNS were of limited utility since malignancy was identified among cystic and hot nodules respectively. Suppressive thyroid hormone therapy was useless in the few cases tried. FNAB in eighteen cases did not limit the number of thyroid resections. This is the result of a higher number of patients with follicular cell cytology in the aspirate.

No attempts should be made to differentiate follicular adenoma from carcinoma since capsular and vascular invasion cannot be adequately assessed by FNAB.

The Jewish community was fined one billion marks to pay for the damage caused by Kristallnacht and told that any insurance settlements would be confiscated.

Emigrants to Palestine were allowed to transfer property there under the terms of the Haavara Agreement , but those moving to other countries had to leave virtually all their property behind, and it was seized by the government.

Like the Jews, the Romani people were subjected to persecution from the early days of the regime. As a non-Aryan race, they were forbidden to marry people of German extraction. Romani were shipped to concentration camps starting in and were killed in large numbers. Action T4 was a programme of systematic murder of the physically and mentally handicapped and patients in psychiatric hospitals that mainly took place from to , and continued until the end of the war.

Initially the victims were shot by the Einsatzgruppen and others; in addition gas chambers and gas vans using carbon monoxide were used by early Mentally and physically ill people were also targeted. Most of the victims came from disadvantaged groups such as prostitutes, the poor, the homeless, and criminals.

Germany's war in the East was based on Hitler's long-standing view that Jews were the great enemy of the German people and that Lebensraum was needed for Germany's expansion. Hitler focused his attention on Eastern Europe, aiming to defeat Poland and the Soviet Union and remove or kill the resident Jews and Slavs.

The Generalplan Ost "General Plan for the East" called for deporting the population of occupied Eastern Europe and the Soviet Union to Siberia, for use as slave labour or to be murdered. Around the time of the failed offensive against Moscow in December , Hitler resolved that the Jews of Europe were to be exterminated immediately.

Some would be worked to death and the rest would be killed in the implementation of Die Endlösung der Judenfrage the Final Solution of the Jewish question. German citizens had access to information about what was happening, as soldiers returning from the occupied territories would report on what they had seen and done. Evans states that most German citizens disapproved of the genocide. In addition to eliminating Jews, the Nazis planned to reduce the population of the conquered territories by 30 million people through starvation in an action called the Hunger Plan.

Food supplies would be diverted to the German army and German civilians. Cities would be razed and the land allowed to return to forest or resettled by German colonists. Poles were viewed by Nazis as subhuman non-Aryans, and during the German occupation of Poland 2. The German authorities engaged in a systematic effort to destroy Polish culture and national identity. During operation AB-Aktion , many university professors and members of the Polish intelligentsia were arrested, transported to concentration camps, or executed.

During the war, Poland lost an estimated 39 to 45 percent of its physicians and dentists, 26 to 57 percent of its lawyers, 15 to 30 percent of its teachers, 30 to 40 percent of its scientists and university professors and 18 to 28 percent of its clergy.

Between June and January , the Nazis killed an estimated 2. Antisemitic legislation passed in led to the removal of all Jewish teachers, professors and officials from the education system.

Frequent and often contradictory directives were issued by Interior Minister Wilhelm Frick, Bernhard Rust of the Reichserziehungsministerium Ministry of Education , and various other agencies regarding content of lessons and acceptable textbooks for use in primary and secondary schools. Detailed National Socialist indoctrination of future holders of elite military rank was undertaken at Order Castles. Primary and secondary education focused on racial biology, population policy, culture, geography, and especially physical fitness.

At universities, appointments to top posts were the subject of power struggles between the education ministry, the university boards, and the National Socialist German Students' League. Religion in Germany []. When the Nazis seized power in , roughly 67 percent of the population of Germany was Protestant , 33 percent was Roman Catholic , while Jews made up less than 1 percent.

Under the Gleichschaltung process, Hitler attempted to create a unified Protestant Reich Church from Germany's 28 existing Protestant state churches , [] with the ultimate goal of eradication of the churches in Germany. Persecution of the Catholic Church in Germany followed the Nazi takeover.

Catholic schools were required to reduce religious instruction and crucifixes were removed from state buildings. Pope Pius XI had the " Mit brennender Sorge " "With Burning Concern" encyclical smuggled into Germany for Passion Sunday and read from every pulpit as it denounced the systematic hostility of the regime toward the church.

Enrolment in denominational schools dropped sharply and by all such schools were disbanded or converted to public facilities. He planned the "extermination of the foreign Christian faiths imported into Germany", and for the Bible and Christian cross to be replaced in all churches, cathedrals, and chapels with copies of Mein Kampf and the swastika.

Nazi Germany had a strong anti-tobacco movement as pioneering research by Franz H. Müller in demonstrated a causal link between tobacco smoking and lung cancer. Government-run health care insurance plans were available, but Jews were denied coverage starting in That same year, Jewish doctors were forbidden to treat government-insured patients.

In , Jewish doctors were forbidden to treat non-Jewish patients and in their right to practice medicine was removed entirely.

Medical experiments, many of them pseudoscientific , were performed on concentration camp inmates beginning in Josef Mengele , camp doctor at Auschwitz. Women were a cornerstone of Nazi social policy and the Nazis opposed the feminist movement, claiming that it was the creation of Jewish intellectuals, instead advocating a patriarchal society in which the German woman would recognise that her "world is her husband, her family, her children, and her home".

Courses were offered on childrearing, sewing and cooking. Women were encouraged to leave the workforce and the creation of large families by racially suitable women was promoted through a propaganda campaign. Women received a bronze award—known as the Ehrenkreuz der Deutschen Mutter Cross of Honour of the German Mother —for giving birth to four children, silver for six and gold for eight or more.

Though the measures led to increases in the birth rate, the number of families having four or more children declined by five percent between and After the war started, slave labourers were extensively used. The Nazi regime discouraged women from seeking higher education since Nazi leaders held conservative views about women and endorsed the idea that rational and theoretical work was alien to a woman's nature since they were considered inherently emotional and instinctive — as such, engaging in academics and careerism would only "divert them from motherhood".

The number of women enrolled in post-secondary schools dropped from , in to 51, in However, with the requirement that men be enlisted into the armed forces during the war, women comprised half of the enrolment in the post-secondary system by Women were expected to be strong, healthy and vital.

The BDM's activities focused on physical education, with activities such as running, long jumping, somersaulting, tightrope walking, marching and swimming.

The Nazi regime promoted a liberal code of conduct regarding sexual matters and was sympathetic to women who bore children out of wedlock. Soldier's wives were frequently involved in extramarital relationships.

Sex was sometimes used as a commodity to obtain better work from a foreign labourer. With Hitler's approval, Himmler intended that the new society of the Nazi regime should destigmatise illegitimate births, particularly of children fathered by members of the SS, who were vetted for racial purity. Existing laws banning abortion except for medical reasons were strictly enforced by the Nazi regime.

The number of abortions declined from 35, per year at the start of the s to fewer than 2, per year at the end of the decade, though in a law was passed allowing abortions for eugenics reasons. Nazi society had elements supportive of animal rights and many people were fond of zoos and wildlife.

In , the Nazis enacted a stringent animal-protection law that affected what was allowed for medical research. The Reich Forestry Office under Göring enforced regulations that required foresters to plant a wide variety of trees to ensure suitable habitat for wildlife and a new Reich Animal Protection Act became law in Evans , The Coming of the Third Reich The regime promoted the concept of Volksgemeinschaft , a national German ethnic community. The goal was to build a classless society based on racial purity and the perceived need to prepare for warfare, conquest and a struggle against Marxism.

In addition to taking control of tens of thousands of previously privately run recreational clubs, it offered highly regimented holidays and entertainment such as cruises, vacation destinations and concerts. Sub-chambers were set up to control aspects of cultural life such as film, radio, newspapers, fine arts, music, theatre and literature.

Members of these professions were required to join their respective organisation. Jews and people considered politically unreliable were prevented from working in the arts and many emigrated. Books and scripts had to be approved by the Propaganda Ministry prior to publication.

Standards deteriorated as the regime sought to use cultural outlets exclusively as propaganda media. Radio became popular in Germany during the s, with over 70 percent of households owning a receiver by , more than any other country. Radio station staffs were purged of leftists and others deemed undesirable by July As with other media, newspapers were controlled by the state, with the Reich Press Chamber shutting down or buying newspapers and publishing houses.

By , over two thirds of the newspapers and magazines were directly owned by the Propaganda Ministry. Under Goebbels, the Propaganda Ministry issued two dozen directives every week on exactly what news should be published and what angles to use; the typical newspaper followed the directives closely, especially regarding what to omit.

Authors of books left the country in droves and some wrote material critical of the regime while in exile. Goebbels recommended that the remaining authors concentrate on books themed on Germanic myths and the concept of blood and soil. By the end of , over a thousand books—most of them by Jewish authors or featuring Jewish characters—had been banned by the Nazi regime.

In Berlin over 20, books being burned at the square at the State Opera. Pacifist works, as well as literature espousing liberal, democratic tendencies and attitudes were targeted for destruction, as well as any writings supporting the Weimar Republic or those written by Jewish authors.

Hitler took a personal interest in architecture and worked closely with state architects Paul Troost and Albert Speer to create public buildings in a neoclassical style based on Roman architecture. Neither structure was built. Hitler's belief that abstract , Dadaist , expressionist and modern art were decadent became the basis for policy.

The exhibition proved wildly popular, attracting over two million visitors. Movies were popular in Germany in the s and s, with admissions of over a billion people in , and Exports of German films plummeted, as their antisemitic content made them impossible to show in other countries.

The two largest film companies, Universum Film AG and Tobis , were purchased by the Propaganda Ministry, which by was producing most German films. The productions were not always overtly propagandistic, but generally had a political subtext and followed party lines regarding themes and content.

Leni Riefenstahl 's Triumph of the Will —documenting the Nuremberg Rally—and Olympia —covering the Summer Olympics —pioneered techniques of camera movement and editing that influenced later films. New techniques such as telephoto lenses and cameras mounted on tracks were employed. Both films remain controversial, as their aesthetic merit is inseparable from their propagandising of National Socialist ideals.

The Allied powers organised war crimes trials, beginning with the Nuremberg trials , held from November to October , of 23 top Nazi officials. They were charged with four counts—conspiracy to commit crimes, crimes against peace, war crimes and crimes against humanity —in violation of international laws governing warfare. The display or use of Nazi symbolism such as flags, swastikas or greetings is illegal in Germany and Austria [] [] and other restrictions, mainly on public display, apply in various countries.

Nazi ideology and the actions taken by the regime are almost universally regarded as gravely immoral. Evans remarks that the era "exerts an almost universal appeal because its murderous racism stands as a warning to the whole of humanity".

The Nazi era continues to inform how Germans view themselves and their country. Virtually every family suffered losses during the war or has a story to tell, though Germans kept quiet about their experiences and felt a sense of communal guilt, even if they were not directly involved in war crimes.

Once study of Nazi Germany was introduced into the school curriculum starting in the s, people began researching the experiences of their family members. Study of the era and a willingness to critically examine its mistakes has led to the development of a strong democracy in today's Germany, but with lingering undercurrents of antisemitism and neo-Nazi thought.

From Wikipedia, the free encyclopedia. Redirected from Third reich. For the book, see Das Dritte Reich. Das Lied der Deutschen "Song of the Germans". Administrative divisions of Germany, January Nazi one-party totalitarian dictatorship. The German state from to , under the dictatorship of Adolf Hitler. Adolf Hitler's rise to power. Anschluss and German occupation of Czechoslovakia. Protectorate of Bohemia and Moravia. Mass suicides in Nazi Germany. Territorial evolution of Germany.

Government of Nazi Germany. Economy of Nazi Germany. Nazism and race and Racial policy of Nazi Germany. Anti-Jewish legislation in prewar Nazi Germany. Porajmos and Nazi eugenics. Nazi crimes against the Polish nation. German mistreatment of Soviet prisoners of war and Generalplan Ost. University education in Nazi Germany. Other religion or irreligious 4. Religion in Nazi Germany.

Women in Nazi Germany. Animal welfare in Nazi Germany. If the experience of the Third Reich teaches us anything, it is that a love of great music, great art and great literature does not provide people with any kind of moral or political immunization against violence, atrocity, or subservience to dictatorship.

List of authors banned in Nazi Germany. Nazi architecture and Art of the Third Reich. See Statistisches Jahrbuch It could not be expected that even for a brief period our Air Force could make up for our lack of naval supremacy. Grand Admiral Karl Dönitz believed air superiority was not enough and admitted, "We possessed neither control of the air or the sea; nor were we in any position to gain it.

The figure of 2 to 2. The German Red Cross still maintains that the death toll from the expulsions is 2. Goebbel's propaganda campaigns carried out in the second half of and again in had failed to convert them".

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Smith, Joseph; Davis, Simon