Achalasia is defined by the following important elements:

• Hypertonic lower esophageal sphincter;
• Absence of lower esophageal sphincter relaxation to swallowing;
• Absence of peristaltic waves in two thirds of the lower esophagus.

Achalasia

In conclusion, the lower esophageal sphincter relaxation dose not occur during swallowing.

Etiopathogenesis is not well known. It incriminate genetic factors, such as the existence of a genetic predisposition, environmental factors, such as infection with certain viruses, the role of emotions and stress in triggering the disease.
Pathological studies have shown an impairment of the nervous control of motility of the esophagus, and esophageal muscles.

The hypothesis of involvement of a virus that secrete a neurotoxin that affects vague nerve is supported by the existence of secondary achalasia in Chagas disease (infestation with Tripanosoma Cruzi), the parasite produces nerve damage that causes the appearance of mega-esophagus.

Clinical picture

The clinical picture is dominated by dysphagia or odynophagia (pain on swallowing). Sometimes swallowing may be paradoxical, difficulty in swallowing liquids, but with good tolerance of solid food. Hiccups may occur late when is an important esophageal dilatation.

Regurgitation of food and saliva is quite common, occurring in several hours after fasting, but over time, by dilation of  the esophagus, regurgitation will diminish. At night, regurgitation can cause coughing and dyspnea.

In advanced stages, patients typically take a position (position of Valsalva), which increases their intrathoracic pressure and ease the transition of the bowel towards to the stomach.

Clinical Picture of Achalasia

Diagnosis

Suspected clinical diagnosis will be confirmed by endoscopy and radiology.

Endoscopy will show a dilated esophagus  with food debris and abundant saliva, but without mucosal injury.

Barium-swallow examination of the esophagus is useful and valuable because it shows a more dilated esophagus, which narrows in the lower portion symmetrically, with an aspect of radish. Tracking swallowing, will reveal a absence of esophageal peristaltic and lack of esophageal sphincter relaxation.

Treatment

Treatment is often difficult and consists of three alternatives:

• Drugs that decrease lower esophageal sphincter pressure, such as nitrates, calcium channel blockers, miofilin, theophylline, anticholinergics. It is administered one or two drugs a day,  in the first stages of the disease can be effective;
• Endoscopic. Consist in expansion technique of the lower esophageal sphincter with inflatable balloon under floroscopic control. It can be used endoscopic injection in the lower esophageal sphincter of botulinum inactivated toxoid, which makes a temporary paralysis of the sphincter. The effect is for several months and after that, the injection can be repeated;
• Surgery. It is rarely indicated in cases where other techniques have failed and consists of Heller cardiomyotomy of the lower esophageal sphincter (longitudinal section of the circular fibers). Ultimately predispose to gastroesophageal reflux disease.

Gastroesophageal Reflux Disease

Gastroesophageal reflux disease includes all symptoms caused by the reflux of stomach contents into the esophagus. Gastroesophageal reflux is a phenomenon of passage of stomach contents into the esophagus, a physiological phenomenon, which becomes pathological when antireflux mechanisms are overcome. Reflux esophagitis represents all esophageal lesions caused by gastroesophageal reflux disease, not seen in all cases of gastroesophageal reflux disease. Gastroesophageal reflux disease is a clinical entity relatively common  in medical practice and is often has a polymorphic symptomatic picture.

GERD

Prevalence

Gastroesophageal reflux disease has a prevalence of 4% in the general population, increasing with age. The current trend is the increase in the incidence of the disease.

Etiopathogenesis

In the case of gastroesophageal reflux disease, are describe two mechanisms that determine the inefficient antireflux mechanism:

Physiological causes:

• decreased lower esophageal sphincter pressure. Under normal conditions, lower esophageal sphincter pressure is 20 to 25 mm Hg and disappears only when swallowing. Gastroesophageal reflux occurs when the lower esophageal sphincter relaxes outside swallowing, or when lower esophageal sphincter pressure falls below 6 mm Hg, thus allowing the passage of stomach contents into the esophagus. Lower esophageal sphincter pressure may be reduced by factors like drugs (anticholinergics, benzodiazepines, calcium channel blockers), food (chocolate, onions, fats, citrus), coffee, smoking and alcohol;
• decreased gastric motility with delayed gastric emptying;
• damage to esophageal clearance by acid stomach contents which flows backward  into the esophagus. This esophageal clearance with the swallowed saliva serve as a tampon mechanism for the acid content of the stomach which flows backward into the esophagus;

Mechanical causes:

• hiatal hernia causes a decrease of the tonus of the lower esophageal sphincter, which favors the reflux;
• increased intra-abdominal pressure leads to widening of the diaphragmatic hiatus, explaining the occurrence of gastroesophageal reflux disease in pregnant women, obese, etc.;
• His angle widening, the angle between the esophagus and stomach is usually very sharp, with the role of a valve at the entrance of the stomach. In obese it widens and loses physiological role.

Development and severity of gastroesophageal reflux disease is caused by the presence of three conditions:

• increased frequency of reflux;
• increased duration reflux;
• aggressive effect of stomach contents on the esophageal mucosa.

LES

Symptomatology

Symptoms are relatively typical, translating by acid regurgitation and heartburn, with continuous or discontinuous character. Symptoms may be occasional or permanent.

Retrosternal pain or dysphagia are relatively rare. The presence of these two symptoms should lead thinking at more severe pathology. Rarely, in atypical forms of gastroesophageal reflux disease, symptoms can mimic a heart disease with angina type of pain or the onset of bronchial asthma.

• pyrosis is the sensation of heartburn that goes to the neck. It is accentuated by maneuvers that increase intra-abdominal pressure (bending forward, going to bed immediately after eating, weight lifting) is sometimes accompanied and acid regurgitation. If lower esophageal sphincter incompetence is increased, then will appear food regurgitation;
• chest pain often provide diagnostic problems with heart disease. Can occur isolated unaccompanied by heartburn, predominantly in irritating food intake;
• odynophagia (pain swallowing) and disafagia (difficult to swallowing) occur in the case of spastic contraction of the lower esophageal sphincter;
• respiratory symptoms (choking, shortness of breath at night, asthma) or otolaryngology symptoms (laryngitis, faringene paresthesia, hoarseness) are due to regurgitation of acid stomach contents and aspiration of this contents.

Paraclinical examination

Paraclinical examination necessary for evidence of gastroesophageal reflux disease are:

Upper gastrointestinal endoscopy: in the presence of esophageal symptoms distressing, persistent, especially in the presence of dysphagia and pain will be performed esophageal-gastroscopy. This can reveal any mucosal lesions, or may exclude them. May reveal a peptic associated lesion which causing symptoms. May be evidence the presence of a hiatal hernia. Also by endoscopy detected lesions can be biopsied. Consequence of gastroesophageal reflux disease, reflux esophagitis is an esophageal mucosa injury under the effect of acid or alkaline reflux.

The severity of endoscopic lesions is estimated by Los Angeles classification of esophagitis which have several degrees:

• A: one or more areas of substance loss, less than 5 mm;
• B: at least one area by substance loss greater than 5 mm, none of which extends between the tops of two mucosal folds;
• C: at least one area by extensive substance loss between 3 or 4 mucosa folds, but which involve less than 75% of the esophageal circumference;
• D: circumferential loss of substance.

Esophageal pH-metric: over 24 hours is very useful to find out the duration of the reflux. It is useful to correlate symptoms with acid pH, but also atypical symptoms may be correlated with the reflux.

Esophageal manometer: coupled with pH-meter allows detection of fine lesions and their correlation with esophageal symptoms.

Barium X-ray: may detect motor disorders of the esophagus.

Complications

Complications which occur in gastroesophageal reflux disease are:

• Reflux esophagitis, in varying degrees, up to esophageal ulcers and esophageal stenosis;
• Barrett’s esophagus, represents a epithelial cylindrical metaplasia of the normal esophageal mucosa, as a consequence of the healing of gastroesophageal reflux disease and is a preneoplastic lesions and cancer of the esophagus;
• Upper gastrointestinal bleeding, a rare complication.

GERD

Treatment

Dietary treatment: a lot of cases of gastroesophageal reflux disease can be solved by diet, these measures are:

• avoiding large meals, avoiding foods that decrease lower esophageal sphincter pressure: coffee, chocolate, carbonated beverages, mint products, fats, alcohol, or avoiding foods that increase acid secretion: orange juice, white wine, acidic foods;
• Avoid smoking because it is believed to increase acid secretion and decreased lower esophageal sphincter pressure;
• avoiding bedtime after eating;
• obese people are advised to lose weight.

Drug treatment: includes the following types of drugs:

Anti-secreting drugs, these drugs reduce acid secretion, the two main groups of anti secretory are:

• histamine H2 blockers: ranitidine, famotidine, which can be used over a period of 4-8 weeks in case of esophagitis, and if the symptoms are occasionally recommended to be administrated on demand;
• proton pump inhibitors, are represented by omeprazole, lansoprazole, esmoprazol and rabeprazol. Treatment duration is 4 to 8 weeks.

In the treatment of gastroesophageal reflux disease, there are two therapeutic strategies, “step down” and “step up”.

The “step down” strategy means starting therapy with a higher dose of proton pump inhibitors, which then, in case of positive response, the dosage can be halved or administration can proceed to histamine H2 blockers.

The “step up” strategy means staring therapy with histamine H2-blockers and in case of failure to pass the administration of proton pump inhibitors.

GERD

Prokinetic medication. In this category are metoclopramide and domperidomul, drugs that increase lower esophageal sphincter tonus and gastric emptying. Treatment strategy in gastroesophageal reflux disease is as follows:  start with taking a proton pump inhibitor, and in case of failure add a prokinetic

 Lactose Intolerance

From an epidemiological point of view, there is great variability of lactase deficiency disease known as lactose intolerance, depending on geographic area. Populations who do not consume milk (Australian Aborigines, Eskimos, American Indians, the Chinese) have an incidence of lactose intolerance in the adult population at the rate of 40% – 90%. Populations which, throughout history, have grown animals (Europeans and their descendants) have an incidence of lactose intolerance in the adult population at a rate of 5% -15%.

Lactose Intolerance

Congenital lactose intolerance, occurs immediately after birth and is manifested by diarrhea. Primary lactase deficiency late-onset or late-onset lactose intolerance is a relatively normal situation. Thus, after weaning, in the infant has place a repression of the lactase activity. An adult has about 5% – 10% of the lactase activity of the newborn. The primary deficit is a hereditary condition regarded to ethnic group without implication of geographical location, environmental conditions and current milk consumption. It is believed that persistence of lactase activity is an adaptive genetic mutation (produced in populations that are growing animals and consuming milk), and lactase deficiency is a relatively normal condition.

Acquired lactase deficiency or lactose intolerance secondary to inflammatory bowel disease: celiac disease, Crohn’s disease, etc.

lactose intolerance

Morphopathology

On microscopic examination the intestinal mucosa has a normal aspect, only the use of immunohistochemical techniques can highlight a decrease or absence of the enzymatic equipment. Only in  secondary lactose intolerance will appear changes which are characteristic for the primary disease.

Symptoms

Clinical signs of disease are relatively typical and yet are ignored by the patient for years. Signs of disease  may vary, depending on lactase deficiency and the amount of lactose that is consumed. Typically, after consuming milk or milk derivatives, in patient with lactose intolerance will occur in  a few tens of minutes bloating, gurgling, explosive watery stools and flatulence.

Diagnosis

From obvious clinical signs or clinical suspicion, may be done a food sample, asking the patient to ingest 250-300 ml of milk on an empty stomach, and to follow the effect for 2 or 3 hours. If there are clinical signs described, the diagnosis is certain.

Lactose tolerance test, which consists of three phases: clinical, biological and radiological. First is determined the patient fasting plasma glucose, then is given 50 g lactose in 400 ml water and a package of barium sulphate. Will be taken glucose level at 1 and 2 hours and will be performed a abdominal X-ray at one hour. Interpretation of results:

• Clinical: diarrhea, flatulence will indicate a positive clinical test;
• Biological: the absence of glucose increased more than 25% of the fasting value is a positive test;
• Radiological: in the lactose intolerance will appear a dilution of the barium, relaxed bowel loops and accelerated bowel movements.

Evolution

Lactose intolerance generally has a favorable evolution, because patients restrict their diet, avoiding milk products In some cases, especially undiagnosed malabsorption can occur because of prolonged diarrhea.

Treatment

lactose Intolerance Treatment

In the case of lactose intolerance, the treatment is clearly dietetic, and consists of a reduction or complete removal of milk and dairy foods.A solution is represented by the appearance of lactase preparations of bacterial origin, such as  Lact-Aid. Coadministration with a meal of 2 or 3 tablets will ensure the assimilation of the lactose and prevent symptomatology.

Celiac Disease

Celiac disease or gluten enteropathy is a chronic intestinal disease characterized by diarrhea, steatorrhea and malabsorption, generated by gluten intolerance. Morphological element is the atrophy of jejunal mucosa and gluten-free diet leads to clinical and histological improvment of the disease. The condition is widespread in temperate climates and has a chronic evolution, with onset or exacerbation after consumption of wheat flour products.

Celiac Disease

Disease prevalence is between 10 and 30 cases per 100.000 inhabitants, and in the last 15-20 years, with typical forms of disease, who present with diarrhea, steatorrhea and malabsorption, have appeared latent form of gluten intolerance, which not necessarily lead to mucosa atrophy, but only  to interstitial inflammation of the jejunal mucosa.

Celiac disease is a genetically determined condition that has a familial character, is 10 times more frequent in first degree relatives of people with celiac disease and 30 times more common in twins.

Etiopathogenesis

In celiac disease, exist in enterocytes a genetic deficiency of peptidases, which lead to sensitization of the enterocytes to alpha-gliadin, a component of gluten. Gliadin is found mainly in wheat and rye and less in barley and oats.

Prolonged contact of the enterocytes with undigested gliadin, will lead to a local immunologic conflict, through formation of immune complexes of gliadin and gliadin antibodies. These immune complexes are fixed on the intestinal mucosa, stimulates T-cell aggregation, leading in this way to the damage of mucosa with the loss of villi and proliferation of cryptic cells.

Interruption of eating gluten favors the restoring of chorionic epithelium, improving transit and malabsorption disorders, with the condition that the diagnosis should be put in the first 3 to 6 years after clinical onset of the disease. In advanced forms of disease, intestinal mucosa regeneration is extremely slow or absent.

celiac disease

Symptoms

Celiac disease may be symptomatic or asymptomatic and may occur at any age, often without diarrhea or steatorrhea.

If it is celiac disease with onset in childhood, the child does not experience symptoms until the introduction of pasta in the diet. Then begin to appear loose stools with foul smell and intestinal cramps. Appear anemia, edema and hypoproteinaemia.

In the adult form of celiac disease, gradually appear diarrhea, steatorrhea and malabsorption syndrome later. Usually patients presents diarrhea for years (3-6 stools / day) associated with abdominal discomfort and gurgling. The onset of diarrheal syndrome is often insidious,in childhood, but sometimes after age 20 to 30. Most often diarrhea occurs in 1 to 2 hours after a meal of pasta wheat, but other intolerances occur during celiac disease that are making difficult to diagnose it. Important elements are the clinical symptoms which are correlate with the consumption of wheat flour and symptom disappear at 2-3 weeks after their interruption.

There are asymptomatic forms celiac disease that is manifested by iron deficiency anemia, short stature, hypocalcemia and dermatological diseases.

Diagnosis

Diagnosis is made mainly in two ways:

• serological: determining anti gliadin antibodies, anti endomisium antibodies and anti reticulin antibodies;
• biopsy: characteristic features are the flattening of intestinal villi.

Intestinal biopsy association with positive serology for celiac disease is the gold standard in terms of the diagnosis of celiac disease.

In terms of histological lesions are evident in the first portion of the intestine.
Determination of anti  gliadin antibodies have a sensitivity of 80-90% of cases of celiac disease. These antibodies become undetectable during gluten-free diet.
Anti endomisium antibodies are very sensitive, over 90% of cases of celiac disease, these antibodies are present.

Determination of antibodies in celiac disease is a useful test, especially in familial and population screenings, and in epidemiological studies, but the test that always confirm celiac disease is the intestinal biopsy.

Other paraclinical examination that can be used in celiac disease are:

• determination of steatorrhea, which is daily between 7 g and 50 g in case of severe celiac disease;
• impaired intestinal absorption tests, such as D-xylose test;
• highlighting the malabsorption syndrome, either selective (iron, calcium, etc.), either global.

celiac disease

Prognosis

The prognosis depends on the time of diagnosis. In cases of undiagnosed celiac disease will appear gradually malabsorption, which will lead in severe cases to death. Another cause of death is the development of lymphoid tumors, especially intestinal lymphoma. Other cancers which are favored by celiac disease are esophageal cancer and small intestine cancer.

In cases of celiac disease diagnosed and subjected to a gluten-free diet, the evolution is favorable, with disappearance of diarrhea, steatorrhea and malabsorption.

Celiac Disease

Treatment

• Dietary: Celiac disease can have a favorable evolution with a gluten-free diet. Wheat, barley, rye and oats will be removed from the diet. Is admitted the consumption of corn flour, rice flour and potato. Completely cure of celiac disease (in pathology exam lesions can not be detect ) in 3 to 5 years of a gluten-free diet, but favorable clinical response can occur from 3 to 6 weeks after starting the diet. Gluten-free diet is for life.
  Supervision of compliance with diet can be done by dosing anti gliadin antibodies, which after a few months to a year of proper diet will be normal, but will increase again in case of stopping the gluten-free diet.
• Medications: When dose not appear a improvement of symptoms after eliminating gluten from the diet, because celiac disease is in an advanced stage, oral corticosteroids is recommended for a period of 4 to 8 weeks.

Irritable Bowel Syndrome

Irritable Bowel SyndromeIrritable bowel syndrome is a functional pathology which is widespread in the population, characterized by a disturbances of the bowel transit, consisting in general by alternation of constipation with diarrhea, diffuse abdominal pain, usually in the form of cramps, sometimes the emission of mucus. Rectoragia, anemia or weight loss are not a part of the pictures of irritable bowel syndrome .
Irritable bowel syndrome received a lot of synonyms, such as diarrhea nervosa, but the most suggestive name would be “unhappy colon.

Irritable Bowel Syndrome

Patients with irritable bowel syndrome generally see a lot of doctors, from the family physician to the gastroenterologist or even surgeon due to fear of cancer, but also because of the long evolution of this disease. Usually, patients are concerned about the disease, anxious, depressed and often working under stressful conditions. Anxiety and depression are common in these patients so that they will present to the doctor a very thorough, detailed history of the disease, often with dramatic characters. The fund of this disease can be constipation, which can be linked to lack of fiber in the diet and sedentary lifestyle, with the appearance of mucus stools or diarrhea, which occur most often under stressful conditions.
Irritable bowel syndrome is a functional disease, so organic lesions, detectable by laboratory techniques are absent.

Irritable Bowel Syndrome

Symptomatology

Irritable bowel syndrome symptoms include:

• Abdominal pain, which can be diffuse or localized on colic tract. The pain may be dull, but often have the character of a cramp, lasting seconds or minutes. Sometimes the patient feels only abdominal discomfort. Symptoms often disappear during periods of relaxation;
• Intesinal transit disorders are common. Their main feature is alternation of constipation with diarrhea. Typically, the stool is hard, fragmented, covered with mucus. Often a false diarrhea can occur, because after the emission of hard stool, will appear the emission of a liquid stools, which is characteristic for colonic irritation. Diarrheal stools occur most often in the form of compelling stools. Diarheal stools appear more often in the morning, postprandial or at emotions;
• Emission of mucus is common and accompanies the stools. In the clinical picture of irritable bowel syndrome, blood does not appear in stool, hard stools can create anal fissures that will bleed;
• Bloating is common in patients with irritable bowel syndrome, the patient feels the bloating diffuse or localized in certain areas of the abdomen. Gas emissions may ease in a transitory way the suffering of the patient.

Diagnosis

The diagnosis of irritable bowel syndrome is made by excluding organic disease of the colon, so based on laboratory exploration.

There are certain criteria that suggest the diagnosis of irritable bowel syndrome called Manning criteria, these are:

1. abdominal pain that fails after the the emission of stools;
2. stools become more frequent and softer in the presence of pain;
3. bloating, abdominal distension;
4. sensation of incomplete evacuation of the rectum;
5. elimination of mucus in the stool;
6. imperative criteria of bowel movement.

Paraclinical examination

Paraclinical examination in irritable bowel syndrome are needed to exclude abdominal organic disease and consists of:

• Anoscopy, rectoscope, colonoscopy to detect colon organic pathology;
• Gastroscopy to exclude gastric pathology;
• Pelvic and abdominal ultrasound to exclude gallbladder, pancreas or genital pathology;
• Radiological evaluation of the intestine (entero-enema or barium-passage) or enteroscopy to exclude enteral pathology.

The diagnosis of irritable bowel syndrome is put on the exclusion of organic lesions in paraclinical explorations, and the Manning criteria for irritable bowel syndrome.

Evolution

Evolution of irritable bowel syndrome is favorable because complications will not arise. In general the disease evolves with time long quiet periods, accompanied by exacerbations, which are usually related to stress. There are some situations in which the colon diverticulosis is associated with irritable bowel syndrome.

Treatment

The treatment of irritable bowel syndrome is generally difficult and the results are often not the expected. Being a functional pathology, the mental component is important, so the role of psychological balancing is also important.

• Diet should be a high-fiber, if constipation is predominant. If the diet is not sufficiently to combat constipation, than should be used laxatives that are growing the volume of the stool. It should be indicated a diet that the patient tolerates and the patient must avoid foods that cause symptoms;
• Antidiarrheal, in cases of diarrhea;
• Antispasmodics indicated for pain control, should be administered only if are necessary;
• Sedatives. Sedative medication is as useful as psychotherapy. Often rule out the diagnosis of colon cancer (the patient imagines it), can lead to symptoms improvement;

In the irritable bowel  syndrome, diet and drug therapy are individualized, and the role of patient trusting in his physician is very important.

Kidney Cancer – Causes, Symptoms Diagnosis And Treatment

Kidney cancer is also called hypernefrom, clear cell carcinoma or Grawitz tumor. Represents 3% of adult cancers and 85% -90% of renal tumors. Most commonly affects the fifth and sixth decade of life, male / female ratio being 2 / 1.

Causes

Reliable cause of kidney cancer is unknown, but have been emitted etiological hypotheses that include occupational and environmental exposures, diet and chromosomal abnormalities. They were discussing of a familial kidney cancer, and one that accompanies congenital afflictions: Hippie-Lindau syndrome (it was found that 2/3 of patients with this disease develop multiple renal caner). Genetic studies have shown changes in chromosomes 3, 8 and 11, both in the hereditary kidney cancer and in the sporadically form of kidney cancer.

Kidney Cancer

There are several risk factors involved in the development of kidney cancer:

• Smoking;
• Obesity;
• Hypertension;
• Nephropathy due to analgesic abuse;
• Genetic factors;
• Occupational exposure to cadmium;
• Gained polycystic kidney disease.

Kidney Cancer

Morphopathology

Kidney cancer develops from the epithelium of renal proximal tubules. It appears with equal frequency in both kidneys, typically in one of the kidney poles. It develops from renal coretx with continuous increase in the tissue surrounding the kidney or renal pelvis, where it can open.

Macroscopically, kidney cancer is a tumor that usually appears as a mass of variable size (3 cm to 15 cm), rounded or lobulated which protrudes on the surface of the kidney and is well demarcated. On the surface section has an orange – yellow or whitish – gray or brown color, with bleeding, necrotic and cystic areas. It gives the false impression of encapsulation by compressing the renal tissue. An important feature is the tendency of the tumor to invade the renal vein and by producing a solid column in this vessel, invade the inferior vena cava, and then giving the lung and bone metastasis.

Microscopically, kidney cancer is usually a mixed adenocarcinoma, which contains clear cells and granular cells. Clear cells are round or polygonal with abundant cytoplasm that contains cholesterol, triglycerides, glycogen and fat. Granular cells contain less glycogen and lipid, and electron microscopy shows a large number of mitochondria.

Kidney Cancer

Pathogenesis

Kidney cancer is a vascular tumor with a tendency to expand either by direct invasion through the renal capsule in the surrounding tissue of the kidney or extending into renal vein. Approximately 1/3 of patients are already diagnosed with metastasis. The most common location of metastasis is in the lung, liver, bone, regional lymph nodes or the other kidney.

Symptoms

Symptoms of kidney cancer can be polymorphic, delaying the diagnosis. Classic triad is represented by:

• Macroscopic hematuria;
• Pain in the flank;
• Palpable tumor mass, which represents an advanced manifestation and occur in 10% – 15% from cases.

Sometimes the first symptoms may be caused by metastasis, such as dyspnea or cough, and bone pain.

Paraneoplazic syndrome

There may be associated with kidney cancer: poliglobulia, hypertension, hypercalcemia and non-metastatic hepatic dysfunction.

Poliglobulia may occur in 10% of patients, the consequence of eritriopoetin secretion from the tumor or because of regional renal hypoxia.

Hypertension occurs in 40% of cases, possibly through the release of renin by the tumoral tissue.

Hypercalcemia occurs as a consequence of the release of parathyroid-like protein from the tumor or bone calcium mobilization in case of metastases.

Non-metastatic hepatic dysfunction (Stauffer syndrome), with increased alkaline phosphatase and increased bilirubin, hypoalbuminemia, prolonged prothrombin time, hyper-gammaglobulinemia, is possibly caused by one tumoral product which is hepatotoxic.

The presence of paraneoplazic syndrome is not a factor of poor prognostic  and its persistence suggests the existence of metastasis after nephrectomy.

Kidney Cancer

Paraclinical investigations

1. Laboratory – besides the changes described above, may occur anemia, hematuria and ESR acceleration.
2. Renal Echography – passed on the first place in detecting and assessing kidney cancer. Kidney cancer occurs as a formation of mixed echogenicity in one renal pole, typically round, with possible calcifications or necrosis inside. Echography, besides location and dimensions, may reveal the existence of mobility of the kidney and associated adenopathy.
3. CT scan – confirm the diagnosis of kidney cancer, contributing to the staging by viewing renal hilum, the tissue surrounding the kidney, renal vein and inferior vena cava visualization  and regional adenopathy visualization.
4. Intravenous urography – may be view the central or peripheral calcifications in direct radiography and urography may reveal changes which are made by a expansive process: amputation of an renal calyx, increasing the distance between the calyx. Sometimes kidney may be radiologicaly muted by parenchyma damage or compression.
5. Magnetic resonance imaging – is a diagnostic method of kidney cancer which is superior than  CT scan.

Kidney Cancer

Treatment

The only possible and necessary treatment for kidney cancer is surgical and consists in total nephrectomy with respecting the oncological principles. Adjuvant methods such as radiotherapy, chemotherapy and hormone therapy were less effective and sometimes are used with palliative purpose.

Ankylosing Spondylitis – Symptoms, Diagnosis And Treatment

Ankylosing spondylitis is a chronic inflammatory systemic disease, with incompletely elucidated etiology, which typically installs in the sacroiliac joints (sacroiliitis) and those of the spine, with evolution to fibrosis, ossification, strain and ankylosis. Peripheral joints and extra-articular structures may be affected, but less frequently. The prevalence of ankylosing spondylitis in Caucasians is 0.5% -1%, with male / female ratio of 3-4/1. Illness begins most frequently in the second, the third and fourth decade of life.

Ankylosing Spondylitis

Ankylosing Spondylitis Causes

So far, different studies did not clear the etiologic agent of ankylosing spondylitis. However, some characters of ankylosing spondylitis are common with those of arthritis in some inflammatory bowel diseases (ulcerative colitis, Crohn’s disease). Elevated titers of anti-Klebsiella pneumoniae antibodies and coprocultures which are frequently positive for enteric germs represent arguments in favor of the role of intestinal microorganisms in producing ankylosing spondylitis.

The genetic predisposition is supported by familial aggregation of cases of ankylosing spondylitis and high frequency with which is met Class I histocompatibility antigen, HLA-B27.

Ankylosing Spondylitis Pathogenesis

There are some arguments that support the role of immune mechanism:

• histological picture of inflammatory process rich in CD4 +, CD8 + lymphocytes and macrophages;
• increased levels of tumor necrosis factor ?  (TNF ? );
• elevated levels of immunoglobulin A.

Morphopathology

Two are the main morphological elements: chronic joint inflammation and peri-articular inflammation and entezitis (localized inflammation at the site of insertion of ligaments, aponeurosis and articular fascia).

• Sacral-iliac joint (sacroiliitis) is the main location of disease, being precocious affected and is characterized by the presence of subchondral granulation tissue, which leads to cartilage erosion of the iliac and sacred cartilage. Subsequently, occurs the replacement of granulation tissue with regenerative fibro-cartilage, followed by the ossification of the joint;
• The joints of the spine: first, there is an inflammatory granulation tissue, located in the insertion of the fibrous ring of inter-vertebral disc, on the edge of inter-vertebral body. The edges of the vertebral body and the peripheral region of fiber ring will be eroded and then will calcify, creating the syndesmophyte;
• Anterior inter-vertebral ligament is suffering a process of inflammation, then will appear fibrosis and  finally will be calcified;
• Peripheral joints (hip, shoulder, knee) are less interested;
• The eye is affected at 20% -35% of patients with the appearance of irides inflammation and iridocilities;
• The heart: aortic valve thickening and fibrosis of excito-conductive system;
• Renal damage, more rare, it is represented by nephropathy with immunoglobulin A;

Ankylosing Spondylitis

Ankylosing Spondylitis Symptoms

The onset of disease, most commonly in young men (between 20 and 40 years), is dominated by lumbosacral or buttock pain, with a character of inflammatory pain:

• Insidious onset
• Pain that appears in the second part of the night, waking up sometimes the patient from sleep;
• accompanied by morning stiffness;
• Improvement after exercise.

Sometimes back pain is radiating to the sciatic nerve path, until the popliteal space, with an alternate character.

During the status period, patients with ankylosing spondylitis present symptoms with progressive, upward character: lumbar, thoracic and cervical:

Articular manifestations of ankylosing spondylitis:

• Increased lumbosacral pain;
• Tenderness at pressing the sacroiliac joint;
• The appearance of thoracic spine pain, exacerbated by coughing or sneezing;
• Limitation of lumbar spine mobility in sagittal plan, evidenced by measuring the finger-ground index and Schober’s maneuver (in healthy individuals during maximal flexion of the spine, the distance between two points, one located at the spinous apophysis of L5 vertebra and another 10 cm above increases by more than 5 cm, while in a patient with ankylosing spondylitis, this distance increases by less than 5 cm or does not modify);
• Limitation of lateral flexion of the spine;
• reducing the mobility of the spine, put out by maneuvers: chin-sternum, occiput-wall, lateral tilt and rotation of the head.

Ankylosing Spondylitis

Extra-articular manifestations in ankylosing spondylitis:

• cardiovascular impairment: aortic regurgitation. AV block;
• pulmonary fibrosis;
• inflammatory lesions of the colon;
• renal impairment: amyloidosis or immunoglobulin A nephropathy;
• neurological manifestations: horse tail syndrome.

Advanced stage is installed, usually late. The column becomes rigid, the lumbar lordosis is erased, thoracic segment of spinal cord get a cyphotic position, and the head is bent, with the eyes directed to ground.

Ankylosing Spondylitis

Ankylosing Spondylitis Diagnosis 

Paraclinical Examinations

Radiological explorations are the most important for the diagnosis of ankylosing spondylitis.

Bilateral sacroiliitis is the main radiological criterion of diagnosis and has four stages:

• Stage I: discrete and wiped aspect of the joint
• Stage II: minimum sacroiliitis characterized by enlargement of the joint space, due to subchondral erosions on the both sides
• Stage III: moderate sacroiliitis, manifested by osteocondensation with decrease of joint space
• Stage IV: stiffness of the sacroiliac joint.

Vertebral radiological changes:

• the presence of the syndesmophytes (calcification of fibrous rings);
• square appearance of the the vertebrae, lumbar X-ray (due to the erosion of the anterior margins of the vertebral body);
• straightness of the lumbar spine;

Other imagistic explorations:

• computerized axial tomography, allows an early diagnosis of the articular affection, in the absence of radiological changes;
• MRI is extremely useful for viewing arahnoidiandiverticula, associated with horsetail syndrome;
• scintigraphy using technetium.

Ankylosing Spondylitis

Biological tests:

• ESR acceleration;
• increased C reactive protein;
• increased fibrinogen;
• increased alpha-2 globulin;
• increased immunoglobulin A and immune complexes;
• the absence of rheumatoid factor.

Diagnosis of ankylosing spondylitis is based on three clinical and one radiological criterion, called Van Der Linden criteria:

Clinical criteria:

1. Lumbar pain accompanied by stiffness, at least three months, reduced by exercise and unimproved by rest;
2. Limitation of the movement of the spine in frontal and sagittal plane;
3. Limitation of chest expansion.

Radiological criterion:

Bilateral sacroiliitis grade II-III or unilateral sacroiliitis grade III-IV.

The presence of sacroiliitis, together with one of three clinical criteria allows diagnosis of ankylosing spondylitis.

Ankylosing Spondylitis

Ankylosing Spondylitis Treatment

Treatment of ankylosing spondylitis aims to reduce inflammation, maintain spinal mobility and prevent stiffness.

Pharmacological treatment with NSAID aims to reduce inflammation, pain and paravertebral contraction:

• indometacin is the moast efficient drug, in doses of 75-100 mg / day;
• oral corticosteroids have a low efficiency in the treatment of ankylosing spondylitis. They are useful in intra-articular administration;
• in severe cases of ankylosing spondylitis : sulfasalazine, methotrexate or cyclophosphamide.

Nonpharmacological treatment is represented by: medical gymnastics, hydrotherapy and practice of sports such as swimming.

Orthopedic and surgical treatment is only recommended in debilitating forms of ankylosing spondylitis and involves performing osteotomies and arthroplasties.

Evolution and prognosis of ankylosing spondylitis:

In most cases, ankylosing spondylitis has a long evolution, with spurts of activity and remission. In severe cases with a onset in adolescence or in case of untreated ankylosing spondylitis, progression is rapid, to stiffness of several joints, giving the patient the appearance which was described in the advanced stages of ankylosing spondylitis.

Polymyositis and dermatomyositis are inflammatory myopathies, usually idiopathic, characterized by diffuse inflammation of striated muscle, expressed clinically by muscle fatigue which has proximal location (in polymyositis) associated with skin rash (in dermatomyositis). Polymyositis and dermatomyositis are rare diseases that can occur at any age, usually between 5 and 15 years in children and among adults between 40 to 60 years. This disease is twice as common in women than in men.

Bohan and Peter proposed the following classification of inflammatory myopathies:

• Type I: Adult idiopathic polymyositis;
• Type II: Adult idiopathic dermatomyositis;
• Type III: dermatomyositis or polymyositis associated with neoplasia;
• Type IV: dermatomyositis or polymyositis of the children associated with vasculitis;
• Type V: polymyositis and dermatomyositis associated with other connective tissue diseases (overlap syndromes);
• Type VI: inclusion-bodies myositis;
• Type VII: eosinophilic myositis, giant cell myositis and ossificant myositis.

Pathogenesis

The pathogenesis is incompletely known, but assume that different causal factors are involved, acting through autoimmune mechanism.

Genetic susceptibility by implication of genes like HLA DR3 and HLA DRW52;

Retrovirus infections, especially Epstein-Barr and Coxsackie. These viruses appear to initiate an autoimmune myositis by molecular mimetism with some muscle antigens.

Pathogenic mechanism is immunological. Antigens against which triggers the autoimmune response are unknown, probably of muscular and microvascular nature.

Antibodies have uncertain role and are represented by:

• cytoplasmic antibodies: anti-RNA t synthetase, anti-SRP;
• nuclear antibodies: Anti I-2.

Symptoms

Muscular manifestations: muscle fatigue accompanied by muscle weakness, usually painless, is the main symptom. It occurs slowly, is symmetrical and can affect any muscle. Appears initially at the proximal segment:

• pelvic belt: is interested in more than 90% of cases and lead to failure in flexion of the copse on the abdomen and difficulties in climbing stairs;
• scapular belt: is affected in more than 85% of cases and makes it difficult to raise arms and combing;
• flexors muscles of the neck: raising the head with difficulty from the pillow and difficulty in maintaining orthostatic position.

Skin Manifestations are present in dermatomyositis and are expressed by:

• heliotrope rash (occurs after exposure to the sun) is located on the face (periorbital red rash with swelling of the eyelids), neck (shaped “V” erythema) and shoulders ( erythema in the form of a scarf);
• Gottron sign: maculopapular lesions, consisting of prominent red, scaly spots, which are localized on the dorsal part of the proximal interphalangeal and metacarpophalangeal joints, elbows, knees and external malleolus;
• nail telangiectasia;
• calcifications of soft tissues (skin, subcutaneous tissue and fascia);
• hands with irregular, thickened and cracked skin.

Articular Manifestations: arthralgia, arthritis which is not erosive and deforming.

Gastrointestinal disorders: dysphagia by involving striated musculature of the oropharynx and upper esophagus (this is a sign of poor prognosis) and gastro-intestinal ulcers.

Cardiac and pulmonary manifestations are less common than in other connective tissue diseases and occur in severe forms of disease. May occur arrhythmias, myocarditis, heart failure, pneumonia and respiratory muscle damage.

Cancers are more common in patients with dermatomyositis: ovarian cancer, breast cancer, colon cancer, lung cancer and melanoma.

Paraclinical examination

• Increased muscle enzymes: CPK, CPK-MM, ALT, AST, LDH. CPK is the most sensitive, it can increase in the active form of the disease up to 10-50 times and evolve parallel with disease activity;
• Electromyography shows suggestive changes of inflammatory myopathy: spontaneous fibrillation at rest, action potential of short duration and small amplitude produced by muscle contraction;
• Muscle and skin biopsy allow the histopathologic diagnosis.

Diagnosis

The diagnosis of polymyositis and dermatomyositis is based on laboratory explorations and on Bohan and Peter criteria for inflammatory myopathies. The criteria are:

1. Symmetric weakness in the belts, neck and extremities with progressive decline within a few weeks or months, which may be associated with dysphagia or respiratory muscle involvement;
2. Increased muscle enzymes: CPK, CPK-MM, ALT, AST, LDH;
3. Electromyographic abnormalities;
4. Muscle biopsy: inflammation, necrosis, regeneration, atrophy.

If there are four criteria the diagnosis is certain, if three criteria are present then the diagnosis is probable and if  two criteria are present then the diagnosis is possible.

Treatment

Therapy aims to suppress the inflammatory process in order to reduce destructive extensive muscle. The treatment depends on the clinical presentation and is done in steps:

• Step I: high-dose of prednisone;
• Stage II: azathioprine or methotrexate;
• Stage III: intravenous immunoglobulin;
• Stage IV: cyclosporine, chlorambucil, cyclophosphamide and mycophenolate.

In exacerbations of disease is recommended bed rest. The surgical treatment is offered only if the cancer is associated and consists of removing the tumor, thereby improving symptoms or even cure the disease.

Mitral Stenosis

Mitral valvular stenosis is characterized by the reduction of mitral orifice area (normally has 4-6 cm2), which is a dam in blood flow from the left atrium to the left ventricle during diastole. Almost all types of mitral stenosis appear as a complication of rheumatic fever. In rheumatic mitral stenosis are characteristic the following pathological changes:

• Valvular narrowing, by the join of the corners;
• Fibrosis, thickening and calcification of the valve;
• Thickening of the pillars and cordages.

mitral-stenosis

Reduction of mitral orifice area determine left atrial dilatation, thickening of the pulmonary veins and pulmonary capillaries, dilation and proliferation of intima and media of pulmonary artery and dilatation and hypertrophy of the right ventricle and right atrium. Mitral Stenosis has hemodynamic consequences in both downstream and upstream:

1. Downstream occur: decreased cardiac output, which lowers the coronary flow;
2. Upstream, the pressure increases in:

• Left atrium, with its hypertrophy and dilation, which favor the occurrence of arrhythmias (atrial fibrillation);

• Pulmonary vein, where the blood stagnates;

• Pulmonary capillary, causing the occurrence of effort dyspnoea, acute pulmonary edema, chronic interstitial edema and pulmonary sclerosis;

• Pulmonary artery, causing its expansion;

• Right atrium and right ventricle, causing their dilatation and hypertrophy with the installation of right heart failure;

Depending on the degree of narrowing, mitral stenosis is classified in:

• Large mitral stenosis (between 2.5 cm2 and 1.5 cm2);
• Moderate mitral stenosis (between 1.5 cm2 and 1cm2);
• Severe mitral stenosis (less than 1 cm2).

Mitral Stenosis

Symptoms

Mitral stenosis mainly affects women, the symptoms became evident between 25 and 30 years. Symptoms depend on the degree of stenosis, the left atrial dilatation and pulmonary stasis. Symptomatology is evident when the surface of mitral hole is less than 1.5 cm2:

• Dyspnoea, the most common symptom may be mild and intermittent (occurs only in situations that increase cardiac output, such as effort and fever), chronic and severe or even permanent, paroxysmal, which is favored by factors such as exercise, excitement, sexual intercourse , labor, menstrual period and pregnancy;
• Dry, persistent cough, caused by compression of the dilated left atrium on the bronchi;
• Palpitations, as expression of compensatory tachycardia occurred during exercise or as expression of arrhithmias;
• Hemoptysis;
• Pain in the left interscapular region;
• Dysphagia due left atrial compression over the esophagus;
• Dysphonia
• Fatigue in the legs.