Respiratory System Infections: Acute Bronchiolitis PDF

Respiratory System infections Acute Bronchiolitis Definition: Inflammatory obstruction of small airways (bronchioles) occurring during Is' two year with peak incidence at 6 months. Etiology: Respiratory syncytial virus (RSV) is the most common cause (80%)...

Respiratory System infections Acute Bronchiolitis Definition: Inflammatory obstruction of small airways (bronchioles) occurring during Is' two year with peak incidence at 6 months. Etiology: Respiratory syncytial virus (RSV) is the most common cause (80%) Para influenza virus, adeno virus and measles virus. Pathogenesis: Invasion of bronchioles by the virus lead to bronchiolar wall inflammation and edema lead to partial bronchiolar obstruction lead to air flow without outflow lead to hyperinflation ( emphysema) lead to defect in gas exchange with retention of CO2 (hypercapnia) and decrease in PaO2 ( hypoxia). Video link: Understanding Bronchiolitis Clinical Picture: Symptoms: J History of exposure to adult with resp. syn. Viral infection V Infant develops fever, rhinitis for 2-3 days. V Cough and shortness of breathing with wheezes develop after. Signs: V Respiratory distress: Tachypnea, retraction, grunting with or without cyanosis V Auscultatory findings: Diffuse wheezes, diminished air entry, fine crepitation may be heard 10 Complication: J Respiratory failure J Heart failure (viral myocarditis) J Dehydration J Metabolic acidosis S 2ry bacterial infection Diagnosis: J Chest X-ray shows hyperinflation J Nasopharyngeal aspirate for detection of RS V antibodies Differential diagnosis: DD of wheezy chest as bronchial asthma. S It may be difficult to differentiate asthma from bronchiolitis by physical examination, but age of presentation, presence of fever, and absence of personal or family history of asthma are the major differential factors. J Bronchiolitis occurs primarily in the first year of life and is accompanied by fever, whereas asthma usually presents in older children with previous wheezing episodes. Treatment: J Humidified oxygenation by nasal cannula or oxygen mask J Hydration with IV fluid or nasogastric tube J Nebulized hypertonic saline: absorption of fluid of bronchial edema J Bronchodilator may be effective J Steroids: oral or parenteral S Antibiotic: only for secondary bacterial infection 11  J Antiviral for high risk group (premature - cong. heart disease) S Endotracheal intubation and mechanical ventilation in respiratory failure. Prophylaxis: J By RSV antibodies J Given only for high risk group  Pneumonia Definition: Inflammation of the lung parenchyma associated with alveolar consolidation Pneumonia is the leading cause of death globally among children younger than age 5 yr. Clinically, pneumonia may occur either as a primary disease or as a complication of another illness. Small Airway With Pneumonia (»cmisr White Blood Cells, and Mucus https://www.cl1iidrensdayt0n.0rg/kidshealth/a/az-pneuIT10nia-bacterial Classification: A- Anatomical classification: Lobar pneumonia: All or a large segment of one or more pulmonary lobes is involved. When both lungs are affected, it is known as bilateral or double pneumonia. Bronchopneumonia: Begins in the terminal bronchioles, which become clogged with mucopurulent exudate to form consolidated patches in nearby lobules; also called lobular pneumonia.  Interstitial pneumonia: Inflammatory process more or less confined within the alveolar walls (interstitium) and the peribronchial and interlobular tissues. B- Etiological classification: 1- Infection: Bacterial: (pneumococci, Streptococci, Staphylococci, Hemlophilius influenza) Non-bacterial: - Viral (RSV) - Mycoplasm - Parasitic - Fungal 2- Non infectious: Aspiration Hypersensetivity Pathological Changes in Tissues Starts with inflammation Congestion: reactive edema of alveolar walls + accumulation of phagocytic cells--thickening of alveolar walls-----------decrease gas exchange--------- hypoxia and acidosis-------------- fast breathing. Two days later, alveoli sac become full of inflammatory exudates consolidation----------crepitation is heard on auscultation. Exudates is transformed into pus which fills the alveoli------------ decreased elasticity and no room for air---------- decrease pressures in alveoli breathing movement will leads to expectoration and chest indrawing. 14 Clinical picture: A- Signs: (Signs of respiratory distress) - Tachypnea - Intercostal and subcostal retraction - Grunting and cyanosis in sever cases B- Symptoms: - Fever, cough and shortness of breathing - Vomiting and diarrhea may be associated C- Chest finding: - Lobar pneumonia: Dullness on percussion - Broncho pneumonia: fine crepitation - Interstitial pneumonia: minimal chest finding with sever resp. distress Diagnosis: CBC: increase WBCs suggest bacterial infection, lymphocytosis suggest viral infection, eosinophilia suggest hypersensitivity pneumonia. CRP: +ve in bacterial and -ve in viral pneumonia. Chest X-ray Culture: blood, sputum or bronchial aspirate, positive only in 10-15% of cases Treatment: A- General: Hospitalization for all who appear toxic, dyspneic or hypoxic Oxygenation if there is respiratory distress IV fluid Control of fever by antipyretics 15 B- Empirical: (determined by age, severity of illness and X-ray findings) 1- Non hospitalized (less sever pneumonia): J Oral amoxicillin for 5-7 days or oral macrolides for child more than 5 years 2- Hospital treatment ( Sever pneumonia): J Stat with combination of parentral antibiotic for 10-14 days Complications: Parapneumonic effusion Empyema. Tension pneumothorax. Bronchiectasis Lung abscess Bronchiolitis Video link: pneumonia 16  Cardiovascular Disorder Classification of cardiovascular disorders in children: (I) Congenital Heart disease: (A) A cyanotic heart disease: 1- With shunt: Atrial Septal Defect Ventricular Septal Defect Patent Ducts Arterioles 2- Without shunt: Aortic co-arcetation Aortic stenosis Pulmonary stenosis (B) Cyanotic Heart disease: 1- With pulmonary oligeamia Fallot's tetralogy Pulmonary atresia Tricusped atresia 2- With pulmonary plethora Transposition of great arteries Single ventricle Total anomalies of pulmonary veins (II) Acquired heart disease: Acute rheumatic fever 17  1) Congenital Heart Diseases (C.H.D) Etiology The etiology of most C.H.D. is unknown, but several factors associated and increase the incidence of the disease such as: 1 - Maternal rubella during pregnancy. 2- Maternal alcoholism, age over 40 yrs., and insulin dependent diabetes. 3- Hereditary Factors. 4- Exposure to radiation in the first trimester. Ventricular septal defect (VSD) https://pediatricheartspecialists.com/heart-education/15-congenital-heart-defects/190- ventricular-septal-defect-vsd Hemodynamics: Blood is shunted from Lt Ventricle to Rt ventricle through VSD lead to Rt ventricle dilatation. The excess blood passes to pulmonary artery to lung to pulmonary veins to Lt atrium to Lt ventricle It leads to: J Rt ventricle, Lt atrial and Lt ventricle enlargement. J Pulmonary artery dilatation and lung plethora 18 Types: 1- Small VSD: usually in the muscular part of the septum 2- Big VSD: usually in the membranous part of the septum Clinical picture: J Absent symptoms in small VSD J Loud murmur on lower left sternal border V Slow weight gain, failure to thrive & feeding difficulties V Tachypnea V Recurrent respiratory infection S Pallor and diaphoresis V Pulmonary artery enlargement, cardiomegaly & biventricular hypertrophy & left Atrial enlargement Investigation: X-ray chest: Biventricular enlargement and lung plethora ECG: BVH ECHO: VSD Cardiac catheterization: for more assessment Treatment: A- Medical treatment: V Chest infection by proper antibiotics V Heart failure: digoxin. Lasix V Prophylaxis for infective endocaradities B- Surgical: Small VSD: (less than 5 mm) Close spontaneously within 1st two year of life 19  Large VSD: Surgical closure at 2- 4 years Tetralogy offallot (TOF) https://www.heart.oig/en/hcalth-topics/congenital-heait-defects/about-congenital- heart-defects/tetralogy-of-fallot The most common congenital cyanotic heart disease. It consists of: J Pulmonary stenosis V VSD V Overriding aorta S Rt. Ventricular hypertrophy Hemodynamics: Venous return passes into Rt atrium to Rt ventricle to VSD to aorta to systemic circulation bypassing the lung. Only small amount of blood passes from Rt ventricle to pulmonary artery to the lung lead to decrease pulmonary blood flow (lung oligeamia). Clinical picture: Cyanosis: The neonate who has tetralogy of Fallot is not cyanotic, because of the presence of patent ductus arteriosus, 20  cyanosis becomes evident after the ductus closes i.e.: after first months of life. Dyspnea: usually exertional dyspnea Clubbing: at age of 1-2 years Anoxic Spells: due to pulmonary stenosis and characterized by: Deep cyanosis Sever dyspnea Irritability Improved by Squatting (knee -chest) position Complication: Anoxic Spells Cerebral infarction: due to brain anoxia Brain abscesses: due to failure of blood filtration by lung Infective endocarditis Growth retardation: due to chronic hypoxia Treatment: A- Medical treatment Treatment of anoxic spells: o Knee chest position o Oxygenation o Correction of acidosis o Treatment of dehydration B- Surgical treatment: 1- Palliative: pulmonary systemic shunt to maintain pulmonary Blood flow and oxygenation. 2- Total surgical correction: at 4-12 months  Acquired heart diseases Acute Rheumatic Fever (A.R.F.) Definition: Is an inflammatory disease of connective tissues involving mainly joints & heart. Incidence More common between the school age children i.e. between 5-10y, boys & girls are equally affected, and with the incidence is still high in the developing countries due to: Overcrowded living conditions. Poor socioeconomic and educational level. Etiology Autoimmune reaction to group A-Beta hemolytic streptococcal (GABHS) that cause upper respiratory tract infection. Pathophysiology R.F. develops in 3 phases: Phase 1: GABHS infection of the URT (pharyngitis). Phase 2: asymptomatic (the symptoms of URTI subside quickly). Phase 3: onset of acute R.F. Hypotheses 1- Hypothesis of abnormal immune response to GABHS: It assumes that: A genetically predisposed person acquires GABHS infection, reacts abnormally, this reaction takes 1-3 weeks, the 22  resulting antigen-antibody complexes will cause the immunological damage to the connective tissues of heart and joints. 2- Hypothesis of autoimmunity: It assumes that: A genetically predisposed person acquires GABHS infection, antigen-antibody reaction that may elicit antibodies capable of reacting not only with the microbial products, but also with the host’s antigens (anti-heart). Thus autoimmunization may be the underlying basis for the cardiac lesion. Assessment 1- Accurate history: It should be taken from parents and child for recent exposure to streptococcal infection. 2- Clinical manifestations: These are divided into: a) Minor manifestations Low grade fever (38-39°C), arthralgia (painful joints), unexplained epistaxis, abdominal pain, weakness, fatigue, pallor, loss of appetite and wt. loss. b) Major manifestations 1- Carditis Mitral & aortic valves are most effective valves. The valve edges become scarred & fibrous, so that they cannot completely close; causing regurgitation when valves close & the following manifestations appear: Tachycardia, Cardiomegaly 2- Migratory polyarthritis Most common criterion occurs in 70% of cases, it lasts for a few days in certain affected joint(s) before moving to other joint(s). 23  The affected joint is red, warm, swollen, and so tender, with limited movements with or without effusion. Several joints are commonly involved, either together or one after another. Typically affect the large joints, as: Knee, ankles, wrists, & elbows. 3- Erythema marginatum It is non-pruritic undifferentiated macules that appear on the trunk and inner surface of the extremities, but never on face. Gradually disappear over a short period of time. 4- Rheumatic chorea Sudden, aimless, involuntary and irregular muscular movements of the extremities, that appears in the form of: Exaggerated muscle movements by anxiety that relieved by rest. Involuntary facial grimaces. 5- Subcutaneous nodules Small nodules that are generally identified as firm without any tenderness or attachment to the skin. They can be palpated over the extensor surfaces of the joints such as elbows, knees, & ankles, or over the scalp and spinous processes of the vertebrae. Gradually resolves over a period of time with no residuals. Diagnostic evaluation To diagnose R.F. the child should have: Two major criteria & evidence of preceding GABHS infection. Or One major, 2 minor criteria, & evidence of preceding GABHS infection. Elevated Antistreptolysin-0 Titer (A.S.O.T.) which is streptococcal antibodies. 24  Increase Erythrocyte Sedimentation Rate (E.S.R.). Increase C-reactive protein. Electrocardiograph. Radiographs. Therapeutic management aimed to: Eradication of group A-beta hemolytic streptococcus infection. Give anti-inflammatory agents for management of carditis, & arthritis. Eradicate the other symptoms. Prevent recurrence of R.F. Penicillin: is the drug of choice to treat group A-beta hemolytic streptococcus infection Give erythromycin as Penicillin substitute for a child who have penicillin allergy. Salicylates: used to control fever & pain and inflammatory process in the joints. Bed rest: is recommended during the acute febrile phase. PrPPkyl^tic_treatment Stall after the acute therapy. It involves monthly IM injection of Benzathine Penicillin (1-2 million IU), or two daily oral doses of Penicillin or Erythromycin (400.000 fU), or one daily oral dose of Sulfadiazine. Video link: Rheumatic Fever Lecture record: https://drive.google.eom/rile/d/lRkziizPFrCR41YipJZrMAP20TBZVzVgN /view 25  Gastrointestinal and hepatic disorders Jaundice in newborn Definition: Yellowish discoloration of the skin and mucus membrane due to the accumulation of bilirubin in these tissues. ♦♦♦ Almost all neonates (Approximately 60 % term and 80 % preterm infants) present with jaundice in the first week of life. If bilirubin levels remain high, infants are at risk of bilirubin-induced neurological dysfunction. Pathogenesis Bilirubin is produced from the breakdown of heme proteins. Afterward, it is transported to the liver with the help of albumin. In the liver, it undergoes conjugation to form water-soluble forms that are excreted through urine and stool..Types: (I) Physiological jaundice: most common, due to: J Increase RBCs volume with short life span J Immaturity of glucoronyle transferase enzyme Characters: Appears on 2nd or 3rd day of life Persist for 1 week in full term and 2 weeks in preterm Total bilirubin is not more than 12mg/dl in full term and 15mg/dl in preterm Rate of daily raise in bilirubin not excess 5mg/dl Good general condition of baby 26  (II) Pathological jaundice: 1- Unconjugated hyperbilirubinemia: A- Hemolytic: S Immune: Rh and ABO incompatibility J Non immune: Infection as septicemia, G6PD and Thalassemia B- Non Hemolytic: Breast milk jaundice 2- Conjugated hyperbilirubinemia: A- Hepatocellular: neonatal hepatitis and metabolic liver disease B- Biliary: Bile duct atresia Treatment: A- Phototherapy: Exposing the skin of the baby to blue light waves. It converts the bilirubin to water soluble metabolite which can exerted easily through bile and urine. Indication: In case of un Conjugated hyperbilirubinemia in which the serum bilirubin is not too high to produce kernicterus. Complication: S Hyperthermia J dehydration S Diarrhea J Eye injury B- Blood exchange: Removal of a volume of infant's blood and replacing by an equal volume of donor blood through umbilical vein catheter over period of 2 hr. Amount^ 2x 85 x body weight 27 Complications: Bilirubin is a potential neurotoxin; free bilirubin can enter the brain and cause necrosis of cells. Bilirubin induced neurologic dysfunction is divided into acute and chronic forms (kemicterus). Acute BIND progresses through three stages early, intermediate, and advanced. Acute BIND can be reversible if treated promptly or it may lead to chronic permanent neurological dysfunction (kemicterus). Kernicterus is the yellowish staining of brain stem nuclei and cerebellum. It develops within the first year of age. It leads to cerebral palsy, hearing loss, upward gaze abnormalities, and dental enamel hypoplasia. Video link: Neonatal Jaundice 28 Diarrhea Definition: An increase in the fluidity, volume and number of stools relative to the usual habits of each individual". Frequent passage of formed stools can't be considered as diarrhea. Classification of diarrhea: 1- Acute diarrhea It defined as a sudden increase in frequency and a change in consistency of stools and is usually self-limited (14 days’ duration) and subsides without specific treatment if dehydration does not occur. Causes of acute diarrhea: A. Infection and Parasitic Infestation as : Bacteria—Salmonella, Shigella, Staphylococcus aureus Viruses—Rotavirus, parvovirus Parasites—Giardia lamblia, Entamoeba histolytica B. Associated Conditions Infection: upper respiratory tract infections, urinary tract infections and otitis media C. Dietary Causes Overfeeding Introduction of new foods Reinstituting milk too soon after diarrheal episode Osmotic diarrhea from excess sugar in formula or juice D. Medications Antibiotics - Laxatives Toxic as ingestion of heavy metals (lead, mercury) 29  2- Dysentery This is diarrhea with visible fresh blood in the stool. Its squeal includes anorexia and damage to the intestinal mucosa. 3- Chronic diarrhea An increase in stool frequency and increased water content with duration of more than 14 days. Chronic nonspecific diarrhea (CNSD), also known as irritable colon in children 6 to 54 months of age. Causes of chronic diarrhea: 1. Malabsorptive Causes as Lactose intolerance, pancreatic insufficiency 2. Allergic Causes as allergic gastroenteropathy. Eosinophilic gastroenteritis 3. Immunodeficiency: as human immunodeficiency virus or acquired immunodeficiency syndrome 4. Inflammatory Bowel Disease as ulcerative colitis. 5. Endocrine Causes as hyperthyroidism, congenital adrenal hyperplasia 6. Motility Disorders as Hirschsprung disease. 7. Parasitic Infestations as ascaris organisms and giardia organisms 8. Other Causes as radiation enteritis, abdominal tumors Complications of diarrhea: J Dehydration J Malnutrition 30  Dehydration Definition: It is caused by the loss of water and electrolytes in liquid stools and vomitins. Fever can make it worse as it causes additional loss of water. Dehydration can lead to hypovolemia, cardiovascular collapse, and death if not treated promptly. Assessment of the degree of dehydration is based on 4 signs: A B C No signs of Sings dehydration loss < Some dehydration loss Severe dehydration loss > 5% of body 5-10% of body weight 10% of body weight weight G-General Well and alert Restlessness and Lethargic, floppy condition irritable unconscious Normal Sunken Very sunken and dry E- Eyes Drinks normally Thirsty, drinks Drinks poorly or unable to M- Thirst eagerly drink. Goes back Goes back slowly Goes back very S-Skin pinch quickly slowly (>2 seconds). Child has no If 2 or more signs are If 2 or more signs are signs of present, there is present there is severe Decide dehydration moderate dehydration. (Mild) dehydration. Select treatment Plan A Plan B Plan C plan Video link: Diarrhea and Dehydration 31  Management of Dehydration: Treatment Plan A Plan B PlanC At home In outpatient In hospital Where rehydration center Fluid Give more fluid than Gives ORS Give IV fluids therapy usual Homemade fluids Gives ORS Pansol Ringer’s lactate. (rice, water, tea Normal saline. What type without sugar, soup, and yogurt). Give after each loose 75ml/kg body weight 100 ml/kg of body wt. given stool for child 2 hr. How much years: 100-200 ml. Next 70 ml/kg given in 2.5 - 5hrs, longer time is used for infant < 1 year. (N.B*) Slowly (lspoon/1-2 Slowly (lspoon/1-2 I.V. min) min) by cup and spoon, cup alone, How given By cup, spoon, cup dropper/syringe. alone, dropper or Nasogastic tube. syringe. Feeding: breast feeding should never be stopped even during initial rehydration. Milk or milk formula given as usual (after rehydration). Soft and semisolid weaning food usually taken by the child, after rehydration (yogurt, mashed potatoes, rice pudding, cereals, vegetable soup and beans) should be given. Avoid giving too sweet foods or food with high fiber content. 32  Nutritional Disorders Rickets Introduction There are many factors contribute to children malnutrition as poor food availability jack of sanitation and recurrent infections. However, the most common and easily preventable cause is bad nutritional habits and lack of nutritional education. Rickets is a common bone disease worldwide that is associated with disturbances in calcium and phosphate homeostasis and can lead to short stature and joint deformities. Rickets can be diagnosed based on history and physical examination, radiological features, and biochemical tests. It can be classified into 2 major groups based on phosphate or calcium levels: phosphopenic and calcipenic Definition It is a systemic metabolic disease caused by deficiency of vitamin D results in inadequate deposition of calcium in developing cartilage and bone leading to bone deformities, hypotonia and sometimes affecting CNS and mostly seen between 6 months and 2 years. It is a disease of growing bone that is caused by unmineralized matrix at the growth plates and occurs in children only before fusion of the epiphyses Types of Rickets Rickets can be classified into the following: 1- Nutritional rickets Prolonged breast-feeding. Fresh cow's milk feeding Un exposure to sunlight 33 2- Non-nutritional rickets A. Inherited rickets as in Hypophosphateniic vitamin D resistant rickets Hypocalcaemic vitamin D resistant rickets Renal tubular acidosis B. Rickets with chronic diseases With chronic malabsorption With chronic liver disease With chronic antiepileptic therapy With tumors D. Conditions resembling rickets There are many causes of rickets including: J Vitamin D disorders: as Vit. D is affecting the reabsorption of calcium andphosphorus at the kidney Nutritional vitamin D deficiency Congenital vitamin D deficiency Secondary vitamin D deficiency Malabsorption Increased degradation Chronic kidney disease J Calcium deficiency Low intake Diet Premature infants (rickets of prematurity) Malabsorption Primary disease 34  Dietary inhibitors of calcium absorption Phosphorous deficiency Inadequate intake Premature infants (rickets of prematurity Akimi num-containing antacids J Distal renal tubular acidosis. S Hereditary rickets J Tumor-induced rickets Populations at risk of vitamin D-deficiency rickets include: I. Exclusive breastfed infants by mothers with an inadequate intake of vitamin D 2. Exclusively breastfed toddlers longer than 6 months without adequate maternal vitamin D intake or supplementation 3. Un-exposure to sun light especially in dark colored infants. 4. Children with diet that low in sources of vitamin D and calcium. 5. Individuals who use milk products not supplemented with vitamin D (yogurtraaw cow’s milk) as the primary source of milk Clinical Manifestations https;//www.trishlaortho.com/rickets-in-children/ 35 General J Failure to thrive J Listlessness Protruding abdomen J Muscle weakness (especially proximal) J Fractures particularly green stick fractur Head Craniotabes (Ping-Pong Ball Skull): is a softening of the cranial bones and can be detected by applying pressure at the occiput or over the parietal bones. The sensation is similar to the feel of pressing into a ping-pong ball and then releasing. Craniotabes is diagnostic criteria of rickets if detected between the 4th and 8th month of age. Frontal bossing Delayed fontanel closure Delayed dentition; caries Changes in the skull also occur causing a distinctive "square headed" appearance known as "caput quadratum". These deformities persist into adult life if not treated. Back: Scoliosis Kyphosis Lordosis Extremities Enlargement of wrists and ankles 36  Valgus or varus deformities Limb deformities as genu varum (bowlegs) or genuvulgum (knock knees) Anterior bowing of the tibia and femur Leg pain Muscular manifestations: Hypotonia of the skeletal and smooth muscles is the main manifestation and it leads to delayed motor development (delayed sitting, crawling, standing, or walking) and abdominal distension. The liver and spleen may be palpated below the costal margin due to their downward displacement secondary to chest deformities and hypotonia tJornval Eio'.degs Knock-Kne https://www.naravanahealth.org/blog/rickets-svmptoms-causes-antl-treatnient- options Neurological manifestations In early cases: anorexia, irritability and sweating may be present. In advanced cases characterized by marked calcium depletion, tetany may occur. However, tetany is considered as a complication rather than a clinical finding. Seizures 37 Diagnosis A doctor will diagnose rickets by checking for symptoms such as bowed legs or a soft skull. They may also ask about a person’s lifestyle habits, such as diet and sun exposure. To confirm a diagnosis, a doctor may recommend: Blood tests: These look for low levels of calcium and phosphorus and high levels of alkaline phosphatase. An arterial blood gas test: This checks for acidity in the blood. X-rays: These may reveal calcium loss in bones or alterations in the structure or shape of the bones. A bone biopsy: This can confirm rickets, but doctors rarely use. Prevention In most cases, people can prevent rickets by consuming enough vitamin Dand getting enough sun exposure. S 400 IU of vitamin D to infants who are breastfed. J Older children should receive 600 lU/day. Children who live in countries with low sun intensity, where there is frequent cloud cover, or where winter days are particularly short may need to take vitamin D supplements to prevent rickets. This may involve: Consuming fortified milk, orange juice, and other products rich in vitamin D Taking a daily vitamin D supplement 38  Taking an occasional high dose of vitamin D when small daily doses are not possible. Treatment J vitamin D and adequate nutritional intake of calcium and phosphorus. J There are 2 strategies for administration of vitamin D: With stoss therapy, 300,000-600,000 IU of vitamin D are administered orally.or intramuscularly as 2-4 doses over 1 day as (Devarol amp). The alternative is daily, high-dose vitamin D, with doses ranging from 2,000-5,000 lU/day over 4-6 wk as (Decal B12 syrup or Vitacal syrup or Calcical syrup). Either strategy should be followed by daily vitamin D intake of 400 lU/day if 1 yr old. A adequate dietary calcium and phosphorus. Radiological signs of healing start after 2 weeks of onset (healing rickets) and become complete after 4 weeks (healed rickets). Video link: Rickets 39  Endocrine System The endocrine system is comprised of several glands throughout the body. Glands release chemical messengers called hormones that control and regulate the activity of target cells and organs. Hormones influence growth, development, and digestion and regulate metabolism and reproduction. The endocrine system maintains homeostasis. Feedback tells a gland to increase or decrease the hormone production so the body returns to homeostasis. When the concentration of a hormone reaches a threshold, hormone productionis turned off. The thyroid gland secretes two types of hormones: thyroid hormone (TH) and calcitonin. TH is made up of the hormones: thyroxine (T4) and triiodothyronine (T3). Regulation of thyroid Hormone The hypothalamic-pituitary-thyroid axis. Thyroid-releasing hormone (TRH) from the hypothalamus stimulates the pituitary gland to secrete thyroid- stimulating hormone (TSH). TSH stimulates the thyroid to produce thyroid hormone (T3 and 40  T4). High circulating levels of T3 and T4 inhibit further TSH secretion and thyroid hormone production through a negative feedback mechanism (dashed lines). Hypothyroidism or hyperthyroidism may result from a defect in the thyroid or from a disturbance in the secretion of TSH Hypothyroidism Hypothyroidism results from deficient production of thyroid hormone, either from a defect in the gland itself (primary hypothyroidism) or a result of reduced thyroid- stimulating hormone (TSH) stimulation (central or hypopituitary hypothyroidism ) Hypothyroidism is divided into: A. Congenital hypothyroidism B. Acquired and may or may not be associated with a goiter  A- Congenital Hypothyroidism Epidemiology The prevalence of congenital hypothyroidism based on nationwide programs for neonatal screening was initially reported al 1 in 4.000 infants worldwide. Over the last 2 decades, the prevalence has dropped to 1 in 2,000, likely the result of detection of milder cases of hypothyroidism. Etiologic Classification of Congenital Hypothyroidism A- Primary hypothyroidism Defect of fetal thyroid development (dysgenesis) 1. Aplasia 2. Hypoplasia 3. Ectopia S Defect in thyroid hormone synthesis (dyshormonogenesis) S TSH unresponsiveness J Defect in thyroid hormone transport S Resistance to thyroid hormone S Maternal antibodies: thyrotropin receptor-blocking antibody J Iodine deficiency (endemic goiter) Maternal medications B- Centra] or hypopituitary hypothyroidism J TSH and TRH deficiency. J Multiple congenital pituitary hormone deficiencies (e.g., septooptic dysplasia) 42 Clinical Presentation A. In neonatal period J Positive family history of pervious affected sibling or maternal goiter J Prolongation of physiological jaundice S lethargy S Poor feeding S Constipation J A hoarse cry J Umbilical hernia J Protuberant abdomen J Mottled skin. S Bradycardia J Wide posterior fontanelle S Hypotonia with delayed reflexes B. In infancy period J Delayed motor development J Delayed mental development J Coarse features (large head, coarse hair, swollen eye lids, depressed nasal bridge, thickprotruded tongue) C. In childhood period J Short stature J Slow growth velocity J Thyroid enlargement J School underachievement and poor learning ability. Diagnosis: 1- Radiological assessment of bone age: 43  Epiphyseal dysgenesis is evidenced by classic features of stippled epiphyses on X-ray. 2- Serum level of thyroid hormones: Low serum levels of T4 and T3 is diagnostic of hypothyroidism (T4 below 6 mcg/dl). Normal level of T4 is 9-18 mcg/dl in neonates and 7-15 mcg/dl in infant and children. 3- Serum level of thyroid stimulating hormones (TSH): The peak serum concentrations of TSH at birth in full-term infants is 40 mu/ml. J High levels above 50 -100 micro unit/ml usually occur in primary hypothyroidism. In secondary and tertiary hypothyroidism the level is low. J Normal level is below 10 micro unit/ml. Treatments J Replacement hormone (levothyroxine, liothyronine). J Serum measurements of T3 and T4 will need to be performed after 6 to 8 weeks to determine if the patient is taking the correct dose. J The patient needs to be aware this is lifetime replacement. S Vitamin D supplement to prevent rickets that might result from rapid bonegrowth. Prognosis Early diagnosis and adequate treatment from the 1st weeks of life result in normallinear growth and development. Video link: Congenital hypothyroidism 44  B. Acquired Hypothyroidism Etiology and classifications: Autoimmune: chronic lymphocytic (Hashimoto) thyroiditis Drug-induced: Anticonvulsants, Antithyroid drugs, and lithium Postablative: Irradiation, Radioiodine and Thyroidectomy Hypothalamic-pituitary disease with multiple pituitary hormone deficiencies Clinical Manifestations Weight gain is mostly fluid retention (myxedema), not true obesity. Myxedematous changes of the skin, constipation. Cold intolerance, decreased energy, and an increased need for sleep bradycardia, muscle weakness or cramps and ataxia. Osseous maturation is delayed, often strikingly. Adolescents typically have delayed puberty. older adolescent girls manifest menometrorrhagia. Younger children might present with galactorrhea or pseudoprecociouspuberty. Galactorrhea is a result of increased TRH stimulating prolactin secretion. Diagnostic Studies Measurement of serum frees T4 and TSH. Because the normal range for thyroidtests is slightly higher in children than adults. Measurement of antithyroglobulin and antiperoxidase antibodies can pinpointautoimmune thyroiditis as the cause. Ultrasound examination can determine the 45  nodule dimensions, texture(solid vs cystic nature) A bone age x-ray at diagnosis is useful. Treatment S Levothyroxine (1-T4) therapy is the treatment of choice in children with hypothyroidism. J The dose on a weight basis gradually decreases with age. For children age 1-3 yr, the average 1-T4 dosage is 4-6 pg/kg/day; for age 3-10 yr, 3-5 p g/kg/day; and for age 10- 16 yr, 2-4 pg/kg/day. J Treatment should be monitored by measuring serum free T4 and TSH every 4-6 mo as well as 6 wk after any change in dosage. Complications seen in severe hypothyroidism Heart failure Ventilatory failure Hyponatremia Ileus Delirium, dementia, seizure, stupor, and coma Adrenal insufficiency Coagulopathy decreased factors VIII, VII, V, IX, and X 46  Renal & Urinary Disorders Nephrotic syndrome Introduction ❖ Nephrotic syndrome is characterized by generalized edema as a result of hypoproteinemia due to excessive protein loss in the urine. Hypercholesterolemia iscommonly associated. ❖ The causes of increased permeability of glomeruli to proteins are not known in 90% of children with nephrotic syndrome hence it is called idiopathic or primary nephrotic syndrome. ❖ The Majority i.e. 80 - 85% of NS respond to steroid therapy and if biopsied will show minimal change. Presentation J Diagnosis of nephrotic syndrome is by simple urine examination in a child who presents with edema. J The edema is generalized involving eyes, abdominal wall, genitals, and ankles; ascites may be present and B.P is normal in the majority of cases. S Urine examination shows the presence of proteins- 3+ to 4+ by dipstick S In the first attack, hospitalize the patient to come to a complete diagnosis and to plan the treatment. S It is important to do the blood tests for serum proteins - Total proteins and albumin (serum alb 200 mg/dl). 47  S Although steroid therapy is the backbone of therapy for nephrotic syndrome; oral prednisolone should be started after checking for infection, hypovolemia, and secondary causes. Relaps_e S After completion of the treatment of first-episode, weekly urine examination for proteinuria is advised for 1 month and subsequently every 2-4 weekly or whenever edema recurs. J If urine shows proteinuria of 3+ to 4+ in 3-4 urine examinations in a week, a relapse is diagnosed. Many relapses occur with infections. J Look for the focus of infection (throat, skin, urine, etc) and treat with appropriate antibiotics e.g. Amoxycillin, Cephalexin, etc for 6-7 days. S After 1 week if urine shows 3+/4+ protein after the infection is controlled, steroid therapy should be started. Video link: Nephritic Syndrome 48  Urinary tract infection J Urinary tract infection is a bacterial invasion of the kidneys and the urinary tract. J It is designated as pyelonephritis or upper UTI when the infection involves mainly renal parenchyma and pelvicalyceal system. J Lower UTI involves infection of the urinary bladder and urethra. Causes J UTI is predominantly caused by enterococci like certain strains of E.Coli and other gram-negative bacilli such as Proteus, Klebsiella, and Pseudomonas. J In children below the age of 3-4 years, UTI is associated with underlying congenital anomalies such as posterior urethral valves, PUJ obstruction, vesicoureteral reflux (VUR), bladder diverticula or ureteroceles, etc. Presentation The clinical manifestations of UTI are non-specific and general. At younger age i.e. in new-borns and infants- fever, failure to thrive, vomiting or diarrhea, convulsions, jaundice predominate, hence UTI can be missed at the early stage when it is important to diagnose. In older children above the age of 4-5 years, UTI manifests with fever, painin the abdomen especially loin pain, vomiting 49  with urinary symptoms such as frequency, urgency, dysuria, painful micturition, and foul-smelling urine. The fever can be associated with chills and recurs frequently. When the UTI is recurrent there is a failure to thrive, anemia, listlessness, and general malaise. Diagnosis J The gold standard for diagnosis of UTI is urine culture, colony count, and antibiotic sensitivity report in a clinical setting of UTI. J The collection of urine under sterile conditions is an important point to remember. In very young infants, suprapubic bladder aspiration, and in females or infants, the use of urinary collection bags may be needed. J In older children, midstream clean catch collection of urine in a sterile container and sending it within an hour or two to the lab are important points to get an accurate diagnosis of UTI. J The presence of pus cells (more than 10/HPF in males and 25/hpf in female children) and gram staining of fresh samples of urine for gm-ve bacilli support the diagnosis of UTI. 50% of cases of UTI may not have pyuria. S There are chemical tests like a) leukocyte esterase test and b) nitrite test, which can be used as screening tests for UTI but the gold standard, is urine culture even in these children. 50 Treatment Treatment of UTI is an early institution of the appropriate drugs according to the antibiotic sensitivity report for a duration of 7-10 days. In sick children especially neonates and infants, intravenous antibiotics are required even before the culture report is available. The recommendation is to use Ampicillin and Gentamicin. This is the treatment given for sepsis. Delay of more than 72 hrs in starting the antibiotic can result in permanent renal damage. A 2nd urine culture after 3-5 days of antibiotic therapy should be sterile while the clinical features such as fever resolve within 2-3 days. 51