פתרון יוני2009
(חפירות מתוך נלסון הגדול, נלסון הקטן ושוחט-אשכנזי אא"כ צוין אחרת)
שאלה 1 תשובה ג'
a) Infantile spasms usually begin between the ages of 4 and 8 mo and are characterized by brief symmetric contractions of the neck, trunk, and extremities.
b) Upper airway obstruction is manifested during inspiration because the pressure within the upper airway is negative relative to the atmosphere… The clinical manifestation most commonly associated with upper airway obstruction is inspiratory stridor, a harsh sound produced usually at or near the larynx by the vibration of upper airway structures.
c) A cyanotic breath-holding spell is usually predictable and is always provoked by upsetting or scolding an infant. The episode is heralded by a brief, shrill cry followed by forced expiration and apnea. There is rapid onset of generalized cyanosis and a loss of consciousness that may be associated with repeated generalized clonic jerks, opisthotonos, and bradycardia. Results of an interictal electroencephalogram (EEG) are normal. A breath-holding spell can occur repeatedly within a few hours or it can recur sporadically, but it is always stereotyped. Breath-holding spells are rare before 6 mo of age, peak at about 2 yr of age, and abate by 5 yr of age.
d) Congenital Central Hypoventilation Syndrome (Ondine Curse) - The typical presentation of children with congenital central hypoventilation syndrome (CCHS, or Ondine curse) occurs in the neonatal period. The initial symptoms include periods of cyanosis when the newborn falls asleep, with diminished chest excursions and prolonged periods of respiratory pauses, with symptoms disappearing when the infant is awakened. CCHS can mimic many diseases; hence, other diagnoses must be entertained before the diagnosis of CCHS is made. There are usually no detectable gross anatomic abnormalities, although brainstem tumors and arteriovenous malformations have been described and other neurologic diseases must be considered. The primary defect is in the CNS, but there may be abnormalities in other elements of the respiratory control system.
e) NIGHT TERRORS.
Night terrors are common, particularly in boys between 5 and 7 yr of age (see Chapter 18 ). They occur in 1–3% of children and are usually short-lived. A night terror has a sudden onset, usually between midnight and 2 a.m. during stage 3 or 4 of slow-wave sleep. The child screams and appears frightened, with dilated pupils, tachycardia, and hyperventilation. There is little or no verbalization; the child may thrash violently, cannot be consoled, and is unaware of parents or surroundings. Sleep follows in a few minutes, and there is total amnesia the following morning. Approximately one third of children with night terrors experience somnambulism. An underlying emotional disorder should be explored in children with persistent and prolonged night terrors. A short course of diazepam may be considered for treatment of protracted night terrors while the family dynamics are under investigation.
שאלה 2תשובה ה'
הילד טופל בבטא אגוניסטים קצרי טווח פחות מפעמיים לחודש, ועל כן ע"פ הטבלה אין צורך בהתחלת טיפול מניעתי.
שאלה 3תשובה ב' (נבדק מול רופא בכיר)
ABLE 397-5--Differentiation of Pleural Fluid
TRANSUDATE / EXUDATE / COMPLICATED EMPYEMAAppearance / Clear / Cloudy / Purulent
Cell count / <1000 / >1000 / >5000
Cell type / Lymphocytes, monocytes / PMNs / PMNs
LDH / <200 U/L / >200 U/L / >1000 U/L
Pleural/serum LDH ration / <0.6 / >0.6 / >0.6
Protein >3 g / Unusual / Common / Common
Pleural/serum protein ratio / <0.5 / >0.5 / >0.5
Glucose[*] / Normal / Low / Very low[*](<40 mg/dL)
pH[*] / Normal (7.40–7.60) / 7.20–7.40 / <7.20, chest tube placement required
Gram stain / Negative / Usually positive / >85% positive unless patient received prior antibiotics
From Kliegman RM, Greenbaum LA, Lye PS:Practical Strategies in Pediatric Diagnosis & Therapy,2nd ed Philadelphia, Elsevier, 2004, p. 30.
* / Low glucose or pH may be seen in malignant effusion, tuberculosis, esophageal rupture, pancreatitis (positive pleural amylase), and rheumatologic diseases (e.g., systemic lupus erythematosus). LDH, lactate dehydrogenase; PMNs, polymorphonuclear neutrophils.ע"פ טבלאות הסיכום של ענבר למבוא לפנימית:
הגורמים השכיחים להיווצרות טרנסודאט- אי ספיקת לב, שחמת של הכבד, סינדרום נפרוטי, דיאליזה פריטוניאלית, תסחיף ריאתי.
הגורמים השכיחים להיווצרות אקסודאט-דלקות ריאה, ממאירות, שחפת, תסחיף ריאתי.
שאלה 4תשובה א'
a) Neurologic findingsare among the most common extraintestinal manifestations of bacillary dysentery, occurring in as many as 40% of hospitalized infected children. EnteroinvasiveE. colican cause similar neurologic toxicity. Convulsions, headache, lethargy, confusion, nuchal rigidity, or hallucinations may be present before or after the onset of diarrhea. The cause of these neurologic findings is not understood. In the past, these symptoms were attributed to the neurotoxicity of Shiga toxin, but it is clear that that explanation is wrong since the organisms isolated from children withShigella-related seizures are usually not Shiga toxin producers. Seizures sometimes occur when little fever is present, suggesting that simple febrile convulsions do not explain their appearance. Hypocalcemia or hyponatremia may be associated with seizures in a small number of patients. Although symptoms often suggest central nervous system infection, and cerebrospinal fluid pleocytosis with minimally elevated protein levels can occur,Shigellameningitis is rare.
b)The most common clinical presentation of salmonellosis is with acute enteritis. After an incubation period of 6–72 hr (mean, 24 hr), there is an abrupt onset of nausea, vomiting, and crampy abdominal pain, primarily in the periumbilical area and right lower quadrant, followed by mild to severe watery diarrhea and sometimes by diarrhea containing blood and mucus. A large proportion of children are febrile, although younger infants may exhibit a normal or subnormal temperature. Symptoms usually subside within 2–7 days in healthy children and fatalities are rare. However, some children develop severe disease with a septicemia-like picture (high fever, headache, drowsiness, confusion, meningismus, seizures, abdominal distention). The stool typically contains a moderate number of polymorphonuclear leukocytes and occult blood. Mild leukocytosis may be detected.
c) addition to profuse watery, nonbloody diarrhea with mucus, vomiting and low-grade fever are common symptoms. Persistent diarrhea (>14 days) can lead to malnutrition; this is an important outcome of EPEC infection in infants in the developing world.
d)Rotavirus infection typically begins after an incubation period of <48 hr (range 1–7 days) with mild to moderate fever as well as vomiting followed by the onset of frequent, watery stools. All 3 symptoms are present in about 50–60% of cases. Vomiting and fever typically abate during the 2nd day of illness, but diarrhea often continues for 5–7 days. The stool is without gross blood or white cells. Dehydration may develop and progress rapidly, particularly in infants. The most severe disease typically occurs among children 4–36 mo of age. Malnourished children and children with underlying intestinal disease such as short-bowel syndrome are particularly likely to acquire severe rotavirus diarrhea. Rarely, immunodeficient children experience severe and prolonged illness. Although most newborns infected with rotavirus are asymptomatic, some outbreaks of necrotizing enterocolitis have been associated with the appearance of a new rotavirus strain in the affected nurseries.
e)
SPECIES / DISEASES IN HUMANSC. jejuni / Gastroenteritis, bacteremia, Guillain-Barré syndrome
שאלה 5 תשובה ב'
Table 135-1. Age-Related Differential Diagnosis of Upper Airway ObstructionNewborn
Foreign material (e.g., meconium or amniotic fluid)
Congenital subglottic stenosis (uncommon)
Choanal atresia
Micrognathia (Pierre Robin syndrome, Treacher Collins syndrome, DiGeorge syndrome)
Macroglossia (Beckwith-Wiedemann syndrome, hypothyroidism, Pompe disease, trisomy 21, hemangioma)
Laryngeal web, clefts, atresia
Laryngospasm (intubation, aspiration, transient)
Vocal cord paralysis (weak cry; unilateral or bilateral, with or without increased intracranial pressure from Arnold-Chiari malformation or other CNS pathology)
Tracheal web, stenosis, malacia, atresia
Pharyngeal collapse (cause of apnea in preterm infant)
Dislocated nasal cartilage
Nasal piriform aperture stenosis
Nasal encephalocele
Infancy
Laryngomalacia (most common etiology)
Subglottic stenosis (congenital, acquired after intubation)
Hemangioma
Tongue tumor (dermoid, teratoma, ectopic thyroid)
Laryngeal dyskinesis
Laryngeal papillomatosis
Vascular rings/slings
Rhinitis
Toddlers
Viral croup (most common etiology in children 6 mo to 4 yr old)
Bacterial tracheitis (toxic, high fever)
Foreign body (sudden cough; airway or esophageal)
Spasmodic (recurrent) croup
Laryngeal papillomatosis
Retropharyngeal abscess
Hypertrophied tonsils and adenoids
Diphtheria (rare)
2-3 Years Old
Epiglottitis (infection, uncommon)
Inhalation injury (burns, toxic gas, hydrocarbons)
Foreign bodies
Rhinitis medicamentosa
Angioedema (familial history, cutaneous angioedema)
Anaphylaxis (allergic history, wheezing, hypotension)
Trauma (tracheal or laryngeal fracture)
Peritonsillar abscess (adolescents)
Mononucleosis
Ludwig angina
Diphtheria (rare)
שאלה 6תשובה ד'?
Acute pancreatitis, the most common pancreatic disorder in children, is increasing in frequency. At least 30–50 cases are now seen in major pediatric centers per year. Blunt abdominal injuries, multisystem disease, biliary stones or microlithiasis (sludging), and drug toxicity are the most common etiologies. Valproic acid is the most common cause of drug-induced pancreatitis in children. Other cases follow organ transplantation, or are due to infections and metabolic disorders. Fewer cases are idiopathic or due to other etiologies.
TABLE 348-2--Differential Diagnosis of Hyperamylasemia
PANCREATIC PATHOLOGYAcute or chronic pancreatitis
Complications of pancreatitis (pseudocyst, ascites, abscess)
Factitious pancreatitis
SALIVARY GLAND PATHOLOGY
Parotitis (mumps,Staphylococcus aureus,cytomegalovirus, HIV, Epstein-Barr virus)
Sialadenitis (calculus, radiation)
Eating disorders (anorexia nervosa, bulimia)
INTRA-ABDOMINAL PATHOLOGY
Biliary tract disease (cholelithiasis)
Peptic ulcer perforation
Peritonitis
Intestinal obstruction
Appendicitis
SYSTEMIC DISEASES
Metabolic acidosis (diabetes mellitus, shock)
Renal insufficiency, transplantation
Burns
Pregnancy
Drugs (morphine)
Head injury
Cardiopulmonary bypass
מכיוון שהיא מחוסנת לחזרת תשובה ד' היא הכי פחות סבירה. גם אי ספיקת כליות אינה סבירה במצב הזה, ובכל זאת כנראה שמטרת השאלה היתה להדגיש כי באי ספיקת כליות קיימת היפר-עמילאזיה.
שאלה 7 תשובה א'
Galactosemia is an autosomal recessive disease caused by deficiency of the enzyme galactose-1-phosphate uridyltransferase (Fig. 52-2). Clinical manifestations are most striking in a neonate who when fed milk generally exhibits evidence of liver failure (hyperbilirubinemia, disorders of coagulation, and hypoglycemia), disordered renal tubular function (acidosis, glycosuria, and aminoaciduria), and cataracts. The neonatal screening test must have a rapid turnaround time because affected infants may die in the first week of life. Infants with galactosemia are at increased risk for severe neonatal Escherichia coli sepsis. Major effects on liver and kidney function and the development of cataracts are limited to the first few years of life, but older children have learning disorders. Girls may develop premature ovarian failure, which occurs despite treatment.
שאלה 8 תשובה ב'
In the metabolictumor lysis syndrome,phosphates and potassium are released into the circulation in large quantities as cells are lysed by treatment. Symptomatic hyperkalemia and hyperphosphatemia with subsequent hypocalcemia may develop in the setting of inadequate renal function.
שאלה 9 תשובה ב'
Duchenne (pseudohypertrophic muscular dustrophy)- המחלה השכיחה ביותר בקבוצת הדיסטרופיות השריריות. היא מופיעה רק בבנים, העברתה היא בצורת X-linked, אך ב-50% מהמקרים מדובר במוטציה חדשה.
קליניקה- הסימנים בד"כ מופיעים מוקדם, סיפור של התפתחות מוטורית איטית. המחלה מתבטאת בחולשת שרירים בעיקר של חגורת המותניים, ומכאן נובע הסימן האופייני של 'הליכת הברווז', וכן קשיים בעליה במדרגות ובקימה מישיבה תוך שימוש ב-gower's sign. חולשת חגורת הכתפיים ניתנת להדגמה ע"י הרמת הילד בבתי השחי; כאשר מרימים אותו כך, הוא מחליק למטה.
קיימת גם היפרטרופיה של שרירי הסובך. השריר נראה היפרטרופי, אך למעשה מוסנן בשומן ולכן חלש.
אי היכולת לנוע קיימת בד"כ בגיל 12 שנים, ורוב הילדים נפטרים בגיל צעיר. במרביתם יש קרדיומיופתיה העלולה לגרום למוות פתאומי.
DD: מחלות אגירת גליקוגן של השריר, פולימיוזיטיס ו-werding Hoffmann.
האבחנה: בד"כ קלה. רמות ה-CPKגבוהות ביותר, וכך גם רמות ה-LDH וה-GOT.
בבדיקת EMG מאובחנת ירידה בגובה ובארוך של פוטנציאל הפעולה.
בביופסיה מוצאים ניוון של סיבי השריר, הסיבים בגדלים שונים, ואלה שנהרסו מוחלפים ברקמת שומן או חיבור. הצביעה לדיסטרופין (החלבון הפגום) שלילית.
בבנות הנושאות את המחלה ניתן למצא לפעמים רמות CPKגבוהות.
אין טיפול. בשנים האחרונות גורמים עיכוב מסוים בהתפתחות המחלה ע"י טיפול ממושך בסטרואידים.
שאלה 10 תשובה א'+ ד' (ד' ע"פ רופא בכיר)
Clinicalpyelonephritisis characterized by any or all of the following: abdominal or flank pain, fever, malaise, nausea, vomiting, and, occasionally, diarrhea. Newborns may show nonspecific symptoms such as poor feeding, irritability, and weight loss. Pyelonephritis is the most common serious bacterial infection in infants <24 mo of age who have fever without a focus (seeChapter 175). These symptoms are an indication that there is bacterial involvement of the upper urinary tract.
Empirical therapy should be initiated for symptomatic children and for all children with a urine culture confirming UTI. For a child who does not appear ill but with a positive urine culture, antibiotic therapy should be initiated either parenterally or orally. For a child with suspected UTI who appears toxic, appears dehydrated, or is unable to retain oral fluids, initial antibiotic therapy should be administered parenterally, and hospitalization should be considered.
Neonates with UTI are treated for 10 to 14 days with parenteral antibiotics because of the higher rate of bacteremia. Older children with acute cystitis are treated for 7 to 14 days with an oral antibiotic. Increasing bacterial resistance has limited the usefulness of some antibiotics, such as amoxicillin. TMP-SMZ is used frequently, although resistance to this drug also is increasing. Oral third-generation cephalosporins (cefixime, cefpodoxime) are effective but expensive. Children with high fever or other manifestations of acute pyelonephritis often are hospitalized for initial treatment with parenteral antibiotics, such as cefotaxime. Patients with systemic toxicity (chills and high fever) should be hospitalized and treated with IV cefotaxime and gentamicin (or another aminoglycoside). When the patient has improved and is afebrile, oral therapy with an agent to which the cultured organism is sensitive is administered to complete 7 to 14 days of total therapy.
הבחורה מיובשת, כדאי להחזיר לה נוזלים לפני טיפול אנטיביוטי כלשהו...אם כי המקרה נראה כ-UTIולא כגסטרואנטריטיס.
שאלה 11תשובה ד'
The most characteristic and specific signs of vitamin A deficiency are eye lesions. Lesions due to vitamin A deficiency develop insidiously and rarely occur before 2 yr of age. An early symptom is delayed adaptation to the dark; later when vitamin A deficiency is more advanced, it leads tonight blindnessdue to the absence of retinal in the visual pigment, rhodopsin, of the retina. Photophobia is a common symptom. As vitamin A deficiency progresses, the epithelial tissues of the eye become severely altered.
שאלה 12 תשובה א'
Among the 5Mycoplasmaspecies isolated from the human respiratory tract,Mycoplasma pneumoniaeis the only recognized human pathogen. It is a major cause of respiratory infections in school-aged children and young adults.
Although the clinical course in untreated individuals is variable, coughing usually worsens during the 1st wk of illness, with all symptoms usually resolving within 2 wk.
Radiographic findings are not specific. Pneumonia is usually described as interstitial or bronchopneumonic; involvement is most common in the lower lobes, with unilateral, centrally dense infiltrates described in 75% of cases.
המחוללים העיקריים הגורמים לדלקת ריאות על פי גיל:
בפעוטות: RSV
מתחת לגיל 5: מזהמים רספירטוריים כגון RSV, פארא-אינפלואנזה, אינפלואנזה ואדנווירוסים
מעל לגיל 5: M.Pneumonia ו –S.Pneumonia
פנאומוניה בפעוטות חולי CF הםS.aureus, P.aeruginosa, Burkholderia capacia.
שאלה 13 תשובה ב'
ויקיפדיה:
The scale reflects what percentage of a certain age group is able to perform a certain task. In a test to be administered by a pediatrician or other health or social service professional, a subject's performance against the regular age distribution is noted. Tasks are grouped into four categories (social contact, fine motor skill, language, and gross motor skill) and include items such as smiles spontaneously (performed by 90% of three-month-olds), knocks two building blocks against each other (90% of 13-month-olds), speaks three words other than "mom" and "dad" (90% of 21-month-olds), or hops on one leg (90% of 5-year-olds).
שאלה 14 תשובה ג'
Respiratory infections caused by adenoviruses (seeChapter 259), influenza viruses (seeChapter 255),Chlamydia(seeChapter 222), andMycoplasma pneumoniae(seeChapter 220) are usually self-limited illnesses but have been associated with prolonged and progressive lung damage, often in the form of bronchiolitis obliterans.
c)Chlamydial genital infection is reported in 5–30% of pregnant women, with a risk for vertical transmission at parturition to newborn infants of about 50%. The infant may become infected at 1 or more sites, including the conjunctivae, nasopharynx, rectum, and vagina. Transmission is rare following cesarean section with intact membranes. The introduction of systematic prenatal screening forC. trachomatisinfection and treatment of pregnant women has resulted in a dramatic decrease in the incidence of neonatal chlamydial infection in the USA. However, in countries where prenatal screening is not done, such as the Netherlands,C. trachomatisremains an important cause of neonatal infection, accounting for >60% of neonatal conjunctivitis.
Inclusion Conjunctivitis.
Approximately 30–50% of infants born to mothers with active, untreated, chlamydial infection develop clinical conjunctivitis. Symptoms usually develop 5–14 days after delivery, or earlier with prolonged rupture of membranes. The presentation is extremely variable and ranges from mild conjunctival injection with scant mucoid discharge to severe conjunctivitis with copious purulent discharge, chemosis, and pseudomembrane formation. The conjunctiva may be very friable and may bleed when stroked with a swab. Chlamydial conjunctivitis must be differentiated from gonococcal ophthalmia, which is sight threatening. At least 50% of infants with chlamydial conjunctivitis also have nasopharyngeal infection.
Pneumonia.
Pneumonia due toC. trachomatisdevelops in 10–20% of infants born to women with active, untreated chlamydial infection. Only about 25% of infants with nasopharyngeal chlamydial infection develop pneumonia.C. trachomatispneumonia of infancy has a very characteristic presentation. Onset is usually from 1 to 3 mo of age and is often insidious with persistent cough, tachypnea, and absence of fever. Auscultation reveals rales; wheezing is uncommon. The absence of fever and wheezing helps to distinguishC. trachomatispneumonia from respiratory syncytial virus pneumonia. A distinctive laboratory finding is the presence of peripheral eosinophilia (>400 cells/mm3). The most consistent finding on chest radiograph is hyperinflation accompanied by minimal interstitial or alveolar infiltrates.
d) Adenovirus- About 7–9% of hospitalized children with acute pneumonia have adenovirus infection. Adenovirus pneumonia often presents with radiologic features more typical of bacterial disease (lobar infiltrates, high fever, parapneumonic effusions), and children with adenovirus pneumonia often have other systemic signs such as diarrhea, abdominal pain, and even coagulation defects. Any of the “respiratory” types can cause pneumonia, but severe infections are most likely caused by types 3, 7, or 21. These infections have a mortality rate as high as 10%, and survivors may have residual airway damage, manifested by bronchiectasis, bronchiolitis obliterans, or, rarely, pulmonary fibrosis. Neonatal adenovirus pneumonia occurs rarely, but may be severe or fatal.
Pharyngoconjunctival fever is a clinically distinct syndrome that occurs typically with type 3 adenovirus. Features include a high temperature that lasts 4–5 days, pharyngitis, conjunctivitis, preauricular and cervical lymphadenopathy, and rhinitis. Nonpurulent conjunctivitis occurs in 75% of patients and is manifested by inflammation of both the bulbar and palpebral conjunctivae of 1 or both eyes, which often persists after the fever and other symptoms have resolved. Headache, malaise, and weakness are common, and there is considerable lethargy after the acute stage.
שאלה 15 תשובה ב'
מתוך "מדריך לאנשי מקצועלהזנת התינוק והפעוט 2009" מאתר משרד הבריאות
"חלב עיזים: חלב זה חסר בחומצה פולית וויטמיןD ועל כן אינו מומלץ כלל להזנת התינוק במקום חלב אם או תמ"ל. אין הוכחות לכך, שחלב עיזים מפחית התפתחות מחלות אלרגיות בתינוקות בסיכון גבוה. תינוקות האלרגיים לחלב פרה יהיו במקרים רבים גם אלרגיים לחלב עיזים, ולכן יש לאסור שימוש בחלב זה במקרים של אלרגיה לחלב פרה."
שאלה 16 תשובה א'
Maternal folic acid status is known to be protective for neural tube defects, primarily spina bifida and anencephaly. To prevent such birth defects, it is recommended that women of childbearing age consume 400 μmg of folic acid from supplements or fortified foods in addition to intake of food folate from a varied diet.
שאלה 17 תשובה ג'
Adverse events from the MMR vaccine include fever (usually 6–12 days following vaccinations), rash in about 5% of vaccines, and, rarely, transient thrombocytopenia. Children prone to febrile seizures may experience an event following vaccination, so risks and benefits should be discussed with parents.
שאלה 18תשובה ג'
Indications for a voiding cystourethrogram (VCUG) are controversial and are changing. Most clinicians recommend it for all children with a febrile UTI. A VCUG also is recommended in girls who have had 2 or 3 UTIs in a period of 6 mo, and for boys with more than one UTI. A VCUG also should be obtained if the renal sonogram shows any significant abnormality, such as hydronephrosis, disparity in renal length, or bladder wall thickening.
ע"פ רופא בכיר- תשובה ג' כוללנית ונכונה יותר ע"פ נלסון למרות הפסקה המודגשת הנ"ל.
שאלה 19 תשובה א'
After acute salicylate ingestion, nausea and vomiting occur due to gastric irritation. Salicylates directly stimulate the respiratory center, leading to hyperventilation and hyperpnea. An increased respiratory rate results inrespiratory alkalosiswith compensatory alkaluria. Both potassium and sodium bicarbonate are excreted in the urine; however, soon after exposure, the serum potassium concentration may be in the normal range. When sufficient potassium has been lost through the kidneys, an exchange of potassium for hydrogen ion occurs and the urine becomes relatively acidic. This “paradoxical aciduria” occurs in the presence of continued respiratory alkalosis. Dehydration and progressivemetabolic acidosis,caused by the accumulation of lactic acid and other metabolic acids, eventually develop. Seriously poisoned patients are >5–10% dehydrated. Patients with chronic salicylate poisoning usually present with metabolic acidosis.