#91
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А какая норма кретинкиназы?
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#92
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Because of the unusual manifestations of the child's muscle weakness involving only the lower extremity, with no cranial nerve involvement, no relationship to activity, worsening of weakness on school days, and improvement during holidays and weekends, in addition to a recent history of school difficulty, a diagnosis of conversion disorder was entertained.
A simple test strongly supported that diagnosis; 1 mL of intravenous normal saline was administered to the child after it was explained to her that this could cure her illness. Shortly after the injection, the child stood up alone and walked unassisted back and forth in the hallway. Psychiatric consultation identified school as a major stressor in the patient's life. The presence of type 1 diabetes, with its daily testing and insulin injections, was identified as a vulnerability that might have triggered the conversion reaction. Physical therapy was initiated and the parents advised about the nature of the problem. Strategies were offered to alleviate the stressors in the child's life. The family also was advised to shift attention from the child's symptoms and to focus on recovery. The girl responded well to treatment, and follow-up showed better coping abilities and amelioration of her muscle weakness. The Condition Conversion disorder should be suspected when a patient's symptoms do not fit into the framework of known medical illnesses or when appropriate evaluation reveals no organic disease or plausible pathophysiologic explanation. Conversion disorders in children do not indicate a major psychiatric disorder but represent the child's subconscious plea for help in situations in which he or she cannot cope. These situations can arise from a variety of stressors, such as struggles in school, family disharmony, and sexual and physical abuse. Symptoms are referable to the CNS in 65% of children who have conversion disorders. The most usual presentations are episodic loss of awareness, such as pseudoseizures and syncope; motor dysfunction, including gait disturbances and paresis; sensory abnormalities, primarily pain and numbness; and disorders of the special senses. Diagnosis Once the diagnosis of conversion disorder is suspected in a child who has persistent and debilitating symptoms, a sensible evaluation plan should be created. In severe cases, hospitalization may be warranted. During the evaluation, focused investigation and testing should be pursued to be reasonably certain that there is no medically treatable cause. Psychiatric evaluation instituted simultaneously should concentrate on five main areas: 1) the levels of stress or anxiety in the child and family, 2) any special predisposing vulnerabilities in the child that might lower the threshold for coping with stress and anxiety (eg, learning disabilities, peer pressures, problems of body image, chronic illness, and family disharmony or conflict), 3) a possible temporal relationship between a specific stress and the onset of symptoms, 4) role models from whom the symptoms might have been learned, and 5) evidence of primary or secondary gain from the symptom. Differential Diagnosis The differential diagnosis of a child presenting with intermittent muscle weakness includes familial periodic paralysis (hypokalemic or normokalemic); metabolic myopathies, including myophosphorylase deficiency and mitochondrial deficiency; limb-girdle muscular dystrophy; myasthenia gravis; and endocrinopathies such as thyroid disorders and adrenal disorders. Delineation of the clinical pattern and laboratory testing should allow the clinician to determine if any of these disorders is present. If no other disorder fits and if significant stress is evident, a psychosomatic cause should be considered. Treatment Once the evaluation has been completed, a treatment plan is presented to the parents and the child. The first step is to explain that the symptom is real but that no organic disease has been demonstrated. Anxiety or stress has led to the symptom, and this element must be understood and relieved for the child to get better. The treatment must be tailored to the problem, with set goals and the provision of positive feedback as goals are achieved. In addition to measures aimed at understanding and relieving stress, treatment for a patient complaining of weakness might involve "graded" physical therapy. Removing the secondary gain achieved by the symptom is essential for recovery and to eliminate perpetuation of the symptom. Examples of secondary gain include missed school days and increased parental attention because of the symptom. It is essential that the treatment provide "escape with honor" and that the regimen give some control to the child. After discharge, continued psychotherapy should be aimed at allowing the child to give up the sick role and cope with future stress and anxiety more productively. Prognosis Except for children who have pseudoseizures, most children who have a conversion disorder have no underlying major mood disorder or psychiatric illness. Major mood disorders have been identified in 32% of children who have pseudoseizures. A history of sexual abuse is common in patients who have conversion disorders. Because children are still in the formative stages of personality development, the adult diagnosis "hysterical personality," now called "histrionic personality disorder," is questionable when applied to children who have conversion disorder. Histrionic personality disorder comprises a constellation of traits, including dependency, immaturity, egocentricity, attention-seeking behavior, and manipulation. With timely intervention, the child who has a conversion disorder will develop better coping abilities and give up the sick role, thus aborting perpetuation of the symptom and progression to an adult histrionic personality disorder. Further Observations This patient had a chronic illness and had become aware of its power to influence the adults in her world. Another example of this effect is that pseudoseizures are common in children who have true epilepsy. Clinical testing should be judicious because the tests themselves promote anxiety and confirm and reinforce the power and seriousness of the symptom. The child herself is deceived about the source of her symptoms, and families of children who have conversion disorders tend to have conversion symptoms, reinforcing the impressionable child's symptomatology. The clinician must be firm in the diagnosis of conversion and resist his or her own anxiety, which tends to produce the need to do more testing. The simple test employing intravenous saline was an effective diagnostic tool in this case, but it is important that clinicians undertake such procedures with sensitivity to avoid their being perceived by the patient as a trick, potentially undermining trust. The use of physical therapy was a face-saving treatment for the patient and more likely to be acceptable to patient and parents than a purely psychiatric approach, which can be counterproductive if instituted at the wrong time. Similarly, early hospitalization can raise the stakes ominously. Sometimes, psychotherapy will be acceptable if the reason given for recommending such treatment is "to help you cope with the stress of being ill for so long." Lessons for the Clinician Conversion disorder represents a child's expression of a difficult or stressful situation through a physical symptom. The pediatrician, being familiar with the child and parents, should be able to gain the trust of the child and identify stressors and difficulties in the child's life. Psychiatric referral and sometimes hospitalization are crucial for the recovery of children whose symptoms are prolonged and unresponsive to counseling by the pediatrician. |
#93
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Цитата:
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#94
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Думаю, что быстрей всех эту задачу решат анестезиологи-реаниматологи.
A 10-month-old boy is brought to a community ED because he started vomiting last night and “has not and “has not been the same since.” This morning, his mother noted that he was breathing fast. He had a low-grade fever this morning, but none prior. He has had no weight loss or diarrhea and has not been exposed to illness. Intravenous access is obtained, and the child is intubated because of respiratory distress and is flown to a children’s hospital.On examination, the boy’s temperaturei s 37.6°F (99.7°C), heart rate is 158 beats/min, and blood pressure is 90/44 mm Hg. His respiratory rate had been 60 breaths/min prior to being intubated.He is a plump, robust infant who is sedated on the ventilator. Faint crackles are audible in his lungs. His skin is warm and well perfused.The rest of his findings are normal. Laboratory findings include glucose, 176 mg/dL (9.8 mmol/L); sodium 140 mEq/L (140 mmol/L); potassium, 3.1 mEq/L (3.1 mmol/); chloride, 115 mEq/L (115 mmol/L); bicarbonate, 10 mEq/L (10 mmol/L); calculated anion gap, 15 mEq/L(15 mmol/L); BUN, 5 mg/dL (1.8 mmol/L); creatinine,0.3 mg/dL (26.5 mcmol/L). A blood gas reveals a pHof 7.29 with a bicarbonate level of 14 mEq/L(14 mmol/L),a carbon dioxide concentration of 14 torr, and a base deficit of 18.6 mEq/L. His WBC count is 14.9103/mcL (14.9109/L) with 71% neutrophils and 2% bands. He is started on vasoactive medication for hypotension and treated for sepsis, but the clinicians are concerned that he has had minimal fever and no rash, and the laboratory findings are puzzling. An additional blood test reveals the diagnosis. |
#95
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Цитата:
Не совсем понимаю, за что ребенок переведен на ИВЛ (только из-за одышки 60 в минуту и крепитации в легких?) Гемодинамика стабильная, нарушений периферического кровообращения нет, тем не менее, проводится инотропная поддержка (кстати, чем и в какой дозе?). Что на рентгеновском снимке? |
#96
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Наталья, вся, имеющаяся в условии информация, выложена.
В качестве подсказки : Какой ведущий клинический синдром имеется в наличии у этого младенца? |
#97
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Метаболический ацидоз с компенсаторной гипервентиляцией. Анионовая щель присутствует. Нужно делать toxscreen и скорее всего получим отравление салицилатами.
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#98
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Discussion
1)The child had an anion gap acidosis, but no evidence of a disorder that would cause lactic acidosis. 2)Although he had been vomiting for 12 hours, he had no other insensible losses to explain the rapid deterioration. 3) Diabetic ketoacidosis seemed unlikely because his glucose level never exceeded 200 mg/dL (11.1 mmol/L). The wide anion gap acidosis prompted measurement of salicylate concentrations, although there was no history of ingestion. Salicylates were present at a toxic level of 60 mg/dL (4.3 mmol/L). The patient was alkalinized immediately and dialyzed. In 24 hours, he was extubated and was doing well. Laboratory Clues Severe dehydration from vomiting or diarrhea can cause a significant lactic acidosis due to tissue hypoxemia and hypoperfusion. However, this child had only a brief history of vomiting. The rapid breathing and respiratory distress that required intubation led the clinicians to consider an underlying metabolic derangement. Other disorders to consider in this case are methanol toxicity, uremia, diabetic ketoacidosis, paraldehyde ingestion, iron and isoniazid toxicities, lactic acidosis, and ethylene glycol ingestion. The addition of salicylism to the list creates the “MUDPILES” mnemonic. In this case, the child’s mother later discovered that one of the siblings had given the infant several adult aspirin tablets. Although the ingestion history was not known at the time of admission, a thorough investigation of the child’s wide anion gap acidosis led to the diagnosis. Other supporting laboratory findings included the potassium concentration of 3.1 mEq/L (3.1 mmol/L) and serum bicarbonate level of 10 mEq/L (10 mmol/L). The Disorder Aspirin toxicity remains one of the most serious ingestions in the pediatric population. Despite the recent declining incidence, there still are approximately 16,000 cases yearly of aspirin overdose in this country, resulting in 30 to 35 deaths. An acute toxic dose for a child is greater than 150 mg (approximately one half of a 325-mg adult tablet) per kg. Although overall use of aspirin has decreased, most households in the United States still contain salicylates in one form or another (tablets, bismuth compounds, keratolytic agents). Pathophysiology Salicylates have a broad scope of action, especially in toxic doses, because of their ability to uncouple oxidative phosphorylation, inhibit amino acid synthesis, and inhibit Krebs cycle enzymes. The emesis and nausea that patients experience after aspirin ingestion are related to direct gastric irritation. Other effects include altered hearing (usually tinnitus), fever, and altered mental status that can range from agitation to seizures to stupor and coma. In the initial phase of aspirin overdose, patients experience respiratory alkalosis due to direct stimulation of the CNS respiratory drive. To correct this alkalosis, bicarbonate is excreted in the urine, causing alkaluria. Initially, potassium also is excreted in the urine, leading to global hypokalemia. In the next phase of salicylate toxicity, the kidneys attempt to preserve the potassium level by an exchange for hydrogen ions in the urine. This paradoxic aciduria in the face of a respiratory alkalosis is a hallmark of aspirin toxicity. In the final stages of aspirin overdose, hyperpnea continues as a response to the primary metabolic acidosis. This exaggerated breathing contributes further to insensible water losses. Although adults tend to have a mixed respiratory alkalosis and metabolic acidosis, acidosis often predominates in young children. Finally, as in this infant, salicylates increase pulmonary vasculature permeability, leading to noncardiogenic pulmonary edema. Laboratory Evaluation Several studies should be ordered in cases of suspected salicylate toxicity. Electrolytes, BUN, creatinine, arterial blood gases, complete blood count, and liver function tests should be checked immediately. Hypokalemia is common and can be severe. Salicylate levels should be checked immediately if ingestion is suspected and subsequently checked every 2 to 4 hours until decreasing or reaching less than 30 mg/dL (2.2 mmol/L). Although levels typically peak 4 to 6 hours after aspirin ingestion and correlate poorly with clinical symptoms, prolonged peaking of salicyindex of suspicion 426 Pediatrics in Review Vol.27 No.11 November 2006 lates occurs with ingestion of entericcoated tablets or in the presence of any obstruction in the GI tract, such as a pill bezoar or pylorospasm. As in this patient, if the diagnosis is uncertain, additional laboratory testing should be undertaken to rule out other causes of anion gap acidosis. Serum lactate, osmolarity, and a urinalysis looking for ketones can be helpful. It also may be useful to measure iron and alcohol levels (looking for methanol and ethylene glycol). As always, a thorough history is indicated, particularly focusing on all medications, including over-thecounter items, in the household. Treatment If the patient is alert, activated charcoal should be administered. Gastric lavage usually is not recommended unless the ingestion is believed to have been a large, life-threatening dose and has been brought to medical attention within 1 hour. Most important in the treatment of salicylism is the correction of multiple metabolic derangements and dehydration. Initially, fluid boluses should be considered in the face of severe dehydration. Caution must be exercised in the presence of pulmonary edema or renal failure. Alkalinization enhances salicylate elimination and should be instituted promptly. A goal for serum pH should be approximately 7.5, with a goal for urinary pH of greater than 7.5. Standard recommendations suggest using a 1 to 2-mEq/kg bolus of bicarbonate followed by a sodium bicarbonate drip. Because persistent hypokalemia may interfere with alkalinization of the urine and salicylate excretion, it is critical to add potassium to fluids in patients who do not have renal failure. Dialysis should be instituted if the serum salicylate level is greater than 100 mg/dL (7.2 mmol/L) in acute toxicity or if there are signs of pulmonary edema, renal failure, persistent altered mental status, worsening vital signs such as fever, or congestive heart failure. Intubation might be considered in the most seriously ill patients. Clinicians should realize, however, that intubation of the patient poisoned by salicylates can be dangerous. The patient’s own ability to hyperventilate and regulate acidosis is suppressed, and he or she may develop worsening acidosis. Rarely do long-term sequelae result from salicylate toxicity. When recognized and treated early, most pediatric patients do very well. Lessons for the Clinician It is important to consider salicylism in the differential diagnosis of a child who has an anion gap acidosis. In this case, a careful review of the initial history, physical findings, and laboratory studies narrowed the differential diagnosis significantly. Early recognition and treatment can be life-saving. |
#99
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Боль в ноге.
A 4-year-old Arabic boy has experienced leg pain for the past 2 months. He localizes the pain to the middle of his right thigh, sometimes extending to his knee. He is able to run and play, but pain recurs at the end of vigorous play, is worse at the end of the day, and often wakes him at night. It is relieved by aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs). There is no history of trauma, redness, swelling, fever, weight loss, or rashes. The patient has had no similar complaints in the past. There is no family history of bone or joint diseases. He was born in the United States but has lived in Lebanon for the past year.
On physical examination, the boy's temperature is 99.1 F (37.3 C), pulse is 108 beats/min, respiratory rate is 28 breaths/min, and blood pressure is 103/68 mm Hg. He localizes pain to the middle of his right thigh, but there is no tenderness, swelling, or erythema in that area. There is full range of motion at both the right hip and knee joints, and strength is 5/5. Laboratory values include a WBC count of 8.5x103/dL (8.5x109/L), Hgb level of 13.3 g/dL (133 g/L), platelet count of 455x103/dL (455x109/L), ESR of 22 mm/hr, and C-reactive protein level of 2.85 mg/dL. Что предложим в плане обследования? |
#100
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Рентген нужно сделать.
Если мне не изменяет память, боли подобного характера, купирующиеся аспирином, характерны для...остеоид-остеомы...Или я ошибаюсь? |
#101
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A plain radiograph of the right hip and femur showed an approximately 1-cm, focal, lytic lesion with sclerotic margins at the interior cortex of the proximal right femoral diaphysis. There was no evidence of periosteal reaction, associated soft-tissue mass, or pathologic fracture. The soft tissue was unremarkable. The visualized hip and pelvis were within normal limits. Findings were believed to be consistent with osteoid osteoma, and the CT scan made that diagnosis more certain . Histologic examination of a specimen obtained by CT-guided biopsy of the lesion confirmed the diagnosis of osteoid osteoma.
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#102
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Мне понравилось.
Новорожденный младенец переведен в отделение патологии новорожденных из-за выраженных усиленных движений грудной клетки при дыхании через 15 минут после рождения. Роды вагинальные, без осложнений, Апгар 9/9.
При осмотре педиатром в отделении патологии новорожденных через 20 минут после рождения отмечается следующее: Т 37 P.R., ЧСС 158, ЧДД 42, АД 55/16, Sat O2 100% Аускультация легких в норме, небольшая ретракция м/реберных промежутков, нет выраженного респираторного дистресса. При аускультации сердца - громкий голосистолический шум, лучше всего слышен по правой стернальной линии сверху, акроцианоз новорожденных, капиллярный возврат 3 сек. Неврология - активный, нормальный новорожденный. ----- Ваши предположения? Что бы это могло быть? |
#104
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