Lipomatosis central non-encapsulated


A rare disorder involving defective fat metabolism which leads to a buildup of fat deposits in neck and shoulder area. Mainly occurs in male alcoholics.


* Enlarged neck * Enlarged shoulders * Reduced neck mobility * Neck pain


Although airways and alveoli of a neonate’s respiratory system are present by 27 weeks’ gestation, the intercostal muscles are weak and the alveolar capillary system is immature. The premature neonate with IRDS develops widespread alveolar collapse due to a lack of surfactant, a lipoprotein pres-ent in alveoli and respiratory bronchioles. Surfactant lowers surface tension and helps prevent alveolar collapse. This surfactant deficiency results in widespread atelectasis, which leads to inadequate alveolar ventilation with shunting of blood through collapsed areas of lung, causing hypoxemia and acidosis. IRDS occurs almost exclusively in neonates born before 37 weeks’ gestation (in 60% of those born before the 28th week). The incidence is greatest in the 1,000 to 1,500 g birthweight group. Infants of diabetic mothers, those born by cesarean delivery, second-born twins, infants with perinatal asphyxia, and those delivered suddenly after antepartum hemorrhage are more commonly afflicted.


Assessment of the respiratory system begins with a thorough patient history. Ask the patient to describe his respiratory problem. How long has he had it? How long does each attack last? Does one attack differ from another? Does any activity in particular bring on an attack or make it worse? What relieves the symptoms? Always ask whether the patient was or is a smoker, what and how often he smoked or smokes, and how long he smoked or has been smoking. Record this information in “pack years”—the number of packs of cigarettes per day multiplied by the number of smoking years. Remember to ask about the patient’s occupation, hobbies, and travel; some of these activities may involve exposure to toxic or allergenic substances. If the patient has dyspnea, ask if it occurs during activity or at rest. What position is the patient in when dyspnea occurs? How far can he walk? How many flights of stairs can he climb? Has his exercise tolerance been decreasing? Can he relate dyspnea to allergies or environmental conditions? Does it occur only at night, during sleep? If the patient has a cough, ask about its severity, persistence, and duration; ask if it produces sputum and, if so, what kind. Have the patient’s cough habits and character of sputum changed recently?


ARF in patients with COPD is an emergency that requires cautious O2 therapy (using nasal prongs or Venturi mask) to raise the PaO2. In patients with chronic hypercapnia, O2 therapy can cause hypoventilation by increasing Paco2 and decreasing the respiratory drive, necessitating mechanical ventilation. The minimum fraction of inspired air (FIO2) required to maintain ventilation or O2 saturation greater than 85% to 90% should be used. If significant uncompensated respiratory acidosis or unrefractory hypoxemia exists, mechanical ventilation (through an endotracheal [ET] or a tracheostomy tube) or noninvasive ventilation (with a face or nose mask) may be necessary. Treatment routinely includes antibiotics for infection, bronchodilators, and possibly steroids.


Lipomatosis central non-encapsulated: Another name for Madelung's disease