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1/9. Nonketotic hyperglycemic hyperosmolar coma. Report of neurosurgical cases with a review of mechanisms and treatment.

    Seventy-eight critically ill patients who died while on the neurosurgical service were studied retrospectively to establish the prevalence of nonketotic hyperglycemic hyperosmolar coma (NHHC). All the patients had been comatose before death, and all underwent necropsy. Criteria for the diagnosis of NHHC included moderate-to-severe hyperglycemia with glucosuria, absence of significant acetonuria, hyperosmolarity with dehydration, and neurological dysfunction. This study revealed seven cases of unequivocal NHHC (9%), and six of hyperosmolarity but with incomplete records. Five of the seven confirmed cases of NHHC demonstrated no evidence of cerebral edema transtentorial herniation, or brain-stem damage, and showed central nervous system (CNS) lesions compatible with survival. Fatal complications of this syndrome, such as acute renal failure, terminal arrhythmias, and vascular accidents, both cerebral and systemic, were common in this series. The mechanism of coma in NHHC is believed related to shifts of free water from the cerebral extravascular space to the hypertonic intravascular space, with subsequent intracellular dehydration, accumulation of metabolic products of glucose, and brain shrinkage. It is uncertain whether injury to specific areas in the CNS is a predisposing factor to the development of NHHC. Factors documented to be significant in its development include nonspecific stress to primary illnesses, hyperosmolar tube feedings, dehydration, diabetes and mannitol, Dilantin, or steroid administration.
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2/9. critical illness with hyponatraemia and impaired cell membrane integrity--the "sick cell syndrome" revisited.

    OBJECTIVE: To determine whether impaired cell membrane permeability exists in critically ill patients with "sick cell" type hyponatraemia. DESIGN AND methods: A 36 year old male patient was identified in an intensive care unit (ICU) with liver disease and multi-organ failure. His initial serum sodium (Na) was 101 mmol/L and osmolar gap 35 mmol/L. A flow cytometric system was used to assess lymphocyte membrane integrity using fluorescein diacetate (FDA) and propidium iodide (PI). Following this, similar studies were carried out in 17 hyponatraemic (Na < 130 mmol/L) and 19 normonatraemic (Na > 136 mmol/L) ICU patients. RESULTS: flow cytometry in the index patient showed two clear populations of cells-one was normal (with identical characteristics to a healthy control) and the other had dysfunctional cell membrane integrity. The extended patient series, however, revealed only 2 other patients with similar flow cytometric patterns-one hyponatraemic and one normonatraemic. CONCLUSIONS: Cell membrane studies in the index patient demonstrated supportive evidence for the "sick cell syndrome" in critically ill patients. The extended series revealed that 3/37 (8%) had this abnormality, which was however not consistently associated with hyponatraemia.
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3/9. Acute brain syndrome as a consequence of the Cronkhite-canada syndrome.

    BACKGROUND: Cronkhite-canada syndrome is a very rare illness and psychical disturbances developed as a consequence of this illness are very rarely described. That is the case because majority of the symptoms of this syndrome are associated with the polyposis of the gastrointestinal tract. AIM: The aim of this case report is to link the development of acute brain syndrome with this rare syndrome. PATIENT: The patient was two times treated under the diagnosis of Cronkhite-canada syndrome in the clinics of internal medicine in Rijeka and Zagreb, and then in the Psychiatric Clinic in Rijeka under the diagnosis of acute brain syndrome (F05.0). RESULT: Therapy with a typical antipsychotic (haloperidol) and corresponding internistic treatment gave results very fast and calmed acute delirium. CONCLUSION: Typical antipsychotics are efficient in the treatment of the acute brain syndrome caused by Cronkhite-canada syndrome. Acute brain syndrome is caused solely by the lack of electrolytes and other important nutrients because of a malabsorption syndrome, conditioned by a diffuse polyposis of intestines.
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4/9. Sick cell syndrome in a burned patient.

    The sick cell syndrome is a disorder of the cellular Na /K pump with several causes which include hypoxia, sepsis, hypovolaemia and malnourishment. We report an example of the sick cell syndrome which occurred twice to a patient admitted to our Burn Centre, the first time due to hyponutrition and the second time septicaemia. The striking features of this syndrome were hyponatraemia (less than 130 mmol) despite an increasing sodium intake, a reduced natriuria (less than 20 mmol), a trend to hyperkalaemia and unchanged haematological parameters. Clinically the syndrome was characterized by confusion and hallucinations, and the problem was solved by appropriate treatment of the cause.
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5/9. Hypoelectrolytemia and metabolic alkalosis in infants with cystic fibrosis.

    The records of all children in the Tucson area diagnosed as having cystic fibrosis (CF) before the age of 12 months were reviewed to ascertain the prevalence of metabolic alkalosis as a major presenting manifestation of CF. Five of eleven infants (46%) in whom CF had been diagnosed between 1 and 12 months of age initially were seen with hypokalemia, hypochloremia, and metabolic alkalosis unassociated with marked dehydration, hyperpyrexia, or major pulmonary and/or gastrointestinal symptoms. Two infants had repeated episodes of metabolic alkalosis; for one of these infants, both episodes of metabolic alkalosis occurred before the diagnosis of CF. It is postulated that chronic loss of sweat electrolytes together with mild gastrointestinal or respiratory illness may predispose the infant with cystic fibrosis to a severe electrolyte and acid-base disturbance. The lack of shock and hyperpyrexia together with the apparent chronicity of electrolyte losses differentiates metabolic alkalosis from the heat prostration syndrome, a more acute complication of cystic fibrosis. Quantitative sweat testing should be part of the evaluation of any infant with unexplained metabolic alkalosis. serum electrolytes should be assessed regularly in infants with cystic fibrosis during hot weather months.
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6/9. Hypoelectrolytemia as a presentation and complication of cystic fibrosis.

    We describe three patients with cystic fibrosis (CF) with four episodes of hypoelectrolytemia, two of which were associated with documented metabolic alkalosis. CF should be included in the differential diagnosis of infants and children presenting with hypoelectrolytemia and metabolic alkalosis. patients with CF are at risk of developing severe hypochloremia in hot weather and during intercurrent illness. An abrupt diminution of oral intake is an important suggestive sign, alerting the physician to look for the development of hypoelectrolytemia.
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7/9. Changing feeding trends as a cause of electrolyte depletion in infants with cystic fibrosis.

    Between June 1979 and June 1980, 16 infants with cystic fibrosis (CF) were cared for at the James Whitcomb Riley Hospital for Children. Five of these children (average age 5.8 months) had a total of eight episodes of electrolyte depletion, with six episodes unassociated with high environmental temperature, fever, or significant gastrointestinal symptoms. Their primary symptoms were poor weight gain and anorexia. According to their dietary records, these five infants, at the time of their initial presentation, had an average electrolyte intake of 8 mEq of sodium, 12 mEq of potassium, and 10 mEq of chloride per day. All infants had been fed either standard infant formula or breast milk. Infant feeding surveys indicate that the estimated average sodium intake of 6-month-old infants has decreased from 45 mEq/day in 1965 to 15 mEq/day since 1977 when manufacturers stopped adding salt to baby foods. In addition, since 1971 the percentage of infants 6 months age receiving breast milk or standard infant formula rather than cow's milk, which is higher in sodium content, has increased from 33% to 72%. This decreased salt intake places the infant with CF at greater risk for electrolyte depletion than in the past. It is expected that a larger percentage of infants with CF will have electrolyte depletion as their initial symptom especially during periods of increased sweating or when electrolyte losses are experienced during gastrointestinal illnesses. CF should be suspected in any infant with electrolyte depletion, and infants known to have CF need daily salt supplementation. serum electrolytes should be measured if the infant is experiencing weight loss or anorexia, particularly during periods of excessive salt losses.
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8/9. Toxic shock syndrome, a newly recognized disease entity. Report of 11 cases.

    The toxic shock syndrome has only recently been described. Eleven female patients aged 13 to 43 years (median 17) with toxic shock syndrome have been seen at the Mayo Clinic since August 1975. One patient died. Seven patients had one or more recurrences. As previously described, the syndrome was often life-threatening, afflicted mostly menstruating females, and was characterized by a very brief prodromal illness consisting of high fever, vomiting, diarrhea, conjunctivitis, headache, irritability, sore throat, myalgias, abdominal tenderness, and erythematous rash. The disorder can progress to hypotension or prolonged refractory shock, adult respiratory distress syndrome, diffuse intravascular coagulation with severe thrombocytopenia, and renal failure. pancreatitis was observed in two cases. During convalescence, pronounced desquamation and peeling of the skin occurred. Numerous laboratory abnormalities are observed. In 5 of the 11 patients, staphylococcus aureus was isolated from conjunctiva, oral cavity or nares, vagina, or stool. A recently described pyrogenic exotoxin was identified in the isolates of three patients; its etiologic role remains speculative. Therapy is mainly supportive. Antistaphylococcal therapy for the acute illness and for prevention of recurrences has not yet proved to be of any benefit. The role of vaginal tampons, if any, in the pathogenesis of this disorder remains unclear.
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9/9. Human rabies encephalomyelitis.

    Seven weeks after he was bitten on the lip by a puppy in the gambia a patient showed symptons of rabies. Passive and active immunisation was begun three days after the onset of symptons. The evidence indicated that death was a direct consequence of the central nervous system disease rather than any associated complication. Our inability to alter the course of the illness appreciably emphasises the importance of immediate postexposure immunisation in rabies and draws attention to the present lack of effective means of preventing virus replication within the central nervous system.
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