[Prolonged spinal and sacral neurostimulation in children with pelvic organ dysfunction: preliminary analysis]. / Dlitel'naya spinal'naya i sakral'naya neirostimulyatsiya u detei pri narusheniyakh funktsii tazovykh organov: predvaritel'nyi analiz.

OBJECTIVE: To evaluate the clinical efficacy of long-term spinal and sacral programmable neurostimulation for pelvic organ dysfunction in patients with myelodysplasia and chronic dysfunction of the bladder and rectum. MATERIAL AND METHODS: A retrospective study included 32 children aged 1-17 years (mean 10.7) with myelodysplasia, pelvic organ dysfunction and ineffective therapy including botulinum therapy and exclusion of tethered spinal cord syndrome. All children underwent comprehensive urodynamic examination with analysis of bladder and residual urine volume, mean flow rate, intravesical pressure and total urine volume, as well as electromyographic examination. Examination was carried out before surgery, after 6, 12 and 36 months. We applied urinary diary, NBSS questionnaire and urodynamic examination data. All patients underwent neurological examinations (neurological status, magnetic resonance imaging of the spinal cord, computed tomography and radiography of the spine, electroneuromyography). The study was conducted at the neurosurgical department of the Republican Children's Clinical Hospital in Ufa between 2014 and 2022. There were 32 implantations of epidural neurostimulators for pelvic organ dysfunctions. RESULTS: Patients used epidural spinal and sacral stimulation up to 6 times a day for 10-15 min turning on the pulse generator. This method significantly increased urinary volume, decreased episodes of urinary leakage and fecal incontinence, residual volume after urination and number of periodic catheterizations compared to baseline data. Sixteen patients were very satisfied, 10 ones were moderately satisfied, and 2 patients were not satisfied with therapy. The number of bladder catheterizations per day decreased by 51.1%. Urine volume significantly increased from 131.5±16.1 to 236±16.7 ml, intravesical pressure decreased from 23.5±4.2 to 18.5±2.1 cm H2O (by 20.3%). CONCLUSION: Chronic epidural spinal and sacral stimulation can improve the quality of life in patients with pelvic organ dysfunction. This technique may be effective for pelvic organ dysfunction caused by myelodysplasia.

Postoperative hyperthermia-induced multiple organ failure in a child with Down syndrome: a case report.

BACKGROUND: Psychological stress has been reported to cause hyperthermia. Persistent excessive hyperthermia can, in turn, cause hypercytokinemia and organ damage. We report a case of postoperative severe hyperthermia leading to a systemic inflammatory response and multiple organ failure in a child with Down syndrome. CASE PRESENTATION: A 10-month-old native Japanese boy with Down syndrome and Hirschsprung's disease is described. Newborn screening showed congenital hypothyroidism and a ventricular septal defect, but these conditions were stable upon administration of levothyroxine and furosemide. His development was equivalent to that of a child with Down syndrome. He developed a noninfectious high fever twice after preoperative preparations at age 8 months and again at 9 months. He was readmitted to hospital at age 10 months to undergo the Soave procedure to correct Hirschsprung's disease. However, he contracted a fever immediately after the surgical procedure. Hyperthermia (42 °C) was refractory to acetaminophen treatment and deteriorated to multiple organ failure due to hypercytokinemia, with increased serum levels of interleukin-6 (44.6 pg/mL) and interleukin-10 (1010 pg/mL). He died on postoperative day 2 with hypoxemia, respiratory/metabolic acidosis, increased serum levels of transaminases, reduced coagulation, and pancytopenia. Various infectious and noninfectious causes of hyperthermia could not be identified clearly by culture or blood tests. CONCLUSIONS: We speculated that the proximate cause of the fever was psychological stress, because he suffered repeated episodes of hyperthermia after the invasive procedure. Hyperthermia, together with the immune-system disorders associated with Down syndrome, may have induced hypercytokinemia and multiple organ failure. This rare case of noninfectious postoperative hyperthermia leading to multiple organ failure may help to shed further light on the currently unclear pathogenic mechanism of hyperthermia and associated multiple organ failure during the perioperative period in children.

Severe clinical manifestation of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase deficiency associated with two novel mutations: a case report.

BACKGROUND: Mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase (mHS) deficiency is an autosomal recessive inborn error of metabolism, which will give rise to failure of ketogenesis in liver during illness or fasting. It is a very rare disease with only a few patients reported worldwide, most of which had a good prognosis after proper therapies. CASE PRESENTATION: We report a 9-month-old boy with mHS deficiency presenting with unusually severe and persistent acidosis after diarrhea and reduced oral food intake. The metabolic acidosis persisted even after supplementation with sugar and alkaline solution. Blood purification and assisted respiration alleviated symptoms, but a second onset induced by respiratory infection several days later led to multiple organ failure and death. Urine organic acid analysis during the acute episode revealed a complex pattern of ketogenic dicarboxylic and 3-hydroxydicarboxylic aciduria with prominent elevation of glutaric acid and adipic acid, which seem to be specific to mHS deficiency. Plasma acylcarnitine analysis revealed elevated 3-hydroxybutyrylcarnitine and acetylcarnitine. This is the first report of elevated 3-hydroxybutyrylcarnitine in mHS deficiency. Whole exome sequencing revealed a novel compound heterozygous mutation in HMGCS2 (c.100C > T and c.1465delA). CONCLUSION: This severe case suggests the need for patients with mHS deficiency to avoid recurrent illness because it can induce severe metabolic crisis, possibly leading to death. Such patients may also require special treatment, such as blood purification. Urine organic acid profile during the acute episode may give a hint to the disease.

Effect of Serum Triglycerides on Clinical Outcomes in Acute Pancreatitis: Findings From a Regional Integrated Health Care System.

OBJECTIVES: The aim of this study was to evaluate the effect of serum triglycerides on the development of multiple or persistent organ failure in patients with acute pancreatitis. METHODS: A retrospective cohort study was conducted among patients hospitalized for acute pancreatitis between 2006 and 2013. Triglyceride levels measured before and within 72 hours of admission were compared. In addition, the effect of triglyceride levels on the development of multiple or persistent organ failure during hospitalization for acute pancreatitis was assessed. RESULTS: Among 2519 patients, 267 patients (10.6%) developed organ failure, of which 75 patients developed multiple system organ failure and 82 patients developed persistent organ failure. Triglyceride levels in patients who developed organ failure were initially much higher than in patients who did not develop organ failure, but by 72 hours into admission, approached levels of patients who did not develop organ failure. Approximately 8% of patients had triglyceride levels greater than 500 mg/dL, the majority of which had similarly high levels before admission. CONCLUSIONS: Increased triglyceride levels were associated with the development of multiple or persistent organ failure among patients hospitalized with acute pancreatitis. Patients with high triglyceride levels at the time of admission were likely to have high triglyceride levels before admission.

Toxic epidermal necrolysis after extensive dermal use of realgar-containing (arsenic sulfide) herbal ointment.

BACKGROUND: Realgar (arsenic sulfide) is thought to be safe with few reports on toxicities or adverse effects and has been used in Traditional Chinese Medicine for many centuries. Serious realgar poisoning is rare, and we report a fatal case resulted from short-term use of realgar-containing herbal medicine through dermal route. CASE DETAILS: A 24-year-old man with atopic dermatitis had received 18 days of oral herbal medicine and realgar-containing herbal ointments over whole body from a Traditional Chinese Medicine (TCM) practitioner. Seven days after the herbal use, he had diminished appetite, dizziness, abdomen discomfort, itching rash, and skin scaling. He later developed generalized edema, nausea, vomiting, decreased urine amount, diarrhea, vesico-edematous exanthems, malodorous perspiration, fever, and shortness of breath. He was taken to the hospital on Day 19 when the dyspnea became worse. Toxic epidermal necrolysis complicated with soft tissue infection and sepsis was noted, and he later died of septic shock and multiple organ failure. The post-mortem blood arsenic level was 1225 µg/L. Herbal analysis yielded a very high concentration of arsenic in three unlabeled realgar-containing ointments (45427, 5512, and 4229 ppm). CONCLUSION: Realgar-containing herbal remedy may cause severe cutaneous adverse reactions. The arsenic in realgar can be absorbed systemically from repeated application to non-intact skin and thus should not be extensively used on compromised skin.

Muscle weakness in septic patients requiring mechanical ventilation: protective effect of transcutaneous neuromuscular electrical stimulation.

PURPOSE: The aim of this study was to evaluate the effect of transcutaneous neuromuscular electrical stimulation (NMES) on muscle strength in septic patients requiring mechanical ventilation (MV). METHODS: Sixteen septic patients requiring MV and having 1 or more organ failure other than respiratory dysfunction were enrolled within 48 hours from admission to the intensive care unit. Neuromuscular electrical stimulation was administered twice a day on brachial biceps and vastus medialis (quadriceps) of 1 side of the body until MV withdrawal. Blinded investigators measured arm and thigh circumferences, biceps thickness by ultrasonography, and muscle strength after awakening with Medical Research Council scale. RESULTS: Two patients died before strength evaluation and were excluded from the analysis. Neuromuscular electrical stimulation was applied for 13 days (interquartile range, 7-30 days). Biceps (P = .005) and quadriceps (P = .034) strengths were significantly higher on the stimulated side at the last day of NMES. Improvement was mainly observed in more severe and weaker patients. Circumference of the nonstimulated arm decreased at the last day of NMES (P = .015), whereas no other significant differences in limb circumferences or biceps thickness were observed. CONCLUSION: Neuromuscular electrical stimulation was associated with an increase in strength of the stimulated muscle in septic patients requiring MV. Neuromuscular electrical stimulation may be useful to prevent muscle weakness in this population.

[Effects of Chinese herbal medicine Dusuqing Granule on toll-like receptor 4 signaling in multiple organ injury induced by bacterial pneumonia in aged rats].

OBJECTIVE: To study the protective mechanism of Dusuqing Granule, a compound Chinese herbal medicine, on the senile multiple organ injury caused by bacterial pneumonia by observing the expression changes of molecules related to toll-like receptor 4 (TLR4) signaling. METHODS: A total of 55 male Sprague-Dawley aged rats were divided into control group, untreated group, Dusuqing group and lomefloxacin group. There were 25 rats in the untreated group and 10 rats in each of the other three groups. Multiple organ injury in a rat model of pneumonia was induced by injection of Klebsiella pneumoniae through tracheal intubation. By means of immunohistochemistry and reverse transcription-polymerase chain reaction (RT-PCR), examinations were made on mRNA expressions of lipopolysaccharide-binding protein (LBP), CD14, TLR4 and interleukin-1 receptor-associated kinase-1 (IRAK-1) in the tissues of the lung, heart and small intestine, and also on the protein expressions of TLR4, tumor necrosis factor receptor-associated factor 6 (TRAF6) and nuclear factor-κB (NF-κB). RESULTS: Expressions of LBP, CD14, TLR4 and IRAK-1 mRNAs in the tissues of the lung, heart and small intestine in the untreated group were stronger than those in the control group (P<0.01 or Plt;0.05). The protein expressions of TLR4, TRAF6 and NF-κB were increased dramatically in the untreated group as compared with the control group (Plt;0.01 or Plt;0.05). Compared with the untreated group, the expressions of LBP, CD14, TLR4 and IRAK-1 mRNAs in the tissues of the lung, heart and small intestine in the Dusuqing group were weakened significantly (Plt;0.01 or Plt;0.05). Meanwhile, the protein expressions of TLR4, TRAF6 and NF-κB were decreased markedly in the Dusuqing group (Plt;0.01 or Plt;0.05). CONCLUSION: Dusuqing Granule is effective in suppressing toll-like receptor signal transduction activation and reducing the secretion of cytokines and inflammatory mediators, which can further reduce the organ tissue injury. Dusuqing Granule can decrease the levels of TLR signal transduction activation including the targets LBP, CD-14, TLR4, IRAK-1, TRAF6 and NF-κB, which is different from the special inhibitor that acts only on some segments.

Multimodal approach to malnutrition in malnourished maintenance hemodialysis patients.

Chronic organ failures, including chronic kidney disease, cardiac failure, and chronic pulmonary disease, share a common phenotype which is characterized by a high prevalence of anorexia, inflammation, oxidative stress, insulin resistance, hypogonadism, and anemia. Because of the systemic dimension of organ failure, such a phenotype results in a decrease in body mass, in addition to alterations of body composition and abnormal muscle structure, metabolism, and performance. The response of both protein-energy wasting and muscle disease to nutritional support given alone was shown to be limited both in chronic kidney disease and chronic pulmonary disease. Data are needed to evaluate the effects of an integrated management taking into account the different factor of muscle anabolism: nutrition support, endurance exercise, and, in selected patients, other anabolic agents such as androgens and omega-3 fatty acids.

[Effects of Tongfu Granule on intestinal dysfunction in patients with multiple organ dysfunction syndrome].

OBJECTIVE: To study the mutual influences between intra-abdominal hypertension (IAH) and intestinal dysfunction, and to observe the efficacy of Tongfu Granule (TFG) in treating IAH of patients with multiorgan dysfunction syndrome (MODS). METHODS: Adopting randomized, double-blinded, controlled method, 60 MODS patients hospitalized in authors' ICU wards from August 2007 to January 2008 were equally assigned to two groups: A and B, that is, the TFG group and the placebo group, all were treated by conventional treatment but different in the administration of TFG and placebo, respectively 1 bag, three times per day, for 7 days via oral intake or gastric infusion. On the 1st, 3rd and 7th day or before death, patients were monitored in terms of symptoms (abdominal distention, defecation, borborygmus, abdominal circumference, intra-abdominal pressure, and occult blood in stool), gastrointestinal function score, APACHE II score, and 28-day outcome. The blind outcome was shown up by specified tester by the end of the observation of 60 cases, A is the TFG group, B is the placebo group. RESULTS: As compared with the placebo group, intestinal peristalsis and borborygmus were increased, the intra-abdominal pressure and circumference were decreased in the TFG group significantly, which could reduce the possibility of gastrointestinal hemorrhage. Moreover, the gastrointestinal function score and APACHE II score were improved and the 28-day mortality was reduced to a certain degree in the TFG group. CONCLUSION: TFG could promote the recovering of gastrointestinal function and relieve the heightened intra-abdominal pressure in patients with MODS so as to improve patients' prognosis to some extents.

Late association of hyperparathyroidism in septic patients with multiple organ failure.

BACKGROUND: Hypocalcemia after severe shock or sepsis stimulates release of parathyroid hormone (PTH), which abates with recovery. Sustained sepsis with multiple organ failure (MOF), however, may cause a resurgent release of PTH and life-threatening hypercalcemia. METHODS: Thirty critically ill patients with prolonged sepsis developed combined hypercalcemia with elevated serum PTH levels. The primary insult was trauma in 12 patients, peritonitis in 14, and pancreatitis in 4. MOF involved the lungs in 30 patients, kidney in 16, gut in 30, brain in 13, and a coagulopathy in 10. There were 12 deaths; hospital stay averaged 81 days. RESULTS: Hypercalcemia with increased serum levels of PTH occurred usually 3-4 weeks after the septic insult. Bradycardia, thought to be caused by the hypercalcemia, occurred in 19 patients, was attributed to a vasovagal reaction, and was treated with atropine. When asystole resulted, epinephrine and cardiopulmonary resuscitation were administered. Five patients required placement of an intravenous pacemaker. Bradycardia was acutely lethal in 4 patients; in a 5th patient, the decision was made for comfort care alone, and he died 9 days later. Bisphosphonate was given to 7 patients with this hypercalcemic-induced bradycardia and, prophylactically, to prevent bradycardia in 9 others. Hypercalcemia corrected in all patients; bradycardia abated in 7 patients. CONCLUSION: Hyperparathyroidism may occur with MOF secondary to sepsis. The mechanism is unclear, but the resultant bradycardia can be life threatening. Treatment with bisphosphonate corrects the hypercalcemia and bradycardia. Both the hypercalcemia and the bradycardia normalize when the MOF resolves.

Protection in rats with heatstroke: hyperbaric oxygen vs activated protein C therapy.

The present study was attempted to evaluate the therapeutic effects of activated protein C and/or hyperbaric oxygen in an animal model of heatstroke. Sixty-eight minutes heat stress (43 degrees C) initiated, the anesthetized rats were randomized to several groups and administered: 1) no resuscitation (vehicle solution plus normabaric air, 2) intravenous activated protein C (1mg in 1ml of normal saline per kg of body weight), 3) hyperbaric oxygen (100% oxygen at 202kpa for 17min), and 4) intravenous activated protein C plus hyperbaric oxygen. Another group of rats exposed to room temperature (26 degrees C) was used as normothermic controls. Blood sampling was 0min, 70min, and 85min after heat stress initiated. When the vehicle-treated rats underwent heat exposure, their survival time values found were to be 19-25min. Resuscitation with activated protein C or hyperbaric oxygen significantly and equally improved survival during heatstroke (134-159min). As compared with those of activated protein C or hyperbaric oxygen alone, combined activated protein C and hyperbaric oxygen significantly had higher survival time values (277-347min). All vehicle-treated heatstroke animals displayed systemic response, hypercoagulable state, and hepatic and renal dysfunction. Combined activated protein C and hyperbaric oxygen therapy reduced these heatstroke reactions better than activated protein C or hyperbaric oxygen alone. The results indicate consequently, combined activated protein C and hyperbaric oxygen therapy heightens benefit in combating heatstroke reactions.

Investigation of the alterations in cellular electrophysiology underlying ventricular arrhythmia in dogs with the multiple organ dysfunction syndrome.

BACKGROUND: Multiple organ dysfunction syndrome (MODS)-specific cellular electrophysiological changes have so far not been reported and it seemed unlikely that they were related to arrhythmogenesis. METHODS AND RESULTS: Twelve dogs, weight 12 +/- 2 kg, were divided into a control group (n = 6) and an MODS group (n = 6). MODS lasting for 72 h was induced by the 'two-hit' method in 6 dogs. Ventricular myocytes were enzymatically isolated. Early afterdepolarizations (EADs), action potential duration (APD) and L-type calcium currents (ICa,L) were assessed. Sinus arrhythmias in all MODS dogs (100%; 6 of 6) and premature ventricular beats in 4 MODS dogs (66%; 4 of 6) were recorded, while no arrhythmias were found in the control animals. The prolongation of the APD was associated with a decreased ICa,L, and frequently provoked EADs were the typical electrophysiological alterations in the myocytes of MODS dogs. The action potential prolongation was shortened, the ICa,L blocked and EAD suppressed by using verapamil (100 micromol/l) in the myocytes of MODS dogs (66%; 4 of 6). CONCLUSION: The changes in cellular electrophysiology within 72 h in the heart of MODS dogs are APD prolongation, markedly decreased ICa,L as well as frequently provoked EAD, the most common types of arrhythmia being sinus arrhythmia and premature ventricular beats. This study suggests that verapamil appears to be an effective agent in reversing alterations in cellular electrophysiology at the early stage of MODS.

[Relationship between immune imbalance and sthenia-asthenia syndromes in patients suffering from severe intra-abdominal infection].

OBJECTIVE: To explore the characteristics of immune imbalance in patients with multiple organ dysfunction syndrome (MODS) induced by severe intra-abdominal infection and its relationship with changing of TCM sthenia-asthenia syndrome. METHODS: Forty-six patients with MODS induced by severe intra-abdominal infection and treated with etiological and syndrome differentiation of integrative medicine were observed in succession. Patients' peripheral blood levels of interleukin-6/interleukin-10 ratio (IL-6/IL-10), human leukocyte antigen DR site (HLA-DR), helper T lymphocyte1/2 ratio (Th1/Th2), and the regulatory T lymphocyte (Treg) were measured on the 1st, 3rd and 7th day of the research respectively. And the distribution laws of TCM syndrome types, sthenia (S), asthenia (A), and mingled sthenia/asthenia (M), in patients were observed as well. RESULTS: IL-6/IL-10 ratio at all the testing time points showed insignificant difference in patients of types S and M, while in those of type A, it was more lowered on the 7th day than that on the 1st day. HLA-DR lowered to <30% on the 7th day in all patients of type A and showed significant difference to that on the 1st day (P <0.05), while HLA-DR <30% was not found in all patients of types S and M. Th1/Th2 ratio in patients of types S and A was insignificant different at the foremost 3 days, but lowered significantly on the 7th day, while in patients of type M, it was unchanged in all the 7 days of observation. Treg level was unchanged in the foremost 3 days in patients of types S and M, while in those of type A, it raised on the 3rd day, but no raising was found in the subsequent 4 days. Comparisons of various indexes detected at corresponding time points respectively among patients with various syndrome types showed that, for levels of IL-6/IL-8, HLA-DR, and Th1/Th2, the sequence was S>M>A; and for Treg, it was A>M>S. CONCLUSION: In the pathological process of MODS induced by severe intra-abdominal infection, the index IL-6/IL-10, reflecting the balance of the pro-/anti-inflammatory cytokines and the indexes HLA-DR, Th1/Th2 and Treg reflecting the immune function, all can exactly reflect the TCM asthenia-sthenia syndrome types. The sequence in patients of various syndrome types for levels of IL-6/IL-10, HLA-DR and Th1/Th2, is S> M>A, but for Treg it is the inverse, as A>M>S.

[Value of four scoring systems for predicting prognosis of severe acute pancreatitis].

OBJECTIVE: To evaluate the value of Ranson, acute physiology and chronic health evaluation II (APACHE II), Balthazar CT and pancreatitis outcome prediction (POP) scoring systems in predicting the prognosis of severe acute pancreatitis (SAP). METHODS: A total of 253 consecutive patients with SAP, admitted into West China Hospital of Sichuan University from January 2004 to August 2007 were studied retrospectively. Data associated to Ranson, APACHE II, Balthazar CT and POP scoring systems after admissions were recorded. Four outcome signals including death, local complications, operation transformation, and development of multiple organ dysfunction syndrome (MODS) were analyzed. The receiver operating characteristic (ROC) curves were also drawn and the areas under the ROC curve (AUC) were obtained to compare the sensitivity, specificity, Youden index, and likelihood ratio positive test (LRPT) of the clinical scoring systems in assessing the prognosis of SAP. RESULTS: Through comparison of AUC, Ranson score could be used to predict the outcomes except for local complications (P=0.00), and all its diagnosis threshold was 5. APACHE II score showed that it could assess the outcomes of SAP except for local complications (P=0.00). Its thresholds in mortality, operation transformation, and incidence of MODS were 14, 10, and 12 respectively. Balthazar CT could predict all the outcomes, and the threshold was 6. POP score could predict all the outcomes except for local complications, and its predictive value in mortality was particularly prominent. The sensitivity and specificity of POP in predicting death were 0.90 and 0.92 as the cutoff was no less than 14. The thresholds of POP in mortality, operation transformation, and incidence of MODS were 14, 8, and 10 respectively. CONCLUSION: The prognostic ability of the four scoring systems is not the same. Ranson, APACHE II and POP scoring systems can predict the severity of SAP and entire complication, but they can't predict the local complications. POP score may predict the mortality well, and Balthazar CT scanning can predict all the outcomes, but the sensitivity and specificity are low.

[Clinical observation on treatment of gastrointestinal dysfunction by fu'an liquid for retention enema in children with critical illness].

OBJECTIVE: To observe the therapeutic effect of Fu'an Liquid (FAL) for retention enema in treating gastrointestinal (GI) dysfunction of children with critical illness. METHODS: Eighty-nine patients were randomly divided into two groups, 52 in the treated group and 37 in the control group. Conventional therapy of western medicine was given to both groups and to the treated group FAL was given additionally. Plasma endothelin (ET) level was measured during admission, GI dysfunction occurrence and after treatment, and the therapeutic effect as well as the recovery of GI condition were observed. RESULTS: The total effective rate of FAL in treating GI dysfunction was 84.62%, which was significantly higher than that in the control group (62.17%) (P < 0.05). In the treated group, 34 cases were treated successfully, 16 died and the other 2 abandoned, the mortality rate being 30.77%, while in the control group, the corresponding numbers were 16, 18, 3 and 48.65%. The mortality rate in the treated group was lower than that in the control group (chi 2 = 4.64, P < 0.05). Level of ET in both groups was higher than normal range during admission (P < 0.01), it further increased when GI dysfunction occurred (P < 0.01), and decreased when successfully treated, the decrease was quicker in the treated group than that in the control group (P < 0.05). CONCLUSION: In children with critical illness, ET level would increase when the patient was complicated with GI dysfunction. FAL for retention enema could reduce the ET level effectively, promote the recovery of patients from GI dysfunction, so as to play a definite role in enhancing the successful rate of rescue.

Multiple organ failure associated with extensive metastatic calcification in a patient with an intermediate state of human T lymphotropic virus type I (HTLV-I) infection: report of an autopsy case.

A patient with an intermediate state of human T lymphotropic virus type I (HTLV-I) infection and in whom autopsy showed multiple organ failure (MOF) associated with extensive metastatic calcification in systemic organs is described. A 56-year-old man presented with signs and symptoms of advanced cardiac insufficiency, respiratory disturbance and renal failure. Serologically, the anti-human T lymphotropic virus type I (HTLV-I) antibody titer and the levels of both calcium and parathyroid hormone-related peptide (PTHrP) were distinctly elevated. These data suggested a diagnosis of adult T cell lymphoma/leukemia (ATLL). However, examination of a peripheral blood sample revealed only a few atypical lymphoid cells (3%) associated with mild leukocytosis (white blood cell count, 13.7 x 10(3)/mm3). Lymph node swelling was systemic but mild, with some nodes up to 10 mm in diameter. The patient died of MOF. Adult T cell leukemia/lymphoma was unable to be diagnosed definitively because of the short duration of laboratory abnormalities and because of the discrepancy between the laboratory data and the magnitude of lymphoproliferation in both the lymph nodes and peripheral blood. At autopsy, the most conspicuous finding was extensive metastatic calcification in the multiple organs, including the heart, lungs, kidneys, tongue, liver, pancreas, spleen and systemic arterial walls. Very small numbers of medium-sized atypical lymphoid cells admixed with small reactive lymphocytes were identified in multiple organs, with no evidence of massive infiltration. Molecular analyses could not detect monoclonal integration of HTLV-I provirus DNA or monoclonality of T cell lineage cells. Parathyroid hormone-related peptide was demonstrated in the cytoplasm of the atypical lymphoid cells on immunohistochemical examination. The bone trabeculae generally showed distinct evidence of resorption associated with marked proliferation of osteoclasts. These findings suggested that the hypercalcemia in the present case was categorized as humoral hypercalcemia of malignancy rather than local osteolytic hypercalcemia.

[L-carnitine levels in critical septic patients receiving parenteral nutrition]. / Niveles de L-carnitina en pacientes críticos sépticos con nutrición parenteral.

Septicemia causes multiple and often not very well understood metabolic alterations. In this sense it is controversial whether or not carnitine is decreased, which may have several implications. Our objective is to measure the plasma carnitine levels in septicemic patients, and to find out whether this is modified if there is a multi-organ dysfunction syndrome (MODS), or if it is dependent on the lipid source. For this we carried out a prospective study in septicemic patients with MODS. These were given exclusively parenteral nutrition (PN) without any carnitine supplementation. The PN of 16 patients contained long chain triglycerides (LCT's), while that of another 12 contained a 1:1 mixture of long and medium chain triglycerides (LCT's and MCT's). We measured the plasma carnitine level at the baseline, after 5 days and after 10 days, using an enzymatic method that measures the total carnitine level (free and esterified). The normal values lie between 35 and 70 mumol/l. We included 28 septicemic patients whose ages were 53.41 +/- 16.51 years, and whose APACHE II on admission was 17 +/- 4. The carnitine levels were: baseline: 60.4 +/- 23.7; 5th day 57.7 +/- 22.9; 10th day 55.7 +/- 21.2 (p = n.s.). Of these patients, 10 had an MODS of septic origins with their baseline levels being: 65.3 +/- 30.9; 5th day 60.3 +/- 23.2; 10th day 61.5 +/- 15.5; while the levels of the 18 septicemic patients without MODS, the baseline levels were 61.9 +/- 13.8; 5th day 58.6 +/- 19.1; 10th day 56.6 +/- 19.3 (p = n.s.). In the patients who were given LCT's the baseline carnitine level was 60.7 +/- 23.1, 5th day 60.1 +/- 23.8; 10th day 58.6 +/- 12.8; while those patients who were given LCT/MCT showed baseline levels of 64.3 +/- 19.5; 5th day 58.6 +/- 19.1; 10th day 57.8 +/- 10.7 (p = n.s.). In our septicemic patients the serum carnitine levels we found were within the normal range, and these remained unchanged during the ten days in those patients with MODS or with the lipid mixture used.