[Chronic liver disease associated with cystic fibrosis in the Hospital Alberto Sabogal Sologuren, Lima, Peru: report of a case]. / Hepatopatía crónica asociada a fibrosis quística en el Hospital Alberto Sabogal Sologuren, Lima, Perú: reporte de caso.

Cystic fibrosis (CF) is the most frequent recessive genetic disorder in the caucasian population and is produced by the alteration of electrolyte and water transport in the epithelial cell membrane. Liver disease is a frequent complication towards the end of the first decade of life, being weird its onset, except in patients with a history of meconium ileus. The characteristic liver injury in CF is focal biliary cirrhosis, but fatty infiltration can also be found. The diagnosis is made considering the clinical, laboratory and imaging results having in consideration that the normal liver function tests do not rule out the disease. Ultrasound is the most widely used and can detect the presence of steatosis, stones, fibrosis, cirrhosis, portal hypertension or abnormalities of the biliary tree. There is an also available technique such as computed tomography or magnetic resonance imaging, which allows a morphological study. Important aspects in the treatment are nutritional management, administration of soluble vitamins and the use of ursodeoxycholic acid (UDCA). In cases of advanced cirrhosis, transplantation, isolated or combined with the lung, is an option to consider, with acceptable survival rates. We report the case of an 11 year old patient with a diagnosis of chronic liver disease associated with cystic fibrosis.

Cor pulmonale due to tumor cell microemboli. Report of a case with occult gastric carcinoma.

Progressive dyspnea developed in a 36-year-old woman. Physical examination and chest roentgenogram showed the signs of pulmonary hypertension. She died of respiratory failure in spite of treatment. Autopsy disclosed gastric carcinoma in the pylorus with metastases to the regional lymph nodes, left adrenal gland, and ovaries. There were no gross pulmonary emboli, but more than 50% of pulmonary microvasculature was occluded by tumor cell microemboli. No parenchymal metastases were found in the lung. This case was remarkable because cor pulmonale due to tumor cell microemboli to the lung was the initial and terminal manifestation of clinically occult, but pathologically advanced, gastric carcinoma.

Electrocardiographic abnormalities in patients with acute aortic dissection.

In conclusion, acute electrocardiographic changes were not uncommon in patients with acute aortic dissection. It was suggested that acute electrocardiographic changes in aortic dissection resulted from at least 1 of the following 3 mechanisms: (1) involvement of the ostium of the coronary artery; (2) shock state, especially tamponade; and (3) preexisting coronary artery disease. We believe that physicians should be aware of the possibility of acute aortic dissection not only in patients with chest pain with a normal electrocardiogram, but also in those with electrocardiographic changes.

Left ventricular function after cigarette smoking by chronic smokers: comparison of normal subjects and patients with coronary artery disease.

Twenty normal subjects and 25 patients with coronary artery disease underwent systolic time interval analysis before and after rapidly smoking two cigarettes. A slight increase in heart rate and arterial pressure was seen in both groups. In patients with coronary artery disease, preejection period/left ventricular ejection time ratio increased; in normal subjects it decreased. Left ventricular performance is diminished after cigarette smoking among subjects who have preexisting significant coronary artery disease.

Motor unit properties of nerve-intact extensor digitorum longus muscle grafts in young and old rats.

Impaired reinnervation has been implicated as the cause of the threefold disparity in the recovery of maximum force (P0) of standard muscle grafts in old compared with young rats. The specific, null hypothesis of this study is that compared with age-matched control extensor digitorum longus (EDL) muscles, nerve-intact EDL muscle grafts in young and old rats show no evidence of an age-related impairment in reinnervation. Nerve-intact grafts were performed in 3-month-old and 23-month-old rats and were evaluated 60 days postoperatively. Compared with age-matched control EDL muscles, nerve-intact grafts in young and old rats showed no difference in muscle mass or motor unit numbers. The mean motor unit P0 for nerve-intact graft muscles in both age groups was significantly lower than that of age-matched control muscles. These data support our hypothesis that if axons are allowed to regenerate in an endoneurial environment, there is no evidence of an age-related impairment in muscle reinnervation.

EMG biofeedback training for a mentally retarded individual with cerebral palsy.

Electromyographic biofeedback training was used to facilitate finger and wrist extension movements in a mentally retarded individual with spastic hemiplegia. The client was a 29-year-old woman with profound mental retardation and cerebral palsy as a result of postnatal meningitis. During treatment, the client demonstrated a short attention span, lack of motivation, low frustration tolerance, and increased spasticity in her left hand. The client was able to hyperextend her fingers following finger extension training, although goniometric measurements showed a marked degree of wrist flexion and wrist ulnar deviation. Therefore, training was implemented to develop wrist extension movements. Biofeedback therapy resulted in a substantial increase in active wrist extension and a marked reduction in wrist ulnar deviation.

Eye socket reconstruction with free-flap transfer.

Patients afflicted with retinoblastoma or other malignant orbital tumors often undergo eyeball excision and irradiation in infancy, which results in severe deformities of the orbit. It is very difficult to achieve the reconstruction of such severely depressed and contracted eye sockets because both augmentation and formation of an eye socket are required. We usually perform a free-flap transfer with microvascular anastomoses in such situations and have experienced 25 cases since 1980. Various kinds of flaps, such as the groin flap, dorsalis pedis flap, scapular flap, and others, were utilized. The degree of depression and whether sufficient conjunctiva for lining of the eye socket remains are important in the selection and design of the donor flap. To obtain an orbit with a natural appearance, many of the patients required subsequent minor revision operations such as debulking, dermal fat grafts, canthoplasty, fascia suspension, and so on.

Neurovascular free muscle transfer combined with cross-face nerve grafting for the treatment of facial paralysis in children.

Microneurovascular free muscle transfer is fast becoming a standardized procedure in the treatment of established or long-standing facial paralysis. However, there is no general agreement as to whether muscle transfer should be employed for children who still are growing. A patient's natural growth may influence or disturb reproduction of a smile, or a child may not be able to collaborate with postoperative rehabilitation sufficiently well to obtain a satisfactory result. One may hesitate to employ the procedure for children aged under 10 out of fear of its technical difficulties. To investigate the influence of age, the results experienced by 23 patients under the age of 15 who underwent free muscle transfer combined with cross-face nerve grafting were compared with those of adult patients. The treatment employed was a two-stage operation consisting of cross-face nerve grafting and subsequent free muscle transfer.

Versatility of a cross-leg free rectus abdominis flap for leg reconstruction under difficult and unfavorable conditions.

A cross-leg free rectus abdominis flap, anastomosing to utilized "carrier" vessels of the contralateral noninjured leg, was used for reconstruction in six cases of extensive traumatic defects and deformities of a lower extremity. This method provides great advantages in free-flap reconstruction of complicated and unfavorable cases in which utilization of what would be suitable recipient vessels in the injured leg is most difficult, but there is good blood circulation of the leg.

Tissue expansion in facial reconstruction.

The tissue expansion technique is advantageous in facial reconstruction because it makes it possible to resurface even wider defects with neighboring skin similar in color and texture and superior to skin obtained elsewhere, thereby surpassing conventional methods. However, there still remain some problems relating to procedural details, such as the selection of tissue expanders and sites of their insertion; the design, elevation, suturing, and fixation of the expanded flap; and the management of free margins such as the lower lip and lower eyelid. In each case, some modification is required with respect to the status of the defect. The experiences encountered in a series of 23 patients are described, with illustration of several representative cases, and advantages and problems are discussed.

Versatility of a free inferior rectus abdominis flap for head and neck reconstruction: analysis of 200 cases.

Between 1984 and 1991, 200 inferior rectus abdominis muscle and musculocutaneous free flap transfers were performed for reconstruction of defects or deformities resulting from removal of cancer in the head and neck region. Primary reconstruction was performed in 190 of 200 cases (95 percent), and secondary reconstruction in 10 cases (5 percent). The most common indication was tongue cancer in 78 patients, followed by cancer of the oropharynx. Total flap necrosis occurred in 10 cases (5 percent) and partial necrosis in two cases (1 percent). Donor-site morbidity was minimal. One of the distinct advantages of this flap is that the volume of the muscle and/or the fatty tissue attached to a given skin paddle can be readily adjusted to suit individual requirements. This flap has proven particularly useful for complex or large tissue defects in the head and neck region.

Mandibular reconstruction using microvascular free flaps: a statistical analysis of 178 cases.

For this article, 178 consecutive cases of mandibular reconstruction using microvascular free flaps and performed from 1979 to 1997 were studied. The purpose of this report is to compare flap success rates, complications, and aesthetic and functional results. The ages of the 131 men and 47 women ranged from 13 to 85 years, with an average of 55 years. Donor sites included the rib (11 cases), radius (one case), ilium (36 cases), scapula (51 cases), fibula (34 cases), and soft-tissue flaps with implant (45 cases). Complications included total flap necrosis, partial flap necrosis, major fistula formation, and minor fistula formation. The rate of total flap necrosis involving the ilium and fibula was significantly higher than that of all other materials combined (p < 0.05). The overall rate of implant plate removal, which resulted from the exposure or fracture of the plate, was 35.6 percent (16 of 45 cases). Each mandibular defect was classified by the extent of the bony defect and by the extent of the soft-tissue defect. The extent of the mandibular bony defect was classified according to the HCL method of Jewer et al. The extent of the soft-tissue defect was classified into four groups: none, skin, mucosal, and through-and-through. According to these classifications, functional and aesthetic assessments of deglutition and contour were performed on 115 subjects, and speech was evaluated in 110. To evaluate the postoperative results, points were assigned to each assessment of deglutition, speech, and mandibular contour. Statistical analysis between pairs of bone-defect groups revealed that there was no significant difference in each category. Regarding deglutition, statistical analysis between pairs of soft-tissue-defect groups revealed there were significant differences (p < 0.05) between the none and the mucosal groups and also between the none and the through-and-through groups. Regarding speech, there was a significant difference (p < 0.05) between the none and the through-and-through groups. Regarding contour, there were significant differences (p < 0.01) between the none and the through-and-through groups and between the mucosal and the through-and-through groups. The points given for each function, depending on the reconstruction material, revealed that there was no significant difference between pairs of material groups. From this prospective study, the authors have developed an algorithm for oromandibular reconstruction. When the bony defect is lateral, the ilium, fibula, or scapula should be chosen as the donor site, depending on the extent of the soft-tissue defect. When the bony defect is anterior, the fibula is always the best choice. When the soft-tissue defect is extensive or through-and-through with an anterior bony defect, the fibula should be used with other soft-tissue flaps.

One-stage transfer of the latissimus dorsi muscle for reanimation of a paralyzed face: a new alternative.

The two-stage method combining neurovascular free-muscle transfer with cross-face nerve grafting is now a widely accepted procedure for dynamic smile reconstruction in cases with long established unilateral facial paralysis. Although the results are promising, the two operations, about 1 year apart, exert an economic burden on the patients and require a lengthy period before obtaining results. Sequelae such as hypoesthesia, paresthesia, and conspicuous scar on the donor leg for harvesting a sural nerve graft also cannot be disregarded. To overcome such drawbacks of the two-stage method, we report a refined technique utilizing one-stage microvascular free transfer of the latissimus dorsi muscle. Its thoracodorsal nerve is crossed through the upper lip and sutured to the contralateral intact facial nerve branches. Reinnervation of the transferred muscle is established at a mean of 7 months postoperatively, which is faster than that of the two-stage method. In our present series with 24 patients, 21 patients (more than 87 percent) believed that their results were excellent or satisfactory, which also compares well with the results of the two-stage method combining free-muscle transfer with cross-face nerve graft.

Skeletal muscle reinnervation by reduced axonal numbers results in whole muscle force deficits.

Patients sustaining a peripheral nerve injury will frequently experience residual muscle weakness after muscle reinnervation, even if the nerve repair is performed under optimal circumstances to allow rapid muscle reinnervation. The mechanisms responsible for this contractile dysfunction remain unclear. It is hypothesized that after peripheral nerve injury and repair, a reduced number of axons are available for skeletal muscle reinnervation that results in whole muscle force and specific force deficits. A rat model of peroneal nerve injury and repair was designed so that the number of axons available for reinnervation could be systematically reduced. In adult rats, the peroneal nerve to the extensor digitorum longus muscle was either left intact (sham group, n = 8) or divided and repaired with either 50 percent (R50 group, n = 7) or 100 percent (R100 group, n = 8) of the axons in the proximal stump included in the repair. Four months after surgery, maximal tetanic isometric force was measured and specific force was calculated for each animal. Mean tetanic isometric force for extensor digitorum longus muscles from R50 rats (2765.7 +/- 767.6 mN) was significantly lower than sham (4082.8 +/- 196.5 mN) and R100 (3729.0 +/-370.2 mN) rats (p < 0.003). Mean specific force calculations revealed significant deficits in both the R100 (242.1 +/- 30 kN/m2) and R50 (190.6 +/- 51.8 kN/m2) rats compared with the sham animals (295.9 +/- 14 kN/m2) (p < 0.0005). These data support our hypothesis that after peripheral nerve injury and repair, reinnervation of skeletal muscle by a reduced number of axons results in a reduction in tetanic isometric force and specific force. The greater relative reduction in specific force compared with absolute force production after partial nerve repair may indicate that a population of residual denervated muscle fibers is responsible for this deficit.

Acute aortic valvular regurgitation secondary to avulsion of aortic valve commissure in a patient with pseudoxanthoma elasticum.

A 68-year-old woman developed acute pulmonary edema due to severe acute aortic valvular regurgitation. At the time of emergency surgery, it turned out to result from spontaneous avulsion of the aortic valve commissure. Later, the patient was diagnosed to have pseudoxanthoma elasticum based on typical skin lesions. Connective tissue abnormalities associated with pseudoxanthoma elasticum might have contributed to the development of the avulsion of the aortic valve in this particular patient.

Beat-to-beat variation of the ST segment in a patient with right bundle branch block, persistent ST segment elevation, and ventricular fibrillation: a case report.

The authors describe a patient with ventricular fibrillation associated with right bundle branch block-like QRS morphology and persisting ST segment elevation in the precordial leads, in whom the morphology of the ST segment changed from the coved type to the saddle-back type or vice versa on a beat-to-beat basis. Such an observation has never been reported in the previous literature.

[A clinical investigation of infective endocarditis at a community hospital in Japan].

There have been few reports on the clinical features of infective endocarditis (IE) in Japan. We clinically investigates 45 episodes (36 cases) of definite IE that were experienced from January 1985 to March 1997 at a community hospital, Okinawa Chubu Hospital, Okinawa, Japan. Regarding age, prior dental procedure, causative organisms and sites of infection, analyses and comparison were performed on a total of 94 episodes, by adding another 49 episodes of IE that were experienced between 1977 and 1984 at our hospital. The mean age was 47 years and majority of patients in the recent 12 years were older than 50 years of age. Mortality of all 94 episodes was 20%, while that of recent 45 cases was 13%. Eight % of all episodes had history of recent dental treatment but significance of the finding remains unclear. Alpha streptococci were most common (33%) and Staphylococcus aureus was the second most common organism (17%). Eleven % of all episodes were culture-negative and there was a statistically significant difference in the histories of prior antibiotic therapy between culture-negative and culture-positive episodes. Regarding sites of infection, 27% of all episodes involved mitral valves, while 24% involved aortic valves. Prosthetic valves were involved in 12%. Ninety-eight % of the recent episodes had fever, 98% had cardiac murmurs and 27% had characteristic mucocutaneous lesions. Heart failure was the most common complication (27%) and half of the cases with prosthetic valve infection had heart failure. Cerebral embolism was most frequently seen among the major arterial embolic complications. Our results were similar to those which were previously reported from other countries. We should have a high index of suspicion for endocarditis whenever we see patients who present various clinical manifestations and fever of which origin remains unclear. Willingness to obtain blood culture before starting antibiotics is most important.

A comparison of temporal muscle transfer and lid loading in the treatment of paralytic lagophthalmos.

Temporal muscle transfer (n = 78) and lid loading with a gold plate (n = 52) were compared in the treatment of paralytic lagophthalmos. Three sizes of gold plate (1.0, 1.2 and 1.4 g) were used. Lid loading was inferior to temporal muscle transfer in improving functional ability to close the eye, but it did protect the cornea from irritation. There were no significant differences among the sizes of plate, but there was most relief from corneal irritation when the 1.4 g gold plate was used. The plate had to be removed in 25% of the patients, but this did not make the condition worse than before lid loading. Lid loading has the advantage that patients can close their eyes without conscious effort. We therefore recommend lid loading as the first choice for correction of paralytic lagophthalmos, and that temporal muscle transfer should be undertaken after plate removal in only those patients for whom lid loading has not given a satisfactory result.

Aging effects in skeletal muscle recovery after reinnervation.

To investigate the influence of age on the process of muscle recovery after nerve repair, the nerves of the right extensor digitorum longus (EDL) and the right soleus muscles of 63 2-month-old and 61 15-month-old rats, respectively, were transsected and resutured. At four, eight, 16, and 24 weeks after nerve repair, functional recovery of the muscle was assessed by electromyographic (EMG) recordings and isometric muscle contraction. Muscle weight, morphological, and morphometric studies were also done. At four and eight weeks after nerve repair the younger age groups showed higher rates of recovery compared with the control side (left EDL and soleus) (recovery rate (%) = operated/control x 100) than the older age groups, and the recovery rates of soleus (slow twitch muscle) in both age groups were higher than EDL (fast twitch muscle). However, the differences between the two age groups decreased at 16 and 24 weeks after nerve repair in both muscles. We therefore conclude that earlier differences were the effects of nerve regeneration on the muscle between different ages and the reason for the reduced differences at later stages was that after reinnervation began, not only the nerve but also the muscle recovered successfully in older age groups, and slow motor units reinnervated faster than the fast units in both age groups. Our present study shows that the older age groups also have a good prognosis for recovery of muscle function after nerve repair.

Evaluation of muscle graft using facial nerve on the affected side as a motor source in the treatment of facial paralysis.

To acquire symmetry of the cheek when smiling, we carried out 39 free vascularised grafts of the muscle, the motor nerve of which was sutured to a stump of the ipsilateral facial nerve, for 39 patients with facial paralysis. We used two methods: an as healthy and fresh as possible facial nerve stump (method 1A, n = 17), or an incompletely affected stump (method 1B, n = 22). The results are classified into grade 1 to 5 indicating increasing efficiency of muscle function. All patients who had method 1A and 14 patients who had method 1B were evaluated grade 4 or better. Both an incompletely affected facial nerve stump and the proximal stump of a facial nerve that had previously been resected have sufficient function to provide contraction in the grafted muscle.