Heart in Friedreich's ataxia.

Most articles on Friedreich's ataxia report that cardiac complications are almost universal in patients with this disease. However, the present study of 30 cases, observed for two to ten years, seems to belie the experience of other writers in the field. Only six (20 per cent) of these patients showed electrocardiographic abnormalities. Only one patient had cardiac enlargement and congestive heart failure. One other patient had paroxysmal tachycardia. Seventy-four family members were also studied and the results were similar. It is suggested that this impressive difference may be a racial one and indeed very few reports on Friedreich's ataxia have come from India. Further study of this group of patients is in progress. The author also includes a review of the literature of neuromyopathic diseases and their relationship to myocardial involvement.

Cardiovascular complications of aluminum phosphide poisoning.

Aluminum phosphide is widely used as a grain preservative. Recently some reports have appeared in the literature in which serious toxic effects of this agent have been reported, and a number of fatal complications, including shock and cardiac dysrhythmias, have been increasingly seen. Of 25 cases of aluminum phosphide poisoning the authors observed over a period of two years, 16 cases (64%) had evidence of cardiac dysfunction. Despite adequate treatment, 40% of the patients died. A plea is being made for recognition of these fatal complications and for early institution of adequate treatment.

Evaluation of enzymes in serum and cerebrospinal fluid in cases of cerebrovascular accidents.

Gamma glutamyl transpeptidase (GGTP), glutamic oxaloacetic transaminase (GOT), and glutamic pyruvic transaminase (GPT) were measured serially in the serum and cerebrospinal fluid (CSF) of 22 patients with fresh stroke and an equal number of age- and sex-matched healthy control subjects. It was observed that levels of these enzymes in the CSF of control subjects were very low but were significantly elevated (p less than 0.001) in both serum and CSF in patients with stroke. The elevation was greater in the CSF than in the serum and was maximum during the first four days of stroke. Thereafter, the enzymatic activity declined. Of all these enzymes, GGTP in CSF correlated best with the clinical picture. It was possible to differentiate between the ischemic and hemorrhagic type of stroke on the basis of CSF levels of GGTP (greater than 60.0 units in hemorrhagic stroke). There was no correlation between GGTP levels in CSF and serum or among GOT, GPT, and GGTP in CSF. It can be concluded, therefore, that estimation of GGTP in CSF is helpful not only in predicting the degree of cerebral damage and functional outcome of the patient following stroke but also in differentiating the type of stroke.

Renal dysfunction in enteric fever.

Twenty-five culture positive cases of enteric fever were studied to see the effect of the disease on various kidney functions. The parameters studied were urine examination, blood urea, serum creatinine, serum electrolytes, 24 hours urinary examination (proteins, creatinine, electrolytes), creatinine clearance rate, plain X-ray abdomen, intravenous urogram and circulating immune complexes. Fifteen patients with fever of non-specific origin served as controls. Renal dysfunction was observed in four patients (16%). Repeat tests showed that abnormalities of renal functions, though significant, were short lasting and reversible. Renal biopsy done in three of these cases did not reveal any abnormality.

Lipid profile in enteric fever.

Lipid profile is known to alter in patients with severe sepsis, but few studies regarding the status of lipid levels in enteric fever are available. Twenty patients with enteric fever, belonging to different age groups and both sexes, along with an equal number of matched patients with fever due to non-enteric causes, were studied with regard to alterations in lipid profile. We observed a severe and protracted hypertriglyceridaemia, decrease in HDL-cholesterol levels and increase in LDL-cholesterol levels in patients with enteric fever at the peak of fever. The values returned to normal on recovery and convalescence. This study serves to highlight the complexity of lipid variation during Salmonella typhi infection.

Plasma cortisol levels in enteric fever.

Plasma cortisol levels were estimated in twenty two culture positive cases of enteric fever and in equal number of patients with fever due to non-enteric causes. Patients of typhoid with severe toxaemia and or some complication were found to have lowered mean levels on the day of admission and at the time of defervescence. The levels returned to normal one week later. Patients of typhoid with mild to moderate illness showed elevated levels of plasma cortisol. To conclude cases of typhoid with severe toxaemia and/or complications have lower cortisol levels and may be given supplementary steroids (if no contraindications exist). Since these values return to normal one week after fever subsides, supplementary steroids need not be given for longer than one week of acute illness.

Autonomic function tests and clinical significance of dysautonomia in chronic liver disease.

Autonomic function in 20 patients with chronic liver diseases was assessed clinically and by various tests like Valsalva ratio, expiratory-inspiratory ratio, postural tachycardia index, atropine ratio, blood pressure response to assumption of erect posture, hand immersion in cold water, and finger wrinkling response to hand immersion in warm water. Eighty per cent of the patients showed some clinical evidence of dysautonomia. While a good correlation between various clinical complaints of dysautonomia and the extent of parasympathetic dysfunction was observed in the individual patient, a significantly greater impairment of sympathetic function tests was observed in oliguric cirrhotics as compared to their non-oliguric counterparts.

Gamma glutamyl transpeptidase in meningitis.

Gamma glutamyl transpeptidase (GGTP) was measured serially in cerebrospinal fluid (CSF) and serum in 23 cases of meningitis (15 pyogenic and 8 tuberculous meningitis) and an equal number of age and sex matched healthy controls, to find out its diagnostic and prognostic significance in meningitis. GGTP activity was significantly elevated (p less than 0.001) in CSF and serum in meningitis as compared to control subjects. Levels were significantly higher in pyogenic as compared to tuberculous meningitis (p less than 0.001) and in CSF than in serum (p less than 0.001). The maximum elevation was seen on the 1st day and thereafter the activity declined in the majority (65.2%) of cases. However, in 3 cases of pyogenic meningitis and 5 cases of tuberculous meningitis, the GGTP activity on subsequent estimation increased serially; all these 8 cases died. It is concluded that CSF GGTP activity is significantly elevated in meningitis and serial rise in its activity is associated with poor prognosis and even fatal outcome.

Autonomic function tests in cases of chronic severe anaemia.

Autonomic functions were studied in 30 adult cases of chronic severe anaemia (CSA) and equal number of age and sex matched healthy controls. Blood pressure and heart rate responses to standing, to respiration (expiratory-inspiratory ratio), to Valsalva manoeuvre (Valsalva ratio) and to hand immersion in ice cold water and given 1.8 mg of atropine intravenously were studied. Patients with CSA had significantly high basal pulse rate and low blood pressure as compared to control subjects (p less than 0.001). The expiratory inspiratory ratio was abnormal in 30% of the cases of CSA (p greater than 0.10) valsalva ratio was abnormal in 50% of cases (p less than 0.01) and postural tachycardia was observed in 60% of cases (p less than 0.001). Normal response to hand immersion in ice cold water was observed in 56.6% of cases (p less than 0.001). Atropine resulted in tachycardia in 73.4% of cases of CSA as compared to 86.7% of controls (p less than 0.10). All the cases of CSA showed one or more abnormal response and in 16.6% of cases all responses were abnormal.

Aluminium phosphide poisoning.

Aluminium phosphide is a solid fumigant pesticide widely used in the Indian subcontinent as a grain preservative. Over the last 5 years there has been a dramatic increase in the number of cases of aluminium phosphide poisoning in India. Ninety-two patients with aluminium phosphide poisoning due to ingestion were studied over a period of 3 years. Abdominal pain, vomiting and restlessness were the common initial features followed by alteration in sensorium and shock unresponsive to conventional treatment. Electrocardiographic abnormalities were very common and highly variable. Routine serum biochemistry was usually unremarkable. Severe metabolic acidosis was common and mortality high (49%). The survivors recover completely without any residual organ damage. There is no known antidote.

Haematological profile in typhoid fever.

The haematological profile in 20 culture proven patients with typhoid fever of varying age and of both sexes was studied. Significant changes observed were anaemia, leucopenia, eosinopenia, thrombocytopenia and sub-clinical disseminated intravascular coagulation. The bone marrow of typhoid patients showed myeloid maturation arrest, decrease in the number of erythroblasts and megakaryocytes with increased phagocytic activity of histiocytes.