USA > Ohio > Hancock County > Findlay > Twentieth Century History of Findlay and Hancock County, Ohio, and Representative Citizens > Part 94
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88
JOHNS HOPKINS HOSPITAL BULLETIN.
[No.]
CASE I .- Miss X., 16 years of age, a school girl, consulted one of us (W. S. T.) on the 30th of May, 1906, with a complaint of jaun- dice. Her father and mother were well and strong. She has one sister, younger than herself, who is perfectly well, and the family history in other respects, is good.
Her mother states that as an infant the patient used to have slight attacks of jaundice for which they were accustomed to give her calomel, but as she grew older the attacks became more frequent until, finally, the jaundice became permanent. The yel- low color has varied in intensity from time to time, but the mother is not sure that it has ever been wholly absent. It has been deeper as a rule at the time of menstruation, and worse after exertion and bad colds, or with any indisposition. Six years ago the patient had a febrile attack which was regarded as typhoid fever. in other respects she has been a strong healthy girl, taking part in all the out-of-door games of her companions. She is not conscious of having been short of breath.
Eight months ago she began to have occasional attacks of pain in the right side of the epigastrium, lasting from ten to twenty minutes. Of these attacks she has had several. Three weeks ago she had a paroxysm of sharp, cramp-like pains in the same posi- tion, so sharp that at times while at school she had to lie down; there has been no fever with any of these attacks, but in the last three weeks she has had more or less pain, usually every other day. Lately the jaundice has been worse, but it is the pain that has excited the attention of her parents.
In other respects she had felt perfectly well and only recently, since she has grown older, she has been somewhat annoyed by the consciousness of her jaundice.
On examination there was a distinct jaundice although in other respects the color of the patient was that of a healthy girl; the tongue was clean; pulse 19 to the quarter, regular, not remarkable. Examination of heart and lungs negative.
Abdomen, natural; no tenderness on pressure in the region of tne gall bladder; palpation, negative, although, on light percus- sion, there is possibly a very slight suggestion of dulness in the region of the gall bladder. The spleen in readily palpable, de- scending on deep inspiration at least 4 cm. below the costal margin.
Widal reaction, negative.
Blood:
R. b. c.
3,680,000
W. b. c.
800,500
Hb.
70-71%
Dried and stained specimens showed the red blood corpuscles of about normal size with but slight variation in diameter; the color is good; no marked polychromatophilia. Their contour is fairly regular, but there is some distortion, a few elliptical and dumbell shapes being found. No nucleated reds.
Differential count:
Polymorphonuclear neutrophiles
Small mononuclears
72.5 %
Large mononuclears 18.0%
Transitionals 7.5 %
Eosinophiles 1.5 %
.5 %
The case was regarded as one of chronic, congenital jaundice with splenomegaly of the type described by Minkowsky. There was a question as to the existence of gall stones, but, in the ab- sence of severe symptoms, operation was not advised. Blaud's pills to be continued through long periods of time, were pre- scribed.
Three years later, having become familiar with the work of Chauffard, Widal and others, the writer requested the parents of the patient to allow him to see her again. She was accordingly seen on the 20th of March, 1909. Jaundice had been present for
the greater part of the preceding three years, although the path had grown and developed normally and was a perfectly heat. looking girl. The mother says that after long periods is to: house she becomes more jaundiced and that dancing, violette; ercise and bad colds make it worse. It is worse at the cataman periods, better just afterwards. The author's note on this oc sion reads:
"Strong, healthy looking girl; color good; tongue det There is a distinct slight jaundice of the skin and confuacts. Pulse 22 to the quarter, regular, not remarkable in qual- Heart sounds, clear at the apex and base.
Abdomen: natural in appearance. Spleen, readily felt just )- low the costal margin, descending on deep breath 3 or 4 cm, beles It seems smaller than when last seen. The liver descends (3 below the costal margin on deep breathing; flatness abore c about the 6th rib, extending to the costal margin. Gall bladiz not felt.
Blood:
R. b. c.
3,200,000
Hb. 75%
Resistance of the red blood cells: 1 cc. of blood was collected : 9 cc. of 1% aqueous sodium fluoride solution, and the corpest: were subsequently washed three times in 0.85% NaCl solution ! 5% suspension of the red corpuscles in 0.85% salt solution va prepared. The corpuscles were then placed in solutions of NaC. E. varying strength and the first trace of hæmolysis (minimal :- sistance) and complete hæmolysis (maximal resistance) moc! The results showed:
Minimal resistance 0.68% NaCl
Maximal resistance 0.45% =
After centrifugalizing the blood-sodium fluoride mixture, supernatant fluid was found to be of a canary yellow color: was pipetted off and tested for urobilin and bilirubin. Neither: pigment could be demonstrated, using the technique describedt; Conner and Roper.
Urine .- Fresh specimen March 20, 1909. Brownish Jelen clear, acid, sp. gr. 1.014; albumen and sugar absent; bile: Is white; tests with yellow nitric acid negative. Trousseau's 5 (tincture of iodine) negative. Urobilin: marked green fluorescente on treating the amylic alcohol extract with 1% ZnCl, in azEs niacal alcohol; also positive on spectroscopic examination.
On the 4th of April, after taking Blaud's pills gr. V, tid tr two weeks, the patient allowed us to make another examination & the blood:
3,800,000
R. b. c.
W. b. c. 5,200
Hb. .84% (Dare.)
Vital staining of fresh blood with Unna's polychrome methyky blue, according to the method of Vaughan, showed 149 red cEls with basophilic granulations out of 1228 cells examined, or abs 12.1% (normal = 0.5% to 1.8% .- Vaughan).
Differential count of 564 cells with Ehrlich's triacid stain:
Lymphocytes 18.6%
Large mononuclears and transitionals. 3.5%
Polymorphonuclears, neutrophilic 73.0%
Polymorphonuclears, eosinophilic 1.2%
Polymorphonuclears, basophilic 0.7%
Myelocyte, neutrophilic 0.17%
Degenerated 2.6%
Wilson's stain (Romanovsky) and the triacid stain were us. in studying the red cells. There was moderate anisocytosis.3 poikilocytosis. The red cells were of good color and no polyters matophil'a, basophilic granules, nuclear particles, Cabot's ral
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Ir few in number in the stained specimens. sistance of the red blood corpuscles:
Minimal resistance 0.72% NaCl. Maximal resistance 0.42% "
e blood plasma-sodium fluoride mixture was again of a bril- canary yellow color. Using the methods recommended by .er and Roper, bilirubin was demonstrated; tests for urobilin negative.
ine .- Fresh specimen, April 8, 1909. Yellowish brown, clear, sp. gr. 1.024; albumen, sugar and bile, absent. Urobilin: ted positive reaction, both chemical and spectroscopic. Micro- cal examination showed a few squamous epithelial cells; 'wise negative.
vol .- Dark brown, small, constipated stool. No blood, pus or IS. Microscopical examination: a few muscle fibers without tions, many yeast cells. No ova or parasites. Schmidt's test centrated corrosive sublimate) positive for urobilin, negative ilirubin.
od .- The average diameter of 500 red corpuscles in dried and ed specimens ( Wilson's modification of Romanovsky) was 4. The variations were between 5 and 9.4 4-essentially nor- measurements.
July of the same year the patient was seen in Europe. At time she seemed perfectly weli but showed, as always, a dis- slight jaundice of the skin and conjunctive.
e general appearance of the patient was very remarkable. out observation one would have picked her out as a perfectly hy girl; her cheeks were red and her expression bright, the 'al nourishment good and it was a matter of great surprise to 30 marked an anæmia. She had never been short of breath complained of no subjective symptoms of anæmia.
e second case was seen in the wards of Prof. Barker, to e courtesy we are indebted for the privilege of reporting ase.
IE II. (Med. No. 23623) .- F. B., single, age 27, a manicurist, .dmitted to the hospital on January 22, 1909, complaining of ice. The family and personal history are good. No other er of the family is jaundiced. The patient has had measles numps at the same age and whooping-cough at 12. During st twelve years there have been occasional attacks of major ia, usually following mental shock. There has been chronic pation. The patient has been jaundiced since she was three old; the jaundice is variable, sometimes very deep and scarcely noticable. Since the age of 16 she has complained at she called bilious spells about twice a month. While ig in the morning she becomes nauseated, vomiting one or nces of fluid, at first like water and then almost pure bile. minutes after vomiting she feels relieved and is able to go k.
e is no history of pain in the region of the gall bladder, or hatemesis. The patient has not passed acholic or bloody to her knowledge. She has had no abdominal pain. In tolecystostomy was performed by Dr. John D. Blake. No mes were found.
t two months ago the patient began to have a " dead, pain " a little below the right scapula; the pain seldom
There has been slight pain occasionally above the left Her average weight is 138 lbs .; in the last three months lost 13 lbs.
! presens. (Dr. Boggs) .- The patient is well nourished. ja marked icteric tint to the entire skin, the scleræ and the membranes. No xanthoma. The lungs are negative. Apex is just felt in the 4th interspace 9.5 cm. to the left
vl the median line. Relative cardiac dulness extends 10.5 cm. to the left in the 5th, 3.5 cm. to the right in the 3rd interspace, and above to the 3d rib. There is a soft systolic blow at the apex, transmitted to the axilla. Pulmonic second sound accentuated. There is a blowing systolic murmur over the conus.
Abdomen: Spleen extends 4 cm. downward and forward beyond the costal margin. Liver not palpable. No hæmorrhages noted.
Jan. 29. (Dr. Thayer). Perfectly healthy looking woman with yellowish type of jaundice. Tongue clean. Abdomen slightly more prominent on left. Spleen readily palpable. Liver not en- larged.
Feb. 5. (Dr. Hanes). Since admission the patient's only com- plaint, jaundice, has varied very markedly from a light yellow to a deep lemon color. The sclera are constantly yellow and do not vary as much as the skin. The heart is as noted before,-the systolic blow is very faint and is not transmitted to the axilla. Spleen still enlarged, edge firm and sharp. Patient discharged.
During the patient's stay in the hospital, her blood pressure (systolic) varied between 100 and 118 mm. Hg. The temperature was between 97.6° and 100.2º F. Pulse 60 to 83, respirations 18 to 20.
Blood examinations:
Jan. 23. R. b. c. 3,800,000
W. b. c. 8,900
Hb.
70%
In the fresh blood the red cells appear normal in color and shape. No nucleated reds or parasites.
Jan. 26. Wasserman reaction negative. Coagulation time, 5 min.
Jan. 27. Blood count practically the same as on the 23d.
Jan. 28. (Dr. Hanes). The patient's blood serum is of a golden yellow color, and the HNO, test for bile is positive.
Jan. 29. (Dr. Rous). "Resistance of the red blood cells to hypotonic salt solution. Into a series of tubes containing 1 cc. each of dilute salt solution 20 mm. of the patient's whole blood were dropped. The dilutions comprised a series from 0.85% to 0.10% NaCl. As control, the blood of S., who had about the same number of cells per cm., was taken. The corpuscles of S. showed complete hæmolysis at a dilution of 0.30% and those of the patient at 0.40%. (This is an immediate observation). After 24 hours the reading showed a trace of hæmolysis at 0.55% for S., and about the same at 0.75% for the patient."
Differential count of 400 cells showed:
Lymphocytes 47.0 %
Large mononuclears and transitionals ... 5.25%
Polymorphonuclear neutrophiles 46.0 %
Polymorphonuclear eosinophiles 0.5 %
Mast cells 0.25%
Unclassified 0.75%
Feb. 3. R. b. c. 3,700,000
W. b. c.
10,500
Hb.
76%
Viscosity 4.1 (in terms of distilled water).
Stool examinations were made on Jan. 27 and 29 and on Feb. 3. Schmidt's test showed only hydrobilirubin. Microscopically noth- ing of importance was found.
Urine was examined on six different days. Sp. gr. 1.018 to 1.030. There was no albumin or sugar. No bile or bilirubin was demon- strated. Urobilin was tested for apparently only on Feb. 4th, when the report says, "Schlesinger's urobilin test negative. Spectroscopic examination inconclusive." The color of the urine was described as brownish yellow and orange.
It is rather unfortunate that more careful tests for urobilin were not made; it must have been present.
All attempts to communicate with the patient since her dis- charge from the hospital have failed.
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90
JOHNS HOPKINS HOSPITAL BULLETIN.
[No. 1.
These cases are typical examples of this condition. Al- though both patients showed a well-marked jaundice, in neither was there the slightest disturbance in the shape of itching; in neither was there bradycardia. The complaint of the second patient bore apparently no relation to her jaundice. The extraordinary manner in which these individuals adapt themselves, as it were, to their anæmia, is strikingly illus- trated by both of our patients, especially by the first, whose appearance and history were not of a delicate but rather of a robust, strong girl.
A striking peculiarity in the observations made upon this case is the remarkably high point of concentration of the solutions of sodium chloride at which complete hæmolysis appears, .42% and .45%, a concentration equal to or above that at which, in the normal individual, hæmolysis begins.
Acquired Hemolytic Jaundice .- This remarkable syndrome, chronic acholuric pleiochromic jaundice without the usual symptoms of biliary intoxication, associated with anæmia, enlarged spleen, and siderosis of the viscera is not, however, always of congenital origin. Soon after Chauffard's first observations, Widal and Abrami, Chauffard and Troisier, Le Gendre and Brulé, von Stejskal, Oettinger, Parkes-Weber and others reported a series of interesting observations in which a similar train of symptoms came on without apparent cause in adult life. The onset of the jaundice in these instances fol- lowed various different accidents-an ischio-rectal abcess (Widal and Abrami), hæmorrhage following a miscarriage (Widal and Abrami), severe nervous shock (Chauffard and Troisier), acute gastro-enteritis (Le Gendre and Brulé). In other instances, such as the two cases of von Stejskal, that of Oettinger, and that of Parkes-Weber, the process seems to have appeared without striking initial symptoms. Again, in a considerable group of cases a jaundice of a transient char- acter presenting similar hæmatological phenomena-an anæmia with signs of regenerative activity of the bone mar- row, granular corpuscles on vital staining and a marked fragility of the red cells, has been observed in the course of a variety of conditions, cancer of the stomach (Chalier) ; cirrho- sis of the liver (Chalier and Le Play) ; malaria (Sacquépée, Chalier) ; repeated hæmorrhages in cancer of the bladder (Widal and Joltrain) ; streptococcus infection (Sacquépée) ; uncinariasis (Darré) ; syphilis (Gaucher and Giroux) ; pul- monary tuberculosis (Landouzy) ; jaundice of the new born (Sabrazès and Leuret; Cathala and Daunay).
This acquired haemolytic jaundice, as it has been called by Widal and Abrami, who described the first cases, does not constitute a definite disease picture to the same extent as does congenital splenomegalic jaundice, but rather an interesting syndrome. Acquired hæmolytic jaundice may be divided, as suggested by Brule, into apparently primary and secondary cases. The former arise either without apparent cause or during the course of some acute transient malady, after which they persist with seeming independence. Secondary hæmo- lytic jaundice is observed in a transient manner associated with acute infections or poisons, or as a terminal phenomenon in the course of some chronic disease.
One essential difference exists between most of the cassa acquired hæmolytic jaundice and the congenital mat; namely : the existence in the former of an anemia suficie grave to occupy a prominent position in the clinical picz: The anemia may, indeed, be intense and is associated with: usual subjective symptoms. There are often striking to tions in the intensity of the jaundice and the grade of anez. Sudden paroxysms of fever associated with painful swel: of the spleen and extremely rapid fall in the number of. corpuscles have been described.
A patient of Widal and Abrami's showed, on the 192 May, 2,600,000 red blood corpuscles; ten days later, while: apparently good condition, there appeared severe heads !- dyspnœa, marked exacerbation of the jaundice, and in a tr or two the blood count showed but 850,000 red elements I: one of von Stejskal's cases the blood count rose in eighty die from 640,000 to 4,000,000.
The blood picture is often very similar to that in pernici: . anæmia; marked oligocythæmia; a high color index; and tosis ; poikilocytosis ; often an increase in the average corpox .: lar diameter. There are almost always marked evidence :: marrow activity in the shape of nucleated red blood corpus's among which megaloblasts are often found. Contrary to wbr one sees in pernicious anæmia, the leukocytes are usta somewhat increased, and, occasionally, there is a well-matte. leucocytosis, which, on differential count, shows a considera: proportion of myelocytes and a large percentage of eos 1- philes. Granular corpuscles are present on vital staining. : in the congenital form of the disease; sometimes, howers these are not so numerous.
There are two striking points of difference in the hema - logical picture between acquired and congenital hæmol ;. jaundice. The corpuscular fragility, so evident in the forza: condition, is often very slight in the acquired disease when t" test is made with unwashed corpuscles.
Widal and Abrami showed early that in acquired hæemo." jaundice the corpuscular fragility may, indeed, be almost : appreciable if the test be made with the whole blood. Wi: deplasmatized corpuscles, however, the resistance is distinct. sometimes very markedly, diminished. These authors show ?. that this increased fragility of the deplasmatized red ar- puscles is common to both congenital and acquired variety- of the disease with the essential difference that while in es- genital hæmolytic jaundice the fragility is usually dem: strable with the whole blood, in the acquired condition *** resistance of the whole blood may be normal, while that of t"> deplasmatized corpuscles may show a very marked reducti: The deplasmatized red blood corpuscles of acquired hæmolrt: jaundice show also a distinct diminution in resistance towsr: other hæmolytic substances, such as anti-human sera of i. sorts and leech extract.
In normal blood the resistance of the deplasmatized (" puscles does not differ essentially from that of the whole blx:"
While it is difficult to avoid the conclusion that this i- creased corpuscular fragility has a definite association vi:" the hæmolysis which occurs in these cases, yet it should k
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Tryguy and the extent of the anemia. The ditions existing in congenital and acquired haemolytic ndice form an interesting paradox, as has been pointed out Brule; on the one hand a marked corpuscular fragility ciated with a moderate anæmia, on the other a moderate ;ility and an intense anæmia. “ It is impossible to draw a ct conclusion as to the fragility of the red blood corpuscles he organism from their fragility in vitro."
'his diminution in the resistance of the deplasmatized cor- les may be restored if they be brought into contact again 1 their plasma. Although there is a slight difference be- en the resistance of the corpuscles according to the manner which they are separated from their plasma (Iscovesco, gnat), yet these differences are so slight that they need not considered; in fact the quantity of serum necessary to ore the resistance of separated corpuscles is so large that, :tically, it is unnecessary to wash the corpuscles deplas- ized in an oxalate solution. The anti-haemolytic power of serum is not restricted to that of the patient himself. It resent in other and heterologous sera. Heating to 56° C. a considerable period of time does not remove the power. re would then appear to be no evidence of the existence of pecific anti-hæmolytic substance. Widal, Abrami and lé believe that the anti-hemolytic power is dependent on e special physical property or properties of the serum. he blood of some individuals with acquired hæmolytic jaun-
possesses one characteristic which, for the moment, ears to be rather special, and is not observed in the con- ital forms, namely, an auto-agglutinative power of the Im. The test is a relatively simple one; it is thus de- Ded by Brulé.
)ne lets fall into a watch glass 10 drops of the patient's serum then a drop of red blood corpuscles isolated by centrifugation: nixture is shaken up and then allowed to rest. Soon the cor- les are seen to gather at the bottom, sometimes appearing to little masses. But if there is no auto-agglutination, we need o shake the glass slightly to restore the mixture to its pre- : homogeneity and to disperse the corpuscles in the serum. e auto-agglutination be positive, one sees after a period vary- ccording to the intensity of the phenomenon, from one to y minutes, a collection of the corpuscles into little granules · perceptible by the naked eye, which give the emulsion an t comparable to brick dust; violent and prolonged shaking t dissociate these corpuscles. Little by little the agglutina- ncreases, the corpuscles gather at the bottom of the watch where they form an homogeneous pellicle, the supernatant
becomes limpid, and shaking no longer dissociates the tic pellicle. If at the beginning of the phenomenon one nes a drop of the serum microscopically it may be seen that Irpuscles, instead of disposing themselves in rouleaux as Hly, gather in little islands, between which there float a few d corpuscles."
B phenomenon has been found in a number of instances hired hemolytic jaundice. In others, such as the cases dal and Joltrain, and that of Parkes-Weber, auto- mation has been absent. The phenomenon is interest- d may be of considerable diagnostic value. It is not in congenital hæmolytic jaundice, although the patient
authors to have been acquired from a wet nurse, might be regarded as belonging to this class. It has, however, been observed in one instance of hepatogenous jaundice by Brulé. It should be said that it has not as yet been studied sufficiently to justify far-reaching conclusions as to its diagnostic value or significance.
Attacks of pain in the region of the gall bladder associated with fever and exacerbation of the jaundice are very common: Indeed, the first three reported cases (Widal and Abrami; Chauffard and Troisier) were all operated upon for suspected gall stones. In one of these cases a little bile sand was found ; in the other instances the gall bladder was quite clear.
Acquired hemolytic jaundice presents itself in a variety of forms. The most important are :
1. Those cases simulating cholelithiasis.
2. Those simulating pernicious anæmia with jaundice.
3. Those simulating a chronic infectious cholangitis, chron- ic, infectious, splenomegalic jaundice (Hayem).
4. It has also been observed in some of those conditions in which the most striking symptoms, anæmia and splenomegaly, leave one in doubt as to whether the case should be classed clinically as Banti's disease or a cirrhosis of Hanot's type. Recent studies by Chauffard and Troisier, as well as by Ar- mand-Delille and Feuille, suggest strongly the possible rela- tionship between some so-called splenic anæmias and hæmolytic jaundice. Attention has also been called to this point by Parkes-Weber.
In other instances the condition may manifest itself under the symptoms of an icterus gravis (Roque and Chalier).
The syndrome is, however, sufficiently definite and easily to be recognized if carefully studied, by the absence of bile in the urine, its presence in the blood serum, the urobilinuria, the presence of a large percentage of granular corpuscles on vital staining, by the fragility of the red corpuscles, especially mani- fest with the deplasmatized elements, and by the commonly present auto-agglutinative power of the serum.
It is important to remember that in Addisonian anæmia and in cholelithiasis the corpuscular resistance is at least normal; indeed, as a rule, it appears to be increased. In one of my cases of pernicious anæmia hæmolysis began at only .325%, and was complete at .275%, a greatly increased resistance.
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