Dermatoglyphic Alterations
Associated With Acute Rheumatic Fever in Children Shyamal
K.
Sanyal, FAAP, FACC; Dilp
P.
\s=b\ The dermatoglyphic configurations of 78 children with acute rheumatic fever were compared with those of 46 first\x=req-\ degree relatives and 1,310 normal subjects. Of the children with acute rheumatic fever, 75% had an ulnar deviation of the axial triradius. In about 40% of this group, the ulnar deviation was associated with a concomitant distal displacement, which resulted in a significantly higher mean maximal angle atd (P < .001) and significantly lower mean ab and td ridge counts (P < .001) relative to normal control values. The palmar dermatoglyphics of patients with acute rheumatic fever were more closely related to the configurations of first-degree relatives than to normal controls. The dermatoglyphic profiles of six patients were nearly identical to those of their first-degree relatives, all of whom had a history of acute rheumatic fever. Presence of abnormal dermatoglyphic profiles in a large proportion of children with acute rheumatic fever supports the hypothesis that certain individuals have a genetic predisposition to this disease. (Am J Dis Child 132:692-695, 1978)
Dermatoglstudy yphics,
the term given to the of configurations formed by the dermal ridges on the palms, soles, and digits of all primates, has become an important diagnostic From the
Cardiopulmonary
Disease Service
(Dr Sanyal), St Jude Children's Research Hospital, Memphis, the Department of Pediatrics (Dr Ahmed), Safdar Jung Hospital, and the Population Genetics and Human Development Division (Dr Mukerjee), Central Family Planning Institution, New Delhi. Read in part before the annual meeting of the American Academy of Pediatrics, Oct 21, 1973. Reprint requests to Cardiopulmonary Disease Service, St Jude Children's Research Hospital, PO Box 318, Memphis, TN 38101 (Dr Sanyal).
Mukerjee, PhD; Syad H. Ahmed, MD, DCH tool in clinical medicine.' Abnormal
dermatoglyphic patterns
are
most
in diseases characterized
by aberrations,24 but have also been reported in patients with congenital malformations pro¬ duced by environmental teratogens, such as rubella' and thalidomide,' in patients with congenital heart dis¬ common
gross chromosomal
ease,6 and even in some individuals without apparent chromosomal errors
evidence of environmental terato¬ genic effects. Hale et al,7 for example, observed distal displacement of axial triradius in 25% to 29% of the adults with acquired heart disease. Other abnormal cases include patients with broad thumb and great-toe syn¬
established between of this disease and the presence of abnormalities in a prena¬ tal hereditary marker, such as an epidermal ridge pattern for which a polygenic mode of inheritance is well documented.1213 Accordingly, we stud¬ ied dermatoglyphic profiles in chil¬ dren with acute rheumatic fever for possible deviations from normal pat¬
tionships the
terns.
or
drome,1 Smith-Lemli-Opitz syn¬ drome,' and acute lymphocytic leu¬
kemia." More recently, dermatoglyphic ab¬ normalities have been reported for patients with mitral valve prolapse syndrome who did not have demon¬ strable chromosomal abnormalities." Since this syndrome frequently occurs in members of the same family, a common genetic factor influencing both the development of the mitral valve and the formation of epidermal ridge pattern has been postulated to account for dermatoglyphic abnormal¬ ities in these patients." Likewise, acute rheumatic fever has a high familial incidence, first reported by Cheadle"' and since confirmed by others," which has led to speculation that some individuals have a genetic predisposition to the disease. Such a hypothesis could be supported if rela-
Downloaded From: http://archpedi.jamanetwork.com/ by a University of Michigan User on 06/16/2015
were
occurrence
MATERIALS AND METHODS Patient Population These observations
drawn from a of 118 chil¬ dren with acute rheumatic fever.14 The were
prospective study comprised
patients were seen during a four-year peri¬ od, (July 1967 to June 1971), at the pediatrie outpatient clinic of the Safdar Jung Hospi¬ tal, New Delhi. Each child fulfilled modi¬ fied Jones's diagnostic criteria for acute rheumatic fever. The dermatoglyphic fea¬ tures of fingers and palms were recorded for 78 of these patients, 42 boys and 36 girls, with ages ranging from 5 to 12 years. The patient population represented four major ethnic groups in north India, Punja¬ bis, Bengalis, Muslims, and those from eastern Uttar Pradesh, in approximately equal numbers. A detailed developmental history and physical examination were done for each patient. Any child with mental retardation, a major congenital malformation, or a family history of consanguinity was excluded from the study.
Dermatoglyphic Finger
Evaluation
palm prints were made with ink-imprinting method; the fingers were carefully spread before a print was made and only those prints judged to be of optimum quality were used a
and
standard
for the study. Each hand was analyzed separately and the results interpreted according to the classification scheme of Penrose."'" The dermatoglyphic features examined included finger patterns, distri¬ bution of ridge pattern, number of triradii on fingers and palms, total absolute ridge count on fingers, ab and td ridge counts, position of hypothenar pattern, ending of main line DII, and position of digital tri¬ radii (a and b) and axial triradii (triradius t). The maximal angle atd was determined by joining triradii a and d with triradius t (Fig 1) and was expressed as an average sum of both hands. The position of the axial triradius, whether in the middle of the palm (tM) or toward the ulnar (t, ) or radial (tR) side, was determined on the basis of the tda angle." The axial triradius was described as tM if the tda angle was between 76° and 85°, as t, if the angle was greater than 86°, and as t„ if the angle was less than 75°. The degree of displacement
of the axial triradius1' '" was defined as proximal when the maximal angle atd (an average sum of both hands) was 45° or less (t„) or was between 45 and 56° (f). Distal displacement was indicated when the maximal angle atd was between 56 and 80° (t") or was greater than 80° (t'").
Fig 1.—Palmar dermatoglyphics
DERMATOGLYPHIC RESULTS
Digital Dermatoglyphics The incidences of whorls, loops, and arches in children with acute rheumat-
vs
patient with
acute rheumatic fever.
Table 1.—Position of Axial Triradius in Patients With Acute Rheumatic Fever vs First-Degree Relatives
Comparison Groups The dermatoglyphic data for children with acute rheumatic fever were compared with data for 1,310 normal Indians and 46 first-degree relatives (fathers, mothers, brothers, and sisters), six of whom had a history of acute rheumatic fever. Socioec¬ onomic background and the frequency distribution of ethnic groups among normal controls and first-degree relatives were similar to those of the patient popu¬ lation. Thus, possible variations in der¬ matoglyphic profile as a result of differ¬ ences in ethnic" or socioeconomic back¬ grounds were uniformly minimized. There was no history of consanguinity in the normal controls. Because dermatoglyphic traits are possi¬ bly related to sex,1516 boys with acute rheu¬ matic fever were always compared with brothers, fathers, and with males in the normal population, whereas girls with the disease were compared with sisters, moth¬ ers, and females of the normal popula¬ tion. Statistical Analysis.—Differences be¬ tween patients, normal controls, and firstdegree relatives were tested for statistical significance by Student's t test.
in normal child
Left, Pattern in normal hand shows position of digital triradii a and d and axial triradius t. Angle formed by a, t, and d represents maximal angle atd, expressed as an average sum of both hands. Note that in normal controls, axial triradius is in midline position (tM or t\,) that is proximal to palmar crease (dashed line). Right, In most patients with acute rheumatic fever, position of axial triradius t is abnormal and shows ulnar deviation (t,) with or without distal displacement (t", or t'", ) resulting in abnormal atd angle.
With Ulnar Shift
Position Proximal t
Without Ulnar Shift
Patients
Relatives
Patients
Relatives
18
10
14
12
20
13
10 Distal
t"
27
14
Total
58
33
ic fever were 38%, 58%, and 4%, respec¬
tively; total and absolute finger ridge counts were 139 ± 10.3 (mean ± SD)
and 187.9 ± 19; and the total number of triradii on fingers was 13.5 ± 0.79. These values were not substantially different from those for first-degree relatives or the normal population, nor were there any substantial differ¬ ences between values for males and females in any of the three groups. Position of the Axial Triradius
An ulnar deviation of the axial triradius (t, ) was recorded for 58 of 78 children with acute rheumatic fever (75%), and in 30 of these patients, the ulnar deviation was associated with distal displacement (t'\ in 27; t'", in three) (Table 1). None of the patients
Downloaded From: http://archpedi.jamanetwork.com/ by a University of Michigan User on 06/16/2015
had
a tR location. In five patients, although the axial triradii were in midline position, there was substan¬ tial distal displacement (t'\, in four; t"'M in one). In the normal population, by contrast, the position of the axial triradius, without exception, was in the middle of the palm and was proxi¬ mal, that is, of t„ or t',, variety (Fig 1).
Ulnar deviation with a concomitant distal displacement of the axial trira¬ dius was not detected in any of the normal controls but was seen in first-
degree relatives, although not as frequently as in the patients (Fig 2). Maximal Angle atd In patients with both ulnar devia¬ tion and distal displacement of axial triradius, the mean maximal angle
Dermatoglyphics in Patients With Acute Rheumatic Fever, First-degree Relatives, and Normal Controls*
Table 2.—Palmar
Normal
Normal Patients Maximal M
angle atdt
101.4
±
Patients
Population
9.2
87.6
±
1.57
101.4
+
Relatives
9.2
95.00
±
Relatives
Population
10.01
87.6 ±1.57
95.00
+
10.01
_(P < .01)_ 102.7
±
6.19
89.2
+
1.57
102.7
±
6.19
96.70 ± 4.64
89.2
+
1.57
96.70
±
4.64
_(P< .01)_ M and F
88.4
102.1+5.51 _
±
1.18
102.1
±
96.20
5.51
±
5.09
88.4 ±1.18
96.20
±
5.09
_(P < .001)_(P < .05)_ Maximal angle M
atdt 80.43 82.03
± ±
2.78 2.38
81.33
±
3.17
87.6 ±1.57 89.20 ± 1.57
(P M and F
Associated With Acute Rheumatic Fever in Children Shyamal
K.
Sanyal, FAAP, FACC; Dilp
P.
\s=b\ The dermatoglyphic configurations of 78 children with acute rheumatic fever were compared with those of 46 first\x=req-\ degree relatives and 1,310 normal subjects. Of the children with acute rheumatic fever, 75% had an ulnar deviation of the axial triradius. In about 40% of this group, the ulnar deviation was associated with a concomitant distal displacement, which resulted in a significantly higher mean maximal angle atd (P < .001) and significantly lower mean ab and td ridge counts (P < .001) relative to normal control values. The palmar dermatoglyphics of patients with acute rheumatic fever were more closely related to the configurations of first-degree relatives than to normal controls. The dermatoglyphic profiles of six patients were nearly identical to those of their first-degree relatives, all of whom had a history of acute rheumatic fever. Presence of abnormal dermatoglyphic profiles in a large proportion of children with acute rheumatic fever supports the hypothesis that certain individuals have a genetic predisposition to this disease. (Am J Dis Child 132:692-695, 1978)
Dermatoglstudy yphics,
the term given to the of configurations formed by the dermal ridges on the palms, soles, and digits of all primates, has become an important diagnostic From the
Cardiopulmonary
Disease Service
(Dr Sanyal), St Jude Children's Research Hospital, Memphis, the Department of Pediatrics (Dr Ahmed), Safdar Jung Hospital, and the Population Genetics and Human Development Division (Dr Mukerjee), Central Family Planning Institution, New Delhi. Read in part before the annual meeting of the American Academy of Pediatrics, Oct 21, 1973. Reprint requests to Cardiopulmonary Disease Service, St Jude Children's Research Hospital, PO Box 318, Memphis, TN 38101 (Dr Sanyal).
Mukerjee, PhD; Syad H. Ahmed, MD, DCH tool in clinical medicine.' Abnormal
dermatoglyphic patterns
are
most
in diseases characterized
by aberrations,24 but have also been reported in patients with congenital malformations pro¬ duced by environmental teratogens, such as rubella' and thalidomide,' in patients with congenital heart dis¬ common
gross chromosomal
ease,6 and even in some individuals without apparent chromosomal errors
evidence of environmental terato¬ genic effects. Hale et al,7 for example, observed distal displacement of axial triradius in 25% to 29% of the adults with acquired heart disease. Other abnormal cases include patients with broad thumb and great-toe syn¬
established between of this disease and the presence of abnormalities in a prena¬ tal hereditary marker, such as an epidermal ridge pattern for which a polygenic mode of inheritance is well documented.1213 Accordingly, we stud¬ ied dermatoglyphic profiles in chil¬ dren with acute rheumatic fever for possible deviations from normal pat¬
tionships the
terns.
or
drome,1 Smith-Lemli-Opitz syn¬ drome,' and acute lymphocytic leu¬
kemia." More recently, dermatoglyphic ab¬ normalities have been reported for patients with mitral valve prolapse syndrome who did not have demon¬ strable chromosomal abnormalities." Since this syndrome frequently occurs in members of the same family, a common genetic factor influencing both the development of the mitral valve and the formation of epidermal ridge pattern has been postulated to account for dermatoglyphic abnormal¬ ities in these patients." Likewise, acute rheumatic fever has a high familial incidence, first reported by Cheadle"' and since confirmed by others," which has led to speculation that some individuals have a genetic predisposition to the disease. Such a hypothesis could be supported if rela-
Downloaded From: http://archpedi.jamanetwork.com/ by a University of Michigan User on 06/16/2015
were
occurrence
MATERIALS AND METHODS Patient Population These observations
drawn from a of 118 chil¬ dren with acute rheumatic fever.14 The were
prospective study comprised
patients were seen during a four-year peri¬ od, (July 1967 to June 1971), at the pediatrie outpatient clinic of the Safdar Jung Hospi¬ tal, New Delhi. Each child fulfilled modi¬ fied Jones's diagnostic criteria for acute rheumatic fever. The dermatoglyphic fea¬ tures of fingers and palms were recorded for 78 of these patients, 42 boys and 36 girls, with ages ranging from 5 to 12 years. The patient population represented four major ethnic groups in north India, Punja¬ bis, Bengalis, Muslims, and those from eastern Uttar Pradesh, in approximately equal numbers. A detailed developmental history and physical examination were done for each patient. Any child with mental retardation, a major congenital malformation, or a family history of consanguinity was excluded from the study.
Dermatoglyphic Finger
Evaluation
palm prints were made with ink-imprinting method; the fingers were carefully spread before a print was made and only those prints judged to be of optimum quality were used a
and
standard
for the study. Each hand was analyzed separately and the results interpreted according to the classification scheme of Penrose."'" The dermatoglyphic features examined included finger patterns, distri¬ bution of ridge pattern, number of triradii on fingers and palms, total absolute ridge count on fingers, ab and td ridge counts, position of hypothenar pattern, ending of main line DII, and position of digital tri¬ radii (a and b) and axial triradii (triradius t). The maximal angle atd was determined by joining triradii a and d with triradius t (Fig 1) and was expressed as an average sum of both hands. The position of the axial triradius, whether in the middle of the palm (tM) or toward the ulnar (t, ) or radial (tR) side, was determined on the basis of the tda angle." The axial triradius was described as tM if the tda angle was between 76° and 85°, as t, if the angle was greater than 86°, and as t„ if the angle was less than 75°. The degree of displacement
of the axial triradius1' '" was defined as proximal when the maximal angle atd (an average sum of both hands) was 45° or less (t„) or was between 45 and 56° (f). Distal displacement was indicated when the maximal angle atd was between 56 and 80° (t") or was greater than 80° (t'").
Fig 1.—Palmar dermatoglyphics
DERMATOGLYPHIC RESULTS
Digital Dermatoglyphics The incidences of whorls, loops, and arches in children with acute rheumat-
vs
patient with
acute rheumatic fever.
Table 1.—Position of Axial Triradius in Patients With Acute Rheumatic Fever vs First-Degree Relatives
Comparison Groups The dermatoglyphic data for children with acute rheumatic fever were compared with data for 1,310 normal Indians and 46 first-degree relatives (fathers, mothers, brothers, and sisters), six of whom had a history of acute rheumatic fever. Socioec¬ onomic background and the frequency distribution of ethnic groups among normal controls and first-degree relatives were similar to those of the patient popu¬ lation. Thus, possible variations in der¬ matoglyphic profile as a result of differ¬ ences in ethnic" or socioeconomic back¬ grounds were uniformly minimized. There was no history of consanguinity in the normal controls. Because dermatoglyphic traits are possi¬ bly related to sex,1516 boys with acute rheu¬ matic fever were always compared with brothers, fathers, and with males in the normal population, whereas girls with the disease were compared with sisters, moth¬ ers, and females of the normal popula¬ tion. Statistical Analysis.—Differences be¬ tween patients, normal controls, and firstdegree relatives were tested for statistical significance by Student's t test.
in normal child
Left, Pattern in normal hand shows position of digital triradii a and d and axial triradius t. Angle formed by a, t, and d represents maximal angle atd, expressed as an average sum of both hands. Note that in normal controls, axial triradius is in midline position (tM or t\,) that is proximal to palmar crease (dashed line). Right, In most patients with acute rheumatic fever, position of axial triradius t is abnormal and shows ulnar deviation (t,) with or without distal displacement (t", or t'", ) resulting in abnormal atd angle.
With Ulnar Shift
Position Proximal t
Without Ulnar Shift
Patients
Relatives
Patients
Relatives
18
10
14
12
20
13
10 Distal
t"
27
14
Total
58
33
ic fever were 38%, 58%, and 4%, respec¬
tively; total and absolute finger ridge counts were 139 ± 10.3 (mean ± SD)
and 187.9 ± 19; and the total number of triradii on fingers was 13.5 ± 0.79. These values were not substantially different from those for first-degree relatives or the normal population, nor were there any substantial differ¬ ences between values for males and females in any of the three groups. Position of the Axial Triradius
An ulnar deviation of the axial triradius (t, ) was recorded for 58 of 78 children with acute rheumatic fever (75%), and in 30 of these patients, the ulnar deviation was associated with distal displacement (t'\ in 27; t'", in three) (Table 1). None of the patients
Downloaded From: http://archpedi.jamanetwork.com/ by a University of Michigan User on 06/16/2015
had
a tR location. In five patients, although the axial triradii were in midline position, there was substan¬ tial distal displacement (t'\, in four; t"'M in one). In the normal population, by contrast, the position of the axial triradius, without exception, was in the middle of the palm and was proxi¬ mal, that is, of t„ or t',, variety (Fig 1).
Ulnar deviation with a concomitant distal displacement of the axial trira¬ dius was not detected in any of the normal controls but was seen in first-
degree relatives, although not as frequently as in the patients (Fig 2). Maximal Angle atd In patients with both ulnar devia¬ tion and distal displacement of axial triradius, the mean maximal angle
Dermatoglyphics in Patients With Acute Rheumatic Fever, First-degree Relatives, and Normal Controls*
Table 2.—Palmar
Normal
Normal Patients Maximal M
angle atdt
101.4
±
Patients
Population
9.2
87.6
±
1.57
101.4
+
Relatives
9.2
95.00
±
Relatives
Population
10.01
87.6 ±1.57
95.00
+
10.01
_(P < .01)_ 102.7
±
6.19
89.2
+
1.57
102.7
±
6.19
96.70 ± 4.64
89.2
+
1.57
96.70
±
4.64
_(P< .01)_ M and F
88.4
102.1+5.51 _
±
1.18
102.1
±
96.20
5.51
±
5.09
88.4 ±1.18
96.20
±
5.09
_(P < .001)_(P < .05)_ Maximal angle M
atdt 80.43 82.03
± ±
2.78 2.38
81.33
±
3.17
87.6 ±1.57 89.20 ± 1.57
(P M and F
