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Influence of Selected Demographic Variables:
A Case Study on Spondyloepimetaphyseal
Dysplasia Handigodu Type in Karnataka
Pulamaghatta N. Venugopal1, I. Arjun Rao2, Devajana C. Nanjunda3,
Adimoolam Chandrasekar4*
1Anthropological Survey of India, North West Regional Centre, Dehradun, India
2Department of Forensic Sciences, GGV, Bilaspur, India
3CSSEIP, University of Mysore, Mysore, India
4Anthropological Survey of India, Southern Regional Centre, Mysore, India
Abstract: Handigodu (HG) disease is a rare and painful osteoarthritis disorder and
remains a major medical problem in Shimoga and Chikkamaglur districts of Karnataka
state. The first case of the disease was reported from Handigodu Village of Sagar
Taluk of Shimoga District. The affected individuals are categorized into 3 phenotypes:
type I (predominantly hip-joint pains), type II (predominantly dysplastic), and type
III (dwarf). Dysplasia of hip joints and spine is often associated with osteoarthritis.
The present study is conducted in 25 villages of the Sagar and adjoining taluks of the
Shimoga district. Socio-demographic information like ethnicity, age, sex, marriage
type, food habits, etc. was collected using structured questionnaire. The analysis is
confined to the present investigation, where the researcher personally collected data
on 300 selected samples (166 HGS affected+ 134 unaffected) in 2 taluks (Sagar and
Soraba) from 25 villages. The result shows the association between various
demographic parameters along with clinical variables.
Keywords: Handigodu, demography, spondyloepimetaphyseal dysplasia.
INTRODUCTION
Handigodu Disease (HGD) is a rare and painful osteoarthritic disorder endemic to
the Malnad region (Shimoga and Chikkamaglur districts) of Karnataka state, India
(Ramamurthy 2005). The first case of the disease was reported from Handigodu
village of Sagar taluk of Shivamogga district in January 1975, hence the name of
the disease. Despite several studies the condition continues to remain a major
medical problem of these socially deprived people. An earlier study identified this
disease as a type of spondyloepimetaphyseal dysplasia (SEMD) which follows a
predominantly autosomal dominant pattern of inheritance (Agarwal et al., 1994).
Further, recently the Handigodu Disease is scientifically named as
Spondyloepimetaphyseal Dysplasia: Handigodu type (SEMDHG) (Venugopal et al.
2011a).
*Address for correspondence: Dr. Adimoolam Chandrasekar, Southern Regional Centre, Manav Bhawan,
Mysor, E-mail: adimoolamchandrasekar@yahoo.com
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
28 Influence of Selected Demographic Variables
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Clinical Symptoms in Identification of Handigodu disease
• Pain in back and hips
• Difficulty in walking
• Deformity of flixion hip, lumbar lordosis
• Short stature
• Other Malformations
Earlier Research
Even before the establishment of etiology and understanding the pathophysiology
of the disease, based on the X-ray findings, an attempt was made at medical
intervention by the surgical approach on selected cases. Though limited only to
very few numbers, because of practical reasons, surgical interventions didn’t
improve the quality of life of the people who underwent the surgical procedure.
Other medical interventions of this disease if any, is limited to anti-inflammatory
and pain relieving medications with little benefit in terms of therapeutic effect.
The Indian Council of Medical Research (ICMR), New Delhi, upon request
from Government of Karnataka, has initiated research to identify the etiology of
this disease and also to recommend appropriate medical intervention measures. In
the year 1975, a team of medical experts which included neurolgist Late Dr. K.S.
Mani and orthopaediatrist Late Dr. H.K. Srinivas Murthy were sent by the
Government of Karnataka to study the problem. The team carried out clinico-
radiological study on 45 patients and controls and certified that it is an osteo-
articular disease predominantly involving joints. However the exact cause of the
disease remained unknown despite the pathological tests on the blood samples of
patients. Further environmental studies carried out by National Institute of Nutrition
(NIN, Hyderabad) could not detect any ecological disturbances, trace metal toxicities
or undue exposure to pesticides. In the year 1982, Dr. Nuruddin of Bangalore
Medical College, suggested the possibility of genetic component contributing to
the disease, when he observed multiple affected individuals within the families.
Encouraged with this observation the Indian Council of Medical Research (ICMR)
in the year 1985 appointed a task force team headed by Dr. S.S. Agarwal, to carry
out a detailed epidemiological, environmental, metabolic and genetic study. At the
end of the first phase of this study, the team concluded the following;
• The trait follows autosomal dominant inheritance pattern.
• Patients can be categorized into 3 phenotypic groups: type I (predominantly
arthritic), type II (predominantly dysplastic) and type III (dwarf and severe
deformities in spine, hip, Knee and wrist).
• The radiological manifestations of HGS are similar to Mseleni joint
disease, except that the latter doesn’t follow a particular inheritance pattern.
Pulamaghatta N. Venugopal, I. Arjun Rao, Devajana C. Nanjunda & Adimoolam 29
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
The second phase of the ICMR project, which involved extensive biochemical
studies, which was carried out at NIMHANS, Bangalore. The study revealed
increased peptide bound proline to 4-hydroxyproline ratio in the urine of patients
compared to controls, suggesting the possibility of defective prolyl hydroxylation,
a post translational modification of proline residues of collagen. (Badadani et al.,
2009). Further, the study revealed low plasma total calcium and phosphorus
associated with low plasma calcitonin without any changes in parathyroid hormone
level among all the three subtypes of HG as compared to the unaffected members
of HG affected families (Badadani et al., 2010).
Possible Causes
Many hypotheses have been put forth as possible causes of this disease:
• The disease may be because the patients consumed dead crabs as food
(which may have high content of pesticide) (Srikanth 2007).
• The prevalence of disease is due to the marriage which have taken place /
taking place in close family members where most of them are affected
(Srikanth 2007) due to consanguineous marriage.
• The possibility of defective prolyl hydroxylation hints off a defect in the
concerned enzyme, the prolyl-4-hydroxylase (Badadani et al., 2009).
• Hypocalcitonemia without change in Parathyroid hormone levels
associated with hypocalcaemia suggests a possibility of a defective Vitamin
D receptor (Badadani et al., 2010).
In brief, what can be said at present is that the studies carried out by various
institutes have led us to the tip of the iceberg and calls for the extended studies on
the crippling disorder of skeletal systems which incidentally affects a socio-
economically backward communities living in certain pockets of Karnataka state.
The need of the hour is to get deep into the problems to understand the basic issues
pertaining to the disease including the epidemiology, biochemistry of the disease,
the gene/s involved and the possible nutritional or environmental factors
contributing to the complexities of the clinical presentations and disease progression.
Thus, the better understanding of the disease in broader perspectives will lead to
the rational medico-social intervention to change the course of disease progression
and to improve the quality of life of HGS affected.
METHODOLOGY
Area Under Study
The present study is conducted in 25 villages (Table 1) of the Sagar and adjoining
taluks of the Shimoga district. The affected villages fall in the malnad area of
Western ghats (fig. 1).
30 Influence of Selected Demographic Variables
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Shimoga district is part of the Malnad region of Karnataka and is also known
as the “Gateway to Malnad” or Malenaada Hebbagilu in Kannada, Located in the
central part of Karnataka state. Shimoga district consists of seven taluks namely
Shimoga, Sagara, Thirthahalli, Hosanagara, Bhadravathi, Shikaripur and Soraba.
Figure 1: Map of Handigodu Disease Affected Area in Karnataka
Pulamaghatta N. Venugopal, I. Arjun Rao, Devajana C. Nanjunda & Adimoolam 31
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Sampling
All the patients included in the study were those identified by Indian Council of
Medical Research (ICMR) based on radiological findings. Information regarding
patients was obtained from the Medical officer of Handigodu disease mobile health
unit of Shimoga district. A structured questionnaire was administered to collect
socio-demographic information like ethnicity, age, sex, marriage type, food habits,
etc. Secondary data from hospitals, dispensaries, District Health Office were also
collected to identify the cases of Handigodu disease in the affected areas. Secondary
data obtained by Handigodu disease mobile health unit.
Data Collection
From each individual personal detail like name, gender, date of birth, cast/
community, religion, nationality and occupation were collected with the use of a
questionnaire. Questions which obtain information about food and lifestyle habits
like tobacco consumption, smoking status, and alcohol consumption status and
health history were also included in the questionnaire.
Figure 2: Distribution of HG type I cases
32 Influence of Selected Demographic Variables
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Figure 3: Distribution of HG type II cases
Figure 4: Distribution of HG type III Cases
Pulamaghatta N. Venugopal, I. Arjun Rao, Devajana C. Nanjunda & Adimoolam 33
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Figure 5: Distribution of HG All Type
Statistical Tools
Descriptive statistics were calculated for all the demographic and clinical
variables. Frequency differences between Affected and Unaffected groups for
the discontinuous variables were checked using crosstab analysis. All the above
analysis was performed with the use of SPSS v 12 software (SPSS for windows,
USA).
RESULTS
The analysis in this part is confined to the present investigation, where the
researcher personally collected data on 300 selected samples (166 HGS affected+
134 unaffected) in 2 taluks (Sagar and Soraba) from 25 villages. The data
has been collected on various demographic parameters along with clinical
variables.
34 Influence of Selected Demographic Variables
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
contd. table 2
Table 1
Details of the Samples Collected from Different Villages in Shimoga District
Sl.No Villages No. of Samples
1 Bandagadde 30
2 Handigodu 24
3 Karehonda 22
4 Lingadahalli 20
5 Beleyur 19
6 Hirale 17
7 Kanelepur 16
8 Keladipura 16
9 Ambapura 16
10 Kolisalu 13
11 Manehale 13
12 Chikkabilagunji 12
13 Kanale 11
14 Malaliyur 11
15 Kalsipete 9
16 Belur 8
17 Sangla 7
18 Othigodu 7
19 M.Kanugodu 6
20 Harokoppa 5
21 Sagar Town 5
22 Gaddemane 4
23 K.Kanugodu 3
24 Mandagalale 3
25 Kerekoppa 3
Total 300
Table 2
Associations of Gender with Various Parameters of HG Disease and Results of
Contingency Coefficient Analysis
Types and deformities Gender
Male Female Total CC Value P Value
Types Un affected F 69 65 134 .201 .006
% 54.8% 37.3% 44.7%
Type 1 F 23 57 80
% 18.3% 32.8% 26.7%
Type 2 F 25 33 58
% 19.8% 19.0% 19.3%
Type 3 F 9 19 28
% 7.1% 10.9% 9.3%
Hip OA No hip OA F 83 108 191 .113 .276
% 65.9% 62.1% 63.7%
Pulamaghatta N. Venugopal, I. Arjun Rao, Devajana C. Nanjunda & Adimoolam 35
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Mild F 12 23 35
% 9.5% 13.2% 11.7%
Moderate F 29 43 72
% 23.0% 24.7% 24.0%
Severe F 2 0 2
% 1.6% .0% .7%
Hip Dysplasia No hip Dysplasia F 83 108 191 .113 .276
% 65.9% 62.1% 63.7%
Mild F 12 23 35
% 9.5% 13.2% 11.7%
Moderate F 29 43 72
% 23.0% 24.7% 24.0%
Severe F 2 0 2
% 1.6% .0% .7%
Spinal No Spinal F 96 125 221 .087 .510
Dysplasia dysplasia % 76.2% 71.8% 73.7%
Mild F 22 37 59
% 17.5% 21.3% 19.7%
Moderate F 7 12 19
% 5.6% 6.9% 6.3%
Severe F 1 0 1
% .8% .0% .3%
Platyspondyly No Platyspondyly F 99 134 233 .044 .900
% 78.6% 77.0% 77.7%
Mild F 7 13 20
% 5.6% 7.5% 6.7%
Moderate F 13 19 32
% 10.3% 10.9% 10.7%
Severe F 7 8 15
% 5.6% 4.6% 5.0%
Knee dysplasia No Knee dysplasia F 112 151 263 .061 .769
% 88.9% 86.8% 87.7%
Mil F 9 12 21
% 7.1% 6.9% 7.0%
Moderate F 4 10 14
% 3.2% 5.7% 4.7%
Severe F 1 1 2
% .8% .6% .7%
Wrist dysplasia No Wrist dysplasia F 123 166 289 .087 .320
% 97.6% 95.4% 96.3%
Mild F 3 5 8
% 2.4% 2.9% 2.7%
Moderate F 0 3 3
% .0% 1.7% 1.0%
Total F 126 174 300
% 100.0% 100.0% 100.0%
Types and deformities Gender
Male Female Total CC Value P Value
36 Influence of Selected Demographic Variables
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Types of HGS: A significant association was observed between types and gender
where the contingency coefficient value of .201 was found to be significant at .006
level. From the table it is clear that among males we find more of unaffected, and
among females we find more of type 1, and type 3 HGS affected.
Hip OA: Between categories of Hip OA and gender, a non-significant
association was observed revealing a similarity in the distribution of frequencies
of male and female HGS affected in different Hip OA categories (CC=.113;
P=.276).
Hip dysplasia: As seen Hip OA, in hip dysplasia also, contingency coefficient
analysis revealed a non-significant association between categories of hip dysplasia
and gender (CC=0.113; P=.276).
Spinal dysplasia: Between categories of spinal dysplasia and gender, a non-
significant association was observed revealing a similarity in the distribution of
frequencies of male and female HGS affected in different spinal dysplasia categories
(CC=.087; P=.510).
Platyspondyly: In platyspondyly also, contingency coefficient analysis revealed
a non-significant association between categories of paltyspondyly and gender
(CC=0.044; P=.900).
Knee dysplasia: Contingency coefficient analysis revealed a non-significant
association between categories of knee dysplasia and gender (CC=.061; P=.769).
Wrist dysplasia: From the table it is clear that a categories of wrist dysplasia
were independent of gender as the observed contingency coefficient value of .087
was found to be non-significant, indicating similarity in the distribution of
frequencies of male and female affected in different categories of wrist dysplasia.
Table 3
Associations of Age Groups with Various Parameters of HGS and Results of
Contingency Coefficient Analysis
Types and deformities Age Group in years Total CC P
< 20 21-35 36-50 51- 65 Value Value
Type Un affected F 16 50 48 20 134 .543 .000
% 100.0% 90.9% 33.3% 23.5% 44.7%
Type 1 F 0 0 36 44 80
% .0% .0% 25.0% 51.8% 26.7%
Type 2 F 0 1 36 21 58
% .0% 1.8% 25.0% 24.7% 19.3%
Type 3 F 0 4 24 0 28
% .0% 7.3% 16.7% .0% 9.3%
Hip OA No hip OA F 16 52 86 27 181 .481 .000
% 100.0% 94.5% 59.7% 31.8% 60.3%
contd. table 3
Pulamaghatta N. Venugopal, I. Arjun Rao, Devajana C. Nanjunda & Adimoolam 37
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Mild F 0 1 33 12 46
% .0% 1.8% 22.9% 14.1% 15.3%
Moderate F 0 1 17 37 55
% .0% 1.8% 11.8% 43.5% 18.3%
Severe F 0 1 8 9 18
% .0% 1.8% 5.6% 10.6% 6.0%
Hip No hip F 16 51 76 48 191 .381 .000
Dysplasia Dysplasia
% 100.0% 92.7% 52.8% 56.5% 63.7%
Mild F 0 1 15 19 35
% .0% 1.8% 10.4% 22.4% 11.7%
Moderate F 0 3 52 17 72
% .0% 5.5% 36.1% 20.0% 24.0%
Severe F 0 0 1 1 2
% .0% .0% .7% 1.2% .7%
Spinal No Spinal F 16 52 92 61 221 .285 .002
Dysplasia dysplasia
% 100.0% 94.5% 63.9% 71.8% 73.7%
Mild F 0 3 39 17 59
% .0% 5.5% 27.1% 20.0% 19.7%
Moderate F 0 0 12 7 19
% .0% .0% 8.3% 8.2% 6.3%
Severe F 0 0 1 0 1
% .0% .0% .7% .0% .3%
Platy- No Platy- F 16 52 91 74 233 .331 .000
spondily spondyly
% 100.0% 94.5% 63.2% 87.1% 77.7%
Mild F 0 0 15 5 20
% .0% .0% 10.4% 5.9% 6.7%
Moderate F 0 2 25 5 32
% .0% 3.6% 17.4% 5.9% 10.7%
Severe F 0 1 13 1 15
% .0% 1.8% 9.0% 1.2% 5.0%
Knee No Knee F 16 52 117 78 263 .215 .105
dysplasia
% 100.0% 94.5% 81.3% 91.8% 87.7%
Mild F 0 2 13 6 21
% .0% 3.6% 9.0% 7.1% 7.0%
Moderate F 0 1 12 1 14
% .0% 1.8% 8.3% 1.2% 4.7%
Types and deformities Age Group in years Total CC P
< 20 21-35 36-50 51- 65 Value Value
contd. table 3
38 Influence of Selected Demographic Variables
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Severe F 0 0 2 0 2
% .0% .0% 1.4% .0% .7%
Wrist No Wrist F 16 53 136 84 289 .118 .646
dysplasia
% 100.0% 96.4% 94.4% 98.8% 96.3%
Mild F 0 1 6 1 8
% .0% 1.8% 4.2% 1.2% 2.7%
Moderate F 0 1 2 0 3
% .0% 1.8% 1.4% .0% 1.0%
Total F 16 55 144 85 300
% 100.0%100.0% 100.0% 100.0% 100.0%
Types of HGS: On the whole, we find most of the sample unaffected (44.7%),
followed by type I HGS (26.7%), type II HGS (19.3%) and remaining 9.3% of
them were with Type III HGS. Age group comparison revealed a significant
association with HGS types and taluks (CC=.543; P=.000). It is clear from the
table that as the age increased unaffected frequencies linearly decreased and in
higher age groups we find affected one’s more.
Hip OA: Of the 300 samples tested in the second phase, majority of the sample
did not have hip OA (60.3%), 15.3% of the sample presented with mild hip OA,
18.3% were with moderate hip OA, and remaining 6.0% of them had severe hip
OA. Contingency coefficient analysis revealed difference in the distribution of hip
OA and age groups (CC=.481; P=.000), where we find that maximum mild hip OA
in the age group of 36-50 years, and maximum moderate Hip OA in the age group
of 51-65 years.
Hip dysplasia: A majority of 63.7% of the selected sample did not have hip
dysplasia, 24.0% of them had moderate hip dysplasia, 11.7% of them had mild hip
dysplasia and remaining 0.7% of them had severe Hip dysplasia. Further,
contingency coefficient analysis revealed a significant association between Hip
dysplasia and age groups (CC=.381; P=.000), where we find that absence of hip
dysplasia decreased as the age increased. Further, it was observed that maximum
mild hip dysplasia was found more in the age group of 51-65 years and moderate
dysplasia was found more in the age group of 36-50 years.
Spinal dysplasia: From the table it is clear that a large majority of 73.7% of the
selected sample did not have spinal dysplasia, 19.7% of them presented with mild
spinal dysplasia, 6.3% of them had moderate spinal dysplasia and remaining 0.3%
of them presented with severe spinal dysplasia. Further, a significant association
was observed between spinal dysplasia and age groups (CC=.285; P=.002), It can
Types and deformities Age Group in years Total CC P
< 20 21-35 36-50 51- 65 Value Value
Pulamaghatta N. Venugopal, I. Arjun Rao, Devajana C. Nanjunda & Adimoolam 39
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
be inferred from the table that absence of spinal dysplasia decreased as the age
increased. We find more number of HGS affected in mild category of spinal dysplasia
in the age group of 36-50 years.
Platyspondyly: Of the 300 samples diagnosed, a majority of the 77.7% of them
did not show Platyspondyly, 6.7% of the sample presented with mild Platyspondyly,
10.70% of them presented with moderate Platyspondyly, and 5.0% of them had
severe Platyspondyly. Contingency coefficient analysis revealed a similarity in the
distribution of Platyspondyly with categories and age groups (CC=.331; P=.000),
where the absence of Platyspondyly decreased as the age increased. We find more
number of HGS affected persons in mild category of Platyspondyly in the age
group of 36-50 years.
Knee dysplasia: Overall, a large majority of 87.7% of the selected sample did
not have knee dysplasia, 7.0% of them had mild knee dysplasia, 4.7% of them had
moderate knee dysplasia and remaining 0.7% of them had severe knee dysplasia.
Further, contingency coefficient analysis revealed a non-significant association
between knee dysplasia and age groups (CC=.137; P=.545).
Wrist dysplasia: From the table it is clear that a vast majority of 96.3% of the
selected sample did not have wrist dysplasia, 2.7% of them presented with mild
wrist dysplasia, 1.0% of them had moderate wrist dysplasia and none of them
presented with severe wrist dysplasia. Further, a non-significant association was
observed between spinal dysplasia and age groups was observed (CC=.118; P=.646).
Types of HGS: On the whole, we find 48.2% of the sample selected in affected
with Type 1 HGS, 34.9% of them were with type 2 and remaining 16.9% were with
type 3 HGS. Further, a significant association was observed between age groups
and types where the contingency coefficient of .673 was found to be significant at
.000 level. From the table it is clear that HG1 was found to be more in the older age
groups of and type 2 and type 3 HGS frequencies were found to be more in younger
age groups.
Hip OA: In hip OA, 28.3% of the sample devoid of hip OA, 27.7% of them
was affected with mild Hip OA, 33.1% of them are with moderate Hip OA and
remaining 10.8% of them are found with severe hip OA. In hip dysplasia also,
contingency coefficient analysis revealed a significant association with age groups
(CC=.529; P=.000). It is evident from the table that moderate dysplasia was found
to be maximum in the age group of 41-50 and 51-60 years of HGS affected, where
as younger age groups did not have much of hip OA.
Hip dysplasia: As far as Hip dysplasia is considered, 34.3% of them did not
have hip dysplasia, 21.1% with mild, 43.4% of them with moderate and 1.2% of
them are presented with severe hip dysplasia. Further, a significant association
was observed between age groups and type of hip dysplasia, where the contingency
40 Influence of Selected Demographic Variables
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Table 4
Associations of Age of Onset with Various Parameters of HGS with Results of Contingency Coefficient Analysis
Types and deformities Age of onset in years Total CC Value P Value
< 10 11-20 21-30 31-40 41-50 51-60
Types Type 1 F 0 7 1 28 29 15 80 .673 .000
% .0% 21.9% 10.0% 82.4% 90.6% 93.8% 48.2%
Type 2 F 19 21 953158
% 45.2% 65.6% 90.0% 14.7% 9.4% 6.3% 34.9%
Type 3 F 23 4010028
% 54.8% 12.5% .0% 2.9% .0% .0% 16.9%
Hip OA No hip OA F 28 11132247.529 .000
% 66.7% 34.4% 10.0% 8.8% 6.3% 12.5% 28.3%
MildF986137346
% 21.4% 25.0% 60.0% 38.2% 21.9% 18.8% 27.7%
Moderate F 2 9 3 14 17 10 55
% 4.8% 28.1% 30.0% 41.2% 53.1% 62.5% 33.1%
Severe F 34046118
% 7.1% 12.5% .0% 11.8% 18.8% 6.3% 10.8%
Hip Dysplasia No hip F 2 5 1 20 19 10 57 .518 .000
dysplasia % 4.8% 15.6% 10.0% 58.8% 59.4% 62.5% 34.3%
MildF86487235
% 19.0% 18.8% 40.0% 23.5% 21.9% 12.5% 21.1%
Moderate F 31 21 556472
% 73.8% 65.6% 50.0% 14.7% 18.8% 25.0% 43.4%
Severe F 1001002
% 2.4% .0% .0% 2.9% .0% .0% 1.2%
Spinal No Spinal F 8 8 0 27 29 15 87 .578 .000
Dysplasia dysplasia % 19.0% 25.0% .0% 79.4% 90.6% 93.8% 52.4%
contd. table 4
Pulamaghatta N. Venugopal, I. Arjun Rao, Devajana C. Nanjunda & Adimoolam 41
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Types and deformities Age of onset in years Total CC Value P Value
< 10 11-20 21-30 31-40 41-50 51-60
MildF2618752159
% 61.9% 56.3% 70.0% 14.7% 6.3% 6.3% 35.5%
Moderate F 85321019
% 19.0% 15.6% 30.0% 5.9% 3.1% .0% 11.4%
Severe F 0100001
% .0% 3.1% .0% .0% .0% .0% .6%
Platy- No Platy- F 5 11 4 32 31 16 99 .611 .000
spondyly spondyly % 11.9% 34.4% 40.0% 94.1% 96.9% 100.0% 59.6%
MildF96311020
% 21.4% 18.8% 30.0% 2.9% 3.1% .0% 12.0%
Moderate F 19 10 210032
% 45.2% 31.3% 20.0% 2.9% .0% .0% 19.3%
Severe F 95100015
% 21.4% 15.6% 10.0% .0% .0% .0% 9.0%
Knee No Knee F 17 25 7 32 32 16 129 .520 .000
dysplasia dysplasia % 40.5% 78.1% 70.0% 94.1% 100.0% 100.0% 77.7%
MildF125310021
% 28.6% 15.6% 30.0% 2.9% .0% .0% 12.7%
Moderate F 12 1010014
% 28.6% 3.1% .0% 2.9% .0% .0% 8.4%
Severe F 1100002
% 2.4% 3.1% .0% .0% .0% .0% 1.2%
Wrist No Wrist F 35 29 10 33 32 16 155 .297 .099
dysplasia % 83.3% 90.6% 100.0% 97.1% 100.0% 100.0% 93.4%
MildF4301008
% 9.5% 9.4% .0% 2.9% .0% .0% 4.8%
Moderate F 3000003
% 7.1% .0% .0% .0% .0% .0% 1.8%
42 Influence of Selected Demographic Variables
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
coefficient value of .518 was found to be significant at .000 level. From the table
it is clear that samples belonging to the age groups <10 and 11-20 years
showed more of moderate hip dysplasia and age had inverse relation with hip
dysplasia.
Spinal dysplasia: 52.4% of the selected sample did not have spinal dysplasia,
followed by 35.5% who showed mild spinal dysplasia, 11.4% of them were with
moderate dysplasia and remaining few 0.6% of them showed severe spinal dysplasia.
In spinal dysplasia also, contingency coefficient analysis revealed a significant
association with age groups (CC=.578; P=.000). We find an age related decrease
in the presence of spinal dysplasia. It is evident from the table that mild spinal
dysplasia was found more in younger age groups.
Platyspondyly: On the whole, majority of the sample did not have platyspondyly
(59.6%), 12.0% of them showed mild, 19.3% of them showed moderate and
remaining 9.0% of them showed severe platyspondyly. In platyspondyly, between
categories of platyspondyly and age groups, contingency coefficient analysis showed
a significant association (CC=.611; P=.000). From the table it is clear that absence
of platyspondyly linearly increased as the age increased. Moderate platyspondyly
was found to be maximum in the age group of younger age groups. Again severe
platyspondyly was found more in younger age groups than older age groups.
Knee dysplasia: As far as knee dysplasia is considered, again a significant
association was observed between categories and age groups, where the contingency
coefficient value of .520 was found to be significant at .000 level. From the table it
is clear that samples in higher age groups we find no dysplasia, moderate knee
dysplasia was found in more in the younger age groups. Relatively we find very
less frequencies of knee dysplasia in all the age groups.
Wrist dysplasia: A large majority of 93.4% of the selected sample was devoid
of wrist dysplasia. 4.8% of them showed mild and 1.8% of them showed moderate
wrist dysplasia. In wrist dysplasia, we find a non-significant association between
categories of wrist dysplasia and age groups (CC=.258; P=.015). None of the
selected sample had severe wrist dysplasia.
Types of HGS: A significant association was observed between types and caste
categories, where the contingency coefficient value of .258 was found to be
significant at .045 level. From the table it is clear that we find more of unaffected
among Chananngi, and Chaluvadi castes, more type I HGS among Chaluvadi and
Vokkalinga castes, more type 2 and 3 HGS among Ediga castes.
Hip OA: Between categories of Hip OA and castes, a non-significant association
was observed revealing a similarity in the distribution of frequencies of HGS affected
in different castes with different Hip OA categories (CC=.133; P=.943).
Pulamaghatta N. Venugopal, I. Arjun Rao, Devajana C. Nanjunda & Adimoolam 43
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Table 5
Associations of Caste with Various Parameters of HGS and Results of Contingency Coefficient Analysis
Types and deformities Castes Total CC Value P Value
Chana- Chalu- Vokaliga Ediga Uppara
nngi vadi
Types Un affected F 75 43 8 3 5 134 .258 .045
% 42.9% 51.2% 38.1% 27.3% 55.6% 44.7%
Type 1 F 53 20 5 1 1 80
% 30.3% 23.8% 23.8% 9.1% 11.1% 26.7%
Type 2 F 37 12 5 3 1 58
% 21.1% 14.3% 23.8% 27.3% 11.1% 19.3%
Type 3 F 10 9 3 4 2 28
% 5.7% 10.7% 14.3% 36.4% 22.2% 9.3%
Hip OA No hip OA F 106 51 13 4 7 181 .133 .943
% 60.6% 60.7% 61.9% 36.4% 77.8% 60.3%
Mild F 24 15 3 3 1 46
% 13.7% 17.9% 14.3% 27.3% 11.1% 15.3%
Moderate F 33 14 4 3 1 55
% 18.9% 16.7% 19.0% 27.3% 11.1% 18.3%
Severe F 12 4 1 1 0 18
% 6.9% 4.8% 4.8% 9.1% .0% 6.0%
Hip Dysplasia No hip dysplasia F 109 59 12 6 5 191 .163 .768
% 62.3% 70.2% 57.1% 54.5% 55.6% 63.7%
Mild F 26 4 3 1 1 35
% 14.9% 4.8% 14.3% 9.1% 11.1% 11.7%
Moderate F 39 20 6 4 3 72
% 22.3% 23.8% 28.6% 36.4% 33.3% 24.0%
Severe F 1 1 0 0 0 2
% .6% 1.2% .0% .0% .0% .7%
Spinal Dysplasia No Spinal dysplasia F 135 61 13 6 6 221 .354 .000
% 77.1% 72.6% 61.9% 54.5% 66.7% 73.7%
contd. table 5
44 Influence of Selected Demographic Variables
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Types and deformities Castes Total CC Value P Value
Chana- Chalu- Vokaliga Ediga Uppara
nngi vadi
Mild F 29 17 8 3 2 59
% 16.6% 20.2% 38.1% 27.3% 22.2% 19.7%
Moderate F 11 6 0 2 0 19
% 6.3% 7.1% .0% 18.2% .0% 6.3%
Severe F 0 0 0 0 1 1
% .0% .0% .0% .0% 11.1% .3%
Platy-spondyly No Platyspondyly F 140 66 14 6 7 233 .255 .052
% 80.0% 78.6% 66.7% 54.5% 77.8% 77.7%
Mild F 11 8 0 1 0 20
% 6.3% 9.5% .0% 9.1% .0% 6.7%
Moderate F 19 4 6 2 1 32
% 10.9% 4.8% 28.6% 18.2% 11.1% 10.7%
Severe F 5 6 1 2 1 15
% 2.9% 7.1% 4.8% 18.2% 11.1% 5.0%
Knee No Knee dysplasia F 157 77 15 7 7 263 .244 .090
% 89.7% 91.7% 71.4% 63.6% 77.8% 87.7%
Mild F 11 2 4 3 1 21
% 6.3% 2.4% 19.0% 27.3% 11.1% 7.0%
Moderate F 6 4 2 1 1 14
% 3.4% 4.8% 9.5% 9.1% 11.1% 4.7%
Severe F 1 1 0 0 0 2
% .6% 1.2% .0% .0% .0% .7%
Wrist No Wrist dysplasia F 172 80 19 10 8 289 .278 .002
% 98.3% 95.2% 90.5% 90.9% 88.9% 96.3%
Mild F 3 4 1 0 0 8
% 1.7% 4.8% 4.8% .0% .0% 2.7%
Moderate F 0 0 1 1 1 3
% .0% .0% 4.8% 9.1% 11.1% 1.0%
Total F 175 84 21 11 9 300
% 100.0% 100.0% 100.0% 100.0% 100.0% 100.0%
Pulamaghatta N. Venugopal, I. Arjun Rao, Devajana C. Nanjunda & Adimoolam 45
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Hip dysplasia: As seen Hip OA, in hip dysplasia also, contingency coefficient
analysis revealed a non-significant association between categories of hip dysplasia
and castes (CC=0.163; P=.768).
Spinal dysplasia: Between categories of spinal dysplasia and gender, a
significant association was observed (CC=.354; P=.000), where we find that more
of mild spinal dysplasia among vokkaligas, more of moderate spinal dysplasia
among Edigas and more of severe spinal dysplasia among upparas.
Platyspondyly: In platyspondyly also, contingency coefficient analysis revealed
a non-significant association between categories of platyspondyly and castes
(CC=0.255; P=.052).
Knee dysplasia: Contingency coefficient analysis revealed a non-significant
association between categories of knee dysplasia and gender (CC=.244; P=.090).
Wrist dysplasia: From the table it is clear that categories of wrist dysplasia
were independent of castes as the observed contingency coefficient value of .278
was found to be significant (P=.002). Though we do not find any case of severe
wrist dysplasia, moderate dysplasia was found to be more among upparas caste.
Table 6
Associations of Types of HGS with Various Parameters and Results of
Contingency Coefficient Analysis
Types and deformities TYPE Total CC P
Value Value
UA Type 1 Type 2 Type 3
Hip OA No hip OA F 134 7 24 16 181 .628 .000
% 100.0% 8.8% 41.4% 57.1% 60.3%
Mild F 0 23 17 6 46
% .0% 28.7% 29.3% 21.4% 15.3%
Moderate F 0 41 10 4 55
% .0% 51.3% 17.2% 14.3% 18.3%
Severe F 0 9 7 2 18
% .0% 11.2% 12.1% 7.1% 6.0%
Hip No F 134 54 2 1 191 .654 .000
Dysplasia hipdysplasia % 100.0% 67.5% 3.4% 3.6% 63.7%
Mild F 0 14 16 5 35
% .0% 17.5% 27.6% 17.9% 11.7%
Moderate F 0 12 39 21 72
% .0% 15.0% 67.2% 75.0% 24.0%
Severe F 0 0 1 1 2
% .0% .0% 1.7% 3.6% .7%
contd. table 6
46 Influence of Selected Demographic Variables
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
Spinal No Spinal F 134 74 11 2 221 .649 .000
Dysplasia dysplasia % 100.0% 92.5% 19.0% 7.1% 73.7%
Mild F 0 5 34 20 59
% .0% 6.25% 58.6% 71.4% 19.7%
Moderate F 0 1 12 6 19
% .0% 1.25% 20.7% 21.4% 6.3%
Severe F 0 0 1 0 1
% .0% .0% 1.7% .0% .3%
Platy- No F 134 78 19 2 233 .643 .000
spondyly Platyspondyly % 100.0% 97.5% 32.8% 7.1% 77.7%
Mild F 0 2 11 7 20
% .0% 2.5% 19.0% 25.0% 6.7%
Moderate F 0 0 21 11 32
% .0% .0% 36.2% 39.3% 10.7%
Severe F 0 0 7 8 15
% .0% .0% 12.1% 28.6% 5.0%
Knee No Knee F 134 77 45 7 263 .584 .000
dysplasia % 100.0% 96.3% 77.6% 25.0% 87.7%
Mild F 0 3 10 8 21
% .0% 3.8% 17.2% 28.6% 7.0%
Moderate F 0 0 3 11 14
% .0% .0% 5.2% 39.3% 4.7%
Severe F 0 0 0 2 2
% .0% .0% .0% 7.1% .7%
Wrist No Wrist F 134 80 55 20 289 .415 .000
dysplasia % 100.0% 100.0% 94.8% 71.4% 96.3%
Mild F 0 0 3 5 8
% .0% .0% 5.2% 17.9% 2.7%
Moderate F 0 0 0 3 3
% .0% .0% .0% 10.7% 1.0%
Total F 134 80 58 28 300
% 100.0%100.0%100.0%100.0% 100.0%
Hip OA: On the whole, we find 60.3% of the sample selected was not affected
with Hip OA, 15.3% of them had mild hip OA, 18.3% of them presented with
moderate hip OA, and remaining 6.0% of them had severe hip OA. Between types
Types and deformities TYPE Total CC P
Value Value
UA Type 1 Type 2 Type 3
Pulamaghatta N. Venugopal, I. Arjun Rao, Devajana C. Nanjunda & Adimoolam 47
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
and categories of hip OA, a significant association was observed (CC=.628; P=.000),
where we find that unaffected ones did not have hip OA, are find more of type 1
HGS affected in moderated hip OA category.
Hip Dysplasia: 63.7% of the selected sample was devoid of hip dysplasia, 11.7%
of them had mild dysplasia, 24% of them had moderate hip dysplasia, and remaining
0.7% of them had severe hip dysplasia. Contingency coefficient analysis (CC=.654;
P=.000), revealed a significant association between categories of hip dysplasia and
types of HGS. As found earlier, unaffected ones did not have hip dysplasia and we
find more frequencies of moderate hip dysplasia in type 2 and type 3 HGS affected.
Spinal Dysplasia: A large majority of the 73.7% of the sample did not have
spinal dysplasia. 19.7% of the sample had mild spinal dysplasia, 6.3% of them
were presented with moderate dysplasia and remaining 0.3% of them showed severe
dysplasia. Between types and categories of spinal dysplasia, a significant association
was observed (CC=.649; P=.000), where we find that unaffected ones did not have
any dysplasia, and more frequencies of moderate spinal dysplasia in type 2 and
type 3 HGS affected.
Platyspondyly: On the whole, we find majority of 77.7% of the sample selected
was not affected with Platyspondyly, 6.7% of them had mild platyspondyly, 10.7%
of them presented with moderate platyspondyly, and remaining 5.0% of them had
severe platyspondyly. Between types and categories of platyspondyly, a significant
association was observed (CC=.643; P=.000), where we find that unaffected ones
did not have platyspondyly, again we find more in moderately affected platyspondyly
in type 2 and type 3 HGS affected.
Knee dysplasia: A large majority of the 87.7% of the sample did not have knee
dysplasia. 7.0% of the sample had mild knee dysplasia, 4.7% of them were presented
with moderate dysplasia and remaining 0.7% of them showed severe dysplasia.
Between types and categories of knee dysplasia, a significant association was
observed (CC=.584; P=.000), where we find that unaffected ones did not have any
dysplasia, and more frequencies of moderate spinal dysplasia in type 3 HGS affected.
Wrist dysplasia: On the whole, we find majority of 96.3% of the sample selected
was not affected with Wrist dysplasia, 2.7% of them had mild platyspondyly and
remaining 1.0% of them had moderate wrist dysplasia. Between types and categories
of wrist dysplasia significant association was observed (CC=.415; P=.000), where
we find that unaffected ones did not have wrist.
A significant association was observed between type of HGS and marital status,
where the contingency coefficient of .479 was found to be significant at .000 level.
From the table it is evident that married Hg persons were more affected with type
1 HGS (93.8%) and type 2 HGS (87.9%) as against unmarried ones. However, in
type 3 HGS we find more of unmarried affected ones (64.3%) than married ones.
48 Influence of Selected Demographic Variables
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
DISCUSSION AND CONCLUSION
Main Findings of the Study
1. The HG disease was found more with older age groups, Type 1 manifestations
were found more with persons aged above 51 years and type was more with
36-50 and 51-65 years. Relatively type 3 was observed in early adulthood and
adult developmental stages.
2. Hip dysplasia, spinal dysplasia, platyspondyly, knee dysplasia and wrist
dysplasia are commonly occur at younger and adult age groups, where as hip
OA relatively occurs among aged persons.
3. HGS type 1 was found occur among aged where as type 2 and type 3 occur
significantly at younger age.
4. The deformities like Hip dysplasia, spinal dysplasia, platyspondyly, and knee
dysplasia begin their manifestations in early ages compared to hip OA, which
begins its manifestation at later ages.
5. Females are more affected in each of the type of HG disease than male samples.
6. Type 1 HGS was found more with moderate hip OA, hip dysplasia, spinal
dysplasia, and knee dysplasia were found to be more with type 2 HG and type
3 HG.
It was also observed that female subjects were more affected than male subjects
in the present study. The results of the present study are in agreement as far as
gender factor is considered, where Bhat et al., (2010) in his recent study reported
that females are more affected than males (38% males and 62% females).
Contradictorily, pedigree analysis conducted by ICMR (1994) revealed both males
Table 7
Associations of Types of HGS with Marital Status and Results of
Contingency Coefficient Analysis
Types Married Un married Total CC P
Type 1 F 75 5 80 .479 .000
% 93.8% 6.3% 100.0%
Type 2 F 51 7 58
% 87.9% 12.1% 100.0%
Type 3 F 10 18 28
% 35.7% 64.3% 100.0%
Total F 136 30 166
% 81.9% 18.1% 100.0%
Pulamaghatta N. Venugopal, I. Arjun Rao, Devajana C. Nanjunda & Adimoolam 49
Journal of the Anthropological Survey of India, 63(1) : (27-50), 2014
and females are equally affected Agarwal et al. (1994). It is now proved that female
subjects were more prone HG syndrome than male subjects.
As far as the onset of the disease is considered, it was found that the age of
onset of type1 HG is usually at adult hood and aged stages, type 2 Hg was usually
observed at late adolescent stage, whereas type 3 in early and late adolescence
developmental periods. These results have agreement with the findings of Agarwal
et al. (1997), who indicated that a single family has 3 types of HG due to difference
in the age of onset.
Previous epidemiological studies stated that the reason may be due to high
inbreeding or consanguinity (Agarwal et al, 1994), where there is the chance of
fixation of defective alleles. Previous studies (Agarwal et al., 1994, 1997) have
identified only Channangi and Cheluvadi castes are affected with HGS, whereas,
Bhat et al., (2010), and (ICMR task force report 2004), and the present study have
identified the HGS affected in other castes also. However, frequency of occurrence
of HGs in other castes is less compared to the Cheluvadi and Channangi caste.
Regarding the marital status, the frequency of unmarried in type 3 is more
when compared to type2 and type1 which is due to social stigma of the handigodu
disease (Bhat 2010 and Venugopal et al., (2011b). The earlier report (Agarwal et
al., 1997) mentioned that unaffected are not marrying affected individuals.
Cultural perceptions of the physical or social conditions vary from culture to
culture. Further, within the same culture stigma vary from time to time. Since
handigodu disease if mainly focused among members of two scheduled caste
communities, the problem of stigma has doubled. They still have to suffer from the
ancient stigma of untouchability attached to their caste but also have to suffer from
the modern stigma attached to Handigodu disease. Patients of handigodu disease
find it difficult to get employment in their villages. The local occupations are related
to agricultural and land owning employers are afraid of contracting the disease
from the patients. The patients are not able to go distant places in search of work
due to physical disability. In the studied area social stigma of handigodu disease is
prevalent; to eradicate this stigma people has to be educated with the information
about the disease that it is not a contagious disease.
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