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CAPENEWS Archive - General

-Contributed by Dr. Anju Virmani, New Delhi and Dr. Vijayakumar, Calicut


Endocrine Patient Information on the netBack to Top
Harry S Glauber, Oregon Health Services Univ., Portland, Oregon, USA. (Reprinted with permission from Endocrine News, Vol. 22, # 6, 1997 <br/>The Endocrine Society, in CAPENEWS 3.1, April 1999)


This article lists some of the sites I have found useful and reliable in providing patient information. The web browser software allows the user to organize these sites through "Bookmarks" or lists of "Favorite Places" which make it easy to return directly to a site of interest. I can print out a single copy of the hand out for the patient at the time of the office visit. I practice in a fully computerized office setting where the web browser is on the same work-station as the Electronic Medical Record. Finding and printing the patient hand-out therefore has become part of my regular office routine.

It is important to recognize that the Inter-net is continually changing and growing. Addresses ("URLs") may change, sites may disappear, and new sites continually appear. In spite of vast amounts of good information, there is also unreliable information and advice out there. Consumers (physicians and patients) need to be aware of the source of information they are reading. I will only share with patients information originating from reputable organizations that I am familiar with.

www Sites Containing Information Of Interest To Patients With Endocrine Disease (Partial and Selective List)

  • American Thyroid Association ( www.thyroid.org ) has useful information on hypothyroidism, thyroid hormone treatment, hyperthyroidism, Graves' disease, etc.
  • Thyroid Association of Canada ( www.thyroid.ca ): Thyroid disease is real: Know the facts, The thyroid gland: a general introduction, To confirm the clinical diagnosis; Hypothyroidism, Thyroid nodules, Thyroiditis, Hyperthyroidism, Graves' eye disease; Thyroid disease in childhood, Common concerns of thyroid patients.
  • www.thyrolink.com is a Merck sponsored site with links to patient information and of interest to professionals on thyroid diseases.
  • The Osteoporosis and Related Bone Diseases National Resource Center (www.osteo.org) links to excellent information on common and uncommon metabolic bone disorders (osteogenesis imperfecta, osteoporosis, etc.)
  • Pituitary Tumor Network Association ( www.pituitary.com ) provides information for patients and physicians and links to other useful sites.
  • The National Adrenal Diseases Foundation ( medhlp.netusa.net/www/nadf.htm#Nadfpubs) has information on Addison's disease, CAH and Cushing syndrome.
  • American Diabetes Association ( www.diabetes.org) provides information on many aspects of diabetes for patients and physicians and links to other useful sites.
  • www.niddk.nih.goc/NutritionDocs.html will provide information on nutrition and obesity.
  • The Medical matrix sites ( www.medmatrix.org ) provide access to a truly vast array of resources of interest including news, abstracts, reviews, articles, practice guidelines, images, meetings, forums and patient education.
  • The Turner Syndrome Society of the United States (www.turner-syndrome-us.org) has a wealth of information for patients about many aspects of this condition.
  • Finally, one of the most useful www sites for the practising endocrinologist is the Endocrine Society at www.endo-society.org

ENDOCRINE COMPLICATIONS IN MULTIPLY TRANSFUSED PATIENTS WITH THALASSEMIABack to Top
Amita Mahajan (from CAPENEWS 8.1, April 2004)


Optimal transfusion regimens and adequate chelation therapy have dramatically extended the life span of thalassemic children1. Despite all our resource limitations there has been major improvement in the overall status of thalassemic children and young adults in India as well, primarily attributable to better transfusion regimes, increased safety of blood, and oral chelation. More than that, however, there has been a perceptible change in the expectations and attitudes of families and physicians leading to overall improvement, both in the survival and general condition. As we come to grips with this cohort of growing thalassemics, it is important to appreciate the endocrinopathies that they are likely to have. As a general rule, it would be fair to say that endocrine complications primarily attributable to hemosiderosis at various sites are likely to be much more severe in under-chelated patients. However, it is important to appreciate that even optimally managed; adequately chelated patients may have endocrine complications, especially short stature.

An international collaborative study2 (n=3817) designed to look at the prevalence of endocrine abnormalities in a large cohort of thalassemic children and young adults showed a high prevalence of various endocrinopathies. Thirty percent were short, with Growth Hormone (GH) deficiency detected in 7.8%. Pubertal failure was seen in 16%. Primary hypothyroidism and insulin dependent diabetes each were seen in 3.2% patients. Hypoparathyroidism was seen in 7%. The extent of iron overload and liver damage seemed to be commonest determinants of the prevalence of endocrinopathies. It is important to highlight that 36.5% of the patients in this cohort were over the age of 16 years.

In another recent study (n=220)3, short stature was seen in 39.3% of patients. Delayed puberty was noted in 80.2% boys and 72.4% girls (22.9% boys and 12.2% girls had hypogonadism). The mean ferritin level of adolescents with delayed puberty was not statistically significantly different from those who underwent puberty at normal age. This emphasizes the fact that delayed puberty and several other endocrinopathies are by and large multifactorial. Hypoparathyroidism and primary hypothyroidism was present in 7.6% and 7.7% respectively. About 13% of patients had more than one endocrine complication with mean serum ferritin of 1678 ± 955 ug/L. This study also systematically looked at osteoporosis. The prevalence of lumbar osteoporosis and osteopenia were 50.7% and 39.4%. Femoral osteoporosis and osteopenia were present in 10.8% and 36.9% respectively. Low serum zinc and copper were observed in 79.6% and 68% respectively of the study population. (Other studies have, however, not shown such high levels of zinc and copper deficiency.) In 37.2% of patients serum levels of 25(OH) D below 23 nmol/L were detected.

Other studies4,5,6,7 have reported a much higher prevalence (up to 70%) of short stature: closer to the figure that we see in most centers in India.

It is now well accepted that the etiology of endocrinopathies in these patients is multi-factorial. Hemosiderosis is the predominant cause, but a number of other factors also influence the endocrine profile in these individuals. These include: liver damage due to viral infections, increased activity of the iron dependent protocollagen proline hydroxylase enzyme, chronic anemia and individual susceptibility to damage from iron overload. Another factor that seems to be important is the genotype in individual patients. The complexity, both in terms of etiology and management, can be appreciated when we look at, for example, the pathogenesis of growth failure in these patients. Factors which contribute to growth failure include:

  • Inadequate transfusions
  • Undernutrition
  • Iron-induced selective central hypogonadism
  • Interference of iron with the production of insulin-like growth factor 1
  • Impaired GH responses to GH- releasing hormone
  • Abnormalities in GH secretion
  • Abnormalities in the GH receptor itself
  • Hyposecretion of adrenal androgens
  • Delay in pubertal development
  • Zinc deficiency
  • Free-hemoglobin induced inhibition of cartilage growth
  • Intensive desferoxamine administration.

Most of the above listed factors are either a direct or indirect consequence of hemosiderosis and free radical oxidant stress. The anterior pituitary gland is particularly sensitive to free radical oxidative stresses. Magnetic resonance imaging shows that even a modest amount of iron deposition within the anterior pituitary can interfere with its function. Endocrine morbidity therefore can be reduced to a large extent by optimizing transfusion and chelation therapy.

Osteoporosis is a major problem in young adults with thalassemia. A number of studies8,9 have conclusively demonstrated a very high prevalence of low bone mass in these patients despite optimal management. In the study by Wonke et al8 51% patients had severely low bone mass and a further 45% had low bone mass. The three factors that seem to have a statistically significant association with severely low bone mass are male sex, lack of spontaneous puberty, and diabetes. In optimally managed patients, bone mineral density measurements do not seem significantly associated with haematological characteristics or treatment details. The severely low bone mass appears to be due to endocrine abnormalities. In contrast, untreated thalassemics characteristically have hematological causes of bone disease.

As the life span of these patients continues to steadily increase, it becomes more important to focus on the quality of life issues. To optimize quality of life, it is mandatory that these patients be followed up regularly by a multidisciplinary team geared up to prevent and manage these complications. Careful monitoring of growth and puberty is vital to detect abnormalities and to initiate appropriate and early treatment.

References:

  1. Olivieri NF, Nathan DG, MacMillan, et al: Survival in medically treated patients with homozygous β-thalassemia. N Engl J Med 1994, 331:574-578.
  2. Sanctis V D, Eleftherion A, Malaventura C. Prevalence of endocrine complications and short stature in patients with thalassemia major: An international multicenter study. Abstract of the paper presented at the 9th International Conference on Thalassemia & Hemoglobinopathies.
  3. Shamshirsaz AA, Bekheirnia MR, Kamgar M et al. Metabolic and endocrine complications in thalassemia major.
  4. Saka N, Sukur M, Bundak R, et al: Growth and puberty in thalassemia major. J Pediatr Endocrinol Metab 1995, 8:181-186.
  5. Italian Working Group on Endocrine Complications in Non-Endocrine Diseases: Multicenter study on prevalence of endocrine complications in thalassemia. Clin Endocrinal 1995, 42:581-586.
  6. Kwan EY, Lee AC, Li AM, et al: A cross-sectional study of growth, puberty and endocrine function in patients with thalassaemia major in Hong Kong. J Paediatr Child Health 1995, 31:83-7.
  7. Kwan EY, Lee AC, Li AM, Tam SC , et al. A cross-sectional study of growth, puberty and endocrine function in patients with thalassaemia major in Hong Kong. J Paediatr Child Health 1995; 31(2): 83-7.
  8. Jensen CE, Tuck SM, Wonke B et al: High prevalence of low bone mass in thalassaemia major. B J Haemat 1998, 103:911-915.
  9. Voskaridou E, Kyrtsonis MC, Terpos E,et al: Bone resorption is increased in young adults with thalassemia major. Br J Haemaol 2001, 112:36-41.
  10. Fuchs GJ, Tienboon P, Linpisarn S,et al: Nutritional factors and thalassemia major. Arch Dis Child 1996, 74: 224-7.

FUTURE OF PEDIATRIC ENDOCRINOLOGY IN INDIABack to Top
P Raghupathy (From CAPENEWS, 10.1, April 2006)


There is no doubt that we have crossed many a milestone in establishing pediatric endocrinology in India. But there are still many more miles to go…. If a genie came out a bottle, what do I wish for the future?

Post doctoral course in Pediatric Endocrinology
Pediatric endocrinology calls for specialized skills which a physician does not acquire routinely during undergraduate or postgraduate training. For those with a special interest in the subject, several centers on the model of the course at SGPGI, Lucknow, should be set up in the country.

Neonatal thyroid screening
A national screening program for congenital hypothyroidism (CH) is imperative in this country and should be freely available as a birthright for every baby born. The prevalence of congenital hypothyroidism being as high as nearly 1 in 1,000 live births as reported from some Indian studies emphasizes the need for immediate action.

Type 1 diabetes mellitus
All state health machineries must provide human monocomponent recombinant insulin free of charge to all children with T1DM on a nationwide basis. Basic insulins such as the intermediate and short acting forms will do. I feel it is a shame that in this day and age, a child with T1DM should die for want of insulin. Children with T1DM should be given the benefits of a 'handicapped' status and given preference in getting education, vocational skills and livelihood oppurtunities. Education and employment in the Government and private sectors should not be denied to individuals with T1DM. Health insurance for T1DM should be available.

Adrenal disorders
Children with congenital adrenal hyperplasia or Addison's disease should be awarded a 'handicapped' status. Investigations for diagnosis and monitoring of these conditions should be available in regional centers in the various states. Antenatal detection facilities for congenital adrenal hyperplasia must be made available for helping the parents of an affected child to be able to decide regarding future offspring.

Medical Insurance
A national medical insurance scheme to cover chronic endocrine conditions requiring lifelong therapy should be made available in order to ensure continuous lifesaving replacement hormonal therapy.

National policy on expensive therapy such as growth hormone
A system should be evolved by which deserving poor children are awarded treatment with expensive medications such as GH to help them lead a normal and productive life. Subsidies may be provided proportionately for those with means.

Use of Media
Endocrine conditions are chronic and need lifelong therapy with highly gratifying results in those who are regular and compliant. Societal attitude towards these handicapped individuals may be changed positively with the help of the media. For example, public awareness of the usefulness of neonatal thyroid screening; the simple, cheap and effective treatment for CH; the irreversible brain damage caused by lack of treatment and such useful health guidelines may need to be constantly provided by the media. It needs to be emphasized repeatedly that these illnesses such as hypothyroidism andT1DM are not contagious. Movies, plays and stories portraying superstitions, traditions and customs which have a positive impact must be encouraged to gain the confidence of the public, while those with a negative impact such as indigenous herbal treatment for T1DM should be actively discouraged.

Schools must have 'personal hygiene' as part of the curriculum where useful evidence-based information can be provided for healthy living. A school health program will be highly beneficial. Promotion of breastfeeding, balanced diet, avoidance of junk food, usefulness of regular physical activity, regular growth monitoring, and awareness of pubertal changes.

Do all these sound like distant dreams? Finally, if a genie were to come out of a bottle and be willing to grant me one
wish, I would certainly opt for a magic wand for reversing childhood obesity.

CHRONIC ILLNESS: ROLE OF THE DOCTOR ALONG WITH THE CAREGIVERBack to Top
Ruchika Mehra Jain (From CAPENEWS, Vol 11.1, April 2007)


Twentieth century advances in medicine have benefited both children and adults. As more children escape or survive life threatening diseases, they run the risk of suffering from chronic illnesses. The burden of chronic illness is not limited to the child alone: there is significant psychological, social and economic impact and burden on the family. Numerous investigators have documented that children with chronic illness suffer more emotional, behavioral, and educational difficulties (Thompson et al, 1993; Wallander & Varni, 1998). Some of the many factors that make the child vulnerable to psychological disturbances include:

  • the child's external health locus of control,
  • maladaptive coping styles, and
  • poor caregiver psychological adjustment.
Amelioration of these psychological responses within the matrix of the doctor-patient relationship is better for overall and long-term outcome. Ideally, every physician needs to address, in every consultation, the biological, psychological and social components of "physical" symptoms, and therefore of chronic physical illnesses, without caring for one component at the expense of the others (Madhu Sridhar 2001). The help of trained professionals can be taken to help meet these psychological needs. However, our poor infrastructure makes it impossible to have the psychological needs of the patients catered to, so it becomes crucial for the primary pediatrician to address the psychological needs of the patient/ family along with the biological interventions required, in order to improve the welfare of the patient and family. Unfortunately, while there has been a lot of research in western countries, there is dearth of literature in the field of such interventions for parents and caregivers of such children in the Indian context. More importantly, communication of the results of these studies to specialists looking after children is largely neglected. Till date, during the training our pediatricians undergo mental health is neglected, cure receives more emphasis than care, and when cure is not possible (as with chronic illnesses), they flounder and are left with few options.

The impact of changes in patient behavior has been observed in several disorders, the most significant being seen in diabetes, ischemic heart disease, airway disease and hypertension (Oldenberg 1985; Johnson 1992). For example, a 70% reduction in visits to the doctor was seen in a chronically ill population following a 10 week biofeedback and stress management program (Kurtz, 1990). As doctors become more aware of the psychological components of physical illness, they realize that a counseling/ behavioral medical intervention can make an impact, which translates into more effective care. Just the process of carefully listening to the patient/ family has also been found to permit the development of trust and enhance the quality of life and well being of the patient (Radha Madhvi, 2001) Some important reasons for focusing on children with chronic illness and their family members are that along with medical care the family must have:

  1. A developmentally appropriate understanding of the illness (both for the child and immediate family),
  2. Compliance with treatment regimens,
  3. Integration of the illness into family life, including a balance between the needs of other family members,
  4. Successful adaptation to important systems, such as the school, peers and the hospital,
  5. Mastery of anxiety and fears related to the illness and its management (Koplewicz, 2001).
  6. These pressing psychological, developmental, and social needs have spurred the development of psychosocial interventions to address mental health problems and maximize the functioning of children and families as productive members of society. It has been shown that even an intervention as simple as listening to the patient and family members can help in reducing their psychological disturbances. Other beneficial interventions include:
    1. Psycho-education: One of the most important components is giving family members complete information about the illness- cause, course, treatment and long-term effects. This information should be honest and appropriate, and given in a form that it is understandable to them, keeping factors such as age and cognitive ability in mind.
    2. Developmental changes: Children move from the more dependent state of childhood to the more independent state of adolescence. These changes affect their disease management in various ways, sometimes detrimental, e.g. issues with body image, refusal to take medication or make lifestyle modifications, etc. The pediatrician is aware of the phases of normal adolescent development. He/ she must take these into account, make the family aware of them and help the patient and family accept and cope with these changes and their impact. Strategies include:
      1. Encouraging adolescents to share their ideas and concerns with healthcare professionals,
      2. If the illness reaches an unstable state due to non-compliance, encouraging discussion of what happened rather than reprimanding non-compliance.
      3. Teaching and encouraging use of problem-solving skills related to the illness. Ask questions such as: "What do you think you would you do if……?" or "What do you think would happen if……..?" Encourage adolescents to ask you the same kinds of questions.
      4. Seeking mental health services when:
        1. the adolescent seems overwhelmed with emotional issues related to living with a chronic illness.
        2. a pattern of non-compliance continues.
        3. the adolescent's development regresses, overly dependent behavior continues, and/or the adolescent withdraws from or gives up interest in age-appropriate activities.
  7. Skill training- The pediatrician has to ensure that the child and family have mastered the skills required for disease management. The family would benefit from finding out from the doctor the changes likely to be seen in the child as a result of the illness.
  8. Socio-economic support- is often crucial, since chronic illnesses tend to be expensive and emotionally draining. Thus if the doctor can put the family in touch with social support groups which help in getting available resources, give an opportunity to interact with others suffering from the same condition, and share the emotional burden, it would be very beneficial.
  9. Family therapy and group work- for the parents regarding their role as caregivers. The pediatrician should help the family internalize the roles and responsibility of each member in handling the situation: this would make for a better family environment:
    1. Each family member should be actively involved in the care of the child and aware of the disease to the extent that age allows. This helps because certain active changes have to be made in the family due to the child's condition.
    2. The patient and family should be encouraged to become self advocates in obtaining information and identifying resources.
    3. Contingency management plans should be formulated in case the family faces difficulties.
    4. The family should be encouraged to make the life of the sick child as close to normal as possible. This would make the child feel active and involved rather than isolated from peers and resentful. e) The family should be constantly counseled that the child should not be made to feel he/ she is a burden or a cause for disturbance for the family.
    5. The family should be encouraged to spend enjoyable quality time together.
    6. The parents and other elders must be reminded to pay attention to the other siblings also so that they do not feel neglected.
A bio-psycho-social approach to helping patients with chronic illness needs medical practitioners to involve families in their treatment of illness and expand their perspective from an individual medical model to a social systemic model. (Madhu Sridhar 2001).

DEVELOPMENTAL ORIGINS OF HEALTH & DISEASE (DOHaD)Back to Top
Gurmeet Singh (From CAPENEWS, Vol 11.3, December 2007)


Poor fetal growth and small size at birth have been shown to be followed by increased risk of coronary heart disease, stroke, hypertension, type 2 diabetes and osteoporosis. This led to the hypothesis that these disorders originate through unbalanced nutrition in utero and during infancy. The DOHaD concept is bringing new insights into the pathogenesis of disease. There is a range of experimental models for investigating the underlying biological mechanisms, and DOHaD is now a burgeoning research area in basic and clinical sciences, involving scientists from many backgrounds. The DOHaD Society aims to promote cohesion and shared knowledge between groups working in different specialties and different countries. Their Congress brings together a wide range of specialists from all over the world, including endocrinologists, cardiologists, perinatologists, gynecologists, pediatricians, geneticists, epigeneticists, public health physicians, fetal physiologists, scientists specializing in animal experiments…..

The 5th International Congress held in Perth, Australia (6-10 Nov 2007), like previous DOHaD conferences, had a great deal of information on a wide range of topics such as "Why does the early life environment have echoes for the rest of our life?", "Growth in early childhood: the catch-up dilemma", "The epigenetic interface between genes, the environment and disease", "Early interventions in pregnancy" and "Early nutrition and long-term cardiovascular health". The 6th International Congress is in Santiago, Chile, from 19th-22nd November, 2009.

Epigenetics, also called the hidden influences on genes, adds a whole new layer to genes beyond DNA. It proposes a control system of switches that turn genes on or off –and suggests that things people experience, like nutrition and stress, can control these switches and cause heritable effects in humans. DNA methylation, histone acetylation, and RNA interference, and their effects in gene activation and inactivation, are increasingly understood to be more than "bit players" in phenotype transmission and development. During the meeting, complex experiments in animals designed to elucidate these mechanisms and their effects were described. With specific emphasis on developing countries, there were discussions on epigenetic mechanisms as the basis of programmed changes and the transgenerational effects of environmental conditions, on the effect of postnatal catch-up growth on later life events, and finally whether there was enough evidence on which one could act.

Earlier evidence came mostly from the developed world. More recently, large cohort studies from developing countries, which have the largest numbers of low birth weight (LBW)babies, are also providing the evidence for the effectiveness of possible interventions to reduce LBW. The main cohorts are from Pune and Delhi in India; the Pelotas (Brazil)birth cohorts, 1882 and 1993; and the 2003 birth cohorts from Latin and Caribbean countries. The Pune Maternal Nutrition Study has studied the effects of maternal nutrition on offspring size and body composition. Compared to babies born in UK during a similar period, Indian babies were smaller but with a higher fat percentage. A Pune birth cohort showed increased insulin resistance at 8years of age. These differences, mediated by the state of the intrauterine environment, might explain partly the increasing propensity of Indians to develop diabetes.

The adverse effects of accelerated postnatal growth led to questioning of the current practice of "fattening up" babies who are born small. Interestingly, it has now been shown that "catch-up growth" in the first two years of life is mainly due to increase in lean body mass, which not only gives a survival advantage, but is also necessary for the development of organ systems, especially the brain. In contrast, a similar acceleration in growth after 4years of age is mainly due to an increase in fat mass, with several adverse metabolic consequences. Recent evidence also shows that improvements in birth weight are difficult to achieve once pregnancy is established. Ensuring a mother who is healthy preconception is a more effective means of ensuring a baby with a normal birth weight.

Evidence also suggests that improving the health of women of child-bearing age to prevent LBW babies, providing adequate nutrition to allow catch up growth in LBW infants till 2 years of age, and preventing the development of obesity after 4 years of age should help prevent the epidemic of obesity and insulin resistance occurring globally, especially in the developing world.