Letters to the editor

satisfactory condition. He was discharged home to continue the dressing of his wound at the PHCC. He defaulted on Discussion
Salmonella arizonae Infection from Snake Bite
Reptiles are the main natural reservoir of S. arizonae but To the Editor: Snakes, lizards, terrapins and other reptiles humans, poultry and other animals have also developed are the main natural reservoir of Salmonella arizonae (S. infection from this organism.1 Nine out of ten reptiles shed arizonae).1 Several investigators have reported the fact that salmonellae in their feces, and attempts at their elimination snakes and other reptiles harbor and transmit S. arizonae to and treatment have largely failed. In the United Kingdom, humans.2-5 Therefore, whenever and wherever human the Chief Medical Officer of the Department of Health has infection due to this organism occurs, it should trigger warned that owners of snakes, lizards, terrapins and other suspicion of a possible connection with reptiles as well as reptiles are at the risk of contracting salmonella from their poultry and egg products. In areas of Saudi Arabia where pets.6 In a recent report, the Public Health Laboratory snakebite is common, deliberate attempts should be made to Services showed an increase in salmonella cases associated exclude secondary bacterial infection due to S. arizonae, with exotic pets in children and infants. The report noted which could be fatal. Our report highlights a case of S. that in the previous two years (1999 and 2000), 13 people, arizonae wound infection in a Saudi boy bitten by a snake. including a three-week-old baby who died, had contracted Reports of S. arizonae infection in cases of snakebite2 A 10-year-old Saudi boy presented at our Emergency and in cancer patients and HIV-positive patients following Department in November 2000 with a history of snakebite consumption of snake powder capsules as a so-called “folk on the dorsum right big toe, having been referred from a remedy” have been reported by many investigators.7-10 The primary health care center (PHCC), where he received clinical spectrum of S. arizonae infections varies and may polyvalent anti-snake venom (equine serum) and a booster include benign gastroenteritis, enteric fever and septicemia, dose of tetanus toxoid. He did not develop any systemic localized infection in diverse organ sites such as the brain, manifestation of the complication of snakebite, but bone, liver, lung, joints and gall bladder, which may or may subsequently had abscess at the site of the bite over the big not be a sequel to septicemia. Well-documented cases of toe, for which he was referred to the Asir Central Hospital osteomyelitis caused by S. arizonae have been reported.11 However, our patient did not develop osteomyelitis of the On admission, the patient was found to be healthy- affected toe, possibly because the infection was detected looking and afebrile, with an abscess over the dorsum of the early and managed appropriately. We wish to draw the first metacarpophalangeal joint of the right big toe. At the attention of clinicians and laboratory staff to the possible center of the abscess was a black penetrating wound mark presence of this organism in cases of wounds caused by (caused by the fangs of the snake) surrounded by an bites of reptiles, especially snakes, both wild and domestic. induration and edema. The movement of the joint was limited, and a diagnosis of septic arthritis of the C.S.S. Bello, FRCPath
metacarpophalangeal joint with overlying abscess was S. Singh, MD
made.
A. Al-Waley, BSc
After preliminary investigations, the abscess was M. Hyde, RN, RM
drained and the pus was sent for Gram’s stain, culture and M.R.N. Khan, FRCS, MSc
sensitivity. The patient was admitted and put on intravenous cefuroxime and amikacin 6 hourly pending culture results. X-ray of the affected toe revealed no bony involvement. Results of the other investigations carried out showed PT of 19 (control 17), APTT 34 (control 30), ESR 22 mm/hour, References
urea 41, creatinine 0.7, RBS 96, Na 131, and K of 3.4. 1. Enterobacteriaceae. In: Koneman EW, Allen SD, Janda WM, Pus from the infected toe yielded pure growth of S. Schreckenberger PC, Winn WC Jr, editors. Color Atlas and arizonae which was sensitive to ampicillin, augmentin, Textbook of Diagnostic Microbiology. 5th edition. New York: cefuroxime, cefotaxime, ceftriaxone, chloramphenicol, gentamicin, amikacin, co-trimoxazole, and imipenem. S. 2. Buck JJ, Nicholls SW. Salmonella arizonae enterocolitis acquired by an infant from a pet snake. J Pediatr Gastroenterol Nutr 1997;25: arizonae was identified using API 20E strip (BioMerieux Vitek Inc., Missouri, USA). After the laboratory report, the Sanyal D, Douglas T, Roberts R. Salmonella infection acquired from patient was reviewed and found to be stable and in reptilian pets. Arch Dis Child 1997;77:345-6. Annals of Saudi Medicine, Vol 21, Nos 5-6, 2001 4. Centers For Disease Control and Prevention. Reptile-associated less than 0.03 mU/L. Radiological examination of her spine salmonellosis: selected states 1996-1998. JAMA 1999;282:2293-4. Oros J, Rodriguez JL, Herraez P, et al. Respiratory and digestive lesions caused by Salmonella arizonae in two snakes. J Comp Pathol generalized severe osteopenia. Dual energy x-ray D.H. Chief Medical Officer. “Salmonella Warning.” Biomedical absorptiometry (DEXA) scan showed a significant loss of bone marrow density (BMD) in the lumbar spine as well as Fainstein V, Yancey R, Trier P, Bodey GP. Overwhelming infection the left hip. Other results included: PTH 70 ng/L (n=10-65), in a cancer patient caused by Arizona hinshawii: its relation to snake pill ingestion. Am J Infect Control 1982;10:147-8. Ca 2.29 mmol/L (2.25-2.62), PO4 1.77 mmol/L (1.07-1.71), Babu K, Sonnenberg M, Kathpalia S, Ortega P, Swiatlo AL, Kocka alkaline phosphatase (ALP) 279 IU/L (160-500), and 25- FE. Isolation of Salmonellae from dried rattle snake preparations. J HO vitamin D of 52 pmol/L (N=25-90). She was started on methimazole 20 mg daily with Ca 300 mg/kg/d and Vitamin 9. Centers For Disease Control. Arizona hinshawii septicemia associated with rattle snake powder. Morbid Mortal Wkly Rep D supplements (1.25 dihydroxy-cholecalciferol, 0.25 mcg/d), and was encouraged to undertake active exercise. 10. Waterman SH, Juarez G, Carr SJ, Kilman L. Salmonella arizonae She showed significant clinical improvement in terms of her infections in Latinos associated with rattlesnake folk medicine. Am J weakness and tremors. Compliance was a major problem. 11. Keren DF, Rawlings W, Murray HW, Leonard WR. Arizona Her Ca, phosphorus and alkaline phosphatase were repeated hinshawii osteomyelitis with antecedent enteric fever and sepsis. Am once and were all normal. She was subsequently lost to follow-up for more than a year. A repeat DEXA scan was Osteopenia and Osteoporosis in Two Children with
Graves’ Disease
A 14-year-old boy presented with neck swelling, To the Editor: The adverse effects of long-standing polyphagia, weight loss, increased sweating, nervousness untreated hyperthyroidism on the bone were first described and insomnia for a period of three weeks. He had previously in 1891 by von Reckling Hausen in a 29-year-old woman been healthy and was not on any medications. His normal who died after five years of hyperthyroidism. The changes diet was rich in calcium (dairy products and vegetables), described were predominantly those of bone destruction, and he was active and athletic. Physical examination with increased osteoclastic and osteoblastic activity, revealed an anxious, thinly built boy (37.7 kg, below the 3rd especially in cortical bones, causing high turnover centile). He had exophthalmus, lid lag and retraction. The osteoporosis and fractures. These changes have been thyroid was diffusely enlarged with positive bruit. His hands described extensively in adults, but data in children are were warm, sweaty and tremulous. His heart rate was lacking. We report two cases of Graves’ disease in children 110/min., and blood pressure was 140/80. He was who had significant osteopenia and osteoporosis at diagnosed with Graves’ disease and was started on diagnosis. methimazole 30 mg daily, as well as propranolol. Thyroid function test showed a thyroxine level of 194 nmol/L, free thyroxine of 76.1 pmol/L and TSH of less than 0.01 mU/L. The patient was an 11-year-old Libyan girl who The thyroid scan was consistent with Graves’ disease. Initial presented with low back pain after sustaining a fall on her DEXA scan showed significant osteopenia with calcium back a day earlier. For the following two weeks, she levels of 2.44 mmol/L, ALP of 467 U/L and phosphate of complained of generalized weakness, shakiness of her hands 1.8 mmol/L. He was continued on methimazole for two and a protrusion of her left eye. There had also been a years, during which his thyroid function was monitored noticeable weight loss over the previous few months. Her regularly. He went into remission after 18 months. The past medical history was significant for congenital methimazole dose was reduced, and finally stopped after six strabismus, for which she had repeated surgeries that had months. His Ca, phosphorus and alkaline phosphatase were resulted in significant proptosis of the right eye. She had repeated at remission and were all normal. He is currently had speech delay since early childhood, which worsened seven months into remission, and an assessment of his with the recent tremors and weakness. On physical BMD has shown no evidence of osteopenia. examination, she looked severely underweight (19 kg, below the 3rd centile), with a heart rate of 130/min., and Discussion
blood pressure of 122/65 mm Hg. Her hands were warm, with obvious tremors bilaterally, and the eyes were Our patients clearly demonstrate that children with proptotic, especially on the right side. She had significant Graves’ disease can have a significant reduction in their kyphosis and tenderness on the thoracolumbar spine. A BMD at diagnosis. Although this has been well established clinical diagnosis of Graves’ disease was confirmed by in adults,1,2 only a few studies have been reported in biochemical findings of FT children.3,4 Our first patient had decreased BMD in the Annals of Saudi Medicine, Vol 21, Nos 5-6, 2001 lumbar spine and the hip, although for the hip there was no Allain TJ, McGregor AM. Thyroid hormones and bone. J Endocrinol age-matched control. The second patient had a low BMD in Lucidarme N, Ruiz JC, Czernichow P, Leger J. Reduced bone the lumbar spine as well, but there was no age-matched mineral density at diagnosis and bone mineral recovery during control either. These results are in accordance with earlier treatment in children with Graves’ disease. J Pediatr 2000;137:56- Lucidarme et al.3 have demonstrated that children with Leger J, Czernichow P, Garabedian M, Brauner R, Rappaport R. Osteopenie grave chez deux jeunes enfants atteints d’hyperthyroidie. Graves’ disease have significant reductions in BMD at presentation, and that femoral and lumbar spine BMD are Wakasugi M, Wakao R, Tawata M, Gan N, Koizumi K, Onaya T. significantly lower than expected for age and sex. Leger et Bone mineral density in patients with hyperthyroidism measured by al.4 reported the case of two children aged two years who dual energy x-ray absorptiometry. Clin Endocrinol 1993;38:183-6. Greenspan S, Greenspan F. The effect of thyroid hormone on skeletal presented with Graves’ disease and severe bone integrity. Ann Intern Med 1999;130:750-8. demineralization. One had spontaneous fractures and 7. Linde J, Friis T. Osteoporosis in hyperthyroidism estimated by collapse in the lumbar vertebrae. The second had complete photon absorptiometry. Acta Endocrinol 1979;91:437-48. resolution of his osteopenia after attainment of 8. Uzzan B, Campos J, Cucherat M, Nony P, Biossel P, Perret G. Effects on bone mass of long term treatment with thyroid hormones: euthyroidism. This condition has been reported in adults,7,8 a meta-analysis. J Clin Endocrinol Metab 1996;81:4278-89. although the degree of recovery of osteopenia has been 9. Siddiqi A, Burrin J, Noonan K, James I, Wood D, Price C, Monson variable. Lucidarme et al.3 also reported significant gains in J. A longitudinal study of markers of bone turnover in Graves’ femoral (P=0.001) and lumbar spine (P=0.02) BMD during disease and their value in predicting bone mineral density. J Clin treatment, and none of his 11 patients showed osteopenia. 10. Rosen C, Alder R. Longitudinal changes in lumbar bone density Our first patient had normal Ca, ALP, 25 OH Vit D and among thyrotoxic patients after attainment of euthyroidism. J Clin PTH, and the second had normal Ca and ALP. This is in agreement with the study by Leger et al.4 Siddiqi et al.9 11. Saggese G, Bertelloni S, Baronelli G, Costa S, Ceccarelli C. Bone reported a rise in the ALP coinciding with euthyroidism at mineral density in adolescent females treated with L-thyroxine: a longitudinal study. Eur J Pediatr 1996;155:452-7. 4-8 weeks, and he attributed that to continuing bone formation. The mean serum OC level was increased in Primary Pulmonary Alveolar Proteinosis: A Case
Leger’s study in the children with hyperthyroidism Report and a Review of the Literature
compared with that in the control group, however, no correlation was found between OC levels and the individual To the Editor: We read with interest the article of Wali et BMD and the thyroid hormone levels. Similar findings were al.1 We have also encountered two similar cases of reported in adults.1,8 The effect of Ca, Vit D pulmonary alveolar proteinosis (PAP) with widespread supplementation and physical activity needs to be evaluated. appearance of ground glass opacities (Figures 1 and 2), In conclusion, our patients, as well as those recently involving two adults aged 43 years. Diagnoses were made reported, show that Graves’ disease can be associated with with bronchial alveolar lavage fluid. In one case, the significant bone demineralization at presentation. The diagnosis was difficult because of pulmonary changes are reversible as euthyroidism is achieved with hemosiderosis. The cases have been followed for several treatment. This was clear from the literature,3 as well as years. Garcia Rio et al.2 have published a series of six cases. from our second case. Whether we need to do a BMD test In one patient, pulmonary tuberculosis appeared three in all children presenting with Graves’ disease is still to be months after the diagnosis of PAP. Both diseases decided since the effect is reversible, and finding age- disappeared with anti-tuberculous treatment. matched controls for BMD in the young age group has not Jean-Claude Hoeffel, MD
C.C. Hoeffel, MD
Dr. Majedah Abdul-Rasoul, MD
P. Fornes, MD
Dr. Bader Abdul-Kader, MD
Dr. Ahmed Al-Haj, MD
References
Wali SO, Samman YS, Altaf F, Abdulla L, Krayem AB, Alyafi WA. Primary pulmonary alveolar proteinosis: a case report and a review References
of the literature. Ann Saudi Med 2000;20:274-8. Garcio Rio F, Alvarez-Sala R, Caballero P, Prados C, Pino JM, Lee M, Kim S, Lee M, Cho B, Lee H, Koh C, Min H. Negative Villamor J. Six cases of pulmonary alveolar proteinosis: presentation correlation between the changes in bone mineral density and serum of unusual associations. Monaldi Arch Chest Dis 1995;50:12-5. osteocalcin in patients with hyperthyroidism. J Clin Endocrinol Annals of Saudi Medicine, Vol 21, Nos 5-6, 2001 issue. Child abuse is the maltreatment of a child by an adult FIGURE 1. Extensive bilateral lesions (Pr Anthoine). in a way which is unacceptable in a given culture at a given time.3 Child abuse is subdivided into physical, sexual, and FIGURE 2. Predominantly right-sided lesions (Pr Bernadac). emotional abuse and child neglect.4 This sociomedicolegal condition is very common in the West. In the US, an estimate of 1.6%-14% has been reported, out of which 4000 children die annually.5-7 In the UK, 4% of children less than To the Editor: I would like to thank Dr. Hoeffel and 12 years suffer from child abuse, 1 in 10,000 dies, and the colleagues for their comments and interest in the said incidence is increasing.3 We are quoting these records just article. I wish to comment on the diagnosis of alveolar for comparison with the incidence of child abuse in Saudi proteinosis by bronchial lavage cytology only and on the Arabia, where it is a newly emerging or a newly identified response of Dr. Hoeffel’s patient to anti-tuberculosis entity.8 Only a few case reports are available in the Saudi medication only. As mentioned in our review, literature,9-13 and the total number of cases is very low.11 bronchoalveolar lavage (BAL) cytology alone has been The letter by Maziak2 commenting on the reports by shown to be a reliable tool in confirming the diagnosis of Elkerdani et al.1 and Al-Ayed11 focused on the social and alveolar proteinosis in the right clinical setting.1,2 This is legal links of child abuse, especially the role of polygamy as again in keeping with Dr. Hoeffel’s cases. More recently, a risk factor. We have some reservations on this point. First, the diagnosis of pulmonary alveolar proteinosis (PAP) has we agree with the author that anecdotal reports do not fit in been performed by the demonstration of autoantibodies scientific documentation, and that the conclusions based on against GM-CSF, although this appears to be highly them are erroneous and misleading. Unfortunately, he did so sensitive (further confirmation studies are needed) in cases in considering polygamy as a risk factor for child abuse. A of idiopathic PAP, but less sensitive for secondary PAP.3 few case reports of child abuse in a country where To date, the only successful treatment for PAP is by a whole polygamy is common is statistically insignificant. To long lavage regardless of whether it is a primary or counter this, one may quote other anecdotal reports where secondary disorder.1 As is known, the natural history of the polygamy has been shown to save some compromised disease is variable, which makes the evaluation of the children from a psychologically unstable mother or from efficacy of any specific treatment difficult. Therefore, it is poverty-related child abuse.7 Shall we conclude then that not clear whether the reason for the recovery of Dr. polygamy prevents child abuse? Certainly not. However, the Hoeffel’s patient was spontaneous or related to the response fact that child abuse is rare in Saudi Arabia, where to anti-tuberculous drugs. On the other hand, patients with polygamy is common, may itself indicate a reverse proof PAP are susceptible to chest infection, and hence the against this claim. Yet, a comparative study between the appearance of pulmonary tuberculosis may represent a prevalence of child abuse in polygamous and monogamous superadded infection, rather than a triggering factor for families is required after fixing the other etiological factors in both research groups. For example, in the report of Elkerdani et al.,1 polygamy was not possibly the only acting Siraj O. Wali, MD, FRCPC, FACP, FCCP
factor to hang the case on. There was the mother herself who was quite young (16 years old), the large difference in age between the mother and the husband, and the illiteracy of both parents. Also there was no elucidation of the mental and psychological status of the mother. It is hard not to attribute some criminal intention and aggression from a References
natural mother, even if she is wife No. 10, against her defenseless infants without some psychotic defect Wang BM, Stern EJ, Schmidt RA, et al. Diagnosing pulmonary alveolar proteinosis: a review and an update. Chest 1997;111:460. underlying it. We cannot blame one likely factor in the 2. Martin RJ, Coalson JJ, Rogers RM, et al. Pulmonary alveolar presence of other factors if we are neutral and scientific. proteinosis: the diagnosis by segmental lavage. Am Rev Respir Dis Second, in a rough review of 23 Saudi cases of child abuse,11,13 polygamy was found in two families only, noting Kitamum T, Urchida K, Tanaka N, Tsuchiya T, et al. Serological diagnosis of idiopathic pulmonary alveolar proteinosis. Am J Respir that some of these children belonged to unknown mothers. Third, child abuse is a multifactorial disorder.14 A review of the literature shows that polygamy has not been reported as Fatal Child Abuse in Two Children of a Family: The
a risk factor or predisposing factor for child abuse.3 On the Alleged Role of Polygamy
contrary, it has been shown that most child abuse cases have been perpetrated by a family member, usually a parent or To the Editor: We would like to thank Dr. Elkerdani et al.1 the mother’s boyfriend.15 There is no available study on the and Dr. Maziak2 for tackling such a sensitive medicolegal effect of polygamy. Fourth, the author may have been Annals of Saudi Medicine, Vol 21, Nos 5-6, 2001 influenced by the Western experience of polygamy, which Maziak W. Fatal child abuse in two children of a family (letter). Ann is quite different from what happens in Saudi Arabia. Meadow R. ABC of child abuse: epidemiology. BMJ 1989;298:727- Polygamy or even “multi-polygamy” is widely practiced in the West, but because it is illegal, it is practiced in the dark, Wissow LS. Child abuse and neglect. N Engl J Med 1995;332: with no responsibility towards the child that comes out of that relationship. They rather try to get rid of the undesired US Department of Health and Human Services. Study findings: Study of national incidence and prevalence of child abuse and pregnancy. So, child abuse there might have a strong link neglect. Washington, DC: Children’s Bureau, National Center of with such illegal polygamy, especially in a situation where the so-called child protection agencies are themselves 6. Kempe CH, Helfer RE. Stress and child abuse in the battered child. ineffective. Thus, the legality and official acceptance 3rd edition. Chicago: University of Chicago Press, 1980;36-104. Moy JA, Sanchez MR. The cutaneous manifestation of violence and change completely the prognosis of polygamy and the children born out of it. Fifth, any legal rule or official 8. Baeesa SS, Jan MM. The shaken baby syndrome. Saudi Med J 2000; regulation, including polygamy, may be abused when put in practice. This should be dealt with separately and 9. Al-Ayed IH. Munchausen syndrome by proxy: the emerging face of accordingly without undermining the rule itself. Sixth, even child abuse in Saudi Arabia. Saudi Med J 1998;19:781-4. 10. Al-Eissa Y. The battered child syndrome: does it exist in Saudi if we propose child abuse as an adverse side effect of polygamy, it should be weighed against the positive social 11. A1-Ayed IH, Shaikh JA, Qureshi MI. Patterns of pediatric effects and benefits of polygamy, which are beyond the emergency room visits at King Khalid University Hospital, Riyadh. scope of this letter. Seventh, it is completely unscientific to 12. Al-Jumaah A, Al-Dowaish A, Tufenkeji H, Frayh HH. Munchausen generalize the suggestion of child abuse on all Middle syndrome by proxy in a Saudi child. Ann Saudi Med 1993;13:469- Eastern countries when the author is commenting on child abuse in Saudi Arabia, especially when polygamy is not 13. Kattan H. Child abuse in Saudi Arabia: report of ten cases. Ann common in other Middle Eastern countries. 14. Kottmeier PK. The battered child. Pediatr Ann 1987;16:343-51. Citing polygamy as a cause of child abuse is unscientific 15. Kennedy CTC. Mechanical and thermal injury. In: Champion RH, without statistical studies. We should differentiate between Burton JL, Burns DA, Breathnach SM, editors. Rook/Wilkinson/ legal polygamy, which is practiced in Saudi Arabia to serve Ebling Textbook of Dermatology. 6th edition. London: Blackwell special social conditions, and the illegal polygamy widely practiced in the West which may be related to child abuse, and which places no responsibility towards the resulting offspring. The rare occurrence of child abuse in Saudi To the Editor: In response to the letter by Dr. Shelleh et al., Arabia, where polygamy is common, may help remove any I start by repeating the part of my published letter that suspected relationship between them. A rough review of 23 initiated the current debate. I said “it is astonishing that in cases of child abuse in Saudi Arabia showed that polygamy the two reports, the father was married to two women, and the involved mothers were the second wives, yet polygamy was not even mentioned as a possible cause of trouble in NB: We are commenting on the issue on principle only. both cases.” I think it is clear to the reader that I am not None of the authors of this article practices polygamy, so stating that polygamy is a risk factor, let alone the risk factor for child abuse, as Dr. Shelleh et al. accuse me of doing. Thus, a simple, correct reading of what I have written renders their rebuttal out of context and Hamdi Hassan Shelleh, MD
unnecessary. Any factor surrounding the case of interest, as Sarosh Ahmed Khan, MD
I have clearly stated in my letter, is a possible attribute to Hussni Al-Hateeti, Arab Board
the case until it is proven otherwise. The acknowledgement Latif Ahmed Khan, MRCP
by Dr. Shelleh et al. that the relationship between polygamy Abdul Fattah M. Saleh, MD (France)
and child abuse needs to be studied is a clear support of my Mohammed N. Qasabah, MD
view that this factor, along with others, should receive due consideration. The fact that only a few cases of child abuse Sheikh Mohammad Al-Askari
have been reported in Saudi Arabia compared to the large numbers in Western countries, as presented by Dr. Shelleh et al., does not mean that it is rare in Saudi Arabia References
compared to the West. Most developed countries have systematic tracking and surveillance systems for various Elkerdani AAF, Al-Eid WM, Buhaliqa AA, Al-Momani AA. Fatal public health problems, including child abuse. From the child abuse in two children of a family. Ann Saudi Med 1999;19: available medical literature, I am not aware of the presence of national tracking and surveillance systems for child abuse Annals of Saudi Medicine, Vol 21, Nos 5-6, 2001 in Saudi Arabia, which means that the assumption that it is In contrast to the statement by Shelleh et al. that rare compared to the West is at least premature. polygamy was the main focus of my letter, the reader can In the response of Dr. Shelleh et al., they repeatedly readily notice that my main concern was the failure of refer to what is scientific and unscientific, of course medical and law enforcement personnel involved in the accusing me of being unscientific and deprived of reported cases to intervene to protect the children and bring neutrality. I am just wondering where science is in their the perpetrators to justice, when they had multiple prejudiced, stereotypic description of the West as the land opportunities to do so. It was also a cry for the medical of shattered families, where sexual promiscuity is practiced community to take an active role in cases of child abuse, in the darkness. And where is science in their classification and in the case of current interest, to try to investigate the of legal polygamy as practiced in Saudi Arabia vs. the destiny of the remaining victim to protect him if he is still illegal polygamy practiced in the West, which is based alive. I am really disturbed by the fact that in the response solely on their personal opinion, and with complete of Dr. Shelleh et al., authored by six practicing medics and disregard to others’ legal and cultural specificities? In fact, a senior judge, there was not a single word about any steps in their rebuttal Dr. Shelleh et al. go as far as to object even taken by them or others to investigate the fate of the to my consideration of polygamy as a risk factor, which remaining unfortunate child, or to bring those responsible makes me wonder if all this is because I have trespassed on for these sad events to justice. Science is an objective study a taboo. For very mysterious reasons as well, they object to of life, yet better science is an objective study of life in the my plea for the establishment of child protecting agencies in face of unfavorable sociocultural circumstances. the whole Middle East to deal with cases of child abuse, which was advanced in the context of providing legal Wasim Maziak, PhD
bodies capable of taking active measures in cases of child
Institute of Epidemiology and Social Medicine In their rebuttal, Shelleh et al. presented arguments in favor of polygamy having nothing to do with child abuse, References
and based on the fact that of the 23 cases of reported child abuse in Saudi Arabia, only two were from polygamous 1. Maziak W, Asfar T, Kilzieh N, Mzayek F, Fouad MF. families, and that child abuse in Saudi Arabia is rare while Sociodemographic correlates of psychiatric morbidity among low-income women in Aleppo, Syria. Soc Science Med 2001 polygamy is common. For those obsessed with scientific objectivity, it seems strange to me that such arguments, 2. Maziak W, Asfar T. Physical abuse in low-income women in which could not pass by even the most lax science reviewer, Aleppo, Syria. J Fam Violence 2001 (in press). did not stimulate their critical faculties. On the other hand, I think it will be interesting for Dr. Shelleh and colleagues to Hb Bart’s Levels in Cord Blood in
know that recently, we have been able to show that women Hb H Disease: Follow-up of Cases with
of polygamous families in Syria were more likely to have “Probable” Hb H Disease
mental distress or to be physically abused, than women of monogamous families, after adjusting for other To the Editor: Hb Bart’s levels of 2%-8% in cord blood are sociodemographic factors such as age, age of marriage, seen in α-thalassemia minor, and it is widely accepted that education, economic status, etc.1,2 Hb Bart’s of >10% is diagnostic of Hb H disease.1,2 Annals of Saudi Medicine, Vol 21, Nos 5-6, 2001 TABLE 1. Red cell indices and Hb electrophoresis results in “probable” Hb H disease at birth and at 3 years of age. 4.62 95.9 25.3 69.3 16.1 – 24 59.9 – 5.37 83.6 24.6 69.3 11.7 – 19.1 69.2 T T=traces. However, it has been reported by Pembrey et al.3 that Hb molecular studies, cases with Hb Bart’s of 10%-20% in Bart’s of even up to 16% might be present in severe cases cord blood should not be labeled as Hb H disease, and such of α-thalassemia in Eastern Saudi Arabia. On the basis of samples should be followed up by repeat electrophoresis at the above reports, we had classified four newborns (Table 4-6 months of age to ascertain the correct phenotype. 1) whose cord blood Hb Bart’s ranged from 10% to 16.1% as “probable” Hb H disease. We now present their follow- References
up findings at about three years of age. Higgs DR, Vickers MA, Wilkie AOM, Pretorius IM, Jarman AP, Discussion
Weatherall DJ. A review of the molecular genetics of the human α- globulin gene cluster. Blood 1989;73:1081-108. In our study, only 1 out of the 4 cases of “probable” Hb 2. Higgs DR. Alpha-thalassemias. Baillieres Clin Haematol H disease was shown to have Hb H disease (Table 1, no. 4). Pembrey ME, Weatherall DJ, Clegg JB, Bunch C, Perrine RP. The patient developed pancytopenia and has been Hemoglobin Bart’s in Saudi Arabia. Br J Haematol 1975;29:221-34. dependent on transfusion for the last 2½ years. The patient Quadri MI, Islam SI, Nasserullah Z. Effect of alpha thalassemia on has been referred for bone marrow transplant. It is evident cord blood red cell indices and interaction with sickle cell gene. Ann that the majority of the newborns with Hb Bart’s of 10%- Jasim N, Al-Arrayed S, Gerard N, Ducrocoq R, Nagel RL, 16.1% do not develop probable Hb H disease and are cases Krishnamurthy R. Molecular basis of α-thalassemia in Bahrain. of severe α-thalassemia trait. Exceptionally high prevalence of the α-T Saudi mutation has been reported from Bahrain, which is geographically and ethnically closely related to M.I. Quadri, MD, PhD
Eastern Saudi Arabia.5 It is likely that our cases of severe Z. Nasserullah, CABP
thalassemia trait resulting in high Hb Bart’s have an Post Box 184 underlying compound heterozygosity for α-T Saudi mutation and some other deletional α-thalassemia. In the absence of Annals of Saudi Medicine, Vol 21, Nos 5-6, 2001

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