Copper and zinc concentrations in the breast milk of mothers undergoing treatment for Wilson’s disease: a prospective study

ObjectiveTo evaluate the concentrations of copper and zinc in the breast milk of mothers undergoing treatment for Wilson’s disease (WD) and clarify whether they can safely breast feed their infants.DesignThis was an observational and prospective study in an individual-based case series.SettingBreast milk samples were collected from participants across Japan from 2007 to 2018 at the Department of Pediatrics, Teikyo University in Tokyo. This was a primary-care level study. Clinical data were collected from the participants’ physicians.PatientsEighteen Japanese mothers with WD who were treated with trientine, penicillamine or zinc, and 25 healthy mothers as controls, were enrolled.Main outcome measuresWhey exacted from the milk was used to evaluate the distribution of copper by high-performance liquid chromatography-inductively coupled plasma mass spectrometry. Copper and zinc concentrations in the breast milk samples were analysed by atomic absorption spectrometry.ResultsCopper distribution was normal in the breast milk of mothers with WD treated with trientine, penicillamine or zinc. No peak was detected for trientine-bound or penicillamine-bound copper. The mean copper concentrations in the mature breast milk of patients treated with trientine, penicillamine and zinc were 29.6, 26 and 38 µg/dL, respectively, and were within the normal range compared with the value in healthy controls (33 µg/dL). Likewise, mean zinc concentrations were normal in the mature breast milk of patients treated with trientine and penicillamine (153 and 134 µg/dL, respectively vs 160 µg/dL in healthy controls). Zinc concentrations in the breast milk of mothers treated with zinc were significantly higher than those in control milk. All infants were born normally, breast fed by mothers undergoing treatment and exhibited normal development.ConclusionsOur results suggest that mothers with WD can safely breast feed their infants, even if they are receiving treatment for WD.

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Author response for "Ammonium tetrathiomolybdate in the decoppering phase treatment of Wilson's disease with neurological symptoms: A case series"

10.1002/brb3.1596/v3/response1 ◽

2020 ◽

Author(s):

Oriol De Fabregues ◽

Jaume Viñas ◽

Antoni Palasí ◽

Manuel Quintana ◽

Ignasi Cardona ◽

...

Keyword(s):

Wilson’S Disease ◽

Case Series ◽

Wilson's Disease ◽

Neurological Symptoms ◽

Author Response ◽

Ammonium Tetrathiomolybdate

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Review for "Ammonium tetrathiomolybdate in the decoppering phase treatment of Wilson's disease with neurological symptoms: A case series"

10.1002/brb3.1596/v1/review2 ◽

2019 ◽

Keyword(s):

Wilson’S Disease ◽

Case Series ◽

Wilson's Disease ◽

Neurological Symptoms ◽

Ammonium Tetrathiomolybdate

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Spectrum of cutaneous manifestations in Wilson’s disease in children in a tertiary care center in South India: a prospective study

International Journal of Contemporary Pediatrics ◽

10.18203/2349-3291.ijcp20174731 ◽

2017 ◽

Vol 4 (6) ◽

pp. 2060

Author(s):

Karrunya K. J. ◽

Sumathi Bavanandam ◽

Nirmala Dheivamani ◽

Vijayabhaskhar C. Reddy ◽

Manoharan K.

Keyword(s):

Portal Hypertension ◽

Skin Biopsy ◽

Prospective Study ◽

Wilson’S Disease ◽

Wilson's Disease ◽

P Value ◽

Indian Children ◽

Cutaneous Manifestations ◽

Mucosal Involvement ◽

A Prospective Study

Background: Wilson’s disease in children is the most common inherited disorder of copper metabolism in India with varied clinical manifestations secondary to deposition of copper in various organs including skin. Methods: The aim of the study was to study various cutaneous manifestations of Wilson’s disease in south Indian children. A prospective study for a period of one year was conducted at Dermatology and Pediatric Gastroenterology clinic in Government Rajiv Gandhi Hospital, Chennai. Clinical, demographic details, various cutaneous manifestations and results of skin biopsy were analysed. Results: Out of the total 30 children with Wilson’s disease, 93.3% had at least one cutaneous, mucosal, hair or nail findings but not statistically significant between naive and on treatment group. Cutaneous findings, mucosal involvement, hair changes and nail findings were observed in 90%, 36.7%, 46.7% and 60% respectively. Cutaneous findings were more common in liver disease (P value<0.05). Xerosis was the most common cutaneous manifestation seen in 63.3% patients, followed by pigmentation in 36.7% and infection in 30%. KF ring was noted in 53.3%. Icterus and pruritus were seen in one fifth of study children and majority had portal hypertension. Vascular changes like spider nevi, purpura and other features of portal hypertension were noted in 16.6% of study population. Trichomegaly was one of the common hair manifestation found in 9 (30% especially in children already on treatment (p value <0.05). There was no evidence of stainable copper on skin biopsy. Conclusions: The physicians should be aware of various cutaneous manifestations of Wilson’s disease in children.

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Liver Stiffness Assessed by Real-Time Two-dimensional Shear Wave Elastography Predicts Hypersplenism in Patients with Wilson’s Disease: A Prospective Study

10.21203/rs.3.rs-612351/v1 ◽

2021 ◽

Author(s):

Jiajia Wang ◽

Minxia Hu ◽

Qiang Zhu ◽

Lanting Sun

Keyword(s):

Risk Factors ◽

Prospective Study ◽

Real Time ◽

Shear Wave ◽

Wilson’S Disease ◽

Liver Stiffness ◽

Shear Wave Elastography ◽

Wilson's Disease ◽

Dynamic Monitoring ◽

Two Dimensional

Abstract Background To explore the value of liver stiffness assessed by two-dimensional real-time shear wave elastography (2D-SWE）in predicting the occurrence of hypersplenism in patients diagnosed with Wilson’s disease (WD). Methods A total of 90 WD patients were enrolled in this prospective study between May 2018 and December 2018. Clinical data and ultrasound imaging including 2D-SWE liver stiffness of WD patients as baseline data were collected. Patients were followed up for 24 months, or patients developed hypersplenism after enrollment. Risk factors for hypersplenism were determined using cox regression and receiver operating characteristic curve. Results Twenty-night (32.2%) patients were found developed hypersplenism. The age, the diameter of portal vein, and the liver stiffness were independent risk factors associated with hypersplenism in WD. The cutoff value of liver stiffness for predicting hypersplenism was 10.45 kPa, with sensitivity and specificity of 75.9% and 73.8%, respectively. When patients were divided into two groups according to liver stiffness ≥10.45 kPa or <10.45 kPa, the incidence of hypersplenism were 57.9% vs. 13.5% (P<0.001), and the median time between the enrollment and the development of hypersplenism was 15 months vs. 22 months (P<0.001) for the two groups, respectively. Conclusion The liver stiffness measured by 2D-SWE was a reliable predictor of hypersplenism in WD patients. Dynamic monitoring WD patients using 2D-SWE is crucial for the early diagnosis of hypersplenism.

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